[Senate Hearing 107-592]
[From the U.S. Government Publishing Office]



                                                        S. Hrg. 107-592



                BIRTH DEFECTS: STRATEGIES FOR PREVENTION

                      AND ENSURING QUALITY OF LIFE

=======================================================================

                                HEARING

                               BEFORE THE

                  SUBCOMMITTE ON CHILDREN AND FAMILIES

                                 OF THE

                    COMMITTEE ON HEALTH, EDUCATION,
                          LABOR, AND PENSIONS
                          UNITED STATES SENATE

                      ONE HUNDRED SEVENTH CONGRESS

                             SECOND SESSION

                                   ON



 EXAMINING PUBLIC HEALTH ISSUES RELATED TO BIRTH DEFECTS, FOCUSING ON 
         STRATEGIES FOR PREVENTION AND ENSURING QUALITY OF LIFE

                               __________

                             JULY 26, 2002


                               __________

 Printed for the use of the Committee on Health, Education, Labor, and 
                                Pensions


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          COMMITTEE ON HEALTH, EDUCATION, LABOR, AND PENSIONS

               EDWARD M. KENNEDY, Massachusetts, Chairman
CHRISTOPHER J. DODD, Connecticut     JUDD GREGG, New Hampshire
TOM HARKIN, Iowa                     BILL FRIST, Tennessee
BARBARA A. MIKULSKI, Maryland        MICHAEL B. ENZI, Wyoming
JAMES M. JEFFORDS (I), Vermont       TIM HUTCHINSON, Arkansas
JEFF BINGAMAN, New Mexico            JOHN W. WARNER, Virginia
PAUL D. WELLSTONE, Minnesota         CHRISTOPHER S. BOND, Missouri
PATTY MURRAY, Washington             PAT ROBERTS, Kansas
JACK REED, Rhode Island              SUSAN M. COLLINS, Maine
JOHN EDWARDS, North Carolina         JEFF SESSIONS, Alabama
HILLARY RODHAM CLINTON, New York     MIKE DeWINE, Ohio
           J. Michael Myers, Staff Director and Chief Counsel
             Townsend Lange McNitt, Minority Staff Director
                                 ------                                

                 Subcommittee on Children and Families

               CHRISTOPHER J. DODD, Connecticut, Chairman
JAMES M. JEFFORDS, Vermont           SUSAN M. COLLINS, Maine
JEFF BINGAMAN, New Mexico            BILL FRIST, Tennessee
PAUL D. WELLSTONE, Minnesota         JOHN W. WARNER, Virginia
PATTY MURRAY, Washington             CHRISTOPHER S. BOND, Missouri
JACK REED, Rhode Island              MIKE DeWINE, Ohio
                     Grace A. Reef, Staff Director
            Priscilla Hobson Hanley, Minority Staff Director


                            C O N T E N T S

                              ----------                              

                               STATEMENTS

                         Friday, July 26, 2002

                                                                   Page
Dodd, Hon. Christopher J., Chairman of the Subcommittee, opening 
  statement......................................................     1
Reed, Hon. Jack, a U.S. Senator from the State of Rhode Island, 
  opening 
  statement......................................................     4
Cordero, Jose F., M.D., Director, National Center on Birth 
  Defects and Developmental Disabilities, Centers for Disease 
  Control and Prevention, U.S. Department of Health and Human 
  Services, Atlanta, GA..........................................     5
    Prepared statement...........................................     7
Pote, Mr. Harold, President, Spina Bifida Foundation, New York, 
  NY; Dr. Nancy S. Green, Medical Director, March of Dimes of 
  Birth Defects Foundation, and Assistant Professor of 
  Pediatrics, Albert Einstein School of Medicine, New York, NY; 
  and Fred Liguori, Parent, Granby, CT...........................    17
    Prepared statements
        Harold Pote..............................................    19
        Nancy S. Green, M.D......................................    25
        Fred Liguori.............................................    32

 
 BIRTH DEFECTS: STRATEGIES FOR PREVENTION AND ENSURING QUALITY OF LIFE

                              ----------                              


                         FRIDAY, JULY 26, 2002

                                       U.S. Senate,
Subcommittee on Children and Families, of the Committee on 
                    Health, Education, Labor, and Pensions,
                                                   Washington, D.C.
    The subcommittee met, pursuant to notice, at 9:40 a.m., in 
room SD-430, Dirksen Senate Office Building, Hon. Christopher 
J. Dodd (chairman of the subcommittee) presiding.
    Present: Senators Dodd and Reed.

 OPENING STATEMENT OF HON. CHRISTOPHER J. DODD, A U.S. SENATOR 
                 FROM THE STATE OF CONNECTICUT

    Senator Dodd. The subcommittee will come to order.
    Thank you all for being here this morning. Let me just 
announce ahead of time that I think we are going to have one or 
perhaps two votes at 10 a.m., which will disrupt the hearing a 
little bit; but my hope would be to complete your work with us, 
Dr. Cordero, and since I do not have to vote immediately--I can 
vote after the vote starts--so we will have about 40 minutes or 
so. And then I will come back right after the vote, and we will 
continue the hearing. So just to announce ahead of time that we 
may have some disruption of the orderly flow of events.
    But I am very grateful to all of you for joining us, those 
of you who are in the audience today as well as those who will 
be testifying, and family members and friends who have come out 
for this hearing of the Senate Committee on Health, Education, 
Labor, and Pensions, and the subcommittee which I am very proud 
to chair, the Subcommittee on Children and Families, which I 
have been proud to be associated with during my entire service 
here in the United States Senate for more than 20 years.
    The title of today's hearing is ``Birth Defects: Strategies 
for Prevention and Ensuring Quality of Life.'' Today the 
Subcommittee on Children will examine two very crucial 
questions--first, how do we best prevent birth defects, and 
second, how do we best support those born with birth defects 
and the families and loved ones who work to assure their care.
    We should begin initially with an examination of the 
problems presented by birth defects. Birth defects are the 
leading cause of infant mortality in the United States and 
affect more than 150,000 infants born each year. In many cases, 
children with birth defects face a lifetime of disability. They 
are the leading cause of death in the first year of life. 
Sadly, the parents of one out of every 28 babies receive the 
terrifying news that their child has a birth defect.
    Both genetic and environmental factors can cause the more 
than 4,000 known birth defects. However, the causes of about 60 
percent of birth defects are currently unknown.
    We have a chart here on my left which will highlight some 
of the points I am about to make. Each and every day in the 
United States, more than 400 children are born with a birth 
defect. Tragically, in the same day, 17 infants will die due to 
a birth defect. Another 78 infants will lose their lives before 
they reach their first birthday because of a critical birth 
defect.
    The wonderful pictures that surround the dais today show 
children--Nicholas Liguori, of Granby, CT, whom I just met and 
who said, ``Hello, Senator Dodd''--I appreciate that, Nicholas; 
I told him I have a brother named Nicholas as well, so it is a 
great name--Nicholas is with us today and is the son of Fred 
Liguori, whom we will hear from shortly on the second panel. 
You are also looking at a picture of Gregory Pote, carrying the 
Olympic torch. We are proud to have you with us, Gregory. Greg 
is the nephew of another witness, Hal Pote, who will also 
testify on our second panel.
    These are children who are, thankfully, very much with us. 
It is because of people like Greg and Nicholas that we are here 
today to talk about this issue. So the question is not only how 
do we best prevent birth defects, but also how do we best 
support those born with birth defects and the loved ones who 
work so hard to provide for their care.
    I know that every parent here with us today will agree that 
there is no greater gift for a parent than that received upon 
the birth of a child. As we also know, with such a great gift 
comes great responsibility. However, for parents of a child 
born with a severe birth defect, such a great gift brings with 
it even more responsibility.
    Every parent wants the very best for their child. Parents 
of children with birth defects are clearly no different. The 
blessing that comes with the birth of a child is clearly no 
less joyous for parents of a special needs child. However, it 
is only reasonable to accept that parents of a special needs 
child may require more assistance in order to ensure that their 
children receive the supportive services that they require and 
deserve.
    I believe that as policymakers here in the United States 
Congress, we have responsibilities as well. Our responsibility 
should lead us to enact effective, helpful systems of care that 
not only prevent birth defects but also provide aid to those 
with birth defects so they can achieve all that their abilities 
allow.
    Thankfully, we have made progress. Since 1960, the number 
of infants dying due to birth defects has been cut by more than 
half, which is great news. We now know that there exist some 
simple measures that can greatly decrease the chances of birth 
defects. We must work to ensure that all women of childbearing 
age are aware that there are certain, often very easy steps 
that will help to ensure a safe and healthy pregnancy.
    For example, we know that a modest 400 microgram daily dose 
of the B vitamin folic acid could prevent 50 to 70 percent of 
all cases of spina bifida and other neural tube defects.
    While encouraging as these numbers may be, we also know 
that about half of all pregnancies are unplanned and that in 
order to be effective, women must be taking the required dose 
of folic acid before they get pregnant. And while studies 
indicate that the message stressing the critical importance of 
folic acid is currently being heard, we all know that behaviors 
do not change overnight. I believe that if we continue our 
educational efforts, the desired changes in behavior will 
occur, as evidenced by the fact that the number of women who 
smoke during pregnancy has dropped approximately 30 percent 
since 1989 alone. However, we must continue to work to spread 
the lifesaving messages that truly prevent birth defects.
    Efforts to prevent birth defects are tremendously worthy. 
In fact, they should be strengthened. And while the importance 
of birth defect prevention cannot be overstated, the fact 
remains that there is no one action that will prevent all birth 
defects.
    While progress has been made both with regard to birth 
defect prevention and efforts to better the lives of those 
living with birth defects, this is not to say that more cannot 
be done. In fact, Congress can and should do more.
    The Birth Defects Prevention Act of 1998 authorized the 
Centers for Disease Control and Prevention to conduct programs 
aimed at the prevention of birth defects through surveillance, 
research, and education. With the authority given by the BDPA, 
the National Center on Birth Defects and Developmental 
Disabilities at CDC provides funding to 28 States to develop, 
implement, and/or expand community-based birth defects tracking 
systems, programs to prevent birth defects, and activities to 
improve health services for children with birth defects.
    However, the BDPA's authorization ends this year. In fact, 
I look forward to working with my colleagues, both Democrats 
and Republicans, Senator Bond in particular, to reauthorize 
this important legislation and maintain our commitment to 
preventing birth defects and ensuring the quality of life for 
those living with birth defects.
    I am also encouraged by the efforts underway to create a 
National Spina Bifida Program. I believe that such a program 
could greatly improve our efforts to prevent the incidence and 
morbidity of spina bifida and other neural tube defects. A 
National Spina Bifida Program would also have the effect of 
greatly improving the quality of life of the more than 70,000 
people presently living with this often severely disabling 
birth defect.
    Thankfully, advances over the years in medical treatment 
such as the development of in utero surgery have allowed 
children with spina bifida to become adults living with spina 
bifida. I believe that a National Spina Bifida Program would 
provide vital resources to help these individuals lead full and 
rewarding lives.
    It is my hope that the discussion we are going to have this 
morning will provide us with an outline that will lead us to 
advances in both prevention and the development of helpful 
systems of care for those living with birth defects.
    So it is with great optimism that we begin today's hearing. 
Again, I want to thank all of our witnesses, particularly the 
families who have come down with Nicholas and Greg to be part 
of this program today to shed some light on these important 
issues.
    I see that my colleague from Rhode Island has joined us, 
and I thank him very much for being here and will turn to him 
for any opening comments or thoughts that he might have.
    Senator Reed?

 OPENING STATEMENT OF HON. JACK REED, A U.S. SENATOR FROM THE 
                     STATE OF RHODE ISLAND

    Senator Reed. Well, Mr. Chairman, you said it so well that 
I cannot add very much at all.
    I want to welcome Dr. Cordero. Obviously, this is a topic 
of great concern. We have made progress over the last several 
years, but we can do much more.
    I would note that your interest in children goes way back; 
it is no longer theoretical. Mine is strictly theoretical, so I 
want to just commend you for holding the hearing, and let us 
get on with the witnesses.
    Senator Dodd. Since you have asked, Grace's pictures are 
right here.
    [Laughter.]
    Senator Reed. Usually I do not have to ask.
    Senator Dodd. She was born a little more than 10 months ago 
and brought great joy. I often say that when I reach into my 
wallet to take out the pictures, there is a 50-50 chance that 
my AARP card will also drop out.
    [Laughter.]
    So I am an older father.
    Let me introduce our first witness--and I thank my 
colleague from Rhode Island for his comments.
    This morning, we are going to hear from two panels, as I 
mentioned at the outset. Our first witness is Dr. Jose Cordero, 
Director of the National Center on Birth Defects and 
Developmental Disabilities at the Centers for Disease Control 
and Prevention.
    Throughout his very distinguished career, Dr. Cordero has 
been a strong and very effective leader in the fight against 
birth defects. He is a pediatrician and joined the CDC in 1979, 
first working in the Division on Birth Defects and 
Developmental Disabilities, and later serving as deputy 
director of the National Immunization Program.
    Since the National Center opened last year, Dr. Cordero has 
undertaken the critical work of saving the lives of infants 
through the prevention of birth defects and developmental 
disabilities.
    He is also a recognized leader in developing effective 
policies to support those who are born with birth defects.
    I thank you, Dr. Cordero, not only for your presence here 
today but for many, many years of commitment to this issue. You 
truly honor us with your presence, and we are anxious to hear 
what ideas, thoughts, and advice you can give this Congress.

STATEMENT OF DR. JOSE F. CORDERO, DIRECTOR, NATIONAL CENTER ON 
                BIRTH DEFECTS AND DEVELOPMENTAL 
DISABILITIES, CENTERS FOR DISEASE CONTROL AND PREVENTION, U.S. 
      DEPARTMENT OF HEALTH AND HUMAN SERVICES, ATLANTA, GA

    Dr. Cordero. Thank you. Good morning, Mr. Chairman, Senator 
Reed.
    I am Jose Cordero, Assistant Surgeon General and Director 
of the National Center on Birth Defects and Developmental 
Disabilities.
    Thank you for the opportunity to be here today to discuss 
public health issues related to birth defects prevention and 
ensuring quality of life for individuals who have a birth 
defect.
    Mr. Chairman, as you mentioned, more than 150,000 infants 
are born with a birth defect each year in the United States. 
Birth defects are the leading cause of infant mortality in the 
U.S. Babies who do survive often experience lifelong 
disability. In addition to experiencing physical impairments, 7 
percent of children with a birth defect also have a serious 
developmental disability such as mental retardation, hearing 
loss, or vision impairment.
    The challenges that confront children over the course of 
their lives, including access to medical care and preventive 
services, the development of secondary conditions, and social 
isolation are critical public health issues.
    With the establishment of the National Center on Birth 
Defects and Developmental Disabilities in 2001, efforts related 
to birth defects, developmental disabilities, and the health of 
people with disabilities have been given new visibility.
    Although the Center is fairly new, CDC's commitment to 
these issues is not. Since 1968, CDC has made birth defects 
monitoring, research, and prevention a priority. In 1985, we 
initiated a similar program for developmental disabilities, and 
in 1988, we established our disability prevention program and 
dedicated ourselves to promoting wellness for the 54 million 
Americans who have a disability.
    When it comes to the prevention of birth defects and 
developmental disabilities, we have already seen a great many 
public health successes. We have virtually eliminated the 
mental retardation, hearing loss and vision impairments 
associated with congenital rubella syndrome, as well as mental 
retardation from Hib meningitis, which I must say when I was a 
pediatrician was a very, very common cause of mental 
retardation.
    We have seen drugs which caused devastating birth defects, 
such as thalidomide, denied entry to the United States market 
or now tightly controlled.
    Another emerging success story is the result of research 
which shows that the B vitamin folic acid prevents serious 
birth defects including spina bifida and anencephaly.
    In 1992, the U.S. Public Health Service issued a 
recommendation that all women of childbearing age should 
consume 400 micrograms of folic acid to reduce their risk for 
these birth defects. CDC and the National Council on Folic 
Acid, a coalition of nonprofit organizations, took action to 
provide women with information about folic acid. The members of 
the National Council, the Spina Bifida Association of America, 
and the March of Dimes will also be testifying here today.
    In 1996, the Food and Drug Administration also responded to 
this compelling scientific information and required all 
enriched cereal grain products to be fortified with folic acid 
by January 1998. The results of these public health information 
and fortification efforts are beginning to bear fruit.
    Data published last week from 24 population-based State 
birth defects surveillance programs indicate that the 
prevalence of spina bifida has decreased 31 percent from 1995, 
before fortification, to 1999, after fortification.
    These results are extremely encouraging as are the data 
emerging from other CDC-supported projects, suggesting that 
folic acid may also prevent or might have a role in preventing 
other birth defects.
    Epidemiologic research like this is the foundation of our 
efforts to identify new causes of birth defects and 
developmental disabilities. For example, CDC supports Centers 
for Birth Defects Research and Prevention in seven States. 
These Centers are undertaking the National Birth Defects 
Prevention Study, one of the largest studies ever conducted on 
the causes of birth defects. This study will provide the Nation 
with a continuing source of information on potential causes of 
birth defects. CDC is also supporting similar research programs 
for developmental disabilities.
    We are very optimistic about our ability to use science to 
identify new causes of birth defects and developmental 
disabilities and the ability of the public health community to 
translate the science into action.
    However, until all causes of birth defects are identified, 
and prevention strategies are implemented, some children will 
still be affected. Many of these children will share the 
experience of tens of millions of other Americans who are 
living with a disability.
    Great health disparities currently exist among people with 
disabilities. For that reason, CDC is supporting research 
related to health promotion and wellness among people with 
disabilities. One promising research project involves a 
community-based wellness program known as Living Well. This 
program has been shown to reduce disabilities due to secondary 
conditions by 37 percent and to reduce physician visits by 45 
percent. We are expanding this program to a number of States.
    Finally, CDC recognizes the importance of ensuring that 
people with disabilities have access to the specific types of 
medical treatment and care that are required as a result of 
their disability but also access to preventive health services. 
For example, CDC is working to ensure that women who use 
wheelchairs have access to mammograms and cervical cancer 
screening.
    However, there are many challenges ahead of us. We can take 
great pride in the fact that individuals with disabilities are 
living longer. But the health care system is not adequately 
prepared to deal with the consequences of our success. Most 
physicians have little experience providing care to adults with 
spina bifida, cerebral palsy, Down syndrome, or other similar 
conditions.
    Young people who have grown up with spina bifida or other 
birth defects often experience serious medical complications 
during their teenage years and need assistance dealing with 
both physical and social challenges associated with their 
disability.
    It is clear that there is a need to educate health care 
providers on the issue of disabilities across the lifespan. We 
have a long way to go, but we are already moving forward. I 
think we can and will do much more in the future.
    I appreciate the opportunity to share with you this 
information on CDC's activities to prevent birth defects and 
developmental disabilities and promote quality of life for 
people with disabilities. It is our goal to ensure optimal 
health and wellness, beginning before birth and continuing 
throughout the lifespan. To achieve this goal, we undertake 
epidemiologic and prevention research, implement tracking and 
monitoring programs, and partner with organizations who share 
our mission.
    I hope you find this overview useful, and I will be very 
happy to answer any questions that you may have.
    Thank you.
    [The prepared statement of Jose Cordero, M.D. follows:]

            Prepared statement of Jose Cordero, M.D., M.P.H.

    I am Dr. Jose Cordero, Director of the National Center on Birth 
Defects and Developmental Disabilities at the Centers for Disease 
Control and Prevention (CDC). I am a pediatrician and geneticist, with 
29 years of public health experience related to birth defects, 
developmental disabilities and children's health.
    I am very pleased to be here today to discuss the extremely 
important public health issues related to birth defects prevention and 
ensuring quality of life for individuals who have a birth defect. More 
than 150,000 infants are born with a birth defect each year. Many of 
these babies do not survive, and birth defects are the leading cause of 
infant mortality in the United States. For those babies that do 
survive, they often experience lifelong disability. In addition to 
physical impairments, birth defects are closely linked with a variety 
of cognitive and developmental disabilities--7 percent of children with 
a birth defect also have a serious developmental disability. The 
challenges that confront these children over the course of their lives, 
including access to medical care and preventive health services, the 
development of secondary conditions and social isolation, are critical 
public health issues.
    With the establishment of the National Center on Birth Defects and 
Developmental Disabilities (NCBDDD) in April 2001, CDC has been given 
increased visibility for its efforts in these areas. However, while the 
organizational structure for NCBDDD is relatively new, CDC's commitment 
to these important issues is not. Since 1968, with the development of 
the Metropolitan Atlanta Congenital Defects Program, CDC has made birth 
defects monitoring, research and prevention a priority. In 1985, we 
initiated a similar program for developmental disabilities. Over the 
years, efforts in these two areas were focused on two primary goals--
the prevention of birth defects and developmental disabilities for 
which causes have already been identified and the identification of 
causes for those which have not. However, we also recognized that the 
public health community's responsibility does not stop with prevention. 
We cannot afford to ignore the public health needs of the 54 million 
Americans who have disabilities. As a result, in 1998, we established 
our disability prevention program and dedicated ourselves to a 
prevention research, monitoring and intervention program aimed at 
identifying and implementing strategies that will eliminate challenges 
to optimal health and development and promote wellness for all 
Americans, including those who have a disability.

                            PREVENTION WORKS

    When it comes to the prevention of birth defects and developmental 
disabilities, we have already seen a great many public health 
successes. Through immunization, we have virtually eliminated the 
mental retardation, hearing and vision impairments associated with 
congenital rubella syndrome, as well as mental retardation from Hib 
meningitis. We have seen drugs which caused devastating birth defects, 
such as thalidomide, denied entry to the United States market or 
tightly controlled. Removing lead from gasoline has meant that far 
fewer children have blood lead levels that might adversely affect their 
intelligence, behavior and development. These successes have been 
achieved through close coordination and collaboration between Federal 
agencies--including CDC, the National Institutes for Health, the Food 
and Drug Administration, Health Resources and Services Administration--
and with the private sector.
    More recently, we have seen the results of many years of prevention 
research pay off when we learned that increasing consumption of the B 
vitamin folic acid resulted in significant reductions in the rates of 
neural tube defects--a category of common birth defects which include 
spina bifida and anencephaly. In 1992, the United States Public Health 
Service issued a recommendation that all women of childbearing age 
consume 400 micrograms of folic acid to reduce their risk for these 
serious birth defects.
    As a result of this recommendation, CDC immediately began The 
National Folic Acid Campaign to provide women with information about 
folic acid. It is especially important for women to know that in order 
to prevent these birth defects, folic acid must be taken before 
conception and during early pregnancy. Educational materials have been 
developed stressing the use of folic acid prior to conception and these 
materials are provided to State and local campaigns. The National 
Council on Folic Acid, a coalition of non-profit organizations, has 
been established to assist in this effort. Two key members of the 
National Council, the Spina Bifida Association of America and the March 
of Dimes, will also be testifying here today. In 1996, the Food and 
Drug Administration also responded to this compelling scientific 
information regarding folic acid and authorized manufacturers of 
enriched cereal grain products to fortify these products with folic 
acid. All such products were required to be fortified by January 1998.
    We may be beginning to see the results of these public health 
education and fortification efforts. Data published just last week from 
24 population-based State birth defects surveillance programs supported 
by CDC indicate that the prevalence of spina bifida decreased 31 
percent from 1995 (pre-fortification) to 1999 (post-mandatory 
fortification). Additionally, the 1999 data from the National Health 
and Nutrition Examination Survey (NHANES) on folate blood 
concentrations reveal substantial increases among women of childbearing 
age. The trend in 1999 already exceeds the Healthy People 2010 goal to 
increase the median red blood cell folate level to 220 ng/mL among 
nonpregnant women 15-44. Further research, which includes not only 
measuring rates of birth defects but also includes measuring women's 
folic acid intake, is needed to confirm that these downward trends are 
a result of the fortification and public health education. However, 
these results are extremely encouraging, as are the data emerging from 
other CDC-supported projects that suggest that folic acid may also play 
a role in preventing other birth defects, such as imperforate anus and 
abdominal wall defects known as omphalocele.

                       RESEARCH HOLDS NEW PROMISE

    Epidemiologic research is the cornerstone for our efforts to 
identify new causes of birth defects and developmental disabilities. 
Translating these epidemiologic findings into effective public health 
strategies through applied research represents the next step to making 
prevention a reality. CDC has underway a broad range of research, 
including both epidemiologic and applied, that will be providing the 
foundation for our next prevention success stories.
    CDC's Centers for Birth Defects Research and Prevention, first 
established in 1996 and formalized with the passage of the Birth 
Defects Prevention Act of 1998 (Public Health Law 105-168), serve as a 
cornerstone for these efforts. The Centers were established in 7 states 
with nationally recognized expertise in birth defects surveillance and 
research. CDC supports and coordinates the overall activities of the 
Centers, and participates as an eight study site in the National Birth 
Defects Prevention Study (NBDPS). This study--one of the largest ever 
conducted on the causes of birth defects--is a key part of the program 
of research for the Centers. The study will provide the Nation with a 
continuing source of information on potential causes of birth defects 
and will serve as a mechanism for identifying new substances in our 
environment that are harmful to developing babies. In addition to 
participating in the NBDPS, each individual Center is undertaking its 
own center-specific research agenda on issues including nutritional, 
environmental and behavioral factors associated with birth defects, 
financial and other costs of birth defects, and birth defect prevention 
research.
    CDC is also supporting similar research programs for developmental 
disabilities. The Children's Health Act of 2000 mandated the creation 
of centers for excellence in autism and developmental disabilities 
epidemiology, and in 2001, CDC established CADDRE--Centers for Autism 
and Developmental Disabilities Research and Epidemiology. This 
collaborative network of four centers plus CDC will collect and analyze 
information on the incidence, correlates, and causes of autism and 
related developmental disabilities.
    Our collaborations on research to identify causes for birth defects 
and developmental disabilities are not limited to institutions in the 
United States. CDC has initiated a very important collaboration with 
Denmark to help improve our understanding of the potential causes of 
cerebral palsy--a condition which affects 10,000 babies born in the 
United States each year. As part of this collaboration, we will be 
undertaking a case-control study of the relationship between infection 
in pregnancy and cerebral palsy, using cases identified in the Danish 
Cerebral Palsy Register and archived newborn blood samples. Denmark has 
a unique combination of national public health data systems that is not 
found elsewhere in the U.S. or abroad. These unique Danish data systems 
can all be linked by a universal personal identifier. Thus, they 
provide much important information (such as health, medical, and 
sociodemographic information) to carry out epidemiologic studies of 
reproductive and developmental outcomes. Also, because these data 
systems contain information on large numbers of individuals over long 
periods of time, studies can be made with an unusually high level of 
statistical power. For reasons like these, such collaborations are 
invaluable in providing timely and relevant information that can be put 
to immediate use in the development of prevention strategies.
    Other prevention-oriented research currently supported by the CDC 
includes Legacy for Children and Project Choices. Legacy for Children 
is a program consisting of randomized, controlled, longitudinal 
research projects being implemented at the University of Miami and the 
University of California at Los Angeles. These projects examine the 
potential for improvement in child developmental outcomes through 
interventions designed to influence parenting behavior. The early years 
of life--especially from birth to age five--are crucial for cognitive 
and social/emotional development. Parents play a critical role in their 
children's development and are responsible for their children's 
environment. Children develop within the context of their family and 
their development is affected by the nature of the relationship with 
their parents. Previous early intervention efforts have typically 
included early education for children in child care centers and social 
or mental health services for the parents in their role as adults, with 
only marginal attention devoted to parenting behavior. The 
interventions initiated by CDC focus on parenting behaviors and the 
relationship of the parent with his or her own child. Results from 
these projects will be available over the next few years and will help 
determine what are the critical components of early intervention 
programs for children at-risk for developmental delay.
    Drinking alcohol during pregnancy poses a serious threat to the 
health of the unborn child. While overall alcohol use during pregnancy 
has been declining since 1995, rates of frequent drinking and binge 
drinking remain high and thus continue to pose a risk for many women. 
Interventions aimed at reducing alcohol consumption must begin before 
pregnancy because birth defects associated with prenatal exposure to 
alcohol can occur in the first 3 to 8 weeks of pregnancy, before a 
woman even knows that she is pregnant.
    In an effort to reduce the number of children born with alcohol-
related birth defects, CDC is working to develop and test interventions 
to identify women at high risk for an alcohol-exposed pregnancy and 
help them to reduce their alcohol intake and postpone pregnancy until 
they stop or limit their drinking. Project Choices is the centerpiece 
of CDC's research efforts aimed at preventing alcohol consumption 
during pregnancy. CDC has demonstrated that women in certain community-
based settings (such as alcohol and drug treatment centers, jails, and 
primary care centers serving low income populations) have a sevenfold 
higher risk for an alcohol-exposed pregnancy. However, information on 
how best to reduce this risk was lacking. To address this data gap, CDC 
initiated Project CHOICES: a multi-site collaborative study involving 
three universities (Nova Southeastern University, University of Texas 
at Houston, and Virginia Commonwealth University). Results from the 
first phase of the study found that two-thirds of all women who were at 
risk prior to the intervention were no longer at risk at the time of 
the 6-month follow-up visit. The intervention is now being tested more 
rigorously through a randomized clinical trial.

           MONITORING OUR PROGRESS AND MEASURING OUR SUCCESS

    As more causes for birth defects and developmental disabilities are 
identified, and prevention strategies are implemented, it will become 
even more important for the public health community to have good 
information regarding the number of cases of birth defects and 
developmental disabilities. Only by having such information will we be 
able to ensure that our programs are having their desired effect and 
make any needed adjustments and improvements. In addition, monitoring 
rates of birth defects and developmental disabilities over time also 
enables us to determine whether rates are varying over time or in 
different geographic regions. Such variations in rates may provide 
clues to additional causes or possible need for concern.
    CDC is working toward the establishment of comprehensive monitoring 
and tracking programs to accomplish these objectives, and currently 
CDC:
     Conducts model surveillance programs to monitor the 
prevalence of birth defects and developmental disabilities in the 
metropolitan Atlanta area.
     Provides financial and technical support for State-based 
birth defects surveillance through cooperative agreements with 35 
states, as well as through a collaborative effort of State programs 
known as the National Birth Defects Prevention Network that collects 
and disseminated State-based birth defects surveillance data.
     Supports the Fetal Alcohol Syndrome Surveillance Network 
(FASSNet) in five states.
     Supports the Autism and Developmental Disabilities 
Monitoring Network (ADDM) in four states.
     Funds 30 State-based Early Hearing Detection and 
Intervention (EHDI) tracking programs.
     Continually monitors folate in women through the NHANES.
     Works with States to improve the timeliness and quality of 
reporting of birth defects through the vital statistics systems.

  ENSURING HEALTH, WELLNESS AND QUALITY OF LIFE COMPLETES THE PICTURE

    While we are very optimistic about our ability to use science to 
identify new causes of birth defects and developmental disabilities and 
the ability of the public health community to translate this science 
into public health action, we know that our prevention efforts will 
never be 100 percent successful. For example, we estimate that folic 
acid has the potential to prevent 50-70 percent of all neural tube 
defects. There are still other causes of neural tube defects that 
remain unknown. Until the causes of all birth defects are identified 
and prevention strategies are implemented, close to 3 percent of all 
babies will still be born with a birth defect. Most of these children 
will share the experience of tens of millions of other Americans who 
are living with a disability. For that reason, CDC has also placed a 
high priority on research focused on promoting health and wellness 
among people with disabilities.
    Working with universities and other research organizations, CDC 
conducts research to develop a better understanding of the varied and 
unique health conditions of people with disabilities. CDC seeks to 
identify health risks and protective factors and to measure and develop 
effective health promotion activities to prevent the secondary 
conditions related to living with a disability. CDC is currently 
funding 11 projects in academic settings that develop new methods for 
promoting health and wellness among people with disability. One 
particularly promising research project will be rigorously testing a 
community-based wellness program, known as ``Living Well,'' which has 
previously been shown to reduce disability due to secondary conditions 
by 37 percent and reduced physicians visits by 45 percent over 6 
months. This empirically grounded health promotion intervention, 
delivered by community-based consumer-controlled independent living 
centers, offers a model for both improving health and independence of 
adults with physical disabilities, as well as containing medical 
services costs within a managed-care context. The new study will 
evaluate the model with a larger, more representative sample and a more 
rigorous design. Additional data on costs and preferences will be 
developed to conduct a cost-effectiveness assessment. The researchers 
will also look at how adults with physical disabilities can maintain 
and improve their health after participating in a health promotion 
program to further enhance cost-effectiveness.
    In addition, CDC is working with State health departments to 
improve their capacity for implementing programs to address the public 
health needs of people with disabilities. Currently, CDC is supporting 
such activities through cooperative agreements with 16 states. Specific 
programmatic efforts include improving State surveillance activities, 
conducting health promotion interventions, developing and monitoring 
policies affecting people with disabilities, and facilitating 
partnerships between State- and research- or service-based agencies. 
These programmatic efforts do not focus on specific types of 
disabilities, but rather they address common issues in a variety of 
cross-cutting disability domains including communication, learning, 
mobility, and personal care/home management.
    Finally, CDC recognizes the importance of working with the health 
care delivery system to ensure that people with disabilities have 
access not only to the specific types of medical treatment and care 
that are required as a result of their disability, but also access to 
important preventive health services. For example, CDC has worked to 
ensure that women with a mobility impairment who use a wheelchair have 
access to mammograms through the Breast Cancer Early Detection Program. 
To ensure this, CDC works with State programs to purchase special 
equipment. In addition, CDC is working to ensure that people with 
disabilities have access to information that will help them reduce 
health risk behaviors such as tobacco use and sedentary lifestyles.
    However, there are major challenges that remain ahead of us. While 
we can take great pride in the advancements that have been made in 
increasing the life expectancy of individuals with disabilities, we 
also must recognize that the health care system is not adequately 
prepared to deal with the consequences of our success. The life 
expectancy of an individual with Down Syndrome has increased from 1 
year to 49 years since the late 1960's. Just several decades ago, very 
few children with spina bifida survived at all. Because these changes 
have occurred over a relatively short period of time, most physicians 
have had no experience dealing with an adult with these types of 
conditions. In 1994, SBAA and United Cerebral Palsy sponsored a 
symposium entitled ``Preventing Secondary Conditions Associated with 
Spina Bifida or Cerebral Palsy.'' Then, as now, one of the major issues 
of concern to people with spina bifida and cerebral palsy, their 
families and their health care providers was the issue of transition 
from pediatric care. The need is particularly acute in teenage years. 
These are difficult years for any young person, but for a young person 
with a disability, such as spina bifida, they can be even more 
challenging. For example, teenagers with spina bifida often experience 
serious medical complications. In addition, these teenagers often need 
assistance in dealing with both physical and social challenges 
associated with catheterization, skin care, and cleanliness. Social, 
educational, and vocational support are also essential to ensuring full 
participation as an adult.
    It is clear that, in addition to developing programs that address 
the public health needs of people with disabilities, there is a need to 
educate healthcare providers on the issue of disability across the 
lifespan. Addressing these issues will also provide an opportunity to 
begin to prepare our public health and health care systems for the 
challenges presented by the aging of the American population. I look 
forward to working toward future successes in these areas, and 
recognize that achieving them will require broad-based partnerships and 
collaborations with research, public health, medical and voluntary 
organizations. We have a long way to go, but we are already moving 
forward. I think we can and will do much more in the future.
    I appreciate this opportunity to share with you this information on 
CDC's activities to prevent birth defects and developmental 
disabilities and ensure quality of life for individuals who have 
experienced disability. Our goal is to ensure optimal health and 
wellness--beginning before birth and continuing throughout the 
lifespan. To achieve this goal, we undertake epidemiologic and 
prevention research, implement tracking and monitoring programs, and 
partner with health care and voluntary organizations who share our 
mission. I hope you found this overview useful, and I will be happy to 
answer any questions that you may have.
    Thank you.

    Senator Dodd. Thank you very much, Dr. Cordero, for your 
work. Of course, the work of the National Center is tremendous, 
and we are very grateful for the efforts that have been made 
and the initiatives that have been undertaken.
    I have a number of questions. Just picking up on one of the 
last points you made about the need to educate health care 
providers and the problem that exists out there with the lack 
of understanding among the physician or health care provider 
population, what specific recommendations would you make? We 
are going to be reauthorizing legislation here, and if there 
are some things we could recommend as part of our efforts that 
could help close that gap, I would think that that would be one 
of the critical things we could try to achieve.
    Dr. Cordero. Yes, Senator. There are two areas that we are 
actually approaching at CDC. One is that we have entered into a 
collaborative agreement with the American Academy of Pediatrics 
to educate physicians about the challenges in the first 18 
years of life of working with individuals with disabilities, 
particularly spina bifida and others.
    Second is that CDC is developing, in collaboration with the 
National Institutes of Health and the AHRQ, to look at a 
consensus on what are the specific activities that can be done 
to help individuals with spina bifida. This consensus 
conference will address issues of medical care, issues of 
functional limitations, and also social and activities of daily 
life. I think that will identify both what are the research 
gaps and what are the things that we can implement currently to 
help individuals with spina bifida.
    Senator Dodd. When do you anticipate those recommendations?
    Dr. Cordero. The meeting is scheduled for April or May of 
2003.
    Senator Dodd. The reauthorization expires this year, so any 
thoughts you have ahead of time would be helpful if we are 
going to reauthorize before the expiration. I do not want to 
have to get to this in the next Congress. We could end up 
losing a year or so. It is going to be tough this fall with so 
much on the agenda, but this will be very important to get 
reauthorization. I anticipate wide support for us, so if you 
could give us some ideas specifically on how we can do that, it 
would be helpful early on.
    Dr. Cordero. Right. We will be happy to provide more 
specifics in writing for you.
    Senator Dodd. Would you, please? That would be very, very 
helpful to us. And I can see heads nodding in the audience, so 
I am sure we will get some additional suggestions on this.
    What I will do is ask a couple more questions and then turn 
to my colleague from Rhode Island. As I announced ahead of 
time, Jack, we will then break to go over and vote, but we will 
at least finish with Dr. Cordero. So I will watch the clock, 
and I may have to submit some additional questions to you in 
writing.
    I wonder if you could give us some idea as to why these 
numbers continue--they are still large numbers. We know that we 
have done a lot with folic acid, yet the numbers still seem 
high in the case of spina bifida among certain communities. I 
wonder if you could shed some light on that.
    Second--and I will ask both questions and then turn to my 
colleague--what has the CDC identified as some of the 
environmental causes of birth defects, and what is the National 
Center doing to provide information about these causes so that 
people have a better level of education about what to avoid and 
what to be careful about?
    Dr. Cordero. Let me answer the first one about the numbers 
in the case of spina bifida. Actually, I think we have made 
great progress, as I mentioned, with the over 30 percent 
decline in spina bifida comparing before and after 
fortification.
    We have made some progress in educating women and families 
about the use of folic acid, but we realize that we still have 
challenges in reaching some communities, particularly the 
Hispanic community. We are actually working to develop a more 
aggressive campaign to educate women in Hispanic communities 
about the use of folic acid. We have actually started a project 
in Miami and San Antonio on how we can be sure to reach the 
Hispanic community, with messages but also working with 
physicians and the communities in those areas to be sure the 
message gets out and that it is translated into action.
    Senator Dodd. That is important. What about the 
environmental issues?
    Dr. Cordero. CDC is also working with the Center for 
Environmental Health and the National Institute for 
Environmental Health Sciences in actually funding a number of 
projects and centers looking at the area of birth defects and 
developmental disabilities.
    As I mentioned, we are funding seven Centers for Birth 
Defects Research and Prevention, and these centers are actually 
conducting the largest study, one of the largest ever, and 
there, we are hoping to identify new causes of birth defects, 
including environmental areas.
    In terms of what we can recommend today, I think the 
important things are, one, that women begin to take folic 
acid--
    Senator Dodd. When they are of childbearing age.
    Dr. Cordero [continuing]. Of childbearing age, yes--and 
also, that they actually stop using alcoholic beverages before 
pregnancy begins, quit smoking, and also, that they review with 
their doctor what kinds of exposures they have in the workplace 
to ensure that they are not exposed to chemicals and other 
things that could be a problem for them during pregnancy.
    Senator Dodd. Are there lists of these things, for 
instance, chemicals or substances in the workplace--there are 
known lists of these materials, aren't there, that women who 
are pregnant or are thinking about getting pregnant should not 
be around?
    Dr. Cordero. Yes. Actually, one of the challenges that most 
women have when they seek information is finding one place 
where they can get all the information. And actually, one of 
our fundings for this year is a center that would provide 
information. This is a center in California which would be able 
to answer women's questions about what are the kinds of 
exposures, so if they have specific questions, they can be 
answered. I think that that is something that hopefully, we can 
expand in the future.
    Senator Dodd. And these health clinics--last Monday, we 
opened a dental division--we have 11 public health clinics in 
Connecticut, but they are all over the country, and they are 
growing, and they are very successful. It seems to me that for 
the audiences that we are talking about here, those places 
would be a very good place to start, given the fact that so 
many people who may not be as well-informed about the problems 
could start getting that information when they show up at these 
health clinics.
    Dr. Cordero. I agree, Senator. I think that that is an area 
that we can look into, how we can expand there.
    Senator Dodd. Yes. I have about 100 more questions for you, 
but let me turn to my colleague from Rhode Island, Senator 
Reed.
    Senator Reed. Thank you very much, Mr. Chairman.
    Thank you, Dr. Cordero. When we were doing the Children's 
Health Act of 2000, I became aware of and interested in the 
Fragile X problem. Is CDC doing any work with respect to 
Fragile X?
    Dr. Cordero. Senator, we have been working with a number of 
colleagues on Fragile X for a number of years. We actually 
funded Dr. Stephanie Sherman, who is one of the world experts 
on Fragile X, to conduct a study looking at two questions--one, 
what can be done to help individuals with Fragile X, and two, 
can we begin screening for Fragile X in the near future. At 
that time--this was a few years ago--the answer was that it was 
not yet the time for that. And we are working with colleagues, 
reviewing those questions, and especially what we can do to 
identify children with Fragile X early and then, what are the 
interventions that may actually work.
    Let me say that we are about to publish a paper in the 
Morbidity and Mortality Weekly Report that has actually been 
looking at the delays in diagnosing Fragile X. I think that 
that is going to give us some information to then move into 
additional actions.
    Senator Reed. Thank you, Doctor.
    This line of questioning raises the issue of the impact of 
genomic research, generic research, on birth defects and to 
what degree CDC or NIH is pursuing the breakthroughs that we 
have seen in genomics.
    Dr. Cordero. I think genomics offers a great opportunity 
for, one, finding more causes of birth defects, especially 
looking at the genetic environment interactions, which is an 
area that we had very little opportunity to look at before. We 
are working very closely with our Office of Genomics at CDC, as 
well as with the NIH, in exploring areas where we can use the 
genomics knowledge to actually find causes of birth defects. In 
fact, several of our centers--a center in Iowa is actually 
looking at the role of genes in cleft palate, and also the 
interaction of cleft palate and genes in causing these serious 
birth defects.
    I think it is the future, and we expect and will continue 
through our Centers on Birth Defects to do more research and 
fund more research in that area.
    Senator Reed. I know the Center on Birth Defects is 
preparing a study about the topic. It seems to me that there 
are so many different ways to approach the issue, since it is 
very complicated, but without being overly simplistic, there 
are some basic public health initiatives--education, good 
nutrition--and then, there are some more sophisticated issues 
in terms of genetic predisposition, environmental factors.
    Within your study, are you also going to send up here or 
devise a strategy that would approach the public health 
problems in terms of the comprehensive way, and let us know how 
much it would cost, and then lay out a strategy to deal with 
the more complicated issues of genetics and environmental? Is 
that something that you are contemplating?
    Dr. Cordero. The answer is yes. Actually, I think that what 
you are outlining is very much the approach that we are 
following in terms of addressing both the issues of birth 
defects and also when we apply genetics into it. For whatever 
we do--let us take the example of even spina bifida--we 
actually need to educate the public, we need to educate the 
health care professionals, and then determine how the 
intervention actually works and how we insert also the 
laboratory work that needs to go into it.
    I agree, and I think that that is exactly the process that 
we are following, and as we get more into the genetics of 
diseases, I think we will have more of that. But the public 
health effort to educate and track and also conduct more 
research will be the basis of what we will continue to do.
    Senator Reed. Thank you very much, Dr. Cordero.
    Thank you, Mr. Chairman.
    Senator Dodd. Thank you, Senator Reed.
    I would ask you if you could, just in response to Senator 
Reed's questions, if you are going to get us some early 
information in the next month or so where you include this kind 
of information. As I said, Doctor, you have a very willing and 
anxious Senator here on this issue. Senator Bond offered the 
original bill back in 1998, I guess it was--he is going to try 
to come by this morning, by the way--but we would very much 
like to get as much of this information as we can so that when 
we craft the reauthorization legislation, we can accommodate as 
many of these ideas as possible in that bill.
    Dr. Cordero. We will be very delighted to provide more 
information in writing.
    Senator Dodd. And before we race to make the vote, I did 
not get to the questions on the additional costs. I mean, $1 
million is not an outrageous number when you start talking 
about the cost of continuum of care. That is a staggering 
figure. And obviously, these disabilities do not choose people 
based on economic circumstances and their ability to afford it; 
they are blind to that. So we need to be talking as well about 
how we can provide some really meaningful support so that with 
the wonderful new breakthroughs now medically, people can live 
very productive lives for a long time, and to enhance their 
ability to do that and support their caregivers and their 
families has got to be a critical piece of any discussion in 
this area. So I would be very interested as well in any ideas 
you can suggest and give to us on how we can be more supportive 
of the families out there and the individuals themselves, so 
they can have lives that are fruitful and productive.
    If you would make that a part of your recommendations to 
us, I would appreciate it.
    Dr. Cordero. We certainly will, and especially the care of 
individuals with birth defects is a key area that needs to be 
addressed.
    Senator Dodd. Yes. Again, I will submit some additional 
questions to you in order not to hold you up, and we will come 
right back as soon as the vote is over.
    [Questions of Senator Dodd were not available for inclusion 
at press time, however, copies are retained in the committee 
file.]
    Dr. Cordero. Thank you.
    Senator Dodd. The subcommittee will stand in recess for a 
few minutes.
    [Recess.]
    Senator Dodd. The subcommittee will come to order.
    My apologies to all of you for being disrupted with the 
vote. Let me thank all of you again for being here.
    I am going to submit for the record a statement from 
Senator Bond who will be unable to join us this morning but, as 
I mentioned a while ago, was the author of the authorization in 
1998. So his statement will be included in the record.
    [Statement of Senator Bond was not available at press time, 
however, copies are retained in the committee files:]
    Senator Dodd. I am pleased now to introduce our second 
panel of witnesses and thank all of you again for being here.
    First, we will hear from Mr. Hal Pote. Mr. Pote is the 
president of the board of directors of the Spina Bifida 
Foundation, which is affiliated with the Spina Bifida 
Association of America, an organization dedicated not only to 
eradicating spina bifida but one that is admirably working to 
better the lives of more than 70,000 individuals living with 
this birth defect.
    In addition to his responsibilities at the Foundation, Mr. 
Pote is a regional bank executive at J.P. Morgan Chase Bank. He 
is the proud uncle of Greg, whom I met before the hearing and 
who is with us here today and whose picture is up behind me 
here, carrying the Olympic torch.
    Mr. Pote, we thank you for joining us today. You are very 
gracious to be here, and I commend you for the wonderful work 
that you are doing.
    Next, we are going to hear from Dr. Nancy Green, who has 
recently been named the medical director for the March of 
Dimes, an organization that we are all familiar with for its 
long record of lifesaving work. Since its founding in 1938 by 
President Franklin Roosevelt, the March of Dimes has proven 
itself an invaluable national resources to the Nation's public 
health.
    Dr. Green received her M.D. from Columbia University 
College of Physicians and Surgeons and currently serves as 
associate professor of pediatrics and cell biology at the 
Albert Einstein College of Medicine in New York.
    We congratulate you on your new position and look forward 
to you working with this committee in the coming years.
    Our final witness is Fred Liguori from Granby, CT. I am 
pleased to have you here, Fred, coming from our State. Fred and 
his wife Jill have a son, Nicholas, whom I have already 
mentioned and talked about, a fine young man.
    We also have up here on the dais a picture of Nicholas 
riding his tractor. He is reading diligently over there with 
his mother at this particular point. Nicholas has spina bifida, 
and Mr. Liguori will testify to some of the unique needs that 
Nicholas has.
    I have already discussed some of the costs that can hit a 
family, and we are anxious to hear your ideas and thoughts, and 
again, we are very grateful to you, as well as to you, Hal, for 
being here. I am always impressed when people are willing to 
come forward in a public setting and talk about these matters. 
It is not easy to do so, and I am glad that you have brought 
family along as well so we will get a chance to meet them--so 
these are not just numbers and statistics and data, but we in 
the Congress here and our staffs and members who can be here, 
those who are watching on CSPAN, which is covering this hearing 
today, can appreciate that this is more than just about data 
and numbers. It is about real human beings and real needs and 
what we can do cooperatively to make those burdens lighter, and 
to see that we maximize the potential of each one of these 
wonderful God-given gifts of life.
    So we thank you for coming today, and we will begin with 
you, Hal, and move right down the table in the order that I 
have introduced you.

STATEMENTS OF HAROLD POTE, PRESIDENT, SPINA BIFIDA FOUNDATION, 
 NEW YORK, NY; DR. NANCY S. GREEN, MEDICAL DIRECTOR, MARCH OF 
   DIMES BIRTH DEFECTS FOUNDATION AND ASSISTANT PROFESSOR OF 
 PEDIATRICS, ALBERT EINSTEIN SCHOOL OF MEDICINE, NEW YORK, NY; 
              AND FRED LIGUORI, PARENT, GRANBY, CT

    Mr. Pote. Thank you, Senator.
    Mr. Chairman and Senator Reed, you are very gracious with 
your remarks. I want both of you to know how pleased we are to 
have this opportunity to be here today, so thank you very much 
for that.
    I am here representing the Spina Bifida Association of 
America, the Spina Bifida Foundation, and our whole spina 
bifida family, and I respectfully request that my testimony be 
included in the record.
    As you mentioned in your opening remarks, Senator Dodd, 
spina bifida is a neural tube defect that results from the 
spine failing to close properly. More important, it is the most 
common birth defect in the United States that results in 
permanent disability, and as you mentioned a minute ago, there 
are currently 70,000 families in our country who have been 
affected by spina bifida.
    Our Association is a national voluntary health agency 
working on behalf of all of these folks and their families 
through education, advocacy, research and service. We are about 
30 years old, and today we have 60 chapters and serve 100 
communities around the country.
    As you mentioned, Senator, my personal passion for this 
issue comes about through my relationship with my nephew, Greg, 
who is here today. He is a terrific kid, and I love him deeply, 
but he has been dealt a tough hand. He has had several major 
surgeries; he has another one scheduled for next month. On the 
other hand, as you can see from that photograph, he is clearly 
the most famous member of our family, and while that was very 
early in the morning, Senators, I am sure that both of you saw 
him on television when he was carrying the torch.
    [Laughter.]
    While prevention of spina bifida is a critical public 
health issue, and we are fully committed to the range of 
activities that can reduce the incidence of spina bifida, in 
the interest of time, I will focus my remarks on three other 
important issues, and my written testimony will deal with 
prevention. And Dr. Green, I am pleased to say, in her role 
here representing the March of Dimes, will be focusing on 
prevention in greater detail.
    The three issues are the following. First, quality of life. 
As a result of this neural tube defect, most babies and 
children with it suffer from a very broad range of challenges, 
including physical, psychological, and educational challenges. 
These include paralysis, developmental delay, numerous 
surgeries, and living with a shunt in an attempt to ameliorate 
their condition.
    After decades of poor prognoses and short life expectancy, 
due to recent breakthroughs in research and treatment, more 
children with spina bifida are becoming adults with spina 
bifida, and that is the very good news. This then provides a 
new range of challenges including education, job training, 
independent living, and health care, now prolonged for 
secondary conditions.
    Even if we are successful in preventing the majority of 
spina bifida cases in the future through folic acid and 
supplementation of grain, our Nation must still take the steps 
to ensure that the thousands of individuals living with spina 
bifida today can live full and healthy lives.
    To that end, improved secondary prevention interventions 
and treatment therapies must be identified, developed, and 
delivered to those in need. Dr. Cordero referenced some of the 
things that we are working on in conjunction with his 
organization, AHRQ, and others.
    Secondary conditions associated with spina bifida--and by 
the way, these secondary conditions are more complicated for a 
person with spina bifida than they are for anyone with any 
other birth defect--can include neurological disorders, 
paralysis, bladder and bowel control difficulties, learning 
difficulties, depression, social and sexual issues, latex 
allergy, obesity, and skin breakdown.
    For example, our children with learning disabilities may 
have difficulty paying attention, expressing or understanding 
language, and grasping reading and math. So a response there--
early intervention with children who have experienced learning 
problems can help them considerably prepare for school.
    With appropriate, affordable, and high-quality medical, 
physical, and emotional care, most people born with spina 
bifida will live a long and productive life. Ensuring access to 
the full range of necessary and appropriate services is 
essential to improve the quality of life for those born with 
spina bifida.
    The second topic, which you have referenced already, 
Senator, is cost. It is in fact estimated--and we do not yet 
have hard data on this, but that is part of what we are doing--
that the lifetime cost of care for individuals with spina 
bifida very often exceeds $500,000 and very often exceeds $1 
million, as you mentioned. And Senator, as you pointed out 
earlier, when you look at that million-dollar cost and then you 
think back to Dr. Cordero's testimony, the decline in spina 
bifida incidence is happening in the socioeconomic groups where 
you would expect to first see it. The challenge is that folks 
who have greater socioeconomic challenges are also the folks 
who are not seeing a decline in spina bifida incidence, and 
they are in fact the folks who are least able to afford these 
burdens of medical cost and probably least likely to have the 
private health insurance that can help ameliorate that 
situation.
    Nationally, we think this is a $750 million per year issue, 
and we know that the Social Security Administration is paying 
out $82 million a year to folks who are affected by spina 
bifida.
    The third issue that I would like to comment on deals with 
the range of choices available to families when they learn of a 
diagnosis of a fetus with spina bifida. Historically, that 
family had two choices--first, to carry full term, to have a 
beautiful child but a child who would be faced with a lifetime 
of challenges; and the second option was to terminate the 
pregnancy voluntarily. There is perhaps now a third option 
developing from in utero surgery, and through the work being 
done now at three hospitals, it is very early, and we are now 
supporting the work being done to go back and look at the 
results, but it appears that there is some encouraging evidence 
coming out that, for example, perhaps 30 to 50 percent of 
births of folks who have received in utero surgery would result 
in their not requiring a shunt. So there could be some very 
exciting things coming out of in utero surgery, but we need 
support to make sure we understand that and that those best 
practices can be promulgated to the community of surgeons as 
well.
    I would like to close by first thanking Dr. Cordero for his 
testimony and for his leadership. There are two specific 
messages that we would like to leave with this subcommittee.
    The first is that the National Center for Birth Defects and 
Developmental Disabilities is critical for our community. It 
was a very important step forward when this Center was founded 
and Dr. Cordero's leadership was established. We would urge 
that it be funded at a level of $115 million this coming year. 
I think that is about $25 million more than the funding it 
received last year. We are well aware that this is a budget 
with lots of challenges, but we would love to see that kind of 
support.
    Then, secondly, as you indicated in your opening comments, 
Senator, identifying $3 million of that to support the Center 
of Excellence for Spina Bifida would be something that would 
also be very important to us as well.
    So in closing, let me again thank you on behalf of our 
community and for the 70,000 families for the leadership that 
you have shown and the work that your subcommittee is doing, 
and thank you for the opportunity to be here.
    Senator Dodd. Thank you very much for very fine testimony. 
I look forward to working with you some more on this.
    [The prepared statement of Mr. Pote follows:]

                   Prepared Statement of Harold Pote

                              INTRODUCTION

    Mr. Chairman, and Members of the Subcommittee, on behalf of the 
Spina Bifida Association of America (SBAA) and the Spina Bifida 
Foundation, thank you for the opportunity to come before you today to 
discuss issues related to Spina Bifida, the nation's most common, 
permanently disabling birth defect.
    I am honored to be here today to discuss with you the importance of 
preventing Spina Bifida and improving the lives of the more than 70,000 
individuals and their families who are affected by this condition. I 
respectfully request that my testimony be included in the Congressional 
Record.
    While in my day job I currently serve as the head of the Regional 
Banking Group at JP MorganChase, I also serve as the President of the 
Spina Bifida Foundation Board, a cause that is near and dear to my 
heart. SBAA is the national voluntary health agency working on behalf 
of people with Spina Bifida and their families through education, 
advocacy, research and service. The Association was founded in 1973 to 
address the needs of the Spina Bifida community and today serves as the 
representative of 60 chapters serving more than 100 communities 
nationwide.
    My personal interest and passion which drives my involvement with 
these organizations stems from the fact that my family is one of the 
70,000 affected by Spina Bifida. My nephew Gregory Pote--who is here 
today in the audience--was born with Spina Bifida 16 years ago. One of 
my proudest moments as Greg's uncle was the morning that our family 
awoke before the crack of dawn and gathered together on the side of a 
street in Philadelphia to watch Greg carry the Olympic torch earlier 
this year. To date, Greg has already had more than 20 surgeries. Double 
digit numbers for surgeries unfortunately are not unusual for children 
living with this condition. I am here today to outline what Congress 
can do to prevent Spina Bifida and help improve the quality of life for 
Greg and the other individuals and their families that live with this 
birth defect.

                               BACKGROUND

    Spina Bifida is a neural tube defect (NTD) and occurs when the 
spinal cord fails to close properly during the early stages of 
pregnancy. This happens within the first few weeks of pregnancy and 
most often before the mother knows that she is pregnant. Over the 
course of the pregnancy--as the fetus grows--the spinal cord is exposed 
to the amniotic fluid which increasingly becomes toxic. It is believed 
that the exposure of the spinal cord to the toxic amniotic fluid erodes 
the spine and results in Spina Bifida. There are varying forms of Spina 
Bifida occurring from mild--with little or no noticeable disability--to 
severe--with limited movement and function. Unfortunately, the most 
severe form of Spina Bifida occurs in 96 percent of children born with 
this birth defect.
    The result of this neural tube defect is that most children with it 
suffer from a host of physical, psychological, and educational 
challenges--including paralysis, developmental delay, numerous 
surgeries, and living with a shunt in their skulls which seeks to 
ameliorate their condition by helping to relieve cranial pressure 
associated with spinal fluid that does not flow properly. It is 
wonderful to report that after decades of poor prognoses and short life 
expectancy, children with Spina Bifida are now living long enough to 
become adults with Spina Bifida. These gains in longevity principally 
are due to breakthroughs in research, combined with improvements in 
health care and treatment. However, with this extended life expectancy, 
our Nation and people with Spina Bifida now face new challenges--
education, job training, independent living, health care for secondary 
conditions, aging concerns, among others.

                               PREVENTION

    While the exact cause of Spina Bifida is unknown, over the last 
decade, medical research has confirmed a link between a woman's folate 
level before pregnancy and the occurrence of Spina Bifida. Sixty 
million women are at risk of having a child born with Spina Bifida and 
each year approximately 4,000 pregnancies in this country are affected 
by Spina Bifida, resulting in 2,500 births. Recent studies have shown 
that if all women of childbearing age were to consume 0.4 mg of folic 
acid prior to becoming pregnant and throughout the first trimester of 
pregnancy, the incidence of Spina Bifida could be reduced by up to 75 
percent. There are few public health challenges that our Nation can 
tackle and conquer by three-fourths in such a straightforward fashion. 
However, we must still be concerned with addressing the 25 percent of 
Spina Bifida cases that cannot be prevented by folic acid consumption 
as well as ensuring that all women of child-bearing age consume 
adequate amounts of folic acid.
    Progress has been made in convincing women of the importance of 
consuming folic acid supplements and maintaining diets rich in folic 
acid. Since 1968, the Centers for Disease Control and Prevention (CDC) 
has led the Nation in monitoring birth defects and developmental 
disabilities, linking these health outcomes with maternal and/or 
environmental factors that increase risk, and identifying effective 
means of reducing such risks. While the CDC has a long-standing history 
of addressing birth defects and developmental disabilities, in 2000--as 
you know--the National Center on Birth Defects and Developmental 
Disabilities (NCBDDD) was established at the agency as created by the 
Children's Health Act of 2000. NCBDDD's mission is to improve the 
health of children by preventing birth defects and developmental 
disabilities. Mr. Chairman we thank you and other members of the 
subcommittee and the full Health, Education, Labor, and Pensions (HELP) 
Committee for your assistance in creating the NCBDDD.
    Former CDC Director Jeff Koplan stated last year that the Center's 
folic acid prevention campaign has reduced neural tube defect births by 
20 percent. This public health success should be celebrated, but it is 
only half of the equation as approximately 2,500 babies are born each 
year with Spina Bifida. This public education campaign must be enhanced 
and broadened to reach segments of the population that have yet to heed 
this call to ensure that as many cases of Spina Bifida can be prevented 
as possible.
    However, even if we are successful in preventing the majority of 
Spina Bifida cases in the future, our Nation must still take steps to 
ensure that the thousands of individuals living with Spina Bifida can 
live full and healthy lives. To ensure the highest quality of life 
possible, prevention interventions and treatment therapies must be 
identified, developed, and delivered to those in need. As I mentioned 
earlier, major medical advances have permitted babies born with Spina 
Bifida to have a normal life expectancy and live independent and 
fulfilling lives. Despite these gains, individuals and families 
affected by Spina Bifida face many challenges--physical, emotional, and 
financial.

                       CHALLENGES OF SPINA BIFIDA

    Secondary conditions associated with Spina Bifida include full or 
partial paralysis, neurological disorders, bladder and bowel control 
difficulties, learning disabilities, depression, latex allergy, 
obesity, skin breakdown and social and sexual issues. Spina Bifida 
children with learning disabilities may have difficulty with paying 
attention, expressing or understanding language, and grasping reading 
and math. Early intervention with children who experience learning 
problems can help considerably to prepare them for school. With 
appropriate, affordable, and high-quality medical, physical, and 
emotional care, most people born with Spina Bifida likely will have a 
normal or near normal life expectancy. Ensuring access to these 
services is essential to improving the quality of life for those born 
with this birth defect.
    Julie Yindra, an active volunteer with the Spina Bifida Association 
of America from North Carolina is with us in the audience today. As an 
adult with Spina Bifida, Julie believes strongly that the key to being 
able to be independent, self-sufficient and productive is education. 
Because of that, Julie is close to completing a PhD in Higher Education 
and working at Greensboro College to increase access for students with 
disabilities.
    It is important to note that the lifetime costs associated with a 
typical case of Spina Bifida--including medical care, special 
education, therapy services, and loss of earnings--are as much as 
$1,000,000. The total societal cost of Spina Bifida exceeds $750 
million per year with just the Social Security Administration payments 
to individuals with Spina Bifida exceeding $82 million per year. 
Moreover, tens of millions of dollars are spent on medical care covered 
by the Medicaid and Medicare Programs. I believe strongly that our 
Nation must do more to help reduce the emotional, financial, and 
physical toll of Spina Bifida on the individuals and families affected.

                IN-UTERO SURGERY TO ADDRESS SPINA BIFIDA

    SBAA seeks to support individuals and families affected by Spina 
Bifida, maximize the prevention of Spina Bifida, and ensure that as 
many Spina Bifida pregnancies go to term as possible. When families 
recently diagnosed with a Spina Bifida pregnancy contact SBAA, the 
organization puts them in touch with another family who has a child 
with the condition so they can learn of the joys and challenges of 
having a child with the birth defect. Unfortunately, traditionally when 
families have faced a Spina Bifida diagnosis they have had two 
difficult options. The first is to continue the pregnancy with the 
expectation of multiple surgeries for the child after birth, uncertain 
life expectancy, and many physical and developmental challenges and 
complications. The second, unfortunately, is to voluntarily terminate 
the pregnancy. Fortunately, now there may be a third option.
    Since the late 1990's, doctors at three U.S. hospitals--Children's 
Hospital of Philadelphia, Vanderbilt University Medical Center in 
Nashville, and the University of California at San Francisco--have been 
operating before birth on fetuses diagnosed with Spina Bifida. Last 
year, the University of North Carolina, became the fourth hospital in 
the Nation to perform these operations. By closing the lesion early in 
pregnancy, these doctors believe they can minimize the damage created 
by fluid leaking from the spine, as well as by the spinal cord's 
contact with amniotic fluid. Surgeons have found that closing the hole 
in the spine in this fashion before birth may correct breathing 
problems in 15 percent of the children receiving the procedure and may 
reduce the need for a shunt to drain brain fluid buildup by between 33 
percent and 50 percent. Given the potential for this surgery in the 
secondary prevention of conditions associated with Spina Bifida, we 
must do a better job of studying this procedure, educating health care 
providers and families about this surgery, and making information about 
it available to more families facing a Spina Bifida pregnancy. It is 
our hope that with improved quality-of-life for individuals and 
families affected for Spina Bifida available through this surgery as 
well as interventions through a National Spina Bifida Program, the 
stigma and fear associated with a Spina Bifida birth will decrease 
significantly. In turn, the number of Spina Bifida pregnancies 
continued to term likely will increase.

              THE NEED FOR A NATIONAL SPINA BIFIDA PROGRAM

    SBAA and the Spina Bifida Foundation are working hard to ensure 
that our Nation is taking all the steps possible to prevent Spina 
Bifida and diminish suffering for those currently living with it. To 
that end, we currently advocate that the CDC receive $3 million in 
fiscal year (FY) 2003 to develop and implement a National Spina Bifida 
Program to promote quality-of-life programs which support people with 
Spina Bifida so they can live fulfilling and productive lives.
    A national program works on two critical levels--to reduce and 
prevent Spina Bifida incidence and morbidity and to improve quality-of-
life for those living with Spina Bifida. A National Spina Bifida 
Program would ensure that what is known by scientists is practiced and 
experience by the 70,000 individuals and families affected by Spina 
Bifida. For example, such an initiative would help individuals with 
Spina Bifida and their families learn how to treat and prevent the 
secondary health problems which range from learning disabilities and 
depression to severe allergies and skin problems that make life 
difficult for these individuals. All of these problems can be treated 
or prevented, but only if those affected by Spina Bifida are properly 
educated and taught what they need to do to keep themselves healthy.
    A National Spina Bifida Program offers young families hope and help 
through a plan to educate the public of the importance of taking a 
vitamin with folic acid and the maintenance of a healthy diet that 
includes foods fortified with folic acid. As SBAA works collaboratively 
with CDC and the March of Dimes to increase awareness of the benefits 
of folic acid for those at risk of having a baby with neural tube 
defects (those who have Spina Bifida themselves or those who have 
already conceived a baby with Spina Bifida) these activities need to be 
expanded to reach the broader population in need of these public health 
education, health promotion, and disease prevention messages. Such 
efforts should include reaching out to at-risk populations (e.g. 
Hispanic-Latino communities), health professionals, and community 
outreach in this critical public health education and awareness effort.
    Second, a National Spina Bifida program offers benefits to those 
who suffer from Spina Bifida and their families by working to improve 
the outlook for a life challenged by this complicated birth defect, 
identifying valuable therapies from in utero throughout the lifespan 
and making them available and accessible to those in need. These 
secondary prevention activities represent a tangible quality-of-life 
difference to the 70,000 citizens in the U.S. with Spina Bifida. 
Focusing on living well with Spina Bifida, the initiatives as part of 
the secondary prevention program should be designed to create and 
implement strategies to improve the quality-of-life. These quality-of-
life efforts would center on reaching the general population with Spina 
Bifida, advancing treatment of Spina Bifida and its related conditions, 
and working with adolescents living with Spina Bifida to address their 
specific academic, psycho-social, and vocational needs. In addition, we 
envision that a National Spina Bifida Program would create and 
implement a comprehensive program to assist teens with Spina Bifida in 
the development of life skills for independence, self-reliance, and 
success in the world.
    Initiating a National Spina Bifida Program will help ensure that 
our Nation mounts a comprehensive effort to prevent and reduce 
suffering from Spina Bifida. SBAA and the Spina Bifida Foundation stand 
ready to work with you and your colleagues to reduce suffering and 
improve the quality of life for the 70,000 individuals and their 
families who live day in and day out with this birth defect. Again, I 
thank you for your calling this important hearing and for your support 
and leadership for establishing a National Spina Bifida Program. I 
would be happy to answer any questions you and your colleagues may 
have. Thank you.

    Senator Dodd. Dr. Green, thank you for being here.
    Dr. Green. Thank you for the opportunity to speak this 
morning, Mr. Chairman, Senator Reed.
    I am Dr. Nancy Green, medical director of the March of 
Dimes Birth Defects Foundation, and I am pleased to have the 
opportunity to speak this morning on behalf of our 3 million 
volunteers and 1,600 staff of the March of Dimes. I want to 
thank others for their comments about the March of Dimes, 
including our work not only on prevention but also treatment of 
neural tube defects and other important and serious birth 
defects.
    As you know, the March of Dimes works to improve the health 
of mothers, infants, and children by preventing birth defects 
and infant mortality.
    The Foundation is a unique partnership of scientists, 
clinicians, parents, volunteers, business and community leaders 
throughout the Nation. We also have a chapter structure in all 
50 States as well as the District of Columbia and Puerto Rico.
    The statistics that were discussing this morning on birth 
defects are very troubling, obviously, to all of us. It is a 
serious health problem facing our Nation. We take a two-pronged 
approach to this problem. One is research to identify causes 
and improve prevention tools and treatment tools, and access to 
health care for women and infants so they can best benefit from 
existing and emerging medical knowledge.
    So I want to support your efforts and those of several 
Federal agencies to prevent birth defects and to improve the 
quality of life for those born with birth defects. In addition, 
I want to use my time to urge Congress to reauthorize the Birth 
Defects Prevention Act of 1998 as a way of ensuring that this 
important work continues.
    The March of Dimes defines birth defects as an abnormality 
of structure, function, or body metabolism presenting at birth 
or in early childhood that results in physical or mental 
disability, or is fatal.
    The entire research budget of the March of Dimes supports 
basic and clinical research, the ultimate aim of which is to 
assure that every baby born is healthy. The research funded by 
the Foundation addresses basic biologic processes that underlie 
development and also examines clinical aspects of pregnancy.
    Research currently funded includes studies of developmental 
biology and genetics and those important processes leading to 
healthy birth outcomes. When these steps go awry, normal birth 
is abrogated, with consequences of miscarriage, stillbirth, or 
live births of infants with birth defects.
    So all research that is supported by the March of Dimes 
deals with prevention of birth defects. In 2001, the 
Foundation's research budget was $39.7 million. In the history 
of our grant program, awards have been made to more than 1,200 
universities including Yale, Brown, Harvard, Columbia, 
Stanford, University of California, and other fine institutions 
throughout the Nation. The average award in 2001 was $72,000.
    As a private foundation, the March of Dimes can respond 
relatively quickly to new directions or promises in research to 
accelerate the pace of scientific discovery. Previous March of 
Dimes research included identification of fetal alcohol 
syndrome, which we heard about this morning, thus establishing 
the association between birth defects and alcohol use. Fetal 
alcohol syndrome is the most common preventable birth defect in 
the United States, affecting about 4,000 infants every year.
    More recently, the Foundation has been funding professional 
and public educational outreach efforts to reach women and 
teach them about healthy behaviors during pregnancy to help 
prevent serious birth defects such as fetal alcohol syndrome.
    The March of Dimes has also funded research on fetal 
surgery, as you heard about just now, for successful 
interventions for congenital diaphragmatic hernia, as well as 
early but promising studies on spina bifida.
    In addition, the March of Dimes has funded research on 
Fragile X, including Steve Warren, who identified the Fragile X 
gene. This is the most common form of inherited mental 
retardation and has generated three important outcomes for 
Fragile X. One, it has increased the understanding in the 
medical community that some forms of mental retardation, 
including some common forms of mental retardation, are 
inherited. This leads to the ability to refer affected families 
for genetic counseling. Second, the work on Fragile X has 
launched a major research effort to identify additional genes 
and disorders causing mental retardation. And third, the work 
on Fragile X has resulted in early screening possibilities for 
this and related disorders and to begin to determine the impact 
of early interventions from that early screening for affected 
children to determine whether their intellectual development 
can in fact be augmented.
    So we are very proud of our research program, and our 
volunteers and staff vigorously support that program.
    In addition, we support Federally-funded research related 
to birth defects. As you know, the March of Dimes was a 
vigorous advocate for two recent legislative efforts on birth 
defects prevention--the Birth Defect Prevention Act of 1998 and 
the Children's Health Act of 2000, through which the Center 
that Dr. Cordero represents so ably was created.
    In addition, the March of Dimes has been reaching out to 
Congress for approval on these important measures. As you know, 
in 1998, Congress passed the Birth Defects Prevention Act with 
broad bipartisan support. This has allowed for, as I mentioned, 
creation of this National Center on Birth Defects and 
Developmental Disabilities.
    As you know, the Prevention Act of 1998 authorized 
allocations through fiscal year 2002. We understand that 
Senator Bond will be introducing the Prevention Act of 2002, 
and we urge you to support this throughout Congress to maintain 
Federal commitment to preventing birth defects and ensuring the 
quality of life for those living with birth defects. And we are 
privileged to be working closely with the Center on their 
efforts in this regard.
    To help find the causes of birth defects, Dr. Cordero 
mentioned the funding of the Centers for Birth Defects Research 
and Prevention. Four months ago, the March of Dimes testified 
before the House Appropriations Committee and submitted a 
written statement to the Senate Appropriations Committee 
requesting an increase in the budget of these centers so they 
can expand and intensify their research pertaining to genetic 
and environmental causes of birth defects.
    These seven regional centers and the eighth site at CDC are 
participating in the National Birth Defects Prevention Study, 
the largest such study ever conducted. I think this research 
holds a lot of great promise for finding etiologies of birth 
defects and appropriate prevention.
    The Birth Defects Center also supports the development, 
implementation, and expansion of State birth defects tracking 
systems, programs to prevent birth defects, and activities to 
improve access to health services for children with birth 
defects. March of Dimes chapters around the country work 
closely with these State-based birth defects surveillance 
programs.
    Finally, the ultimate goal of tracking and research is the 
development and implementation of effective programs to prevent 
birth defects and developmental disabilities. One such example 
of this success has been in the national folic acid campaign, 
led by the March of Dimes and, as Dr. Cordero mentioned already 
this morning, with the CDC and many partner organizations 
including, of course, the Spina Bifida Association. As you 
heard, we know that this campaign is getting results both 
through outreach to professionals and to the public, women of 
childbearing age, as you know so well, as well as grain 
fortification. So we are cautiously optimistic that this trend 
will continue and that there will continue to be a decrease in 
the number of children born with spina bifida and related 
neural tube defects, and we continue our work on folic acid.
    In addition to our support of the National Center, the 
March of Dimes works collaboratively with other Federal 
agencies. For instance, we have been a very outspoken advocate 
for the doubling of the NIH research budget. We also work 
closely with HRSA and CMMS on initiatives to treat children 
with birth defects and other special health care needs.
    Improving access to newborn screening is one of the 
Foundation's highest advocacy priorities here in Washington as 
well as in every State capital. This year, the March of Dimes 
asked the Appropriations Committee to allocate $25 million for 
implementation of the newborn screening program authorized in 
Title XXVI of the Children's Health Act of 2000. If adequately 
funded, this program would make it possible for States to 
develop, evaluate, and acquire innovative testing technologies 
and improve the screening, counseling and special services for 
those at risk, these newborns and children.
    In conclusion, on behalf of the March of Dimes, I want to 
thank you, Mr. Chairman, Senator Reed, for holding this hearing 
today. The March of Dimes volunteers and staff around the 
country are ready to work with you and other members of this 
committee to support public policies and provide the resources 
necessary to prevent birth defects and ensure that those who 
are born with birth defects enjoy the best life possible.
    Thank you for your attention.
    Senator Dodd. Thank you, Dr. Green, very much.
    [The prepared statement of Nancy Green, M.D. follows:]

                Prepared Statement of Nancy Green, M.D.

                              INTRODUCTION

    Good morning, Mr. Chairman. I am Dr. Nancy Green, medical director 
of the March of Dimes Birth Defects Foundation. I am pleased to have 
the opportunity to testify this morning on behalf of the over 3 million 
volunteers and 1600 staff of the March of Dimes, and share with you the 
Foundation's views on ``Birth Defects: Strategies for Prevention and 
Ensuring Quality of Life.''
    As you know, the March of Dimes is a national voluntary health 
agency founded in 1938 by President Franklin D. Roosevelt to find a 
scientific prevention of the threat of polio to the public. Today, the 
Foundation works to improve the health of mothers, infants and children 
by preventing birth defects and infant mortality through research, 
community services, education and advocacy. The March of Dimes is a 
unique partnership of scientists, clinicians, parents, members of the 
business community, and other volunteers in every state, the District 
of Columbia and Puerto Rico.
    The statistics on birth defects are very disturbing and illustrate 
a serious health problem facing our nation. Of the four million babies 
born each year in the United States, approximately 150,000, or 34 
percent of all live births, have at least one serious birth defect. 
Severe birth defects often require lifelong medical treatment. Because 
many conditions cannot be fully corrected, birth defects are a major 
cause of childhood and adult disability. Birth defects are also the 
leading cause of infant mortality. The March of Dimes believes a two-
pronged approach to prevention and treatment of birth defects is 
required: research to identify causes and improve prevention tools, and 
access to health care so women and infants can benefit from existing 
medical knowledge. I am grateful for the invitation to come before this 
subcommittee to share the March of Dimes agenda for prevention and 
treatment. I am here today to support your efforts and those of several 
Federal agencies to prevent birth defects and to improve the quality of 
life for those born with birth defects. In addition, I want to use my 
time with you to urge Congress to reauthorize the Birth Defects 
Prevention Act of 1998 as a way of ensuring that this important work 
continues.

                      BACKGROUND ON BIRTH DEFECTS

    The March of Dimes defines birth defects as an abnormality of 
structure, function or body metabolism (inborn error of body chemistry) 
presenting at birth or early childhood that results in physical or 
mental disability, or is fatal. There are more than 4,000 known birth 
defects. Both genetic and environmental factors can cause birth 
defects. In some cases, a child inherits a genetic disease when one 
parent (who has the disease) passes along a single faulty gene; 
examples of birth defects caused by dominant inheritance include Marfan 
syndrome and achondroplasia (a form of dwarfism). More frequently, a 
child inherits a genetic disease when both parents (who are healthy) 
pass along the same faulty gene; this is called recessive inheritance. 
Examples include Tay-Sachs disease and cystic fibrosis. Abnormalities 
in the number or structure of chromosomes can cause numerous birth 
defects. Down syndrome, in which a baby is born with an extra 
chromosome 21, is one of the most common chromosomal abnormalities.
    Birth defects also result from environmental factors such as drug 
or alcohol abuse, infections (such as rubella) or exposure to certain 
medications (such as the acne drug Accutane) or other chemicals. Often, 
birth defects appear to be caused by a combination of one of more genes 
and environmental factors, such as cleft lip/palate, clubfoot and some 
heart defects.
    Birth defects generally are grouped into three major categories: 
structural/metabolic, congenital infections, and other conditions. When 
a baby has a structural birth defect, some part of the body is missing 
or is malformed. Heart defects are the most common type of structural 
birth defects, affecting one baby in 125. Spina bifida affects one in 
2,000 babies. Metabolic disorders are not visible, but can be harmful 
or even fatal, and affect one in 3,500 babies. PKU (phenylketonuria) is 
an example of a metabolic disorder, in which affected babies cannot 
process a part of protein, which builds up in blood and results in 
brain damage. Fortunately, this disorder is routinely detected with 
newborn screening tests, so affected babies can be placed on a special 
diet that prevents mental retardation.
    Rubella (German measles) is probably the best known congenital 
infection that can cause birth defects. If a pregnant woman is infected 
in the first trimester, her baby has a one-in-four chance of being born 
with one or more symptoms of congenital rubella syndrome (deafness, 
mental retardation, heart defects, blindness). Fortunately, because of 
aggressive immunization programs, this syndrome has been eradicated in 
the United States and many other countries.
    Other causes of birth defects include substance abuse during 
pregnancy. For example, fetal alcohol syndrome (FAS), which affects one 
baby in 1,000 (about 4,000 babies per year in the United States) and 
results in mental and physical birth defects, is common in babies whose 
mothers are heavy drinkers of alcohol during pregnancy. FAS is the most 
common preventable birth defect in the United States. Babies of mothers 
who use cocaine early in pregnancy are also at increased risk of birth 
defects. Studies suggest that these babies are five times more likely 
to be born with urinary tract defects than babies of women who do not 
use cocaine while pregnant.

                MARCH OF DIMES RESEARCH ON BIRTH DEFECTS

    The entire research budget of the March of Dimes supports basic and 
clinical research, the ultimate aim of which is assurance that every 
baby is born healthy. Research supported by the Foundation addresses 
basic biological processes that underlie development and also examines 
clinical aspects of pregnancy. Research currently being conducted 
includes studies of developmental biology and genetics aimed at 
explaining fundamental steps that lead to healthy birth outcomes. When 
these steps go awry, normal birth is abrogated with the consequence of 
a miscarriage, still birth, or live birth of an infant with a birth 
defect. If we improve our understanding of these normal steps and their 
occasional aberration, we will be in a better position to devise means 
of prevention and intervention. Thus, all research that is supported by 
the March of Dimes deals with the prevention of birth defects.
    In 2001, the Foundation's research budget totaled $39.7 million. In 
the history of our grant program awards have been made to more than 
1,200 institutions, including Yale, University of Connecticut, 
Washington University, Harvard, Columbia University, Cornell, 
University of California--Los Angeles, Stanford, University of 
California--San Francisco, and Johns Hopkins University. The average 
award in 2001 was $72,000 per year; the median $73,000; the range 
$48,000-97,000. Typically grants are awarded for 3 years. As a private 
foundation, the March of Dimes is able to act quickly to fund creative 
new approaches, accelerating the pace of scientific discovery.
    Previous March of Dimes research achievements include 
identification of fetal alcohol syndrome (FAS), thus establishing the 
association between the birth defects and alcohol use. More recently, 
findings from research supported by the Foundation have been used to 
develop professional and public educational messages that aim to change 
unhealthy behaviors of pregnant women. The March of Dimes has also 
supported research to refine fetal surgery techniques, a successful 
intervention for congenital diaphragmatic hernia and spina bifida. In 
addition, the March of Dimes supported research on Fragile X, the most 
common form of inherited mental retardation, which has generated three 
important outcomes. First, it has increased understanding in the 
medical community that some forms of mental retardation are genetic in 
origin, leading to referral of affected families for genetic 
counseling. Second, it has launched a major research effort to 
investigate other heritable and potentially preventable forms of mental 
retardation. And, third, it has resulted in early screening for 
disorders such as Fragile X and in clinical studies to ascertain the 
impact of early interventions for affected children to determine 
whether their intellectual development can be augmented.

        FEDERALLY SPONSORED BIRTH DEFECTS RESEARCH AND SERVICES

    Although we at the March of Dimes are justifiably proud of the 
Foundation's research program, the volunteers and staff of the March of 
Dimes strongly support federally funded research related to birth 
defects and programs that provide services to those living with birth 
defects. For example, the March of Dimes was a vigorous advocate for 
two recent legislative initiatives that focus on birth defects 
prevention: the Birth Defects Prevention Act of 1998 (P.L. 105-168) and 
the Children's Health Act of 2000 (P.L. 106-310) through which the CDC 
National Center on Birth Defects and Developmental Disabilities was 
created. In addition to Chairman Dodd, Senators Kennedy and Bond who 
are members of this subcommittee were instrumental in obtaining 
Congressional approval of these important measures.

              BIRTH DEFECTS PREVENTION ACT REAUTHORIZATION

    In 1998, Congress passed the Birth Defects Prevention Act with 
broad bipartisan support. The act, first introduced by Senator Bond, 
authorized the Centers for Disease Control and Prevention to (1) 
collect, analyze, and make available data on birth defects; (2) operate 
regional centers for applied epidemiological research on the prevention 
of birth defects; and (3) inform and educate the public about the 
prevention of birth defects. CDC's current birth defects prevention 
activities described below are made possible by the Birth Defects 
Prevention Act and conducted under the auspices of the National Center 
on Birth Defects and Developmental Disabilities.
    The Birth Defects Prevention Act authorized these prevention 
programs, as well as the appropriations that support the National 
Center on Birth Defects and Developmental Disabilities, through fiscal 
year 2002. We understand that Senator Bond will be introducing the 
``Birth Defects Prevention Act of 2002'' shortly to revise and extend 
the Birth Defects Prevention Act. Mr. Chairman, the March of Dimes 
urges you and the members of the full HELP Committee to complete action 
on the Senator's bill before the end of this Congress and thereby 
maintain the Federal commitment to preventing birth defects and 
ensuring the quality of life for those living with birth defects.

    NATIONAL CENTER ON BIRTH DEFECTS AND DEVELOPMENTAL DISABILITIES

    Two years after passage of the Birth Defects Prevention Act, 
Congress took another major step toward preventing birth defects and 
improving the lives of those born with a birth defect when it created 
the National Center on Birth Defects and Developmental Disabilities 
(NCBDDD) as part of the Children's Health Act of 2000. The NCBDD was 
officially launched in April 2001. The mission of the Center is to 
improve the health of children and adults by preventing the occurrence 
of birth defects and developmental disabilities; promoting optimal 
child development; and promoting health and wellness among children and 
adults who have a disability.
    The Center focuses on identifying the yet unknown causes of birth 
defects and developmental disabilities through research programs; 
preventing birth defects and developmental disabilities through public 
as well as professional education; and promoting wellness for all 
Americans, including those with a disability. The March of Dimes is 
privileged to work closely with the Center in carrying out activities 
to meet these objectives.
    To help find the causes of birth defects, NCBDDD currently funds 
regional ``Centers for Birth Defects Research and Prevention'' in 
Arkansas, California, Iowa, Massachusetts, New Jersey, New York, and 
Texas. Each center is funded at a level of approximately $900,000 per 
year. Four months ago, the March of Dimes testified before the House 
Appropriations Committee and submitted a written statement to the 
Senate Appropriations Committee requesting an increase of $6 million in 
the budgets for these centers so that they can expand and intensify 
their research pertaining to genetic and environmental causes of birth 
defects.
    These seven regional centers and the eighth site at the CDC are 
participating in the National Birth Defects Prevention Study, the 
largest study on the causes of birth defects ever conducted. Now, with 
information collected on more than 11,000 families, the data are being 
used in studies designed to help identify the causes of birth defects. 
For example, work now underway focuses on the effectiveness of various 
methods for the primary prevention of birth defects, the mechanisms of 
inducing birth defects by various drugs, the environmental causes of 
birth defects, the genetic factors that make individuals susceptible to 
birth defects, and the behavioral causes of birth defects. Moreover 
other studies examine the costs associated with birth defects. This 
exciting leading edge research holds great promise and merits an 
increase in funding.
    NCBDDD also supports the development, implementation, and expansion 
of State birth defects tracking systems, programs to prevent birth 
defects, and activities to improve access to health services for 
children with birth defects. March of Dimes chapters around the country 
work closely with these State-based birth defects surveillance 
programs.
    Finally, the ultimate goal of tracking and research is the 
development and implementation of effective programs to prevent birth 
defects and developmental disabilities. One example of a success in 
this area is the national folic acid education campaign, lead by the 
March of Dimes, the CDC and its partner organizations, such as the 
Spina Bifida Association. This is a multi-year national education 
campaign designed to increase the number of women taking folic acid 
daily. Each year in the United States, approximately 2,500 babies are 
born with neural tube defects (NTDs). NTDs are severe birth defects of 
the brain and spinal cord, including anencephaly and spina bifida. CDC 
estimates that the annual medical care and surgical costs for persons 
with spina bifida in the United States exceed $200 million, and that up 
to 70 percent of NTDs could be prevented if all women of childbearing 
age consumed 400 micrograms of folic acid daily, beginning before 
pregnancy. We know that this campaign is getting results. In fact, a 
study published in the Journal of the American Medical Association in 
2001 showed that NTDs in newborns decreased 19 percent between 1995 and 
1999 in the wake of this campaign. However, we at the Foundation 
believe that many more fatal or disabling birth defects could be 
prevented if more women of childbearing age were to take a multivitamin 
with folic acid daily.
    The Foundation also supports the vital work of the National Center 
on Health Statistics which offers information essential for these 
research and programmatic initiatives. Investment in the National Vital 
Statistics System, a major source of information on birth outcomes and 
birth defects, would allow CDC to modernize this system using web-based 
technology that would provide for rapid compilation of data as well as 
tools to improve the accuracy and completeness of information obtained 
from physicians and hospitals. These improvements are needed to better 
detect trends in birth outcomes and to support birth defects 
registries. Additional resources would also support expansion of the 
National Survey of Family Growth to provide essential information on 
factors that affect birth outcomes.
    In addition to our support of the National Center on Birth Defects 
and Developmental Disabilities, the March of Dimes also works 
collaboratively with agencies that fall within the jurisdiction of this 
committee. In particular, the Foundation has been an outspoken advocate 
of doubling the research budget of the National Institutes of Health. 
With an increase in funding, NIH could expand its research into the 
causes of birth defects, and also the causes of prematurity. Increased 
Federal funding would also help accelerate the timetable for 
implementing a much needed analysis of environmental influences on 
child health and development that is contemplated by the National 
Children's Study authorized by this Committee in the Children's Health 
Act of 2000.
    In addition to our work on behalf of a more robust Federal research 
agenda, the March of Dimes also works closely with Health Resources and 
Services Administration and the Centers on Medicare and Medicaid 
Services on initiatives to treat children with birth defects and other 
special health care needs. Improved access to newborn screening is one 
of the Foundation's highest advocacy priorities here in Washington and 
in every State capital. One of the great advances in preventive 
medicine has been the introduction of newborn screening to identify 
certain genetic, metabolic, hormonal and/or functional conditions in 
newborns. As the Chairman knows, such disorders, if left untreated, can 
cause death, disability, mental retardation and other serious lifelong 
problems. This year the March of Dimes asked the Appropriations 
Committee to allocate $25 million for implementation of the heritable 
disorders (newborn screening) program authorized in Title XXVI of the 
Children's Health Act of 2000. If adequately funded, this program would 
make it possible for states to develop, evaluate, and acquire 
innovative testing technologies and improve these screening, 
counseling, testing and special services for at risk newborns and 
children. On June 14, 2002, a March of Dimes volunteer testified before 
this subcommittee about the importance of expanding the Federal role to 
assure that newborn screening programs in every State have access to 
the technical guidance and financial resources they need to make it 
possible for every child born in the United States to be screened using 
State of the art diagnostic and treatment tools.

                               CONCLUSION

    On behalf of the March of Dimes, I want to thank you, Mr. Chairman, 
for holding this hearing today. March of Dimes volunteers and staff 
around the country stand ready to work with you and the other members 
of this committee to support public policies and provide the resources 
necessary to prevent birth defects and ensure that those who are born 
with birth defects enjoy the best possible quality of life.

    Senator Dodd. Mr. Liguori, thank you for being here, and 
thank you, Jill, as well for coming, and Nicholas.
    Mr. Liguori. Thank you, Mr. Chairman, Senator Reed. Thank 
you very much for inviting me to testify before you here today. 
I greatly appreciate this opportunity to help provide you with 
a parent's perspective on spina bifida.
    I respectfully request that my testimony be included in the 
Congressional Record.
    Senator Dodd. It will be.
    Mr. Liguori. I am Fred Liguori from Granby, CT. I have been 
a pilot for American Airlines for 16 years. I am also a 
lieutenant colonel in the Massachusetts Air National Guard, 
where I have flown the A-10 aircraft for the past 21 years. 
However, my most important role is that of father to my 3\1/2\-
year-old son, Nicholas, who was born with spina bifida.
    As I am mindful of the time today, I will provide you with 
an abbreviated version of my family's experience with spina 
bifida. However, I would like to draw your attention to my 
written testimony, as it contains a longer, more detailed 
account and information about this all too common birth defect.
    When my wife Jill and I decided to start a family, it did 
not go as expected. After losing two pregnancies, Jill became 
pregnant for a third time. All went smoothly until an 
ultrasound exam during the 18th week. As the doctor scanned the 
baby's back, he stopped and exclaimed, ``Your baby has spina 
bifida.''
    I asked him what spina bifida was, and he explained that it 
was an opening of the baby's spine and informed us that babies 
born with spina bifida may walk as children with the aid of 
braces and crutches, but normally end up in a wheelchair as an 
adolescent. They have no control of their bowel or bladder. 
They need to have a shunt placed in their brain to control the 
hydrocephalus. And he told us to expect some degree of mental 
retardation.
    After telling us of our options, the doctor asked if we had 
any questions. My questions were: Am I awake, or is this just a 
nightmare, and if I am awake, who just hit me in the head with 
that baseball bat?
    By the next day, we began to focus on the decision before 
us, which we felt should be based on quality of life issues, 
and unfortunately, the medical professionals painted a very 
bleak picture. We spent the next few days in the public library 
and medical libraries. We read sections of textbooks and 
research papers. We contacted physicians and other health care 
professionals around the country who were involved with spina 
bifida children.
    Finally, we contacted the Spina Bifida Association of 
America, SBAA, and asked if we could talk to parents of 
children with spina bifida. They put us in contact with several 
families within the State chapter in Connecticut.
    After speaking with a number of families, we noticed a big 
discrepancy between what the medical professionals told us to 
expect and what the parents thought regarding quality of life 
of their affected children.
    One of the families invited us to meet their 9-year-old 
daughter, Jaclyn, who was born with spina bifida. We arrived at 
the house and rang the bell. As the door opened, there was a 
beautiful, smiling little girl with pink crutches and braces up 
to her hips welcoming us into their home. In that instant, we 
knew what kind of quality of life Jaclyn had.
    We spent the night talking to Jaclyn about her hobbies and 
her friends, and we spoke with her parents, Janet and Anthony, 
about her numerous surgeries and their battle with the school 
system. We left relieved that our decision was made. Now we 
needed to get as much information as possible before our son 
was born. On our way out the door, Janet handed us a copy of 
``Insights into Spina Bifida,'' a newsletter published by SBAA.
    While reading through ``Insights,'' we came upon an article 
that concerned in utero surgery on unborn fetuses to reduce the 
effects of spina bifida. The reasons for doing the surgery as 
early as possible made sense, and years of animal research 
yielded some positive results.
    After very careful consideration of the risks versus the 
hoped for rewards for both our unborn son and my wife, we 
elected to proceed with the fetal surgery at Children's 
Hospital of Philadelphia, one of the two hospitals performing 
the surgery at the time.
    After the surgery, we were to remain in Philadelphia until 
the birth of our son. The surgery was performed at 25 weeks 
gestation. Jill and I spent the next week in the hospital and 
the next 3 months at the Ronald McDonald House in Philadelphia.
    On January 14, 1999, the joy of our life, Nicholas 
Ferdinand Liguori, was born. Nicholas spent 8 days in neonatal 
intensive care. We then returned home to Connecticut. Almost 
immediately, the doctor and therapist visits began. He has been 
evaluated by a neurosurgeon, an orthopedic surgeon, a 
urologist, a cardiologist, and a host of other health care 
professionals.
    Today, Nicholas' bladder and bowels do not function 
properly. He requires two medications daily to allow his 
bladder to fill at low pressure, to prevent bladder spasms, and 
to prevent urinary tract infections. We catheterize Nicholas 
five times a day to completely empty his bladder, and we are 
working on a bowel training program to hopefully achieve social 
continence.
    At 3\1/2\ years old, Nicholas has attended spina bifida 
clinics in Connecticut, Pennsylvania, Springfield, MA, and 
Boston, MA, where he is currently treated. He receives physical 
therapy, occupational therapy, speech therapy, and 
developmental therapy--each once per week.
    Despite all this, Nicholas has what is considered a 
``mild'' case of spina bifida. He walks on his own without the 
aid of braces or crutches. Due to the positive changes that 
occurred in his brain as a result of the fetal surgery, he has 
not required a shunt. At 3\1/2\ years old, Nicholas had his 
first surgery since birth this past July 5. The little girl 
sharing his hospital room also had spina bifida, was the same 
age as Nicholas, and she was recovering from her 23rd surgery.
    As parents of a child with spina bifida, we are very 
grateful to Congress for its assistance and leadership 
regarding programs for the prevention of spina bifida and 
improving the quality of life for individuals with spina 
bifida. The establishment of a National Spina Bifida Program at 
the Centers for Disease Control and Prevention would provide a 
crucial necessary next step to help reduce the occurrence of 
spina bifida and to help those already affected.
    A national program would help improve public awareness, 
which I believe should be considered a top priority. More also 
needs to be done to improve the quality of life for those 
living with spina bifida. The number one concern of parents and 
individuals is that few improvements have been made in the area 
of bowel and bladder continence, which has devastating 
emotional and social consequences for the individual.
    A national program would also help with the establishment 
of multidisciplinary care facilities. Spina bifida is a complex 
defect that cannot be handled by ordinary health care 
professionals. With clinics established, the health care 
providers within that facility will become more experienced 
with the problems affecting spina bifida patients and how those 
problems interrelate.
    In addition, we must have educated professionals within the 
school system. Educators need to understand the types of 
learning disabilities associated with spina bifida and develop 
alternative methods of learning. They also need to teach 
independent living to those individuals who may not have a 
support network at home.
    Our collective goal should be for the majority of 
individuals with spina bifida to live long, healthy, 
independent, and productive lives.
    Finally, research into the cause of and a cure for all 
neural tube defects must be increased. Our job will not be 
complete until there is no longer a need for a National Spina 
Bifida Program.
    Again, on behalf of my son, my wife, and myself, thank you, 
Mr. Chairman, for giving me the opportunity to tell our story 
to you today. It has meant a great deal to us, and we stand 
ready to be of any assistance to you and your colleagues as you 
consider issues relating to this all too common birth defect.
    Thank you.
    Senator Dodd. Great job.
    [Applause.]
    [The prepared statement of Mr. Liguori follows:]

                   Prepared Statement of Fred Liguori

    Mr. Chairman and Members of the Subcommittee, thank you very much 
for inviting me to testify before you today. I greatly appreciate this 
opportunity to help provide you with a parent's perspective on Spina 
Bifida.
    I am Fred Liguori from Granby, Connecticut. I have been a pilot for 
American Airlines for 16 years. I also am a Lieutenant Colonel in the 
Massachusetts Air National Guard where I have flown the A-10 aircraft 
for the past twenty-one years. However, one of my most important roles 
is that of father to my three-and-a-half year old son, Nicholas, who 
was born with Spina Bifida.
    When my wife, Jill, and I decided to start a family it did not go 
as expected. After losing two pregnancies Jill became pregnant for a 
third time. We were excited but apprehensive. Given our past history, 
Jill was scheduled for routine ultrasound exams at six, twelve, and 18 
weeks into the pregnancy. All went smoothly until the eighteenth week. 
I remember looking at the screen as the technician scanned our baby's 
head thinking that it didn't look right to me--but what did I know, I'm 
a pilot not a radiologist. The technician excused herself and returned 
with the doctor.
    As the doctor scanned the baby's back he stopped and exclaimed, 
``There it is!'' He turned to us and said, ``Your baby has Spina 
Bifida.'' I asked him what Spina Bifida was and he explained that it is 
an opening of the baby's spine. I then asked, ``What does that mean? 
After our baby is born you sew up his spine and we go home, right?'' 
``Not exactly'', he said. ``Babies born with Spina Bifida may walk as 
children with the aid of braces and crutches but normally end up in a 
wheelchair as an adolescent. They have no control of their bowel or 
bladder. They need to have a shunt placed into the brain to control the 
hydrocephalus. In addition, you should expect some degree of mental 
retardation. Your options are to continue the pregnancy, continue the 
pregnancy and give the baby up for special needs adoption, or terminate 
the pregnancy. Do you have any questions?'' My questions were: am I 
awake or is this just a nightmare and, if I am awake, who just hit me 
in the head with that baseball bat?
    Next, the technician handed us a box of tissues and escorted us 
into a room across the hail. The genetics counselor came in with charts 
and graphs of ``x's'' and ``y's.'' Jill and I sat with tears in our 
eyes just wanting to go home to try to absorb what was happening.
    By the next day we began to focus on the decision before us, which 
we felt should be based on quality of life issues. We knew we had many 
questions that needed to be answered. What exactly is Spina Bifida?
    How does it affect the child? What is the quality of life for 
children with Spina Bifida? Unfortunately, the medical professionals' 
responses painted a very bleak picture.
    So, we decided to take matters into our own hands and educate 
ourselves. We spent the next few days in the public library and the 
medical libraries at UCONN, Yale and UMASS. We read sections of 
textbooks pertaining to neural tube defects and research papers in 
medical journals regarding the physical issues and cognitive 
development of children with Spina Bifida. We contacted physicians and 
other health care professionals around the country who were involved 
with Spina Bifida children. Finally, we contacted the Spina Bifida 
Association of America (SBAA) and asked if we could talk to parents of 
children with Spina Bifida. They put us in contact with several 
families within our State chapter in Connecticut.
    After speaking with a number of families we noticed a big 
discrepancy between what the medical professionals and the parents 
thought about Spina Bifida's quality of life. One of the families we 
spoke with invited us over to continue our discussion and meet their 9-
year-old daughter, Jaclyn, who had Spina Bifida. We arrived at their 
house and rang the bell. As the door opened, there was a beautiful, 
smiling little girl with pink crutches and braces to her hips welcoming 
us into their home.
    In that instant we knew what kind of quality of life Jaclyn had. We 
spent the night talking to Jaclyn about her hobbies and her friends. 
She was excited to show us her room and her American Doll collection--
including the one in the wheelchair. We spoke with her parents, Janet 
and Anthony, about her numerous surgeries and their battle with their 
school. We left relieved that our decision was made. Now we needed to 
get as much information as possible before our son was born. On our way 
out, Janet handed us a copy of 'Insights,'' a newsletter published by 
SBAA.
    A few days later, while reading through `Insights into Spina 
Bifida,' we came upon an article that was of great interest to us. It 
concerned in utero surgery on unborn fetuses to reduce the effects of 
Spina Bifida. The reasons for doing the surgery as early as possible 
made sense and years of animal research yielded some positive results. 
The article indicated that there were two centers in the country 
performing fetal surgery for Spina Bifida, Children's Hospital of 
Philadelphia (CHOP) and Vanderbilt University Hospital. We contacted 
the surgeons at both centers and traveled to both locations for 
evaluation and additional information pertaining to the surgery.
    After very careful consideration of the risks versus the hoped-for 
rewards for both our unborn son and for my wife, we elected to proceed 
with the fetal surgery at CHOP. After the surgery we would remain in 
Philadelphia until the birth of our son. The surgery was performed 
October 30, 1998 at 25 weeks gestation. Jill and I spent the next week 
in the hospital and the next 3 months at the Ronald McDonald House in 
Philadelphia. On January 14, 1999 the joy of our life, Nicholas 
Ferdinand Liguori, was born. One chapter was closed and another about 
to begin.
    Nicholas spent 8 days in neonatal intensive care. We then returned 
home to Connecticut. Almost immediately, the doctor and therapist 
visits began. We met with `Birth To 3' therapists who set a schedule 
for follow-up. At 2 months we attended our first multi-disciplinary 
Spina Bifida clinic. Actual therapy began around 6 months.
    As you might expect, Nicholas generated a lot of interest as a 
fetal surgery patient. He was evaluated by a neurosurgeon, an 
orthopedic surgeon, a urologist, a cardiologist and a host of other 
health care professionals. Of primary concern was the function of his 
kidneys and bladder. The urodynamic testing revealed high bladder 
pressures, bladder spasms, incomplete emptying of the bladder and urine 
reflux into the kidneys. We were scheduled for a head and spine MRI 
that showed a tethered spinal cord (common in children with Spina 
Bifida) and was therefore inconclusive. The neurosurgeon wanted to 
perform surgery to `de-tether', or release, the spinal cord. We wanted 
another opinion. After additional tests and consultations in 
Philadelphia and Boston, it was decided that surgery was not required 
but urologic intervention was necessary.
    Today, Nicholas' bladder and bowels do not function properly. He 
requires two medications daily to allow his bladder to fill at low 
pressure, to prevent bladder spasms and to prevent urinary tract 
infections. We catheterize Nicholas five times each day to completely 
empty his bladder and we are working on a bowel-training program to 
hopefully achieve social continence. At three-and-a-half years old 
Nicholas has attended Spina Bifida clinics in Connecticut, 
Pennsylvania, Springfield Massachusetts, and Boston, where he is 
currently treated. He receives physical therapy, occupational therapy, 
speech therapy and developmental therapy--each once per week.
    Despite all of this, believe it or not, Nicholas has what is 
considered a ``mild'' case of Spina Bifida. He walks on his own without 
the aid of braces or crutches. Due to the positive changes that 
occurred in his brain as a result of the fetal surgery, Nicholas has 
not required a shunt. At three-and-a-half years old Nicholas had his 
first surgery since birth this past July 5th. The little girl sharing 
his room also had Spina Bifida, was the same age, and was recovering 
from her 23rd surgery. Can you imagine the physical, emotional, and 
financial toll that must have on such children and their families?
    With Spina Bifida, the physical ability you have today may be gone 
tomorrow. It is an ever-changing defect, which rarely, if ever, 
improves. The main problems that plague individuals with Spina Bifida 
fall into four main categories: neurological, orthopedic, urologic and 
developmental. Most children with Spina Bifida have a tethered spinal 
cord, meaning the cord is attached usually at the level of the spinal 
defect. This mainly occurs as a result of scarring from the original 
closure surgery. As the child grows their spinal cord is stretched. 
This often requires surgery to de-tether, or release, the spinal cord 
and occasionally surgery to decompress the brainstem where it enters 
the top of the spinal column. These are extremely critical procedures 
with a high degree of risk. Left uncorrected, they can result in loss 
of motor function, paralysis, and in the case of brainstem compression, 
even death. Chronic bladder problems and urinary tract infections can 
result in loss of kidney function. Furthermore, the problems associated 
with incomplete bowel and bladder control can have a huge effect on an 
individual's self-esteem and overall social interaction. Finally, shunt 
malfunctions and infections can result in increased developmental 
delays.
    As parents of a child with Spina Bifida, we are very grateful to 
Congress for its assistance and leadership regarding helping to reduce 
and prevent Spina Bifida and improving the quality of life for 
individuals with Spina Bifida. The Early Childhood Intervention program 
(ECI), in particular, has made a big difference in the early lives of 
children with Spina Bifida. However, there is a lot more that needs to 
be done with respect to prevention and quality of life issues.
    A Federal allocation of $3 million in fiscal year 2003 for a 
National Spina Bifida Program would provide a crucial necessary next 
step to help reduce the occurrence of Spina Bifida and to help those 
already affected. Such a national program would help improve public 
awareness--which I believe should be considered a top priority. Most 
people I have spoken with during ``awareness events'' have never heard 
of Spina Bifida. Once the public knows what Spina Bifida is and 
understands how it affects the individual, prevention will evolve 
automatically.
    More also needs to be done to improve the quality of life for those 
individuals and families--such as mine--living with Spina Bifida. The 
No. 1 concern from parents and individuals is that few improvements 
have been made in the area of bowel and bladder continence. This is a 
problem that has devastating emotional and social consequences for the 
individual. Families often have to change plans because a facility or 
area is not equipped to handle the needs of an individual with Spina 
Bifida. Children with Spina Bifida often are excluded from events and 
activities because a facility or even a friend's home cannot 
accommodate them and their needs.
    A National Spina Bifida Program also will help with the 
establishment of multi-disciplinary care facilities. Spina Bifida is a 
complex defect that cannot be handled by ordinary healthcare 
professionals. With clinics established, the healthcare providers 
within that facility will become more experienced with the problems 
affecting Spina Bifida patients and how they interrelate. Through the 
National Center for Birth Defects and Developmental Disabilities at the 
Centers for Disease Control and Prevention, health care providers can 
become better educated about the full range of issues and challenges 
that face individuals and families affected by Spina Bifida. This in 
turn with help improve the quality of care they receive and 
subsequently improve quality of life.
    In addition, we must have educated professionals within the school 
systems. Educators need to understand the types of learning 
disabilities associated with Spina Bifida and develop appropriate 
alternative methods of learning. They also need to teach independent 
living to those individuals who may not have a support network at home. 
Our collective goal should be for the majority of individuals with 
Spina Bifida to live long, healthy, independent, and productive lives.
    Lastly, research into the cause of--and a cure for--all neural tube 
defects must be increased. Our job will not be complete until there is 
no longer a need for a National Spina Bifida Program.
    Again, on behalf of my son, my wife, and myself, thank you Mr. 
Chairman for giving me the opportunity to tell our story to you today. 
It has meant a great deal to us and we stand ready to be of any 
assistance to you and your colleagues as you consider issues relating 
to this all too common birth defect.
    Thank you.

    Senator Dodd. Let me turn to my colleague, Jack Reed, who 
has some other obligations, so I am going to have him go first 
with his questions, and I will follow up with some. I thank all 
three of you.
    Senator Reed. Thank you, Mr. Chairman.
    I concur that it was extraordinary testimony by Mr. Pote, 
Dr. Green, and Mr. Liguori. Thank you so much. And let me once 
again thank the chairman for holding this hearing, but not just 
this hearing--I cannot think of any of my colleagues who has 
been more committed to helping the welfare and the health of 
children throughout their career than Chris Dodd, so thank you, 
Mr. Chairman, for that and for all that you do.
    Let me raise just two questions. First, we talk about the 
cost of birth defects to the child and to the society, but it 
strikes me that any child with an illness of this severity has 
huge costs to other children in the family, to the family 
itself, costs that are very difficult to objectify, to put into 
numbers. But Mr. Pote and Mr. Liguori, if you could just 
comment on the impacts and the costs in regard to other 
siblings that we have to capture, because this too is a burden 
that we have to address.
    Mr. Pote. I will just offer a couple of personal 
observations and then generalize it a bit. There are two 
significant impacts that we see in our families, and we are 
very lucky in our family--my older nephew, Tim, who is a very 
strong young man and has been a very important part of helping 
his family deal with the challenges of spina bifida. But that 
is not always the case. Many siblings grow up feeling like they 
are not getting the attention from especially their mother, 
because the other important impact here is that the nature of 
spina bifida--and you heard Fred list the number of care 
providers that their family has to deal with--one of our 
chapter folks in Nashville told me one day that there are 15 
doctors that she regularly visits with her son. It is a full-
time job for one member of the family--it is almost always the 
mother, as it turns out--and that has tremendous demoralizing 
impacts. Women have given up their careers. They often feel 
guilty that they are not spending time with their other kids, 
and the siblings often feel like they are not getting their 
fair share. Those are all very important psychological issues.
    Senator Reed. Indeed. Thank you so much for your testimony.
    Mr. Liguori, again, thank you for your testimony, and thank 
you also for flying those A-10s. That helps a great deal.
    Mr. Liguori. Thank you, Senator.
    Senator Reeds. Could we have your comments, because you see 
it as a parent--and by the way, I have the same tractor at home 
for myself; it is a perfect size for me.
    [Laughter.]
    Go ahead.
    Mr. Liguori. That is his favorite.
    As far as how it affects other children, it is a little 
early for us to tell. We do have another child who is 8 months 
old--he is not here today, thank God.
    [Laughter.]
    He is not quite as mellow as Nicholas. But we do see 
already that Nicholas does require a lot of attention just from 
the therapies that he receives, and of course, now that he has 
transitioned from birth to 3 into our local school system in 
Granby, which is doing a phenomenal job, he does receive 
therapy just about every day. So that means loading everybody 
into the car, taking him to the school, and it does take a lot 
of time away from the second child.
    As far as the health care costs, right now at American 
Airlines, we have a $1 million lifetime cap on our health 
insurance. That is something that I am very concerned about 
with Nicholas, and we do everything we can to keep his health 
care costs as low as possible, because that can be a factor.
    One big thing with spina bifida is that it is an ever-
changing birth defect, and just because he is doing so well 
today does not mean that that is the case tomorrow, next week, 
or next year. That can change.
    So those are our biggest concerns. We will see how it is 
when he gets a little older, how it affects our family. Like I 
say, right now, he does require a lot of extra care, and 
certainly the cost is a factor for us.
    Senator Reed. Thank you so much.
    The other point that I think both you, Mr. Liguori, and Mr. 
Pote made is that the progress and the response from families 
that are well-educated and affluent and have access to health 
care is challenging, but just think of families who do not have 
a health care plan, and maybe do not have English as their 
first language. It is a huge challenge, and that is our 
responsibility; that is where we have to step up to the plate, 
particularly helping those families.
    Dr. Green, it is encouraging to hear about Nicholas' 
surgery, and I wonder if you might talk about the impact of in 
utero surgery in terms of limiting the number of surgeries 
going forward and potentially alleviating many of the symptoms 
of spina bifida. Could you comment on that?
    Dr. Green. Sure. Fetal surgery is not all that new, and in 
fact it has been very well-established for quite successful 
treatment of other disorders that I mentioned--congenital 
diaphragmatic hernia, urinary tract obstructions. So that while 
there are issues which I think you heard around risks of pre-
term birth, and so on, I think it is a well-established option 
for a number of different birth defects.
    The spina bifida story is still early, as I mentioned. 
There are procedures being done in three centers, and as Mr. 
Pote correctly mentioned, the data seem most encouraging about 
one of the major difficulties with spina bifida being the need 
for a shunt for draining spinal fluid from the head, and it 
actually goes into the abdomen. I can tell you from taking care 
of lots of kids with those shunts that they are often a problem 
and result in innumerable surgeries and problems as well as 
potential association with learning problems and mental 
retardation if shunts are not adequately taken care of.
    So the early data is that the fetal surgery may help avoid 
the need for a shunt and some of the complications associated 
with it. But again, it is early, and these programs are being 
evaluated, so at this point, it is at the clinical study stage.
    Actually, there was a report in this week's New England 
Journal about three kids who had had fetal surgery for spina 
bifida, and they developed another condition associated with 
the surgery that they may or may not have had had they not had 
this fetal surgery, which in two of the three cases was 
treatable with another surgery, in one of the cases not.
    So I think there is a lot to be learned and that we need to 
be cautious and study this very carefully, both in terms of 
short-term as well as long-term outcomes. The long-term 
outcomes, it is too early to say, so we do not know.
    Senator Reed. The other point and my last question--because 
the chairman has been very kind to let me go first--is there 
are three centers doing this type of surgery, which underscores 
the reality that to get access to the sophisticated health care 
and the counseling, you have got to be fortunate enough to live 
in one of these cities. Probably if you are in a rural area, 
you would be unlikely to logistically be able to access these 
services. You have got to be fortunate to live in a school 
system that is really--and Granby, I think, is--sensitive and 
is working with the families.
    So we have a huge challenge to whatever we develop in terms 
of medical responses and educational responses to make sure 
that they are diffused fairly across the country. Otherwise, 
rural Americans and poor Americans--even if you are doing 
everything you can, you do not have the resources available.
    Does that make sense? I see affirmation.
    Mr. Chairman, thank you.
    Senator Dodd. Thank you, Jack, very much for your presence 
here today and your commitment to this issue as well. It has 
been tremendously helpful to have you with us here this 
morning.
    I probably should have noted at the outset that one of the 
reasons that I have been involved in this issue is because I 
have been pleased every year for I do not know how many years 
now--I am trying to recall the number--I have been the honorary 
co-chairman with Bob Dole, my wonderful friend and colleague, 
former Senator, of the spina bifida dinner we have each year to 
raise dollars. I have enjoyed those roasts that we have here in 
Washington, where I have mostly been a ``roaster''--and by the 
way, that is not an invitation for me to become the 
``roastee,'' having seen what they do to people.
    Mr. Pote. It has been duly noted.
    [Laughter.]
    Senator Dodd. I am glad to be the honorary co-chair with 
Bob Dole and those involved. Judy Woodruff and Al Hunt are 
great friends, and they have done a wonderful job bringing us 
all together for that particular event.
    Let me run down some questions for you--and this is an 
informal enough setting here so I want you to jump in and 
comment on these matters if you feel so inclined.
    Let me begin with you, Mr. Pote. You mentioned in your 
statement that prevention, intervention and treatment therapies 
must be identified, and as you said in your statement, in order 
to identify these, it would require, in your view, either new 
medical breakthroughs, more research, or just funding.
    Where should the emphasis be from our standpoint?
    Mr. Pote. I will just mention two, and I will reinforce 
something that Dr. Cordero mentioned. We will be cofunding and 
participating with CDC, NIH, and AHRQ in the coming year in a 
consensus conference which is, as you know--and you probably 
know this better than I--designed to bring together health care 
professionals, scientists and researchers, public health 
professionals, and members of our community to figure out what 
is it that we know about best practices, and what do we not 
know, and then to establish the agenda which hopefully will 
help us, with Dr. Cordero, figure out what the priorities are, 
but then will also lead to the promulgation of best practices 
with the participation of the academies and the Agency for 
Health Care Research and Quality.
    So from the point of view of our families, I would say that 
our number one priority now is that the quality of care 
delivered to our families around the country is very uneven, 
and we need to establish those standards.
    The second issue--and this is also why it is very 
complicated--is that there is no such thing as a spina bifida 
secondary condition. It is a urological or a neurological or a 
dermatological, and that makes it difficult for us to 
specifically pinpoint what we need to pursue next. That is why, 
as Dr. Green said, with the help of people like the March of 
Dimes and these other professional academics, we can focus on 
the two or three most important secondary conditions in each of 
those areas of treatment and make sure we have identified the 
research priorities to bring the level of care up in each of 
those specific areas.
    Senator Dodd. That is great.
    Tell me--and this seems sort of self-evident, but I would 
like you to explain why you think this is important for an 
audience that may not be familiar with this and wants to know 
more--I find the idea of locating the National Spina Bifida 
Program in the National Center to be a wise one. But tell me 
what you think that is going to mean for spina bifida families. 
What does it really do for those living with spina bifida? What 
sort of difference does that make?
    Mr. Pote. I think that it helps raise awareness, first of 
all. The fact that we are sitting here in 2002 talking about 
this issue says something about the level of awareness, and we 
in the spina bifida community accept our share of that 
responsibility, because we need to make sure this is a higher 
priority item.
    I think the most important outcome of that center will be 
that we will be able to once and for all establish a clear 
national agenda and will be able to focus the efforts of the 
legislative world, the medical community, and the research 
community and make sure we are dealing with the most important 
issues in a very coordinated and focused way.
    Senator Dodd. That is a good enough reason.
    Dr. Green, do you have any comments on that?
    Dr. Green. I will give you three statistics. Up to 70 
percent of neural tube defects can be prevented with folic 
acid. As Dr. Cordero mentioned, there is a 31 percent decrease. 
So there is a gap between what could be prevented and what has 
been prevented. And the third statistic is that only one-third 
of women of childbearing age take folic acid during the 
preconception period.
    So we want to get to 100 percent prevention, so we need 
more research on what the other causes are that cannot be 
prevented by folic acid; we need to reach out to the public 
through health care providers directly to the public to get as 
much prevention as possible.
    And I think Dr. Cordero's center at CDC is well-poised to 
do that and also to do the surveillance.
    Senator Dodd. Mr. Pote, in your written testimony, you 
mention that ``With appropriate, affordable, high-quality 
medical, physical, and emotional care, most people born with 
spina bifida can lead relatively normal lives.''
    And your testimony, Mr. Liguori, about the difference 
between what the medical people told you and what you 
encountered with the family in Connecticut that you met was 
certainly instructive. It is also clear from your statement 
that ensuring access to this care is obviously necessary if we 
are to improve the quality of life for people.
    But my question is this. How do you know how affordable it 
must be to be truly accessible? This is a complicated question, 
but I am trying to get some sense of this--and are there other 
obstacles such as lack of trained specialists--you have 
mentioned that to some degree already--which make this care 
inaccessible to those with spina bifida.
    I would like all three of you to comment on that if you 
can.
    Mr. Pote. I guess the point that I would emphasize is that 
once quality of care is accessible because the health community 
has been trained to the same level of awareness and skill, I 
think the next issue, then, is making it clear to folks who 
have a child with spina bifida that there are places where they 
can go to get that kind of specialized attention.
    The third issue is how do they pay for it. And as you 
mentioned earlier, Fred is fortunate enough to have a good, 
solid private insurance policy. The good news on those policies 
is that for the most part, they do fund what they should be 
funding, and as you know, that is not always the case. That is 
not to say that the families do not have to fight to get there, 
but most of those policies do pay where they should. The issue 
is the number of people who are not covered by those kinds of 
policies and are therefore totally reliant on the public safety 
net, and as you know here also, that does not always result in 
the highest-quality care.
    Senator Dodd. You mentioned earlier, Mr. Liguori, that the 
Granby school system has been pretty good. We have been trying 
up here for the last number of years--I started about 10 years 
ago--to have the Federal Government live up to its commitment 
on special education costs. We have not gotten there yet, and 
when I go back home, and I meet with my first selectmen and 
mayors in the 169 cities and towns in Connecticut, one issue 
that is right at the top of the list all the time for them is 
special education dollars. And we are getting better at this--
we are at about 12, 13 percent. We tried last year in the 
Elementary and Secondary Education Act to get those dollars up, 
and the Senate was overwhelmingly in support of it, but the 
House was not, and we did not have a lot of support out of the 
administration on this. I am hopeful that this year, that may 
change.
    There are a lot of problems obviously with the fiscal 
issues associated with that. But I have also been concerned 
about how in communities, because of the costs associated with 
special education, in smaller towns, the pressures that can be 
exerted because people start to complain about the cost of 
taking care of a handful of children.
    Tell me about the response in the community of Granby. 
Obviously, it has been a good one, but I would like people to 
hear about how that happened. How do you educate people about 
the importance of Nicholas getting the kind of education that 
the Granby school system is giving him?
    Mr. Liguori. I have to go back to what Mr. Pote said. I 
think it comes back to public awareness. Public awareness is 
the most important thing in my mind, because I think that once 
the public is aware of what spina bifida is and how it affects 
children, you will see a change in prevention, you will see a 
change in fundraising--all that stuff will follow on, and it 
will also help to educate people on what the defect is, the 
developmental disabilities of these children, and the needs 
that they have in the school system.
    We were with the birth to 3 program initially in 
Connecticut when we got back, and we were quite happy with the 
care that Nicholas was getting at that time--we did not know 
anything better, though--but he was continuing to fall behind 
developmentally. Once he turned 3, and he stepped into the 
Granby school system, they came up with a program for him that 
was significantly more than what birth to 3 was giving him, and 
he has just started that--he is only 3\1/2\ years old--but we 
are very happy with the system that he has, and we hope to see 
him improve quite a bit.
    But to answer your question, I think it just comes back to 
raising public awareness on the defect and how it affects 
children.
    Senator Dodd. I probably should have said this ahead of 
time, for those people who are not from Connecticut or from the 
area--Granby is a small town. It is not a large metropolis in 
the State of Connecticut, like Hartford or Bridgeport. Granby 
is a small community. So education is important, and local 
support is based on people being aware of the kind of 
assistance and support that Nicholas would need. And that made 
a difference in terms of the system being willing to step up to 
the plate and see that he gets that kind of help.
    Mr. Liguori. Yes. And Senator, if I could make one more 
comment as well, as far as the health care professionals, like 
I said, a lot of the information that we got from them 
conflicted with the information that we got from the parents. 
And again, I think that goes to education, because a lot of the 
information that we did get from the professionals we were 
dealing with was--I do not want to say it was incorrect--it was 
old information. And with early intervention and 
multidisciplinary care, the children today do much better than 
they did 20 years ago.
    Senator Dodd. Dr. Green, why don't you pick up on that 
point? I would love to see us do something in this 
reauthorization that did a lot more in terms of providing that 
kind of support for health care professionals to have a far 
better understanding of these issues. What can we do that will 
achieve that? It seems to me that that is a major question 
here, and we need to put more emphasis on it.
    Dr. Green. I think Dr. Cordero mentioned the program with 
the American Academy of Pediatrics and his Center, and that is 
a major mechanism for outreach to professionals, for 
pediatricians. It would also seem that it is important to reach 
out to the obstetrics side as well, perhaps to the American 
College of Ob/Gyn, since many of these decisions are made prior 
to the delivery of the child.
    In fact, the March of Dimes has been working with ACOG, the 
College of Ob/Gyn, on educational efforts in this and other 
aspects of health care for infants and children. That is not 
it--obviously, outreach needs to be made to the nursing staff, 
and as you have heard, there is a whole spectrum of health care 
needs and health care providers involved in care for children 
with serious birth defects like spina bifida. So educational 
outreach really needs to be directed at this spectrum so that 
you get coordinated care.
    Senator Dodd. Let me ask three other quick questions if I 
can. One, can you go into a little more detail about what is 
being done on the research side--we have talked about it--but 
what areas of research are showing some real promise in the 
prevention or treatment in the area of birth defects?
    Dr. Green. The question came up earlier about genomics, and 
certainly identifying genes so there can be screening and 
prediction of course--maybe some children are affected with 
specific birth defects because of some genes in their mothers 
or themselves. The genetic diversity may help to explain some 
of the spectrum of disorder, so that is important to know in 
terms of making decisions, planning treatments and 
interventions.
    So that, for instance, the 30 percent or so of women who 
are resistant to folic acid, so are at risk of having affected 
pregnancies, despite folic acid may need ten times the dose of 
folic acid or more, or other kinds of medical interventions for 
prevention.
    So I think that gene identification is critical for 
prevention as well as for prediction of clinical course and 
therefore allows families to plan. So that is one aspect.
    And certainly fetal surgery, as we have heard, has been 
important in a careful approach to assessing impact on these 
birth defects.
    And then, the other issue is we should remember that 
research involves surveillance and keeping track, so for 
instance, that is how we know that Hispanics are at greater 
risk of having children affected by neural tube defects, and 
that has then led to outreach to providers and outreach to 
communities. So I think that surveillance is a very important 
aspect of this that the CDC helps to provide.
    Senator Dodd. The March of Dimes and the public educational 
messages that they have developed over the years for newly 
pregnant parents--have they been targeted to them, or is it 
sort of a general message?
    Dr. Green. It is an interesting question, because if you 
think about folic acid needing to be taken by women of 
childbearing age, once you know that you are pregnant, if you 
start taking folic acid, it may be too late. Then, you have to 
really reach women from the ages of 16 to 45. That is very 
challenging.
    If you think about other disorders like fetal alcohol 
syndrome, which was brought up today, similarly, you have to 
reach women early, although a lot of the public outreach on 
fetal alcohol and also smoking that is associated with pre-term 
delivery and low birthweight of children, you can target more 
toward pregnant women.
    It is obviously easier to reach more well-defined 
populations, and pregnant women are known to be more receptive 
to educational messages. They want healthy babies as well. So 
it depends on the disorder and the cause.
    Some disorders associated with, say, medications--the 
thalidomide story is old, but one of the more active stories is 
with accutane, an acne medication. Again, you have to deal with 
the young adult female population. So one size does not fit all 
for this.
    Mr. Pote. Senator, I would just add in terms of some of the 
outreach that the March of Dimes is doing, that next time you 
are in an airport, look for Daisy Fuentes talking about folic 
acid. And I am sure that, like me, you are an avid watcher of 
MTV, and you are very familiar with Daisy Fuentes, but she is 
exactly the kind of spokesperson that we need--a young, 
attractive Hispanic woman who is ``cool,'' and it is ``cool'' 
to take folic acid.
    One of the things that we have said about food 
fortification is that if we could ever find a way to fortify 
soft drinks with folic acid, or if we could find a way to 
convince young women that--
    Senator Dodd. I was curious about that, whether we could 
incorporate it in food sources, working with the Department of 
Agriculture and others, so that this could become as much of a 
conscious choice as just normal eating habits and patterns.
    Mr. Pote. You find it today in breads and cereals, and if 
you look on the back of most cereal boxes, I believe that what 
you will see is 170 micrograms in many cases--
    Senator Dodd. Not the 400.
    Mr. Pote [continuing]. Not the 400--and then, of course, 
the question is when you look at the population that we are 
targeting, are they really eating the recommended daily dosage 
of breads and other grains than cereals.
    Senator Dodd. One thing you might think about doing--I just 
spoke over the weekend at the national convention of La Raza in 
Miami, and 2 weeks before that, I spoke at the national 
convention of LULAC. I was in the Peace Corps, so I have a 
strong interest in the culture and history. And I would think 
that through Telemundo and these other groups, and the 
``novellas'' which are tremendously popular, obviously, in any 
language--these are the soap operas and so forth--among younger 
women, these are tremendously popular--I would bet that if you 
talk to these folks, with Dr. Cordero, about how you might 
incorporate it as part of the commercial programming, as part 
of the stories, it would have a tremendous impact.
    With all due respect--and I am very grateful to CSPAN for 
covering a hearing like this--but the audience that we are 
going to reach probably is not going to be the one that we 
would like to be reaching. Maybe we should do this with 
simultaneous translation for people who speak other languages. 
But nonetheless, there is a greater likelihood, I think, 
through the commercial popular programming that we are able to 
get a better education through, and I bet if we had some 
contacts with them, they would be more than willing, 
particularly with the information you have shared with us here 
today about the particularly high incidence in the Hispanic-
Latino community. I would bet you would have a tremendous 
response to this, and I will be happy to join you in getting in 
touch with them, if you would like to do that.
    Mr. Pote. Thank you.
    Senator Dodd. I apologize--this could go on and on. I have 
been so impressed with all of you. I am tremendously impressed 
with you, Mr. Pote, for the work that you have done, a 
tremendous effort.
    And Dr. Green, we are excited to have you with the March of 
Dimes, with your background as a pediatrician.
    It has been great to have Nicholas and Greg here with us. I 
cannot tell you how much I appreciate having these young people 
with us today. It makes a big difference.
    And Mr. Liguori, you and Jill are terrific parents.
    Mr. Liguori. Thank you, Senator.
    Senator Dodd. Okay. We will leave the record open for some 
additional questions.
    I thank all of you for coming, and the committee stands 
adjourned.
    [Additional material follows.]

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    [Whereupon, at 11:34 a.m., the subcommittee was adjourned.]
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