[Congressional Record Volume 150, Number 52 (Wednesday, April 21, 2004)]
[Extensions of Remarks]
[Page E584]
From the Congressional Record Online through the Government Publishing Office [www.gpo.gov]




       NATIONAL PRIMARY IMMUNE DEFICIENCY DISEASES AWARENESS WEEK

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                         HON. SHERWOOD BOEHLERT

                              of new york

                    in the house of representatives

                       Wednesday, April 21, 2004

  Mr. BOEHLERT. Mr. Speaker, I rise today to ask my colleagues to join 
me in recognizing the week of April 19th as National Primary Immune 
Deficiency Diseases Awareness Week. Primary immune deficiency diseases 
(PIDD) are genetic disorders in which part of the body's immune system 
is missing or does not function properly. The World Health Organization 
recognizes more than 150 primary immune diseases, which affect as many 
as 50,000 people in the United States.
  I am familiar with primary immune deficiencies because of a family in 
my district, the Driscolls. Kerstin and Dean Driscoll are from Greene, 
NY and have two sons who were born with an extremely rare PIDD called 
X-Linked Agammaglobulinemia, or XLA. Zack, 11, and Alex, 9, are 
confronting XLA head on. XLA is a genetic disorder that prevents 
production of B-cell antibodies that fight infection. When Zack was 3 
years old and Alex only 9 months, they suffered chronic ear and sinus 
infections. Their recurring illnesses remained a mystery to their 
parents and doctors, until tests were done to confirm that the boys had 
primary immune deficiency diseases.
  Soon after their diagnosis, Zack and Alex were treated with an immune 
globulin infusion (IGIV), which is prepared from the plasma of many 
blood donors, to support their immune systems. Zack and Alex receive 
their IGIV infusions once every 3 weeks and the infusion takes 
approximately 4 hours.
  Zack and Alex are lucky because their primary immune deficiency 
disease was diagnosed early. However, despite the recent progress in 
PIDD research, the average length of time between the onset of symptoms 
in a patient and a definitive diagnosis of PIDD is 9.2 years. In the 
interim, those afflicted may suffer irreversible damage to internal 
organs. That is why it is critical that we raise awareness about these 
illnesses.
  Mr. Speaker, the Driscolls face their sons' PIDD head on, by becoming 
active with the Immune Deficiency Foundation. Therefore, I commend the 
Immune Deficiency Foundation for its leadership in this area and I am 
proud to join them in recognizing the week of April 19th as National 
Primary Immune Deficiency Diseases Awareness Week. I encourage my 
colleagues to work with us to help improve the quality of life for PIDD 
patients and their families.

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