Baby Brady case inspires representative to sponsor HB 716, additional screenings

Representative Tom Todd's is currently the chief sponsor of recently proposed House Bill 716, which was introduced in a Health Care Policy Committee meeting last week.

The bill establishes the Brady Alan Cunningham Newborn Screening Act, which will require, by July 1, 2010, the Department of Health and Senior Services to expand newborn screening requirements to include certain lysosomal storage diseases.

The department will be authorized to increase the current fee associated with newborn screening tests, in order to cover additional costs of the expanded newborn tests, according to Todd. Currently in Missouri, 67 disorders are screened, however, the bill will add five more disorders to the screening list.

According to Todd, the hospitals will not see anything different, as the blood drawn from your baby's ankle after birth will not change in amounts. A drop of this same blood will be used for the new testing at the state lab.

Brady, the baby the bill is named after, is 10-months-old and lives with his parents in District 163. At birth, he appeared to be picture perfect, and continued to be the typical baby of every parent's dream until the age of four months, according to the family. At that time, Brady started crying non-stop, not smiling, eating, and was no longer able to suck or swallow. One month after visiting the emergency room, Brady was diagnosed with leukodystrophy or Krabbe, a disease that destroys the central nervous system, according to Todd.

The life expectancy for children with this disease is about 13-14 months. Currently, Brady can still open his eyes and has some eye movement. He can still hear but eventually will lose both eyesight and hearing. Brady must receive his food through a feeding tube and is on Morphine and Ativan every four hours for pain. He also takes chloral hydrate at night in order to sleep.

Eventually respiratory complications will begin, according to doctors.

All of this could have been avoided if Missouri had the proposed bill in effect, according to Todd.

"A minimum of 10 children in Missouri are born each year with the five metabolic disorders addressed in House Bill 716, referred to as lysosomal storage disorders," added Todd. These disorders can be invariably fatal if treatment is not instituted promptly. In the case of Krabbe disease, treatment is only effective if provided prior to the onset of any clinical symptoms. The hope for children with these disorders and the hope for their families is newborn screening, according to the state representative.

"I am hoping insurance companies will be on board with us," Todd said. "I can't imagine testing for $50 to not outweigh the costs incurred once the disease is allowed to progress."

Todd added that it is important to remember that children will be born with these diseases whether or not there is medical screening. "They will continue to suffer and die if undiagnosed, but with early detection and effective treatment obtained, Krabbe disease no longer needs to bring death and despair to those who receive its diagnosis," Todd said.