Benefits of newborn screening for cystic fibrosis in Shreveport, Louisiana, Cystic Fibrosis Center.
BACKGROUND
Cystic Fibrosis (CF) is the second most common, life-shortening, inherited disorder occurring in childhood in the United States, after sickle cell anemia. More than 10 million Americans are unknowing, symptomless carriers of the defective cystic fibrosis gene. Cystic Fibrosis was included in the newborn heel stick screening in Louisiana, beginning July 2007.


OBJECTIVE
1) To assess and compare the nutritional status of Cystic Fibrosis patients in the newborn screened cohort (NBS) compared to the non-newborn screened cohort (non-NBS) by measuring their weight for length/height percentile at 1, 2, and 3 years of age, 2) To compare the vitamin levels of CF patients in these two groups, 3) To compare the number of hospitalizations in the two groups, and 4) To compare the incidence of colonization of significant bacteria in the airways in these groups.


METHODS
Out of 91 CF patients, 26 were excluded due to insufficient data. The sample was stratified into two groups; 1) Newborn screen (July 2007 to present) and 2) Non-Newborn screen (1971 to present). Recording and comparison of their weight for height or length; percent of patients showing decreased fat soluble vitamin A, D, and E levels; rate of hospitalization; and cultures at 1- 3 years of age were made.


RESULTS
CF patients below 50th percentile of weight for height/length were 48.88% in Non-NBS and 33.33% in NBS at one year, 47.73% in Non-NBS and 40% in NBS at two years, and 45.45% in Non-NBS and 25% in NBS at three years. CF patients below 10th percentile of weight for height/length were 13.33% in the Non-NBS and 5% in NBS at one year, 11.66% in Non-NBS and 0% in NBS at two years, and 6.81% in Non-NBS and 8.33% in NBS at three years of age. There was also reduction of vitamin A, D, and E deficiencies at one to three years in NBS compared to Non-NBS. However, these results were not statistically significant. The hospitalization rate of CF patients during the first three years of life was 86.66% in Non-NBS as compared to 68.42% in NBS. The colonization of (i) MRSA was 18.75% in Non-NBS and 36.84% in NBS, and (ii) Pseudomonas aeruginosa in Non-NBS was 59.37% and 36.84% in NBS at any point during the first three years.


CONCLUSIONS
The study has shown that there has been an improvement in the nutritional status shown by a decrease in the percentage of patients below the 50th percentile and below 10th percentile of weight for height at one to three years of age after the implementation of NBS three years ago. There has also been a reduction in the vitamin deficiencies, hospitalizations, and colonization by Pseudomonas aeruginosa among the screened patients as compared to the CF patients diagnosed clinically.
Department of Pediatrics, Pulmonary Diseases Section, Louisiana State University Health Sciences Center, Shreveport, USA.
Jyothsna Akam
JA
Venkata
Kimberly L
KL
Jones
D001827:Body Height; D001835:Body Weight; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis*; D006304:Health Status; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008145:Louisiana; D015997:Neonatal Screening; D009752:Nutritional Status; D012189:Retrospective Studies