Impact of Supplemental Therapies and Nutritional Support on the Duration of Nasogastric Feedings in Infants with Prader-willi syndrome

AUTHORS: LaFuente N^*, Orellana D^*, Lee PDK^@, Klish W^*, Scheimann, AO⁺.

Affilitiations:

^*Baylor College of Medicine, Houston, TX

⁺ Johns Hopkins School of Medicine, Baltimore, MD

^@ University of Calfornia-Los Angeles School of Medicine, Los Angeles

Introduction:  Prader-Willi syndrome(PWS) is a complex genetic disorder characterized by failure to thrive during infancy followed by insatiable appetite and weight gain after 2 yrs of age.  Nasogastric tube feedings has been the standard approach with neonates when failure to thrive becomes life threatening, including the Prader-Willi infant who will have decreased energy needs.

With the emergence of early genetic detection of PWS, it is this author’s hypothesis that use of nasogastric tube feedings can be minimized if not eliminated completely in Prader-Willi patients who receive occupational and physical therapy along with formula concentration.  

Materials and Methods: Retrospective data review of medical records of Prader-Willi patients included outpatient and inpatient records, when applicable.  15 medical records of PW pts dx at <2 yrs of age at Texas Children’s Hospital were evaluated for factors surrounding nasogastric tube placement including frequency and duration of nasogastric tube feedings, availability of supplemental therapies and usage of formula concentration.  

Results:  When occupational and physical therapy was employed, nasogastric tube feedings were limited to 40.5 days.  However, if physical therapy or occupational therapy was not utilized and formula not concentrated, mean nasogastric tube feedings lasted 234 days (p< 0.003).  Symptoms of hypotonia, low wt/ht, and poor appetite may lead a practitioner to recommend feeding by nasogastric tube feedings.  

Conclusion:  When occupational and physical therapy is employed, patients can thrive resulting in a decreased number of days required for nasogastric tube feedings.  Management of the young child with  Prader-Willi syndrome should emphasize occupational and physical therapy, thereby reducing the need for nasogastric tube feeds.  Once off nasogastric tube feedings, the incorporation of concentrated formula may aid in growth and development.

July 2002