Abstract

Author(s): Bajana, Stephany2; Beltran, Tanya2; Penafiel, Daniela1,2

Introduction: Wolf Hirschhorn Syndrome is a genetic disorder, occurring in 1 in 50000 births, resulting from the deletion of the short arm of chromosome 4. There is limited information on the correct management of enteral feeding in home patients.

Objectives: To evidence the contribution of using enteral nutrition education specifically designed for a patient with Wolf Hirschhorn Syndrome to the improvement of the nutritional status.

Methods: This research includes the follow up of a female adolescent, diagnosed with Wolf Hirschhorn Syndrome who had an initial body weight of 25.8 kg, and 134 cm of length. Medical history includes swallowing disorder, absence of chewing reflex, enteral feeding via gastrostomy, gastroesophageal reflux, constipation, absence of motor, intellectual and language skills. Enteral feeding was tracked for 8 weeks.

Results: We found that nutrition education promotes a significant improvement in the nutritional status of patients with Wolf Hirschhorn Syndrome. Body weight gain, bicipital and tricipital folds were increased by 1 kg, 2 mm and 2.3 mm, respectively; and also an increased intake of micro and macronutrients (p<0.001).

Discussion: It is essential to educate the staff in charge of feeding home patients with Wolf Hirschhorn Syndrome using a guide.

Conclusions: Enteral feeding at home was successful using the designed tool for the specific context.