Hirschsprung Disease Patient on Home Parenteral Nutrition during COVID-19 Pandemic

Amran MHH, Wan Razdi WN and Jusoh NA

Department of Pharmacy, Hospital Sultanah Nur Zahirah, Ministry of Health, Malaysia
Department of Pharmacy, Hospital Pengajar Universiti Sultan Zainal Abidin, Malaysia

^*Correspondance to: Amran Mohd Haz Hairul 

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Abstract:

Hirschsprung Disease (HD) is a rare neonatal congenital disorder with an absence of parasympathetic ganglion cells in the distal colon. Adequate nutrition support is important in HD management. Parenteral Nutrition (PN) and Home Parenteral Nutrition (HPN) is an alternative nutrition support for the HD patient to grow in psycho-familial environment. A female patient weight 3 kg was born with no bowel opening, abdominal distension and bilious aspirate in 2014. Biopsies and laparoscopic biopsies had confirmed the presence of ganglion cells up to 20 cm distal to the stoma. She was diagnosed with Hirschsprung Disease-Total Colonic Aganglionosis. She underwent laparotomy, adhesiolysis and serial transverse enteroplasty STEP procedures for bowel lengthening initiative. She started with 2-in-1 parenteral nutrition via internal jugular vein in 2015 and discharge with home parenteral nutrition in 2017. Prior to discharge, patient mother has demonstrated competence in HPN management. Her family made the necessary adjustment and renovation of their house to provide a dedicated room for PN administration. Discussion and arrangement have been made with related authorities for PN supply, routine home visit, emergency health management, PN transportation and routine medical follow-up. She was 13 kg at the beginning of HPN program, and currently her weight is 15.8 kg, active and no other medical issues. PN solution and lipid solution were packed in separate bags and infused using infusion pump for 12 h each starting at 6.00 pm daily. The HPN treatment continue during COVID-19 pandemic. She had never been infected by COVID-19 and not yet vaccinated. HPN is a strategy to support patient live a normal life and should remain uninterrupted yet flexible, even in the era of pandemic.

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Cite the Article:

Amran MHH, Wan Razdi WN, Jusoh NA. Hirschsprung Disease Patient on Home Parenteral Nutrition during COVID-19 Pandemic. Clin Case Rep Int. 2023; 7: 1549.