Patient Introduction

Mohammad Hashir, an 18-month-old baby boy from Pakistan was suffering from Severe Jaundice and Liver Failure. The doctors in Pakistan had advised him to undergo a Liver Transplant. For a small child, it was a very risky procedure and his parents were very apprehensive of getting such a procedure. After that Hashir’s uncle got in touch with MediGence in November 2018. He briefed the team about the condition of his nephew, who had been advised liver transplantation in Pakistan. Hashir was suffering from severe jaundice, loose motions, and loss of appetite at the time his uncle contacted the team. However he was stabilized and because of unavailability of funds at the moment, the family decided to medically manage his condition in Pakistan itself and do watchful waiting.

Treatment Decision

His uncle kept connected to the team and it was in April that he finally asked the team to make the necessary arrangements and that they now have the funds for his treatment in India. However, by this time, Hashir’s condition had been affected tremendously and he had been hospitalized in Pakistan in the last 3 months multiple times for management of symptoms.

The team quickly made arrangements and visa invitation letter was sent to them, following which they collaborated with the embassy to make sure that they get a visa on an urgent basis. They finally traveled to India in May.  They were picked up from the airport and transported directly to Indraprastha Apollo Hospital, New Delhi upon arrival. After the first consultation with Dr. Neerav Goyal, it was decided that Hashir be admitted for two days to complete all investigations for the purpose of transplantation. In parallel, tests for her aunt, who was supposed to be her donor, were also initiated.

During hospitalization, it was realized that Hashir had a severe urinary infection, which extended his hospital stay because of antibiotic administration through IV. Also, the team suspected that the issue was not with his liver but something else. The CT scan did not reveal massive damage to the liver, which should make the specialists consider liver transplant straight away.

Upon further evaluation, they suspected an underlying genetic issue that could have been leading to the symptoms triggering local doctors in Pakistan to think that it is a case of liver failure. The biopsy confirmed that progressive familial intrahepatic cholestasis (PFIC), and further advanced biopsy sent to a liver institute in Delhi also confirmed it’s typing.

Since the liver was not damaged completely and since PFIC was confirmed, the surgeons instead decided to go ahead with partial external biliary diversion (PEBD) surgery to improve the clinical status of the patient. It was decided that transplant is not required at the moment and that if it is done, the outcome may not be very good or long and the child will need it again in the future.

Recovery

The family was counseled and the surgery was successfully performed to improve the bile acid transport and excretion in Hashir. As of today, he is recovering after the surgery and his symptoms are gradually improving. The bilirubin levels went down from 25 to 19 and two weeks after the surgery, it has come down to 11. The family has been asked to wait for another 2 weeks to monitor the progress before he can travel back again.

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