Doctors at Sakra World Hospital Save Life of a Five-Year-Old Suffering from Rare Liver Condition

By / 9 months ago / News Update / 21 Comments
Doctors at Sakra World Hospital Save Life of a Five-Year-Old Suffering from Rare Liver Condition

Doctors at Sakra World Hospital have recently saved the life of a five-year-old Assamese girl suffering from rare liver disease by performing an emergency liver transplant. Koel Bohra (name changed) visited Sakra World Hospital after complaining of rapidly progressive jaundice that developed within the span of a week.

“Initial investigations revealed that her bilirubin was 34mg% and, the International Normalized Ratio (INR) was 6.6.  High bilirubin level in blood is indicative of jaundice and is normally less than 1mg%.  The INR reflects coagulation function of the liver.  A value of over 6.5 revealed that the liver was not producing enough protein required for clot formation in the body.  Hence, Koel was diagnosed with acute liver failure,” said Dr. Sadiq Sikora, Director – Gastrointestinal Surgery, Sakra World Hospital.

 Koel was immediately admitted to the Paediatric Intensive Care Unit and her condition was stabilised.  Further investigation revealed that she suffered from Wilson’s Disease.  It is a rare condition in which copper accumulates in the liver leading to progressive deterioration of normal hepatic functions. The symptoms of this condition usually start between the age of 12 and 23.  However, in the case of Koel, the very first presentation of Wilson’s Disease was acute liver failure. The condition is extremely fatal if not treated immediately.

In order to counter the rapid advancement of her liver failure, Koel’s name was added to the top of Bengaluru’s cadaver liver transplantation list.  Fortunately, her mother was also of the same blood group and volunteered to be a liver donor.    Since two out of the eight segments of her mother’s liver along with the blood supply and biliary drainage was found to be suitable and matched with Koel, the clinical team decided to go ahead with Koel’s mother as her liver donor.

On July 28, 2018 Koel underwent a seven-hour long live donor liver transplant procedure.  The mother and the daughter tolerated the procedure well.  The mother recovered within the period of a week and was discharged.  She subsequently participated in the care of her daughter.

“Koel had a smooth post-operative course.  Post-surgery patient care, especially after organ donation transplant, is critical.  Medication to suppress the body’s natural immune response is administered to prevent the body from rejecting the transplanted organ.  Immunosuppressants must be continued lifelong to preserve the function of the new organ.” said, Dr. Kishore GSB, Senior Consultant, Hepato Pancreato Biliary Surgery & Liver Transplantation, Sakra World Hospital.

 Living donor liver transplantation is a lifesaving option for patients with acute liver failure, especially in a country like India wherein donation rates of cadaveric organs from brain dead donors does not match up to the need of the recipients awaiting liver transplant.  Living donor liver transplantation for a child with acute liver failure is perhaps the best case of double equipoise where the risk to the donor is minimal and benefits to the recipient is phenomenal.

Koel was discharged from the hospital on the 19th day after surgery in excellent condition.  Back in her bubbly cheerful self once again, “I am extremely grateful to the Dr Kishore and the entire team at Sakra World Hospital for saving my daughter’s life.  The happiness of watching my daughter running around laughing gives me so much joy.” said Bhoopendra Bohra.

 

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