The life of the Ibrahim family from Iraq came to a standstill when they realized that their young children, Ibrahim, Sozan and Lozan, aged 4, 10 and 11 years respectively were suffering from a rare genetic disorder called Progressive familial intrahepatic cholestasis (PFIC), a class of chronic cholestasis disorder (a condition where bile cannot flow from the liver to the duodenum) that begins in infancy and usually progresses to liver cirrhosis within the first decade of life.
A team of surgeons, anaesthetists and 30 nurses led by Dr Vivek Vij, Director GI Surgery and Liver Transplant at Fortis Hospital, Noida persevered for nearly 12 hours for each liver transplant for the three Iraqi siblings, to save their precious life.
Dr Vivek Vij, Fortis Hospital, Noida said This was a rare case and very few such cases have been reported in the medical literature. This is for the first time that we come across a family wherein all their young children were suffering from PFIC, a rare genetic disorder which had led to stunted growth, low immunity, severe nutritional deficiency, progressive jaundice, bile excretion disorder and diseased liver. The children had recessive genes from both parents as they were born out of consanguine marriage. It also presented a challenge for finding three donors for recipients of the same family. Ideally, the sooner the transplant is performed in such cases, the better is the outcome. But since the two siblings, Lozan and Sozan, were already 11 years and 10 years old respectively, it made the surgery more challenging.
Lozan (oldest sister) aged 11 years, weighed 24 kgs at the time of the surgery. Her aunt Sherivan was the donor. Sozan, (middle sister), aged 10 years weighed 18 kg. Her maternal uncle Nisrat was the donor. Ibrahim (youngest brother), aged 4 year old weighed only 12 kg at the time of surgery. His mother Bushra willingly agreed to donate a part of her liver.
Dr Vij further explained, “There are three types of PFIC (PFIC1, PFIC2 and PFIC3). Fewer than 200 patients with PFIC1 or PFIC2 are reported in the medical literature. PFIC 3 is even rarer.
It has been the most challenging phase of our life and I am glad that it has been a happy ending for our family. We are thankful to the doctors of Fortis Noida who have miraculously saved all our three children, said the elated mother Bushraabsullah Ahmed.
After the surgery, the patients were kept under the close observation for a week. The children have been discharged and are ready to go back to Iraq.
Fortis Noida has performed more than 150 Liver transplants till date.