It is not easy when the joy of parenthood is overcome by the fear of disease, that too only a few months after your child’s birth. Unfortunately, this was the reality faced by a young couple in Pune over the last few months. Their child was diagnosed with a rare condition called Tyrosinemia a few months after his birth. Child had Acute Liver failure due to Tyrosinemia and suspected Hepatocellular Carsinoma(HCC).
In this disease the body lacks enzymes that breakdown an amino acid called Tyrosine properly. It is usually an essential building block in proteins found in the body. This improper breakdown leads to an accumulation of toxic products which make life difficult for the patient. Often without treatment survival beyond a few months is not likely. The child was under treatment for this condition but unfortunately the disease progressed. In advanced stages, the only treatment option left is a liver transplantation. In such cases a liver is taken from a person, usually a family member, who does not have the disease. The healthy donor’s liver produces adequate amounts of the enzymes that breakdown tyrosine properly. Replacing the patient’s old liver with a part of the donor’s new liver provides this critical enzyme, essentially curing the disease. Since a liver transplantation often can last a lifetime, it offers a permanent cure to this condition.
Liver transplantation in children is often very challenging. Especially in children like this who was 6 months old and weighed only 5.8 kgs. They have very small blood vessels which need to be connected to the new liver and the operation is technically challenging. There is no room for error. The complexity is often understood even by lay people. Even though they were faced with such a difficult situation the parents of this child decided to pursue a liver transplant. A member of the family agreed and was found fit to donate a small part of her liver to the child. All necessary testing and diagnostic procedures were carried out and the transplant was performed recently. The complex surgery which took place over 12 hours at Jupiter hospital in Baner, Pune was performed by a team of transplant surgeons headed by Dr Gaurav Chaubal, Director- Transplant.
Dr Chaubal said a small part of the donor’s liver, the medical term being ‘segment 2 liver graft’, was taken. The procedure to take such a liver graft from the donor itself is a challenge. However, it was performed successfully (and the donor went on to make a complete recovery within a week). The old liver from the child was removed surgically and the donor’s liver was connected. The transplanted liver began working straight away. At the end of the surgery the recipient was stable and shifted out to the intensive care unit for further care.
In the post-operative period the child recovered in the ICU over the next few days. During this time a team of surgeons, liver specialists, intensive care doctors, nurses and pediatricians provided round the clock care for this child. The child made a complete recovery over a 2-week period and was discharged from hospital. He continues to follow up at the Jupiter Hospital in Baner where his team of doctors continue supervising his care. He is expected to make a complete recovery from his disease and have a normal life.
Only a couple of decades ago this family would have had to seek medical care abroad. The case has highlighted how providing access to life saving surgeries like liver transplantation are changing the outcomes in diseases where historically outcomes were dismal. The expertise to carry out such complex surgeries is now available at centers of excellence like Jupiter Hospital in Pune. The hospital has a large team of doctors from all specialties to address the needs of such complex cases.
