Nephrotic Syndrome is one of the commonest childhood kidney diseases that occur in India. In this condition, protein leaks from the kidneys into the urine, causing deficiency of protein in blood and swelling of the body. Fortunately in more than 95% of children, this disease is responsive to medications, and gradually resolves over time. However, in the rare 5% patients, this condition does not respond to any medications, and gradually progresses to chronic kidney failure.
Sneha was first detected to have Nephrotic Syndrome just after she turned 1 year of age. She received all standard medicines in succession but did not respond to any of them. Out of desperation, the parents took opinions from different doctors from different parts of the country, and also from alternative medicine practitioners. But since the treatment of this disorder is totally standardized all over the world, there was no difference of opinion regarding her management. She required several admissions throughout her childhood for different complications of Nephrotic Syndrome. This greatly disturbed her normal activities, school and social interactions. About 1.5 years ago, her renal function deteriorated to such a level that she had to be started on dialysis. Her parents were trained to perform peritoneal dialysis on her at home. Although in this way too many hospital visits could be avoided, however since her parents opted to perform the dialysis manually during the day, this caused a significant restriction to normal activity for the whole family. She was also subjected to a lot of restrictions regarding water and food intake, as is essential in most dialysis patients.
The best treatment for children with severe chronic renal failure is a renal transplant. When successful, this procedure allows freedom from dialysis, normal amounts of water and food intake, improved growth and well-being, and close to normal life activities. The transplant work-up for both donor and recipient takes a few months since the donor has to undergo rigorous tests to ensure that he / she is healthy and able to withstand the procedure. Likewise the recipient undergoes tests to ensure optimum clinical condition, and a series of vaccinations to provide protection from infections. Tests are also done to confirm that there is no mismatch between donor and recipient. Usually best results are obtained when the donor is a close first degree relative.
After such rigorous process, Sneha received a kidney transplant from her mother about a month ago. Renal transplantation in children is technically more difficult than in adults since children’s blood vessels and organs are smaller requiring great surgical skill during anastomosis… Similarly the fluid and electrolyte balance and drug dosing after the procedure can also be tricky to manage. However the long terms results are actually better. She is currently stable and doing well, but will need close monitoring for any signs of rejection or infection. She will also need to remain on anti-rejection medications life-long; however the doses will be reduced gradually. The marked improvement in her wellbeing is evidenced by a complete change of personality in hospital after the transplant….this little girl who was withdrawn and uncommunicative all through her long illness, was smiling and playful just a few days after transplant. The battle will continue for her – renal transplants do not last a lifetime, especially in a child –however, if everything goes well; she will have a vastly improved quality of life and be able to perform all normal life activities for many years. Please join us in wishing her and her family a long and happy life.
Photograph by-নিজস্ব প্রতিনিধি