FSGS is a rare kidney disease. It is a condition for which there is no proven treatment. Despite the use of cytotoxic agents, steroids, immunosuppressants and plasma exchange, approximately 50% of those who do not respond to medication progress to renal failure, half within 2 years and the majority within 5 years. Many sufferers will be looking at a transplant within 20 years. For a child this can mean multiple transplants in a lifetime because they don’t often last more than 10 years. Worse, in FSGS the disease often returns in the new kidney, sometimes within hours of the transplant, resulting in another decline towards end stage renal disease and dialysis before another transplant.

Life expectancy for kidney transplant patients is lower than the average life expectancy, mostly due to cardiovascular complications resulting from taking ciclosporin to prevent organ rejection. My son is already on ciclosporin, so I assume he already falls into this category although we don’t kow how long he’ll be able to take ciclosporin for.

It’s a gloomy picture for a five year old boy.

Additionally, there are often problems before the onset of renal failure secondary to complications of the nephrotic state which often accompanies FSGS, some of which are life-threatening (we make an emergency trip to the hospital most weeks, sometimes ending in hospitalisation).

Remission of proteinuria (protein in the urine), either complete or partial, is a strong indicator of increased renal survival rates. (William is not showing any signs of remission despite nearly three months of treatment although we remain hopeful he will. Our biggest hope now is that he can get a long enough remission so that he doesn’t have to face dialysis or a transplant while he is a child.)

Nephrotic syndrome, along with the side effects of the very powerful drugs he is on, make him feel really crappy. So crappy that he has not been to school at all since Christmas. So crappy that he rarely wants to play outside. There are bad headaches and lots of vomiting. And lots of unpleasant medicines to take several times a day. His appearance has changed so much due to the corticosteroids that some of his former classmates barely recognise him. There are dietary restrictions which means some of his favorite foods are off-limits. Then there are weekly routine visits to the hospital for bloods and other tests.

He is 5 and his childhood has been taken away from him. His future is likely to be very hard. We will learn to deal with that, but we can’t just yet.

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For more information on kidney disease, nephrotic syndrome and FSGS, visit the kidneybeans pages.