(by Tom Melang)
My name is Tom. I am 32 years old and have cystinosis. I was diagnosed with the disease at the age of 13, but did not begin Cystagon [cysteamine] until I was 29 years old. I took cysteamine when it was in the experimental phase when I was 15 years old, but stopped taking it because I did not see an immediate benefit and there was not substantial proof that the drug worked. With the benefit of hindsight, this was the WRONG decision.
There is substantial evidence that Cystagon (or cysteamine) not only works to protect the kidney from the damage of cystine buildup, but also protects or slows down the deterioration of other cells in the body. I have other symptoms that have developed over the years including muscle loss and swallowing difficulities. These progressed slowly over the years, and there is a good chance that consistent Cystagon treatment could have slowed or stopped this development. When I began Cystagon at the age of 29, I had encountered 3 or 4 choking incidents the prior year. Since then, I have noticed improvement in swallowing, and have not had any choking incidents. The muscle loss seems to slow as well.
As the life expectancy dramatically increases for cystinosis patients, there is evidence that Cystagon is proven to be our best defense against the progression of this disease. Those who take Cystagon may spare themselves of a whole host of problems that would have developed in later life. Regardless of what your symptoms are now, I am a strong believer that anyone with cystinosis should take the medicine.
Taking medication every 6 hours is not easy. It may not seem worth the hassle because you don't feel any different. But believe me, the quality of life over the long term will make it well worth it.
Cystinosis Central | Patients