Slow the Aging Kids

Dave and I complain about getting old, but that's because together we have more than a hundred years. In children with a genetic disorder called Progeria, they're old and frail before kindergarten and most die in their early teens from heart disease or stroke.

Now, for the first time, a drug called Zokinvy can extend their lives. It blocks an enzyme that modifies cellular proteins. This enzyme adds molecules such as fats onto proteins so that they can attach to cell membranes.

In children with Progeria too much fat is added onto these proteins, causing cell nuclei to become unstable and leading to early cell death. Children with progeria appear normal at birth and then fail to grow at a normal rate. They develop prominent eyes, a thin nose, small chin, protruding ears, hair loss, loss of fat under the skin and begin to look aged. Their arteries also start to harden so that they face the risk of heart failure, heart attack and stroke; however, their intelligence and motor coordination are unaffected.

But for the eighty children in clinical trials with the drug, Zokinvy, their lives were increased. In the first three years of treatment, their lifespan grew by three months. After eleven years of the drug, the children lived an average two and a half years longer.

That's significant given the short lifespan of those with Progeria and offers hope that one day, it will be stretched even more.