Thursday, March 22, 2012

Ostrich Syndrome

A man of my acquaintance has 2 boys, both of whom are the product of a marriage where their mother has Huntington's disease, a progressively debilitating disease that affects the brain and body and ends in death, but not before destroying every ounce of humanity and personality. In the television show House, 13 had Huntington's, as did her brother and mother, and she went to prison for euthanizing her brother at his request. She asked the same from House, to ease her out of this world when she could no longer live her life as she wished.

One of the things I remember most is from Jacqueline Susann's novel, Valley of the Dolls, when Tony, a singer and actor, who had Huntington's, suddenly lost coordination and ended up in a sanitarium, something his older half-sister had prepared for, and Tony's wife worked in French art films to subsidize.

The question is whether a parent, who knows his children may carry the gene for the disease, has the right to deny his children early detection. Is a parent obligated to find out if his children carry the disease and pass that information on or is it all right to follow the path of not knowing being the best defense?

Since Huntington's strikes in the 30s and mid 40s, it would at least give his children a chance to live and to explore all options for treatment and mediation of the disease. I've always believed that knowledge is power, especially when not knowing could mean the difference between making informed decisions and choosing whether or not to have children. There is a 50% chance of both boys having the disease and being a carrier, passing the gene and the disease on to their children. Is it irresponsible to bury his head in the sand and refuse to have the boys tested? Does it not continue the cycle of disease in future generations, and what does that say for disease eradication?

There are some people who believe that if a person carries a degenerative disease that is transmitted to future children, they should be sterilized or at least not have children. By so doing, the disease is rooted out much like rooting out a dangerous plant and destroying its chance to propagate. That does interfere with free will and the right to choose one's life, but what is technology for if not to detect and prepare for potentially life-altering diseases?

That brings up another issue, genetic therapy. Genetic therapy has done wonders for so many illnesses and near catastrophic situations, like restoring natural tissue after extensive burns. Instead of the corded, ropy scars of yesterdays' burn victim, genetic therapy can bring a person's natural skin back so there is no discernible evidence of being burned. Genetic therapy has also progressed to the point that the tip of a finger that has been amputated can be regrown, fingernail and all, with only minor differences in length. Genetic therapy continues to progress and there could well be a treatment for those afflicted with Huntington's disease, but how would you know unless you were diagnosed before the disease progresses to the acute phase?

Some people would rather not know the worst -- or anything at all -- than to have to deal on a day to day basis with the facts of a devastating diagnosis like cancer, brain tumors, or Huntington's. Are they helping or hindering the process of future treatments or being irresponsible, not to mention cruel, to themselves and their children? Who is to say?

I know what I would do. I'd have my children tested and then sit down and discuss the situation with my children one way or the other, give them the information necessary for them to make a decision about their own lives and future. If it means not having children, then its best to make the decision early and not subject a future partner to the news that offspring have a 50% chance of being born with the disease.

What if both people weren't told they had Huntington's, or some other genetic disorder, and by marrying they have increased the odds of their children getting the disease?

Knowledge is power. Yes, the news could be difficult for a children, even teenagers nearing the time when they will finish high school and move away to make their own lives, but knowing, in my opinion, is better than not knowing. From my point of view, not knowing is tantamount to playing Russian roulette where most of the cylinders are filled with bullets.

The children may not have the disease. Wouldn't it be best to know that and to know whether or not they are carriers?

It's always a matter of choice and not choosing or making a decision is a choice. A bad choice, not only for one's children but for any future children and the progression and spread of genetic disorders like Huntington's.

The only thing left to do is pray. For that family in this situation, there are no other options.

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