DRHOOK- Wasteful inheritance: Huntington's disease devastates everyone

the handsome doctor John Hong

"Inheritance is the retirement plan for "gold diggers." 

As much fun as I thought Anna Nicole Smith was, let's face it: she wouldn't have married a man 63 years older than she was (wasn't he 88 when she pushed his wheelchair down the aisle?) if he weren't a billionaire. (On the flip side, he wouldn't have married her if she were unattractive.) Look at Larry King and the late Dennis Hopper–- wealthy men like them dilute the inheritance every time they get married, divorced, and pop out more children.

But there are some things you just don't want to inherit, such as Huntington's Disease (HD) which is most common in people of European ethnicity. Worldwide, there are about 2 to 4.7 million new cases of HD occur each year, and because the gene for this disease is autosomal dominant, there's a 50-50 chance of getting it from the parent who has HD.

In HD, the neurological system gradually degenerates, and this process cannot be stopped. The symptoms start at any time but usually in middle age. Juvenile onset is the most unfortunate, but most people with HD symptoms pass away within 10-30 years.

Chorea is the hallmark sign of HD. Chorea (sounds almost like my parents' place of origin, Korea) is abnormal movement of the face, trunk, and limbs. They are rapid and non-repetitive, so other people easily misinterpret this body language to be restlessness.

To adapt to the extra movements (consciously or unconsciously), the person with HD incorporates the unintentional movement into a purposeful action. I know I talk with my hands (watch me on the news!), so I can see how easy it would be to cover up the chorea with "passionate" movements.

The chorea might eventually be taken over by Parkinson type problems: poor muscle tone, slow movement, rigidity. Speaking problems and even involuntary vocalizations from diaphragm movement disorder can occur as the disease progresses.

Because swallowing difficulties make it hard to clear the throat, things can get into the lungs to cause pneumonia– the main cause of death in HD. 

Since the brain deteriorates, psychiatric problems typically occur. Some families and friends do not deal well with this and think the person with HD is lazy or crazy. It is not the person's fault for being apathetic (they don't care about anything or want to do anything) because it is the awful nature of HD. Depression, anxiety, delusions, paranoia, aggression, and irritability can turn the life upside down for an HD person, as well as the family. 

Dementia occurs in the later stages, though often this stage is limited because life is often cut short by this disease. It might be difficult for caretakers to work with a person with HD because of their loss of insight (people may think the HD person is clueless, which isn't their fault), poor judgment, poor concentration (people may think the HD person has ADD) and memory loss.

Persons with HD tend to wither away, even when taking in a lot of calories. Part of the reason for losing so much weight is the constant uncontrollable movements of the chorea. 

Genetic testing is a very accurate way to diagnose HD. However, it is a huge decision to get tested because what do you do if you find out you have HD? Folks who are counseled about testing and followed afterwards (if they do have HD) have a suicide rate of seven percent.

There's no cure and no way to slow down the progressive deterioration of HD. Some things can help the symptoms, though. Support and love are also the best medicine because going through it alone (or the family going through it without understanding the disease) is a terrible loss.

It makes you think twice about inheritance.

