Dr. Cliff's Disease of the Week!
On this page I'll take a disease at a time, not really once a week since I actually work and stuff like that, and write a little blurb about what it is, who gets it, how it may affect them, how often it occurs, and so forth. This could serve several potential purposes. You could:
The first problem I want to talk about is cancer- specifically, cancers of the head & neck area. These add up to more than 40,000 new cancer cases per year, in the US alone. Typically there is an association with smoking, and drinking as well. The World Health Report 1997 tells us that about one in seven cancer deaths are directly attributable to tobacco- that means a million people die every year because they smoke(d). On a lighter note, non-smokers die too, and, well, like cancer patients tend to say, you gotta die from something.
Head & neck cancers carry a very poor prognosis, and 5-year survival estimates range from 5% to 50% depending on who you ask. There are several contributors to the low survival-
At the NIH, patients are given the best options and receive state-of-the-art treatment. This usually involves a combination of the 'big three'- surgery, radiation, and chemotherapy. A delicate combination of these unpleasantries, ideally, would destroy the tumor(s) but not the patient. It doesn't always happen that way. A healthy 25-year-old would be gravely challenged by treatment this invasive, and most head & neck patients are neither healthy nor 25.
One thing to expect, is that the hospital dentist may want to remove several or all of your teeth. During and after therapy your body can't tolerate routine dental care very well. If you have a cavity or a broken tooth, that tooth has to come out before therapy begins. Otherwise, later, you could die from a tooth extraction (because of compromised wound-healing) after surviving a tumor. The irony would be lost to you.
Even the lucky survivors have serious changes in the quality of their life. Surgery may have taken something they now miss dearly- their teeth, their voice, an eye. Maybe the patient has a tracheostomy tube now, talking and/or feeding through a hole in their neck.
Radiation therapy leaves tissue with a very poor blood supply, which delays wound healing. Little pieces of dead bone (sequestrae) may break off and begin to migrate, slowly, painfully, out into the patient's mouth. Salivary glands in the radiation field don't work any more. This chronic dryness leads to markedly increased oral infection and tooth decay problems. It is also difficult to wear a denture because the supporting tissue is so fragile, and injury from an ill-fitting denture could lead to life-threatening problems. So patients may be on a soft-food diet for the rest of their lives. This can be a major contributor to the depression which often overwhelms these patients.
A lot of current patients learned to smoke when we were all a little less aware of the dangers, and my heart truly goes out to them. Younger people have no such excuses, and in twenty years, I will be hard-pressed to grieve for someone who has deliberately destroyed him/herself. I think that tobacco may be the most powerful threat to human health ever encountered, and the suffering and death created are totally preventable! Please don't smoke (or chew, or dip).
Is tooth decay really a disease? Yes, it is. It is an infectious disease, caused by specific bacteria. These bacteria are present in everyone's mouth, and will thrive and multiply anytime they get the chance. When these bacteria metabolize sugar (specifically sucrose, or table sugar), they produce acids that leach minerals from the teeth. This softens and discolors the tooth, resulting in what we all know as a cavity. Left untreated, the decay will spread into the tooth's pulp chamber, which is filled with nerves and blood vessels. At this point, the only hope for saving the tooth is a root canal, where the pulp chamber is cleaned of all living tissue and sealed. If the root canal fails, the tooth has to come out.
So, if this problem is so simple to understand, why do we still get cavities? Why hasn't some Jonas Salk or Albert Schweitzer cured tooth decay? Hey, we're working on it. In the meantime, patients need to learn the best ways to take care of their teeth. Prevention is still the best course of action. Patients say things like "I got my dad's bad teeth", when really they probably got their parents' bad habits. Teeth are teeth, with few exceptions.
Fluoride toothpastes and dental floss are the big weapons against tooth decay, and used regularly, they're very effective. So brush twice a day, floss once a day, and get to your dentist for a check-up. Most of the horror stories associated with dental care can be avoided by proper oral hygiene.
Table of Contents
is an extremely rare disorder, first reported in 1979. Only a few dozen patients have been diagnosed since. It was named after the Greek god Proteus, who could change his shape at will to avoid capture. The clinical manifestations are extensive, including abnormal outgrowths of cranial bones, progressive asymmetry and disfigurement of the skull, hemihypertrophy (one side of the body is larger than the other), 'lumpy', irregularly pigmented skin, due to several types of benign tumors, partial gigantism of the hands or feet, scoliosis, and marked asymmetry of the limbs.
Lesch-Nyhan syndrome (LNS)
is yet another complex problem brought on by a single genetic defect. One defective enzyme, out of the thousands working in the human body, can lead to problems of staggering complexity.
LNS children do not process one particular metabolic waste chemical properly, resulting in a buildup of uric acid in the body. This is the same 'uric acid buildup' commonly associated with gout, and in fact the same treatment, allopurinol, can save LNS patients from gout symptoms. But there is something more; something terrifying yet fascinating.
LNS children develop 'itches' or 'funny feelings', typically in their lips and fingers, but sometines also in their ears, nose, or toes. These areas will be compulsively chewed or clawed apart by the patient, even though the patient still feels pain like you or me. Children with LNS will often 'eat' their own lips and fingers if not physically prevented from doing so. Although LNS children are often behind the average in mental & social development, the social stigma of being a 'lip-eater' is still unfathomable to you or me.
Even more bizarre, LNS patients often develop 'fetish' areas which must be mutilated at all costs, while other, similar body parts are of no real interest.
A colleague who is faculty at UCLA Medical School relates a chilling anecdote on the mystery of LNS:
Bear in mind, again, this complex and disturbing behavior is caused by a problem in a single enzyme. For these reasons, it strikes me as unforgivable hubris to declare that the 'Human Genome Project' will result in the cure or prevention of even one single disease. There's a little more to human behavior than single genes making single proteins which do single jobs. The human genetic sequence is of absolutely no use without a better understanding of its meaning, much as a phone book means nothing to a man with no phone.