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Hope & Healing: Packed tool box is essential to manage ALS symptoms
Wednesday, November 12, 2008

I have gathered to myself a collection of machines, gadgets and appliances meant to make life easier as my body loses more and more function. I am living with Lou Gehrig's disease, also called amyotrophic lateral sclerosis or ALS.

The disease, which so far has no cure, kills the motor neurons that feed and signal the voluntary muscles of the body. In the United States, about 5,000 people are diagnosed with ALS each year. Usually, it shows up when one reaches middle age, but I know of one victim who was diagnosed at 14. He died at 16. ALS is almost always fatal.

As the illness progresses, the person with ALS has to find ways to carry on necessary functions like speaking, eating, walking, dressing, coughing, breathing, swallowing, even turning in bed during sleep. So far I have at least a dozen tools that have either taken over a life task or aid in performing it.

First, in the winter of 2007 I was fitted with an AFO. AFO stands for ankle-foot orthotic. This is a hard plastic brace that supports a weak ankle and corrects the resultant foot drop that can be an early symptom of ALS. I don't use this one any more since walking on my own is out of the question because my legs are extremely weak now.

Almost simultaneously with the foot drop my speech showed signs of being affected, too. I did not lose the ability to talk until mid-summer, however. But by July I was totally dependent on pen and paper to communicate. So, the next item I got was a DynaVox electronic speaking device. I was so fortunate that the patient services nurse with the ALS Association of Western Pennsylvania and West Virginia, Linda Talmon, helped me secure a loaner DynaVox through the association.

The machine is actually a computer that can function as a computer, meaning it can access the Internet, send e-mail, produce documents, etc.

This 8 1/2-by-8-by-1 1/2-inch heavy box that costs nearly $10,000 allows me to "say" what I'm thinking. I type into it my thoughts, punctuate the sentence that appears on the screen and an electronic voice articulates the words. Of course, it has flaws. The voice often sounds mechanical and the inflection often lacks context, but it beats a pad and paper, especially while traveling in the car.

What's more, it's conversant in Spanish, German or Canadian French. If I ever find I need to speak English with a French accent, I'm set.

Along with the DynaVox I have a $33,000 electric-powered wheelchair for getting around on the first floor at home, a manual wheelchair for use away from home and two suction machines -- one stationary, one portable -- to clear away choking secretions that I can no longer swallow or cough out.

Another device that helps me is called a BiPAP machine. BiPAP is the acronym for bi-level positive airway pressure. Not to be confused with the CPAP, which stands for continuous positive airway pressure, the BiPAP exerts two air strengths, more at inhalation and less at exhalation.

These machines are often prescribed for people with sleep apnea, a condition in which a person stops breathing during sleep. In my case, ALS has begun to cause my diaphragm to lose strength -- and my breathing is shallower than it should be.

The diaphragm is essential for breathing. When one breathes, the diaphragm contracts and pulls down. Thus, the lungs have room to expand and fill with air. At exhalation the lungs deflate and the diaphragm arches upward as it relaxes. As it does so, it helps to push air out of the lungs.

ALS steals strength from the diaphragm, a voluntary muscle.

Consequently, an ALS victim loses "forced vital capacity," or FVC. This is air that can forcibly be blown out after a deep breath. The normal measure is 80 to 100 percent. My FVC was 30 percent in July. Fifty percent is the danger point.

So the BiPAP is set up in the bedroom for me to use during the night.

It's about the size of a large loaf of bread. Three-fourths of it is a computerized motor. One-fourth is a reservoir for warm distilled water.

Off the reservoir is a 6-foot plastic hose with a harness that goes over my head in order to hold a nosepiece at my nose. That's where the air comes out. It reminds me of scuba gear.

Another acquisition I've gotten for nighttime use is a mattress overlay.

The overlay is made of plastic that is honeycombed with air pockets that are automatically filled and deflated as I sleep on it. This helps prevent pressure sores. With this illness, muscle weakness reaches a point where it is impossible to turn from side to side during the night.

There's a bedside commode, a walker with wheels and one without (both loaned by kind neighbors), a buttonhook, a water distiller, an elevated toilet seat, a stair lift and a PEG tube.

What's a PEG tube?

PEG stands for percutaneous endoscopic gastrostomy. This is a small tube that is inserted into the stomach to allow a patient, who cannot swallow, to take nourishment and yet bypass the esophagus.

Here's a description, adapted from Wikipedia, of the procedure to place the PEG tube:

The PEG tube is placed endoscopically. The patient is sedated, and an endoscope, or camera with surgical tool attached, is passed through the mouth and esophagus into the stomach. The position of the endoscope can be visualized on the outside of the patient's abdomen because it contains a powerful light source.

A needle is inserted through the abdomen, visualized within the stomach by the endoscope, and a suture or thread passed through the needle is grasped by the endoscope and pulled up through the esophagus. The suture is then tied to the end of the PEG tube that will be external, and pulled back down through the esophagus, stomach, and out through the abdominal wall. The insertion takes about 20 minutes.

The tube is kept within the stomach either by a balloon on its tip (which can be deflated) or by a retention dome, which is wider than the tract of the tube.

According to this online reference, a PEG tube can last up to six months. I've had mine since Oct. 4, 2007.

All told, that's some of what it takes to help an ALS patient continue to function. No wonder ALS Association officials say it costs upwards of $250,000 to keep a person with ALS going.

M. Tinsley-Crabb can be reached at health@post-gazette.com.
First published on November 12, 2008 at 12:00 am
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