Lou Gehrig's Disease

WHAT YOU SHOULD KNOW

In Lou Gehrig's disease, the nerve cells that govern the muscles begin to shrink and die, leaving the victim unable to use them. As more and more muscle groups become affected, the condition becomes increasingly debilitating. Although commonly known as Lou Gehrig's disease, after the famed New York Yankees baseball player who died from this disorder in 1941, this condition is medically known as amyotrophic lateral sclerosis or ALS. It usually strikes in mid-life, affects more men than women, and typically proves fatal within five years after its discovery.

Causes

The progressive weakness that marks ALS is due to degeneration within the brain and spinal cord of specific nerve cells that control voluntary movement. Why these cells begin to atrophy is still unknown.

Signs/Symptoms

The first sign of ALS is often weakness in the hands, and less frequently the feet. The weakness tends to grow worse more rapidly on one side of the body, proceeding up the arm or leg. Victims are prone to tripping and falling. They gradually lose motor control in the arms and hands and experience difficulty speaking and swallowing. Many suffer constant fatigue, twitching, tremors, muscle spasms, muscle cramping, and breathing difficulties. Sensation, however, remains intact, as does the brain's ability to think and reason.

Care

There is no cure for ALS, nor any way to halt its progress&#151although the Food and Drug Administration (FDA) recently approved a drug called riluzole that can prolong life for several months. Treatment generally focuses on relieving discomfort and controlling the symptoms as much as possible. There are a number of support devices to aid patients in performing daily activities.

WHAT YOU SHOULD KNOW

At first, the problems brought on by ALS are fairly simple to manage, and sufferers can usually help themselves for a while. However, as the disease progresses, ALS patients become totally incapacitated and require 24-hour care. Here are a few tips for coping with some of the many problems brought on by this debilitating disease:

• Begin assessing home and workplace safety. Eliminate slippery floors, scatter rugs, and similar hazards. Use a cane to provide stability when weakness is mild. A brace, called an ankle-foot orthosis, that supports the foot and ankle can aid with walking when weakness affects the feet. As weakness progresses, a wheelchair may become necessary for almost all activities.
• Work closely with doctors and physical and occupational therapists to learn the special techniques needed to transfer the patient to and from a bed, chair, wheelchair, or vehicle. There are simple tools, such as transfer boards, slides, and mechanical lifts, which can make these maneuvers easier.
• Employ hand and wrist splints to aid in eating and writing.
• Consult a speech therapist or a speech-language pathologist to learn ways of preserving the ability to speak and swallow as much as possible. As weakness grows worse over months or possibly years, additional assistance from low-tech or high-tech mechanical aids will eventually be needed.
• The patient will need help with basic hygiene and skin care. A person with advanced ALS will require a bed bath at least once a day and will also need assistance in brushing his teeth.
• Work closely with a physical therapist to learn the special exercise techniques that help prevent muscle contractures&#151abnormal tightening of the muscles that can immobilize the joints.
• Soften or blenderize foods. As the muscles that control chewing and swallowing weaken, eating and drinking become more difficult, finally culminating in an inability to eat regular food. In the later stages of the disease, a feeding tube is usually inserted into the stomach or small intestine.
• Raise the head of the bed, even during sleep, to make breathing and swallowing easier. Help the patient with a technique called assisted cough, which involves pressing on the patient's abdomen as he or she coughs. After the assisted cough, use a mechanical suction device to remove secretions from the mouth.
• Use a physical therapy technique known as percussion and postural drainage to help move mucus from deep in the lungs up through the bronchial tree to a point where it can be coughed up. This technique calls for rapidly tapping or vibrating the patient's back with your hands.
• If paralysis sets in, the patient will need to be turned from side to side every few hours, around the clock, to relieve discomfort and prevent bed sores. Special mattresses, called alternating pressure pads, discourage bed sores by inflating and deflating air pockets underneath different parts of the body on a rotating basis. Other supports, such as soft booties, special pillows, and elbow pads, can also be useful in alleviating discomfort.
• Make provisions for bathroom needs. Although ALS does not have a direct effect on bowel and bladder functions, they will be affected, especially if paralysis sets in. Suppositories and stool softeners can be helpful at this stage, particularly if the patient can't eat normal meals. To help prevent urinary and bowel complications, make sure the patient gets plenty of fluids.
• At some point in the later stages of the disease, a mechanical ventilator will become necessary to sustain life. Although it's difficult to even think about it, try to decide well in advance&#151while the patient can still discuss the issue&#151whether such measures are wanted. Make certain that the doctor is aware of the patient's decision.
• For more information, contact the ALS Association at 800-782-4747.

Call Your Doctor If...

• Symptoms worsen or new ones develop.

Seek Care Immediately If...

• Breathing or swallowing becomes difficult.
• Episodes of apnea (lapses in breathing) occur.

Return to top