Caring for the Person With ALS
Index to This Chapter:

• The Early Stages
  
• The Later Stages
  
• Hygiene and Skin Care
• Nutrition and Swallowing
  
• Preventing Contractures
• Respiratory Care
  
• Communication
  
• Meeting Psychological and Emotional Needs

ALS is a progressive disease, and caring for someone with this disorder requires constant adaptation to continued loss of function. You can learn to meet these demands, and in this guide you’ll find many resources for information and help.

About 70 percent of people with ALS die within five years of the onset of the first symptoms, but about 10 percent live for 10 years. Only a few (for example, British physicist Stephen Hawking) have lived 20 years or more.

Thus, taking care of someone with ALS isn’t like taking care of a person with an acute illness lasting a few weeks; nor is it like caring for someone with a stable, chronic condition, such as someone who has had a stroke. It’s somewhere in between.

The Early Stages

Most people with ALS experience their first weakness or spasticity in an arm or leg. These symptoms usually progress and may soon involve the other arm or leg. At first, the problems will be fairly simple to manage. When the person with ALS first gets a diagnosis, he will likely still be able to walk and use his arms and hands. However, he may experience fatigue, lose balance, have difficulty using his hands or trip when walking.

In this early stage of ALS, safety needs, around the house and outside the home, are important. Slippery floors, scatter rugs and other hazards should be eliminated, both at home and at work.

A cane may provide stability when weakness is mild. A brace that supports the foot and ankle (called an ankle-foot orthosis) can keep a dragging foot from interfering with walking (see Figure 1). He may need to use a wheelchair for especially tiring excursions, such as going to a mall.

Your local MDA office is a good source of information about equipment vendors in your area. MDA provides some financial assistance with the purchase and maintenance of wheelchairs and leg braces.

Figure 1

As weakness progresses, over several months or possibly years, a wheelchair may become necessary most or all of the time. Many people say the wheelchair is, at this stage, a welcome relief from the constant strain of walking with weakened muscles and the fear of falling. The wheelchair can be thought of as preserving, not decreasing, a person’s mobility and independence.

You’ll need to learn about special techniques for lifting a weak or paralyzed person and helping him transfer to and from a bed, chair, wheelchair or vehicle without injuring yourself. A physical or occupational therapist can help you learn these techniques and can be consulted through your MDA clinic or ALS center.

The patient with this type of ALS — bulbar-onset ALS — has needs that are different from the more common, limb-onset type of ALS. (Lower motor neurons in the brainstem control the muscles of the face, mouth and throat, and are called bulbar neurons.)

A speech therapist (usually called a speech-language pathologist at a medical center) can help the person with ALS make the best use of his remaining muscles for both speaking and swallowing. However, as weakness progresses over months to years, additional solutions — low-tech or high-tech mechanical aids — must be found to aid both functions.

The Later Stages

No matter what part of the body is first affected by ALS, the disease eventually involves the entire body, including the muscles involved in speech, swallowing and breathing.

In the late stages of ALS, the affected person is totally paralyzed, and providing care at home is multifaceted and demanding.

Hygiene and Skin Care

The person with late-stage ALS will need to have his basic hygiene and skin care provided by others. Nurses at your MDA clinic or ALS center may be able to provide you with guidance in this kind of care, but you may wish to arrange for a visiting nurse to come to your home at least once or twice to demonstrate techniques and watch you perform them. (You can look in the yellow pages of your phone directory under "Nurses" or "Nursing," but your local MDA office, MDA clinic or MDA/ ALS center will probably be able to offer some guidance on which services are the most reliable and cost-effective.)

You will probably need to give the person with advanced ALS a bed bath at least once a day, and provide oral care, such as brushing his teeth.

Having your loved one sit up in a chair or wheelchair at least once a day is a help to his body and mind, as well as to your ability to change the bed sheets, turn the mattress and so forth. There are simple tools, such as transfer boards and slides, which make moving from bed to chair easier. An occupational therapist can advise you about these.

Equipment can sometimes be rented or can be borrowed from your local MDA office, rather than purchased.

A mechanical lift (see Figure 4) allows even a small caregiver to safely lift a large person and transfer him between a bed and a chair.



Figure 4

A paralyzed person must be turned from side to side every few hours, including during the night. It's very uncomfortable to stay in one position for long periods of time.

Special mattresses called alternating pressure pads can help prevent skin discomfort and damage by inflating and deflating air pockets underneath different parts of the body on a rotating basis. Soft booties and elbow pads and other such devices can also be useful, as can special pillows and other kinds of supports, but there is no substitute for frequent turning of the patient. (Speak with a physical therapist or local orthopedic equipment vendor about which mattress is most appropriate for your loved one.)

ALS doesn’t have any direct effect on bowel and bladder function, but, in a paralyzed person, these functions are often somewhat altered. Provisions have to be made for collecting and disposing of urine and feces, and constipation and fecal impaction may occur in people who can’t use voluntary muscles to defecate. Your doctor or nurse can advise you and your loved one on how to develop a bowel routine to prevent these complications. Suppositories and stool softeners can be helpful at this stage, especially if the person isn’t able to eat a normal diet.

Adequate fluid intake will help prevent urinary and bowel complications.

Nutrition and Swallowing

As the muscles that control chewing and swallowing weaken, eating and drinking become more difficult, until they’re impossible to manage safely. At first, foods with a softer consistency can still be swallowed safely. (See Resources.) Some people with ALS find that blenderized foods can be sucked through a large, sturdy straw more easily than spooned up, probably because the muscles that move food from the front to the back of the mouth aren’t as necessary with this technique.

As throat muscles weaken, the possibility of choking on food or "swallowing the wrong way" increases markedly and poses a real danger. Swallowing the wrong way means breathing food or liquid into the lungs instead of routing it down the esophagus into the stomach. Doctors call this aspiration, and it can cause respiratory infection or a frightening choking sensation.

Also, when eating is laborious, it’s usually difficult for the person to take in enough calories to maintain weight. This is especially true if respiratory muscles are also weakened (which they usually are at this stage), requiring the person to expend enormous energy (burn calories) just to breathe. The nutritionist or dietician at your medical center can instruct you on using soft or blenderized foods.

When throat muscles weaken to the point where choking spells occur often and the patient is losing weight, his doctor will probably raise the question of a feeding tube, known as a gastrostomy tube (see Figure 5). This is a tube that’s inserted directly into the stomach (or occasionally into the small intestine, in which case it’s called a jejunostomy tube). The tube can usually be inserted under local anesthesia.

Figure 5

Liquid nourishments, either commercial or homemade, are poured down the tube into the stomach, bypassing the mouth and throat entirely and thus eliminating any chance of choking or aspirating the food. Adequate calories can be taken in with little effort expended by the patient.

The nutritionist or dietician at your medical center can help plan and supervise nutritional requirements. Your pharmacy or supermarket will probably carry at least one brand of nutritionally balanced liquid food, such as Ensure or Jevity.

Food put down a feeding tube should be clean, but it doesn’t have to be sterile.

Inserting a feeding tube is a decision that should be made by the person with ALS, after consulting with medical professionals and family members. A feeding tube can prolong the life of a person with ALS by months or even years.

Preventing Contractures

Contractures, or abnormal tightening of muscles so that joints become immobilized, are a common problem in many people with neuromuscular disorders.

Your physician, nurse or physical therapist can show you how to exercise the patient’s limbs and position him in bed or in a chair so as to prevent or minimize contractures.

Respiratory Care

Loss of the muscles involved in breathing is the gravest problem in advanced ALS.

The respiratory diaphragm — a muscle that lies under the lungs — normally moves up and down when we breathe. As we breathe in, the diaphragm moves down, allowing the lungs to fill with air; as we breathe out, it moves up, letting the lungs expel air.

There are also other muscles, located between the ribs, and in the neck and abdomen, that aid in respiration. All these muscles are weakened or paralyzed in ALS.

As the respiratory muscles are losing strength, the person with advanced ALS is also having difficulty coughing and clearing his throat because of weakened abdominal and throat muscles.

Normally, the lungs are constantly performing "housecleaning" chores, moving excess mucus and inhaled particles up toward the mouth, to a spot where they can be coughed up.

In the person with ALS, this material doesn’t get coughed up, but falls back down into the lungs, where it can cause respiratory irritation and infection. Weakened swallowing muscles make aspirating (inhaling) food and liquids into the lungs more likely, and these inhaled particles add to the irritation of the respiratory tree. They can also lead to pneumonia or acute choking.

Raising the head of the bed, including during sleep, is a simple but helpful step. This makes both breathing and swallowing easier. (You may want to purchase or rent a hospital bed, which is fully adjustable.)

You can help the person you’re caring for rid himself of respiratory irritants by learning how to do an assisted cough. Your MDA clinic doctor, nurse or respiratory therapist can show you how to do this maneuver, which involves pressing on the patient’s abdomen as he coughs.

After the assisted cough, you’ll need to use mechanical suction to remove secretions from the mouth. This is also something you can learn from a health professional. You’ll need to rent or buy the suction device from a local medical equipment vendor.

A physical therapy technique known as percussion and postural drainage can also help to move mucus from deep in the lungs up through the bronchial tree, to a point from which it can be coughed up. A professional can instruct you in how to perform this technique at home. It involves using your hands to vibrate the chest wall from the back, with the patient in different positions.

As the person with advanced ALS breathes less effectively, the exchange of oxygen and carbon dioxide that’s supposed to occur in the lungs becomes less effective.

A person in respiratory distress because of weak muscles can only speak in short phrases. He doesn’t seem to have enough air to speak in full sentences. He can’t sing or shout. He’s unable to cough effectively or sniff hard. His rate of breathing increases, and the breathing appears labored. Sometimes you can see muscles working in the neck or abdomen, trying to compensate for lost function in the diaphragm.

When respiratory distress causes a person to retain excess carbon dioxide, he may have early morning headaches (after sleep), and may be excessively sleepy during the day but unable to sleep well at night. The person may look exhausted and may lose weight rapidly.

At this stage, your doctor will probably begin to discuss respiratory (ventilatory) support and ask your loved one to begin thinking about what kind of support he wants to have.

There are several steps between "being on a ventilator" full time and allowing nature to take its course without doing anything. It’s a good idea for the patient and caregiver to think through their options at this point.

If the patient doesn’t already have a gastrostomy tube, now is a good time to consider inserting one. Not only will it eliminate the danger of aspirating food into the lungs, but respiratory function often improves with better nutrition and less energy expended on eating.

Some doctors prescribe drugs that dilate the bronchial tree (bronchodilators) to ease respiratory problems, but some don’t think these are helpful. Some prescribe drugs that help the patient cough up respiratory secretions more easily (expectorants). Increasing the person’s intake of fluids is also often recommended.

There are drugs that have an adverse effect on respiratory function. A list of the patient’s medications should be reviewed with the doctor, with an eye to switching him to drugs that have fewer respiratory side effects.

Some studies have suggested that respiratory exercises, prescribed by a doctor or respiratory therapist, may help postpone the loss of respiratory function.

In recent years, many forms of "noninvasive" ventilation have been developed. These devices are called noninvasive because they don’t involve a surgical invasion of the body. Often, they can be used part-time for several hours a day.

"Invasive" ventilation is via a tracheostomy, a surgically created hole in the trachea through which air is mechanically forced.

Noninvasive ventilation comes in many forms. One common way to deliver positive-pressure, noninvasive ventilation is with a facial mask (see Figure 6). Air is forced through the mask under pressure on a timed cycle. One type of machine, called a BiPAP, delivers air at two pressures — one for inspiration and one for expiration. If this kind of device is going to be used for a long time, it’s best to have a custom-fitted mask made through your hospital’s respiratory therapy department.

Figure 6

Another way to support respiration without invasive techniques is with a negative-pressure ventilator (see Figure 7). Instead of forcing air into the person, these ventilators — descendants of the old "iron lung" — create negative pressure around the chest, allowing the lungs to expand. The negative pressure is applied on a timed cycle, so that the lungs alternately inflate and deflate, as in regular breathing.

Figure 7

The most complete type of ventilation is the positive-pressure ventilator with a surgically created tracheostomy. A positive-pressure ventilator is attached to the hole in the trachea, through which it delivers air on a timed cycle. It can be adjusted to respond to the person’s own efforts to breathe, allowing him to take breaths on his own but ensuring he gets a minimum number of breaths per minute, or it can be set to completely override the person’s own breathing efforts.

To use this type of ventilation, the patient first has to have a surgical procedure to make the hole in the trachea (see Figure 8). This can often be done under local anesthesia with sedation.

The decision to start tracheostomy-delivered ventilation often becomes a permanent one, since it’s usually impossible for a person with ALS to breathe on his own after being "weaned" from tracheal ventilation. Mechanical ventilation through a tracheostomy can prolong life by years, which for some people seems very worthwhile, and for others does not.

Life isn’t as bleak for people on invasive ventilation as it once was, when people were kept in institutions because of their machinery. Actor Christopher Reeve provides a good example of someone on total ventilatory support, living at home and enjoying many of the good things in life — family, friends, movies, even working.

Ventilators are now small, portable and relatively quiet. However, maintaining a person at home on a ventilator can be extremely taxing for the caregiver and very expensive. (See Resources.)

Figure 8

Many people with ALS have written that losing the power of speech is the worst part of the disease. Speech is something most people take for granted, and it’s a vital tool that keeps us connected with loved ones, friends and co-workers.

Speech may be partially lost relatively early in the course of ALS. Eventually, usually late in the disease, speech may be lost entirely, because of loss of the muscles that allow the vocal cords to function and loss of muscles in the lips and tongue that are used to form words.

Before speech is lost, a referral to your medical center’s speech therapy or speech pathology department can be helpful. You can get this referral through your MDA clinic or ALS center.

Speech-language pathologists help a person with ALS make the best use of the muscles he still has.

Also while speech is still possible, it’s a good idea to start planning for a time when this ability will be lost. Solutions for those who have lost the power of speech range from very low-tech to very high-tech.

At the low-tech end are writing tools, such as a pad and pencil, magic slate (you lift a piece of cellophane to erase what you’ve written), or chalk board.

However, many (not all) people with advanced ALS will have lost the ability to use their fingers to write before they lose their ability to speak. For them, a low-tech solution might include using a pointer and a board with letters, phrases or pictures, either purchased from a commercial supply house or made at home.

In the mid-tech range are ordinary computers, which can be improved considerably by software programs that allow whole words and phrases to be typed when the operator presses only one key ("word prediction" programs).

Moving toward high-tech solutions, you can purchase computers that speak for you as you type, and you can buy computer "switches" (what computer people usually think of as a type of "mouse") that let the operator use the computer with any muscle he has — mouth, forehead, little toe and so forth.

There are also systems that allow an operator to use the computer just by focusing his eyes on different parts of the screen. (See Resources.)

MDA now provides financial assistance with the purchase of communication devices prescribed by MDA clinic physicians.

Meeting Psychological and Emotional Needs

Doctors once thought ALS didn’t affect the psyche or the emotions. But recent studies have explored a phenomenon of unwanted laughing and crying that occurs in some people with ALS. Researchers believe this an effect of neurological damage in the disease.

Certainly, the thinking and feeling areas in the brain aren’t severely affected by ALS, as they are in, for example, Alzheimer’s or Huntington’s disease.

Of course, a disease like ALS has a profound effect on the psyche and emotions of the person with the disease and anyone close to him. This topic is explored in "Your Loved One's Feelings."