Care of the ALS Patient


ALS is a disease that has no cure and no medical treatment beyond supportive care. Therefore the focus is on providing nursing care that helps the patient and family cope with the ever increasing disability and deteriorating breathing, maximizes communication, maintains comfort, and prevents further complications of immobility. The list of potential problems and nursing interventions for any immobilized patient is long and rather than reiterate common nursing interventions for common problems, the focus here will be on those that are additional or need to be tailored to meet the specific needs of the ALS patient, particularly those that apply to patients in later stages of ALS.

Communication

Although the loss of the ability to communicate is not a life threatening complication nor physically painful, it is perhaps the most emotionally and mentally devastating aspect of the disease, the most frightening, and surely the most frustrating. The loss of input into one's own care represents a loss of independence that makes paralysis seem a minor problem. The degree of helplessness is frightening. Most frustrating of all is the fact that this problem is "treatable" -- not by medicine but by technology -- yet many patients are not being helped. For example, finding a nurse call system that the patient can activate represents the very minimum in care and yet all too often it is not done.

A lack of information about the technology available, intimidation by high tech equipment, and high cost all combine to prevent ALS patients from getting the needed equipment. A simple Internet search will turn up several suppliers for the equipment. Early use of the equipment will allow the ALS patient to become comfortable and proficient with it before it is desperately needed.

In 2001 Medicare began covering the cost of some communication aids. With the help and active support of care givers, few if any ALS patients need to be left "Locked In" by the disease.

If the problem is primarily lack of breath control for speaking, equipment is available to magnify a weak whisper into audible speech. If the problem is paralysis of the muscles needed to form words, magnification won't help, but as long as the patient retains the ability to move nearly any body part -- hand, knee, foot, mouth, eye brow -- that small movement can unlock the potential for at least basic communication.

There are a multitude of communication devices available, ranging from simple nurse call buttons to portable boxes that can speak a few pre-recorded words/messages, to larger ones that allow the patient to "type" in his own messages and even have it spoken aloud. Any of these can be operated using easy to press "switches" that replace hard to press buttons. These are called "switches" for their electronic function but are actually simply modified push buttons. Some are extra sensitive to pressure and can be activated with slight pressure from a hand, foot, knee, elbow, etc. Some are extra large for easy pressing by clumsy hands that cannot handle small buttons. Others switches are activated my slight movement rather than pressure. A tilt of the head, blink, lift of an eyebrow can be used. Mouth/breath control by "sip" or "puff" is also possible.

Ordinary laptop and desktop computers can also be set up for use by patients with near total paralysis. One type of desktop set up allows the user to move the cursor simply by moving his eyes across the monitor screen. The mouse is replaced by whatever type of switch the patient finds easiest to use. Although it is a slower process than for other computer users, with this equipment an ALS patient has the potential for communication, entertainment, and even productivity. The fact that this technology is readily available and yet so many paralyzed people are left unable to communicate their simplest and most basic needs to their care givers is a tragedy.

What can a care giver do when the equipment is not available or the patient cannot use it anymore? Assuming that every effort has been made to get the equipment or further modify it to fit the patients deteriorating strength, the care giver can only fall back on low-tech aids such as letter boards. For a patient whose only movement is eye gaze, a clear plexiglass board with minimal words or symbols arranged on it is easy to make and works best for simple and fast communication. Facing the patient with the board held up between them, the caregiver can follow the gaze of the patient as he looks look toward the word. A number of boards can be made, each dealing with specific situations - activities, positioning, comfort, etc. A board set up with the alphabet can be used to allow the patient to spell out his own messages for specific communication.

If even this method fails, the care giver can only go about providing care while remembering that inside that motionless body and expressionless face, there is still a person who sees, hears, understands, feels. Provide distractions - TV, books on tape, time out of his room. And above all, talk to him, not about him.


Pseudobulbar Emotionalism and Depression

Pseudobulbar Emotionalism (emotional lability) is not a mood disorder but does generally respond to amitriptyline (Elavil) or fluvoxamine (Luvox). Depression is common as it is in any devastating disease and needs to be treated if persistent. Remember that the lack of facial expression may be due to weakness of the facial muscles, not depression. Nor does lack of expression indicate anger or ingratitude! We rely so much on non-verbal response that an expressionless patient seems angry, snooty, or depressed. It is amazingly frustrating, even irritating, to care for a patient who never smiles a thank you, never reacts to your best bedside chat. All you can do is assume they are smiling on the inside!


Respiratory Care

Nursing assessment of respiratory status includes all the basics (respiratory rate, depth, effort, use of accessory muscles, breath sounds, color, sputum production). One key point differs from standard assessment however. An ALS patient with significant impairment of respiratory muscles will not have "labored respirations" as evidenced by increased depth and use of accessory muscles. (If he had the muscles for increased depth he wouldn't be in trouble!) The real indication of respiratory distress is in the rate of respirations and heart rate. An ALS patient in trouble will have rapid but shallow respirations and increased heart rate.

Although the ALS patient is at risk for respiratory crisis from pneumonia, a simple cold, and even pulmonary emboli, sudden respiratory deterioration usually follows a slow slide.

In addition to patient complaints about the room being too warm or stuffy, chest aching discomfort, difficulty breathing especially when lying down or after meals, and headaches (especially morning headaches), other indicators of deteriorating respiratory status can include lethargy, drowsiness, confusion, anxiety, irritability, loss of appetite, fatigue, depression. In short, if there is a way to feel all-around lousy, respiratory insufficiency will do it!

Although respiratory deterioration is expected and respiratory failure is generally the cause of death in ALS, there are interventions that can significantly delay the need to chose between a ventilator or death, and, more importantly, greatly improve the patients quality of life. Unfortunately, many physicians are unfamiliar with these interventions or misinformed. For example, ALS patients are sometimes told by their doctors not to get flu shots. Flu and pneumonia vaccines are NOT known to have adverse effects on ALS patients beyond those seen in the general population however. Although the flu vaccine will not protect the patient from all respiratory infections and the pneumonias usually seen in late stage ALS are caused by organisms not covered by the pneumonia vaccine, they will protect the patient from most common "bugs" passed around among family, friends, and care givers.

One common problem for ALS patients is the inability to cough strongly enough to clear the airway of even the normal accumulation of mucus. In addition to making certain that fluid intake is sufficient to keep the secretions thin, an over-the-counter cough medicine containing the expectorant guaifenesin can help loosen the thick phlegm. A beta blocker, such as propranolol (Inderal) or metoprolol (Toprol) may also help reduce the amount of phlegm produced.

A weak cough can be made more effective by Quad coughing (assisting a cough by applying Heimlich-like pressure as the patient coughs), giving deep breaths with an ambu-bag to improve the cough, or using a "Cough Assist" device (a device which delivers a couple of deep breaths through a mask and then abruptly reverses to negative pressure to simulate / assist a cough).

Morning headaches are often the first sign that the next step in respiratory care, BiPAP, is indicated. Even in healthy people, breathing is shallower during sleep. For people with ALS, that little extra drop in volume can mean trouble since they are breathing shallowly to start with. As the disease progresses, patients find themselves waking up with headaches because the shallow breathing causes them to retain CO2 which gives them a headache. After they wake up and begin breathing more deeply, the headache goes away.

Other patients may wake up repeatedly during the night as the shallow breathing or even apnea triggers an internal alarm that wakes the patient. This can cause a sudden awakening jolt or just restless, fitful sleep. With broken sleep, the patient is deprived of REM sleep, the stage of sleep considered the most important. Sleep deprivation causes daytime sleepiness, lethargy, anxiety, irritability, confusion, difficulty thinking clearly and remembering things as well as physical problems such as poor appetite, nausea, increased heart rate and fatigue and weakness.

At this point, use of BiPAP (Bi-level Positive Airway Pressure) non-invasive ventilation is clearly indicated. The introduction of BiPAP in the early 1990's represented a major advance in respiratory care for neuromuscular disease giving the patient the opportunity for respiratory assistance short of a ventilator. Unlike a ventilator, no tracheostomy is needed. BiPAP is done using a mask over the nose that can be removed when not needed. Because it does not require a trach, it does not interfere with speech or swallowing. Unlike a full ventilator however, BiPAP requires that the patient be able to take a breath. BiPAP is NOT a life support machine -- it cannot take over breathing for the patient completely. It delivers a pressurized breath of air into the lungs, then drops the pressure to allow the patient to exhale.

The most common use of this type of machine is a CPAP machine for people with sleep apnea. CPAP is not tolerated by people with ALS. They require the extra settings provided by a BiPAP machine. Unfortunately many doctors are unfamiliar with its use in neuromuscular diseases even though BiPAP is now part of the Standard of Care/Practice Parameters for ALS patients. Too often the orders for the pressure settings are wrong, or oxygen is ordered. BiPAP uses only room air and that is all the ALS patient generally needs, but oxygen can be added in later stages if needed.

Pulmonary studies of FVC (Forced Vital Capacity) may be ordered to support the clinical evidence that BiPAP is needed. Ideally FVC readings should be done with the patient lying down because that is when the problem is most evident. There is no set FVC at which BiPAP is indicated however. Overnight monitoring of O2 Saturation can be done with a simple "clothes pin" monitor on the finger to detect drops in oxygenation from shallow breathing or apnea. ABG's may be ordered but are seldom necessary at this point unless the patient also has some other lung disease. If ABG's are done, it is important that they be done immediate on awakening: CO2 levels will begin to return to normal once the patient is awake. A full Sleep Study to prove nighttime breathing problems is seldom needed in ALS.

BiPAP is generally initiated for overnight use and most patients find that assisted breathing overnight also improves unassisted daytime breathing. This may be due to the rest given respiratory muscles during the night, but probably owes as much to the restorative powers of a good night's sleep. Similarly, appetite, strength, stamina, and mental and emotional state improve.

As breathing deteriorates, BiPAP is used for rest periods during the day, often after meals when breathing is more difficult, and eventually it is used continually. BiPAP can improve quality of life while delaying the need for invasive ventilation by months or years.

Although most patients adapt quickly to the annoyances and are successful in using BiPAP, patients who already have significant bulbar weakness may have problems. Weakness of the jaw and lip muscles make it impossible to close the mouth. Many physicians automatically assume that ALS patients cannot use BiPAP for this reason but jaw support straps or full face masks that cover the mouth as well as the nose may work.

When the oropharyngeal muscles weaken to the point where the glottis can't close off the esophagus, the air is pushed into the stomach rather than the lungs and the patient awakens uncomfortable and having difficulty breathing from the abdominal distention. Adjustment of BiPAP pressures and sleeping position may help, but this is often a signal that a change to invasive ventilation is necessary.

The pros and cons of invasive ventilation should have been discussed with the patient well before the time when the decision has to be made. In addition to quality of life, the financial cost and burden of care are huge considerations in the decision about going on a ventilator.

Patients need to know that the decision to go on a ventilator is not irreversible. If at some point they wish to discontinue ventilation and be allowed to die, it can be done legally and with all the medication needed for a peaceful death.

Some people use BiPAP as an intermediary step before going on a ventilator, others find that by the time BiPAP is no longer sufficient and they need to consider full (invasive) ventilation, their level of paralysis and quality of life is such that they do not want to prolong life with a ventilator.

When the patient has either rejected BiPAP, or has used it as long as possible but elects not to go on to a ventilator, the focus is on measures to reduce the discomfort and anxiety of insufficient respirations. For most patients, this end stage is peaceful with slowly deteriorating level of consciousness and death. For others it can be a nightmare of slow suffocation. Medications such as Ativan are given for anxiety and opiates, generally morphine, can be added if dyspnea is severe or constant. Morphine diminishes the respiratory drive that causes "air hunger" as well as relieving the discomforts of joint pain that is usually significant by this stage. Thorazine may also be used for restlessness.

Although these medications are used freely without (pointless) concern about addiction, the goal is not to stop respirations but only to ease discomfort. The use of these medications may further diminish respirations and death, already inevitable and imminent, may occur somewhat sooner that it otherwise might, but it will be peaceful. A similar protocol is used for patients who wish to be removed from a ventilator.

Nutrition

As swallowing problems develop, the patient needs to consider whether or not to have a feeding tube placed for feeding. Patients often put off doing this until weight loss is dramatic and every meal a frightening and exhausting battle with choking. The general consensus among ALS patients who have had it done is that they only wish they had done it sooner. Improved nutrition and fluid intake results in a big improvement in their general condition.

Patients whose respiratory status is deteriorating need to consider having the tube placed even if swallowing is not yet a major problem. Once FVC falls below 50%, the procedure for placing the feeding tube is complicated by the patients poor respiratory status. Weakness of the diaphragm (the muscle between the lungs and stomach) and shallow breathing have also been reported to allow the stomach to shift upward further under the rib cage making tube placement difficult. It is not at all uncommon for the procedure to be tried and canceled if left until breathing is significantly impaired. Earlier insertion of the feeding tube is now recommended in order to avoid both weight loss and problems in placing it. The patient can continue to enjoy eating orally and gradually supplement oral intake with tube feeding as swallowing deteriorates. Once placed, care of the tube fed ALS patient does not differ from other patients with tube feedings.

Bowel and Bladder

Although ALS is not proven to directly affect digestion and bowel motility, constipation is a frequent and often severe problem. The ALS patient's weak abdominal and chest muscles and difficulty taking or holding a deep breath make it hard to bear down and supply the push needed to have a bowel movement.

In addition to attention to a bowel program addressing diet, fluids, regular timing, careful selection of pain medications (non-constipating meds for frequent or regular use), and judicious use of laxatives, the care giver can help improve bowel function by providing optimal positioning. Contrary to the design of most toilets and commodes and especially high rise toilets and commodes designed to make lifting the patient to a standing position easier, the optimal position for a bowel movement is a squat -- fanny low, knees high. Any parent of a diapered toddler recognizes the position! A squatting position gives the best mechanical advantage to the muscles of the pelvic area and abdomen to supply extra force in pushing. Placing a footstool under the feet of the patient can help. With a safety belt on to prevent falling, the patient can lean forward on a pillow placed on his lap to further increase intra-abdominal pressure.

Comfort is important in order to concentrate on the job at hand. ALS patients have no loss of sensation and atrophied muscles in the buttocks offer little padding on a hard toilet seat. Most are reasonably comfortable but a bad fit between backside bones and the seat is really painful and may necessitate a different one, possibly cushioned.

Skin Care

Pressure sores are less common in ALS patients than in other immobilized patients. Some textbooks and articles suggest that there are changes in the skin that account for this, but one obvious reason is that ALS patients have full sensation. Pressure areas become very painful before breaking down. As long as the patient can communicate, pressure areas are unlikely to be allowed to reach the point of breaking down. Pressure relief cushions, mattresses, elbow pads, etc., are needed early in the course of the disease. In later stages when the patient is thin from muscle wasting, poorly nourished because of swallowing problems, and unable to communicate discomfort, the risk increases and more frequent repositioning and skin checks are necessary.

Eye Care

As paralysis spreads, the patient may develop weakness of the muscles of the eyelids. Most commonly the problem is an inability to close the eye. This quickly leads to severe drying of the cornea. Redness, itchiness, infection, scarring and vision loss can result. The eyelid does not have to be wide open all the time for this to begin. Early weakness can leave the eyes open just a bit during sleep. A simple observation of the patient during sleep will identify the onset of the problem and allow early intervention --- frequent eye drops while awake and taping the lids closed at night.

Droopy lids are less common but inability to see is another devastating blow to the alert and aware patient who is already dealing with difficulty communicating. Eyeglass frames with "lid crutches" attached can be used during waking hours. Frequent eye drops will be needed to prevent drying.

Mouth Care

Brushing the teeth of an ALS patient with spasticity can be very difficult because the jaw clamps shut in an involuntary spasm. Although it may seem impossible to get anything else into their mouths for cleaning, sponge "lollipops" should never be used with these patients. If the sponge gets pulled off the stick when you are trying to get it out of his mouth, he could choke on it. Instead use a bite block or make one out of old-fashioned white adhesive tape and sturdy wooden tongue depressors. Stack several tongue depressors together and wrap adhesive tape thickly around one end (half way up the stick) in layers. Make sure that it is securely taped down so it won't slide off. When finished, the taped end needs to be 1/2 to 3/4 of an inch thick. The layered tongue depressors give it strength and the adhesive tape hold them together and pads it.

To do mouth care, you will have to "sneak up" on those hyper-reflexive jaws. You have to get the bite block in place before the jaw clamps down. Try doing it when the patient is relaxed, even snoozing or yawning. (If spasticity is severe, this will be a job that you will have to do when the opportunity strikes, and not necessarily as part of his morning bath.) Quickly put taped end of the bite block to the back and side of his mouth between his molars. Do not turn it on edge, just put it in flat. *****DO NOT put it between the front teeth! ****** The jaw clamping reflex can be strong enough to break front teeth, especially if they are weak to start with. The molars are a flatter surface, much stronger and intended to withstand grinding pressure. The tape will give some padding to the sticks to protect the teeth, and putting the bite block in flat will spread the pressure out evenly over the teeth. If he does get it between his front teeth, just let go and wait for the muscles to relax. Pulling on it will only increase the pressure and keep the jaw tight longer.

With the bite block in place, his jaws will be held far enough apart for you to maneuver the tooth brush around surprisingly well. If you can't, get a smaller tooth brush or make the next bite block thicker. Doing this while the patient is sitting up or turned at least part way on his side and/or using a suction machine while allow you to use a little more water without choking him. Be careful with the round plastic "wand" of the suction equipment however. It does not make a good bite block at all! To get the bite block out, just let go and wait for the muscles to relax. Sometimes by the time you are done with a good brushing, the muscles are already relaxing.

If it becomes impossible to even get a bite block in place, there is a nasty little tool called a jaw screw that can be used. It is basically a short, fat, plastic screw. The tip is placed between the molars and as the screw is turned, the jaw is wedged open. This is very hard on the teeth, even the molars, and there is always some risk of breaking them. You also have to be very careful not to catch and grind up the corner of the mouth in it. In short, it is something that should be used only if the jaw is constantly tight so that getting a bite block in is impossible, and then only by someone trained in its use.

Sialorrhea is the correct term for excess salivation and drooling. ALS patients do not produce extra saliva but swallowing problems reveal the surprising amount of saliva we normally produce and swallow without thought. Sialorrhea is understandably distressing to patients and is a problem often under treated because it may take trials of several medications before one is found that provides some relief without undesirable side effects. Often the patient is unwilling to continue the medication long enough for the side effects to lessen.

Medications that can be tried include:
  1. glycapyrrolate (Robinul))
  2. Amitriptyline (Elavil)
  3. benztropine (Cogentin)
  4. trihexyphenidyl hydrochloride (Artane)
  5. transdermal hyoscine (Scopolamine)
  6. Atropine

For or thick mucus production associated with sialorrhea, the addition of a beta blocker, such as propranolol (Inderal) or metoprolol (Toprol) may help. External beam irradiation to a single salivary (parotid) gland to reduce saliva production is being used with good success by some specialists. Botox injection of the salivary glands is used with success in some patients. The effect lasts several months.

Range of Motion

As with any immobilized patient, passive range of motion and gentle stretching exercises are important to prevent contractures and frozen joints. Although such exercises serve no purpose in restoring function in the ALS patient, they are very important in preventing pain. With contractures and frozen joints, it becomes very difficult to position the patient comfortably.

Comfort

One of the most common misleading statements about ALS in textbooks is that there is no pain associated with it. Although it is true that for most patients there is no great pain directly due to the disease process, there are a few patients who do experience severe unexplained muscle and joint pain -- and nearly all patients experience significant discomfort at some point. Muscle cramping and spasticity can be very painful, joints ache as muscles weaken. The small discomforts of sitting or lying in one position reach a whole new level of misery when you cannot shift position enough to relieve them.

In ALS patients all the problems experienced by patients paralyzed by stroke or spinal injury have the added dimension of occurring in the presence of full sensation. Long before there is any visible skin breakdown, pressure areas burn and ache fiercely. Foot drop pulls the skin on the top of the foot until it feels like it will tear. Swollen ankles burn. Subluxed shoulders and frozen joints defy any attempt at comfortable positioning. And then there is the unreachable, unscratchable itch . . .

Most of these aches and pains can be minimized with correct and frequent repositioning and range of motion exercises. Even with good care however, they do tend to become problematic over time but generally can be relieved with simple analgesics (Tylenol, Ibuprofen). End stage patients often have significant discomfort and require stronger medications.

Patients with spasticity can experience severe discomfort. Spasticity is an upper motor neuron problem and is present to some degree in ALS. For some patients it is minimal, for others extreme. Spasticity can actually be helpful in maintaining function as the rigidity helps replace normal muscle strength, but it causes jerky, hard to control movements. Spasticity causes a tightening of muscles that results in a stiffening of that part of the body in an exaggerated reflex. It is actually triggering both the muscles to flex and the muscles to extend that part of the body at the same time. Spasticity tends to affects larger areas of the body - arm, leg, trunk, neck. All the muscles in the area tighten up and the entire area becomes so tight it hurts. A simple touch can trigger it and it may persist indefinitely.

Medications usually help, but spasticity is sometimes is a very stubborn problem. One consideration in treating spasticity is to find a balance between relieving excessive and painful spasticity and maintaining a certain level of spasticity which can be helpful by replacing muscle strength. The meds for spasticity are primarily Baclofen and Zanaflex. In 1996 the FDA approved the use of Baclofen delivered directly into the spinal fluid by an implanted pump for the treatment of spasticity due to spinal cord injury and this is now being used with good results on ALS patients with severe spasticity.

Muscle cramps are very common in ALS. They can occur in small muscles or as large "charlie horses" affecting any part of the body - fingers, hands, neck, jaw as well as arms and legs. The cramping becomes less severe with time because the weakening muscles simply can't work up a good cramp anymore. Quinine seems to be the most effective medication for muscle cramping. A low dose (half of a 260mg tablet) once or twice a day is usually sufficient. Higher doses can cause muscle weakness. Quinine has a very bitter taste so taking crushed tablets requires a lot of pudding! Baclofen is often ordered for muscle cramps but is seldom effective unless spasticity is being mistaken for cramps or triggering them.

Fasciculations (muscle twitchings) are probably due to nerve irritability. They occur in smaller muscle bundles inside large muscle bundles and can be observed as well as felt. Fasciculations are not so much painful as irritating and have been described as feeling like someone is popping corn inside the muscle. They can be incredibly persistent and strong enough to prevent sleep. No medication has been found reliably effective in stopping them but some patients find that a few minutes massage of the area will reduce them.

Positioning

As with any paralyzed patient, correct positioning is important to prevent contractures, skin breakdown, etc. Because the ALS patient has no loss of sensation, good positioning becomes a critical factor in comfort. One hour spent with an arm unsupported is miserable and can result in days of shoulder pain and sleepless nights.

Being confined to a bed is not comfortable and is generally unnecessary if a chair is adapted for the patient. Being able to sit up for several hours a day will have a positive effect on the patient breathing, digestion, skin, musculoskeletal system, and sleep pattern, not to mention his emotional well-being.

Of course the ideal chair is a wheelchair which has been customized to fit the patient. When such a chair is purchased, it will be fitted by OT/PT. However, with the cost of such chairs, all too often the patient is assigned to a "one size fits none" standard issue wheelchair, geri-chair or recliner. Regardless of what type of chair is used it should be customized to the patient and needs to be "assigned" to that patient. OT/PT should be called in to help the nursing staff make adaptations to the chair. Some important adjustments are:
  • Size of seat/distance from front to back (knees to tail bone) If this is too large, it either cuts off circulation to the lower legs or causes lower back pain and skin breakdown from excess pressure on the tail bone when leaning back.
  • Position/height of neck rest/pillow. When neck weakness causes the head to droop, a different style of head rest and/or a neck brace is needed. There is absolutely no need for any patient to sit with his chin on his chest or shoulder. It is every bit as uncomfortable as it looks!
  • Height from seat to floor/foot rests. This is extremely important in reducing swelling of the feet and development of blood clots.
  • Height of arm rests. Too high causes shoulder pain and elbow pressure. Too low leads to subluxation of the shoulder. This leads to aching discomfort when sitting and real pain when lying on the side.
  • Comfortable cushions. No standard seat cushion provides sufficient pressure relief for someone paralyzed. A ROHO air cushion or gel cushion is needed. Foam cushions are not recommended. They are better than nothing, but no variety of foam cushion (not even the "egg crate" style) provides adequate pressure relief for sitting. When a cushion is added, the height of both the foot rests and the arm rests needs to be readjusted.

A recliner is probably the worst choice for seating. Few have adjustable seat depth or arm rests (which are generally too low). Most importantly, using the recline position is likely to increase swelling of the feet and ankles rather than reduce it. The problem is that the human body was not designed to bear the weight of the leg on either the calf or the heel which is exactly where most recliner foot rests place it. Weight on the calf cuts off circulation and causes foot swelling. Weight on the heel causes a painful pressure area that will in time break down into a pressure sore. The ideal chair would be shaped to the curve of the leg to spread the weight evenly, but most recliners have a large gap between the seat and the foot rest, leaving no support behind the knee and upper calf -- all the weight is on the lower leg and heel. If a recliner must be used, use it as a regular chair. The foot rest should be used only for short periods of time if at all.

When trunk weakness causes slumping to the side, foam wedges that support the trunk are needed. If the knees/ankles turn in or out, a foam wedge between the knees can improve alignment and greatly increase comfort. A lap desk that wraps around to the sides to support the patients arms is not just a positioning aid. If the patient still has any finger dexterity, the support of the elbows and forearms can also greatly prolong the patients ability to use his hands.

Time invested in getting an ALS patient comfortable in bed is time well spent for any care giver. Because the ALS patient has full sensation, the little discomforts created by awkward positioning can make sleep impossible. Those little discomforts quickly graduate to pain when one is alone, unable to sleep, unable to make even the minor adjustments needed to ease the problem. The only hope for getting to sleep is to call the care giver back again and again until all the nit-picky positioning quirks are addressed. Every patient has their own individual "pre-flight checklist" for comfortable sleep, but some of the basic needs are a comfortable mattress, a foot board to keep the weight off the blankets off the feet and reduce foot drop. (Yes, foot drop is miserably uncomfortable!) More common sleep destroyers are bad pillow position, a folded-over ear (incredibly painful after a bit!), heavy or tight blankets that restrict any weak movement the patient has left. A bedside checklist can help when care givers change often.

Swelling of legs

One very common source of discomfort for the ALS patient is the swelling of the feet and ankles. This begins when leg weakness prevents walking because muscle action is needed to help pump the blood back up the legs. When muscle movement is lost, blood pools in the veins. Water leaks from the distended veins out into the surrounding tissue creating the swelling (edema). With repeated episodes of swelling, water seeps into the tissues even more easily. At the same time, the one-way valves that help move blood upward are collapsing from the weight of pooled blood. That damage is permanent and swelling occurs even more readily.

Doctors often prescribe diuretics, but unless the patient has kidney or heart problems this should be the last resort, not the first. Diuretics remove fluid, putting the patient at greater risk of blood clots and don't address the underlying problem of poor blood flow.

First, make certain that when the patient is up in the chair, the distance from the seat to the floor/foot rests is correct. Having the legs "dangle" is a sure-fire way to cause swelling!  Put a box/platform under the feet if necessary to make sure that there is minimal pressure at the back of the lower thigh and knee.

Elevating the feet can help but only if it is done properly. The foot rest cannot be just under the calves and heels as that only further impairs circulation and leads to pressure sores on the heels. Putting the feet up without "unfolding" at the hips is very minimally helpful, possibly even detrimental as that bend interferes with the already difficult job of moving blood upward to the heart. Elevating the legs effectively requires lowering the back rest to a reclining position so that the feet are level with or higher than the heart. Keeping the patient in this position defeats the entire purpose of getting the patient out of bed however. Elevating the feet for a short time several times a day or putting the patient back in bed for an hour or so in the afternoon is a much better solution.

Other interventions:
  • Muscle activity also helps even if it is only through passive range of motion exercises.
  • Although swollen legs feel cold and the impulse is to warm them, heat will only increase the swelling. Many ALS patients find that just a few minutes of sitting with feet by a heater or in hot sunshine will dilate blood vessels and set off the fierce burning pain of extreme swelling.
  • Limiting salt intake is often recommended, but again doesn't address the underlying circulation problem. It is probably sufficient to limit indulgence in very salty foods.
  • TED (elastic or compression) stockings can help and ALS patients with severe swelling problems are finding that "boots" that inflate and deflate to help pump the blood along work very well.

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