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.
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
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
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
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!
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
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
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
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
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
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
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
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.
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
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
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.
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
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.
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:
trihexyphenidyl hydrochloride (Artane)
transdermal hyoscine (Scopolamine)
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
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.
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
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
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.
Muscle activity also helps even if it is only through passive range of motion
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
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.