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 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
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
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
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 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
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
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
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
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
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 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.
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
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
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.
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
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
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
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 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