AMYOTROPHIC LATERAL SCLEROSIS

Background information – Amyotrophic Lateral Sclerosis (ALS) is a rare neurological disease characterized by progressive muscular weakness, dysphagia and dysarthria. It is also called Lou Gehrig’s Disease, and (in Britain) Motor Neurone Disease. There is no known cause and no specific treatment. It is invariably fatal. Patients benefit considerably from skillful, supportive management.

It is rare under the age of 30. The peak age of onset is 50 to 70 years.

The early symptoms are usually clumsiness in one hand, falls or slurred speech. As the disease progresses weakness tends to spread and become bilateral.

The pattern of involvement varies. Some patients have weak limbs only, some have bulbar symptoms only, but most patients progress to both.

Bulbar symptoms affect speech and swallowing. Emotional lability can occur, with uncontrolled crying (or laughing) due to loss of control of reflexes (not intellectual impairment). This can be partially controlled by breathing exercises.

The patient becomes progressively more dependent on the help of others for transport, transfer, feeding, washing and eating. (Regular respite care is essential to avoid exhaustion of family carers.)

Eventually the respiratory muscles are involved, with poor basal expansion of the chest (a clinical clue to a shortened prognosis), and either a chest infection supervenes or the patient develops terminal dyspnea for 24 to 48 hours (controllable with morphine), or sometimes dies suddenly while being moved.

Choking is very rarely the cause of death, occurring in only 1% of patients.

Many patients remain mentally alert right up to the moment of death, and explanation and encouragement continue to be important.

Diagnosis relies on clinical examination (there are no diagnostic tests).

The classical features are:

•  Fasciculations

•  Flaccid weakness in the arms

•  Spastic weakness in the legs

•  Bulbar signs

Generalized fasciculation is diagnostic and occurs in no other disease. It is most easily seen in the tongue and deltoid muscles. A fasciculation is a visible muscle twitch. (Compensatory branching of normal nerve fibers occurs so each nerve now stimulates a whole group of muscle fibers.)

The combination of lower motor neuron weakness and wasting (Amyotrophic) with upper motor neuron weakness and spasticity (due to Lateral Sclerosis of the spinal cord) is highly suggestive of ALS - especially if wasting and spasticity occur in the same muscle group. A myelogram is occasionally necessary to exclude cervical spondylosis which can mimic ALS by causing wasted arm muscles (due to nerve root damage) with spastic leg weakness (due to cord pressure).

Prognosis varies. The majority of patients die within 4 to 5 years after diagnosis. 25% of patients present with bulbar symptoms, and their prognosis is worse (2 to 3 years). In some patients the disease is confined to the limbs and respiratory muscles, and they have a better prognosis (10% survive 10 years).

Explanation of the cause of weakness is valuable (for patient and family). Explanation is supportive. Patients who are getting weaker, falling, and fearing what is wrong, are always relieved to know that it is a recognized condition with a name. Isolating a person from the truth increases his despair. It is essential to be open, and then to provide support. The diagnosis is not grasped in a moment. It usually takes the patient several weeks or months to adjust to the new situation.

It is especially helpful to explain which functions are not affected:

•  Sensation (pressure sores unlikely)

•  Intellect (unlike multiple sclerosis)

•  Eyesight

•  Hearing

•  Bladder control

•  Bowel control

•  Sexual potency

(Many persons mistakenly confuse ALS with multiple sclerosis.)

There are many types of specialized equipment available to help someone with ALS. These can improve a patient’s quality of life.

The key is skillful assessment and timely prescribing of appropriate equipment. Introduction of equipment before it is necessary can be demoralizing, but there is no point in equipment arriving too late for the patient to use because the disease has progressed. This increases the patient’s sense of frustration. (“If only I’d had this two months ago.”)

Some helpful equipment can include:

•  Communication aids (printer, computer equipment)

•  Mobility aids

•  Transfer aids (boards, hoists, slings, turning disc)

•  Armchairs, Spenco cushions

•  Wheelchairs (push, powered, reclining)

•  Mobile arm supports

•  Bathing aids (shower chair, bath lift)

•  Special toilet equipment

•  Eating aids (heated plate, 2-handled mug, straws)

•  Special beds and mattresses

•  Backrests, bed elevators

•  Special car equipment (car hoists, rotating seats)

•  Collar, head and neck supports

•  Page turner

•  Various environmental control systems

As the disease progresses, a patient usually needs differing types of particular aids and appliances (for example, one person may need 4 or 5 different types of communication aids at different times).

Expert advice from a skilled occupational therapist is essential.

Teamwork among professionals and family carers, especially including good communication, is essential.

The best way of coordinating care and involving appropriate professionals at the right time is by means of a key worker supporting the family.

Symptom control – A retrospective review of 100 patients with ALS at St. Christopher’s Hospice emphasized the differences that a determined program of symptomatic treatment coupled with a positive attitude can make to a patient’s quality of life and to the support of the family.

«  Attention to details is essential: What helps one patient may be unbearable for another.

80% of patients benefited from oral opioids, often initially only at night, with excellent results. (Being unable to turn over in bed causes very uncomfortable “bedache”.)

Morphine does not have to be reserved for the terminal stages. Low doses can transform a patient’s condition by abolishing the generalized aching that occurs if a person cannot change position frequently. Some patients take 5mg to 10mg of morphine at night for months with no danger of tolerance. Laxatives are essential with morphine.  (see Analgesics, Morphine)

Common symptoms in ALS are:

•  Weakness (100%)

•  Communication problems (90% eventually)

•  Dysphagia (75% eventually)

•  Dyspnea (60% eventually)

•  Falls

•  Muscular aching

•  Night cramps

•  Muscle spasms

•  Stiff joints

•  Constipation

•  Swollen legs

•  Poor sleep

•  Dribbling from mouth

•  Choking episodes

•  Sore eyes (reduced blinking)

•  Sore bottom

•  Anxiety

•  Panic attacks

•  Boredom

Dysphagia is often partially relieved by sucking ice before meals and applying an ice pack to the front of the neck for a few minutes, which reduces muscle spasm and improves the swallowing reflex. The patient should eat slowly and focus on lip closure (the first part of the reflex). Food must form a firm bolus to initiate the pharyngeal reflex (semisolid foods are usually best). Food may need to be placed at the back of the mouth (tongue movement is usually poor). Neck position is important (it is difficult to swallow with the neck either too flexed or too extended).

Dehydration or taking an unacceptably long time to eat are indications for a nasogastric tube. Fine bore tubes are well tolerated. Some patients like to try to eat normally again once they are rehydrated and rested, and some succeed. Severe persistent dysphagia is an indication for a gastrostomy.

Choking is a defensive cough reflex to prevent aspiration of food into the lungs. Explanation of this fact can help to overcome some of the fears. Choking can usually be considerably relieved by skillful nursing care. Meals need to be slow and relaxed to avoid muscle fatigue. Cough can be made more forceful by abdominal compression. Cricopharyngeal myotomy is likely to be helpful only if there is pharyngeal spasm (which tends to cause choking after eating as pooled food spills into the larynx). Repeated severe choking is an indication for a gastrostomy, although most patients can be successfully managed without one.

Dribbling (due to an inability to contain or swallow saliva) is a common problem. Normally 1 to 2 liters of saliva are produced and swallowed automatically each day. Some patients with ALS fear that the disease is making them produce excessive saliva, and that they may drown. Others fear that dribbling is a sign they are losing their minds.

Explaining the causes of dribbling can reassure the patient.

Atropine can be very useful. The patient may prefer the tablets or the liquid preparation. The dose must be carefully titrated (up to 1.2mg every 4 hours on occasion). Excess dosage will cause the equally unpleasant problem of a dry mouth. (It is important to warn the patient that blurred vision may occur as a side effect of atropine, and not as a progression of the disease.)

Sucking ice can sometimes reduce spasm and bunching of the tongue, and allow saliva to be channeled to the back of the throat.

A palatal lift (a plastic disc attached to an existing denture or removable plate) can help to close the soft palate during the first phase of swallowing, and can improve swallowing and reduce dribbling.

A portable suction machine used a few hours a day can improve comfort if saliva is pooling at the back of the throat. Carers need to be carefully instructed on the proper use of the machine.

Polo-neck bibs (“dickies”) with velcro fasteners can be changed regularly to prevent soiled clothing. These are more comfortable and less demeaning than regular bibs.

Bilateral neurectomy of the chorda tympani nerves (which innervate the salivary glands) is possible via the middle ear, and may be considered in cases of severe dribbling. Other techniques (including irradiation of the salivary glands) are rarely necessary.

Terminal phase – The patient must be comfortable, repositioned regularly, free of pain and dyspnea, and sleeping well. Regular mouth care is essential after every meal.

As the patient gets weaker and more dependent, communication and companionship become even more important. It is essential to support the family to enable them to support the patient. These patients become overwhelmingly dependent and vulnerable. They are often unable to communicate, yet remain mentally alert. Extra effort by carers is needed to maintain the patient’s self-esteem and sense of control.

A continuous subcutaneous infusion of drugs can be very useful in the terminal phase. (see Subcutaneous Infusions, Terminal Phase)

Artificial ventilation for terminal dyspnea is contraindicated. The patient can be kept free of distress with titrated doses of morphine. Ventilation simply prolongs the process of dying.

«  “It is madness to demand from medicine a cure which it cannot give, or for the body to resist a disease which is irresistible.” (Hippocrates)

These comments, made by patients with ALS, may help to draw attention to their special needs (and to the needs of many other patients, too):

“When a person is paralyzed quite ordinary discomforts assume the character of minor tortures.”

“For the average homebound invalid, conversation with visitors is the highlight of the day.”

“Loneliness is not so much a matter of being alone as of not belonging.”

“It is not realized that fear amounting to panic occurs by day or night, and it is therefore essential that help should be readily available. Contact can be made by means of a bell or buzzer which responds to the slightest touch.”

“Being told the disease was a progressive one enabled me to do things while I could.”

“I feel that patients and families should be told as much as possible in the home, in preference to the doctor’s office or the rather tense atmosphere of a hospital clinic.” 

“I find it difficult to talk to people who tower above me, because of difficulty with neck control.”

“Breathing exercises combined with simple speech exercises have not only kept my speech understandable but have also helped me with my swallowing.”

“An understanding of my unusual tiredness is very important. This illness needs rest during the day and lots of sleep at night. Over a period of time I have come to recognize within myself waves of energy, which are followed by long periods of exhaustion. Things can be tackled while my energy lasts which should not be attempted once the exhaustion has set in.”

“With the practical help and prayers of others I have found it possible to set aside the physical and let the mental and spiritual take over. This is a very enriching experience.”

“Doctors of today should not feel that because they cannot cure patients with ALS they cannot help them. They can indeed help them by their compassionate understanding and friendship.”

The author and publisher have taken precautions to ensure that the information in this book is error-free. However, readers must be guided by their own personal and professional standards of good practice in evaluating and applying recommendations made herein. The contents of this book represent the views and experience of the author, and not necessarily those of the publisher.