Amyotrophic Lateral Sclerosis: An Overview for Pharmacists

Amyotrophic Lateral Sclerosis: An Overview for Pharmacists
Andrew Skouby, a second-year pharmacy student from Mentor, Ohio; Andrew M. Roecker,
PharmD, ’00, BCPS, Associate Professor of Pharmacy Practice
This knowledge-based activity is targeted for all pharmacists and is acceptable for 1.0 hour (0.1
CEU) of continuing education credit. This course requires completion of the program evaluation
and at least a 70 percent grade on the program assessment questions.
ACPE Universal Activity Number 0048-0000-11-032-H01-P
Objectives
After completion of this program, the reader will be able to:
1. Describe and differentiate between the three types of ALS.
2. Describe the process used to diagnose ALS.
3. Explain the perceived mechanism of action for riluzole.
4. Discuss the factors thought to have an influence on the pathogenesis of ALS.
5. List several treatment modalities that may improve the quality of life for ALS patients.
6. Describe the role of a pharmacist in the life of an ALS patient.
Overview
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative muscular disease
that predominately occurs in the adult population. The disease has also been known as motor
neuron disease, Charcot’s disease, and Lou Gehrig’s disease. 1 Prevalence of ALS is
approximately 20% higher in men than it is in women. 2 ALS is increasing in incidence at a rate
that is faster than can be explained by the aging population. 3 It is estimated that 5600 new cases
of ALS are diagnosed each year in the United States, and that around 30,000 people may have
the disease at any given time. ALS affects between 1.2 and 1.8 per 100,000 individuals.
However, as this number takes into account the entire U.S. population, and ALS is a disease that
most commonly presents later in a patient’s life; this number is heavily skewed when it comes to
overall risk. 2
ALS is one of the more complex and misunderstood diseases that healthcare
professionals may encounter for various different reasons: (1) there are multiple different forms
of the disease 3 , (2) the pathogenesis is not fully delineated 1 , (3) diagnosis can occur only by
exclusion, (4) there exists only one FDA approved medication, riluzole, for the treatment of
ALS 2 , and (5) there is no cure. Several research designs are currently in progress in order to try
to understand the disease so that it is possible to treat and potentially discover a cure. As the
disease continues to increase in incidence, pharmacists practicing in both a hospital and
community setting are becoming more and more likely to come into contact with patients
plagued by ALS. This article is meant to prepare the pharmacist for that proposition by
providing an insight into disease pathogenesis and prognosis, the treatment plans for patients
with the disease, and the role that the pharmacist may hold in ALS patients’ lives.

Disease Types
There exist three forms of ALS and each affects the human body in similar, yet distinctly
different ways. Least prevalent, the Western Pacific form of ALS was originally observed in
Guam and the surrounding Trust Territories. 2 Along with possessing the general characteristics
of the disease, to be discussed in the forthcoming section, patients with this form of ALS exhibit
an abnormally high incidence of dementia 3 . Fortunately, prevalence of this type of ALS is near
negligible in the United States and is therefore beyond the scope of this article.
However, familial ALS (fALS) is more common. The familial form of the disease,
accounting for approximately 5-10% of all diagnosed ALS cases 2 , can be inherited from
generation to generation. Because of its obvious link to genetics, fALS is especially intriguing to
researchers. Though there are several genes that cause motor neuron diseases, only three are
directly linked to the ALS phenotype. 3 And though the complete route through which ALS is
transmitted via the genetic material is still not understood, researchers are most interested in one
specific gene whose mutation occurs in somewhere between 20-25% of all fALS patients. The
cytosolic Cu/Zn superoxide dismutase 1 (SOD1) gene is transmitted via an autosomal dominant
route. 3 Therefore, there is a 50% chance that the mutation will be transmitted to offspring of the
diseased. 1 Specifically a concern for fALS, the mean age of onset for the disease is 55-65 years
of age. 4 Thus, uninformed individuals with an ALS-causing mutation could easily pass the
mutation on to their progeny without any knowledge of the disease that may be present in their
genome.
In contrast to Western Pacific and Familial ALS, Sporadic ALS is the most prevalent
form of the disease in the United States. Sporadic ALS (to be referred to simply as ALS)
accounts for 90-95% of newly diagnosed cases in the United States. 2 Patients afflicted with this
subset of the disease are clinically known as typical ALS patients and exhibit the most common
features of the disease. 3
Clinical Signs
ALS clinical signs and symptoms are typically divided into two distinct subtypes: those
of the upper motor neurons and those of the lower motor neurons. Symptoms of upper motor
neuron destruction include, but are not limited to, moderate weakness, loss of dexterity, slowed
movements, spasticity, and hyperreflexia. Symptoms of lower motor neuron destruction include,
but are not limited to, severe weakness, hyporeflexia, muscle atrophy, fasciculations (involuntary
muscle twitches), muscle cramps, and muscle hypotonicity. In addition to these clinical signs,
there are also several neuropathological aspects of the disease, usually associated with loss of
muscle control, and occasionally with cases of dementia. 3
Pathogenesis
The full pathogenesis of ALS is not well understood as it has not been fully elucidated by
medical research. As with other neuromuscular degerative diseases, several key factors can be
noted when it comes to ALS pathogenesis. These factors include: (1) Genetics, (2)
Excitotoxicity, (3) Oxidative stress, (4) Mitochondral dysfunction, (5) Impaired axonal transport,
(6) Neurofilament aggregation, (7) Protein aggregation, (8) Inflammatory dysfuction and
contribution of non-neuronal cells, (9) Deficits of neurotrophic factors and dysfunction of
signalling pathways, and (10) Apoptosis. 1,5
(1) Genetics: As previously mentioned, around 20% of fALS patients and 2% of
sporadic ALS patients present with the SOD1 gene mutation. It is known that there may be as

many as six gene loci that code for the ALS phenotype, but only three have been identified.
Several other mutations have also been documented to possibly take part in the pathogenesis of
ALS. Most of the genetics are passed via the autosomal dominant route, though some are
autosomal recessive and others may be sex-linked. 1
(2) Excitotoxicity: Excitotoxicity is a term used to signify the damage undertaken by
neuronal cells that possess overstimulated glutamate receptors, as glutamate is the major
excitatory neurotransmitter in the human central nervous system. 8 As SOD1 codes for the major
reuptake protein of glutamate, a mutation limits the concentration levels of that reuptake protein,
allowing an excessive amount of glutamate to be present in the neuronal synapse. It is also
postulated that glutermatergic toxicity plays a direct role in the destruction of neuronal cells in
patients with ALS. 5
(3) Oxidative stress: Oxidative stress is of particular interest to researchers due to the
fact that the SOD1 gene mutation that is known to cause ALS normally codes for an anti-oxidant
protein. 8 Post-mortem studies of ALS victims have shown that diseased cells show a higher
sensitivity to radicalization than those of a control. Also, free radical tissue damage is an
accepted and acknowleged symptom of ALS. 1
(4) Mitochondrial dysfunction: There are many new data that have come to support
the theory that mitochondrial dysfunction plays a large role in the pathogenesis of ALS.
Multiple cases of dysfunctional mitochondria have been noted in post-mortem analyses of ALS
patients. 1 Dysfunctional mitochondria have also been linked to the SOD1 gene mutation in mice
models. 5
(5) Impaired axonal transport: The theory that axonal transport is key to ALS
pathogenesis stems from SOD1 transgenic mice models. Mice from these models often show
slowed anterograde and retrograde axonal transport. No human ALS patient has yet to present
with this problem, yet it is known to occur in several other neuromuscular disorders of the
human body. 1
(6) Neurofilament aggregation: Abnormal neuronal assembly, including those with
accumulation of neurofilaments, are often seen in ALS patients. Neurofilaments can combine
with a toxic form of peripherin, an intermediate filament protein, and be toxic to neurons even at
modest concentration levels. This combination has been found in the spinal cord of ALS patients
and not in controls. This evidence points to neurofilament aggregation as being a part of ALS
pathogenesis. 1
(7) Protein aggregation: Long debates as to whether protein aggregation take a part in
disease pathogenesis have been had. It is possible that these inclusions may simply be innocent
bystanders, or even beneficial to the cells. 1 In mutant SOD1 transgenic mice models, protein
inclusion bodies are seen in most neurons of the afflicted mice, making it possible that protein
aggregation exists as a part of ALS pathogenesis. Several theories have been put forth as to how
protein inclusions in neurons may lead to cell toxicity, but nothing has yet been confirmed. 5
(8) Inflammatory dysfunction and contribution of non-neuronal cells: It has been
recently discovered that SOD1 mutations alone are insufficient to cause ALS in transgenic mice,
making the case that non-neuronal cells may play a part in ALS pathogenesis. 5 ALS patients
commonly experience activation of the non-neuronal microglial and dendritic cells. This
activation has been shown to produce inflammatory cytokines such as interleukins and tumor
necrosis factor (TNF). However, recent trials have yet to see success in utilizing
immunomodulatory drug therapies to curve the progression of ALS. 1

(9) Deficits of neurotrophic factors and dysfunction of signalling pathways:
Lowered levels of neurotrophic factors have been noticed in post-mortem analysis of several
ALS patients. In addition, three mutations in the Vascular Endothelial Growth Factor (VEGF)
gene were thought to be associated with an increased risk for developing ALS. However,
recently these finding have come under scrutiny due to an inability to replicate research results. 1
(10) Apoptosis: Current research also skews towards examining if ALS motor neuron
destruction occurs via a cell programmed death, or apoptosis. Several studies have shown that
cell death due to ALS often occurs because of this programmed apoptosis, yet these findings are
still being reviewed and discussed heavily. 5
Diagnosis
The diagnosis of ALS in a patient is not an easy one to come to, and the time that passes
between symptom onset and diagnosis is often up to an entire year. 6,7 Because of the noted lack
of understanding in the pathogenesis of ALS, the diagnosis is done almost purely by excluding
other neurodegenerative disorders. The El Escorial diagnostic criteria are the most commonly
accepted for the diagnosis of ALS. Using these criteria, ALS is diagnosed if the following
conditions are met:
A: the presence of the following:
1. Evidence of lower motor neuron (LMN) degeneration,
2. Evidence of upper motor neuron (UMN) degenration, and
3. Continuing spread of symptoms within one body region to other body regions, and
B: the absence of the following:
1. Any kind of pathological evidence of a disease state that may account for the LMN or
UMN degeneration, and
2. Neuroimaging evidence that may explain any of the experienced symptoms 7
It is because of these complex requirements that ALS often goes so long before being
diagnosed. ALS diagnoses are therefore classified into six separate “El Escorial” categories: (1)
suspected ALS, (2) possible ALS, (3) probable ALS, (4) probable (laboratory supported) ALS,
(5) definite fALS, and (6) definite ALS. The main factor that allows a patient to move through
the ranks from suspected ALS to definite ALS is simply time to allow the symptoms to progress
and become more defining. fALS becomes definite if signs and symptoms exist in the presence
of the SOD1 gene mutation discussed above. 3,7
Riluzole
There exists only a single FDA approved medication for the treatment of ALS. The drug
is called Riluzole, has a brand name of Rilutek®, and is manufactured by Rhone Poulenc Rorer
Pharmaceutical Inc. The medication comes in the single dose of a 50 mg tablet. The drug
maintains the lone indication of aiding the prognosis of patients with ALS by extending survival
time or extending the time until a tracheostomy is required. The tablet is to be taken at the same
times each day once every 12 hours, at least an hour before, or two after meals in order to
decrease the likelihood of a food-decreased bioavailability.

As noted above, the pathogenesis of ALS is not fully delineated. It is therefore not
surprising that the mode of action for riluzole is also not fully understood. Although the reason
for the effects on ALS prognosis are not clear, riluzole exerts the following pharmacologic
effects:
1. An inhibitory effect on glutamate release
2. Inactivation of voltage-dependent sodium channels
3. Ability to interfere with intracellular events that follow transmitter binding at excitatory
amino acid receptors 8
It is commonly thought that the ability to inhibit the effect of glutamate release has the strongest
effect on ALS pathogenesis. It has been hypothesized that in ALS, glutamate concentrates in the
synapses of motor neurons and causes them to die. 9 In rat-tested animal trials, riluzole protected
rat motor neurons from the excitatory effects of glutamic acid and prevented the death of cortical
neurons. 8 Recent studies indicate that riluzole will prolong median survival rates by anywhere
from two to three months 4 , and has a 9% chance to increase lifespan by a full year. 6 The drug is
usually well tolerated, and the most common side effects include asthenia, nausea,
gastrointestinal tract upset, and abnormal liver function tests. Therefore, liver function should be
monitored at baseline and at regular intervals while taking riluzole.
Pharmacokinetics of Riluzole
Absorbtion/Distribution
Riluzole is absorbed well from the gastrointestinal tract with approximately 90%
absorption with absolute bioavailability of the medication at roughly 60%. This absorption is
adversely affected when taken with a high-fat meal as area under the curve (AUC) decreases by
20% with corresponding peak blood levels of riluzole decreased by 45%. This strengthens the
need for appropriate patient education on timing of riluzole administration to provide more
consistent levels of the medication. Riluzole is highly protein bound (96%) to albumin and
lipoproteins which allow for wide distribution including areas of the central nervous system. 8,10
Metabolism / Excretion:
Metabolism of riluzole is dependent on cytochrome P450 1A2 (CYP1A2) activity and is
entirely hepatic in activity. CYP1A2 is responsible for hydroxylation and glucuronidation of
riluzole to 6 major metabolites and several minor metabolites. There is little evidence that other
CYP enzymes are involved in the metabolism of this medication. Potential drug interactions
could occur with activitites/medications that either induce (smoking) or inhibit (fluvoxamine or
fluoroquinolone antibiotics) CYP1A2 and should be closely monitored for. There are also a
number of CYP1A2 polymorphisms that help explain interpatient variability with riluzole
metabolism. It is important to monitor closely for signs of adverse reactions upon initiation of
therapy with riluzole. 8-10
The mean elimination half-life of riluzole is 12 hours with 90% excreted in urine. Most
is eliminated as glucuronidated metabolites (85%) with only 2% eliminated as unchanged drug.
Only 5% of riluzole is excreted in feces. During clinical trials, there was still evidence of
riluzole elimination up to 7 days following initial dosing with this effect explained by slow direct
glucuronidation of riluzole in humans. 8-10

Symptomatic Treatment
As riluzole is the only approved treatment of ALS, several ancillary treatments are
necessary to relieve ALS symptomatology, in an effort to improve patient quality of life. As
such, it has been noted that patients who seek relief from multidisciplinary healthcare settings
see, on average, a 7.5 month increase in lifespan compared to those who strictly seek relief from
a neurology clinic. 6
Symptom management and ancillary treatment aimed at symptomatology was found to be
increasingly beneficial. 1 The following figure presents several symptoms of ALS and provides
possibilities for both pharmacologic and non-pharmacologic approaches to reduce the negative
effects of these symptoms.
Symptom 1,8-10 Treatment (Pharmacologic) Treatment (Nonpharmacologic)
Muscle Cramping
• carbamazepine: commonly
used as anti-seizure medication,
• physiotherapy
has muscle relaxant and
anticholergenic properties
• phenytoin: commonly used as
• physical exercise
• massage
anti-convulsant and works by
directly interacting with sodium
ion pumps in the motor cortex
• hydrotherapy
Muscle Spasticity
Excess/Watery Saliva
• quinine sulfate: proposed as an
incredibly effective treatment,
however the FDA has recently
warned against its use against
cramping symptoms
• baclofen: has labeled
indication for reversible
spasticity by inhibiting the
synaptic reflexes at the spinal
cord level
• tizanidine: has labeled
indication for muscle spasticity
by decreasing excitatory input to
alpha motor neurons
• dantrolene: has labeled
indication for spasticity
associated with UMN disorders
by interfering with calcium ion
channels
• atropine: labeled indication for
inhibiting salivary secretions by
blocking the action of
acetylcholine in secretory glands,
and by drying salivary secretions
• physiotherapy
• hydrotherapy
• cryotherapy
• home suction device
• drinking dark grape juice
• using sugar-free citrus lozenges

Pain
• hyoscine drugs: labeled
indication to decrease salivary
secretions by blocking the action
of acetylcholine in secretory
glands and by drying secretions
• glycopyrrolate: labeled
indication to inhibit salivation
and excessive secretions by
blocking the action of
acetylcholine in secretory glands
• amitriptyline: labeled
indication is for depression, yet is
occasionally used for excessively
watery saliva
• propranolol: labeled indication
is for management of
hypertension, yet is occasionally
used to control persistent salivary
secretions
• metoprolol: labeled indication
for management of hypertension,
yet is occasionally used to
control persistent salivary
secretions
• analgesics
• NSAIDs
• opioids
• nebulization
• steam inhalation
• injections of botulinum toxin
into the parotid glands
• irradiation of the salivary
glands
• use of an assisted cough
insufflator-exsufflator
• rehydration with jelly or ice
• drinking pineapple or papaya
juice
• reduced intake of dairy
products, alcohol, and caffeine
• offering comfort
In addition to the symptoms listed in the above table, some patients present with
excessive or violent yawning. These patients may find relief by taking baclofen. For
laryngospasms, patients should take lorazepam. Also, patients may experience constipation, for
which lactulose or senna with hydration and increased fiber intake should help with this
symptom. 1
Malnutrition is commonly seen in ALS patients due to the myriad of ALS-associated
symptoms contributing to the etiology. The health care team needs to be wary of the possibility
that the patient will stop eating certain foods due to a difficulty of swallowing or constipation.
Dieticians should be consulted in order to ensure that correct fluid consistancy of food is utilized
in order to aid in proper nutrition for patients. 6
Depression, anxiety, insomnia and fatigue are other common symptoms that ALS patients
are forced to deal with. Common prescription and over-the-counter medications that are utilized
for these symptoms can be prescribed and dispensed to the ALS patient. In addition to these
medications, non-pharmacologic treatments such as phychological support, counseling, and
comfort should be provided to patients experiencing these symptoms.
In addition to physical symptoms, the emotional stress of having difficulties
communicating often takes its toll on patients. Possible treatments for symptoms such as this
include: learning new speaking techniques, low-tech augmentative and alternative

communication tools, voice amplifiers, light writers, scanning systems operated by switches, and
brain-computer interfaces. Some of these solutions are only for very rare cases. It is important
that, when confronted with an ALS patient, a pharmacist be empathetic to the patient’s individual
disease characteristics and how he/she may work to help relieve that symptomatology. 1
Prognosis
Large patient samples of those diagnosed with ALS consistantly show a median survival
time of somewhere between 2-5 years, depending on the location and onset of symptoms. For
those experiencing bulbar onset ALS (whose symptoms begin in the brainstem), the median
survival time is 2-3 years, and for those experienceing limb onset ALS the median survival time
is 3-5 years. Only around 4% of patients will live 10 years from the onset of symptoms.
However, some population based studies note that a 5 year survival rate may actually be as low
as 4%. 1
As previously noted, treatment of ALS with riluzole may extend life (or time until
tracheostomy) by around 2-3 months. In addition to riluzole use, other indicators of survival
time include: site of symptom onset, age of symptom onset, time between symptom onset and
diagnosis, and El Escorial category at presentation. 1
Current Research
As noted several times over, both pathogenesis and treatment of ALS are not understood
to an adequate degree. Thus far, the scientific community has merely been able to make
educated guesses as to the causes of ALS and, therefore, how to treat the disease state. ALS is a
hotbed for research due to the devastating effects that it has on the human body and the lack of
information that currently exists. Several recent and/or current studies are evaluating different
perspectives in the overall treatment and outcomes of ALS.
In early 2009, there were several clinical trials that were in the midst of being conducted.
There were four separate Phase I studies, eight Phase II studies, and four Phase III studies. 6
These tests have all yet to publish any data or conclusive findings relating to an improvement in
the current treatment modalities used in ALS patients today.
To diverge from unpublished data, one recent research model worked off of the body of
evidence emerging that hypothesized that lithium carbonate was successful in significantly
slowing the progression of ALS. This evidence emerged from a trial treating solely male
transgenic mice models that concluded that lithium carbonate was effective at increasing lifespan
in male mice. A research model was then crafted to attempt to universalize this treatment to
several mice models that included only female subjects. The mice in this model were treated
with lithium carbonate 37 mg/kg in an effort to prolong lifespan. The conclusion of the study
was that lithium carbonate presented no positive therapeutic effects in female SOD1 transgenic
mice. 11 The researchers were unable to exclude gender as the factor that prevented the results
from the earlier trial from being replicated. However, it is well known that SOD1 transgenic
mice show gender differences in terms of disease progression and lifespan, and that there are
noted treatments that will work for one gender, but not the other. 11
In addition to the above trials, several other pharmacologic agents have been tested for
effect on the progression of ALS. In transgenic mice models, treatment with thalidomide or
lenalidomide enhanced motor neuron performance and elongated lifespan. In the human clinical
trial, thalidomide was given to patients at a dose of up to 400 mg/day as was suggested for use in
other neurodegenerative diseases. The conclusion of the study failed to note any positive effects

produced by thalidomide in terms of disease progression or prolonged survival, and actually
noticed several negative side effects associated with thalidomide treatment, including
constipation, fatigue, neuropathy, and rash development. Researches note that, as lenalidomide
is a more potent analog and has a different side-effect profile, may be more effective as an ALS
treatment in humans. 12
Thinking in a different manner, Garvey et al. conducted a survey in order to determine
how the use of total laryngectomies (TL) were being used as symptomatic treatment for bulbar
dysfunction in ALS patients. The study concluded that TL are a relatively safe and effective
symptomatic treatment for ALS patients to seek out when that have severe bulbar dysfunction. 13
Additionally, more positive news coming out of recent research is the Qureshi et al. hypothesis
that ALS survival rates in placebo clinical trial groups have been steadily increasing over time
since 1990. The main reason that the authors propose for this noted statistic is simply improved
symptomatic care of the ALS patient population. 14
The regulation and control of ALS symptoms is one specific arena that the pharmacist in
particular can participate heavily in. The pharmacist is commonly noted as the most accessible
health-care professional, and this is especially important to terminal patients, as symptomatic
control is all that they can hope to acheive.
Pharmacist’s Place
At the culmination of every pharmacist’s education, he/she takes an oath that includes a
vow to place the welfare of humanity and the relief of suffering at the top of his/her priorities. It
is with this goal that a pharmacist must approach being a part of the treatment of a terminal
patient.
In the case of ALS patients, the pharmacist is likely to be most involved at the terminal
portion of the disease. At this point in their lives, patients will be experiencing many of the
symptoms noted above and will be seeking relief. The pharmacist can make it his/her objective
to aid these individuals by providing them with a means to be comfortable in the end stages of
their lives.
In their article, “Coping with Terminal Illness,” Dr. Guido Zanni and Dr. Charles Browne
cite the fact that different patients will want differing amounts of information depending on their
individual symptoms. 15 They also believe that pharmacists hold a key role in helping patients to
cope with their symptoms and their fate.
First off, pharmacists need to engage in an active partnership with their patients. They
need to explain options and symptomatic treatments both clearly, and in a way that an elderly
individual will be able to understand. Pharmacists are experienced in this type of activity, and
need to be sure to be especially diligent in providing information to terminal patients.
Also, it has been shown that terminal patients prefer to be told information in person and
not over the phone. As pharmacy becomes increasingly pace-motivated, especially in the retail
sector, it is critical that pharmacists still allocate time to face-to-face patient counseling.
Terminal patients in particular desire this one-on-one counseling time in order to ensure that they
are doing all that they possibly can in order to alleviate their symptoms.
In addition, ALS patients are extremely interested in symptomatic progression and how it
is going to be dealt with. It is true that a physician will undoubtedly discuss possible symptoms
with the patient, but Zanni notes that patients only recall around 50% of the information
presented to them by their physicians. Therefore, it is critical that the pharmacist be able to
explain current and progressive symptoms, as well as treatment options to an ALS patient.

To further extrapolate on the specific role of the pharmacist, it has been shown over and
over that a patients understanding of their situation directly impacts patient outcomes. Patients
lacking full knowledge of their conditions are at an increased risk for inadequate management of
end of life care, unnecessary hospitalizations, and (especially relevant to the pharmacist) poorer
symptomatic control. 15
It is the unique opportunity of the pharmacist to be able to help ALS patients with
specific symptoms that they experience. Many of the symptoms that ALS patients experience
are able to be managed with appropriate use of over the counter (OTC) medication and nonpharmacologic
intervention. It is also highly likely that new symptoms will arise without the
patient seeking out new prescriptions from his/her physician. It is the pharmacist’s place to
initiate patient-physician contact in order to facilitate the patients’ procurement of adequate and
satisfactory symptomatic management.
In addition to ensuring that the patient is receiving all necessary medications and
treatments, it is also the role of the pharmacist to take note of any possible side effects that are
being experienced. Asking open-ended questions about possible side effects will allow the
pharmacist to determine whether these adverse symptoms are simply symptoms of the disease
itself or true side effects from symptomatic treatment, as the two are easily confused by the
patient.
Additionally, a pharmacist must ensure that meetings with terminal patients occur in
appropriate meeting areas. Patients need to feel comfortable and that their right to privacy will
not be broken. True insight into the lives of the patient will not be had unless the patient feels
comfortable discussing their condition. Therefore, it is critical that the pharmacist maintain a
positive relationship with the patients that he/she serves on a daily basis. 15
Similarly, most research stipulates that health care professionals must avoid being too
pessimistic in initial discussion about terminal illness. 16 By the time an ALS patient comes into
the pharmacy, they will know the general characteristics of the disease. However, it is still
critical to assume a stance of positivity when it comes to symptomatic treatment. Be sure to be
truthful and realistic when it comes to symptom management, but never intimidating or overly
morbid.
It is the duty of a pharmacist to serve the public. ALS patients represent a very unique
subset of the American population, yet one that is unfortunately growing in prevalence every
year. This article is meant to give the pharmacist an insight into the condition, and provide a
means for one to understand how it causes damage, what this damage means for a patient, and
the best way to provide for these patients as a pharmacist.

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