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Clinical Considerations
for Treating the Dental
Patient with ALS
A Peer-Reviewed Publication
Written by Kimberly M. Parsons, MEd, CDA, EFDA, RDH and Amy J. Schneider, LDH, CDA, BS
Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder that affects the nervous system, particularly the
motor neurons. It may affect either spinal or bulbar neurons,
which classifies how an individual’s symptoms present. The
prognosis for individuals with ALS is not promising, usually
resulting in death within three to five years of diagnosis.
Most management of amyotrophic lateral sclerosis focuses
on palliative care, determined by the affected individual’s
ongoing symptoms. An essential component of palliative
care is management of the individual’s oral conditions.
Manifestations of ALS may affect the dental practitioner’s
care of patients with this disease. These modifications will
be dependent upon the individual’s current symptoms and
needs.
Educational Objectives
At the conclusion of this educational
activity participants will be able to:
1.Describe amyotrophic lateral
sclerosis (ALS) and the manner(s) in
which it may present
2.Recognize common types of palliative care offered to individuals with
ALS
3.Describe the symptoms associated
with spinal and bulbar degeneration
4.Integrate appropriate modifications into the dental treatment of
patients with ALS
Author Profile
Kimberly M. Parsons, MEd, CDA, EFDA, RDH, is the dental assisting program chair
and an assistant professor of dental assisting/dental hygiene at the University
of Southern Indiana. Her scholarly activities include research in the areas of
educational technologies and dental education. Ms. Parsons has been a dental
hygienist for 13 years, practicing in Arizona, Indiana, Kentucky, and Michigan. She
can be reached at [email protected].
Amy J. Schneider, LDH, CDA, BS is an instructor of dental assisting/dental hygiene
at the University of Southern Indiana. She has been practicing dental hygiene
for 18 years in the state of Indiana. Ms. Schneider has personal experience with
individuals with ALS.
Author Disclosure
Kimberly M. Parsons and Amy J. Schneider have no commercial ties with the
sponsors or providers of the unrestricted educational grant for this course.
Go Green, Go Online to take your course
Publication date: June 2014
Expiration date: May 2017
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This educational activity was developed by PennWell’s Dental Group with no commercial support.
This course was written for dentists, dental hygienists and assistants, from novice to skilled.
Educational Methods: This course is a self-instructional journal and web activity.
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Requirements for Successful Completion: To obtain 1 CE credit for this educational activity you must pay the required
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CE Planner Disclosure: Heather Hodges, CE Coordinator does not have a leadership or commercial interest with products
or services discussed in this educational activity. Heather can be reached at [email protected]
Educational Disclaimer: Completing a single continuing education course does not provide enough information to result
in the participant being an expert in the field related to the course topic. It is a combination of many educational courses
and clinical experience that allows the participant to develop skills and expertise.
Image Authenticity Statement: The images in this educational activity have not been altered.
Scientific Integrity Statement: Information shared in this CE course is developed from clinical research and represents
the most current information available from evidence based dentistry.
Known Benefits and Limitations of the Data: The information presented in this educational activity is derived from the
data and information contained in reference section. The research data is extensive and provides direct benefit to the patient
and improvements in oral health.
Registration: The cost of this CE course is $20.00 for 1 CE credit.
Cancellation/Refund Policy: Any participant who is not 100% satisfied with this course can request a full refund by
contacting PennWell in writing.
Educational Objectives
At the conclusion of this educational activity participants will be
able to:
1. Describe amyotrophic lateral sclerosis (ALS) and the
manner(s) in which it may present
2. Recognize common types of palliative care offered to
individuals with ALS
3. Describe the symptoms associated with spinal and bulbar
degeneration
4. Integrate appropriate modifications into the dental treatment
of patients with ALS
Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder that affects the nervous system, particularly the
motor neurons. It may affect either spinal or bulbar neurons,
which classifies how an individual’s symptoms present. The
prognosis for individuals with ALS is not promising, usually
resulting in death within three to five years of diagnosis. Most
management of amyotrophic lateral sclerosis focuses on palliative care, determined by the affected individual’s ongoing
symptoms. An essential component of palliative care is management of the individual’s oral conditions. Manifestations of
ALS may affect the dental practitioner’s care of patients with
this disease. These modifications will be dependent upon the
individual’s current symptoms and needs.
Introduction
Amyotrophic lateral sclerosis (ALS), commonly referred to as Lou
Gehrig’s disease, is a progressive degenerative disorder that affects
the nervous system, particularly the motor neurons.1, 2 It may affect either spinal or bulbar neurons, which classify how symptoms
present in the individual.1 Currently, the cause of ALS is unknown,
although research indicates the possibility of genetic and environmental factors affecting its incidence.3 It is important for health care
practitioners to understand amyotrophic lateral sclerosis to enable
them to make modifications to patient care as needed.
Epidemiology and Prognosis
The sporadic form of amyotrophic lateral sclerosis, characterized
by motor neuron degeneration and premature death, comprises
the majority of all ALS cases.4 Juvenile-onset ALS, where disease onset occurs before age 30, comprises only five percent of
all ALS cases. Familial ALS occurs only five to ten percent of
the time. The more common sporadic form comprises the vast
majority of cases. The average onset of sporadic ALS occurs between 55 and 65 years, with a median age of 64 years.4 There is
a slightly higher incidence of sporadic ALS in men than women,
with a ratio of 1.5:1.4 The age of onset of familial ALS occurs
approximately ten years earlier than sporadic ALS, with males
and females equally affected.
The prognosis for individuals with amyotrophic lateral
sclerosis is not promising. Although there are possible treat-
84
06. 2014 | www.DENTALECONOMICS.com | @dentalmagazine
ments on the horizon, they are still in early developmental
stages. This rapidly progressive disease usually results in death
within three to five years of diagnosis, primarily from respiratory complications.5 Slower disease progression can occur in
younger individuals and those whose disease first presents in
the extremities. Only ten percent of individuals with ALS live
longer than eight years.5
Management of the Disease
Currently, management of ALS patients primarily focuses on
palliative care.6,7,8,9 Palliative care is a philosophy of care that
addresses the prevention and relief of suffering.6 Symptoms of
ALS greatly impact an individual’s quality of life and, therefore,
must be continually managed.8 A multidisciplinary approach
to care provides the individual with ALS with the most optimal
care choices, thus enhancing their quality of life.6 The National
Consensus Project Clinical Practice Guidelines for Quality
Palliative Care (NCPQPC) outline eight domains of palliative
care.6 These include: (1) physical care, such as pain management and physical symptoms; (2) psychological care that addresses depression, loss, grief, and other symptoms experienced
by both the patient and his/her loved ones; (3) social systems
available for the patient; (4) faith-based beliefs; (5) cultural
norms that are integrated into patient care; (6) ethical and legal
choices for the patient and family to define; (7) designation of
property and other legal aspects of care for the imminently dying patient; and (8) structure and processes associated with the
outcomes of ALS.6,8
The affected individual’s ongoing symptoms determine the
type of palliative care provided.7 Some common types of palliative care offered to individuals with amyotrophic lateral sclerosis
may include medication for the following symptoms: depression
or anxiety, drooling, bronchial secretions, pain, pathological
laughing, constipation, gastro-esophageal reflux, sleep disturbances, and venous thrombosis or pulmonary embolism. In
addition, dietary changes, increased hydration, physiotherapy,
and/or stretching may be utilized to address other symptoms.7
Management of oral conditions may be required as part of
the palliative treatment of an individual with ALS. Oral conditions may arise due to the effects of medication, sialorrhea
caused by ineffective clearance of saliva, thickened mucus, and/
or xerostomia that arises from mouth breathing.10 Management of these oral conditions is essential for patient comfort.
Adequate hydration and the use of a room humidifier can be
effective in decreasing xerostomia from mouth breathing during
sleep.10 In addition, an expectorant or beta-adrenergic blocking
agents, such as propranolol or metoprolol may be effective in
75% of patients experiencing thickened secretions.10 To address
sialorrhea, an anticholinergic, such as glycopyrolate can be
90% effective.10 Another option for sialorrhea is the injection
of botulinum toxin into the parotid and submandibular glands.
However, this type of care may result in temporary side effects,
such as bulbar and masseter muscle weakness.10
Table 1
Dental Treatment Modifications
ALS Symptom
Treatment Modification
Advanced disease
Care provider to learn oral home care
Dysphagia
Semi-supine treatment position
Dysphagia
Nutritional counseling
Excessive saliva
Additional practitioner to assist with
HVE
Muscle weakness/spasticity
Shorter appointment time or frequent
breaks during appointment
Muscle weakness/spasticity
Bite block/assistance with holding
mouth open
Currently, the only medication approved to slow the course
of the amyotrophic lateral sclerosis disease process is riluzole.3, 11
Riluzole, originally developed as an anti-epileptic drug, inhibits
the release of glutamate. However, this drug’s mechanism of action in ALS is currently unknown.3 Presently, the use of riluzole
prolongs the life of an individual with ALS by approximately four
months. Its safety and efficacy have been established for individuals with ALS, including those with advanced disease and in elderly
patients.3 More than half of ALS patients in the United States
utilize it.3
Symptoms Associated with Spinal Degeneration
Individuals that experience degeneration of the spinal neurons
usually exhibit weakness or spasticity of the lower limb muscles.
As a result, they may also experience disturbances in gait.1 Initial
presentation of symptoms in an individual with spinal neuron
involvement includes changes in the functional strength and
coordination of lower extremity muscles. Eventually this progresses to muscles of the upper extremities, and finally to the
bulbar muscles.1
Symptoms Associated With Bulbar Degeneration
Individuals with involvement of the bulbar neurons present
initially with speech problems resulting in dysarthria (motor
speech disorder), deterioration of respiratory function, and
weakness of the muscles of deglutition, which results in dysphagia.1 Dysphagia occurs in each ALS patient with bulbar
involvement and eventually occurs in all ALS patients, regardless of the time of disease onset. Dysphagia in ALS patients is
characterized by impairment of the oral and pharyngeal phases
of swallowing. Patients with amyotrophic lateral sclerosis
present with abnormal lingual movement. This abnormality
makes the act of swallowing effortful, inefficient, and fatiguing.1,12 Decreased tongue strength may also result in decreased
frequency of swallowing. This leads to accumulation of saliva
in the oral cavity, which can result in drooling, aspiration, and
coughing.9 Salivary control, along with other secretions, is a
common challenge for patients with ALS.1
Dental Treatment Modifications
Manifestations of amyotrophic lateral sclerosis affect the dental
practitioner’s care of patients with ALS (Table 1). Due to the high
occurrence of dysphagia in individuals with ALS,1,12 modification
of patient treatment in the dental chair is often necessary. First,
the dental practitioner may need to modify patient positioning.
Since pooling of saliva can occur in the oral cavity, the patient may
be more comfortable in a semi-supine position to help control
aspiration of excess saliva. In addition, the dentist and dental
hygienist may need another practitioner present to assist him/
her with high volume evacuation to help keep saliva from pooling
and interfering with dental work, and to make the patient more
comfortable. Third, shorter appointments, or appointments with
built in breaks, may be necessary for the patient with ALS. Since
individuals with ALS experience muscle weakness and spasticity,1
it can be difficult for them to keep their mouths open for the time
necessary to complete treatment during a regular appointment.
Treatment may need to be broken down into smaller divisions to
allow for shorter appointment times. Frequent breaks may also
need to occur during the appointment. In addition, a bite block
may be necessary to hold the mouth open for the duration of the
dental procedure.
Many ALS patients have difficulty swallowing,9 which can
affect an individual’s intake of food. As a result, nutritional counseling by the dental practitioner may be warranted. Oral health
education regarding nutrition and its effects on the oral cavity is
important in emphasizing how food intake can affect the health of
an individual’s intraoral tissues. The health of the intraoral tissues
can also influence the individual’s food choices and nutrient intake. This important cycle should be addressed with ALS patients
so that the dental professional and patient can work together to
devise a nutritional plan that will complement the patient’s current
physiological needs.
Lastly, oral health education may need to address modification
of home care due to loss of muscular function.1 These alterations
will include modification of the daily homecare regimen, such as
modified toothbrushes or brushing techniques. In addition, the
introduction of auxiliary aides may enable the patient to enhance
biofilm removal. Individuals who have lost substantial motor function or who experience severe spasticity may need to bring a care
provider, such as a family member, to their dental appointment to
assist in the nutritional and homecare planning.
Conclusion
Amyotrophic lateral sclerosis, a degenerative neural disorder,
ultimately affects multiple body systems. Its poor prognosis and
rapid progression lead to quick, visible symptoms.5 The initial
symptoms vary according to the type of neural involvement in the
individual. All patients afflicted with ALS show signs of dysphagia and other oral complications. The dental implications of ALS
span both home and professional clinical care. As such, it is important for the dental practitioner to have an understanding of the
disease and the modifications needed to treat patients with ALS.
@dentalmagazine | www.DENTALECONOMICS.com | 06.2014
85
References
1.
Easterling C, Antinoja J, Cashin S, Barkhaus PE. Changes in tongue pressure, pulmonary
function, and salivary flow in patients with amyotrophc lateral sclerosis. Dysphagia 2013;
28(2): 217-225.
2. Paris G, Martinaud O, Petit A, Cuvelier A, Hannequin D. Oropharyngeal dysphagia in
amyotrophic lateral sclerosis alters quality of life. J Oral Rehabil 2013; 40(3): 199-204.
3. Gordon P. Amyotrophic lateral sclerosis: pathophysiology, diagnosis, and management.
CNS Drugs 2011; 25(1): 1-15.
4. Escuro AA. Nutrition support in amyotrophic lateral sclerosis: from diagnosis through
terminal phase. Support Line 2012; 34(2): 8-19.
5. Fallon L. Amyotrophic lateral sclerosis. Gale Encyclo Neurol Disor 2005. Available at:
http://www.encyclopedia.com/topic/amyotrophic_lateral_sclerosis.aspx
6. Davis M, Lou JS. Management of amyotrophic lateral sclerosis (ALS) by the family nurse
practitioner: A timeline for anticipated referrals. J Am Acad Nurse Pract 2011; 23(9); 464472.
7. Corcia P, Meininger V. Management of amyotrophic lateral sclerosis. Drugs 2008; 68(8);
1037-1048.
8. Gardner DD. Amyotrophic lateral sclerosis in the older adult. AARC Times. November
2011; 35(11): 22-25.
9. Britton D., Cleary S., Miller R. What is ALS and what is the philosophy of care? Perspect
Swallow Swallow Disorders 2013; 22: 4-11.
10. Valente SM. Life with Lou Gehrig’s disease: managing ALS symptoms. The Nurs Pract
2007; 32(12); 26-34.
11. Blackhall LJ. Amyotrophic lateral sclerosis and palliative care: where we are, and the road
ahead. Muscle Nerve 2012; 45(3): 311-318.
12. Fattori B, Grosso M, Bongioanni P, et al. Assessment of swallowing by
oropharyngoesophageal scintigraphy in patients with amyotrophic lateral sclerosis.
Dysphagia 2006; 21(4): 280-286.
Author Profile
Kimberly M. Parsons, MEd, CDA, EFDA, RDH, is the dental assisting program chair and an assistant professor of dental assisting/dental
hygiene at the University of Southern Indiana. Her scholarly activities
include research in the areas of educational technologies and dental education. Ms. Parsons has been a dental hygienist for 13 years, practicing
in Arizona, Indiana, Kentucky, and Michigan. She can be reached at
[email protected].
Amy J. Schneider, LDH, CDA, BS is an instructor of dental assisting/
dental hygiene at the University of Southern Indiana. She has been practicing dental hygiene for 18 years in the state of Indiana. Ms. Schneider
has personal experience with individuals with ALS.
Author Disclosure
Kimberly M. Parsons and Amy J. Schneider have no commercial ties with
the sponsors or providers of the unrestricted educational grant for this course.
Online Completion
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Questions
1.What is the name for the
progressive degenerative disorder
that affects the nervous system,
particularly the motor neurons?
a. Amyotrophic latent sclerosis
b. Amyotrophic lateral spasticity
c. Amyotrophic lateral sclerosis
d. Alveolar lateral sclerosis
2.What form of ALS comprises the
majority of ALS cases?
a. Juvenile onset
b.Sporadic
c.Familial
d. Geriatric onset
3.Which of the following groups is
most often inflicted with sporadic
ALS?
a.Men
b.Women
c.Children
d. Men and women are equally inflicted
4.Death in patients with ALS occurs
primarily from which of the following complications?
86
a.Cardiac
b.Skeletal
c.Nervous
d.Respiratory
5.What determines the type of palliative
care provided to individuals with ALS?
a. Length of time afflicted with ALS
b. Individual’s current financial situation
c. Individual’s ongoing symptoms
d. Individual’s prognosis
6.Which of the following oral conditions may arise as a result of medications used for ALS patients?
a. Thickened mucus
b.Xerostomia
c.Sialorrhea
d. All of the above
7.Management of sialorrhea may
be achieved through which of the
following?
a. Use of an anticholinergic
b. Chewing medicinal gum
c. Use of a room humidifier
d. Adequate hydration
06. 2014 | www.DENTALECONOMICS.com | @dentalmagazine
8.A medication currently approved to
slow the course of ALS is:
a.Metoprolol
b.Warfarin
c.Riluzole
d. There is no approved medication
9.Individuals with degeneration of the
spinal neurons initially exhibit:
a. Difficulty maintaining control of the head
and neck
b. Weakness or spasticity of the lower limb
muscles
c. Problems with speech
d. Tingling or numbness of the hands
10. Individuals with degeneration of
the bulbar neurons initially exhibit:
a. Difficulty maintaining control of the head
and neck
b. Weakness or spasticity of the lower limb
muscles
c. Problems with speech
d. Tingling or numbness of the hands
ANSWER SHEET
Clinical Considerations for Treating the Dental Patient with ALS
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Educational Objectives
Academy of Dental Therapeutics and Stomatology,
1. Describeamyotrophiclateralsclerosis(ALS)andthemanner(s)inwhichitmaypresent
P.O. Box 116, Chesterland, OH 44026
or fax to: (440) 845-3447
2. Recognize common types of palliative care offered to individuals with ALS
A Division of PennWell Corp.
3. Describe the symptoms associated with spinal and bulbar degeneration
4. Integrate appropriate modifications into the dental treatment of patients with ALS
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