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HUNTINGTON’S DISEASE

Huntington's disease is an inherited disease that causes the progressive


breakdown (degeneration) of nerve cells in the brain. Huntington's disease has a
broad impact on a person's functional abilities and usually results in movement,
thinking (cognitive) and psychiatric disorders.

Most people with Huntington's disease develop signs and symptoms in their 30s or
40s. But the disease may emerge earlier or later in life.

When the disease develops before age 20, the condition is called juvenile
Huntington's disease. An earlier emergence of the disease often results in a
somewhat different set of symptoms and faster disease progression.

Medications are available to help manage the symptoms of Huntington's disease,


but treatments can't prevent the physical, mental and behavioral decline
associated with the condition.

SYMPTOMS
Huntington's disease usually causes movement, cognitive and psychiatric
disorders with a wide spectrum of signs and symptoms. Which symptoms appear
first varies greatly among affected people. During the course of the disease,
some disorders appear to be more dominant or have a greater effect on
functional ability.
Movement disorders

The movement disorders associated with Huntington's disease can include both
involuntary movement problems and impairments in voluntary movements, such
as:

 Involuntary jerking or writhing movements (chorea)

 Muscle problems, such as rigidity or muscle contracture (dystonia)

 Slow or abnormal eye movements

 Impaired gait, posture and balance

 Difficulty with the physical production of speech or swallowing


Impairments in voluntary movements — rather than the involuntary movements
— may have a greater impact on a person's ability to work, perform daily
activities, communicate and remain independent.

Cognitive disorders

Cognitive impairments often associated with Huntington's disease include:

 Difficulty organizing, prioritizing or focusing on tasks

 Lack of flexibility or the tendency to get stuck on a thought, behavior or


action (perseveration)

 Lack of impulse control that can result in outbursts, acting without thinking
and sexual promiscuity

 Lack of awareness of one's own behaviors and abilities

 Slowness in processing thoughts or ''finding'' words

 Difficulty in learning new information


Psychiatric disorders

The most common psychiatric disorder associated with Huntington's disease is


depression. This isn't simply a reaction to receiving a diagnosis of Huntington's
disease. Instead, depression appears to occur because of injury to the brain
and subsequent changes in brain function. Signs and symptoms may include:

 Feelings of irritability, sadness or apathy

 Social withdrawal

 Insomnia

 Fatigue and loss of energy

 Frequent thoughts of death, dying or suicide


Other common psychiatric disorders include:

 Obsessive-compulsive disorder — a condition marked by recurrent,


intrusive thoughts and repetitive behaviors

 Mania, which can cause elevated mood, over activity, impulsive behavior
and inflated self-esteem

 Bipolar disorder — a condition with alternating episodes of depression and


mania
In addition to the above symptoms, weight loss is common in people with
Huntington's disease, especially as the disease progresses.

Symptoms of juvenile Huntington's disease

The start and progression of Huntington's disease in younger people may be


slightly different from that in adults. Problems that often present themselves early
in the course of the disease include:

Behavioral changes

 Loss of previously learned academic or physical skills

 Rapid, significant drop in overall school performance

 Behavioral problems
Physical changes

 Contracted and rigid muscles that affect gait (especially in young children)
 Changes in fine motor skills that might be noticeable in skills such as
handwriting

 Tremors or slight involuntary movements

 Seizures

CAUSES
Huntington's disease is caused by an inherited defect in a single gene.
Huntington's disease is an autosomal dominant disorder, which means that a
person needs only one copy of the defective gene to develop the disorder.

With the exception of genes on the sex chromosomes, a person inherits two
copies of every gene — one copy from each parent. A parent with a defective
gene could pass along the defective copy of the gene or the healthy copy.
Each child in the family, therefore, has a 50 percent chance of inheriting the
gene that causes the genetic disorder.

Autosomal dominant inheritance pattern


COMPLICATIONS

After the start of Huntington's disease, a person's functional abilities gradually


worsen over time. The rate of disease progression and duration varies. The time
from disease emergence to death is often about 10 to 30 years. Juvenile
Huntington's disease usually results in death within 10 years after symptoms
develop.

The clinical depression associated with Huntington's disease may increase the
risk of suicide. Some research suggests that the greater risk of suicide occurs
before a diagnosis is made and in the middle stages of the disease when a
person has begun to lose independence.

Eventually, a person with Huntington's disease requires help with all activities of
daily living and care. Late in the disease, he or she will likely be confined to a
bed and unable to speak. However, he or she is generally able to understand
language and has an awareness of family and friends.

Common causes of death include:

 Pneumonia or other infections

 Injuries related to falls

 Complications related to the inability to swallow

DIAGNOSIS

A preliminary diagnosis of Huntington's disease is based primarily on your


answers to questions, a general physical exam, a review of your family medical
history, and neurological and psychiatric examinations.

Neurological examination

The neurologist will ask you questions and conduct relatively simple tests in the
office to judge:
Motor symptoms

 Reflexes

 Muscle strength

 Muscle tone

 Coordination

 Balance
Sensory symptoms

 Sense of touch

 Vision and eye movement

 Hearing
Psychiatric symptoms

 Mental status

 Mood
Neuropsychological testing

The neurologist may also perform standardized tests to assess:

 Memory

 Reasoning

 Mental agility

 Language function

 Spatial reasoning
Psychiatric evaluation

You'll likely be referred to a psychiatrist for an examination to judge a number of


factors that could contribute to your diagnosis, including:

 Emotional state

 Patterns of behaviors
 Quality of judgment

 Coping skills

 Signs of disordered thinking

 Evidence of substance abuse


Brain imaging and function

Your doctor may order brain-imaging tests for assessing the structure or function
of the brain. The imaging technologies may include magnetic resonance
imaging (MRI) or computerised tomography (CT) scans that provide detailed
images of brain structures.

These images may reveal structural changes at particular sites in the brain
affected by Huntington's disease, although these changes may not be
apparent early in the course of the disease. These tests can also be used to rule
out other conditions that may be causing symptoms.

Genetic counseling and testing

If symptoms strongly suggest a diagnosis of Huntington's disease, your doctor


may recommend a genetic test for the defective gene.

This test can confirm the diagnosis, and it may be valuable if there's no known
family history of Huntington's disease or if no other family member's diagnosis
was confirmed with a genetic test. But the test won't provide information that
might help determinine a treatment plan.

Before undergoing such a test, the genetic counselor will explain the benefits
and drawbacks of learning test results. The genetic counselor can also answer
questions about the inheritance patterns of Huntington's disease.

Predictive genetic test

A genetic test can be given to someone who has a family history of the disease
but shows no signs or symptoms. This is called predictive testing. The test result
has no treatment benefit, and it doesn't indicate when disease onset will begin
or what symptoms are likely to appear first.

Some people may elect to do the test because they find it more stressful not
knowing. Others may want to take the test before they make decisions about
having children.

Risks may include problems with insurability or future employment and the
stresses of facing a fatal disease. In principle, federal laws exist that make it
illegal to use genetic testing information to discriminate against people with
genetic diseases.

These tests are only performed after consultation with a genetic counselor.

MEDICAL MANAGEMENT

No treatments can alter the course of Huntington's disease. But medications can
lessen some symptoms of movement and psychiatric disorders. And multiple
interventions can help a person adapt to changes in his or her abilities for a
certain amount of time.

Medication management is likely to evolve over the course of the disease,


depending on the overall treatment goals. Also, drugs to treat some symptoms
may result in side effects that worsen other symptoms. Therefore, the treatment
goals and plan will be regularly reviewed and updated.
Medications for movement disorders
Drugs to treat movement disorders include the following:

 Tetrabenazine (Xenazine) is specifically approved by the Food and Drug


Administration to suppress the involuntary jerking and writhing movements
(chorea) associated with Huntington's disease. A serious side effect is the
risk of worsening or triggering depression or other psychiatric conditions.
Other possible side effects include drowsiness, nausea and restlessness.
 Antipsychotic drugs, such as haloperidol (Haldol) and chlorpromazine,
have a side effect of suppressing movements. Therefore, they may be
beneficial in treating chorea. However, these drugs may worsen
involuntary contractions (dystonia) and muscle rigidity.

Other drugs, such as risperidone (Risperdal) and quetiapine (Seroquel), may


have fewer side effects but still should be used with caution, as they may also
worsen symptoms.

 Other medications that may help suppress chorea include amantadine,


levetiracetam (Keppra, others) and clonazepam (Klonopin). At high
doses, amantadine can worsen the cognitive effects of Huntington's
disease. It may also cause leg swelling and skin discoloration.

Side effects of levetiracetam include nausea, stomach upset and mood swings.
Clonazepam may worsen the cognitive side effects of Huntington's disease and
cause drowsiness. It also has a high risk of dependence and abuse.
Medications for psychiatric disorders

Medications to treat psychiatric disorders will vary depending on the disorders


and symptoms. Possible treatments include the following:

 Antidepressants include such drugs as citalopram (Celexa), escitalopram


(Lexapro), fluoxetine (Prozac, Sarafem) and sertraline (Zoloft). These drugs
may also have some effect on treating obsessive-compulsive disorder.
Side effects may include nausea, diarrhea, drowsiness and low blood
pressure.

 Antipsychotic drugs — such as quetiapine (Seroquel), risperidone


(Risperdal) and olanzapine (Zyprexa) — may suppress violent outbursts,
agitation, and other symptoms of mood disorders or psychosis. However,
these drugs may cause different movement disorders themselves.
 Mood-stabilizing drugs that can help prevent the highs and lows
associated with bipolar disorder include anticonvulsants, such as
valproate (Depacon), carbamazepine (Carbatrol, Epitol, Tegretol) and
lamotrigine (Lamictal).
Psychotherapy

A psychotherapist — a psychiatrist, psychologist or clinical social worker — can


provide talk therapy to help a person manage behavioral problems, develop
coping strategies, manage expectations during progression of the disease and
facilitate effective communication among family members.
Speech therapy

Huntington's disease can significantly impair control of muscles of the mouth


and throat that are essential for speech, eating and swallowing. A speech
therapist can help improve your ability to speak clearly or teach you to use
communication devices — such as a board covered with pictures of everyday
items and activities. Speech therapists can also address difficulties with muscles
used in eating and swallowing.
Physical therapy

A physical therapist can teach you appropriate and safe exercises that
enhance strength, flexibility, balance and coordination. These exercises can
help maintain mobility as long as possible and may reduce the risk of falls.

Instruction on appropriate posture and the use of supports to improve posture


may help lessen the severity of some movement problems.

When the use of a walker or wheelchair is required, the physical therapist can
provide instruction on appropriate use of the device and posture. Also, exercise
regimens can be adapted to suit the new level of mobility.
Occupational therapy

An occupational therapist can assist the person with Huntington's disease, family
members and caregivers on the use of assistive devices that improve functional
abilities. These strategies may include:
 Handrails at home
 Assistive devices for activities such as bathing and dressing

 Eating and drinking utensils adapted for people with limited fine motor
skills

NURSING INTERVENTION
1. Provide emotional support for client and family.

2. Allow client and family to express feelings about progressive deterioration


and ultimate death.
3. Encourage family members to seek genetic counseling.
4. Modify environment to increase safety.
5. Assess ability to swallow.
6. Provide nutritional support as needed.
7. Encourage client to remain as active as possible.

8. Provide respiratory support based on changing needs of client such as


airway, suctioning, oxygenation).
9. Utilize community agencies to provide emotional support.

AMYOTROPHIC LATERAL SCLEROSIS/LOU GEHRG’S DISEASE

Amyotrophic lateral sclerosis), or ALS, is a progressive nervous system


(neurological) disease that destroys nerve cells and causes disability.

ALS is often called Lou Gehrig's disease, after the famous baseball player who
was diagnosed with it. ALS is a type of motor neuron disease in which nerve cells
gradually break down and die.

Doctors usually don't know why ALS occurs. Some cases are inherited.
ALS often begins with muscle twitching and weakness in a limb, or slurred
speech. Eventually, ALS affects control of the muscles needed to move, speak,
eat and breathe. There is no cure for ALS, and eventually the disease is fatal.

SYMPTOMS
Early signs and symptoms of ALS include:

 Difficulty walking or doing your normal daily activities


 Tripping and falling
 Weakness in your leg, feet or ankles
 Hand weakness or clumsiness
 Slurred speech or trouble swallowing
 Muscle cramps and twitching in your arms, shoulders and tongue
 Difficulty holding your head up or keeping good posture

ALS often starts in the hands, feet or limbs, and then spreads to other parts of
your body. As the disease advances and nerve cells are destroyed, your
muscles progressively weaken. This eventually affects chewing, swallowing,
speaking and breathing.

ALS doesn't usually affect your bowel or bladder control, your senses or your
thinking ability. It's possible to remain actively involved with your family and
friends.

CAUSES
ALS is inherited in 5 to 10 percent of cases, while the rest have no known cause.

Researchers are studying several possible causes of ALS, including:

 Gene mutation. Various genetic mutations can lead to inherited ALS,


which causes nearly the same symptoms as the noninherited form.
 Chemical imbalance. People with ALS generally have higher than normal
levels of glutamate, a chemical messenger in the brain, around the nerve
cells in their spinal fluid. Too much glutamate is known to be toxic to some
nerve cells.
 Disorganized immune response. Sometimes a person's immune system
begins attacking some of his or her body's own normal cells, which may
lead to the death of nerve cells.
 Protein mishandling. Mishandled proteins within the nerve cells may lead
to a gradual accumulation of abnormal forms of these proteins in the
cells, destroying the nerve cells.

RISK FACTORS

Established risk factors for ALS include:

 Heredity. Five to 10 percent of the people with ALS inherited it (familial


ALS). In most people with familial ALS, their children have a 50-50 chance
of developing the disease.
 Age. ALS risk increases with age, and is most common between the ages
of 40 and 60.
 Sex. Before the age of 65, slightly more men than women develop ALS.
This sex difference disappears after age 70.
 Genetics. Some studies examining the entire human genome
(genomewide association studies) found many similarities in the genetic
variations of people with familial ALS and some people with noninherited
ALS. These genetic variations might make people more susceptible to ALS.

Environmental factors may trigger ALS. Some that may affect ALS risk include:

 Smoking. Smoking is the only likely environmental risk factor for ALS. The risk
seems to be greatest for women, particularly after menopause.
 Environmental toxin exposure. Some evidence suggests that exposure to
lead or other substances in the workplace or at home may be linked to
ALS. Much study has been done, but no single agent or chemical has
been consistently associated with ALS.
 Military service. Recent studies indicate that people who have served in
the military are at higher risk of ALS. It's unclear exactly what about military
service may trigger the development of ALS. It may include exposure to
certain metals or chemicals, traumatic injuries, viral infections, and intense
exertion.
COMPLICATIONS
As the disease progresses, people with ALS experience complications, which
may include:

Breathing problems

Over time, ALS paralyzes the muscles you use to breathe. You may need a
device to help you breathe at night, similar to what someone with sleep apnea
might wear. For example, you may be given continuous positive airway pressure
(CPAP) or bi-level positive airway pressure (BiPAP) to assist with your breathing at
night.

Some people with advanced ALS choose to have a tracheostomy — a


surgically created hole at the front of the neck leading to the windpipe
(trachea) — for full-time use of a respirator that inflates and deflates their lungs.

The most common cause of death for people with ALS is respiratory failure. On
average, death occurs within three to five years after symptoms begin.

Speaking problems

Most people with ALS will develop trouble speaking over time. This usually starts
as occasional, mild slurring of words, but progresses to become more severe.
Speech eventually becomes more difficult for others to understand, and people
with ALS often rely on other communication technologies to communicate.

Eating problems

People with ALS can develop malnutrition and dehydration from damage to the
muscles that control swallowing. They are also at higher risk of getting food,
liquids or saliva into the lungs, which can cause pneumonia. A feeding tube can
reduce these risks and ensure proper hydration and nutrition.

Dementia

Some people with ALS experience problems with memory and making
decisions, and some are eventually diagnosed with a form of dementia called
frontotemporal dementia
MEDICAL MANAGEMENT
Treatments can't reverse the damage of amyotrophic lateral sclerosis, but they
can slow the progression of symptoms, prevent complications and make you
more comfortable and independent.

You may need an integrated team of doctors trained in many areas and other
health care professionals to provide your care. This may prolong your survival
and improve your quality of life.

Your team will help you select the right treatments for you. You always have the
right to choose or refuse any of the treatments suggested.

Medications

Two medications are currently approved by the Food and Drug Administration
for the treatment of ALS.

 Riluzole (Rilutek) — This drug appears to slow the disease's progression in


some people, perhaps by reducing levels of a chemical messenger in the
brain (glutamate) that's often present in higher levels in people with ALS.
Riluzole is taken as a pill and may cause side effects such as dizziness,
gastrointestinal conditions and liver function changes.
 Edaravone (Radicava) — The FDA approved edaravone in 2017 based on
six-month clinical trial that showed it reduced the decline in daily
functioning associated with ALS. The drug is given via intravenous infusion
(typically 10-14 days in a row, once a month), and side effects may
include bruising, gait disturbance, hives, swelling and shortness of breath.

Edaravone contains sodium bisulfite, which may cause serious allergic reactions
in people with sulfite sensitivity.

Your doctor may also prescribe medications to provide relief from other
symptoms, including:

 Muscle cramps and spasms


 Spasticity
 Constipation
 Fatigue
 Excessive salivation
 Excessive phlegm
 Pain
 Depression
 Sleep problems
 Uncontrolled outbursts of laughing or crying

NURSING MANAGEMENT
PHYSICAL

I. Muscle Weakness/Skin Status

1. Assessment
o Assess motor strength; presence of spasticity of flaccidity.
o Assess for presence of contracture.
o Assess skin daily, especially those areas susceptible to breakdown.
2. Common Nursing Diagnoses
o Potential for injury related to impaired physical mobility.
o Knowledge deficit regarding prevention of injury while promoting mobility and
self-care.
o Alteration in comfort.
3. Management
o Promotion of activity and exercise.
 Encourage continuation of daily routines and activities.
 Maintain exercise program to tone and strengthen unaffected muscle groups
without fatiguing affected muscles.
 Range-of-Motion (ROM) exercises to prevent contracture and pain in joints; first
Active ROM, then passive. Refer to physical therapist for exercise program and
see recommended passive range of motion description in Managing ALS (MALS)
Manual II.
 When weakness in the extremities begins to compromise mobility, safety, or
independence in ADL, refer to a physical or occupational therapist.
o Promotion of proper positioning to prevent decubitus ulcers
 Use as many different positions as possible when in bed. Change positions every
two hours, or on skin tolerance. After each change of position, check for redness
over bony
 prominences, and provide an eggshell or circulating mattress when immobility
prevents independent repositioning.
 Repositioning in the wheelchair based on the patient’s skin tolerance. Use of a
wheelchair cushion to prevent skin breakdown.
 Proper positioning when ambulating or in a wheelchair, i.e., use of a sling for a
weak upper extremity.
 Promote adequate nutritional intake.
II. Urinary Function

1. Assessment
o Assess urination pattern and patterns of fluid intake.
o Assess ability to transfer to toilet or commode, or standing ability for a male.
o Assess for signs and symptoms of urinary tract infection; frequency, urgency,
painful urination, fever, etc. (urinary tract infections are rare in ALS).
2. Common Nursing Diagnoses
o 1. Impaired urinary elimination related to:
 Progressive loss of mobility
 Dehydration
1. Management
o Encourage daily fluid intake of 2,500cc’s per day unless contraindicated
because of swallowing ability.
o Consult physician if there are signs of urinary tract infection.
III. Altered Bowel Function

1. Assessment
o Assess bowel pattern (for constipation, diarrhea, impaction.
o Assess diet, fluid intake, and swallowing ability.
o Assess activity level.
2. Common Nursing Diagnoses
o Impaired bowel elimination.
o Progressive loss of mobility.
o Progressive decline of dietary fiber.
o Often mile/moderate dehydration.
o Progressive inability of the trunk muscles to support a normal posture to assist in
defecation.
3. C. Management – ideally focused on preventative measures. Unlike a neurogenic
bowel, person with ALS have slowed bowel motility.
1. Encourage patient to keep daily record of bowel movements (include
consistency and amount), particularly if there has been change to a more
constipated or less frequent stool.
2. Encourage daily fluid intake of 2,500cc’s per day unless contraindicated
because of swallowing ability.
3. Provide dietary instruction regarding the importance of fiber, use of bran
cereals, prune juices, etc., unless contraindicated because of swallowing ability.
4. Daily use of stool softeners and Metamucil are often recommended. Laxatives
may be used, but chronic use may be harsh on the bowel. Mineral oil should not
be used because of the danger of aspiration.
5. As mobility progresses and/or diet and fluid intake is altered, a more aggressive
bowel program may be indicated, daily or every other day.

NUTRITION

I. Nutritional Needs

A. Assessment
1. Anthropometric Measures:
a) Height
b) Pre-morbid “usual” weight
c) Current Weight
d) Weight gain/loss pattern since onset of disease
e) “Ideal body weight”
2. Laboratory Test – indicated if patient has lost ten percent or more of body
weight in the last two months or weighs fifteen percent or more below “ideal
body weight”.

3. Hydration status – determined by careful recording of fluid intake and output


and by test of urine specific gravity. The average person needs 35cc fluid/kg
body weight for adequate renal function. This figure can be used to calculate
fluid requirements.

B. Dysphagia (difficulty in swallowing)


1. Assessment
a) Assess gag, cough and swallowing reflexes, and chewing.
b) Assess patient’s ability to swallow liquids and solids.
c) Assess weight serially.
2. Common Nursing Diagnoses
a) Potential for injury.
b) Impaired nutritional status.
c) Knowledge deficit regarding alternatives for diet, food preparation, and
alternative procedures for supplemental feeding.
3. Management
a) Discuss techniques for protecting airway, i.e., sitting up straight, putting chin
on chest when swallowing, concentrating while eating. Avoid increased
stimulation, i.e., talking at mealtime.
b) Refer to dietician for determination of adequate nutritional intake with
continued weight loss.
C. Nutritional Management of the Dysphagic Patient
1. Promotion of optimum weight
a) Smaller, more frequent meals.
b) Foods that are “easy-to-eat”, i.e., adjusted mechanical soft diet.
c) Increased daily activity with non-fatiguing exercise.
d) Liquid supplement between meals.

RESPIRATORY

A. Assessment
1. History: subjective symptoms (starving for air, shortness of breath and relation
to position changes, fatigue).
2. Assess: changes from baseline respiratory rate, depth, pattern, chest
expansion; adequacy of respiratory exchange and effort; retraction of
intercostals spaces; abdominal breathing; diaphragmatic breathing; nasal
flaring and use of cervical accessory muscles of respiration; color (cyanosis);
cough, gag, and swallow reflexes.
3. Auscultate: decreased breath sounds, presence of extra or adventitious
sounds (i.e., sales, wheezes).
B. Common Nursing Diagnoses
1. Ineffective airway clearances related to:
a) Impaired/absent gag reflex.
b) Impaired/absent swallowing reflex.
c) Impaired/absent cough/sneeze reflex.
2. Impaired gas exchange related to:
a) Aspiration secondary to impaired/absent gag, swallowing and cough/sneeze
reflexes.
b) Atelectasis secondary to impaired cough/sneeze reflexes and/or
hypoventilation.
c) Ineffective breathing pattern.
3. Knowledge deficit regarding:
a) Airway clearance and gas exchange.
b) Considerations regarding mechanical life supports.
C. Management
1. Maintain airway patency
a) Use of aspirator to suction secretions and prevent choking.
b) Use of medications (i.e., Elvail – 25 mg t.i.d. or 25-75 mg. at hs to reduce
secretions).
c) If the patient has a tracheostomy, suctioning and “trach care” should be
done prn to prevent buildup of secretions, possible obstruction and infection.
2. Maintain adequate ventilation
a) Deep breathing and coughing exercises and use of incentive spirometer to
encourage lung expansion.
b) Chest physiotherapy: percussion, assisted cough and postural drainage when
indicated.
c) Oxygen, when ordered.
d) Elevation of the head of the bed with a foam wedge or hospital bed to ease
shortness of breath and provide comfort during sleep.
e) When air hunger is chronic and distressing, help monitor and titrate
medications that will help provide comfort.
3. Provide information and opportunities to discuss options for mechanical
ventilation. Generally, if there are early signs of dysphagia, dyspnea, or after the
patient has had time to adjust to his diagnosis, the patient and family should be
assisted in considering options regarding life support systems. The important
thing to remember is the patient should be thinking about these decisions
before he/she needs them.

4. Provide information and opportunity to discuss Physician’s Directive (also


called Living Will or Durable Power of Attorney for Medical Care). The pros and
cons of life support should be discussed in detail prior to the patient filling out a
Physician’s Directive.

COMMUNICATION

A. Assessment
1. Assess volume and clarity of speech. 2. Assess ability to communicate needs
to family/significant others.
B. Common Nursing Diagnoses
1. Impaired verbal communications related to:
a) Altered volume of speech.
b) Altered clarity of speech (dysarthia).
c) Loss of speech.
C. Management
1. With dysarthria or with total loss of speech and no use of upper extremities.
2. There are a variety of alternative modes of communication.
3. Refer to a speech pathologist for evaluation and intervention.

PSYCHOLOGICAL ADAPTATION

A. Assessment
1. Evaluate the patient and family’s support systems and coping patterns with
awareness that with ongoing loss of independence, there will be ongoing
grieving by patient and family.
B. Common Nursing Diagnoses
1. Ineffective coping strategies
2. Knowledge deficit regarding alternatives for mobility and comfort.
3. Impaired support system.
C. Management
1. Provide an accepting environment in which the patient and family can share
concerns and fears with each other and members of the health care team.
2. Help the patient and family anticipate care needs and implications.
3. Anticipate and support emotional responses to loss as a normal reaction, thus
enabling the family to take in information and cope more effectively with
practical decision-making.
4. Provide encouragement and anticipate unasked questions.
5. Encourage realistic expectations of the disease whenever possible.
6. Refer, if needed, to a counselor, psychiatrist, psychologist or psychiatric
clinical nurse specialist.
7. Refer to an ALS support group if available.
ARELLANO UNIVERSITY
College of Nursing

HUNTINGTON’S DISEASE
&
AMYOTROPHIC LATERAL
SCLEROSIS/LOU GEHRG’S
DISEASE
Submitted by:

Denise M. Espinosa
BSNIII-OS

Submitted to:

Ma’am Arlene Latorre, RN, MAN


Clinical Instructor