Als Management

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ALS MANAGEMENT ( namamanage yung sakit throught the treatment given )

Currently there is no cure for ALS, yet patients suffering from the disease can be made more
comfortable with the following options:
 medications to relieve painful muscle cramps, excessive salivation and other symptoms.
 heat or whirlpool therapy to relieve muscle cramping.
 exercise, although recommended in moderation, may help maintain muscle strength and
function. physical therapy to maintain mobility and ease the discomfort of muscle
stiffness, cramps and fluid retention.
 nutritional counseling to promote good nutrition and offer other dietary options when
swallowing becomes difficult.
 speech therapy and communication training to maintain as many verbal communication
skills as possible. Communication training also indicates non-verbal techniques.
 devices such as splints, corrective braces, grab bars, reach-extenders, etc. to help with
daily activities such as dressing, eating, using the toilet and bathing.
 special equipment such as wheelchairs, electric beds or mattresses to maximize
functional independence.
Recently, the US Food and Drug Administration (FDA) approved Rilutek®, the first drug that has
reliably prolonged the survival of persons with ALS. Patients, however, will not get stronger nor
regain lost strength with this drug.
Managing the symptoms is a process that may be challenging for people with ALS, their care
givers and the medical team, but there are many community resources available for support and
assistance.

This medication is used to treat a certain type


of nerve disease called amyotrophic lateral
sclerosis (ALS, also commonly called Lou
Gehrig's disease). Riluzole helps to slow down
the worsening of this disease and prolong
survival.
(Rilutek Oral: Uses, Side Effects, Interactions, Pictures, Warnings &
Dosing - WebMD, n.d.)

Heller, L. (n.d.). ALS Treatment, Amyotrophic Lateral Sclerosis, Lou Gehrig’s disease. ALS
Treatment, Amyotrophic Lateral Sclerosis, Lou Gehrig’s Disease. Retrieved October 8, 2022, from
https://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/als/conditions/
als_treatment.html
Andrews, & Moroney. (2008, August 6). Amyotrophic Lateral Sclerosis. Google Books. Retrieved
October 8, 2022, from https://books.google.com.ph/books?
id=rq0qiZYYYicC&printsec=frontcover&dq=how+to+manage+the+disease+als&hl=en&sa=X&redir_esc=y
#v=onepage&q=how%20to%20manage%20the%20disease%20als&f=false
The effect of available treatment for ALS is modest. Two drugs, Riluzole and Edaravone,
are approved for ALS; they have modest effect in slowing the disease progression. The
antiglutamate agent Riluzole, when given orally, was shown to slow the progression of ALS and
improve survival in patients with disease of bulbar onset; it prolonged survival by about 3
months. The antioxidant Edaravone has been shown to slow the clinical progress of ALS in
select patients in limited trials; but again, the benefit has been marginal 

At all stages of ALS, physical therapy is useful in maintaining mobility. Physical therapy
is invaluable, for example, for avoiding contractures of the fingers and shoulders. Occupational
therapy is likewise helpful, particularly assessments of the patient’s function in the home. A
range of personalized orthotic devices, often guided by the physical and occupational therapists,
may be of assistance to the patient as the disease progresses.
Important in the management of ALS is periodic monitoring of respiratory function and
nutrition. Significant practical advances have been made in multidisciplinary ALS clinics with
regard to respiratory and nutritional management in ALS. As the respiratory muscle weakness
compromises breathing, the use of bilevel positive airway pressure (BiPAP) allows patients to
sleep better and reduce daytime somnolence. With effective noninvasive respiratory support,
tracheostomy can be deferred for months or years in most cases. Ultimately, as the disease
progresses further and diaphragm fails, BiPAP becomes necessary not only at night but also
during the day. When BiPAP use approaches 20 to 24 h per day, patients and their families
must address the difficult question of tracheostomy and mechanical ventilation or hospice care.
As oropharyngeal muscles become weak and dysphagia progresses, meals need to be
modified to prevent choking, aspiration, and complications. In initial stages, fruits, vegetables
and meat should be cut into small pieces and dry foods, such as toast should be avoided. Milk
shakes and thicker consistency foods are ideal at this stage. Speech therapists at ALS clinics
are helpful in teaching patients and their caregivers methods to adapt to declining bulbar
function and minimizing aspiration. Eventually, most ALS patients will need a feeding tube to
maintain normal hydration and caloric intake.
The American Academy of Neurology has published guidelines for management that
have been of aid to patients and physicians; they emphasize the complex and multidisciplinary
needs of ALS patients

Verma, A. (2021, July 25). Clinical Manifestation and Management of Amyotrophic Lateral
Sclerosis - Amyotrophic Lateral Sclerosis - NCBI Bookshelf. Clinical Manifestation and Management of
Amyotrophic Lateral Sclerosis - Amyotrophic Lateral Sclerosis - NCBI Bookshelf. Retrieved October 8,
2022, from https://www.ncbi.nlm.nih.gov/books/NBK573427/
Who should be on an ALS care team?
People with ALS get the best care from a team of specialists from many fields of medicine. This
team works together to help you manage symptoms, avoid complications and live as best you
can with ALS. This team may include:
 Doctors.
 Speech, occupational and physical therapists.
 Pharmacists.
 Nutritionists.
 Respiratory therapists.
 Psychologists.
 Social workers.
 Home care and hospice nurses.

What are communication tips for people with ALS?


People with ALS can use these strategies to communicate effectively:
Plan ahead:
 Ask your speech-language pathologist which exercises are right for you.
 Set aside a rest period before a conversation or phone call. Being tired affects your
speaking ability.
Choose an environment that is:
 Quiet, since it can be tiring to try and talk over TV or other loud noises.
 Well-lit to enhance face-to-face communication.
When you’re talking:
 Choose a comfortable posture and position, especially if you’re having a long or stressful
conversation.
 Establish the topic before speaking.
 Face your listener. Look at them while you talk.
 Over articulate your speech. Prolong the vowels and exaggerate the consonants to be
as clear as possible.
 Speak slowly.
 Use short phrases. Try saying one or two words or syllables per breath.
 Use telegraphic speech, leaving out unnecessary words.
Consider using devices:
 An amplifier helps if your voice is low or soft.
 An electrolarynx or respiratory tube provides an alternative air source if you want to
communicate while you’re on a respirator.

If people are having trouble understanding you, try to:


 Carry pen and paper with you as a backup, so you can write down what you want to say.
 Use an alphabet board if writing is difficult. Point to the first letter of words you want to
say.
 Spell words out loud or on the alphabet board if people don’t understand you

What exercises can help people with ALS?


Staying active can help with muscle strength and overall well-being. There are several
approaches you can take:
 Aerobic exercises increase your heart and breathing rates and improve heart health.
They also help your muscles work better and increase endurance. Exercise in
moderation. If you get fatigued, you may make your muscles weaker.
 Stretching exercises can relieve muscle cramping. Do them every day to prevent pain
and stiffness.
 Range of motion exercises (ROM) can help your joints move fully. Do these every day
to prevent pain and stiffness.
Avoid strengthening exercises, such as exercising with weights. They won’t strengthen
weak muscles, and they could even make your muscles weaker.

C. (n.d.). Amyotrophic Lateral Sclerosis: What Is It, Symptoms & Management. Cleveland Clinic.
Retrieved October 14, 2022, from https://my.clevelandclinic.org/health/diseases/16729-amyotrophic-
lateral-sclerosis-als
Symptom Management
Many patients fear the loss of control. It is important for the caregiving team to reassure
the patient that symptoms can be managed through medication and nonmedication therapies.

Nutrition and Difficulty Swallowing: As ALS progresses, patients experience greater


difficulty in swallowing. A few tips to help with swallowing include changing food consistency,
tilting the chin towards the chest when swallowing, and swallowing a few times per bite. As
swallowing becomes increasingly difficult, the concern shifts to the patient’s nutritional status.
Good nutritional status will help the patient maintain a higher quality of life. A percutaneous
endoscopic gastrostomy (PEG) tube may be offered to patients who are experiencing weight
loss. (It involves placing a tube into the stomach through the abdominal wall.)

Muscle Cramps: Patients may experience muscle cramps ranging in severity from mild
to severe. Adequate hydration and stretching exercises are the best preventive measures for
cramps. If cramps are severe, quinine sulfate, baclofen, dantrolene, gabapentin,
benzodiazepines, or carbamazepine may be prescribed. Vitamin E 400 IU twice daily and
magnesium supplement 5 mmol 3 times daily may also be recommended.

Respiratory Management: Respiratory depression is an indicator of disease progression


and should be handled aggressively. The patient and caregiver need to discuss respiratory
management with the physician every 3 months since the patient’s desire for life-sustaining
treatment may change over time. Treatment options range from noninvasive positive pressure
ventilation (NIPPV) for symptomatic relief to tracheostomy for long-term survival. 9,17,18 If the
patient is experiencing an increase in shortness of breath due to a decline in lung function,
breaks throughout the day or NIPPV may be beneficial.

Sialorrhea and Secretions: Sialorrhea (excessive salivation or drooling) due to a


decrease in facial muscle tone and involuntary swallowing of saliva are common. This can be
very frustrating and embarrassing. If drooling becomes bothersome, a trial of amitriptyline,
hyoscyamine, atropine, diphenhydramine, glycopyrrolate, or scopolamine may be tried.9,17,18 If
these fail, botulinum toxin injected directly into the salivary gland may be useful. 19 If the saliva is
thick and phlegmlike, an increase in fluid intake, humidified air, or beta-blocker therapy
(propranolol or metoprolol) may be beneficial.

Pseudobulbar Affect: At times, patients may find that they cannot control their emotions
and may start laughing or crying uncontrollably at inappropriate times. Treatment includes
amitriptyline and fluvoxamine (a selective serotonin reuptake inhibitor [SSRI]). 9,17,18 A new
combination agent consisting of dextromethorphan and quinidine has shown promise in a phase
II/III clinical trial for decreasing pseudobulbar symptoms.
Pain: Patients may experience pain from joint immobilization, skin breakdown, and
muscle spasms. Frequent changing of position can help decrease skin breakdown and improve
joint immobilization. If pain still occurs, nonsteroidal anti-inflammatory drugs (NSAIDs) should be
the first option. Opioids should be reserved for pain that is nonresponsive to NSAID therapy.
There are no specific recommendations for the choice of NSAID or opioid therapy.

Fatigue: Fatigue may be due to general muscle wasting or a decrease in respiratory


function. Additionally, as nerves die, the remaining nerves must take their place, increasing the
burden placed on these remaining nerves. Taking life easy, with naps and breaks throughout
the day, can be an effective treatment.9 Methylphenidate, pyridostigmine, amantadine, and
pemoline may provide short-term relief.9 A recent study of modafinil showed promising results.
Patients experienced a decrease in daytime sleepiness and fatigue and an increase in attention,
alertness, and focus.

Constipation: Constipation can significantly decrease a patient’s quality of life.


Constipation can be caused by a decrease in fluid intake, decreased physical activity, diet, and
numerous medications. Increases in fluid and fiber intake, along with an increase in activity, if
appropriate, are the first steps in prevention. For acute constipation, nonprescription laxatives,
suppositories, and Fleet enemas may be helpful. No specific product recommendations have
been made for the treatment of acute constipation. Stool softeners and osmotic laxatives can be
effective for long-term therapy

Depression: Depression, which affects up to 44% of patients, can significantly impact a


patient’s quality of life and decision making toward life-sustaining treatment in the terminal
phase of the disease.22 There are currently no clinical trials assessing the effectiveness of
different antidepressants, but treatment in clinical practice is fairly consistent. By taking
advantage of the common side effects of drowsiness and dry mouth, amitriptyline should be
first-line therapy in patients who are experiencing insomnia and/or sialorrhea. If treatment fails
or if the patient does not need to take advantage of amitriptyline’s side effects, SSRIs such as
sertraline, fluoxetine, and paroxetine may be used. Psychotherapy is an essential part of any
treatment for depression and can help improve a patient’s quality of life.

Anxiety: Anxiety can affect up to 30% of patients with ALS. While there are no trials
assessing the effectiveness of different treatments, clinical practice includes the use of
benzodiazepines such as lorazepam, diazepam or alprazolam, and buspirone.

Dysarthria: Almost all patients will experience dysarthria (speech difficulties) at some


point during the course of the disease. Since there are no medications available for this, speech
therapists may help maintain verbal communication. Slow speaking and overexaggeration of
words may be helpful. Writing boards and electronic communication devices may be useful tools
if verbal communication becomes too difficult.
DeSimone II, & Schlegel. (2009, August 20). The Management of Amyotrophic Lateral Sclerosis.
The Management of Amyotrophic Lateral Sclerosis. Retrieved October 14, 2022, from
https://www.uspharmacist.com/article/the-management-of-amyotrophic-lateral-sclerosis

C. (n.d.). Amyotrophic Lateral Sclerosis: What Is It, Symptoms & Management. Cleveland Clinic.
Retrieved October 14, 2022, from https://my.clevelandclinic.org/health/diseases/16729-amyotrophic-
lateral-sclerosis-als

Verma, A. (2021, July 25). Clinical Manifestation and Management of Amyotrophic Lateral
Sclerosis - Amyotrophic Lateral Sclerosis - NCBI Bookshelf. Clinical Manifestation and Management of
Amyotrophic Lateral Sclerosis - Amyotrophic Lateral Sclerosis - NCBI Bookshelf. Retrieved October 8,
2022, from https://www.ncbi.nlm.nih.gov/books/NBK573427/

Andrews, & Moroney. (2008, August 6). Amyotrophic Lateral Sclerosis. Google Books. Retrieved
October 8, 2022, from https://books.google.com.ph/books?
id=rq0qiZYYYicC&printsec=frontcover&dq=how+to+manage+the+disease+als&hl=en&sa=X&redir_esc=y
#v=onepage&q=how%20to%20manage%20the%20disease%20als&f=false

Heller, L. (n.d.). ALS Treatment, Amyotrophic Lateral Sclerosis, Lou Gehrig’s disease. ALS
Treatment, Amyotrophic Lateral Sclerosis, Lou Gehrig’s Disease. Retrieved October 8, 2022, from
https://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/als/conditions/
als_treatment.html

Rilutek Oral: Uses, Side Effects, Interactions, Pictures, Warnings & Dosing - WebMD. (n.d.).
Rilutek Oral: Uses, Side Effects, Interactions, Pictures, Warnings & Dosing - WebMD. Retrieved October
8, 2022, from https://www.webmd.com/drugs/2/drug-12146/rilutek-oral/details

 ALS Association. Understanding ALS.  (https://www.als.org/understanding-als)  Accessed


7/23/2021.

 Centers for Disease Control and Prevention. Amyotrophic Lateral Sclerosis. What Is
ALS? (https://www.cdc.gov/als/WhatisALS.html)  Accessed 7/23/2021.
 Muscular Dystrophy Association (MDA). Amyotrophic Lateral Sclerosis
(ALS). (https://www.mda.org/disease/amyotrophic-lateral-sclerosis) Accessed 7/23/2021.
 National Institute of Neurological Disorders and Stroke. Amyotrophic Lateral Sclerosis (ALS) Fact
Sheet.  (https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/
Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet) Accessed 7/23/2021.

(CDC, 2022)
Pulley, M. T., Brittain, R., Hodges, W., Frazier, C., Miller, L., Matyjasik‐Liggett, M., ... & Berger, A.
R. (2019). Multidisciplinary amyotrophic lateral sclerosis telemedicine care: the store and forward
method. Muscle & nerve, 59(1), 34-39.

Paganoni, S., & Simmons, Z. (2018). Telemedicine to innovate amyotrophic lateral sclerosis
multidisciplinary care: The time has come. Muscle & Nerve, 59(1), 3-5.

Rashed, H. R. (2021, October 4). How COVID-19 pandemic changed our management strategies
for amyotrophic lateral sclerosis (ALS) patients: Egyptian study - The Egyptian Journal of Neurology,
Psychiatry and Neurosurgery. SpringerOpen. Retrieved October 14, 2022, from
https://ejnpn.springeropen.com/articles/10.1186/s41983-021-00331-2

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