Amyotrophic Lateral Sclerosis (ALS)

Which of the following is the nurse's role in the care of clients with Amyotrophic Lateral Sclerosis (ALS)? Select all that apply: a. Act as advocate for the client and family b. Provide supportive care c. Coordinate timely referrals d. Provide client and family education e. Provide nutritional advice and make meal plans f. Provide psychosocial support to the client and family

All are correct except E a. Act as advocate for the client and family b. Provide supportive care c. Coordinate timely referrals d. Provide client and family education f. Provide psychosocial support to the client and family

Which of the following best describes dysarthria? a. It is the difficulty in controlling or coordinating the muscles used when speaking. b. It is the difficulty in chewing or swallowing foods c. It is excessive drooling or hypersalivation d. It is excessive hunger or increased appetite

a. It is the difficulty in controlling or coordinating the muscles used when speaking.

To prevent aspiration in clients with dysphagia, the nurse teaches the caregiver the following appropriate interventions, except? a. Limiting the amount of food the client eats b. Administering soft, mechanical or pureed foods c. Allowing client ample time to eat d. Elevating the head of bed during mealtime and keeping head of bed elevated for 1 ½ hours after meals.

a. Limiting the amount of food the client eats A client with dysphagia should be assisted with meals to prevent aspiration and to maintain adequate nutrition and fluid balance. The amount of food the client eats should not be limited unless the HCP

The following materials can appropriately aid in communication barriers in a client with Amyotrophic Lateral Sclerosis (ALS) who has dysarthria and is unable to use his hands, except? a. Magic slate b. Laser pointers in combination with letter board c. Headmouse d. None of the above

a. Magic slate A magic slate can not be used as an alternative mode of communication if a client is unable to use his hands because it requires gripping a pen to write on a slate.

In a client with Amyotrophic Lateral Sclerosis (ALS), difficulty in swallowing or chewing or dysphagia can happen at any point. The nurse knows that the following are appropriate actions to prevent aspiration, except? a. Assessing gag reflex before giving food or fluid by mouth b. Positioning the client in an upright position during mealtime and for ½ afterward c. Having a client sip through a straw when drinking while lying down d. None of the above

c. Having a client sip through a straw when drinking while lying down

A client newly admitted in with Amyotrophic Lateral Sclerosis with respiratory insufficiency. Which of the following should be the main goal of the charge nurse? a. Establish effective means of communication b. Prevent aspiration. c. Maintain adequate oxygenation. d. Maintain normal bowel and bladder function

c. Maintain adequate oxygenation. When bulbar symptoms occur and respiratory insufficiency is evident, maintaining adequate oxygenation should be priority and the main goal of the nurse.

When giving discharge instructions to the caregiver of a client with Amyotrophic Lateral Sclerosis (ALS), when should the drug be taken with regards to meals? a. One hour before meals b. Two hours after meals c. With meals d. A and B.

d. A and B. Medication should be taken on an empty stomach.

During assessment of a client with amyotrophic lateral sclerosis (ALS), which finding should the nurse identify when planning care for this client? A) Muscle weakness. B) Urinary frequency. C) Abnormal involuntary movements. D) A decline in cognitive function.

A) Muscle weakness. Amyotrophic lateral sclerosis (ALS) is characterized by a degeneration of motor neurons in the brainstem and spinal cord and are manifested by muscle weakness (A) and wasting. ALS does not manifest (B and C). In ALS, the client remains cognitively intact, not (D), while the physical status deteriorates.

The nurse teaches the discharge instructions to a client newly diagnosed with Amyotrophic Lateral Sclerosis (ALS). Which of the following adverse effects should the client contact his physician for? Select all that apply: a. Chest discomfort b. Lightheadedness c. Yellowing of the eyes d. Drowsiness e. Stomach pain f. Fever

A, C, D, & E a. Chest discomfort c. Yellowing of the eyes d. Drowsiness e. Stomach pain

A patient with amyotrophic lateral sclerosis (ALS) is hospitalized with pneumonia. Which nursing action will be included in the plan of care?

Assisting the patient with active range of motion (ROM)

The early manifestations of amyotrophic lateral sclerosis (ALS) and MS are somewhat similar. Which clinical feature of ALS distinguishes it from MS?

Impairment of respiratory muscles In ALS, there is progressive muscle atrophy until a flaccid quadriplegia develops. Eventually, there is involvement of the respiratory muscles, which leads to respiratory compromise.

The wife of a newly diagnosed client with Amyotrophic Lateral Sclerosis (ALS) asks the nurse if her husband can see their newborn baby graduate college, the nurse correctly responds: a. "Clients with ALS usually have a median survival rate of three to five years" b. "The life expectancy of clients with ALS ranges from three to five months" c. "There is hope because Glatiramer acetate (Copaxone) is now being used as a treatment that can improve prognosis in clients with ALS" d. "Of course he can as long as he keeps taking his maintenance medications and goes to physical therapy."

a. "Clients with ALS usually have a median survival rate of three to five years" ALS is incurable and fatal.

Which of the following statements, when made by the client newly diagnosed with Amyotrophic Lateral Sclerosis (ALS), needs further teaching? a. "If I lose strength in my upper extremities, I can regain it by having my physician increase the frequency that of my Rilutek." b. "This disease can affect the way I talk and my ability to swallow in the future." c. "This can give me at least two to five years, so it's best to enjoy my life while I can." d. None of the above

a. "If I lose strength in my upper extremities, I can regain it by having my physician increase the frequency that of my Rilutek." During treatment of ALS, the client must have a clear understanding of the expectations of drug therapy. Motor neurons that are affected by the disease process cannot be regenerated, and the client should be made aware that no drug treatment as of now can help ALS clients regain strength.

A client with Amyotrophic Lateral Sclerosis (ALS) has developed muscle flaccidity. The following are appropriate interventions to prevent developing decubitus ulcers, except? a. Providing a hard mattress to provide adequate back support b. Using of a wheelchair cushion to prevent skin breakdown c. Wash well and keep skin dry after each bowel movement or urination d. Changing of position or turning every two hours

a. Providing a hard mattress to provide adequate back support to prevent skin breakdown in ALS clients who have developed muscle weakness and flaccidity and eggshell or circulating mattress is used.

The nurse case manager talks to the wife of the client who has been discharged with early onset of Amyotrophic Lateral Sclerosis (ALS) and explores her knowledge in the care of her husband. Which of the following signs and symptoms should the wife need to notify her husband's MD for? Select all that apply? a. difficulty sleeping when lying supine b. fever c. difficulty swallowing d. urine output of 35 cc/ hour e. difficulty swallowing f. breathing at 22 breaths per minute

a. difficulty sleeping when lying supine b. fever c. difficulty swallowing e. difficulty swallowing

As the nurse makes her assessment in the 58-year old client for degenerative disorders, which are the clinical manifestations consistent with Amyotrophic Lateral Sclerosis (ALS)? Select all that apply: a. Polyphagia b. Fatigue c. Progressive muscle weakness d. Dysarthria e. Cramps f. Twitching

b. Fatigue c. Progressive muscle weakness d. Dysarthria e. Cramps f. Twitching Dysphagia is another manifestation NOT polyphagia.

The nurse caring for the client with Amyotrophic Lateral Sclerosis (ALS) has developed urinary frequency. The nurse does the following interventions, except: a. Place urinal/bed pan where the client can reach if able to do so b. Leaving the door of the bathroom open for the client c. Instruct the client the use of the call bell in case he needs assistance with elimination d. Anticipate urinary catheterization or external catheterization when necessary

b. Leaving the door of the bathroom open for the client To ensure safety, the client should have assistance when moving around the room. A urinal or bed pan that can be easily reached by the client is an appropriate nursing intervention, the call bell should be functional in case he needs assistance and catheterization as ordered by the HCP is anticipated, especially the use of external catheters during the night to not disrupt client's sleep.

Which of the following statements about the intake of Riluzole (Rilutek) made by the client newly diagnosed with Amyotrophic Lateral Sclerosis (ALS) needs further teaching? a. "I should not drive after taking this medication or when I feel dizzy." b. If I think that I am pregnant, I should call my doctor." c. "I can take my Omeprazole whenever I my stomach hurts as a side effect of this medication" d. "If I forget to take this medication when it is almost time for the next one, I should skip the missed dose and take the next dose as scheduled."

c. "I can take my Omeprazole whenever I my stomach hurts as a side effect of this medication" When taking Riluzole, Omeprazole should not be taken without a HCP order because it can affect liver enzymes that can also affect the absorption of the drug.

The following are potential complications in clients with Amyotrophic Lateral Sclerosis (ALS), except: Select all that apply: a. Respiratory Insufficiency b. Dementia c. Blindness d. Dysarthria e. Dysphagia f. Sialorhea

c. Blindness

The following materials can appropriately aid in communication barriers in a client with Amyotrophic Lateral Sclerosis (ALS) who has dysarthria, except? a. Magic slate b. Word or letter boards c. Electro-larynx d. Laser pointers in combination with letter board

c. Electro-larynx An Electro-larynx is a device used to produce clearer speech for those who have undergone laryngectomy and is not an appropriate device to be used in clients with dysarthria.

As the nurse takes charge of a client with Amyotrophic Lateral Sclerosis (ALS), which of the following nursing diagnosis should be her utmost priority? a. Potential for injury related to impaired physical mobility b. Impaired urinary elimination related to progressive loss of mobility c. Ineffective gas exchange related to aspiration secondary to absent gag and swallowing reflexes d. Impaired verbal communications related to altered clarity of speech

c. Ineffective gas exchange related to aspiration secondary to absent gag and swallowing reflexes oxygenation and air exchange is priority for patients with ALS who's gag and swallowing reflexes have been effected by this condition.

The client newly diagnoses with Amyotrophic Lateral Sclerosis (ALS) tells the nurse: "This is terrible, all of my plans for the future are crushed." Which of the following is an appropriate reaction of the nurse? a. Inform the client that there is hope for cure b. Tell client success stories of other ALS clients c. Stay with the client and encourage verbalization of feelings d. All of the above

c. Stay with the client and encourage verbalization of feelings The nurse should focus on the patient at the crucial time and should allow verbalization of feelings to explore behavioral changes and the grieving process. False reassurance is not a correct option.

The nurse knows that the following description is true of Amyotrophic Lateral Sclerosis (ALS), except? a. It is a rapidly progressive, invariably fatal degeneration of the upper and lower motor neurons controlling voluntary muscles. b. The word amyotrophic refers to the increase in muscle growth that occurs as a consequence of degeneration of the lower motor neurons. c. When neurons can no longer transmit impulses to the muscles, the muscles begin to atrophy and weakness increases overtime. d. Lateral sclerosis is the demyelination and gliosis occurring in the corticospinal tracts located in in the lateral spinal cord and corticobulbar tracts.

c. When neurons can no longer transmit impulses to the muscles, the muscles begin to atrophy and weakness increases overtime. The word amyotrophic refers to the decrease instead of increase in muscle growth that occurs as a consequence of degeneration of the lower motor neurons.

In giving discharge instructions to a client newly diagnosed with Amyotrophic Lateral Sclerosis (ALS), which of the following information is not consistent with the medication Riluzole (Rilutek)? a. It promotes prolong survival rates in clients with ALS by modestly slowing the disease progression b. It protects motor neurons from degeneration and death c. Within five years of continued use, it is able to cure ALS by regeneration of motor neurons. d. If administered early in the course of the disease, this medication helps client stay functional and independent longer.

c. Within five years of continued use, it is able to cure ALS by regeneration of motor neurons. Riluzole dose not cure ALS but instead, it prolongs survival and extends time that the clients are in the earlier phases of the disease and helps them stay functional and independent longer.

A client with Amyotrophic Lateral Sclerosis can have weakened articulation, in this case, the nurse should do the following communication strategies, except: a. Sit face to face with the client in a quiet, well lit room and limit the amount of distraction. b. Repeat the words that are understood to validate with the client. c.Establish the type of communication environment that is comfortable for the client. d. Finish the client's sentences for him and encourage him to say his ideas fast.

d. Finish the client's sentences for him and encourage him to say his ideas fast. Hurrying the client does not help him articulate his ideas better. The nurse should have the client with ALS slow down and take his time when talking.

The client with Amytrophic Lateral Sclerosis asks the nurse in charge what does "sialorrhea" mean. The nurse correctly defines it as: a. A defect in the utilization and interpretation of language symbols b. Difficulty in forming words c. Difficulty in swallowing d. It is the excess amount of saliva in the mouth

d. It is the excess amount of saliva in the mouth Sialorrhea is excessive salivation which is a common problem with neurological disorders. A defect in the interpretation in language symbols is called aphasia, while difficulty in forming words is dysarthria and difficulty in swallowing is dysphasia.

The following are appropriate teaching applications to the caregiver of a client with Amyotrophic Lateral Sclerosis (ALS) with dysarthria, except: a. Anticipatory care and referral to speech pathologist and using alternative modes of communication b. Give ample support to alleviate frustration related to difficulty in communicating c. Allow the client sufficient time to respond to verbal communication d. None of the above.

d. None of the above.

The client with Amyotrophic Lateral Sclerosis (ALS) states "This is not the life I have dreamed of, I can't even button my shirt!" The nurse recognizes that this statement supports which nursing diagnosis? a. Disturbance in body image b. Risk for disuse syndrome c. Grief d. Powerlessness

d. Powerlessness The comment directly points to powerlessness which is commonly seen in patients with ALS because they lose their ability to preform simple daily tasks when they are unable to use their hands and arms.