ALS

In a client with amyotrophic lateral sclerosis (ALS) and respiratory distress, which finding is the earliest sign of reduced oxygenation? a) Decreased level of consciousness (LOC) b) Decreased heart rate c) Increased restlessness d) Increased blood pressure

c) Increased restlessness

The client with end-stage ALS requires a gastrostomy tube feeding. Which finding would require the nurse to hold a bolus tube feeding? 1. A residual of 125 mL. 2. The abdomen is soft. 3. Three episodes of diarrhea. 4. The potassium level is 3.4 mEq/L.

1. A residual of 125 mL. 1. A residual (aspirated gastric contents) of greater than 50 to 100 mL indicates that the tube feeding is not being digested and that the feeding should be held. 2. A soft abdomen is normal; a distended abdomen would be cause to hold the feeding. 3. Diarrhea is a common complication of tube feedings, but it is not a reason to hold the feeding. 4. The potassium level is low and needs intervention, but this would not indicate a need to hold the bolus tube feeding.

The client is diagnosed with ALS. Which client problem would be most appropriate for this client? 1. Disuse syndrome. 2. Altered body image. 3. Fluid and electrolyte imbalance. 4. Alteration in pain.

1. Disuse syndrome. 1. Disuse syndrome is associated with complications of bedrest. Clients with ALS cannot move and reposition themselves, and they frequently have altered nutritional and hydration status. 2. The client does not usually have a change in body image. 3. ALS is a disease affecting the muscles, not the kidneys or circulatory system. 4. ALS is not painful.

The client is in the terminal stage of ALS. Which intervention should the nurse implement? 1. Perform passive ROM every two (2) hours. 2. Maintain a negative nitrogen balance. 3. Encourage a low-protein, soft-mechanical diet. 4. Turn the client and have him cough and deep breathe every shift.

1. Perform passive ROM every two (2) hours. 1. Contractures can develop within a week because extensor muscles are weaker than flexor muscles. If the client cannot perform ROM exercises, then the nurse must do it for him—passive ROM. 2. The client should maintain a positive nitrogen balance to promote optimal body functioning. 3. Adequate protein is required to maintain osmotic pressure and prevent edema. 4. The client is usually on bedrest in the last stages and should be turned and told to cough and deep breathe more often than every shift.

The client diagnosed with ALS asks the nurse, "I know this disease is going to kill me. What will happen to me in the end?" Which statement by the nurse would be most appropriate? 1. "You are afraid of how you will die?" 2. "Most people with ALS die of respiratory failure." 3. "Don't talk like that. You have to stay positive." 4. "ALS is not a killer. You can live a long life."

2. "Most people with ALS die of respiratory failure." 1. This is a therapeutic response, but the client is asking for specific information. 2. About 50% of clients die within two (2) to five (5) years from respiratory failure, aspiration pneumonia, or another infectious process. 3. The nurse should allow the client to talk freely about the disease process and should provide educational and emotional support. 4. This is incorrect information; ALS is a disease that results in death within five (5) years in most cases.

The client with ALS is admitted to the medical unit with shortness of breath, dyspnea, and respiratory complications. Which intervention should the nurse implement first? 1. Elevate the head of the bed 30 degrees. 2. Administer oxygen via nasal cannula. 3. Assess the client's lung sounds. 4. Obtain a pulse oximeter reading.

2. Administer oxygen via nasal cannula. 1. Elevating the head of the bed will enhance lung expansion, but it is not the first intervention. 2. Oxygen should be given immediately to help alleviate the difficulty breathing. Remember that oxygenation is priority. 3. Assessment is the first part of the nursing process and is priority, but assessment will not help the client breathe easier. 4. This is an appropriate intervention, but obtaining the pulse oximeter reading will not alleviate the client's respiratory distress.

Which diagnostic test is used to confirm the diagnosis of ALS? 1. Electromyogram (EMG). 2. Muscle biopsy. 3. Serum creatine kinase (CK). 4. Pulmonary function test.

2. Muscle biopsy. 1. EMG is done to differentiate a neuropathy from a myopathy, but it does not confirm ALS. 2. Biopsy confirms changes consistent with atrophy and loss of muscle fiber, both characteristic of ALS. 3. CK may or may not be elevated in ALS so it cannot confirm the diagnosis of ALS. 4. This is done as ALS progresses to determine respiratory involvement, but it does not confirm ALS.

The son of a client diagnosed with ALS asks the nurse, "Is there any chance that I could get this disease?" Which statement by the nurse would be most appropriate? 1. "It must be scary to think you might get this disease." 2. "No, this disease is not genetic or contagious." 3. "ALS does have a genetic factor and runs in families." 4. "If you are exposed to the same virus, you may get the disease."

3. "ALS does have a genetic factor and runs in families." 1. The son is not sure if he may get ALS, so this is not an appropriate response. 2. This is incorrect information. 3. There is a genetic factor with ALS that is linked to a chromosome 21 defect. 4. ALS is not caused by a virus. The exact etiology is unknown, but studies indicate that some environmental factors may lead to ALS.

The client is diagnosed with ALS. As the disease progresses, which intervention should the nurse implement? 1. Discuss the need to be placed in a long-term care facility. 2. Explain how to care for a sigmoid colostomy. 3. Assist the client to prepare an advance directive. 4. Teach the client how to use a motorized wheelchair.

3. Assist the client to prepare an advance directive. 1. With assistance, the client may be able to stay at home. Therefore, placement in a long-term care facility should not be discussed until the family can no longer care for the client in the home. 2. There is no indication that a client with ALS will need a sigmoid colostomy. 3. A client with ALS usually dies within five (5) years. Therefore, the nurse should offer the client the opportunity to determine how he/she wants to die. 4. ALS affects both upper and lower extremities and leads to a debilitating state, so the client will not be able to transfer into and operate a wheelchair.

The nurse is caring for the following clients on a medical unit. Which client should the nurse assess first? 1. The client with ALS who is refusing to turn every two (2) hours. 2. The client with abdominal pain who is complaining of nausea. 3. The client with pneumonia who has a pulse oximeter reading of 90%. 4. The client who is complaining about not receiving any pain medication.

3. The client with pneumonia who has a pulse oximeter reading of 90%. 1. Refusing to turn needs to be addressed by the nurse, but it is not priority over a life-threatening condition. 2. Nausea needs to be assessed by the nurse, but it is not priority over an oxygenation problem. 3. A pulse oximeter reading of less than 93% indicates that the client is experiencing hypoxemia, which is a life-threatening emergency. This client should be assessed first. 4. The nurse must address the client's complaints, but it is not a priority over a physiological problem.

The client diagnosed with ALS is prescribed an antiglutamate, riluzole (Rilutek). Which instruction should the nurse discuss with the client? 1. Take the medication with food. 2. Do not eat green, leafy vegetables. 3. Use SPF 30 when going out in the sun. 4. Report any febrile illness.

4. Report any febrile illness. 1. The medication should be given without food at the same time each day. 2. This medication is not affected by green leafy vegetables. (The anticoagulant warfarin [Coumadin] is a well-known medication that is affected by eating green, leafy vegetables.) 3. This medication is not affected by the sun. 4. The medication can cause blood dyscrasias. Therefore, the client is monitored for liver function, blood count, blood chemistries, and alkaline phosphatase. The client should report any febrile illness. This is the first medication developed to treat ALS.

The client is being evaluated to rule out ALS. Which signs/symptoms would the nurse note to confirm the diagnosis? 1. Muscle atrophy and flaccidity. 2. Fatigue and malnutrition. 3. Slurred speech and dysphagia. 4. Weakness and paralysis.

4. Weakness and paralysis. 1. These signs and symptoms occur during the course of ALS, but they are not early symptoms. 2. These signs and symptoms will occur as the disease progresses. 3. These are late signs/symptoms of ALS. 4. ALS results from the degeneration and demyelination of motor neurons in the spinal cord, which results in paralysis and weakness of the muscles.