Amyotrophic lateral sclerosis (ALS)

2 Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease Patho: Amyotrophic lateral sclerosis ( ALS) is also known as Lou Gehrig's disease. This disease is a neurodegenerative disorder that is progressive. It primarily affects motor neurons in both the brain and spinal cord. Degeneration of upper motor neurons in the cerebral cortex. Lower motor neurons in the brainstem and spinal cord are also affected. The exact cause of ALS is not fully known. It likely involves both genetic and environmental factors. One theory in ALS involves glutamate excitotoxicity. Evidence shows there is excessive glutamate release from neurons. Additionally, abnormal protein aggregation is another assay mark of ALS. This leads to toxic protein deposits in motor neurons. Signs and Symptoms Muscle weakness and atrophy Loss of voluntary muscle control Difficulty breathing Dysphagia Constipation Respiratory paralysis, which turns into respiratory failure Medications Riluzole-Called benzothiazoles Action: Works by changing the activity of certain natural substances in the body that affect nerves and muscles. Specifically by blocking glutamate. Edaravone- Improved functional ability. Action: unknown (Davis Drug Guide 2010) Relyvrio- Action: reduce the unfolded protein response, preventing Cell death resulting from the unfolded protein response. shown to help slow disease progression. Tofersen- Action: antisense oligonucleotide that targets SOD1 mRNA to reduce the synthesis of the SOD1 protein. Baclofen- Helps with spasticity Dantrolene Sodium- Helps with spasticity Modanifil- useful for fatigue (Brunner 2021) 3 - Diagnostic Tests, Procedures, Labs - “ALS is diagnosed on the basis of signs and symptoms because no clinical test or laboratory test is specific for this disease.” (Hinkler,2022) - Neurological assessment consists of function in muscle reflexes, muscle strength, and coordination. - Electromyography - A series of small electrodes are inserted into the muscle. The electrodes then pick up electrical activity and display the findings on a monitor, where they are portrayed in waves. - This test shows if muscles are responding properly to nerve conduction, distinguishing if the problem is caused by nerve function or muscle function. - Nerve Conduction Study - Two electrode patches are applied to the skin surface over particular nerves. One of the electrodes provides an electrical impulse while the other records the speed of conduction. - This diagnostic test is used in conjunction with EMG to best diagnose ALS. - Diagnostic tests that rule out other diseases - MRI - Used to rule out structural abnormalities. 4 - Lumbar Puncture - - Used to rule out bleeds as well as meningitis. Muscle Biopsy - It is used to rule out muscle diseases that share the same signs and symptoms. - LABS - White Blood Cell Count - Used to rule out infections such as meningitis and those that diminish muscle function/reflex. - Cerebrospinal Fluid - The lab is used to exclude other possible conditions with similar symptoms. - Thyroid Function Tests - These labs can determine if the cause of the signs and symptoms are related to Thyroid dysfunction. - Both Hyper and Hypothyroidism can cause muscle tremors, fatigue, and muscle weakness. - Creatine Kinase - Elevated creatine kinase can be caused by muscle damage in diseases not specific to ALS. 5 A- Assess patient's muscle strength, assess for a patent airway, assess patient's ability to swallow, assess nutritional status, assess patient's ability to perform any activities of daily living, assess skin daily, assess fall risk, D N/A P have speech therapist perform a swallow evaluation I Perform range of motion to prevent contracture, proper positioning and turning, and a Daily intake of 2,500mL to maintain hydration unless contraindicated due to swallowing. Record strict I and O. Discuss changes in body image and what they mean to the patient. Encourage the patient to focus on positive aspects of self. Suction and maintain a patent airway. Administer 02 as necessary. Maintain elevated HOB at least 30 degrees at all times. Auscultate the chest frequently to assess the lungs and airway. Help the patient to cough and deep breathe every two hours or more if needed. Teach and have the patient return to demonstrate the use of incentive spirometer, b if necessary or due to chest physiotherapy. Elevate HOB at mealtime and keep it elevated for at least 30 minutes after meals. Soft, mechanical soft, or pureed diet as needed. High protein, high fiber diet, Bring in a dietician to teach the basics of good nutrition and in-between meal snacks. Take meds as prescribed. E Input and output recorded with daily input of 2500 through staff I&O monitoring. The head of the bed is kept in Fowler and semi-Fowler’s position at all times. The patient continues to have intermittent shortness of breath upon auscultation, with a patent airway noted. The patient is able to successfully demonstrate the use of the incentive spirometer but expresses he does not like doing it. The patient will need continued encouragement from staff alongside patient education 6 on the importance of the use of incentive spirometer and chest physiotherapy. Patient education on risk for pneumonia. The patient taking medications as prescribed successfully. Case Study https://www.physio-pedia.com/Amyotrophic_Lateral_Sclerosis_(ALS):_A_Case_Study Overall this was a case study about a 24-year-old male named Jared Miller. He has been diagnosed with ALS, a progressive neurological degenerative disease that affects the motor neurons. Prior to his diagnosis Jared was previously a soccer player. Jared experienced a wide variety of symptoms, such as muscle weakness, cramping, and pain in his arms and legs. Furthermore, he suffers from balance difficulties and decreased hand dexterity. These symptoms Jared experiences have affected every part of his life, including his ability to perform basic ADLs. Jared's diagnosis of ALS was made six months before the case study, following initial signs of muscle weakness and frequent falls during soccer games. Jared’s current treatment is the prescribed medication Riluzole (Amyotrophic Lateral Sclerosis (ALS): A Case Study, n.d.). The medication Riluzole works specifically by blocking the release of glutamate, as it is thought that too much glutamate can cause injury to nerve cells. While Riluzole is often prescribed to patients suffering from ALS, it is not known for its ability to change or improve symptoms (Wexler & Inácio, 2022). Despite the lack of symptom management, it is working to prolong the overall survival rate and life span of the ALS patient by slowing the progression of ALS. Even with medication management Jared's quality of life score is 7.2/10, and he rates his pain as 4/10 (Amyotrophic Lateral Sclerosis (ALS): A Case Study, n.d.). This study provides an all-encompassing overview of Jared's medical history, physical examination findings, test, functional status, and treatment of medication regimen. As indicated above, the most prominent findings include muscle weakness and atrophy, altered gait, hyperreflexia, and movement and balance impairments (Amyotrophic Lateral Sclerosis (ALS): A 7 Case Study, n.d.). Jared’s treatment is comprised of multiple interdisciplinary teams. The purpose of utilizing interdisciplinary team care is to have specialists from different areas collaborate together to attempt to improve the quality of life and treatment for the patient. In Jared’s case, specialists include physiotherapists, occupational therapists, registered dietitians, speech-language therapists, social workers, and palliative care providers. Specifically, in Jared's case, the physiotherapy intervention plan is to maintain his strength and improve his motor skills and overall balance. Specific exercises, endurance training, dexterity training, stretching, and thermotherapy are included in his program (Amyotrophic Lateral Sclerosis (ALS): A Case Study, n.d.). The interdisciplinary team as a whole is responsible for providing continuing education on ALS management for Jared. Throughout the case study, Jared's progress is assessed, and his functional abilities are maintained, despite the progressive nature of ALS. Jared’s case study focuses on the importance of early intervention for patients with ALS. Early intervention can provide the patient with more individualized care and therefore includes the use of interdisciplinary teams as well. Through this form of treatment, both the patient's lifespan and quality of life can be improved. Jared's journey provides medically educational insight into the challenges faced by those living with ALS. It also examines the role of the multiple different healthcare providers involved in the care of patients with ALS (Amyotrophic Lateral Sclerosis (ALS): A Case Study, n.d.). References