Multiple Sclerosis - Medicine Study Guide

2 Multiple Sclerosis Pathophysiology Multiple sclerosis (MS) is a disease involving plaque demyelination in the CNS, - the destruction of the fatty substance that protects and coats the nerve fibers in the spinal cord and brain – making it difficult for the brain to send signals to the rest of the body (Hinkle et al., 2022, p. 2095). This is comparable to the insulation coating on electrical wires. If nerve fibers are exposed and myelin is damaged, messages that travel along that nerve fiber may be blocked or slowed. MS presents with focal sensory disturbances, double vision, sight damage, limb weakness, unsteady gait, and urinary retention (Hinkle et al., 2022, p. 2095). It is suggested environmental and genetic factors are influenced through an underlying pathophysiology widely believed to be immune; onset trends occur in ages 20-40 and female-male ratio of 1:2 (Hinkle et al., 2022, p. 2095). Signs and Symptoms Figure 1 Multiple sclerosis anatomical view Note. By Mayo Clinic, Online image, Copyright 2022 by Mayo Foundation for Medical Education and Research. Multiple Sclerosis can easily be understood as having multiple scars due to having multiple lesions. Because of these lesions/scars that are caused by the body attacking the myelin sheath, the body reacts to different signs and symptoms depending on where these lesions/scars are located (Mayo Clinic, 2022). 3 1. When lesions/scars are noted in the optic nerve, clients eye sight can be affected. They will have s/s of diplopia (having double vision or possibly complaining of seeing two images of one object). This can affect one or both eyes. Other eyesight concerns are also blurred vision, having patchy or total blindness/loss of vision (Mayo Clinic, 2022). 2. When lesions/scars are noted in the area of the cerebral cortex or brain stem, clients movement/motor pathways in the spinal cord can be affected. The s/s that will occur is spasticity. Clients will complain of having weakness, spasms, loss of power/strength on the arm/leg, numbness to extremities (Hinkle et al., 2022, p. 2095). 3. When lesions/scars are noted in the sensory pathway, s/s of pain can occur. Neuropathic pain happens from “short circuiting” of the nerves that carry signals from the brain to the body because of damage from MS. These pain sensations feel like burning, stabbing, sharp and squeezing sensations (Pain and Itching, National Multiple Sclerosis Society, n.d.). Additional sensory s/s are paresthesias, dysesthesias, and proprioception loss (Hinkle et al., 2022, p. 2095). 4. If the frontal or parietal lobe are affected, s/s of cognition may occur. Memory loss and decreased concentration are possible complaints of clients (Hinkle et al., 2022, p. 2095). 5. If the cerebellum or basal ganglia are affected, s/s of ataxia and tremor may occur (Hinkle et al., 2022, p. 2095). 6. Depending on where the lesions/scars are noted, other s/s can occur such as bladder, bowel, and sexual dysfunctions; emotional lability and euphoria; fatigue, difficulty in coordination, loss of balance (Hinkle et al., 2022, p. 2095). 7. The most disabling symptom of all is fatigue. Many factors can contribute to fatigue such as heat, depression, anemia, deconditioning, and medication (Hinkle et al., 2022, p. 2096). 8. Due to these s/s, complications can occur such as UTI, constipation, PI, contracture deformities, dependent pedal edema, PNA, osteoporosis (Hinkle et al., 2022, p. 2096). Different Types of MS (see Figure 2): 4 Figure 2 Types of Multiple Sclerosis Note. By M. Wexler, Online image, Copyright 2023 by Multiple Sclerosis News Today. 1. Radiologically Isolated Syndrome (RIS) - will have no symptoms (Hinkle et al., 2022, p. 2095). 2. Clinically Isolated Syndrome (CIS) - includes optic neuritis, focal symptoms, or partial myelopathy (Hinkle et al., 2022, p. 2095). 3. Relapsing-remitting MS (RRMS) - the most common disease course. RRMS are followed by periods of partial or complete recovery, or remission. In remissions, all symptoms may disappear or some symptoms may continue and become permanent. Most clients will move to the secondary progressive form in which disease progression occurs with or w/o relapses (“Types of multiple sclerosis. National Multiple Sclerosis Society,” n.d.). 4. Secondary progressive MS (SPMS) - neurologic function worsens progressively or disability accumulates overtime (“Types of multiple sclerosis. National Multiple Sclerosis Society,” n.d.). 5 5. Primary progressive MS (PPMS) - disabling symptoms steadily increase, with rare plateaus and temporary minor improvement. PPMS may result in quadriparesis, cognitive dysfunction, visual loss, and brainstem syndromes (Hinkle et al., 2022, p. 2095). Labs and Diagnostics Neuropsychological testing Assess cognitive impairment (Hinkle et al., 2022, p. 2096). Urodynamic studies Assess any bladder dysfunctions (Hinkle et al., 2022, p. 2096). Blood tests Blood tests can aid in ruling out additional illnesses that present with symptoms similar to MS (Hinkle et al., 2022, p. 2096). Lumbar puncture (spinal tap) (see Figure 3): This procedure extracts a small amount of cerebrospinal fluid for laboratory examination Antibody abnormalities linked to multiple sclerosis can be seen in this sample while also ruling out infections and disorders similar to MS (“Multiple Sclerosis - Diagnosis and Treatment,” 2022). Antibody a Figure 3 Spinal tap anatomical view Note. By Mayo Clinic, Online image, Copyright 2022 by Mayo Foundation for Medical Education and Research. 6 MRI (see Figure 4): An MRI can show where the brain, cervical, and thoracic spinal cord have MS lesions. A contrast agent may be injected intravenously to draw focus on lesions that signify an active stage of MS (Hinkle et al., 2022, p. 2096). Figure 4 MRI multiple sclerosis lesions Note. By Mayo Clinic, Online image, Copyright 2022 by Mayo Foundation for Medical Education and Research. Evoked potential tests These tests can be done to capture the electrical impulses the nervous system produces in response to stimuli, in which both optical and electrical stimuli can be used (Hinkle et al., 2018). Short electrical impulses are administered to the nerves in the arms or legs while a moving visual pattern can be seen during these examinations (Hinkle et al., 2022, p. 2096). Medications - Pharmacologic Therapy ● There is no cure for multiple sclerosis. 7 ● There exists drugs that instead provide relief: disease modifying drugs and symptom managing drugs. ● Disease modifying drugs delay the progression of disease. Notable examples include the IV immunosuppressant mitoxantrone and the corticosteroid methylprednisolone . ● Symptom managing drugs are aimed at the symptoms that accompany MS (Adams et al., 2020, p. 273) Disease Modifying Drugs ● Useful for RRMS (remitting-relapsing multiple sclerosis) but not PPMS (primary progressive multiple sclerosis). The three goals of disease modifying drugs are: ● to reduce the frequency of relapse ● the duration of relapse ● number and size of plaques observed on MRI (Adams et al., 2020, p. 274) Interferon beta-1a and interferon beta-1b ● Subcutaneous every other day ● IM once a week (different version) ● Best used as early as possible in disease progression ● Side effects: (Adams et al., 2020, p. 274) ○ Dizziness, headaches, weakness, confusion, anxiety, mental depression, conjunctivitis, constipation, diarrhea, sexual dysfunction, sweating, menstrual disorders, neutropenia, flu like symptoms, spasticity, pain and itching reactions at the injection site, seizures, anaphylaxis, hepatotoxicity Glatiramer acetate ● Subcutaneous daily ● Reduces rate of relapse ● Adverse effects: (Adams et al., 2020, p. 274) ○ injection-site reactions such as pain, itching, swelling and redness and flushing 8 ○ ● Self limiting adverse effects There is nothing necessary to monitor Teriflunomide, fingolimod, and dimethyl fumarate ● PO ● Used for patients that can’t tolerate consistent injections ● Has a significantly reduced relapse rate in several types of MS (Bradshaw & Houtchens, 2018 as cited in Hinkle et al., 2022, p. 2097). Ocrelizumab ● Has a 6% annual relapse reduction rate in patients with PPMS (Bradshaw & Houtchens, 2018 as cited in Hinkle et al., 2022, p. 2097). Methylprednisolone (Depo-Medrol, Medrol, others) ● IV/IM ● Immunosuppressant ● Treats acute exacerbations, shortens the duration of relapse but has not been found to have long-term benefit (Bradshaw & Houtchens, 2018 as cited in Hinkle et al., 2022, p. 2097). ● Treatment is followed by an oral taper of prednisone ● Adverse effects: (Adams et al., 2020, p. 704) ○ Mood swings, weight gain, acne, facial flushing, nausea, insomnia, sodium and fluid retention, impaired wound healing, menstrual abnormalities, hyperglycemia, hypocalcemia, immunosuppression Mitoxantrone ● IV every 3 months ● Reduces frequency of clinical relapse in secondary progressive MS or RRMS ● There is a maximum lifetime dose (Hickey & Strayer, 2020 as cited in Hinkle et al., 2022, p. 2097). ● The maximum lifetime dose for mitoxantrone is 120 mg/m2 (Kingwell et al., 2010) 9 ● Side effects: (Adams et al., 2020, p. 275) ○ Nausea, vomiting, fever, mouth sores, diarrhea, hair loss, anemia, increased susceptibility to infection ○ Cardiotoxicity, dysrhythmia, shortness of breath, myelosuppression Symptom Management Drugs: Treating Spasticity Baclofen ● Medication of choice ● Gamma-aminobutyric acid agonist ● PO ● Intrathecal injection for severe spasticity (Hickey & Strayer, 2020 as cited in Hinkle et al., 2022, p. 2097) Benzodiazepines (e.g., diazepam), tizanidine, and dantrolene ● Treats spasticity and improves motor function (Hickey & Strayer, 2020 as cited in Hinkle et al., 2022, p. 2097). ● Diazepam ○ Antiseizure drug ○ Benzodiazepine: GABA receptor agonist ○ IV/IM ○ When administering IV, monitor reparations every 5-15 mins ○ Don’t take with alcohol ○ Side effects: ■ Drowsiness, sedation, ataxia ■ Laryngospasm, respiratory depression, cardiovascular collapse, coma (Adams et al., 2020, p. 274) ● Dantrolene ● Skeletal muscle relaxant 10 ● Direct-acting antispasmodic; calcium release blocker ● PO ● Side effects: ○ Muscle weakness, dizziness, diarrhea ○ Hepatic necrosis ■ There is also a higher proportion of hepatic events with fatal outcomes in older patients receiving dantrolene (Adams et al., 2020, p. 174). Symptom Management Drugs: Treating Ataxia ● Chronic and resistant to medication (Hinkle et al., 2022, p. 2097) Beta-adrenergic blockers (propranolol) ● Side effects: ○ Fatigue, insomnia, drowsiness, bradycardia, confusion ○ Agranulocytosis, laryngospasm, Stevens Johnson syndrome, anaphylaxis ● Gabapentin ● Benzodiazepines (clonazepam) (Hinkle et al., 2022, p. 2097) Symptom Management Drugs: Treating Bladder and Bowel Problems ● Anticholinergics ● Alpha-adrenergic blockers ● Antispasmodic agents ● Nonpharmacologic strategies (Hinkle et al., 2022, p. 2097) Symptom Management Drugs: Treating UTI ● Occurs on top of neuro ● Logical disease ● Antibiotics given as needed ● Increased fluid intake and good perineal care prevent UTI (Hinkle et al., 2022, p. 2097) 11 A_PIE Assessment ● Assess for neurological problems due to myelin damage. Such problems may include paresthesia, numbness, unsteady gait and posture, blurry or double vision, slurred speech or affected cognition, fatigue or vertigo (Hinkle et al., 2018, p. 536; “Multiple Sclerosis - Diagnosis and Treatment,” 2022). ● Monitor for other secondary complications. These complications may include at-home lifestyle management, sexuality changes, or suicidal ideation (Hinkle et al., 2018, p. 536). ● Assess the psychosocial influences of the diseases on the patient’s relationships; for example, coping mechanisms, family conflict, separation (Hinkle et al., 2018, p. 536). Planning Planning for the patient focuses on physical mobility, fall risks, psychosocial influences, and absence of complications (Hinkle et al., 2022, p. 2098). Goals for this patient would be for the patient to: ● exercise once a day ● not experience injuries related to diagnosis ● express and show improvement in swallowing ability ● be bladder and bowel continent ● take frequent rests throughout the day (Hinkle et al., 2018, p. 536) Implementation ● Encourage exercise ○ Daily to stretch muscles and minimize joint contractures ○ Encourage patient to push self to continue just before fatigue ○ Encourage increasing resistance in exercises in order to build strength in weaker muscles ○ Examples: ■ Walking to improve gait ■ Swimming and stationary biking 12 ■ Progressive weight bearing ■ Swallowing exercises ● Encourage patient to take frequent rests throughout the day in order to prevent extreme fatigue ● Keep bedpan and urinal nearby ● Set a schedule for voiding, gradually increasing the time in between ● Provide bowel training, adequate fluids, and dietary fibers ● If diplopia occurs, provide patient with an eyepatch/eyeglass occluder ● Teach patient and family pathophysiology and symptoms about diagnosis ● Educate patient on importance of medications ● Encourage patient to take medications ● Maintain appropriate temperature in patient’s environment ● ○ Increased muscle weakness and fatigue may occur from a warm/hot environment ○ Increased spasticity may occur from a colder environment Provide patient with emotional support and assess for any disturbances in mental behavior and coping mechanisms ● Make referrals for counseling, support, and speech therapy ● Assess for pressure ulcers if frequently in wheelchair or bed ● Encourage patient to contact health care provider if any symptoms worsen or changes at home Evaluation Goals will be evaluated to be met if the patient: ● reports improved physical ability by the end of the week ● experiences no injuries at the end of every shift ● expresses and shows improvement in swallowing function by the end of the week ● achieves or maintains bladder and bowel continence in 72 hours ● expresses little to no fatigue throughout the day (Hinkle et al., 2018, p. 540) 13 CASE STUDY Educational Case: Multiple sclerosis In this Multiple Sclerosis Case Study from the National Library of Medicine, the patient is a 32-year-old woman who came to the emergency room presenting with signs and symptoms of Multiple Sclerosis. The patient reported that she experienced recurrent “unilateral visual impairment” and “facial numbness” on the right side (Pape, MD et al., 2022, pg. 1). In the last six months prior to the present ER visit, the patient had three episodes of these symptoms, each lasting for multiple weeks (Pape, MD et al., 2022, pg. 1). On the same day she presented to the emergency room, the patient noted when she woke up, she experienced “sudden onset of blurry vision in her right eye” and “numbness of the right side of her face” (Pape, MD et al., 2022, pg. 1). Her vital signs are stable, however, the neurologic results for her right eye showed she had 20/100 vision and her left vision was normal (Pape, MD et al., 2022, pg. 1). The patient had equal and strong muscle strength for all extremities but had lost sensation on the whole side of her right face (Pape, MD et al., 2022, pg. 1). As referenced by this case study, the physiologic analysis of MS is primarily achieved by analyzing multiple diagnostic data which ultimately demonstrates the dissemination of lesions in the white matter of the CNS (Pape, MD et al., 2022, pg. 1). This collection of data consists of diagnostic imaging, laboratory testing, and electrophysiologic testing. In this scenario, the patient has tested negative for Romberg's sign and her neurologic examination has only revealed deviations from baseline such as 20/100 vision in the right eye (Pape, MD et al., 2022, pg. 1). Under diagnostic imaging, the most important is an MRI of the brain and spinal cord because this is crucial for the observation/analysis of a “typical MS lesion” that is “round/ovoid” in shape, and “1-2 cm in size” or another MS related white matter abnormality (Pape, MD et al., 2022, pg. 1). Laboratory testing should include a CSF analysis that includes WBCC, RBCC, protein concentration, glucose level, immunoglobulin G (IgG) index, and oligoclonal band testing. Indicative of MS, the patient’s CSF lab exposed a significantly elevated IgG (7.2 mg/dl) and 10 oligoclonal bands were identified in the CSF (Pape, MD et al., 2022, pg. 1). 14 Electrophysiologic testing is indicated in the form of evoked potentials to measure electrical activity in the CNS. Because no cure is available for MS, treatment is symmetrical to the nursing management discussed in the A-PIE and Interventions section above. As stated in the article, treatment consists of counseling, physical therapy, exercise, and pharmacotherapy. In alignment with the nursing management of patients with multiple sclerosis, pharmacological management calls for the use of disease modifying therapeutic agents specific to RRMS, Injections such as recombinant human interferon beta-1b and beta 1a, as well as fumarates and sphingosine 1-phosphate receptor modulators to combat patient intolerance to injectable agents (Pape, MD et al., 2022, pg. 1). Overall, this clinical case traversed major clinical assessment findings, significant diagnostic tests and values, and crucial treatment options that are each important to the nursing management of a patient with multiple sclerosis.