Upon completion of this activity, participants should be able to:
- Describe pathophysiological changes that occur in Multiple Sclerosis
- Compare and contrast the four possible courses of Multiple Sclerosis
- List proposed risk factors for Multiple Sclerosis
- Describe clinical manifestations of Multiple Sclerosis as they relate to destruction in the various areas of the central nervous system
- Describe pharmacological management of Multiple Sclerosis during an exacerbation, in modulating the disease course and in symptom management
- Discuss nursing care aimed at managing/controlling symptoms, promoting independence and dealing with the chronicity of the disease
The purpose of this learning activity is to increase the nurse’s knowledge of the disease process of multiple sclerosis as well as the medical and nursing management of the affected individual.
Multiple sclerosis (MS) is an immune-mediated demyelinating disease of the central nervous system (CNS). Demyelination is the destruction of the myelin, a fatty protein substance that covers the nerve fibers and acts as an insulator. This demyelination results in impaired transmission of nerve impulses in the brain, optic nerves and spinal cord (Honan, 2019).
MS can occur at any age but typically becomes apparent in young adults between the ages of 20 and 40 and rarely affects those over 60 years old (Honan, 2019). MS affects women up to three times more often than men (Bauldoff, Gubrud, & Carno, 2020). Approximately 400,000 people in the United States are affected by MS (Hoffman & Sullivan, 2017). There is currently no cure for MS, so treatment goals are instead focused on intervening during exacerbations, delaying progression of the disease and managing chronic symptoms (Honan, 2019).
Anatomy and Physiology
The basic cells of the CNS are neurons, which are composed of axons and dendrites (see Figure 1 below). The neuron is responsible for transmission of electrochemical impulses. Dendrites (thin projections that extend from the neuronal body) receive impulses that are passed down the axon for transmission to other cells. Axons are surrounded by a myelin sheath that provides insulation and helps to speed nerve impulse transmission (Bauldoff et al., 2020).
In MS, an autoimmune process is triggered, the blood brain barrier is disrupted and lymphocytes enter the CNS tissue. The lymphocytes produce IgG, an antibody that attacks and damages myelin (demyelination) and also causes the release of inflammatory chemicals and resultant edema (Bauldoff et al., 2020). Once the demyelination of axons occurs, nerve impulse flow is interrupted, and a variety of clinical manifestations may present depending on the area(s) affected. The areas in the CNS that are most often affected in patients with MS include: optic nerves, chiasm and tracts, cerebrum, brainstem and cerebellum and the spinal cord (Honan, 2019). As inflammation subsides, myelin regenerates and the symptoms of the disease may temporarily subside (Bauldoff et al., 2020).
Unfortunately, after repeated inflammatory attacks on the myelin the damage becomes irreparable and areas of the underlying axons begin to degenerate leading to scarring or plaques. As a result, the individual will be left with permanent, irreversible damage (Honan, 2019).
Figure 1: Normal Neuron vs. MS Neuron
(National Institutes of Health, 2019)
There are four possible courses of MS: (a) relapsing-remitting, (b) primary progressive, (c) secondary progressive, and (d) progressive-relapsing.
The relapsing-remitting form of MS (see Figure 2 below) is characterized by periods of remission interspersed with exacerbations (or relapses) of clinical manifestations. During relapses, new symptoms may develop, and old symptoms may reappear. Relapses or exacerbations can last anywhere from days to months (Hoffman & Sullivan, 2017). When remission occurs, it can result in almost complete recovery, yet over time remissions often become less complete with more residual baseline symptoms present during remissions (Honan, 2019). Remission may be instantaneous or occur slowly (Hoffman & Sullivan, 2017). Between relapses there is absence of disease progression. Approximately 85% of individuals with MS have this course of the disease (Hinkle & Cheever, 2018).
Figure 2: Relapsing-Remitting MS
(Lublin et al., 2014 as cited in National Multiple Sclerosis Society [NMSS], n.d.b)
The primary progressive form of MS (see figure 3 below) is characterized by continuous neurologic deterioration from the onset of clinical manifestations. With this form, there are no remissions, but the individual may experience temporary minor improvements. Disabling symptoms steadily increase as the disease progresses over time. Approximately 10 to 15% of individuals with MS have this course of the disease (Hinkle & Cheever, 2018).
Figure 3: Primary Progressive MS
(Lublin et al., 2014 as cited in National Multiple Sclerosis Society [NMSS], n.d.a)
The secondary progressive form of MS (see Figure 4 below) is characterized by gradual deterioration with or without relapses which develops after an initial period of relapsing-remitting disease. Although some improvement may be noted after a relapse, there is no real recovery (Bauldoff et al., 2020). Approximately 40% of those who have the relapsing-remitting form will progress to this course of the disease (Honan, 2019).
Figure 4: Secondary Progressive MS
(Lublin et al., 2014 as cited in National Multiple Sclerosis Society [NMSS], n.d.c)
The progressive-relapsing form of MS is characterized by gradual progression of neurologic deterioration right from the onset with superimposed relapses. The relapses may or may not have intermittent periods of recovery. This is the least common form of the disease, affecting approximately 5% of individuals with MS (Hinkle & Cheever; Honan, 2019).
Although the etiology of MS is unknown, there is speculation that environmental factors and genetic susceptibility play a role in triggering an immune response that results in damage to the myelin sheath, which can also progress to damage to the oligodendrocytes, axons and neurons. Research suggests that exposure to the Epstein-Barr virus and mononucleosis may play a role in the development of MS (Honan, 2019). Other suggested environmental risks include smoking and vitamin D deficiency (Bauldoff et al., 2020; Hinkle & Cheever, 2018). Genetic predisposition is seen as an increased risk for Caucasians (Hoffman & Sullivan, 2017; Honan, 2019).
Prevalence of MS is highest in the northern United States, southern Canada, Europe, New Zealand and southern Australia. There is greater frequency in temperate regions and latitudes away from the equator. MS is rare in the Asian population (Hinkle & Cheever, 2018).
Signs and Symptoms
Fatigue is one of the more common complaints of MS and is poorly understood. It is often one of the most disabling symptoms (Hinkle & Cheever, 2018). Individuals with MS may describe it as lack of physical or mental energy to complete daily activities. Fatigue is typically reported to be worse in the afternoon. Influencing factors may include: depression due to the chronicity of the disease, deconditioning, and heat (Honan, 2019).
Clinical manifestations of MS are varied and are primarily determined by the location of any current lesion or lesions. For example, if there are lesions or plaques in the optic nerves or their connections, the individual may experience diplopia (double vision), blurred vision, nystagmus (jerking movement of the eyes either vertically or horizontally), scotomas (blind spots), and even periods of total blindness (Honan, 2019). Additionally, if the cranial nerves innervating speech and swallowing are affected, the individual may have dysarthria and dysphagia (Hinkle & Cheever, 2018).
Lesions on the sensory pathways may cause the individual to experience pain which can be debilitating and lead to daily use of opioid analgesics, anti-seizure medications or antidepressants (Hinkle & Cheever, 2018); (Honan, 2019). A physical assessment finding associated with sensory pathway involvement is Lhermitte’s sign, which is a shock-like sensation that is felt down the arms and back when the individual flexes the head forward (Honan, 2019). Disruption of these sensory axons can also lead to numbness, paresthesias (tingling or burning) or dysesthesias (abnormal sense of touch) (Hinkle & Cheever, 2018; Honan, 2019).
When patients have active lesions in the cerebellum or basal ganglia, they may demonstrate ataxia (impaired coordination of movements) or tremor (Hinkle & Cheever, 2018; Honan, 2019). Cognitive and psychosocial problems may be indicative of frontal or parietal lobe involvement and may be displayed by memory impairment and loss of concentration (Honan, 2019).
When the main motor pathways of the spinal cord are involved, the individual may have spasticity (muscle hypertonicity) of the extremities, most commonly the legs (Hinkle & Cheever, 2018; Honan, 2019). This can result in painful spasms and affect the individual’s mobility, functional independence and disrupt sleep (Honan, 2019). Additionally, with spinal cord involvement there may be effects on bladder, bowel and sexual function. When individuals experience bladder dysfunction it typically falls into one of the following categories: (1) hyperreflexic bladder, or inability to store urine; (2) hyporeflexic bladder, or inability to empty the bladder; or (3) a mixture of both types (Hinkle & Cheever, 2018). Bowel problems can result in incontinence or constipation. Sexual dysfunction can be due to erectile of ejaculatory dysfunction in males and orgasmic dysfunction and vaginal dryness in females (Bauldoff et al., 2020).
Multiple Sclerosis can be difficult to diagnose because symptoms may be intermittent and also mimic other disease processes. A detailed history and physical along with an extensive neurological examination are essential (Hoffman & Sullivan, 2017).
Magnetic resonance imaging (MRI) is used to identify the presence of lesions (plaques) within the CNS and is the most definitive test available. However, it is only one of several diagnostic tests. Examination of cerebrospinal fluid through lumbar puncture for presence of increased number of T lymphocytes (indicative of an immune response) and elevated levels of immunoglobulin G (IgG) also provides support of the diagnosis. A CT scan of the brain may reveal atrophy or white matter lesions (Bauldoff et al., 2020).
During an exacerbation, the goal is to decrease inflammation and induce remission. A high dose glucocorticoid, such as intravenous methylprednisolone, is used to exert anti-inflammatory effects on T cells and cytokines. This medication is administered over a 3 to 5-day period followed by a short taper with oral prednisone (Honan, 2019). The nurse should actively monitor for hyperglycemia and hypokalemia during this time (Vallerand & Sanoski, 2017).
Disease-modifying medications are useful in reducing the frequency of relapses, the length of the relapse and the number and size of the lesions (plaques) visible on the MRI. These medications should be started as early as possible following diagnosis (Honan, 2019).
Interferon Beta-1a is available in an intramuscular form administered weekly (Avonex) and in a subcutaneous form administered three times per week (Rebif). Interferon Beta-1b (Betaseron) is administered subcutaneously every other day. The main mechanism of action of interferons is reducing inflammation by limiting the movement of inflammatory cells to the CNS through the blood-brain barrier (Smelkowska, Wilkiewicz, Grabowska-Fudala, & Jaracz, 2018). A common side effect of all interferon beta medications is flu-like systems, such as fevers, chills, and body aches, which can be reduced by concurrent administration of acetaminophen or ibuprofen and by administering the medication at bedtime (Honan, 2019). Additional side effects include leukopenia, thrombocytopenia, anemia, liver dysfunction, fetal abnormalities and depression. Individuals taking these medications should have routine laboratory tests, including complete blood counts with differential and liver function studies. The individual and family should be advised to notify the health care provider (HCP) if thoughts of suicide are experienced or expressed to others. Individuals should contact the HCP if pregnancy is planned or suspected (Honan, 2019; Vallerand & Sanoski, 2017).
Glatiramer acetate (Copaxone) is another subcutaneous injection administered daily but may take up to six months to demonstrate effectiveness (Honan, 2019). It is believed to affect T-cell activation and proliferation. Adverse reactions include swelling, inflammation and reddening at injection sites (Smelkowska et al., 2018). As with all injectable medications, the nurse should include medication safety, preparation, proper injection technique, and disposal in their patient education (Vallerand & Sanoski, 2017).
Fingolimod (Gilenya) is a daily oral medication that helps to trap immune cells in lymph nodes resulting in a decrease in frequency of relapses and a delay in disability (Bauldoff et al., 2020). Monitoring is required for at least 6 hours after the first dose and periodically during therapy due to the risk for bradycardia (Vallerand & Sanoski, 2017).
Dimethyl fumarate (Tecfidera) and teriflunomide (Aubagio), are two addition oral medications indicated for the treatment of the relapsing forms of MS (Vallerand & Sanoski, 2017). The therapeutic mechanism of both dimethyl fumarate and teriflunomide are not well understood, however both have demonstrated immunodulator properties that suppress the inflammatory process (Smelkowska et al., 2018). As both of these medications can decrease the ability to fight infection and cause hepatotoxicity, individuals will need to have routine complete blood counts and differentials as well as liver function studies (Vallerand & Sanoski, 2017; Wilson, Shannon, & Shields, 2017).
Natalizumab (Tysabri), a laboratory-produced monoclonal antibody, is designed to hinder movement of damaging immune cells from the bloodstream across the blood-brain barrier into the brain and spinal cord (National Multiple Sclerosis Society, n.d.d). It is administered as a one-hour infusion every 4 weeks and is used as monotherapy for treatment of the relapsing forms of MS in highly active disease where conventional treatment with interferons or glatiramer acetate has not been effective (Smelkowska et al., 2018). Due to the severity of the side effects the risk/benefit ratio requires consideration. Although rare, progressive multifocal leukoencephalopathy (PML), an opportunistic infection, is a potential adverse effect caused by the human polyomavirus 2 (previously the JC virus) (Smelkowska et al., 2018; Wilson et al., 2017). PML is characterized by onset of dementia, progressive weakness, vision changes, coma and even death (Smelkowska et al., 2018).
Mitoxantrone (Novantrone), given via intravenous infusion every three months, can help reduce the frequency of relapses in individuals with secondary progressive or worsening relapsing-remitting MS. Cardiac toxicity is a serious adverse effect and close monitoring is required to evaluate for a decrease in left ventricular function through studies such as echocardiogram (Hinkle & Cheever, 2018; Wilson et al., 2017).
Medications for Symptom Management
Muscle spasticity may be experienced by individuals with MS. Muscle relaxants help to depress the CNS to reduce pain and inhibit reflexes at the spinal level thus reducing muscle spasm. Medications that can be administered include baclofen (Lioresal), diazepam (Valium), tizanidine (Zanaflex) and dantrolene sodium (Dantrium) (Bauldoff et al., 2020; Hoffman & Sullivan, 2017). If spasticity is severe and not sufficiently controlled with oral medications, placement of an intrathecal baclofen (Lioresal) pump may be indicated (Honan, 2019).
Fatigue can interfere with performance of activities of daily living. Medications such as amantadine (Symmetrel) or pemoline (Cyclert) may be prescribed to combat fatigue (Hinkle & Cheever, 2018). Modafinil (Provigil) can also be used to combat fatigue and improve wakefulness in clients with MS (Hoffman & Sullivan, 2017).
Medications that can be used to treat ataxia include the beta-adrenergic blocker propranolol (Inderal), the anticonvulsant gabapentin (Neurontin) and the benzodiazepine clonazepam (Klonopin) (Hinkle & Cheever, 2018).
Anticholinergics may be prescribed for bladder spasticity and cholinergic agents may be prescribed for urinary retention. Ascorbic acid may be recommended to acidify the urine, making bacterial growth less likely, thus decreasing the risk for urinary tract infection (Hinkle & Cheever, 2018).
A physical therapy consultation will help the client with exercises to improve muscle function and determine the need for assistive devices that are aimed at maintaining safety through fall prevention (Hinkle & Cheever, 2018). An occupational therapy consultation will help determine if the client needs assistive devices for fine motor skills to promote independence and suggest measures to promote energy conservation (Hinkle & Cheever, 2018). Consultation with a speech pathologist will be needed if the individual is experiencing speech and swallowing problems (Honan, 2019).
Nursing Care and Implications
The nurse should work collaboratively with the affected individual to develop a plan of care that addresses the physiologic, social and psychological problems associated with MS.
Promoting Physical Mobility
Measures should be implemented to improve mobility. Progressive resistive exercises help to strengthen weakened muscles. Walking can help to improve gait and proprioception of the legs and feet. Because muscle spasticity may interfere with normal function, an orthotic may be prescribed to maintain normal functional position and help to reduce the risk for contracture. Activities that help to decrease spasticity include swimming and stationary bicycling along with progressive weight bearing exercises. Individuals should exercise to just before the point of fatigue because fatigue can aggravate symptoms. If ataxia is present, this patient is at increased risk for falls, so individuals should be taught to walk with the feet apart to widen the base of support which helps to improve stability. If the individual experiences loss of proprioception, watching the feet while walking may help. Assistive devices (walker, cane, or braces) may be needed to facilitate walking and training with a physical therapist in the proper use of these devices is important. If gait is not stable enough to ensure safe mobility, a wheelchair or motorized scooter may be needed (Hinkle & Cheever, 2018).
Along with immobility comes the risk for complications, such as pressure ulcers and deconditioning of the muscles of respiration. Skin assessments should be performed frequently especially for those who are wheelchair bound. Deep breathing and coughing exercises help to prevent pooling of bronchial secretions (Hinkle & Cheever, 2018).
Perimenopausal women with MS are at increased risk for osteoporosis not only as a result of estrogen loss, but due to decreased mobility and high-dose corticosteroid therapy during exacerbations (Hinkle & Cheever, 2018).
Activities of daily living (ADLs) should be arranged to include rest periods to preserve energy. Individuals should be encouraged to prioritize activities which helps to promote independence and control. More important activities should be performed in the morning when energy reserves are the greatest. Heat can exacerbate fatigue (for example, hot showers) so it should be avoided when possible. Pain can also exacerbate fatigue, so adequate pain management should be a priority (Bauldoff et al., 2020).
Enhancing Bladder and Bowel Control
The individual with urinary frequency or urgency as well as incontinence may benefit from specialized bladder training, called pelvic floor training or Kegel exercises. This includes immediate attendance to the urge to void (readily accessible bedpans or urinals) and creation of a voiding time schedule. Developing a voiding time schedule starts with short intervals of 1.5 to 2 hours where the individual drinks a measured amount of fluid and then attempts to void 30 minutes after drinking the fluid. The use of an alarm or timer may serve as a reminder to attempt to void if the individual does not have the natural urge to void. The time intervals should be gradually lengthened to increase tolerance. Individuals with urinary retention may perform self-catheterization as a method of achieving bladder control. Males may choose to use condom catheters if incontinence is a concern. Bowel problems may include constipation or incontinence. Constipation can be helped through adequate fluid and fiber intake and stool softeners (Hinkle & Cheever, 2018; Honan, 2019). A bowel retraining program, or sphincter training, may be beneficial for the individual with constipation or fecal incontinence.
Improving Visual Disturbances
If the individual is experiencing diplopia, an eyepatch or a covered eyeglass lens can be used to block the visual impulse of one eye so that only one image is seen. If the individual is confined to bed, prism glasses may assist the individual with reading while in the supine position. Also, large-print and audio books may be obtained from local libraries (Hinkle & Cheever, 2018).
Improving Cognitive Function
Some individuals with MS may become forgetful, easily distracted and emotionally labile. A structured environment with minimal distractions and a consistent use of lists and other memory aids may help the individual with MS to maintain a daily routine. Additionally, adaptation to illness occurs in a variety of ways including denial, depression, withdrawal and displays of hostility to others. Family members should be advised that these responses may occur and be as supportive as possible. Unfortunately, MS causes disruption not only in the life of the affected individual but in the lives of family members as well (Hinkle & Cheever, 2018).
Promoting Sexual Function
Individuals may benefit from meeting with a sexual counselor to explore other ways of sharing intimacy and to also be educated on other available options depending on the type of dysfunction experienced (Hinkle & Cheever, 2018). Factors such as bowel and bladder incontinence may also inhibit sexual desire (Honan, 2019).
Males experiencing erectile dysfunction may benefit from oral medications such as sildenafil citrate (Viagra), tadalafil (Cialis), or vardenafil hydrochloride (Levitra). Other options to obtain an erection include injectable medications, such as papaverine or prostaglandin E and mechanical devices (Bauldoff et al., 2020).
Females experiencing vaginal dryness may benefit from use of water-soluble lubricants (Bauldoff et al., 2020).
Strengthening Coping Mechanisms
Because MS typically appears in individuals in the early stage of life, career and family responsibilities are often affected. It is important to stress that no two individuals with MS will have identical symptoms or course of disease. While some experience significant disability early after the onset of the disease, others have a near-normal lifespan with minimal disability. Role function within a family may change with others taking on the previous role of the affected individual as well as the role of caregiver. The role of the nurse includes supporting the affected individual and family members in efforts to reduce stress and cope with the chronicity of the disease. These include referrals to home health services, individual and family counseling, and local chapters of the National MS Society (Hinkle & Cheever, 2018).
Future Research and Trends
Interestingly, women who are pregnant typically do not experience relapses during pregnancy. This suggests that further research needs to be done on the protective effects of hormones (Honan, 2019). The long-term adverse effects of the newer oral immunomodulators need further study and comparison to each other with respect to effectiveness and severity of adverse effects.
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Wilson, B., Shannon, M. & Shields, K. (2017). Pearson Nurse’s Drug Guide. Hoboken, NJ: Pearson Education, Inc.