Multiple sclerosis (MS) is a chronic, demyelinating disease affecting approximately 400,000 people in the United States alone. This article provides evidence-based practice tools and explores care needed by the small and challenging number of patients whose MS progresses, leading to increasing disability and life-limiting situations. MS is a unique and extremely variable disease, often manifesting "invisible" disabilities and symptoms. Nurses and other clinicians need to know of the nuances of MS to provide the care and resources available to this group.
Tara: A Patient Case Conference
Tara is a 42-year-old woman who has been living with progressive multiple sclerosis (MS) for a decade. Formerly a high school teacher, Tara has had a fruitful and full career. Tara has been slowing down for 2 years and rapidly declining for the past 6 months. She has not been able to teach full time for 5 years.
Diagnosed with relapsing–remitting multiple sclerosis (RRMS), she was initially treated with a disease-modifying therapy that is proving to be no longer effective for her. Because Tara can no longer get to her MS doctor on a regular basis, her symptoms are not being adequately managed. Fortunately, a friend in her apartment building checks on her and helps her to make doctor's appointments with her urologist for her frequent urinary tract infections and her speech/language therapist who is using breathing exercises to help her to speak more effectively. With her difficulty ambulating, Tara tends to sit for long periods and has developed pressure sores.
Tara states that she lacks energy and a zest for life. She suspects she is depressed and has difficulty sleeping. Not surprisingly, thinking about the future is frightening for her. Tara feels "cheated" by not having had children, but at the same time knows that she would probably be leaving them prematurely by an earlier than average death. She has designated her cousin her healthcare proxy and made known her funeral requests. Unable to drive or walk on her own, Tara spends much of her time in her apartment, and sometimes neglects to change her underwear and bedclothes since "it takes so much energy and no one knows anyway."
Tara's former social circle has tried to keep her in the loop but she has lost her frame of reference—the classroom—by being at home. One time when her former "group" took her to lunch in a nearby restaurant, Tara had an incontinence episode and has since refused subsequent invitations.
For Tara, worsening symptoms, coupled with diminished social contacts and emotional distress, demand palliative care initiatives.
MS is a chronic, demyelinating disease that affects the central nervous system (CNS). The progress, severity, degree of disability, and specific symptoms of MS are unpredictable and may vary greatly from one day to another and from one person to another.
Although believed to be an autoimmune disease, the cause of MS is unknown (Lode et al., 2007). A combination of immunologic, epidemiologic, and genetic factors are thought to contribute to the etiology of MS. Various infectious triggers have been studied, but no clear culprit has been identified. The myelin that surrounds and insulates the nerve fibers in the CNS is the target of the autoimmune attack in MS. The loss of myelin and nerve fibers results in the variety of symptoms that can occur (Randall & Schneider in Halper & Holland, 2002).
MS typically strikes young adults as they begin families and careers. Although young children and older adults can also develop MS, the diagnosis is most commonly made between the ages of 20 and 50. It is estimated that at least 400,000 Americans are living with MS. Women outnumber men by a ratio of at least 2–3:1. MS occurs most commonly in temperate areas that are further from the equator. It is most common in whites of northern European ancestry, but can occur in African Americans and Hispanics/Latinos as well. The disease is relatively rare in Asians (Kantarci & Wingerchuk, 2006).
The symptoms of MS are many and varied. According to the National Multiple Sclerosis Society (NMSS, 2009) the most common symptoms of MS include the following:
- Fatigue is the most common symptom of MS and reported by many to be their most disabling symptom.
- Visual problems, a common first symptom of MS, can include optic neuritis (temporary or permanent blurred vision, pain behind the eye, distortion of color). Other problems: diplopia, nystagmus.
- Sensory changes including numbness, tingling, itching, and pain. The pain in MS is of two general types—primary, neurogenic pain caused by damage in the CNS and secondary, nociceptive pain resulting from orthopedic or other causes.
- Bladder (urgency, frequency, nocturia, incontinence, often accompanied by frequent urinary tract infections) and bowel dysfunction (primarily constipation) that can contribute to social isolation, morbidity, and mortality in MS.
- Mobility issues caused by weakness, spasticity, imbalance, lack of coordination.
- Cognitive dysfunction, which along with fatigue, is the primary reason people with MS leave the workforce prematurely.
- Mood changes, including depression, anxiety, and mood swings, are more common in MS than in other chronic illnesses and are thought to be a symptom of the disease as well as a reaction to it (http://www.nationalMSsociety.org/about-multiple-sclerosis/symptoms/index.aspx).
Less common symptoms of MS include the following:
- dysarthria and dysphonia
The Clinical Courses of MS
People with MS typically experience one of four disease courses, each of which might be mild, moderate, or severe as listed below (Lublin & Reingold, 1996).
- –Clearly defined attacks of worsening neurologic function (relapses, flare-ups, exacerbations) followed by remissions, during which no apparent disease progression occurs
- –About 85% of people are initially diagnosed with RRMS
- Primary-Progressive MS (PPMS)
- –Slowly worsening neurologic function from the beginning with no distinct relapses or remissions
- –Progression varies over time, with occasional plateaus and temporary minor improvements
- –About 10% of people have PPMS
- Secondary-Progressive MS
- –After an initial period of RRMS, a secondary-progressive disease course develops in many people. In this phase, the disease worsens steadily, with or without occasional flare-ups, minor remissions, or plateaus.
- Progressive-Relapsing MS
- –Relatively rare course of MS (5%)
- –Steadily worsening disease from the beginning, with clear attacks of worsening neurologic function
- –May/may not experience some recovery following relapses, but disease continues to progress without remissions.
Most people with MS are followed by a neurologist—ideally one with MS expertise. Comprehensive care of the MS patient requires that the neurologist work in collaboration with other clinicians including nurses and rehabilitation and mental health professionals. The patient is seen at intervals for evaluation and medical management, with referrals made, as necessary, to the other specialists. Assistive and adaptive equipment can be recommended. The medical, psychological, and social complexities of this disease call for this kind of interdisciplinary approach.
Currently there is no cure for MS. Treatment strategies fall into the following categories:
- Modifying the disease course—ongoing treatment with an immunomodulatory or immunosuppressive medication. The first-line therapies, all of which are delivered by injection or infusion, slow the disease process and lessen disability in relapsing forms of MS (Goodin et al., 2002; Hutchinson et al., 2009). These medications do not cure MS, nor do they treat the symptoms; they are, however, the best available intervention for use at present. Since 1993, the Federal Drug Administration (FDA) has approved seven medications for use in MS. Currently approximately 60% of people with MS are taking one of the approved disease-modifying therapies. No medication is yet available for PPMS. The most current information about the six current FDA-approved immunomodulating drugs and the one FDA-approved immunosuppressant drug that alter the function of the immune system can be found on the NMSS website at http://www.nationalMSsociety.org/Treatments.
- Because clinical trials are ongoing with newer medications for the treatment of MS in the pipeline (Cohen, 2009), it is important to check for updated information at http://www.nationalMSsociety.org/ClinicalTrials.
- Managing acute relapses—most often with high-dose, intravenous corticosteroids, with or without an oral taper.
- Managing symptoms with a combination of medication, rehabilitation interventions, and support to promote comfort and function while preventing unnecessary complications.
- Providing psychosocial support for patients and their families.
Most newly diagnosed patients will be prescribed one of the currently available disease-modifying drugs and followed on an outpatient basis with nursing and pharmacy support as needed. Various symptoms caused by MS, such as the very unique MS fatigue, will be handled by additional medication and other strategies such as environmental adaptations and mobility aids. The nurse must use observation, instruction and case management skills, and coordinate care accordingly.
During the course of MS, nurses need to be vigilant for signs of depression in the patient and make appropriate referrals to psychiatric home care nurses or social workers, psychologists, psychiatrists, or other mental healthcare providers per physician orders (use the Patient Health Questionnaire—PHQ9) (Kroenke et al., 2009).
It is generally very difficult to determine the prognosis for people with MS with any accuracy as the disease progression and symptoms are so variable. Most people with MS can expect a near normal life span (Ragonese et al., 2008), with death attributable to cancer, heart disease, and stroke as in the general population. Premature death in MS can result from disease complications such as overwhelming infection or suicide resulting from untreated depression. A very small subset of MS patients die from a rapidly progressive, fulminating form of MS that is fortunately very rare (Capello & Mancardi 2004).
Of those diagnosed with MS, approximately one-third will experience a mild, very manageable form of the disease; one-third will experience a moderate level of disability; and one-third will become severely disabled (Minden et al., 2006). In this last group are patients who progress beyond the ability to care for themselves, requiring home healthcare or some type of long-term care.
Caring for Those With Advanced MS
So what happens to the progressive MS patient who is no longer responding to disease-modifying medication and is becoming more disabled? Whose physician says that he or she has nothing more to offer? Who can no longer make the arduous trip to the doctor's office? Whose disease progression seems relentless? This is the time when many clients fall off the clinicians' radar screen. Moreover, although they may not yet be terminally ill, they may miss receiving many of the services that would make life more comfortable. It is at this very time in their disease that more oversight and assistance are needed and more referrals processed. Palliative care is a good option at this stage because it promotes comfort care and is interdisciplinary.
Before 1993, when the first disease-modifying therapy was approved for MS, the "Diagnose and Adios" model of care described by Dr. Labe Scheinberg, a neurologist dedicated to caring for people with MS, was predominant (Kalb, 2006). Today, the client with more advanced disease—for whom the disease-modifying therapies are no longer effective—is apt to see the results of the unspoken but implied, "Off your med, go to bed." As people are no longer seen regularly in the clinic, they may be deprived of the MS team relationship, as well as referrals to other important and needed resources. Without this care, they may gradually lose their ability to participate in the world around them.
For a person with advancing MS, many care factors need to be considered, not least of which are the patient's personal preferences for care. Choices range from being cared for at home by family members, or by paid assistants. The person may best be served by participating in an adult day care program or by moving into an assisted living facility. Further along on the continuum is the long-term-care facility. However, the MS client, who is generally younger and has a family, is likely to have unmet needs in a traditional nursing home that cares primarily for older adults. Fortunately, several facilities around the country now address the special needs of the younger person with MS, which range from the need to have meaningful work to establishing a social network of peers. Being "different" or needing to rely on mobility aids, for example, often results in anger or resentment. Relationship needs of younger patients involve sexual intimacy and parenthood.
Palliative Care Overview
The clinician—most likely the home healthcare nurse—is in a unique position to map disease progression, make ongoing assessments, and initiate palliative care strategies. However, it is imperative to set in motion the components of palliative care before contact with the patient is lost to follow-up.
Palliative care relieves the pain and other symptoms patients suffer due to serious illness. The goals of palliative care include reducing suffering (physical, emotional, and spiritual), improving quality of life, and supporting the ill person and his or her family during and after treatment. Indeed, the American Academy of Hospice and Palliative Medicine (AAHPM) states "The goal of palliative care is to prevent and relieve suffering and to support the best possible quality of life for patients and their families, regardless of the stage of the disease or the need for other therapies" (http://www.aahpm.org/about/index.html#Mission).
Palliative care should not be confused with end-of-life and hospice care, which are part of the palliative care continuum. Palliative care may be delivered for many years before death, whereas hospice care is generally provided to those with a life-limiting prognosis, and usually for less than 6 months.
According to the World Health Organization, palliative care:
- provides relief from pain and other distressing symptoms;
- affirms life and regards dying as a normal process;
- intends neither to hasten or to postpone death;
- integrates the psychological and spiritual aspects of patient care;
- offers a support system to help patients live as actively as possible until death;
- offers a support system to help the family cope during the patient's illness and in their own bereavement;
- uses a team approach to address the needs of patients and their families, including bereavement counseling, if indicated;
- will enhance quality of life and may also positively influence the course of illness;
- is applicable early in the course of illness, in conjunction with other therapies that are intended to prolong life, such as chemotherapy or radiation therapy, and includes those investigations needed to better understand and manage distressing clinical complications.
Triggers to Initiate Palliative Care
Knowing when to initiate palliative care depends on various factors. Information about the patient's status comes to the clinician from colleagues, the client, or the patient's family. A patient's actions or words may act as a trigger to a dialogue about palliative care, such as "I know I'm not going to get well," or "Please don't let me be a burden to my family."
In addition to verbal clues, clinicians are in a unique position to observe the following physical and social situations:
- The patient's mild or moderate, intermittent or permanent loss of cognitive or physical ability for independent self-care, acknowledged by the patient.
- Change in the patient's role, family structure, or potential care network.
- Unrelieved psychological, social, or spiritual distress (expressed or observed).
- Questions from the patient or family asking about the dying process in MS.
- Stressors related to lack of financial planning for the future.
- Pain associated with chronic or progressive disease process unrelieved after 48 hours.
- Complex symptoms such as fatigue, weakness, pain, tremor, dysphagia, loss of skin integrity, and dyspnea.
- Repeated urinary tract infections, aspiration pneumonia, or other infections leading to repeated hospitalization.
- Bowel incontinence.
- Depression and/or suicidal ideation (Brandis et al., 2008).
Assessing the Need for Palliative Care
The following functional ability or status scales may assist the clinician in determining the need for either palliative care or hospice services.
- Karnofsky scale (Yates et al., 1980):
The Karnofsky score runs from 100 to 0, where 100 is "perfect" health and 0 is death. The scale, originally devised for cancer patients, was named after Dr. David A. Karnofsky, who described the scale with Dr. Joseph H. Burchenal in 1949.
–100%—normal, no complaints, no signs of disease
–90%—capable of normal activity, few symptoms or signs of disease
–80%—normal activity with some difficulty, some symptoms or signs
–70%—caring for self, not capable of normal activity or work
–60%—requiring some help, can take care of most personal requirements
–50%—requires help often, requires frequent medical care
–40%—disabled, requires special care and help
–30%—severely disabled, hospital admission indicated but no risk of death
–20%—very ill, urgently requiring admission, requires supportive measures or treatment
–10%—moribund, rapidly progressive fatal disease processes
- Palliative Performance Scale (PPS):
The PPS (Anderson et al., 1996) is a modification of the Karnofsky Performance Scale intended for evaluating patients requiring hospice care.
- Adult Failure to Thrive Measures:
Clinicians can use several established Hospice Eligibility Guidelines to help the client qualify for palliative care. Although there is no specific MS Local Coverage Determination (LCD), the amyotrophic lateral sclerosis (ALS) LCD can be adapted to document eligibility. The Adult Failure to Thrive (AFTT) Guideline (Robertson & Montagnini, 2004) that has been used for older adults can be adapted to the chronically ill, for example, people with MS.
In chronically ill patients, failure to thrive describes a state of decline that may be caused by chronic concurrent diseases and functional impairments. Examples include weight loss, decreased appetite, poor nutrition, and immobility. Four syndromes are prevalent and predict adverse outcomes in patients with failure to thrive: impaired physical function, malnutrition, depression, and cognitive impairment (Robertson & Montagnini, 2004). All four syndromes can occur in the client with advanced MS. Initial assessments should include information on physical and psychological health, functional ability, socioenvironmental factors, and nutrition. A medication review should be completed to ensure that side effects or drug interactions are not a contributing factor to failure to thrive. The impact of existing chronic diseases should be assessed. Interventions should be directed toward easily treatable causes of failure to thrive, with the goal of maintaining or improving overall functional status. Physicians should recognize the diagnosis of failure to thrive as a key decision point in the care of a person with progressed MS. The diagnosis should prompt discussion of palliative or end-of-life care options to prevent needless interventions that may prolong suffering.
Interim Visit With Tara
Tara is now the focus of an interdisciplinary team, led by the home healthcare nurse and carefully selected experts in chronic neurological conditions. They include Tara's neurologist, who specializes in MS; a physical therapist; a urologist; a nurse aide; a psychiatric social worker; and a volunteer trained in performing some basic, low-risk amenities. The volunteer, Emma, also has a young daughter, who sometimes visits Tara with her mom.
Tara seems more at ease and states she is "feeling less nervous" about her situation. An antidepressant has been added to her medications, along with amantadine to help her deal with overwhelming fatigue. Each day she rests after lunch, usually with music playing. She is having less trouble speaking due to the speech/language pathologist's interventions and, with assistance, can talk to her neurologist or nurse at least once per week. These phone visits are regularly scheduled and Tara looks forward to them. A dietitian, called in by the nurse, has recommended Tara's favorite high-protein food to help prevent skin breakdown and suggested a daily pudding made with prunes and a whipped dessert mix to help with Tara's constipation.
As the Christmas holidays approached, Tara's psychiatric social worker tried to prepare Tara for some possibly very emotionally conflicting feelings as Tara expressed "this may be my last...."
FDA-Approved Disease-Modifying Agents
Avonex (interferon beta-1a)
Betaseron (interferon beta-1b)
Copaxone (glatiramer acetate)
Extavia (interferon beta-1b)
Rebif (interferon beta-1a)
- AARP (end of life, wills, estate planning, etc.): http://www.aarp.org/families/end_life
- American Academy of Hospice and Palliative Medicine: http://www.aahpm.org
- Karnofsky Performance Status Scale Definitions Rating (%) Criteria: http://www.hospicepatients.org/karnofsky.html
- National Association for Continence: http://www.nafc.org/bladder-bowel-health/neurological-disorders
- Palliative care nursing: http://www.palliatvecarenursing.net
- Promoting Excellence in End-of-Life Care: http://www.promotingexcellence.org
- Byock I. (1997). Dying Well: Peace and Possibilities at the End of Life. New York: Putman, Riverhead.
- Kalb, R. (Ed.). (2006). Multiple Sclerosis: a Guide for Families (3rd ed.). New York: Demos Medical Publishing, New York.
- Kalb R. (Ed.). (2008). Multiple Sclerosis: The Questions You Have, the Answers You Need (4th ed.). New York: Demos Medical Publishing.
- Kalb R, Holland NJ, Giesser B. (2007). Multiple Sclerosis for Dummies. Hoboken, NJ: Wiley Publishing.
- Nuland S. (1993). How We Die: Reflections on Life's Final Chapter. New York: Vintage.
- Holland NJ, Northrop DE . (2006).Young Adults with Multiple Sclerosis: Management in the Home. Home Health Care Management and Practice, 18(3):186–195.
- Northrop DE, Frankel D. eds. Caring for Loved Ones with Advanced MS: A Guide for Families National Multiple Sclerosis Society, 2007.
- Resource Guide for Clinicians. National Multiple Sclerosis Society, 2009
Long-Term Care (book series)
- Assisted Living for Individuals with MS: Guidelines and Recommendations (2004).
- Nursing Home Care of Individuals with Multiple Sclerosis: Guidelines and Recommendations for Quality Care (2009).
- Serving Individuals with MS in the Home: Guidelines and Recommendations (2006).
- Serving Individuals with MS in Adult Day Programs: Guidelines and Recommendations (2005).
Talking with Your MS Patients about Difficult Topics (booklet series)
- Brandis M, Reitman NC. (2009). Talking about Palliative Care, Hospice and Dying.
- Goodman D. (2009). Talking about Primary –Progressive MS.
- Miller A. (2009). Talking about progressive Disease.
- Brandis M, Reitman NC MA, Gruenewald D, Del Bene M. (2008). Opening Doors: The Palliative Care Continuum in Multiple Sclerosis.
- Maloni H. (2008). Pain in Multiple Sclerosis.
THE NATIONAL MULTIPLE SCLEROSIS SOCIETY
Clinicians have a valuable resource in the National Multiple Sclerosis Society. Contact the Professional Resource Center (PRC) for information, clinical consultations, and library services by emailing [email protected]. Chapters of the National Multiple Sclerosis Society (1-800-344-4867) can provide information about local hospice and palliative care programs, names of mental health professionals, support groups, respite programs and educational material. Encourage your clients to make chapter contacts.
The National MS Society has created educational materials on a wide range of topics. Your patients can obtain these and other materials free of charge from their local chapter or in the Multimedia Library section of the website at http://www.nationalMSsociety.org/library.
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