DRG Category: 947
Mean LOS: 4.8 days
Description: Medical: Signs and Symptoms With Major Complication or Comorbidity
Chronic fatigue syndrome (CFS, also known as myalgic encephalomyelitis [ME]) is a unique, controversial, and poorly understood chronic disease that has a sudden onset and lasts for at least 6 months. Some experts recommend that the condition be termed systemic exertion intolerance disease (SEID) to reflect the cardinal sign, postexertional malaise. It is a multiple symptom disease that affects the immune and neurological systems and suggests chronic mononucleosis. The pathophysiology of the syndrome is unclear, but the immune system is upregulated, or stimulated, and the levels of antibodies, especially immunoglobulin G, are increased. Experts suggest that an estimated 800,000 to 2.5 million people have CFS in the United States, but most (up to 90%) have not been diagnosed with the condition.
CFS has been mentioned throughout history but only recently has been defined for adults as a distinct disorder. It is characterized by debilitation, chronic fatigue, and a duration of at least 6 months and often much longer; it causes impaired overall physical and mental functioning. The fatigue is not the result of excessive exertion and is not alleviated by rest. People note worsening of symptoms after mental, emotional, or physical exertion. The Centers for Disease Control and Prevention (CDC) criteria have been formulated to standardize diagnosis and include cognitive difficulties, pharyngitis, lymphadenopathy, muscle pain, joint pain, headache, sleep disturbance, poor sleep, and postexercise malaise. Like many chronic illnesses, CFS is often accompanied by depression. People with CFS generally improve over time, although remissions and relapses are common.
CFS is different from but related to fibromyalgia, a condition that generally occurs in young or middle-aged females and is manifested by pain, stiffness, fatigue, disrupted sleep, and problems with cognition. As compared to the development of CFS, fibromyalgia is related to genetic vulnerability and abuse or other traumatic experiences that occur during brain development (childhood) and persistent stress or distress.
The cause of CFS is unknown but is possibly related to an infectious process such as Epstein-Barr viral infection with immune manifestations. Researchers are investigating whether or not the disease is a syndrome triggered by a virus with multiple contributing factors such as age, sex, toxic and environmental exposure, stress, depression, anxiety, and perhaps a precipitating event (recent trauma or surgery).
CFS appears to have both a genetic and an environmental component, with heritability estimated at 20% to 50%. Several studies have found genetic variants that may influence the development of CFS, but more work is needed to define the pathways that contribute to disease. Several genes have been implicated, including the glutamate receptor ionotropic kinase 2 (GRIK2) and neuronal PAS domain protein 2 (NPAS2). Additionally, an association has been made with HLA-C and HLA-DQB1 alleles, linking the immune system to CFS pathogenesis.
CFS is more prevalent in females than in males, and it tends to affect persons between the ages of 40 and 60 years. It is more common in adolescents than children. It may be underdiagnosed in children and older adults.
Most people who have been diagnosed with CFS are White persons, but experts suggest that the condition is at least as common among Black and Hispanic persons but is underdiagnosed. While little is known about the numbers of gender and sexual minority persons who have CFS, these individuals are at risk because of issues with healthcare access, the stress of discrimination and violence, and higher odds for depression, multiple chronic conditions, and substance use (Downing & Przedworski, 2018).
CFS exists worldwide, but the prevalence is uncertain because of the lack of consistent definitions. In the United States, up to 4 million people may have CFS, but more recent statistics indicate that as much as 1% to 2% of the population is affected. Experts believe that this prevalence statistic is likely also applicable to global populations.
ASSESSMENT
History
Establish a history of the sudden onset of flu-like symptoms accompanied by intense, postexertional fatigue that does not resolve within 6 months. Approximately 25% of patients will acknowledge that they have been bed- or housebound because of symptoms. Ask if the patient has experienced sleep disturbance or fatigue after long periods of rest or sleep. Determine if the patient has experienced any other symptoms of a neurological or psychological nature such as problems with short-term memory or fluency of speech. Ask if the patient has been exposed to a toxin or has recently experienced stress. Determine if the patient's occupation involves interaction with the public leading to viral or environmental exposure. It is important to remember that symptoms can vary widely with CFS.
The most common symptoms include prolonged fatigue, difficulties with short-term memory, and verbal dyslexia (difficulty speaking accurately and fluently, problems with verbal memory and verbal processing speed). The physical examination may reveal flu-like symptoms such as sore throat, low-grade fever, chills, muscular pain, and swollen, painful lymph nodes. Neurological assessment findings may include sensitivity to light, headache, inability to think clearly or concentrate, short-term memory loss, sleep disorders, equilibrium problems, and depression. Some patients have orthostatic intolerance with symptoms that worsen when they stand upright.
Psychosocial
Patients with CFS are often depressed because of the stress of dealing with a chronic, debilitating illness that affects their total lifestyle and cognition. Anxiety and mood swings are common, and there are increased rates of divorce and suicide among these patients. Assess the effect of the disease on the patient's job and childcare responsibilities.
There is no definitive method of diagnosing CFS, but the National Academy of Medicine (formerly the Institute of Medicine) has set forth diagnostic criteria (Box 1). The following diagnostic tests are often drawn: complete blood count, serum electrolytes, liver function tests, thyroid function tests, erythrocyte sedimentation rate, C-reactive protein, creatine kinase, cortisol level, and antinuclear antibody test.
Diagnosis requires that the patient have the following three symptoms:
At least one of the following two manifestations is also required:
Note that the frequency and severity of symptoms should be assessed. The diagnosis of CFS should be questioned if patients do not have these symptoms at least half of the time with moderate, substantial, or severe intensity.
Diagnosis
DiagnosisActivity intolerance related to inflammation as evidenced by muscle and/or joint pain, malaise, and/or fatigue
Outcomes
OutcomesEnergy conservation; Coping; Knowledge: Disease process; Mood equilibrium; Symptom severity
PLANNING AND IMPLEMENTATION
Because there is currently no known cure, treatment of CFS focuses on symptom relief. Some patients experience relief of the symptoms by avoiding environmental irritants and certain foods. Exercise therapy has been shown to improve sleep, physical function, and general health as well as to help people feel less fatigued. Current research has not shown that exercise therapy improves pain, quality of life, anxiety, or depression.
Pharmacologic Highlights
Medication or Drug Class | Dosage | Description | Rationale |
---|---|---|---|
NSAIDs | Varies by drug | May reduce inflammation, thus reducing symptoms | Efficacy uncertain |
Other Medications: Most medications have proved ineffective. Nonsedating antihistamines, antianxiety agents such as alprazolam, and tricyclic antidepressants may be helpful. Experimental treatments include the antiviral acyclovir and selected immunomodulating agents, such as IV gamma globulin, rintatolimod (Ampligen), transfer factor, and others. Doxycycline is used to treat people with elevated immunoglobulin M C pneumoniae titers.
It is important to set realistic goals when planning care with the patient with CFS. Teach patients not to overexert themselves. It is believed that stress can prolong the disease or result in an exacerbation. Relaxation and stress-reducing techniques such as hypnosis, massage, biofeedback, and meditation may be useful if sleep patterns are altered. Explain that although the symptoms tend to wax and wane, they are often debilitating and may last for months or even years. The patient therefore needs to reduce their activities when symptoms are more pronounced but also needs to avoid bedrest, which has no proven therapeutic value for patients with CFS. Encourage a graded exercise program and provide an appropriate referral for continuing exercise. Stress the need to progress slowly with exercise to avoid overfatigue. Referring the patient and family to counseling and support groups may assist in developing appropriate coping skills for dealing with a chronic, debilitating illness.
Evidence-Based Practice and Health Policy
Larun, L., Brurberg, K., Odgaard-Jensen, J., & Price, J. (2019). Exercise therapy for chronic fatigue syndrome. Cochrane Database of Systematic Reviews. https://doi.org//10.1002/14651858.CD003200.pub8
Instruct the patient to report any increase in physical symptoms or suicidal thoughts to the primary caregiver. Instruct the patient to obtain assistance as necessary to complete self-care activities and to meet family responsibilities. Teach the patient the proper route, dosage, and side effects to monitor with all medications. Make necessary plans for referrals and follow-up appointments.
Discuss the role of exercise therapy to improve some symptoms.