Initiating care of a patient with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS), by Charles W. Lapp in Front. Pediatr., [Published 23 January 2019]
This paper introduces the primary care physician to the unique and challenging aspects of initially diagnosing and managing a complex condition for which there are a plethora of symptoms, few physical findings, no known cause, and no specific treatments. While daunting, the rewards are many, and those who pursue an interest in ME/CFS find themselves at the forefront of medicine.
The approach to any complex problem is to break it down into small steps, and ME/CFS is no exception. The first office visit should be devoted to a history of the present illness, a physical examination, and collection of exclusionary laboratory tests. On follow-up the differential diagnosis and a treatment plan can be addressed. Many individuals with ME/CFS have been humiliated or dismissed by other providers, so one will need to be as non-judgmental as possible and acknowledge that ME/CFS is not a psychological condition but a real illness. They need reassurance that you will work with them to seek a unifying diagnosis and prioritize management.
Many patients will be seeking rapid relief and even a cure for their illness, but foremost they must have realistic expectations: ME/CFS is a chronic illness for which there is currently no known cure. Nevertheless, there are many treatments that can be helpful to reduce symptoms and improve functionality.
Most experts would agree (9) that it is most important to address exertion intolerance and post-exertional malaise first; then sleep and pain, followed by cognition and the co-morbidities. Experts will agree that patients must avoid over-exerting and then flaring or relapsing—referred to as “pushing and crashing”—which clearly exacerbates the illness and hinders improvement. The controversy surrounds how to best prevent that.
One technique is interval activity or time-based activity. If an individual knows that they can be active for a period of time without triggering symptoms—say 15 min—then he or she can shop or work for 15 min, take a break, then shop or work for another 15 min, and so on. Over time, the activity interval can be increased (10).
Another technique is to monitor steps per day by wearing a step meter or pedometer (11). It is important for patients to take at least 1,000 steps per day in order to avoid deconditioning; but patients are encouraged to calculate their average steps per day during a good week with no flares or relapses. This is typically about 2,500–3,500 steps per day. They are then encouraged to not exceed that number of steps. So if a patient went shopping or sightseeing one day and reached her average limit of 3,500 steps, she would know to quit and rest as soon as possible to avoid a flare or relapse.
Scientific evidence is mounting that patients should not exceed their Anaerobic Threshold, an activity level at which the heart and lungs cannot supply enough oxygen to the mitochondria. In the absence of oxygen, glucose metabolism is much less efficient and produces lactic acid and other toxins that seem detrimental to our patients. The Anaerobic Threshold is usually determined by specialized exercise testing, but is related to one’s heart rate. So if a patient can monitor heart rate, he or she can estimate the maximum heart rate (frequently under 110 in adults) that can be tolerated without triggering a flare. Then avoid exceeding that heart rate except for short periods (12).
In short, it is very important to balance any activity with generous amounts of rest. So the patient should be encouraged to remain active, but not so active as to trigger flares and relapses.
Sleep is the next most important area to address. Start with typical sleep hygiene principles. Patients may consider over-the-counter sleep aids such as melatonin, theanine, valerian, tryptophan, antihistamines (diphenhydramine, doxylamine), or proprietary sleep aids. Low dose tricyclic or tetracyclic antidepressants, cyclobenzaprine, or low dose tizanidine are frequently prescribed to maintain sleep. If necessary, consider prescribing the usual benzodiazepine -based sleep medications to initiate sleep. Between 18 and 62% of persons with ME/CFS have primary sleep disorders, so highly consider referral to a sleep specialist if a sleep disorder is suspected (13).
Pain is another major symptom to address as it may affect sleep, mood, mobility and other domains. First identify the sources of pain: Fibromyalgia? Myofascial pain? Headache? Arthralgia? Inflammatory joint pain? Then assess the patient’s need for pain intervention. Will non-pharmacologic therapy suffice such as hot packs, cold packs, liniments, baths or showers, massage, chiropractic, acupuncture, or TENS? If pharmacologic therapy is indicated, have non-opioid therapies been tried such as Cymbalta/duloxetine, Savella/milnacipran, or Lyrica/pregabalin? (7) In the last 10 years Low Dose Naltrexone has become a primary consideration in opioid-naïve individuals (14). If opioid medications are indicated, tramadol has been very effective, but many providers would be most comfortable referring to a pain specialist for anything more potent. In the case of migraine or rheumatic pain, specialists might also be indicated.
Cognitive problems tend to wax and wane, much as fatigue does. Patients need to be reassured that they are not developing Alzheimer’s or dementia, and there is no evidence that such cognitive losses are permanent. While medication has helped little to improve cognition, the provider can suggest helpful techniques such as:
- Keep a calendar, notebook and calculator at hand.
- Always carry a cell phone to call for assistance, use as a GPS, or photograph your location in a parking lot or unfamiliar area.
- Develop the habit of always putting up important items such as keys, purses, wallets, and glasses in the same place.
- Plan important tasks to be done during the “best time of your day.”
- Avoid chaotic, stressful, or multisensory situations or events.
Autonomic, (auto)immune, (neuro)endocrine, psychological and co-morbid issues are managed as you would normally in your medical practice. It is imperative to address co-morbidities because they confound the ME/CFS. Consultants may be required. It is very important not to attribute all new symptoms to ME/CFS alone. Lastly, patients must maintain adequate hydration and nutrition although they tend to neglect these areas due to fatigue.