Chronic fatigue in Ehlers–Danlos syndrome—Hypermobile type – Hakim – 2017 – American Journal of Medical Genetics Part C: Seminars in Medical Genetics – Wiley Online Library
It is the authors’ opinion that the criteria used for diagnosing CFS and hEDS are inadequate and contribute to diagnostic confusion.
To meet a diagnosis of CFS, fatigue must be “unexplained by other conditions.”
Therefore, a diagnosis of hEDS must exclude a diagnosis of CFS.
However, hEDS is likely to be substantially under-diagnosed and it is likely that some patients diagnosed with CFS may meet or would previously have met the criteria for diagnosis of hEDS.
Also it is the authors’ opinion that the literature and diagnostic methods for CFS and hEDS are of insufficient strength to reliably differentiate between these conditions in a given individual. We would conclude that in the context of hEDS one would simply use the term chronic fatigue.
To facilitate effective management the clinician needs to establish a collaborative relationship with the patient and their carers
The patient and their clinician should share decision making both in identifying the causes of, recognising the impact of, and the phases in the management of fatigue.
Together this might include:
- Understanding the need to exclude underlying diseases and disorders.
- Recognizing the reality and impact (physical, emotional, social [including education and employment]) of the condition and the symptoms.
- Setting realistic goals and timelines for improvement, being prepared to manage set-backs/relapses.
- Exploring the range of interventions and management strategies available, taking account of the patients’ age (particularly for children), the severity of their symptoms, their preferences and experiences, and the outcome of previous treatment.
- Negotiating other areas of healthcare provision, supporting applications for financial benefits and social care, as well as concerns related to education or employment.
People with severe fatigue may need support from a multidisciplinary team for example, nursing, occupational therapy, dietetics, psychology, physiotherapy, and pain management.
This should be coordinated by a named healthcare professional, and usually their general practitioner/general physician.
Treatment is based on addressing the underlying issues.
These might include
medications directed at orthostatic intolerance,
management of allergies,
use of sleep aids, and
pain management [Castori et al., 2012],
as well as a lifestyle changes including pacing, changing sleep pattern, exercise, and even a change of job or hours of work.
There is no known pharmacological treatment or cure for fatigue per se.
Large systematic reviews have not identified consistently effective medications for CFS symptoms in general, but many medications are effective for specific symptoms (e.g., headaches) and co-morbid conditions that result in fatigue [Smith et al., 2014].
Unless there is an underlying medical disorder, the following medications should be avoided as they may cause harm if used inappropriately:
- Glucocorticoids (in the absence of other indications),
- thyroxine (in the absence of hypothyroidism),
- antiviral agents2 (in the absence of confirmed active viral infection).
There is insufficient evidence to recommend the use of complementary therapies and supplements for fatigue. However, some patients choose to use these therapies and find them helpful, and there is little evidence of harm.
Typical agents include
- co-enzyme Q10,
- alpha-lipoic acid,
- nicotinamide adenine dinucleotide (NADH), and
- multivitamins and minerals.
Co-enzyme Q and Riboflavin have been shown to be effective in migraine prophylaxis.
Clinicians and patients should remain aware that some patients do not show meaningful responses to therapy.
Patients with intractable, chronic and disabling conditions are prone to feelings of abandonment and may be vulnerable to potentially toxic therapies or exploitative practitioners.
This is the absolute truth. Patients in serious constant pain must face doctors prescribing a series of ineffective drugs, treatments, and therapies instead of effective opioid medication.
Our government, through the CDC, has effectively abandoned pain patients, taking away effective treatment and providing no alternatives.
Instead, we are questioned and suspected and stigmatized and rejected by our doctors, who value preventing addiction much more than treating pain.
For people with moderate or severe fatigue that has not responded to treatment, equipment and adaptations (e.g., a wheelchair) should be considered as part of the management plan
Disruption of education or employment is generally detrimental to health and wellbeing. The ability to continue in these should be addressed early.
This is exactly what does NOT happen.
Sleep management, rest, and relaxation and key approaches. Advice on sleep management includes:
- Explaining the effect disordered sleep or a poor sleep pattern can have on causing and exacerbating fatigue can help an individual understand how their behaviors might affect the normal day–night sleep cycle.
- Identifying poor sleep patterns such as insomnia, hypersomnia, an altered sleep–wake cycle, and non-refreshing sleep despite either an apparent normal cycle or prolonged sleep.
- Providing general advice on what good sleep hygiene means (see below).
- Introducing changes to sleep patterns gradually.
Good sleep hygiene includes:
- Avoiding stimulants such as caffeine or nicotine too close to bedtime.
- Exercise during the day to promote good sleep.
- Avoiding large meals too close to bedtime.
- Relaxation techniques—establishing a regular relaxing bedtime routine, avoiding emotional upset/dwelling on problems before trying to go to sleep. Calm music or reading may be relaxing for some.
- Associate bed with sleep. Avoid watching TV, playing computer games, sending text messages, e-mails, etc. Calm music or reading may be relaxing for some.
- The sleep environment should be pleasant and relaxing. The bed should be comfortable, and the room dark, quiet, and neither too hot or too cold.
During the day, exercise may also encourage wakefulness, as should adequate exposure to natural light. Especially early in the day, light exposure helps to maintain a healthy sleep–wake cycle.
Chronic pain may be a dominant influence on sleep; it should be managed accordingly. Prescribing of low-dose tricyclic antidepressants, specifically amitriptyline, should be considered.
Other medications may assist sleep. These include:
- a variety of others including benzodiazepines, beta blockers, muscle relaxants, and eszopiclone.
As part of pacing, rest periods may be required. Rest periods can be introduced into daily routine, but the frequency, length, and types of activities undertaken should be adapted for each individual. It is important to maintain as normal a level of activity as possible, whilst avoiding over-exertion
Rest advice might include:
- Limiting the length of rest period to, say, 30 min.
- Undertaking low-level physical (arts, crafts, etc.) and cognitive (reading, puzzles, etc.) activities depending on the severity of symptoms.
- Using relaxation techniques.
- Trying to avoid complete rest as the only management during a setback/relapse.
Prolonged bed rest should be avoided if possible. It is associated with significant physical deconditioning, psychological risks, and medical disorders including severe postural hypotension, venous thrombosis, osteoporosis, and pressure sores
Common relaxation techniques include:
- Visualization. For example the individual may imagine a peaceful setting and then focus on controlled, relaxing breathing, slowing the heart rate.
- Other techniques include:
- music and/or art therapy.
Graded exercise therapy (GET) and management of daily activities are also fundamental therapeutic approaches.
Before advising on exercise the clinician should consider the impact of joint hypermobility and joint instability in EDS, as well as the negative influence on exercise that arises from uncontrolled pain, fear of movement (kinesiophobia), and other associated conditions such as cardiovascular autonomic dysfunction
The main objectives in EDS of an exercise programme are the progressive prevention of physical deterioration, optimization of functional capacity without triggering injury, and pain control.
I see so many recommendations for exercise as a cure for anything that ails you physically, yet I and several others I know with EDS were stricken with disabling pain while we were “exercising” in various sports.
All our years of strenous physical activity, such as what the “experts” recommend for pain, did NOT save us from developing chronic pain.
Recommendations such as “go to the gym,” “exercise more,” or “go swimming” are not helpful without supported advice on what this actually means, and should be avoided.
It is recommended that exercise should be supervised, structured, and gradually increased in intensity (both in muscle strengthening and aerobic fitness as appropriate).
Unstructured and unsupervised exercise may worsen symptoms, as can a rigid or inflexible escalation of activity
When planning GET, it is important clinicians and/or therapists:
- Undertake an assessment of current activity analysis—while also ensuring that this does not of itself already lead to a “boom and bust” cycle.
- Discuss both short and long-term goals important and relevant to the individual.
- Agree on a level of additional low-intensity exercise that is sustainable
- Recognize that it can take weeks, months, or even years to achieve goals, and ensure that this is taken into account in the therapy structure and the ways services deliver treatment.
- Advise that increased levels of exercise may increase symptoms for a few days (e.g., stiffness and fatigue), but explain that this is normal.
Treatment of existing movement restrictions and biomechanical dysfunction using manual techniques can be a bridge to tolerating exercise for some individuals.
Activity management is a form of pacing, controlling activities by means that include:
- Planning daily activities to allow for a balance and variety of different types of activity, rest, and sleep.
- Spreading out difficult or demanding tasks over the day or week.
- Splitting activities into small achievable tasks.
- Monitoring, regulating, and planning activities to avoid a “boom and bust” cycle.
- Goal setting, planning, and prioritising activities.
Cognitive Behavioral Therapy (CBT) has both formal and informal approaches.
At an informal level, this involves education about symptoms, demystification of the medical problems, and explanation of how inactivity can aggravate a number of the problems that contribute to fatigue (e.g., deconditioning, orthostatic intolerance).
Yet running half-marathons and bicycling 100 miles/day did NOT prevent me from developing chronic pain.
None of these experts can explain why athletes develop the pain that is supposed to be ameliorated by exercise.
An individualized, person-centred programme should be offered to people with fatigue.
The objectives of the programme should be to:
- Sustain or gradually extend, the person’s physical, emotional, and cognitive capacity.
- Manage the physical and emotional impact of their symptoms.
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