Health Professional & Carers Guide to Severe ME/CFS
ME/CFS affects the immune system, endocrine system, brain, spinal cord, peripheral nerves, muscles, joints, gastro-intestinal tract, heart, lungs, reproductive system, liver and all other organs, tissues, cells and exocrine glands. There is presently no known cause, although the novel retrovirus XMRV is being investigated (J. Mikovits, V. Lombardi et al., 2009). There is also no known allopathic or naturopathic cure, though various modalities have been successful at alleviating some symptoms. Pacing of activities, proper nutrition, adequate sleep, resting and meditation are all musts (Bested, 2008). So is a healing environment.
Right down to all energy producing cells, there is an enormous dysfunction. In people with CFS (PWCs), there is the inability to produce adequate mitochondrial energy (S. Myhill, N.E. Booth, J. McLaren-Howard, 2009).
There are also significant neurological symptoms including, but not limited to: overload phenomena; seizures; extreme weakness; paralysis;difficulties processing information; sleep disturbances; cognitive dysfuntion; inability to multi-task; hyper-responsiveness to sound, smell, touch, taste, visual information and movement; loss of adaptability and loss of cognitive maps (Canadian Expert Medical Consensus Panel, 2003).I have added ‘movement ‘ to the list to reflect the experience of ME/CFS in the very severely ill; these patients may not even be able to tolerate a trip to the hospital via ambulance. They also may not be able to tolerate passive range of motion exercise without crashing. They may not be able to tolerate the movement/touch of having someone dress or wash them.
Other signs and symptoms of ME/CFS may include, but are not limited to: neurally mediated hypotension, postural orthostatic tachycardia syndrome, delayed orthostatic hypotension, light-headedness, palpitations, fluid retention, extreme palor, bruising, lump in throat, nausea, heartburn, abdominal pain, irritable bowel syndrome, loss of thermostatic stability, excessive sweating/night sweats, hot flushes, feelings of feverishness, feelings of cold extremities, heat/cold intolerance, abnormal appetite, marked weight change, hair loss, myalgia, muscle cramps, chest pressure/pain, arthralgia, TMJ, paralysis, muscle weakness, motor/balance problems, visual and auditory disturbances, severe immune abnormalities, reproductive system problems, respiratory problems and problems with the urinary system (Canadian Expert Medical Consensus Panel, 2003).
Deaths occurring in people with ME/CFS are largely due to heart or kidney failure, cancers and suicide. Often hospital conditions, misdiagnosis and/or allied staff ignorance lead to patient worsening, and can result in death (eg: Sophia Mirza, Alison Hunter).
Autopsy results of PWCs include:
cause of death: CFS. Post-mortem detected viral infection of the heart muscle, showing fibrosis indicating the presence of a long-standing infection (Casey Fero, 23 year-old male; US, 2005).
cause of death: acute aneuric renal failure due to dehydration as a result of CFS. Post-mortem evidence detected severe inflammation throughout 75% of spinal cord caused by dorsal root ganglionitis (Sophia Mirza, 32 year-old female; UK, 2005).
oedema of the lower limbs; alveolar spaces of the lungs filled with inflammatory cells and small emboli scattered throughout the arteries; marked congestion of the liver and spleen; bowel ischaemic; mild inflammation of the kidneys; also evidence of rhabdomyolysis (the breakdown of muscle fibers resulting in the release of muscle fibre contents into the circulation, some of which are toxic to the kidney); the bladder showing a hyperplastic epithelium; the thyroid showing colloid filled follicles, with scattered dystrophic calcifications and calcification of the small arterial walls; the right occipital lobe of the brain showing areas of degeneration and degenerated astrocytes, and the white matter surrounding this defect appeared puckered (unidentified PWC; US, 2005).
cause of death: severe progressive ME/CFS. Autopsy showed severe oedema of the heart, liver and brain (Alison Hunter, 19 year-old female; AUS, 1996).
cause of death: suicide. Autopsy using polymerase chain reaction showed: enteroviral sequences in samples from her muscle, heart, hypothalamus and brain stem. No enteroviral sequences were detected in any of the control tissues. The researchers stated: “The findings further support the possibility that hypothalamic dysfunction exists in the pathogenesis of (ME)CFS (and) they suggest that the chronic fatigue syndrome may be mediated by enterovirus infection and that persistent symptoms may reflect persistence in affected organs” (McGarry et al. Ann Intern Med: 1994:120:11: 972-3) (30 year-old female; UK, 1992).
Autopsy results show various severe pathological states, even when there are no diagnostic tests to indicate such states exist while the PWC is still alive. Don’t wait until your patient dies to believe they were ill.
Note that while patients functioning at a 3/10 - 5/10 (using Bested’s Functional Capacity Scale (BFCS)) may experience ‘exertional dyspnea’ upon walking up a few stairs, patients functioning at less than 1/10 may experience dyspnea just upon receiving a wash by an attendent because even this seemingly passive act is exertion for them. Similarly, patients functioning at any level may experience excruciating neurological pain upon hearing a jack-hammer; patients functioning under 1/10 may crash for weeks from the same sound. Most PWCs can tolerate moving their heads; a bedridden PWC functioing at 0/10 on the BFCS can crash horribly from attempting this simple movement.
The symptom picture for the very severely ill patient may appear unfamiliar, even to the specialist, because the symptoms are so severe and such patients are atypical and therefore rarely seen. ME/CFS specialists are also learning, and that is what makes them unique and helpful to their clients: the ability and willingness to take in and integrate new information that the patient presents. Understanding this illness is an evolving work in progress. To quote J. Audoban: “If the bird and the book disagree, believe the bird”… Specialists are willing to consider things in this light.
Patients at this level of functioning (usually 0.0/10 - 1.0/10 on BFCS) can rarely be seen by specialists as they are unable to travel and/or endure an appointment. They are often unable to speak.They are often misdiagnosed by doctors working in hospitals because cases such as theirs have not been seen there before. These clients represent an important, yet virtually unknown and under-recognized entity. They are the most likely to receive a psychiatric diagnosis such as ‘depression’, ‘somatization disorder’ and/or ‘dependent personality traits’, which further adds to the problem of receiving the respect and treatment that is appropriate for them in the hospital setting and beyond; it can also lead to death.
Symptoms and their severity vary from person to person, day to day, and with their degree of illness. PWCFS may ‘look fine’. If they don’t, it is because they have exceeded their capabilities and have entered a relapse with an almost complete loss of functioning. This is called a crash and needs to be avoided if any type of recovery from the illness is to be made. A crash can happen in an instant causing the inabilty to process all incoming information, a state of unresponsiveness and/or the inability to move. At this point, do not worsen the situation by patting, slapping, checking vital signs, repeating questions, etc. Just finish up with the most important steps of care, prepare the room as per usual instructions, and leave the client alone with lights off. Ensure that the environment is quiet. After crashing, the client may need assistance with Activities of Daily Living (ADLs) that she could manage prior to crashing. This may include, but is not limited to, help with eating, drinking, handling medications or passing urine (i.e. catheterization). If the patient was drinking liquified food from a cup prior to relapse, she may be unable to do so after crashing because her mouth and lip muscles may not be working well enough to manage the wide diameter of a cup. In this case, having someone pour the food into a small plastic water bottle can help as it greatly reduces the diameter of the food vessel making it easier to feed. The duration of a crash can only be known by living through it.
Generally, the very severely ill client may be totally bedridden (using diapers and/or bedpan); paralyzed; unable to stand or sit independently; unable to tolerate a transfer using a lift; unable to speak or tolerate being spoken to; unable to listen to the radio or watch television. They may not be able to tolerate having their body moved, or having the head of their bed raised. They may shake or have seizures. They may not be able to tolerate having their hair washed in a bed ‘sink’. They may be unable to open their eyes because light may cause intense neurological pain, and may require a darkened room. Even trying to look at a blank wall is often overstimulating; thus, people’s faces can become too complex to comprehend and eye contact will be avoided. If the patient is crashing from having others dress her, or is refusing to change clothes, it may be because her regular clothes are not presently appropriate and modified clothing or hospital gowns need to be used. At this level there is often excruciating, unrelenting pain with the complete awareness of what one is experiencing. Such PWCs may not be able to think on demand, or be able to hold thoughts together at all. It is unimaginably difficult just to get through a few hours. There is no quality of life. There is nothing in the healthy person’s realm that this experience can be compared to.
Studies have demonstrated that PWCs are among the sickest and least functional of many people with chronic illnesses. They may be the same as, or worse than, very late-stage AIDS patients (N. Klimas, 2009). Although it is atypical for PWC to die from their condition, they often lose the ability to experience their lives.
In 1998, an ME/CFS sufferer wrote: “I’ve had ME for nearly five years, 18 months of which were a living hell. The physical suffering (inability to walk unaided, chew, swallow, breathe properly, hold my head up, hands which became spastic) was bad enough, but the brain symptoms were at times unbearable – my brain exploding with stimulus until I thought I’d gone mad (and) the room spun like I was drunk, making me feel physically sick. The bed felt like it was moving. I had explosions of light before my eyes. Worst of all were the ‘seizures’, which felt like I was having a stroke – pins and needles on my head and face, drooping muscles around my mouth, my head would start to tip backwards, absolutely terrifying. I live alone, yet have been refused home care, disability living allowance or any form of medical advice. The public need to be shocked by seeing the severely affected, those being tube fed, shaking, uncontrollable, paralysis, unable to hold up their head, speak, see, control bowel movements. The myth that ME is never fatal must be dismissed. I know of several people who have died of the complications ME can bring” (Perspectives, September 1998:26).
Rehabilitation at this point is probably not possible, as just breathing and taking in food and liquid expends the energy held in a PWC reserve. The PWC at a 0 -1/10 on BFCS is often mistaken as being ‘lazy’, ‘unmotivated’ or ‘depressed’. Judging patients as such increases their feelings of being misunderstood and unsupported and is not helpful. Forcing graded exercise therapy (GET) on such patients can result in a relapse of several years and/or in death.
Positive feedback on anything that a PWCFS can do is helpful, as it creates feelings of being understood and supported. Drop judgements and accusations; stick to celebrating abilities, however small they may appear to an outsider looking in. You may not be able to verbally express this feedback, but the PWC will recognize it by your smile.
PWCs are extremely sensitive to their environments, and most have the co-existing condition of Environmental Sensitivity. Thus, the behaviours and skill set of attendants and allied health care staff is of utmost importance. Attendants/Staff must:
pay attention during orientation; take notes to help yourself
approach client with mindfulness; be grounded and gentle.
observe the general ‘no talking’ rule (if there is one), unless asked a question; keep answers simple
be able to follow oral, written and gestured diretcives; watch for body language; if a client has written, “brush teeth and give bedpan”, then only do these tasks; if she wants to add anything she will let you know. Continually asking if you should wash arms, etc. creates brain overload: she won’t be able to respond and it may cause a crash.
know your skills: how to move a body properly; how to insert tampons; how to wash and rinse a human being with care; how and when to use bed controls; how to brush teeth, how to place a bedpan, etc.
be able to manage time effectively
have a professional appearance and behaviours: do not waste a client’s time and energy by having them explain why your clothes need to be clean, nails short, hair tied back, scarves/toggles secured so they don’t fly into the client’s crotch, shirt-sleeves up, etc.
practice proper hygiene without being asked
Depending on the client’s requests, please rememer that the following may be necessary:
do not talk ‘out of the blue’; point to your lips and wait for permission to speak
do not try to engage or redirect a clients’ attention, especially if she’s engaged in a task (e.g. do not comment on the weather if client is already engaged in another activity); just follow written directives. PWCs are often not able to do simple multi- tasking such as this.
keep routine predictable, simple and flowing; just the touch of washing can be overload for the brain, so please do not add anything else to the picture
do not make frantic gestures to get the client’s attention; it will create the opposite result
allow the client the time and space to indicate her needs; do not confound her by continually asking her questions. Multiple choice questions are often not tolerated at all.
be on time. PWC have lost adaptability and rely on schedules. Even putting clocks back in the fall and consequently having service one hour later can cause a crash.
abide by the ‘scent-free policy’; do not wear scented products to a CFS booking as most PWC also have environmental sensitivities(ES)/multiple chemical sensitivities (MCS). If you are a healthcare worker, refrain from using perfumes, colognes, air deoderizers, spray sanitizers, or anything alse that has a scent.
re: housekeeping: use cleansers as per the instructions on the labels; check with the PWC about their tolerance of commercial cleaning products before using them
renovating and painting patient rooms is not acceptable if MCS is also present
keep hospitals/clinics/offices quiet! A private room for medical reasons should be provided for those clients that cannot tolerate sound without it causing severe pain, crash and/or seizure. A private room for hospitalized PWCs is also neccessary to ensure that proper sleep hygiene can be practiced. Lights out at 9pm; no talking, radio or visitors after this time. PWCs need 8-10 hours of continuous sleep nightly and may refuse vitals checks during the night, if it is their wish.
turn cell-phones off before entering a room where a severly ill PWC resides
Caring for someone with ME/CFS is likely more difficult than caring for someone with any other illness, as there are so many ‘small’ potential factors that can cause sustained worsening of health or even death. ME/CFS behaves differently than other diseases, and these differences must be recognized, acknowledged, and worked with to give the PWC their best chance at recovery.