Severe/Very Severe ME equals severe illness and multi-system dysfunction.
Receiving care in a hospital setting is not easy, straightforward, nor is it necessarily obvious as to how and when to interact safely and in the best way for the person.
Personal Care Plans are individual files on each patient put together by the patient and/or carer together with information added from the patient’s primary carer and the specialist consultants the patient attends.
There are strict protocols included in the Personal Care Plan to make arrival, admission, stay, and discharge, as well as medical assessments and procedures as easy on the person with Severe/Very Severe ME as possible, and to prevent major relapse.
The Personal Care Plan below is a sample only including sample case details re a person with Severe ME, which can be edited to suit your own needs. Each patient’s Personal Care Plan would be different and personal to the individual so changes will need to be made to that sample personal care plan to suit you or the person you are caring for.
Organising a personal care plan takes time and it is important to remember to carry the file for any hospital admission including to ED (A&E)
Personal Care Plan
Warning LDN User – opiate use contraindicated whilst using LDN
(See pages 16 & 17)
Content Page no.
1. Contact Details ………………………….. 2
2. Diagnoses ………………………….. 3, 4, 5
3. Crash Phase/Paralysis: ………………………….. 6
Patient’s Vital Signs
4. Emergency Department ………………………….. 7
5. Crash Management ………………………….. 8, 9
6. Inpatient information ………………………….. 10
7. IV Fluids/ ………………………….. 11
Medical Team Awareness
8. Surgery and Dental Treatment ………………………….. 12
9. Discharge Planning ………………………….. 13,14
10. Outpatient Attendance ………………………….. 15
11. Low Dose Naltrexone LDN ………………………….. 16,17
Next of Kin:
Permission is given to share my personal details or a condition update with those named under Next of Kin
Brother: Home Tel:
Brother: Home Tel:
Sister: Home Tel:
Personal Assistant Mobile:
HSE Key Worker:
In his absence only if urgent contact:
Name Hospital Tel:
1. Myalgic Encephalomyelitis
WHO Classification ICD 10 G93.3 classified as a Neurological disorder
WHO Classification ICD 11 8E49 classified as a Neurological disorder
SNOMED Classification SCTID: 118940003 Disorder of the nervous system (Disorder)
NASS (HRB) G93.3
When does an illness become a disease?
When the underlying biological abnormalities that cause the symptoms and signs of the illness are clarified.
“…. At that time, nothing was known about its underlying biology. Indeed, because many standard laboratory test results were normal, some clinicians explained to patients that “there is nothing wrong.” There was, of course, an alternative explanation: the standard laboratory tests might not have been the right tests to identify the underlying abnormalities.
Over the past 35 years, thousands of studies from laboratories in many countries have documented underlying biological abnormalities involving many organ systems in patients with ME/CFS, compared with healthy controls: in short, there is something wrong. Moreover, most of the abnormalities are not detected by standard laboratory tests. In 2015, the Institute of Medicine of the National Academy of Sciences concluded that ME/CFS “is a serious, chronic, complex systemic disease that often can profoundly affect the lives of patients,” affects up to an estimated 2.5 million people in the United States, and generates direct and indirect expenses of approximately $17 billion to $24 billion annually.”
AHRQ 2016 Addendum
Diagnosis and Treatment of Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome
“Conclusions: Although future studies should refrain from using the Oxford (Sharpe, 1991) case definition as eligibility requirements, this early work provided a foundation on which future work can expand. This addendum has delineated differences in treatment effectiveness and harms according to case definitions, highlighting studies that used the Oxford (Sharpe, 1991) case definition and how these studies impacted our conclusions. Additionally, results of studies evaluating CBT have been considered independently from other counseling and behavioral therapies. Our sensitivity analysis would result in a downgrading of our strength of evidence on several outcomes which can be attributed to the decrease in power, dominance of one large trial, or lack of trials using criteria other than the Oxford (Sharpe, 1991) case definition for inclusion. Blatantly missing from this body of literature are trials evaluating effectiveness of interventions in the treatment of individuals meeting case definitions for ME or ME/CFS.
Essential Reading for staff unfamiliar with ME:
Myalgic Encephalomyelitis – Adult and Paediatric: International Consensus Primer 2012 Pages 3-12
Myalgic Encephalomyelitis: Symptoms and Biomarkers
General Information on Myalgic Encephalomyelitis
Overload Phenomena: hypersensitivity to many kinds of sensory input can cause a crash
Crash – a temporary period of immobilizing physical and/or cognitive exhaustion.
Hypersensitivity to and overload of sensory stimuli
· more than one source of information
· mixed modalities of input – auditory and visual, physical and cognitive
· physical or mental exertion
· fast paced or confusing environments
· extremes of temperature.
PENE (Post Exertional Neuroimmune Exhaustion) is the pronounced summation effects and after-effects of numerous interactive dysfunctions. (Often termed PEM, Post Exertional Malaise)
This feature is characterized by a pathologically low threshold of physical and mental fatigability, exhaustion, pain and an abnormal exacerbation of symptoms primarily in the neuroimmune regions.
Fatigue and pain are indispensable bioalarms that alert patients to modify their activities in order to prevent further damage
Effects: physical and mental exhaustion, weakness, symptoms flare and a prolonged recovery
Pathological components: neuroimmune exhaustion
· decreased cerebral oxygen and blood volume flow, cardiac output and pain threshold
· impaired aerobics metabolism and oxygen delivery to muscles
· elevated sensory signalling to the brain perceived as fatigue and pain
· immune activation.
Treatment: Pacing • stay within current` energy availability
• keep cardiovascular response below the anaerobic threshold.
Manage sleep, pain, fatigue, fluids
A pain syndrome is part of the Myalgic Encephalomyelitis (ME) presentation.
The pain is extreme in arms, legs, and torso and includes cardiac pain.
General: Myalgic Encephalomyelitis is a multi-system, multi organ disease.
The ability of the body to create energy is flawed
Mitochondrial tests show the ATP production is poor:
Low whole-cell ATP
Low mt-ATP and very poor provision of ‘new’ mt-ATP
Very rapid depletion of ATP on increased energy demand
When there is no energy, systems in the body shut down – this is what happens in a crash,
when extreme, paralysis can occur.
2. Coronary Microvascular Dysfunction
‘Many patients undergo coronary angiography because of chest pain syndromes believed to be indicative of coronary artery disease (CAD). A significant proportion of such patients, however, are found to have normal-appearing coronary arteries. Several published series have reported that up to 40% of patients undergoing coronary angiography fall into this category.1
In 1985, Cannon and Epstein2 introduced the term ‘microvascular angina’ (MVA) for this patient population, in view of what appeared to be heightened sensitivity of the coronary microcirculation to vasoconstrictor stimuli associated with a limited microvascular vasodilator capacity. They proposed that dysfunction of small intramural prearteriolar coronary arteries might be the pathogenetic cause of this syndrome.3 Although there was an initial attempt to group all these patients into one category, it was soon realized that they represent a spectrum from both the pathophysiological and clinical viewpoint.
In the past 20 years, a large number of studies using both invasive and non-invasive techniques for the assessment of coronary physiology have produced a large wealth of data leading to a better understanding of coronary microvascular dysfunction (CMD) and microvascular ischaemia.
The availability of normal values of MBF and CFR has allowed the investigation of coronary physiology in subjects at an increased risk of CAD and also in different categories of patients with symptoms and signs, suggestive of myocardial ischaemia despite normal coronary angiograms5 (Figure 1).
chest pain syndromes with normal angiography represent a wider category including,
among others, MVA.
Coronary microvascular dysfunction: an update
3. Further considerations
· Type II Muscle Atrophy, severe with focal loss of mosaic pattern
· CKD III
· White matter hyperintensities (MRI)
· Gastro-intestinal issues
If a patient enters a crash phase
Canadian Consensus Document on Myalgic Encephalomyelitis 2003 defines a crash as
“a temporary period of immobilizing physical and/or cognitive exhaustion”
the patient may become paralysed and unable to inform the HCP of the extent of the pain.
Paralysis may be limited to limbs only or may affect the whole body.
Standard protocols for pain control are NOT effective .
BP can vary hugely, usually in the 130-160/85-95
· Going as high as 224/164 or
· in a crash state BP will drop to <120/<80 (e.g. 96/64) which results in the Patient feeling drained & unwell.
Temp basal temp usually around 35.5, can increase in warmer weather
· When exhausted or experiencing PENE (Page 4) temperature can drop as low as 33.8.
· Legs/arms will be very cold & head very hot with raised temp, in this case cover limbs and cool head, fan/cool cloth
· whole body temp lowers & all body requires warming, especially extremities.
HR Resting HR varies 40-50 depending on current baseline.
· Heart rate varies hugely depending on current baseline and ‘activity’
· ‘Activity’/effort can increase heart rate to 130+, going to bathroom, eating etc
· During a crash or when exhausted HR can drop to 30, this is part of the ME ‘dauer’ state which will
resolve without intervention.
· current Medication Sheet at the back of the Emergency File carried by Patient
· Vital Signs, do not rely on standard norms for this Patient (See Page 6 Vital Signs)
Early intervention with relevant pain medications/Diazepam
will limit exacerbation of ME symptoms caused by the particular environment of the ED
· Be aware that BP can spike but also fall markedly which affects a patient’s ability to function.
· Changes can be rapid and may not be triggered by movement, position, IV fluids/fluids
· Do not assume therefore that “standard” BP norms apply (see Page 6)
If brought in by Ambulance:
Arriving at ED Reception
Crash Management (See Page 6: Do/Do Not and Vital Signs)
After any procedure or increased activity please ensure you are aware of the following needs for this patient
Important to note
Pain Medication See Current Medication sheet – at end of Emergency Folder carried by Patient
Up to 10mg
IV Fluids See page 11
Crash state - Do not assume she is peacefully asleep – she is not!
· Unresponsive - unable to move any part of her body/speak/open her eyes.
· She can hear everything.
· Pain is magnified and becomes very difficult to bear.
· Noise and light hurt
· She cannot ask for what she needs as she cannot move to ring a bell or speak to an HCP.
She will potentially need:
· The call bell to be placed in her hand (not on her body).
· Pain medication given (Pain may be very severe in this phase & requires increased pain medication)
· Give IV fluids – begin with 11 over 8hrs. If no fluids have been taken in recent hours, increase the rate of administration of the first bag (See page 11)
· An HCP/Staff member to offer and help her to use a bed pan, if she is able to (unlikely) as she cannot ask for one
· Incontinence pad (large) may be required, as a short-term measure. This has proved the best method in the past
Note to be taken of the fact that
· the patient can hear everything that is said and appreciates being spoken to – it is reassuring to the patient that the HCA/other staff know the phase that she is in
· with effort, she can move a finger to answer “yes” to a simple question that requires a “yes” answer,
· time needs to be given for the Patient to respond
· she should not be asked to open her eyes as she cannot.
Ø Over several hours, she will slowly regain the ability to move her hands /arms/ legs/head and subsequently to speak very quietly using single words or abbreviated phrases – this is an immense effort and drains her.
Ø As her energy increases, she will be able to communicate more freely and speak more loudly. Eventually she will be able to open her eyes. By choice, she will keep her eyes closed as much as possible, as the sensory information received drains energy which needs to be conserved to recover.
This phase usually lasts for around 4-8 hours
She recovers fastest if provided with as much support as possible and allowed rest as much as possible.
Every small action drains the energy that has been regained.
Once she can physically move in the bed, the less she has to do for herself in the first few hours after a crash, the more rapidly she will regain her independence.
· lifting anything with her arms hurts, is severely draining of energy or is impossible
· regard her as totally dependent for all her needs – feeding, drinking.
· regular fluid is essential hence IV fluids (see page 11)
· toileting – whilst immobile, she is best using an incontinence pad which has been found to be the best method for relief at this stage. She will later graduate to the bed pan and when energy increases will ask to use the commode, with help.
As sitting upright at this stage may trigger another crash,
it is not appropriate to leave her alone on a commode.
Any form of exertion (this may be a wash, telephone call, listening to someone, being animated, sitting up, having a shower, trying to walk) may cause her to crash, as described above, or enter a partial crash but without the total inability to move.
‘Encouragement’ to do more is counterproductive.
Reminding her to stay within her current limits is the ‘pacing’ strategy which enables her to improve.
The more rest and support received in the early stages,
the greater the chance the patient has of regaining her previous baseline.
Once admitted to a ward she is usually either within, or immediately after, a crash.
· Needed is peace, quiet, minimal interventions, sleep
· Pain medication (Page 8)
· IV fluids (Page 11)
· She will usually have ear plugs and an eye mask with her to facilitate same
· At this stage it may still be impossible/difficult for her to ask for help or to reach for items
· The patient is best helped by asking if she needs them and passing them to her
As she improves, she will use in ear headphones with an audio book to cut out background noise
· Fluids are vital to quality rest.
· Access to a bedpan or if too weak an appropriate incontinence pad (large).
· Ensure the call bell is in her hand as she cannot move to reach for it.
Support needed when she “comes round” and is able to move be aware:
All movement, activity, interaction with people, light and sound around her
will create sensory and physical input which will exhaust her very quickly & cause deterioration
· She is likely to be able to eat breakfast but then need to rest.
· She may be able to use a commode with help.
Do not leave her sitting upright or unattended, she may crash – it only takes seconds of “doing too much” for this to happen.
· If she is apparently asleep and misses a meal – check on her.
It is likely that she has “done too much”, is exhausted and needs intervention, pain medications/fluids/incontinence pad or the bed pan/call bell in my hand.
· At the stage where she can be taken to the bathroom, be aware that using the lavatory and washing her hands is the limit of her energy at this state.
After a rest a bowl at the bed is adequate for washing.
· Do not expect her to sit up and eat breakfast, use the bathroom facilities – lavatory/wash hands and have a complete wash – she cannot do this at this stage without deteriorating.
Each step has to be done with rest periods in between. Washing frequently becomes an afternoon activity.
· It will take her a few days to be able to manage a daily routine which includes all meals and full use of bathroom.
· The more help she can be given to avoid activity during this time will decrease the time she needs to reach a baseline which means discharge can be planned for.
· Do not expect her to sit upright, sit in a chair or stand – each of these activities increases the time for her to reach her baseline.
Attempts to “motivate” her will tend to hinder her recovery.
1) A functioning cannula should be present at all times
2) Pump delivery
3) Place cannula in hand/wrist not sited in the anticubital fossa
4) IV Fluids to be available at all times – see table
If patient has had no fluids in recent hours OR is in a crash phase
Can initially be given at rate of 250 mls an hour
Thereafter 1 litre over 8 hours
Fluids PRN to be available at all times
1 litre over 12 hours or 0.51itre over 6 hours
Medical Team awareness:
a) to ensure that prior to weekend the cannula is fully functioning
b) fluids have been charted in line with above directions for the whole weekend and until Monday rounds
c) replacing a cannula can be difficult.
Patient has no issue with needles, repeated attempts are preferable to leaving her without a cannula
d) fluids are permanently charted for PRN or crash situations
Pain Medication & Diazepam in the home
By choice the Patient prefers to use LDN and therefore avoids opiate use and uses minimum pain killers.
Diazepam is used on a needs basis.
The Patient prefers to acknowledge pain and fatigue as
‘Fatigue and pain are indispensable bioalarms that
alert patients to modify their activities
in order to prevent further damage’
Attempts by staff:
· unaware of the condition or her history,
· who follow standard cautions/concerns for opiate and diazepam usage
· will result in a draining of the Patient’s energy whilst she explains the long-standing management techniques which have served her well, with her Consultants’ oversight, during many admissions and at home for several years
Surgery and Dental Work
Surgery: Prior to surgery alert the Surgeon & Anaesthetist to the important issues in the management of ME
· General Information Page 2
· Patient is an LDN User Pages 16 & 17
o hypersensitivities to drugs, cat scan dye, anaesthetic containing adrenalin, Vit D injection
o low circulating blood volume, low intracellular magnesium and potassium levels
o rapid fatigability and elevated pain and fatigue levels.
Ensure Patient is well hydrated prior to surgery (Page 11)
Patient takes longer to recover and may need extra time in hospital.
Experience is that any dental work is highly likely to cause a:
· Crash causing paralysis in limbs or whole body (Page 6)
· Prolonged recovery period
Response to dental work is not an ‘anxiety’ state as BP & HR drop, HR has lowered to 30bpm
Dr X, Oral Surgeon/Principal Dental Surgeon HSE West Dental Department
manages my dental care and has experienced a number of crash episodes during/post dental work, referring to them as a ‘neurological shutdown’
Avoid drugs containing adrenaline
If a Crash occurs refer to:
Crash Phase/Paralysis Page 6
Crash Management Page 8 & 9
IV Fluids Page 11
Preparations for Discharge are to be agreed and co-ordinated between the patient, the Consultant(s), Ward Sister and the Discharge Co-ordinator if necessary.
Contact will need to be made with Community Health Organisation Services to discuss discharge package based on Patient’s current needs. (See Key Worker Page 2, or Service Manager in his absence)
Information for noting:
· The discharge process and the journey will reduce her current state of activity to a level below which it is safe for her to be moved without causing deterioration
· Having reached a level of activity which would enable her to be at home, with an appropriate care package and single PA, does not necessarily mean that she can transfer that level of activity to the new environment
· The process of discharge, the journey and settling in to the new environment will inevitably cause deterioration which may result in a return to acute care with the availability of appropriate supports for a crash state.
Prior to planning discharge, the following baseline should have been achieved:
1. Patient is active around the bed including being able to sit upright in bed for periods of time
2. Patient is using the mobile phone and computer
3. Patient is eating three meals a day
4. Patient can access the bathroom using a mobility scooter or wheelchair unaided
5. Patient is demonstrating a level of energy and interest when awake, although she may still be sleeping for long periods
6. Patient has discontinued use of IV fluids
All of the above should be occurring, without deterioration, for two days after first achieving this level.
o Being pushed in a wheelchair/on a trolley - normal speed results in sensory sensitivity so she will have to close her eyes. Same applies to going around corners, facing a different direction or being moved lying flat.
o The discharge process itself is extremely tiring as she will need to check her medications for home, organise belongings, discharge letter and ensure home is ready for her return etc.
o During the journey home, she will need to be flat and still, with her eyes closed.
Sudden activity should not take place to plan her discharge as this risks precipitating a crash
Any meetings/discussions, even at the bedside, need to be:
· advised with advance notice
· a planned part of her day’s schedule
so that the Patient can plan activities such as washing/eating/sleeping and being “active” to include planning for discharge.
Prior to leaving, appropriate pain medication and diazepam should be given if required to help limit the effects of the journey.
Actions prior to discharge:
It is essential to ensure that:
· her GP is aware of her return home,
· her PHN is aware of her return home
· any additional medication is communicated to Ryan’s Pharmacy (see Page 2) for collection by her PA (not available on a Sunday) or provided by the hospital until the Pharmacy can be accessed
· PA’s have prepared the home and done appropriate shopping
· Ambulance is booked
Opiate Prescription for returning home:
Once home and stable, the Patient will reduce and discontinue opiate use and will then re-introduce LDN beginning at 0.5ml and titrating up to the 4.5ml dose.
completing the discharge prescription, opiate medication needs to be discussed
and agreed between the Patient and her Consultant and the discharge prescription
needs to contain a month’s supply of the opiates agreed upon.
The patient will bring a typed list of:
a) Presentation since last consultation
b) Clinical issues which ‘fit’ her current presentation of ME & CMVD (See Pages 2-4)
c) Any other relevant matters
If the Patient has concerns about anything not related to her current ME & CMVD diagnosis:
· she will attend her GP
Her GP may choose to:
· contact the Patient’s Consultant directly
· refer her to the OPD of her attending Consultant
· refer her elsewhere.
1. The Patient will be accompanied and will require her companion to be with her:
· in all clinical areas for observation prior to the Consultant appointment, as she sees fit
· during the Consultation, as she sees fit.
· during any examination/procedure, as she sees fit (other than the Operating Theatre)
2. The area in which she is to be seen or is required to wait (e.g. clinic room, waiting area) will require a facility for her to recline if needed e.g. an examination couch
3. Space is required in the waiting area for her Mobility Scooter. Chairs may need to be removed to allow access
4. The appointment slot should be of 30 minutes duration and ideally at 13.30
5. Ideally the Consultant should have appraised her/himself of Myalgic Encephalomyelitis and the Patient’s specific care plan
Low Dose Naltrexone
Many autoimmune diseases seem to respond to LDN.
The first thing to understand is that Naltrexone – the drug in LDN – comes in a 50:50 mixture of 2 different shapes (called isomers).
It has been recently discovered that:
· one particular shape binds to immune cells
· whilst the other shape binds to opioid receptors.
Although consisting of exactly the same components, the two isomers appear to have different biological activity.
LDN can be taken with other medications or supplements as long as they do not contain opiates or synthetic narcotics, examples of which include fentanyl, meperidine (Demerol, Pethidine), tramadol, morphine, oxycodone and hydrocodone. Naltrexone blocks the opioid receptors. Therefore pain medications will be blocked from working and could lead to withdrawal problems. Check with your doctor and pharmacist to make sure that none of your medications are contraindicated. They can also advise you on stopping pain medications that might interfere with LDN and offer advice and amount of time to allow between stopping opiates and starting LDN.
After starting LDN, if you have surgery scheduled or a procedure that may require pain medications, consult with your doctor to determine the amount of time needed to clear again from your system so that it does not interfere with anesthesia or pain medications. LDN must also be stopped if your doctor plans to prescribe opiate-based medications for postoperative use. The time required to clear naltrexone for the body may vary, based on dosage and body weight.
After a procedure under anesthesia or requiring pain medications allow adequate time for the opiates to clear from your system before restarting LDN.
Summary of mechanism of action
The summary of 10 years of research is that LDN works because:
Levo-Naltrexone is an antagonist for the opiate/endorphin receptors
· This causes increased endorphin release
· Increased endorphins modulate the immune response
· This reduces the speed of unwanted cells growing Dextro-Naltrexone is an antagonist for at least one, if not more immune cells
· Antagonises “TLR,” suppressing cytokine modulated immune system
· Antagonises TLR-mediated production of NF-kB – reducing inflammation, potentially downregulating oncogenes
Taking Naltrexone in larger doses of 50-300mg seems to negate the immunomodulatory effect by overwhelming the receptors, so for the effect to work, the dose must be in the range of 0.5-10mg, usually maxing at 4.5mg in clinical experience.
To summarize, when glial cells are activated they release chemicals and neurotransmitters that cause NMDA receptors to be activated which cause nerves to fire.
LDN (Low Dose Naltrexone), by its ability to inhibit microglial activation, suppresses activation of NMDA receptors by decreasing the release of glutamate neurotransmitter.
Whether to try LDN for CRPS must be seriously considered, especially since it can have interactions with existing medical regimens, particularly if medications are opioids (morphine like drugs). It should not be taken by patients who are on opioids or tramadol. Often, the choice is easiest for patients who are not on opioids. Fortunately, LDN has a low risk of side effects before taking LDN, one must consider current research, clinical trials, strength of anecdotal reports, severity of CRPS, response to other therapies, drug interactions and any contraindications.
Most physicians are unfamiliar with LDN. Be prepared to discuss LDN with your physician and acquaint him or her. There are some resources at the end of this article to help you acquaint yourself and your physician.
What to expect from LDN
LDN does not work immediately. It may take anywhere from a few weeks to many months. Users have reported to notice a difference after 9 to 12 months. After the initial response, it continues to show a benefit. The main goal of LDN is to slow or halt the progression of disease. In addition, symptoms may improve. Improvements seen in pain include decreases in exacerbation of pain, symptom improvement, improved functioning and better tolerance to pain.
LDN may increase endorphins (morphine like substances produced by the body) which may result in a feeling of well being. Human trials have demonstrated improvement in mood and in quality-of-life scores. This feeling helps lower stress, reduce depression, and increase healing. This is especially true for conditions like CRPS where stress can lead to exacerbations.
Naltrexone was initially tested in humans for safety at the 50 to 100 mg dose level. There have been a number of studies such as a Crohn's disease study. Studies have assessed naltrexone administered at low-dose for safety and found no major issues to date.
Physicians who prescribe LDN feel that at such a low dose, it is unlikely to cause any harm. At high doses (50mg to 300mg of naltrexone) it may affect the liver. Patients with pre-existing liver and kidney conditions using LDN should have their metabolic functions monitored by their doctors.
LDN does not stay in the body very long, hence if an emergency arises and a patient has to be administered an opioid for managing severe pain, they are unlikely to see any withdrawal effects.
Post a Comment