Primer for family, friends and care providers

Primer for family, friends and care providers is for those who know or care for someone with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), a complicated and life-altering systemic, biological, neuroimmune disease. Patient care and severity of the disease is poorly understood due to decades of research neglect and a lack of clinical care worldwide.

Diagnosing ME/CFS is difficult as there is no clinically accepted biomarker and there are numerous definitions and criteria that have been developed and utilized over the decades. https://www.youtube.com/watch?v=pqDubEeIBtA

The core ME/CFS symptoms are: post-exertional malaise (PEM); chronic fatigue (CF), unrefreshing sleep/sleep problems; and cognitive impairment/brain fog and/or orthostatic intolerance (OI). A patient can have many more symptoms and meet different criteria. Systemic Exertion Intolerance Disease (SEID), an ME/CFS criteria, allows for a patient to be diagnosed with the minimum core symptoms and the patient may have other symptoms. The Canadian Consensus Criteria (CCC) requires the core symptoms and neurological, autonomic, neuroendocrine, immune system, and myalgia to meet its ME/CFS diagnostic criteria. The International Consensus Criteria (ICC) is used to diagnose myalgic encephalomyelitis (ME) which requires the core symptoms and neurological, immune/gastrointestinal/genitourinary impairment, and energy metabolism/ion transport impairment symptoms for a diagnosis.

Although the Fukuda and the Oxford Criteria are sometimes used for diagnosing chronic fatigue syndrome (CFS), they are both overly broad and there is criticism that patients are being misdiagnosed in both clinical and research settings as having CFS when in fact they are suffering from the symptom chronic fatigue (CF). There have been media reports of athletes diagnosed with myalgic encephalomyelitis (ME) or chronic fatigue syndrome (CFS) who have recovered in a relatively short period of time after rest, supplementation, and diet changes;    these athletes may have had overtraining syndrome (which has the symptom of CF) and not ME, CFS. CF is a symptom of many diseases and illnesses, and should not be confused with the grossly misnamed disease CFS. Some people with adrenal failure are sometimes wrongly diagnosed with CFS.

The acronym ME/CFS is widely used in research, by clinicians and patient organizations, and patients.

"The most common overlapping condition with ME/CFS is fibromyalgia." While some have posited ME/CFS and fibromyalgia are variants of the same illness, Benjamin Natelson, MD summoned considerable amounts of data that suggest the two illnesses differ with different pathophysiologic processes leading to different treatments.

Unfortunately, a psychiatric approach has been taken with ME/CFS in the past, but this is changing. At this time there are no approved drug treatments. Until 2021 graded exercise therapy (GET) and cognitive behavioral therapy (CBT) were used in the UK for treating ME/CFS, and caused significant harm. The Centers for Disease Control and Prevention (CDC) website states "ME/CFS is a biological illness, not a psychologic disorder" and impacts multiple body systems. The CDC recognizes the hallmark symptom of post-exertional malaise which is a worsening of symptoms after physical or mental activity and says ME/CFS is a "disabling and complex disease."



Recommended reading for care providers
"Myalgic Encephalomyelitis (also known as Chronic Fatigue Syndrome or ME/CFS) is a complex, challenging disease for everyone involved - not least the caregiver. Caring for someone with ME will require adaptability, steadfast patience and commitment over many years, if not a lifetime."
 * Caring for People with Myalgic Encephalomyelitis by #MEAction


 * Table of Contents:
 * 1) Educate Yourself
 * 2) Take Care of Yourself
 * 3) Family Dynamic
 * 4) Relationship with the Patient
 * 5) Resources
 * 6) Advocate
 * 7) Power of Attorney
 * 8) Get Organized
 * 9) Join the Community
 * 10) Read more

Nurse Greg Crowhurst has cared for his wife Linda for decades as she is very severely ill with ME. Greg has written several books on caring for someone with ME.


 * Severe ME - Featuring: "Justice for Karina Hansen"
 * Severe ME: Notes for Carers
 * Caring for ME

Other books/guides
 * A Beginner's Guide to ME/CFS
 * Caring For The M.E. Patient
 * Chronic Fatigue Syndrome/ME: Support for Family and Friends
 * Understanding M.E. - A Guide For Friends, Family & Carers
 * What Is M.E.? A Guide For Children

Disease onset and course of illness
ME/CFS can begin from many acute/sudden events: usually viral or bacterial infections, but also trauma, surgery or childbirth, allergic reaction, and stress. There is also a gradual onset in some people, that is not attributed to any one event. Occasionally, ME/CFS has been triggered by environmental toxins or the receipt of an immunizing injection. Some say that the disease ME always has an acute/sudden infectious onset.

The disease ME/CFS is often diagnosed when a person does not recover from a flu-like illness, mononucleosis or another herpesvirus, Q fever, an unidentified virus, or another infection, and meets one or more diagnostic criteria for either ME, CFS, or ME/CFS. Patients experience numerous symptoms and disease severity which fluctuate from day to day, week to week, month to month, year to year, and even decade to decade. Furthermore, symptoms and disease severity vary among patients.

The CDC recognizes that there can be different causes and it is possible that two or more things could cause the illness. Areas of research include infections, immune system changes, stress affecting body chemistry, changes in energy production, and a possible genetic link. The CDC notes abnormalities with the immune system, cellular metabolism, neuroendocrine disturbances, and blood pressure or heart rate regulation.

Not a mental health disorder
In the past, CFS was believed to be a mental health disorder. This is why the CDC now states: "ME/CFS is a biological illness, not a psychologic disorder. Patients with ME/CFS are neither malingering nor seeking secondary gain. These patients have multiple pathophysiological changes that affect multiple systems."

No clear evidence that ME/CFS is contagious
Infectious episodes have led to outbreaks over the years and 72% of ME/CFS patients report an onset of a viral or bacterial infection. Nowadays, the vast majority of ME/CFS cases are sporadic rather than epidemic, and there is no clear evidence that sporadic ME/CFS cases are contagious.

Families, partners, and friends do not report contracting ME/CFS from someone with the disease nor do patients report passing it on to others. Underhill and O'Gorman (2006) researched 219 patients with ME/CFS, and concluded that close household contact and genetics were both risk factors for CFS, finding 3.2% of spouses/partners of those with CFS also had the illness. This was a higher prevalence than all genetic relatives except children of patients. Because ME/CFS can run in families, a genetic link is a line of research recommended by the CDC.

Michael Sikora and collaborators at the Open Medicine Foundation hope that their research on the role of T cells and immune-related genes will help address "whether ME/CFS is an autoimmune or infectious disease, or simply an activation of the immune system".

Blood donation and organ transplant
Blood donation prohibition had been enacted in the United States (US) and United Kingdom (UK) at one time due to the research of XMRV as being the infectious trigger of CFS and that patients carried the virus. Two papers on XMRV were retracted as it was a laboratory contamination. The US American Red Cross and UK National Health Service Blood and Transplant sites no longer have statements barring transfusions or transplants for CFS. Australia has a prohibition on blood transfusion as a precaution.

Mini-Docs
https://www.youtube.com/watch?v=Fb3yp4uJhq0 What happens when you have a disease doctors can't diagnose By Jen Brea/TED

Five years ago, TED Fellow Jen Brea became progressively ill with myalgic encephalomyelitis, commonly known as chronic fatigue syndrome, a debilitating illness that severely impairs normal activities and on bad days makes even the rustling of bed sheets unbearable. In this poignant talk, Brea describes the obstacles she's encountered in seeking treatment for her illness, whose root causes and physical effects we don't fully understand, as well as her mission to document through film the lives of patients that medicine struggles to treat.

https://www.youtube.com/watch?v=9_HwOUiImvw

Invisible Illness - Stories of Chronic Fatigue Syndrome By Veronica Weber/Palo Alto Online

This mini documentary reveals 3 stories of people who have been impacted by Chronic Fatigue Syndrome - a little known disease that affects roughly 836,000-2.5 million people in the U.S. and receives little research funding. They share emotions of treating loved ones with the disease, their frustrations of being ignored by members of society and the healthcare industry and express hopes of treatment and research. Video by Veronica Weber/Palo Alto Online

https://www.youtube.com/watch?v=RC9TjgE_PlU

Diagnosis and Management of Myalgic Encephalomyelitis and Chronic Fatigue Syndrome By Dr. David Kaufman/Unrest

This video on the diagnosis and management of myalgic encephalomyelitis and chronic fatigue syndrome is part of the Unrest Continuing Education module, made available through the American Medical Women's Association and Indiana University School of Medicine, and in partnership with #MEAction. US medical providers can visit: https://www.unrest.film/cme to register to watch Unrest online for free and receive Continuing Education credit.

Epidemiology
In the United States, 836,000 - 2.5 million people suffer from ME/CFS. "The total economic costs of ME/CFS are estimated at $17 to $24 billion annually." Some CFS patients can work with job accommodations but 1/3 to 1/2 become unemployed and many rely on SSDI/SSI.
 * Epidemiology
 * All races and cultures are afflicted with ME/CFS. Children and adolescents are also diagnosed.
 * Female predominant diseases
 * Like many autoimmune and neuro-immune diseases where mostly women are afflicted, the ME/CFS female/male patient ratio per Capelli et al. is 6:1 while the CDC states 4:1.


 * Pediatric
 * Pediatric ME/CFS is defined by the CDC and the National Academy of Medicine (NAM) although it is usually diagnosed in adults. "Children below the age of 8 or 9 do not have the symptom pattern of adolescents past puberty. If the onset of the disease occurs during adolescence, the most common time of onset, the pattern is similar to that of adults." The prognosis in adolescents is considered to be better than in adults. Children are diagnosed with ME/CFS at three months of illness under SEID and CCC and ME is diagnosed immediately under International Consensus Criteria.


 * Prevalence
 * "Worldwide, there may be as many as 17 – 24 million people with ME/CFS." 25% of ME/CFS patients are housebound or bedbound at some point in their illness. 90% of patients are undiagnosed.


 * Prognosis
 * The prognosis for a patient diagnosed with ME/CFS isconsidered to be poor with only a minority (a median estimate of 5%) returning to pre-morbid levels of functioning. The majority of patients remains significantly impaired. A substantial improvement however is noted in an estimated 40% of patients.

Symptoms
Symptom presentation varies enormously between individuals. Symptom presentation also varies within individuals, as individuals often report that symptoms change over time (increasing or decreasing) and new symptoms may appear while others disappear. There are many symptoms which people with ME/CFS experience, though those listed below are the core symptoms found in all patients.













"This is a term which describes a symptom in which exercise or exertion can bring on malaise (illness). In the case of people with ME/CFS, malaise often occurs during a period some 24-72 hours after exertion. For example, in some cases, a short walk can worsen ME/CFS symptoms two days later. This lack of understanding about the delayed onset of symptoms has, in the past, made it harder to test for ME/CFS. Follow up tests, taken less than 24 hours after an initial exercise test, may show that the patient can still perform activities at the same level (before post exertional malaise has a chance to take hold)."
 * Post-exertional malaise (PEM) is the hallmark symptom of ME/CFS. After physical or mental exertion (which for some patients can be a shower or making out a to-do list, others grocery shopping, socializing or reading a news article, while some just walking to the mailbox, getting to the doctor or mentally following a T.V. program) there is a payback of worsening ME/CFS symptoms which can be delayed 24-72 hours or more  and can last 24 hours and even days, weeks, or months.. The patient will experience even greater fatigue as well as exasperate the flu-like symptoms and body pain. Every patient experiences different symptoms and symptom severity from different activities and exertion output according to how sick he or she is with the disease.  Regarding PEM the CFIDS Association of America states:
 * chronic fatigue
 * cognitive dysfunction
 * orthostatic intolerance (OI) such as postural orthostatic tachycardia syndrome (POTS) or neurally mediated hypotension (NMH)
 * unrefreshing sleep See also: sleep disturbance

Other possible symptoms

 * chronic pain
 * immune system
 * neurological signs and symptoms See also: neuroinflammation.
 * List of symptoms in ME/CFS

Comorbids

 * fibromyalgia (FMS)
 * irritable bowel syndrome (IBS)
 * multiple chemical sensitivity (MCS)
 * temporomandibular joint disorder (TMJ)

Government guides on symptoms
US Government guides on symptoms
 * Clinicians Guide from the Institute of Medicine report
 * CDC - ME/CFS Symptoms

Canada guides on symptoms
 * Alberta Clinicians Guide

Tests

 * Natural killer cell (NKC) Blood Test


 * Sleep study for sleep apnea, restless leg syndrome, and other sleep disturbances.
 * Tilt table test for OI or POTS.

Drugs, treatments, and therapies
There are currently no Food and Drug Administration (FDA) approved treatments for ME/CFS. Treatments consist mostly of symptom management, rather than treatment of the underlying cause of the illness, which is not yet understood. There are many potential treatments, though their evidence base is limited, as most research into treatments has gone into unsuccessful psychological approaches to treatment.

Drugs


Two treatments that have garnered much attention are Ampligen, produced by Hemispherx Biopharma, and Rituximab. Many people have reported enormous benefit from Ampligen; some doctors have been prescribing it for ME/CFS for decades.

Attempts to obtain FDA approval for Ampligen in the US have failed so it is unavailable to many. Argentina has approved the use of Ampligen for severe ME/CFS in 2016. Also in 2016, it was made available on a limited basis in Europe. July of 2018, Hemispherx Biopharma, Inc "announced the immediate expansion of its Treatment Protocol/Expanded Access Programs for ME/CFS in the United States, known as AMP-511, to new enrollees for the first time in more than a year." AMP-511 "will allow treatment of up to 100 ME/CFS patients at any one time at approved clinical infusion therapy sites."

Rituximab, a lymphoma drug, had shown promising results in initial trials in Norway, and there were groups crowdsourcing funding for further trials in other countries. In 2017 Drs. Øystein Fluge and Olav Mella announced that their Rituximab trial had failed. They stated that they would focus their efforts on attempting to identify a subgroup of ME/CFS patients with an immune profile that would be responsive to Rituximab.

Jarred Younger announced in early 2016 that he will be undertaking a trial of low dose naltrexone (LDN) in ME/CFS.

Treating other conditions
Different forms of OI are treated with beta-blockers (Metopropol), Fludrocortisone (Florinef), and Pyridostigmine. When treating other diseases, illnesses, and conditions, and ME/CFS comorbids and overlapping conditions such as sleep difficulties, cognitive problems, pain, and other symptoms it is important to remember that patients are sensitive to medications. The CDC advice to health care practitioners is to be aware that because all drugs can cause side effects, ME/CFS patient symptoms can worsen. "This is particularly true of any medication that acts on the central nervous system, such as sedating medications: therapeutic benefits can often be achieved at lower-than-standard doses. Patients with ME/CFS might tolerate or need only a fraction of the usual recommended doses for medications. After initial management with lower dosing, one or more gradual increases may be considered as necessary and as tolerated by the patient." For instance, tricyclic drugs can improve mood and help with sleep and pain. However, in some in can worsen OI.

Controversy
. Psychiatry has taken an inappropriate lead and treatment path for an organic biological disease, although this is now changing. In the UK, graded exercise therapy (GET) and cognitive behavioral therapy (CBT) were employed by ME/CFS Clinics until 2021. Exercise or stepped increases in activity exacerbates symptoms and were found to harm most patients, so this treatment is now advised against. Depression and anxiety drugs have been utilized, but are ineffective.

Treating a biological disease as a mental illness is not cost effective. The drugs are inexpensive and biomedical research is not pursued. People struck down and disabled with ME/CFS are labeled with a mental health issue; disability insurance having clauses excluding a mental illness kick in stopping any payment or shortening the payout time-frame.

Graded exercise therapy & Cognitive behavioral therapy
GET and CBT are usually employed in the UK, Ireland and some parts of Europe through ME Clinics. They are highly controversial and not recommended by patients, most patient advocates, and research organizations nor by many doctors or researchers outside of the UK. Based on the flawed PACE trial which used the flawed Oxford criteria to diagnose and recruit patients, GET and CBT found its way into treating people with ME/CFS.

Exercise, especially GET, can injure an ME/CFS patient further.


 * Jennifer Brea walked home from her Neurologist's office and became wheelchair bound.
 * This is an experience of serious injury due to GET shared by K. Miles, via Tom Kindlon on Twitter.
 * Emma Shorter was able to walk a few minutes a day until GET put her in a wheelchair.
 * Doctor Speedy was also seriously injured by GET.

Claims of curative treatments
Charlatans claim they can cure CFS (per the CDC "there is no cure" ) when in reality they may be able to treat chronic fatigue (CF). ME/CFS patients experience an ongoing improper immune response and other medical abnormalities for at least 6 months straight with a reduction in activity with a specific symptom set for at least 50% of the time.

Additionally, there have been media reports of some athletes diagnosed with CFS that recovered in a relatively short period of time after rest, supplementation, and diet changes   may have had overtraining syndrome, a condition they sometimes acquire, while some people with adrenal failure or other fatiguing illnesses are sometimes erroneously misdiagnosed with CFS.

Working with government to move forward
Advocates met with senate staffers the week of March 16, 2016, and discussed research and drug development by the NIH, the CDC reinstating and increasing funding and education based on IOM recommendations and the HHS funding to develop Centers of Excellence. #MEAction reports on this ongoing process.

CDC Website updates

 * Jul 3, 2017, the CDC's Chronic Fatigue Syndrome page has been changed to Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and GET and CBT recommendations have been removed.


 * Jul 12, 2018, the "Information for Healthcare Providers" tab and its sub-tabs were updated.
 * Feb 25, 2019, Dr. Elizabeth Unger, Chief of CDC's Chronic Viral Disease Branch (CVDB), that houses the ME/CFS program, in collaboration between Medscape and the CDC put out brief video and commentary page with links to the CDC's July 12th, 2018 updates for medical professionals: Chronic Fatigue Syndrome: It's Real, and We Can Do Better.

MEAction input
 * Jul 29, 2018, CDC Revises its Information on ME #MEAction report on the CDC July 2017 and July 2018 website updates. Recommendations for the CDC's ME/CFS page have been created from patient feedback on the updates as well as asking for further patient input.

Documentary
Films
 * Forgotten Plague is a US documentary about Ryan Prior, an afflicted journalist who investigates the disease and its history and why the CDC has left millions sidelined from life; available for streaming and DVD purchase. (2015)
 * Unrest is a US documentary film that aired on PBS's Independent Lens in 2018 and is available for streaming and DVD purchase. Jennifer Brea, a person with ME, directs. (2017)

Short film

 * The Last Great Medical Cover Up is a UK short Film on Vimeo with several patient interviews. (2015)

News media

 * Chronic Fatigue: Missing Millions (Carte Blanche DSTV) TV report, South Africa (2017)

Talks and interviews

 * Jennifer Brea is interviewed for the upcoming BRIC Flix on her ME/CFS disease experience and her 2017 documentary film Unrest at 5:30 and 10:20 - Youtube (2016)

Government health organizations

 * US


 * Centers for Disease Control (CDC)
 * Factsheets - Pediatric Fact sheets for Healthcare Professionals, Parents/Guardians, and Education Professionals. (2017)


 * National Institutes of Health (NIH)
 * Moving Toward Answers in ME/CFS - NIH Director's Blog (2017)


 * Institute of Medicine (IOM)
 * Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) Key Facts From the Institute of Medicine report. (2015)


 * Beyond Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Redefining an Illness Report Brief on the Institute of Medicine report. (2015)

ME/CFS research foundations

 * UK


 * Invest in ME
 * Frequently Asked Questions


 * US


 * Open Medicine Foundation (OMF)
 * What is ME/CFS?

ME/CFS organizations

 * Australia


 * Emerge Australia
 * What is ME/CFS?


 * UK


 * ME Association
 * What is ME/CFS?


 * Tymes Trust (Pediatric)
 * Pediatric ME, CFS, SEID for Families and their GPs (2016)


 * US
 * Massachusetts CFIDS/ME & FM Association
 * About ME/CFS


 * Solve ME/CFS
 * What, Exactly, is a Biomarker Anyway? And Why Don't We Have One for ME/CFS?


 * Get the Facts about ME/CFS (2016)


 * What is ME/CFS? (2013)


 * International
 * MEAction
 * Caring for People with Myalgic Encephalomyelitis (2018) Guide for Caretakers


 * Facts about Myalgic Encephalomyelitis (ME) (Also called chronic fatigue syndrome or CFS) (2015)


 * Reports and Fact Sheets (2015)

Other organizations

 * National Organization for Rare Disorders (NORD)
 * Myalgic Encephalomyelitis

Other reading

 * What Do You Do When a Loved One Becomes Chronically Ill?

Research avenues

 * Dr. Anthony Komaroff Webinar Hot Areas in ME/CFS Research (2016)
 * Beyond the Data – Chronic Fatigue Syndrome: Advancing Research and Clinical Education CDC Video (2016)

History

 * Osler's Web is a book by Hillary Johnson on the early history of CFS.
 * Thirty Years of Disdain by Mary Dimmock and Matthew Lazell-Fairman picks up on the ME/CFS history where Osler's Web leaves off.

Deaths of ME/CFS patients

 * Causes of death
 * Suicide

Death certificates with ME or CFS
 * Merryn Crofts' death is attributed to ME.
 * Sophia Mirza's death is attributed to CFS.

In the UK, United States, and Australia there are claims loved ones have died due to ME/CFS. See Editor's Note

Notable studies
"Bilateral white matter atrophy is present in CFS. No differences in perfusion were noted. Right hemispheric increased FA may reflect degeneration of crossing fibers or strengthening of short-range fibers. Right anterior arcuate FA may serve as a biomarker for CFS."
 * 2014, Right Arcuate Fasciculus Abnormality in Chronic Fatigue Syndrome
 * 2014, Brains of People With Chronic Fatigue Syndrome Offer Clues About Disorder By David Tuller - New York Times: Well


 * Cytokine ("Notable studies")
 * List of enterovirus infection studies
 * 2016, Metabolic features of chronic fatigue syndrome


 * 2019, Evidence of widespread metabolite abnormalities in Myalgic encephalomyelitis/chronic fatigue syndrome: assessment with whole-brain magnetic resonance spectroscopy
 * 2018, ME/CFS Involves Brain Inflammation: Results from a Ramsay Pilot Study (TALK)

Learn more
Potential treatments and research
 * Ampligen - Used in Argentina for the treatment of severe ME/CFS.
 * Autopsy
 * Brain
 * Vagus nerve infection hypothesis
 * Metabolic trap

Guides and reports
 * Caring for People with Myalgic Encephalomyelitis (2008) Guide for Caretakers
 * "In the Expectation of Recovery" by Centre for Welfare Reform UK

Patient mental health


 * Patients that had been deemed as suffering from mental health and not a biological illness:
 * Karina Hansen is a young Danish woman taken from her family for 3 1/2 years due to Per Fink's insistence her ME was due to mental health issues.
 * Sophia Mirza was taken forcibly from her home to a mental hospital only to be returned shortly after; she then died of ME.
 * Ean Proctor was taken from his parents as a young boy and he was told his "parents were letting him die." He had become paralyzed and mute and was let go in the deep end of a pool to make him swim, and put on a scary theme park ride to scare him into moving.


 * ME/CFS is not depression. It causes a lack of energy, not a lack of desire to do activities.

Outbreaks
 * Incline Village outbreak of 1984 began the research that led to the diagnostic construct Fukuda and name of chronic fatigue syndrome


 * List of outbreaks