A Newsletter About Caring for the High Maintenance Child by Kate Andersen, M.Ed. Editor's Note: Kate Andersen is a parent educator and mother of a difficult child. She writes, lectures and consults with parents about dealing with their spirited children. Kate is listed on the Providers page. Kate also writes a monthly column called 'Ask Kate' to answer individual questions about real children and situations. 'Ask Kate' is now included as part of every issue of BDINews. Send your "Ask Kate" question to bdi@temperament.com. Due to the volume of mail, not all individual questions can be answered in this column. BDINews SPECIAL ISSUE: Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome in Children and Adolescents Published by Behavioral-Developmental Initiatives ISSUE THEME: Behavior Problems Volume 4, Issue 9 - May, 2002 www.b-di.com or www.temperament.com Dear BDINews Subscribers: A reader wrote recently: "I see things in BDINews that I don't hear about anywhere else. Please keep sending me this newsletter". Thanks for the compliment. One reason that the information and approach in this newsletter are unique is that the research on temperament individuality truly alters the standard way of looking at children handed down by decades of work in clinical psychology and psychiatry. These fields, and others, continue to ignore, or fail to fully appreciate, the substantial body of evidence about important temperament differences among children. Another reason that BDINews touches on many intriguing topics is that Sean C. McDevitt, Editorial Consultant, is one of the most open-minded and respectful people I have ever worked with. He has permitted me to include in BDINews observations, insights and syntheses that have arisen from my interesting, if challenging, personal and professional experiences, even if the issue discussed was something that was controversial or not fully researched. This special issue is devoted to myalgic encephalomyelitis (ME), also known as chronic fatigue syndrome, in children and adolescents (CFS). Dr. McDevitt knows that I have a strong interest in ME/CFS, in children and in adults. The reason is simple: I have had ME/CFS since 1983. While the cause of ME/CFS is still being debated, I confess to be biased in favor of the "enteroviral" theories. This is because I fell ill with ME/CFS at precisely the time my infant son was diagnosed with an enteroviral meningitis, a Coxsackie B1 infection. I had a mild "flu" at the time (recorded on his hospital admission chart as "Mom, six days ago, fever, myalgia"), but the symptoms of ME/CFS came on in full over that winter. At the time I had never heard of ME or the Coxsackie virus, CFS had not yet earned its new name, and I had only a vague notion of the possible consequences of meningitis. I had absolutely no idea that an enteroviral meningitis was akin to a non-paralytic polio infection and I doubt whether my child's doctors knew this, either. As a result of my personal experiences, I joined the National ME/FM Action Network of Canada as a volunteer working on behalf of parents and youth. In this role I hear about the terrible effect ME/CFS can have on the life of a child, about the horrendous time that parents have getting their children diagnosed, and about the deplorable lack of education in the fields of medicine, psychology and education about the true nature of this very real illness. The key reason, however, for attention to ME/CFS in this newsletter is that children with ME/CFS may be misdiagnosed as having difficult temperaments, ADD, learning disabilities or other disorders. While differentiating young people with severe ME/CFS from those with behavioral or emotional disorders may be relatively easy, this may not be the case for youngsters with atypical or mild presentations, where the overlap in symptoms may be substantial. Much more research on all aspects of pediatric ME/CFS is needed. Although there remain many unanswered questions, there is a great deal about ME/CFS in children that has been known for more than half a century! I urge the professional readers of BDINews to educate themselves about pediatric ME/CFS. To do this properly they must first pay attention to the pioneering work of Drs. Melvin Ramsay and Elizabeth Dowsett in the United Kingdom. This extensive body of knowledge is being ignored by too many contemporary researchers on "CFS", as well as by diagnosticians. May 12 is International ME/CFS Day Please read this issue and then forward it to someone in a position to help a young person diagnosed with ME/CFS or who can help one who may have been misdiagnosed. Sincerely, Kate LETTER TO KATE Dear Kate: My husband and I were invited to review a book on the special education needs of children with ME/CFS to be published by the National ME/FM Action Network of Canada. We have a twelve year old daughter with ME/CFS. As we read the book, we found new insights into her special education needs. My husband is an educator and had childhood ME/CFS, and while reading the book his own childhood memories surfaced - I now understand him more and my child more. My assumption that I clearly understood my daughter because I also have ME/CFS was appropriately challenged. I now understand better what my child's special education needs and emotional needs are. I am fine tuning my handling of our daughter. I pause and think before I make motherly demands on her, and she is becoming more relaxed and responsive with me as a result. She seems a little warmer in her good moments, a little surer of me in her off moments. I really am talking about the small things: is she able and comfortable to do the one thing I've asked of her? I don't talk about the next task until she is finished and rested. For example, putting away her clothing is important, but not the most important thing at this moment, or today, or even next week. Well, that throws "normal" parenting out the window! "Pause" - that is the word I try to remember now. Pause and notice how she is before intruding on her peace and quiet. Pause and notice what she is doing and assess whether it will be productive to introduce a new element into her activity, or leave her alone with it. Pause and delight in something that delights her, no matter how small, because it is the delight that is so important. Pause and remember how much we love her. I engage with her but with a lighter touch, letting her see my happiness when she is enjoying herself, but not overpowering her with my own emotions or anxieties. Happiness is harder to come by when you have a chronic illness, so I want to be sure to allow her ownership of her own joy. I think she feels more acceptance from this, and there is more closeness growing between us. Delicacy is what comes to mind. The same goes for her other emotional moments. ME/CFS has been hard for her. She is strong, but tender in her present state, a flower that has closed in on itself and needs to be gently and carefully coaxed to open again. A person needs to know how to help this process, or get some guidance in how to help, so as not to cause any bruises or further hurt. I am willing and needing to learn for her sake. As I better understand her special needs, I'm finding her again. I have faith and trust that she will find herself again, the self that includes feeling intelligent, capable and unique. It can be hard to accept the situation: denial is easy for the parent and tough on the kid. Adjusting academic expectations downwards has felt tragic and terribly unfair. A better grasp of things has helped me to see that it is not a lowering of expectations, but a change in expectations, and that our daughter's natural intelligence, sensitivity and wit are all still there, just needing special help and lots of nurturing and acceptance from us. We, together, can overcome. This wonderful book has been a blessing in understanding and assisting our child. I'm very much looking forward to seeing it published so other parents may be benefited as well. Loving Mother Kate's Answer Dear Loving Mother, Thank you for your letter. You validate our key message at BDINews that parents need to adjust their expectations to fit the child they have, not the child they dreamed of having. To make a major adjustment of expectations but still see the enormous potential and gifts our children have is the essence of love itself. Thank you for your very moving letter. Sincerely, Kate P.S. This parent is referring to "A Sourcebook for Teachers of Children with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome and Fibromyalgia" being published by the National ME/FM Action Network of Canada. See the announcement below. TEMPERAMENT, ME/CFS, POLIO, and POST-POLIO SEQUELAE: Uncovering Hidden Brain Stem Connections. An Interview with Dr. Richard L. Bruno, by Kate Andersen In his new book, The Polio Paradox: Uncovering the Hidden History of Polio to Understand and Treat "Post-Polio Syndrome" and Chronic Fatigue, Dr. Richard Bruno describes his twenty year journey studying the cause and finding treatments for Post Polio Sequelae, the unexpected and often disabling symptoms -- overwhelming fatigue, muscle weakness, muscle and joint pain, sleep disorders, heightened sensitivity to anesthesia, cold and pain, and difficulty swallowing and breathing -- that occur about 35 years after a poliovirus attack. BDINews readers may be wondering how a book about polio is relevant to this newsletter, and particularly this issue. Read on! Kate : Many people think polio is over, thanks to vaccines, and we can forget about it. Is polio is over? Dr. Bruno : Unfortunately, polio is not over. Beside the thousands of cases in India and Africa, oral poliovirus vaccine (OPV) strains can mutate and cause paralytic polio, as we have seen in the Caribbean during the past year. With poliovirus mutation those in a community who are not vaccinated are at risk for getting paralytic polio. Since many North American parents believe polio is over they don't vaccinate their children. One study estimated that only 50% of American children receive all three doses of the OPV. If a child from the Dominican Republic carrying the mutated poliovirus came to New York last December, there could have been a large polio outbreak. Poliovirus is now being considered a possible biowarfare agent because US vaccination rates are so low. So polio may be forgotten but it is far from gone. Parents must insure their children's vaccination. Kate : What is the relationship between polio and ME/CFS? Could the poliovirus be causing chronic fatigue today? Dr. Bruno : There is no evidence that either the naturally-occurring poliovirus or OPV vaccine strains cause ME/CFS. But almost eliminating the natural supply of poliovirus through vaccination may cause ME/CFS, indirectly. Something unexpected, frightening and totally unrecognized happened after the polio vaccine was distributed: the number of cases of ME went through the roof. British ME pioneer Dr. Elizabeth Dowsett reviewed the 2,500 ME patients she and Dr. Melvin Ramsay had seen since 1919, and plotted the cases of ME against reported cases of polio. When the Salk and Sabin vaccines brought the yearly number of British polio cases below 25 in the early 1960s, the number of ME patients took off. In Ramsay's and Dowsett's practice alone, between 1960 and 1980 the number of ME patients increased by fifty fold. Between 1980 and 1990, the number of patients with ME increased yet again by a factor of fifty! Throughout the world, 32 ME outbreaks were recorded after the polio vaccine was distributed. Kate : How would the lack of poliovirus cause ME/CFS? Dr. Bruno : Dr. Dowsett thinks that the elimination of poliovirus left a vacuum that had to be filled by another enterovirus -- a virus that multiplies in your intestines -- similar to the poliovirus that also damages the brain activating system. In 1990, Dr. Dowsett looked for antibodies to non-polio enterovirus in her ME patients. Fifty percent had antibodies to the first non-polio enterovirus ever discovered, the Coxsackie B virus, named after Coxsackie, New York, the town where it was found to have it paralyzed children in 1948. Yes, paralyzed. It's not just the polioviruses that enter and kill neurons in the spinal cord and brain. Other non-polio enteroviruses that cause damage and symptoms similar to the polioviruses include all the other Coxsackie viruses, the ECHO viruses and Enteroviruses 71. So there are many viruses that could take the place of the poliovirus, damage the brain activating system -- the brain stem and subcortical neurons that activate the brain and focus attention -- and cause ME/CFS in children and adults. Kate : Your research on fatigue in adult polio survivors shows that their brain activating systems are damaged. Is there similar damage in those with ME/CFS? Dr. Bruno : We and other researchers have found remarkable similarities between the signs and symptoms of post-polio brain fatigue and ME/CFS. The lesions of the brain on MRI, attention deficits on neuropsychologic testing, reduced levels of the brain activating hormones ACTH and dopamine, and brain wave slowing on EEG are identical to abnormalities seen in patients with ME/CFS. So we think brain activating system damage is the cause of fatigue in both polio survivors and those with ME/CFS. Kate : Are these findings the same in children with ME/CFS? Dr. Bruno : We did the first study of the psychophysiology of young people reporting chronic fatigue using the same techniques we used to test polio survivors with fatigue. We evaluated thirty-eight young people reporting fatigue and studied thirteen subjects, who were on average 16 years old and met the 1994 Centers for Disease Control diagnostic criteria for Chronic Fatigue Syndrome. We also studied 10 non-fatigued control subjects. We gave 12 neuropsychologic tests of attention and did EEGs and found three things. First, 75% of the young people with CFS had no psychiatric diagnoses at all, not even an adjustment disorder with depressed mood. This finding disproved the notion that ME/CFS is a psychiatric disorder that could not and should not be diagnosed in children. Second, subjects' reports of difficulty staying awake during the day and difficulty concentrating -- the same symptoms reported by adults with ME/CFS and polio survivors with fatigue -- uniquely predicted the diagnosis of CFS, and difficulty staying awake during the day, concentrating and focusing attention were significantly correlated with daily fatigue severity. Third, scores on four of the neuropsychologic tests of attention were clinically abnormal in the CFS group, while scores on two additional tests of attention were statistically significantly lower in the CFS subjects and significantly correlated with daily fatigue severity. There was no difference in EEG between the two groups, probably because the age span of the subjects caused their differing basal EEG frequencies to obliterate changes related to ME/CFS. So in terms of symptoms and impaired attention - signs of brain activating system damage - ME/CFS kids looked just like adults with ME/CFS and fatigued polio survivors. Kate : So what is the treatment for children with ME/CFS? Dr. Bruno: It's the same as for adults with post-polio fatigue and ME/CFS: "The Golden Rule - If anything causes fatigue, weakness or pain DON'T DO IT! (Or do much less of it.) Dr. Ramsay himself said, "The basic fundamental tenet of the management of a case of ME is REST with graduated activity well within the limitations which the disease imposes." Unfortunately doctors choose to ignore more than 80 years of research suggesting that ME/CFS are the result of brain activating system damage and are likely caused by one or more of the enteroviruses. Even more than polio survivors with PPS, those with ME/CFS have been dismissed as lazy, crazy or outright liars by the medical community. It's time that doctors read the medical literature on these conditions and discover that PPS and ME/CFS are medical - not mental - illnesses. Kate : You said 75% of the young people with ME/CFS had no psychiatric diagnoses. What about the 25% that had mental illnesses? Dr. Bruno : Before discussing "mental illness" in the same breath with ME/CFS we need to remember that people with ME/CFS are very sensitive to and angered by being told they have a psychiatric diagnosis, and rightly so. Until just this year, ME was dismissed in the UK as a form of depression, the treatment for which was medication, cognitive behavioral therapy and graded exercise, basically "Mind Over Matter:" If you don't mind, fatigue won't matter. Fortunately, this notion has been discarded by the 2002 British Chief Medical Officer's report on ME. Kate : Do some people with ME/CFS have psychiatric problems? Dr. Bruno : Some have psychiatric problems, as do others with disabling medical illnesses. Fifteen percent of our patients at The Post-Polio Institute have a major depressive episode that occurs when their PPS symptoms prevent them from functioning. But no one would now tell polio survivors they don't have fatigue, muscle weakness and pain because they are "just depressed." Depression in polio survivors results from disability, not the other way around. Kate : Was it only depression you found in the 25% of young people with ME/CFS who had psychiatric diagnoses? Dr. Bruno : No. But the kids with psychiatric diagnoses other than depression did not have ME/CFS. There was Munchausen's by proxy, factitious disorders and schizoid personality disorder, which raises important points about diagnosis and treatment. Just because young people report chronic fatigue doesn't mean they have ME/CFS. ME/CFS is still a diagnosis of exclusion and both psychiatric disorders and medical causes for symptoms must be excluded. And you can exclude psychiatric diagnoses through clinical evaluation and testing. A higher percentage of subjects reporting chronic fatigue and having those psychiatric diagnoses also complained of moderate to severe muscle weakness, which is not a symptom of ME/CFS. We found that these patients also had low scores on the Sensitivity to Criticism and Failure Scale of our Reinforcement Motivation Survey. We have found identical low sensitivity scores in inactive chronic pain patients who don't get better. What's more, neuropsychologic tests scores in those with these other psychiatric diagnoses showed moderate to severe impairments, while the ME/CFS kids were mildly to moderately impaired. This suggests that those with psychiatric diagnosis were allowing themselves to score poorly, their scores being in the brain injured range. If these kids had been that severely impaired they wouldn't have been able to carry on a fluent conversation during the clinical evaluation, and all could. Kate : BDINews talks a lot about self-regulation being a critical developmental task and is focused on helping parents and others accept and work positively with children's individual differences. What do your findings and clinical experience say about self-regulation and dealing with chronic fatigue in children? Dr. Bruno : Self-regulation is the heart of managing chronic fatigue in young people as well as adults. It is the responsibility of parents to help kids with chronic fatigue to self-regulate and to follow "The Golden Rule." Kids with ME/CFS are often on what we call the "roller coaster" - working, being very active and then crashing in bed for days. Kids must even-out their activity, pace and conserve energy, as Melvin Ramsay said, "REST with graduated activity well within the limitations which the disease imposes." If they do, chronic fatigue symptoms and disability decrease while ability increases. It is sad to say that we have had no success in treating the kids with chronic fatigue plus those psychiatric diagnoses. The stumbling block is always parents' unwillingness or inability to set limits for their children, to help them to self-regulate and to reinforce them for increasing activity within their limitations. Not one of these patients improved and all were allowed by their parents to stop treatment. Kate : Does your new book, The Polio Paradox: Uncovering the Hidden History of Polio to Understand and Treat "Post-Polio Syndrome" and Chronic Fatigue, have anything to offer early intervention professionals, pediatricians and parents of children exhibiting difficulties with fatigue, attention, sleep and mood? Dr. Bruno : The Polio Paradox presents a comprehensive program to help those with chronic fatigue from any cause to follow "The Golden Rule," change their behavior, treat their own symptoms and take back their lives. The book also describes the long parallel history of polio and ME/CFS since 1934 and shows how ME/CFS and Post-Polio Sequelae are two sides of the same coin, both being associated with similar damage to the brain activating system. Kate : Speaking of the brain activating system, do you agree with my theory there might be a relationship between hidden brain stem injury from a "silent" or disregarded viral infection early in life and "temperament" differences in children affecting activity level, mood, intensity of reaction, sensory threshold, adaptability, distractibility, persistence and attention span? Dr. Bruno : I certainly do. You can construct a simple (more like simplistic) model of temperament based on prenatal or early-life damage to the reticular formation, the part of the brain activating system within the brain stem, plus the brain stem neurons that produce dopamine, the principal brain activating neurochemical. Take a finding of Jerome Kagan's group. Children classified as high reactive at four months old, who tend to become inhibited or fearful young children and adult introverts, have a faster brain stem auditory evoked response than do low reactive children, who tend to become extroverts. So the more "active" the brain activating system and the more "reactive" the brain, the more likely kids are to feel hyperstimulated and unpleasantly overloaded; therefore, the more likely it is they will "shy away" from environmental stimulation and be less physically active. Taken to the extreme a hyperactive brain activating system due to too much dopamine could be responsible for profound withdrawal from stimulation and result in autistic behavior and the negative symptoms of schizophrenia. The opposite might also apply. Those with an hypoactive brain activating system and a low reactive brain would be more tolerant of stimulation. Their brains might even want more stimulation, causing them to be more eager to explore their environments. Taken to the extreme, a hypoactive brain activating system due to too little dopamine could be responsible for attention deficit hyperactivity disorder and explain why ADHD symptoms decrease with drugs that increase dopamine, stimulate the brain activating system and the entire brain. Kate : So increased brain activation would cause some children to be less active, while decreased brain activation would cause increased activity. Your findings in kids with CFS show decreased brain activation and decreased physical activity? How would that happen? Dr. Bruno : To explain that you need a more complicated model. The brain activating system is more than just the brain stem reticular formation. The BAS includes the basal ganglia, which regulate both stimulation of the brain and physical movement, also as a result of the release of dopamine. The reticular formation, dopamine neurons and basal ganglia are damaged in polio survivors and patients with ME/CFS. Simultaneous damage to the reticular formation, dopamine neurons and basal ganglia would decrease stimulation of the brain, cause symptoms of fatigue -- difficulty staying awake and focusing attention -- plus actually inhibit physical activity. The combined activity of reticular formation and basal ganglia neurons is what we call the "Brain Fatigue Generator." You have to remember, too, that while chronic fatigue is abnormal, the generation of fatigue after activity is both normal and important for survival. Animals must have a Brain Fatigue Generator. An animal that continues to explore its environment even though it is fatigued and its attention is impaired would be less able to direct and sustain attention on the goal of its exploration, like searching for breakfast, and would waste already diminishing energy stores. More importantly impaired attention could also render an animal unaware of or slow to respond to dangers in its environment, like a predator stalking the animal in search of its breakfast. There is important survival value in a Brain Fatigue Generator that forces an animal to feel fatigued, to stop moving and promotes sleep when attention is impaired. In a way, chronic fatigue syndrome is too much of a good thing. Kate : So whether it's ME/CFS, Post-Polio Sequelae, ADHD or shyness, the brain stem and subcortical areas are playing an important role. Dr. Bruno : Yes. I think the brain stem and "lower" brain areas like the basal ganglia are overlooked when people think about behavior. And prenatal injury, causing decreased oxygen to the brain, or the same viruses that we think may cause ME/CFS could damage the brain areas and may be a cause of temperament differences as well as ADD and ADHD, not to mention autism and schizophrenia. Central nervous system infections during childhood are being associated with an increased risk of adult onset psychoses, Coxsackie B5 virus infections in newborns in particular being associated with schizophrenia. But whatever is going on in the brain -- and wherever it's going on -- the focus of parents and professionals must be on each child's individual temperament and special needs. As our work has shown, even when treating something as seemingly homogeneous like "chronic fatigue," one size does not fit all. Dr. Richard Bruno is Director of Fatigue Management Programs and The Post-Polio Institute at Englewood (NJ) Hospital and Medical Center. His new book, The Polio Paradox: Uncovering The Hidden History Of Polio To Understand Treat "Post-Polio Syndrome" And Chronic Fatigue, will be published by Warner Books in June. (Go to AOL Keyword POLIO PARADOX). E-mail questions to him at PolioParadox@aol.com. REFERENCES Dr. Bruno's articles can be found in Post-Polio Library at: http: //members.aol.com/harvestctr/pps/lib2.html Bruno RL. The Polio Paradox: Uncovering the Hidden History of Polio to Understand and Treat "Post-Polio Syndrome" and Chronic Fatigue. Warner Books, June 2002 Bruno RL, Zimmerman JR. Rehabilitation of CFS: A multidisciplinary, behavioral approach. In J. John and J. Oleske (Eds.) A Consensus Manual for the Primary Care and Management of Chronic Fatigue Syndrome. Trenton: The Academy of Medicine of New Jersey, 2002. Bruno RL. Paralytic versus "non-paralytic polio: " A distinction without a difference? American Journal of Physical Medicine and Rehabilitation, 1999; 79: 4-12 Bruno RL, Creange SJ, Frick NM. Parallels between post-polio fatigue and chronic fatigue syndrome: A common pathophysiology? American Journal of Medicine, 1998, 105 (3A): 66-73. Bruno RL, Creange SJ, Zimmerman JR, Frick NM. Elevated plasma prolactin and EEG slow wave power in post-polio fatigue: Implications for a dopamine deficiency underlying chronic fatigue syndromes. Journal of Chronic Fatigue Syndrome, 1998; 4: 61-76. Bruno RL. The psychophysiology of chronic fatigue in young people. Invited presentation, N.I.H. State of the Art Workshop on Chronic Fatigue in Adolescents. Washington, D.C.: April, 1998. Bruno RL. Chronic fatigue, fainting and autonomic dysfunction: Further similarities between post-polio fatigue and Chronic Fatigue Syndrome? Journal of Chronic Fatigue Syndrome, 1997; 3: 107- 117. Bruno RL, Frick NM, Creange SJ, et al.. Polioencephalitis and the brain fatigue generator model of post-viral fatigue syndromes. Journal of Chronic Fatigue Syndrome, 1996; 2: 5-27. Bruno RL, Zimmerman JR, Creange SJ, et al... Bromocriptine in the treatment of post-polio fatigue: A pilot study with implications for the pathophysiology of fatigue. American Journal of Physical Medicine and Rehabilitation, 1996; 75 (5): 340-347. Bruno RL, Sapolsky R, Zimmerman JR, Frick NM. The pathophysiology of a central cause of post-polio fatigue. Annals of the New York Academy of Sciences, 1995; 753: 257-275. Bruno RL. Predicting hyperactive behavior as a cause of non-compliance with rehabilitation: The Reinforcement Motivation Survey. Journal of Rehabilitation, 1995; 61 (2): 50 - 57. Bruno RL, Frick NM, Cohen, J. Polioencephalitis, stress and the etiology of Post-Polio Sequelae. Orthopedics, 1991; 14 (11): 1269-1276. Woodward SA, McManis MH, Kagan J, et al. Infant temperament and the brainstem auditory evoked response in later childhood. Dev Psychol, 2001; 37:533-538. Rantakallio P, Jones P, Moring J, Von Wendt L. (1997). Association between central nervous system infections during childhood and adult onset schizophrenia and other psychoses: a 28-year follow-up. International Journal of Epidemiology, 26(4), 837-843. Parenting a Child with ME/CFS Mary Z Robinson Up until around 1994, my life as a parent was pretty much the same as all parents. I was at home with 3 young children enjoying the everyday activities that parenting brings. We liked going for long walks, going camping, and playing with the neighborhood kids outside until it was time to come in for baths and bed. It was a very active lifestyle where we tried to keep the TV off and physical activity on. A child's scraped knee could always be cured with a kiss and a hug from a loving mom. When my son turned 5 it was time to venture out of our cozy cocoon at home to kindergarten. He loved school and the new friends he made. So it was hard to understand why there were days when he seemed to not want to go complaining of headaches, and sore throats and stomachaches. I would often send him off to school telling him to just "tough" it out. The doctor thought it was related to allergies or maybe school phobia. The years passed and my son was treated for allergies and the symptoms seemed to come and go. Then in the 4th grade they got much more severe and a thorough medical workup was required. He was poked and prodded and scoped and scanned and nothing unusual could be found. He had his tonsils out, but that didn't seem to make him any better. By age 10 he had begun a pattern of symptoms where he would become ill in the fall after enjoying 4 months or so of pretty good health and regress to practically being bedridden by Christmas. He would struggle through the winter with traveling muscle and joint pain, headaches, sore throats, stomach pains, cognitive problems, and a severe limitation to his activity. We did all we could to keep his schoolwork up to date. Then come May he would start to slowly improve and by June he was able to enjoy a little bit more outdoor activity and time at school. He was officially diagnosed with ME/CFS by Dr. David Bell in the beginning of 6th grade. He had gone from being a very active elementary aged child who loved baseball, playing the trumpet and riding his bike to a child who could barely be off the couch for more than 2-4 hours total a day. The lights in the room always needed to be dimmed due to his extreme sensitivity to light. The school provided a home tutor and a special education program and I felt like my life had taken a turn I never dreamed it could. About this time, my youngest child, then about aged 4 to 5 began exhibiting many of the strange symptoms her brother had experienced. I remember sitting by her bed one night as she finally drifted off to sleep in agony from the pain, and I cried. I asked God to not let another of my children be stricken with this awful sentence to a painful childhood, but by age 6 it was confirmed that this prayer would not be answered. My youngest child was diagnosed in first grade as also suffering from ME/CFS. She too would attend school part time at first, but by age 7 was mostly homebound and suffering greatly from the pain and limitations the illness imposed. Dr. Bell was very concerned by the severity of the children's illness and how they seemed to be getting worse with each passing year. In attempting to get help from someone that would help explain my son's condition he was led to a wonderful doctor in Syracuse who would prove to change the course of Dr. Bell's research into this mystifying illness. Dr. David Streeten was a leading researcher in the USA of a condition known as Orthostatic Intolerance or dysautonomic conditions. He discussed doing a relatively new procedure on my son that intrigued Dr. Bell. He proposed testing him for Orthostatic Intolerance and also measuring his blood volume. The test for OI was done by having my son stand perfectly still for as long as he could without moving at all. During this time, Dr. Streeten continually monitored his pulse and blood pressure. As the minutes passed my son became very symptomatic and as his BP dropped and his pulse quickened he felt very faint and ill. Dr. Streeten had him lay down where his symptoms subsided and his vital signs returned to normal. Dr. Streeten explained to us that his condition is one he commonly saw in his studies. He said that for whatever reason when some people stand, such as my son did, the blood would pool in the extremities. He felt that there was possibly a malfunction of the veins in the legs where they could not contract to send the blood back to the heart and to the head. This pooling of blood caused the heart to pump feverishly trying to get the blood to return and the lack of blood flowing caused the BP to drop. He also did a blood volume study and found my son's volume to be about 60% of what it should be. He proposed a course of treatment that would attempt to expand the blood volume while also attempting to get the veins in the legs to contract. My son began the new medicines and within a year had substantially improved in every way. By 1? years after seeing Dr. Streeten he was back to normal activity and a full day at school with sports and high academic honors. This experience led to collaboration between Dr. Bell and Dr. Streeten to study a multitude of patients diagnosed with ME/CFS to see if they also suffered from the orthostatic problems Dr. Streeten studied. They published several studies on blood volume irregularities in patients with ME/CFS and were very excited by the direction their research was taking when Dr. Streeten suffered an untimely passing. Dr. Bell continues to feel that the orthostatic problems that Dr. Streeten studied are a large part of the illness we call ME/CFS. He tested my daughter when she was 8 and she too suffered from the same problems and fits the criteria for a condition known as POTS, which stands for Postural Orthostatic Tachycardia Syndrome, which is basically what my son suffered from also. But in my daughter's situation the treatment that gave my son his health back has had no effect on her. Dr. Bell said of all the people he has tested 50% fit the criteria for POTS while all of these ME/CFS patients suffered some form of orthostatic dysfunction. My daughter just turned 12 and suffers on a daily basis with a multitude of symptoms. At her worst she must totally rely on a wheelchair to get around as she is unable to bare any weight on her legs. If she stands for too long (often only 15 to 30 seconds) she may fall to a faint on the floor. She can manage only a tiny bit of tutoring a day, and even that is done reclined on the couch. She daily must endure headaches, muscle aches and pains, and the isolation that having this illness brings. During her good spells she is able to have friends over to play, she can attend her dog club meetings and in the summer improves enough to add horseback riding to her physical therapy regime. What sustains her is a tremendous and powerful faith that she has a reason for this suffering and that one day its purpose shall be made clear to her. In the meantime she maintains a mostly upbeat attitude, thankful for what she does have. She has a loving family, a few good friends, some great kids online she connects with, and her animals. She gives me strength also. We both find comfort in helping other parents and kids deal with the struggles this illness brings. We feel a camaraderie in being able to share our story and knowing we are not alone. It has been a long time since my kids played wildly for hours together outside, without a care in the world. It was a lifetime ago that my "mother's kisses" could heal their pain, but we endure and we go on. We pray that the doctors and researchers that are working to understand these illnesses will soon find some answers for us all. We pray that more doctors who have been unwilling to learn about these conditions will pay attention to their patients, believe them and join the fight to find answers to the many questions surrounding these illnesses. Mary Robinson is an American educator and research assistant for Dr. David S. Bell and is the co-author of a follow-up study of children with ME/CFS published in Pediatrics in 2001. She is one of the co-authors of a book with Dr. Bell entitled, A Parents' Guide To CFIDS: How To Be An Advocate For Your Child With Chronic Fatigue Immune Dysfunction Syndrome and for 3 years was and editor and co-author of a bimonthly newsletter with Dr. Bell for CFS/ME patients. Mary has 3 children, 2 of whom have been stricken with CFS/ME. She counsels parents on how to work with their schools to get the best educational plan for their child and provides parenting support. She can be contacted at maryz@rochester.rr.com TEACH-ME: A Sourcebook for Teachers of Children With ME/CFS and/or FMS An Introduction by Mary Ellen, Manager, Special Projects, National ME/FM Action Network of Canada and former secondary teacher, now disabled with ME/CFS This Sourcebook offers information and educational principles to teachers who may have students with Myalgic Encephalomyelitis (ME), also known as Chronic Fatigue Syndrome (CFS) and/or Fibromyalgia Syndrome (FMS). The authors are Canadian teachers, disabled with ME/CFS and/or FMS, who are members of the National ME/FM Action Network. We developed a unique e-mail conference, known as TEACH-ME, in which we discussed ways that children with ME/CFS and/or FMS could be provided with uninterrupted, high quality education. As people with ME/CFS and/or FMS ourselves we were devastated to imagine the effects of ME/CFS and/or FMS on young people. As teachers, we remained unwaveringly committed to the importance of providing ongoing and enriching education to all children, no matter how ill or disabled. Yet, as adults who were homebound by the illness, we knew this could not be an easy task for the young person or for the classroom teacher. We wanted to use our combined expertise in teaching and experience of the illness to help these courageous youngsters. We were very privileged to have the consultation of Dr. David S. Bell and Mary Z. Robinson. Dr. Bell is a renowned pediatrician and the leading researcher on ME/CFS and/or FMS in children. Ms. Robinson is a US educator, research assistant to Dr. Bell, co-author of "A Parent's Guide to CFIDS", and parent of two young people with ME/CFS and FMS. Our collaboration with Dr. Bell and Ms. Robinson enabled us to link our personal experience and teaching expertise with the most up-to-date and sound international research knowledge. Not only that, but Dr. Bell's and Ms. Robinson's knowledge of ME/CFS and FMS is unique in that it has been acquired from following real families and children coping with this illness over many years. We are also greatly indebted and make much use of the pioneering work of many other researchers. Parents and young people with ME/CFS also made important contributions by sharing their real experiences. This book has international applicability, with very little content that is specific to Canada. Cost: to be announced. To order a copy of the Sourcebook, contact: The National ME/FM Action Network 3836 Carling Avenue Ottawa, Ontario, Canada K2K 2Y6 Tel & Fax: (613) 829-6667 Email:ag922@ncf.ca Please put SOURCEBOOK in the subject line of the e-mail. MORE BOOKS!! A Book About Coping For Young People With ME/CFS Zoe's Win, by Jane Colby. Published by Dome Vision. ISBN 0953733009. Order from Young Action Online, PO Box 4347, Stock, Ingatestone, CM4 9TE. For further information, contact Jane Colby at Tymes Trust on +44 1245 401080 or e-mail jane@youngactiononline.com . Zoe's Win can also be ordered from http://www.amazon.co.uk This is a wonderful book of hope and practical solutions for young people with ME/CFS. Young people reading it will have a better grasp of their symptoms and will learn ways of coping with them. A Book For Parents A Parent's Guide To CFIDS: How to Be an Advocate for Your Child with Chronic Fatigue Immune Dysfunction Syndrome, by David S. Bell, Mary Z. Robinson, Jean Pollard, Tom Robinson, Bonnie Floyd. Available from the CFIDS Association of America; call 704/365-2343 to order. The price is $18 plus $4 shipping & handling. This book is the starting-point for parents with a child just diagnosed with ME/CFS. It gives parents the encouragement they need to become their child's advocates. There's lots of great advice for maintaining balance in the family and working with schools. ME/CFS in Children and Adolescents by Kate Andersen Myalgic encephalomyelitis, also known as chronic fatigue syndrome and CFIDS, is not a new illness. Most historical accounts of the emergence of the term "ME" (myalgic encephalomyelitis) point to outbreaks of a similar set of illnesses referred to by various terms. The authors of a British report state: An illness with clinical features of muscle pain, neurocognitive problems and exercise-induced fatigue has existed for centuries. In the 20th century, epidemic outbreaks were recorded from 1934 until the 1950s. Earlier names included 'English sweats', epidemic neuromyesthenia, and 'atypical polio'. The term myalgic encephalomyelitis (or ME) was coined in 1956, (Ramsay, 1985) following a series of world-wide outbreaks, and ME has been well documented in children. When seemingly new cases of a fatiguing illness began to appear in large numbers in the 1980's, a group of U.S. researchers developed a new term, chronic fatigue syndrome (CFS) and a slightly different set of diagnostic criteria from the original criteria for ME. These new criteria, along with the new name, have led to a state of confusion and a body of recent research with messy and difficult-to-interpret results. As a result of this state of affairs, a prominent group of researchers is asking tough questions about the samples used in research on CFS and is insisting that we all pay attention to the substantial body clinical knowledge and research on ME accumulated in earlier decades (De Becker et al., 2000; Dowsett, 2001; Hyde, 1998). Dr. Byron Hyde (1998), a Canadian ME/CFS researcher and physician has stated: Definitions are not diseases, they are often simply the best descriptions that physicians and researchers can offer, with their always imperfect knowledge, to describe a disease. Good definitions are good because they correspond closely to the disease state being described. It is thus important that those that attempt to define any disease or illness to have long term clinical experience with patients with this illness. There is simply no place for the bureaucrat in defining illness. All definition of epidemic or infectious illness must be based upon persistent clinical examination of the afflicted patient, an understanding and exploration of the environmental factors producing that illness, and pathophysiological examination of tissue from those patients. For similar reasons, I believe that the inclusion of psychiatrists in the defining of an epidemic and obviously disease of infectious origin simply muddies the water for any serious understanding of that disease. The UK definition of CFS was developed by a panel of physicians who were primarily psychiatrists, with few if any clinicians who had ever looked at an epidemic of CFS. A serious attempt must be made to look at epidemic disease as and where that disease starts. This has not been done by those who have defined CFS in the USA nor in the United Kingdom and this factor alone is probably the single greatest reason why we know so little about CFS today that we did not know in 1984 Along with other misconceptions about the so-called new illness, CFS, some assumed that lifestyle factors of the past two decades were associated with developing it. The inaccurate and offensive term for CFS, "yuppie flu," arose from the fact that one of the big outbreaks of the illness in the 1980s occurred at Lake Tahoe, a resort where many young and upwardly mobile professionals take vacations. However, parallel with this epidemic was one in Lyndonville, New York, in which children were the first to be diagnosed with ME/CFS and where adult patients were often poverty-stricken. In spite of what was really happening, the media latched on to the misconception that ME/CFS affects overachieving young professionals and since then it has been difficult to get the true facts across to doctors and the general public. Regardless what name you give the illness, ME/CFS is not more likely to be experienced by any particular social group or by adults in their twenties. On the basis of a major epidemiological study conducted in the United States, the CFIDS Association of America has reported: "CFIDS does not discriminate. It strikes people of all age, racial, ethnic, and socioeconomic groups" (CFIDS Association, 2002). A recent British study of children found cases of ME/CFS in all social classes (Speight et al., 2001). How Many American Children Have ME/CFS? There are no reliable figures of the number children and adolescents with ME/CFS in the U.S. One attempt to ascertain the prevalence of CFS in the United States found that CFS was clearly present among adolescents, but that the prevalence was lower than for most adult age groups. The prevalence in the United Kingdom is discussed in the article below by Dowsett and Colby, Bell (2001) cited an Australian study which reported 5.5 CFS cases per 100,000 children up to age 9, and 47.9 per 100,000 in ages 10-19 (Lloyd et. al., 1990) Female:Male Ratio ME/CFS is more commonly diagnosed in girls. In Speight et al.'s (2001) study the female to male ratio was 2.5:1. In Krilov et al.s' (1998) follow-up study 71% were girls and in Bell at al.'s (2001) follow-up study the percentage was very close, with girls representing 68.6% of the sample. The disregard of decades of early research and clinical experience means there is not much good information readily available for doctors through the usual channels. A Medline search of work conducted in the past two decades is likely to bring up a series of confusing papers, most of which are of very poor quality and which have wholly misleading conclusions and recommendations. This shocking situation leads to many misdiagnoses and erroneous treatments. One parent reported the following about her daughter, who was missing school a great deal because of unexplained flu-like episodes: "I became dissatisfied with the family doctor always brushing off Karen's health problems and requested a referral to a pediatrician. During this office visit, Karen sat unresponsive, staring into space. 'She's just not with us," commented the physician. He pointed to her budding breasts and stated: "I think this is what's going on. She's entering puberty.' Then, when Karen left the room to dress, he stated his skepticism about the existence of ME as an illness, commenting 'After all, I am very tired, too". He said this to me, even after I had told him that I had ME myself! Then he referred Karen for a study of her sinuses and when the results came back negative he wrote a report to our family doctor suggesting that Karen had psychological problems." Karen's mother reported to me that a year or so after this visit, after missing more and more school and failing her courses, Karen became suicidal. "We immediately asked for a referral to specialists who knew about ME/CFS. The immunologist, an adolescent psychiatrist, and a clinical psychologist then combined the results of their evaluations and determined that Karen had mild, atypical, insidious-onset chronic fatigue syndrome with a reactive depression. Alternate schooling was arranged and Karen's depression began to subside but her fatiguability and cognitive problems remained. Over several years, Karen's health and grades began to improve and she graduated from her alternate school with honors. Although Karen is a happy and active young adult today, she has never forgotten the day that that insensitive pediatrician pointed to her breasts. She told me that she found it extremely humiliating and has hated visiting doctors ever since that time. She also remembers, with great anger, the school counselor who accused her of having 'an attitude problem' and the teacher who, noting her extreme forgetfulness, asked her if she was 'retarded or something'". Fortunately, things have changed somewhat in the years since Karen was diagnosed, and there is a growing international agreement about the reality of ME/CFS as a serious, potentially very disabling illness. This situation should lead to fewer instances of educational and medical mistreatment of young people like Karen. Still, families have a difficult - sometimes nightmarish - time getting the help they need for their child. The ill child has an even more difficult time. Seeing how the illness begins sheds light on some of the problems obtaining a clear diagnosis. When do parents first think that something is wrong with their child's health? Although children as young as five years of age have been diagnosed with ME/CFS, the average age of onset appears to be about 11 or 12 (Bell et al., 2000; Speight et al., 2001). Dr. Bell's (1997) research has demonstrated that onset of CFS in children and adolescents can be acute or insidious. In their 2001 follow-up study, Bell et al. reported that 77.1% had a gradual onset. In Speight et al.'s (2001) study the onset was insidious in 38/49 cases. In Krilov et al.'s (1998) study, 60% had acute onset. When the illness comes on suddenly, it usually follows a virus-like episode and, on occasion, the virus involved is identified. At first, everyone expects the young person to recover from this so-called "flu", but often the youngster just does not get better. He or she may return to school only to fall ill again, a pattern that can be repeated for months until a diagnosis is obtained. Some develop the symptoms after a trauma, such as spinal injury. A few youngsters become extremely ill and are even hospitalized. A few never go back to school. And then, there are cases that come on gradually. A child starts to have "too many" bouts of "the flu" or periods of feeling unwell. Headaches that don't respond to painkillers often become part of the picture; muscle pain can be excruciating; dizziness may be debilitating. Symptoms such as forgetfulness, difficulty comprehending what people are saying and expressing oneself, difficulty doing simple calculations, inability to concentrate, heightened sensitivity to sound, smells and chemicals, and mood swings or episodes of weeping, can become part of the picture. The symptom complex is described in greater detail below. The British ME/CFS pioneer, Dr. Elizabeth Dowsett states: It is important to remember that ME/CFS is a syndrome (a linked group of symptoms) which usually follows a common and apparently trivial virus infection (often described as a self-limiting respiratory/gastrointestinal upset with headaches, malaise and dizziness from which the majority of people recover). However, after an interval, a second more serious multi-system disease can develop with variable involvement of cardiac or skeletal muscle, liver, pancreas, lymphoid or endocrine organs. Nevertheless, ME is primarily a neurological illness with well-documented encephalitic features and classified as such by the WHO [World Health Organisation] international classification of diseases (ICD 10)." You may be surprised to learn that an illness associated with flu-like feelings, a host of physical symptoms and fatigue is primarily neurological and has "encephalitic" features, that is, symptoms known to occur in encephalitis, or brain infection. People tend to think of brain infections as very rare, especially in children. Yet polio is another illness that begins with an encephalitis and which seemed to single out children so much earlier in this century that it was called "infantile paralysis". (Read the interview of Dr. Richard Bruno in this issue and learn about the startling parallels between ME/CFS and post-polio sequelae.) It seems that since the advent of polio vaccinations the developed world has lost its memory about the vulnerability of children to viral infections. Adults can get ME/CFS, too, and research has found that ME/CFS sometimes runs in families (MacIntyre, 1999), though fortunately it appears to be relatively rare for several family members to have it. Autoimmune factors, known to have a genetic component, are being explored by several research groups. In Speight et al.'s (2001) study, there was a positive family history of CFS in a first degree relative in 7 cases (14%), a past history of migraine in 33 cases (67%), and a positive family history of migraine in a first degree relative in 28 cases (57%). While there may be a genetic susceptibility, cross-infection of family members may play a role, too. In an autopsy of a man with ME/CFS who committed suicide, Dr. John Richardson found evidence of active enterovirus (Coxsackie B1) infection of the brain cells. While this case is tragic enough, Dr. Richardson's theory about the spread of such viruses within a family, allowing the development of ME/CFS to arise in other family members over time, is both worrying and intriguing. He writes: From my study of a few hundred families and the sequelae of viral illness, it appears logical that the host both replicates and mutates the virus, no doubt trying to make it "more like self." In the process other members of the family, sharing similar genetic features, who have had the infection as demonstrated by high serological titres, but were not ill, are then at more risk and in fact may later succumb, because their autoimmune system is disadvantaged by the modified 'virus' which has been mutated by the previous host. However, in spite of the published evidence of multiple family members being affected, some doctors don't even realise that a parent and child can have it in the same family! In Karen's case, the first time that her mother suggested that Karen had ME/CFS too, she was accused of imagining things and projecting her own illness on to the child. The distance between the time that her mother fell ill and the time that Karen presented with symptoms may have played a role in the physician's doubt. Too many parents who see symptoms of ME/CFS in their children have been accused of overreacting or imagining things. If it hadn't been for her knowledge of the illness, Karen might be among the many children still undiagnosed. She may even have been committed suicide. Fortunately, things are changing and it is now somewhat easier to obtain a diagnosis for a child with ME/CFS. However, even when a family has a well-informed doctor, part of the problem is that the symptoms differ somewhat in young people from adults so there can be misconceptions about whether they add up to ME/CFS. Here's what is stated in the British report, "Childhood ME": Special diagnostic criteria for children and adolescents have not yet been defined. The diagnostic criteria for CFS in adults (Fukuda 1994) were defined chiefly for research purposes. Young patients may not always fulfil CFS criteria, and yet still have a typical clinical picture of ME/CFSÉ There are some clinical features in children that are different from those in adults, especially in children under 10 years of age. The onset is often more gradual in young children, and their daily behavior is more variable, without a clear history of an initiating infection. Certain symptoms - intractable headache, abdominal pain, loss of appetite, and nausea - are more common in children. There is no clinical difference, once the illness is established, between children whose onset is rapid, usually following an acute febrile episode, and those with a gradual onset, as is seen more often in young children. Especially problematic for children is the criterion of 6 months of activity limiting symptoms required of adults. MacIntyre and associates (1999) recommend: It is better for the child and family to allow for a firm diagnosis after 2 to 3 months of symptoms, instead of the minimum of 6 months as required for adults (Royal Colleges 1996), because good management can be instituted early in the illness and might prevent further deterioration. Even three months of waiting to know what the diagnosis is can be a terrible ordeal. These young people need immediate help - especially with schooling. Parents can help prevent deterioration by following "The Golden Rule" discussed by Dr. Bruno above. Teachers can help greatly by doing what they can to make educational accommodations, even if a child is not yet diagnosed and does not yet have 'special needs' status. Doctors can help by writing letters that enable schools to provide services for the 'at risk' child. Another complicating factor in diagnosis is that a child's own lack of awareness of the symptoms may lead to such youngsters not talking about them: The diagnosis is more difficult in young children because they cannot articulate symptoms such as fatigue and cognitive difficulties. Parents and teachers need to observe and assess symptoms such as onset of pallor and exhaustion, both in and out of school. Their observations of children or pupils whom they know well should be respected. (MacIntyre, 1999). ÉYounger children, who learn at such a rapid pace, have not yet become aware of their learning strengths and weaknesses, making it almost impossible for them to judge the cognitive problems they may be experiencing (Bell et al., 1999, pp. 68-69). Children's lack of awareness of what it feels like to be well may also lead to what Dr. Bell and associates (1999) have described a phenomenon they call "health identity confusion" in young people with ME/CFS and/or FMS: Many [young people with ME/CFS and/or FMS] have no memory of being completely well and are therefore nor sure what health is and what illness is. Adults who became ill as children have stated that they are in good to excellent health while disclosing a significant number of current symptomsÉ.(pp. 68-69). Health identity confusion may be more likely to arise in children who had insidious onset or who fell ill early in life. The Spectrum of Illness There is a range of illness severity in ME/CFS. Diagnosis can be especially difficult to obtain when a child has a mild or atypical presentation. Some young people with ME/CFS, may have quite a few days when they can be active for some of the time. Very severe cases, however, are devastating: The more severe forms of the illness in children and adolescents include symptoms such as dizziness, unremitting headache, severe muscle spasms that may require splinting to prevent contractures in the hands or feet, shaking episodes or pseudo-seizures without loss of consciousness, difficulty swallowing and paresis or paralysis of limbs, bladder irritability and a disturbed mental state. (MacIntyre, 1999). Signs and Symptoms of ME/CFS In Children And Adolescents Understanding the unique symptom complex of ME/CFS is the key to recognizing that this is a physical, not mental, illness that involves the central nervous system. The lack of response of symptoms to medical treatment is one of the most puzzling, and concerning, aspects of the illness. The effects of chronic pain on young people have been studied in other illnesses but far too little attention has been given to this issue in pediatric ME/CFS. As well, the inability of parents and doctors to relieve a child's agony with medical treatment must be recognized as potentially contributing to vicarious traumatization in caregivers, including professionals. a. The commonest feature for diagnosis of ME is persistent fatigue. This is better described as exhaustion, asthenia or weakness, which is usually post-exertional, developing up to 3 days following moderate effort, and is not relieved by rest/sleep. The fatigue may appear as orthostatic intolerance (dizziness or faintness when upright) rather than simple tiredness or sleepiness. The fatigue may be physical or mental, can be severe and often fluctuating, and leads to significant reduction in normal activities. b. Severe malaise (feeling 'poisoned'), particularly following physical or mental exertion c. Persistent headache, not responding to painkillers. d. Disturbance of normal sleep pattern. Hypersomnolence is commonest initially, often progressing to sleep reversal, or else insomnia. e. Neurocognitive disturbance is invariably present (e.g. loss of attention, concentration, and short-term memory, forgetting names, inability to understand a written paragraph). f. Visual disturbance (eye pain, blurring, especially when reading). g. Sensitivity to sound and/or light. h. Recurrent sore throat and/or swollen glands (misleading in children, who develop them with every infection. Prolonged adenopathy may need investigation to exclude TB or malignancy). i.Muscle or joint pain, especially of lower back and lower limbs. j. Nausea, abdominal pain, loss of appetite. k. Balance disturbance, or dizziness on sudden change of position. l. Altered subjective temperature regulation (inappropriate sensations of fevers or chills, night sweats), and maybe objective reversal of sleep/temperature rhythms. m. Facial pallor, especially with the onset of severe fatigue (Ramsay 1986). n. Altered skin sensitivity, paraesthesiae (numbness, tingling), transient rash.. o. Mood changes (irritability, depression, anger and frustration) that are out of character. (MacIntyre, 1999). Orthostatic Intolerance In the past few years there has been a dramatic increase in research on a group of problems in ME/CFS and/or FMS related to blood pressure. In "Chronic Orthostatic Intolerance", Dr. Frank Albrecht, an American counsellor with special knowledge of ME/CFS and/or FMS, has listed the various names for these problems as postural orthostatic intolerance, dysautonomia, neurally mediated hypotension (NMH), neurally mediated syncope, orthostatic syncope, postural tachycardia syndrome (POTS). Although these problems can exist without the presence of ME/CFS, they are commonly found in adolescents with ME/CFS and are not unknown in younger children. Here I will refer to these symptoms as chronic orthostatic intolerance (COI). One of the best descriptions of the effects of COI is provided by Dr. Albrecht: In all of these conditions, upright posture makes the person sick. Walking and running are actually easier than standing or sitting still. The symptoms caused by standing or sitting may include any of the following: fainting, dizziness, nausea, feeling very tired, feeling excessively jittery, having difficulty concentrating or remembering things, experiencing pain in the lower parts of the abdomen or in the legs, mottling or purpling of hands, ankles, feet and legs, hot flushes, sweating, and headache. The underlying cause is a disruption of the autonomic nervous system. This system automatically regulates bodily functions, including heart rate and blood pressure. It also responds to postural or orthostatic stress. Blood is a liquid. It tries to flow downward. When you are standing it tends to run into your legs and feet. This deprives your upper body and, especially, your brain, of an adequate blood supply. The autonomic nervous system prevents this by regulating how the heart beats and by constricting blood vessels in the lower parts of the body, forcing blood upward. In people with COI, this system doesn't work right and blood tends to pool in the belly, legs, and feet. It will pool in the hands, too, if the arms are dangling. This is what causes the mottling or purpling of the skin usually seen in COI. When the blood pools in the lower parts of the body, the heart tries to compensate by beating more rapidly. But because the blood isn't flowing correctly to the heart and brain, efficiency drops and things get worse. In many cases this leads to a sudden drop in blood pressure, which may cause severe symptoms and even fainting. The fast heart beat typical of upright posture in COI is called "tachycardia." This is why the condition is often called Postural Tachycardia Syndrome. Low blood pressure is called hypotension. Most people with COI have normal blood pressure when it is taken in a doctor's office. The sudden drop in pressure typical of NMH can only be detected when it happens--after the person has been standing or sitting quietly for a period of time (several minutes to an hour or more). By the time this happens the person is already feeling sick--dizzy, tired, or in pain--from postural stress. Many people with severe COI have Chronic Fatigue Syndrome too. And those with CFS frequently (but not always) have COI. I don't know why this is. And nobody knows what causes COI itself (though it does often getting suddenly worse following a viral infection). Low Blood Volume As Mary Robinson writes in the article above, groundbreaking new research by Drs. Bell and Streeten has found low blood volume in adults with ME/CFS and a relationship between COI and low blood volume (Streeten & Bell, 1998; Streeten et al, 2000; Stewart & Weldon, 2001). About the role of low blood volume in ME/CFS and/or FMS, Joan Livingston reports: Bell hypothesized that the low blood volume could help account for the prevalence of orthostatic intolerance (worsened symptoms upon standing) in CFIDS, because the limited amount of blood tended to pool in the legs and feet, with a corresponding drop in the amount available to the brain. The result? That common sensation of overwhelming gravity and of wearing lead boots. Other research has added to the mounting evidence that this is a core problem in CFIDS, including reduced cerebral blood flow on SPECT scans and neurally mediated hypotension on tilt-table tests. The new research on orthostatic intolerance and low blood volume may provide important clues about two key symptoms of ME/CFS - fatigue and cognitive problems. Fatigue, in a very special sense, is a central symptom of ME/CFS. But we need to be clear about the quality of the fatigue and how it "works". Low energy reserves It is difficult for some to understand the fluctuating energy levels associated with ME. But it helps to understand energy levels in ME in terms of bank savings and spending. While a person with ME may be able to attend a concert like anyone else, that effort may drastically deplete their energy reserve for a while. It's like they could afford the $50 ticket, same as a millionaire, but once that $50 is spent, they have nothing or little left for anything else, whereas the millionaire can keep on spending with no serious consequences. Likewise, a person with ME may appear to have the same amount of energy in a given situation as a healthy person, but that doesn't mean they can sustain similar levels of activity over time as a healthy person can. And it may take a long time for their energy reserve, like a bank account, to build up again to allow them another activity 'spree.' Absurd Assumptions Not understanding this principle of inconsistent energy levels, many doctors and psychologists who assess people with ME assume that if they show any evidence of being able to do any little thing they therefore can work full-time! So if a person with ME drives to an appointment, doctors may assume they can drive everyday and in all conditions. They don't understand that the drive and the appointment may wipe the person out for days afterwards. Making these kinds of assumptions is as absurd as seeing a person dashing for half a block to catch a bus and assuming they can therefore also run a marathon! To avoid jumping to similar absurd conclusions, doctors need to carefully ask people with ME about their energy limits; e.g., how often they can drive a car and in what conditions and at what subsequent energy and pain costs to themselves! (MEBC, 2002). This very accurate description of the 'fatigue' in ME/CFS applies to children and adolescents as well as adults. Imagine the effects of this fatigue on a young person trying to grow up, play, see friends, join in on the family fun, and go to school. You can see that a child or adolescent may not in fact be exhausted all the time. If he or she uses some energy from the reserves, however, for attending school, for example, there will be a 'payback' later. If that payback is an hour or so of exhaustion followed by recovery and a good night's sleep, things are not too bad. That's how it was with Karen after about three years of the illness. Remember, Karen had mild ME/CFS. However, if the payback is very great, the child may not be able to sleep that night (a common, paradoxical phenomenon in ME/CFS) or attend school the next day. This is a very big price to pay for attending school for a couple of hours. In time, the relapses can start to blend into each other. Relapses and setbacks are commonly referred to as 'crashes' by patients young and old. The 'crashes' that young people with ME/CFS experience have numerous triggers, including exposure to chemicals, loud noise, immunisations, studying hard, and even mild physical exertion. Emotional Symptoms Children and adolescents may be especially likely to receive misdiagnoses of primary psychiatric problems because they cannot describe or explain the origins of their brain-based emotional symptoms and because their parents do not have a framework for explaining these. While emotional symptoms have been historically linked with ME/CFS, they may have been underreported in the more recent literature on CFS. Numerous researcher/clinicians have stated that irritability, anxiety-like and panic-like symptoms in ME/CFS in children and in adults may arise from the physical disruptions in the brain rather than from psychological factors. In "What is ME/CFS?", Dr. Byron Hyde, Canadian physician and ME/CFS researcher, extracted symptoms in children from the work of Wallis and Behan and noted that, among the many other symptoms, children often had "weeping tendencies, and [these symptoms] appeared early [in the course of the illness]. Nearly all affected children are first diagnosed as hysterical, depression or 'parental over-involvement'..Temper tantrums were frequent in young children. In older children unsociability, lack of attention and effort on return to school was frequent. If behaviour was checked, children tended to weep". In a discussion entitled "The Psychosocial and Psychiatric Aspects of CFIDS", child psychiatrist, Dr. Alan Gurwitt (1995) states: Among the major primary biologically caused symptoms are acute and chronic anxiety episodes and panic attacks, emotional lability, sleep problems, and depression. The limbic system, linking several brain areas is complex, contains areas that are the sites of emotions, and has connections with both the endocrine and immune systems such that there are probable inter-reactive feedback mechanisms. Many of these biologically-based problems may fluctuate in severity often in conjunction with other physical symptoms. Cognitive Problems in Children with ME/CFS Children and adolescents who have previously been good students are also likely to be at a loss to explain why they can no longer concentrate or remember familiar details. These symptoms have been written off as 'subjective' or as symptoms of depression by too many. In this issue, Dr. Bruno discusses the clear evidence of attention deficits on neuropsychological testing in adolescents with ME/CFS without psychiatric disorders. While the neurocognitive difficulties of children and adolescents with ME/FMS may not appear to be severe on testing (and that may be because the wrong tests are used), they potentially have significant long term consequences. ME/CFS researcher Dr. Elizabeth Dowsett discusses the potential long term implications of memory deficits, in particular, for children's mental development and educational outcomes: A good memory is the corner stone of the human mind and deprivation of special educational provision in their most formative years is the greatest disability inflicted on young people with ME/CFS. It is also important to recognize that children and adolescents with a milder course and those who have partially "recovered" may have more physical energy but may be plagued by ongoing and very handicapping neurocognitive and learning problems. Though more research on this question is needed, these problems may create similar educational and social difficulties as those of young people with attention deficit disorder and learning disabilities. It is also important to recognise that research on child temperament has shown that difficulties with attention that are milder than those in attention deficit disorder can contribute to lower achievement in the classroom (Martin et al., 1988). Another complicating factor in ME/CFS is how well a child who is ill may look, even being rosy-cheeked and cheerful in demeanor. Research on other disabilities which are 'invisible' in children has shown that the psychosocial consequences of invisibility are more serious than of having a disability that others can see (Davis, 1993). Psychiatric Problems The topic of psychiatric disorders in children with ME/CFS is addressed by Dr. Bruno in the interview in this issue. It is easy to understand why a large number of children and adolescents with ME/CFS are at risk of developing a reactive depression. This is true for children with other chronic illnesses. Although the researchers of the British report "Childhood ME" suggest that family dynamics can provide a clue about depression in these children, my view is that experiences in school may play an even larger role in this situation. This is a question calling for more research. "Karen's marks began declining rapidly in the tenth grade. Her teachers would not help her to catch up when she missed school due to frequent episodes of illness. We tried to be as supportive as we could at home. However, by mid-year, Karen was severely depressed and suicidal. She was very confused about her cognitive problems and emotional sensitivity and expressed her fear that she was losing her mind. Her teachers repeated criticisms for being away 'too much' and for forgetting her assignments and books chipped away at her self-esteem until she broke down one day, weeping: 'I'm not smart any more.'" Everyone needs to be aware of the risk of depression and suicide in young people, including those with ME/CFS. Parents are often the first people to appreciate how deeply their child is suffering and they can become very sad in the face of their child's daily struggles. Even when depression is not present, professionals and relatives need to listen and understand the roller coaster of parents' lives with an ill child and to see the anxiety and alarm experienced as normal and understandable. It can be difficult to sort out the direction of effect between parental despair and a child's own mood. Families should not be blamed for the altered dynamics in their homes while clinicians must work hard and skillfully to restore feelings of hope. People should not be pathologized because of professional ideologies and ignorance. While the presence of competing theories, the use alternative medicine, and that preponderance of conspiracy theories have been regarded as detrimental to these families, the fact is that the vast majority of parents and patients are far better informed about the illness than the average helping professional. Whereas ME/CFS patients have been scoffed at for raising the issue of biowarfare, since Sept 11 of this year this topic is no longer considered an irrational concern in public discussions of health. Quality of Life Both acutely and less severely ill children and adolescents with ME/CFS experience time away from school, loss of contact with peers, and often daily bouts of intractable pain, exhaustion and flu-like feelings. They may not have sufficient attention, energy or tolerance of stimuli to cope with being read to, to watch television or otherwise distract themselves from their plight. What is the quality of life of these young people? Mary Robinson provides a compelling glimpse in her story in this issue. To understand ME/CFS from the young person's perspective, I recommend reading their own words, posted on many of the ME/CFS web sites around the world. A young Canadian wrote: So, much of my life is now dependent on other people. I was always very independent at a young age but now I require help with so many things. At times I need help with such simple things as drying my hair or dressing. I need someone to prepare my meals (I used to love to cook), do my laundry, make my bed, help me with projects. I know many people think this it what most teenagers want. Help with everything. But, it is not true - when independence is taken away from you because your body won't co-operate it is very difficult. I was a dancer, a gymnast, a swimmer. I was on the basketball team at school and very involved in school activities. I now require the aid of a wheelchair for outings and have difficulties walking around the yard. Any exercise (something I loved) is now followed by extreme exhaustion that can put me to bed for days or even weeks. I still attempt gentle exercise but it is always followed by pain, exhaustion, and a host of other symptoms. There are days when I can't seem to keep my balance, days with severe nausea, dizziness, terrible migraine headaches......and there is always extreme exhaustion even after rest. Always there seems to be a dependence on my family. I am doing better than years ago when I was virtually paralysed. I could not bathe, feed or toilet myself - I could not turn over in my bed without help.... I still have bad days but I also have better days." Will These Young People Get Well? The British report, "Childhood ME." draw some cautious conclusions about prognosis in children and adolescents. The quotation below is long, but it makes some very important points: At present there is not enough evidence to make a definitive statement. Several authors have published follow-up studies of paediatric ME/CFS patients. Dale and Strauss (1992) considered that young patients with CFS have a high probability of recovery within the first 1 -2 years, but that after longer periods of illness the recovery rate is less. There was an outbreak in Lyndonville, N.Y. state, in 1985: "104 patients were identified who retrospectively met criteria (for CFS). 44 patients were under 18. All were followed up for at least two years, when only four children had made a complete recovery." (Bell 1992). In 1998 David Bell presented findings (at a conference) from 15 year follow up, which found that after 15 years 20% had not recovered. Feder (1994) in the USA claimed about 90% recovery over 5 years, most of whom recovered in the first 2 years. This was a selected district population of mild to moderately ill children and did not include the more severely disabled." All follow-up studies report improvement or recovery in over 50% patients. Some young people have ongoing disability. ..... however, it is significant that some children in each group continue to experience significant fatigue and disability. It is possible that these children who do not improve represent a subset of paediatric CFS (ME) patients, potentially having a more severe form of the illness or differing in other important traits. There are some indicators as to which young people may experience persistent disability: Bell (1995) noted that this group with persistent disability tends to have symptoms that are worse from onset, resulting in severe activity limitation. In addition, they tend to have more severe neurologic symptoms including myoclonus, paraesthesia, and seizure-like episodes." (Jordan et al, 1998). The age of the child at onset and onset type may play a role in prognosis: Clinical experience suggests that younger children recover more fully than adults, but that younger children with a gradual onset may run a more protracted course. MacIntyre et al. (1999) comment: Because ME is characterised by fluctuations in energy and symptoms, often with remissions of weeks or months followed by relapses - which usually result from stress or over-exertion, a child who appears to be recovering needs to be careful about exercise for at least a year. The more severely ill children may take a very long time to recover (>7 years), but such assessments are seriously flawed by the absence of a reliable comparison. Since they wrote this report, there has been a little more published research on recovery which has had similar findings. Rangel et al. (2000) considered that most children recover although some children had a very severe course. Bell et al. (2001) reported that twenty percent of participants remain ill with significant symptoms and activity limitation 13 years after illness onset. However, Speight et al. (2001) reported a much higher level of recovery at the Sydney 2001 Conference: Full recovery was seen in 15 cases (31%) (5 mild, 8 moderate and 2 severe). Mean duration of illness in this group was 5.1 years. Significant improvement (change to a less severe category) was seen in 7 cases (14%). Twenty-seven cases (55%) were static at the time of follow-up, of whom 7 were still in the severe category. How Do You Define Recovery? A lot, of course, depends on how you define "recovery". This is what the authors of the British report, "Childhood ME" had to say on this topic: 'Recovery' means the achievement of an acceptable life style in comparison with a 'normal' adult population. There are different degrees of recovery from a return to a tolerable energy level and lifestyle, to complete recovery to the pre-illness activity potential. It is possible that many people with ME/CFS who 'recover' have in fact adapted to a lower energy level and modification of lifestyle. What is unknown is what quality of life would have been achievable had the person not become ill in the first place. We remain very guarded as to individual prognosis. David Bell's study, presented in San Fransisco, concluded that there were no clear pointers to the predicted course of the illness in any individual child. (Bell 1996). Even if the word "recovery" is not completely accurate, there is no question that the vast majority of young people with ME/CFS improve over time. What Prevents Recovery? Dr. Bruno addresses this question above. Except in very mild cases, trying to push though the fatigue and overcome the limits imposed by the illness causes an exacerbation of symptoms. These symptoms may be delayed for 48 even 72 hours after the exertion. They are often experienced away from everyone but the family. When the effects are not witnessed, reports about them may not be believed. Even some family members may note the child or adolescent "lying around" but not fully appreciate the agony and misery the young person is experiencing. "The Enigma and the Paradox of ME", Dr. Dowsett describes the numerous factors which trigger relapse: a) Immuno suppressive events such as concurrent infection with other microbes, immunisation, steroid or cytoxic therapy. NB smoking reduces local mucosal immunity (b) Hormone disturbance, including puberty, menstruation, pregnancy but following the menopause, new onset of illness in females falls sharply. (c) Mental or physical stress arising from head injury, whiplash, surgery, malnutrition, climatic change, domestic problems, litigation, social security assessments etc.. (d) Exposure to drugs which are psychoactive or vasoactive including alcohol, anti-depressants or recreational substances and to neurotoxins, pesticides and drugs which interfere with specific neurotransmitters (eg. acetylcholine). The figures about recovery and the possibility of relapse call for caution in two respects. The first is the management of activity level and attempts to return to school when a child has been home ill. This is a very complex issue that needs to be addressed on an individual basis. Flexible educational systems are the keys to success but not all children will be able return to school. The second matter is the critical one of the messages that parents and teachers give to the young person who is ill. It is my impression that some clinician/researchers disagree about being so pessimistic in tone about prognosis, even if the figures seem to speak for themselves. I can see their point. From a medical point of view, when you have been treating a child who has been bedridden for years and he or she is active again, this is a marvellous recovery. However, my guess is that when there is more research that is from an educational and psychosocial perspective, we will learn that there are often residual problems that may or may not be disabling, depending on the goals the young person sets in life and the accommodations he or she is able to obtain. Whether they have partly recovered or are severely ill, young people are not helped when they are surrounded by pessimism and doubt. It is also absolutely true that we do not know what science may bring in the way of effective treatments tomorrow, next week or next year. We owe it to youth to be hopeful but also mindful of the cost of their current situation. Developmental Costs How do all the losses young people with ME/CFS experience affect their overall development? Do they have big gaps in their cognitive abilities and/or general knowledge? Do they lack "street smarts"? And, are there any gains to be achieved by living through this very tough ordeal? Young people with ME/CFS and/or can grow into wise and patient young adults, unusually gifted in many ways. Although many have described the remarkable resiliency of this group of young people, readers will recognize the potential developmental costs associated with this type of maturity as well as with the losses and frustration that these young people endure (MacIntyre, 1999). Teachers also know the important role that social contact and education play in nurturing growing young minds and souls. Families should not have to try to provide these without support from society. Most especially, the medical profession needs to start becoming the advocates of these children and their families, instead of their adversaries. Follow-up "Karen, 21 at the time of this writing, is now attending college full-time and doing well. Has she truly recovered? She sleeps late every weekend and catches naps as often as she can. She still has some cognitive problems, especially with memory. She can't concentrate on something like reading for very long, either." Remember that Karen had only mild ME/CFS. And while she is doing far better than many young people with ME/CFS, her parents believe she is not really meeting the potential she would have had had she not been ill. On the other hand, Karen herself said she is glad she went through her illness, including her depression: "Having ME/CFS has made me stronger and I have more empathy for other people. I have more confidence in myself and I know what my goals are. It has also strengthened my religious faith." Karen, and the other young people described in this issue, are living embodiments of a statement made by Dr. David Bell and associates in their book, "A Parents' Guide to CFIDS" (Haworth Press): Steady, but slow progress, is being made toward uncovering the cause [of ME/CFS]. Until this becomes known, children with CFIDS will continue to face the medical, educational and social dilemmas of this illness. Remarkably, they tend to do so with a courage unknown to the healthy adults who judge them. Key Reference MacIntyre, A. (Ed.) (1999). Childhood ME: A Report by an Independent Group of Advisers. To read the full report, Childhood M.E., go to http://www.youngactiononline.com/docs/chldhdme.htm To save space, a complete list of references for this article has not been included. To obtain a reference list, please contact Kate Andersen at bdi@temperament.com International Web Sites on Young People with ME/CFS and FMS Association of Youth With ME (UK) http://www.ayme.org The CFIDS Association of America (USA) www.cfids.org/youth/default.htm The Parents and Youth Pages of the National ME/FM Action Network of Canada www.geocities.com/canadianyouthmefm/index.html South Australian Youth with ME (SAYME) http://www.sayme.org.au Young Action Online (UK) www.youngactiononline.com Improving Communication Between Doctors and Young People With Chronic Fatigue Syndrome (YPWCs) By Sharon Walk (Originally published in Youth Allied By CFIDS, Fall 1996) All too often, doctors and patients fail to communicate effectively. They go their separate ways after office visits, each unaware of the other's thoughts and concerns. Sharon Walk and other young persons with CFIDS (YPWCs) have joined efforts here, suggesting ways for doctors and patients to bridge the gap of miscommunications and understand each other better. -The Editors For Our Doctors: Ways to better understand and communicate with YPWCs 1. Make time to listen to our concerns. So often we feel rushed through appointments and don't get to ask the questions we have. Please make sure your appointment scheduler understands that we may occasionally ask to arrange for longer visits when we have severe relapses or confusing treatment problems. Try to make sure that near the end of an appointment you take a minute to ask if we have any further questions and if we have understood everything that was discussed. If you need to keep track of the time, you might hang a clock in your line of vision so you can see it without your patient noticing. When you glance at your watch repeatedly, we hesitate to take your time with our questions and concerns. 2. Talk so we can understand you. Try to remember that you are talking to a younger person and take the extra time to explain medical terms. Of course, how much you need to do this will vary depending on the ages of your patients and the amount of CFIDS and general medical knowledge they have. 3. Talk to us without our parents during each appointment. Often those of us who are younger CFIDS patients come to appointments with our parents. Try to set aside at least a few minutes to talk to us by ourselves. Sometimes we have questions and concerns that we might not want to discuss in front of our parents, but that we really need to address with our doctors. 4. Let us be part of the decision-making process. As YPWCs, we often feel that we lack control over our lives. It sometimes seems that all of our decisions about treatments, school and social life are dictated by our doctors, schools or parents. As a doctor treating YPWCs, you can help return some of that control to us. Try not to tell us what we have to do; rather, suggest something that you would like us to do and ask us how we feel we can make it work. By giving us choices and listening to our opinions, you may have happier and more secure patients. 5. Encourage us to think of creative solutions to problems. What works for one YPWC may not work for another, even if they appear to be similarly affected by CFIDS. Give our parents and us opportunities to share ideas for increasing our social and activity levels and improving our quality of life. We'll work together to create individualized treatment and schooling plans. 6. Show trust in us. As YPWCs, we are constantly facing people who do not believe we are sick and do not understand CFIDS. It helps us to feel that we are trusted and believed by our doctors. 7. Understand and help us through our fears about not being believed. Sometimes we assume that if we are successful at doing one thing we will be expected to continue to push ourselves until we near a relapse. Some of us fear that our parents and doctors will push us until we question our own judgment about whether or not we're really sick. This comes, in part, from our experiences with doctors, teachers and parents prior to being diagnosed with CFIDS. Many of us were often told that we weren't sick and that all we needed to do was to "do more" and get back into the world. Sometimes we still associate someone wanting us to try to do more with being told that we're not really sick, or with being pushed harder than is good for us, and having to fight to say "no." As a CFIDS doctor you can help us avoid this. From the first appointment, emphasize to the newly diagnosed YPWC that you believe what we are going through is real, and that you are going to do your best to improve our quality of life. Tell us whether you have seen cases like ours before. Listen to our reactions with care and understanding, and know that we are usually eager to socialize and attend school as much as we are physically able. 8. Don't be afraid to say, "I don't know. " We understand that most doctors, including those knowledgeable about CFIDS, have experiences when they are perplexed by a patient. Maybe the patient in their care has unusual symptoms or isn't responding to a treatment as expected. But when this happens to us, we often leave our doctors' offices feeling that they think we aren't following their recommendations or just don't want to get well. That makes us feel awful about ourselves. Be willing to admit if you just don't know why a treatment isn't working as expected, or why we have an unusual symptom or problem. CFIDS confuses us, too, and we will respect you for your honesty. For YPWCs: Ways to better communicate with your doctors and increase their understanding of your life with CFIDS 1. Make sure your appointments are the length you need. If you expect to need a longer appointment than usual, perhaps because of new symptoms or a confusing relapse, it's important to be considerate and tell that to the appointment scheduler. Otherwise, your visit may be rushed and not as helpful as you need and your doctor will likely be late for appointments with other patients. 2. List your three worst symptoms. Before seeing your doctor, decide which three symptoms have been most disabling for you since your last visit. Be sure to discuss them with your doctor, so you can work together to prioritize your treatment plan. 3. Make a list of the questions you have for your doctor. It helps to write down the questions and concerns you think of between visits. Before your appointment arrange them in order of importance and consider adding a brief explanation. That way, if you are having a difficult day, you can hand the list to your doctor and he or she will understand what you wanted to know. 4. If you have questions about your CFIDS treatments, ask them! When your doctor suggests trying drugs or other treatments, feel free to ask him or her to explain what each is and what symptoms he or she hopes it will help. This will help you to understand and participate in your treatment plan, and help to prevent misunderstandings. For example, antidepressants are used to treat many different conditions - not just depression. If your doctor prescribes one and you leave without asking questions, you may find yourself wondering whether your doctor thinks you're depressed. You may not find out until your next visit that antidepressants are often used to treat sleep disorders and chronic pain. Misunderstandings like this can be prevented by asking your doctor to explain what your new medicines are, and what you should expect while you take them. 5. Tape record your visits with your CFIDS specialist. When a YPWC goes to see a CFIDS doctor, there are often many questions answered, research breakthroughs explained and details to remember. If you record your appointments, you can listen to them again and better understand what your doctor said. (Before you tape record a visit, ask your doctor for his or her permission.) 6. Tell your doctor if you're having problems with people who don't understand CFIDS. It is common for YPWCs to have difficulties with school personnel, and sometimes even their parents, not understanding their illness and the limitations it places on their lives. Your doctor can help resolve this if you tell him or her what is happening. Ask for his or her assistance in explaining your disability and how others can support your efforts to lead as normal a life as possible. 7. Tell your CFIDS doctor if you are depressed. Many YPWCs go through periods of depression as they adjust to living with a chronic illness. Your doctor will know that in most cases, depression is a result of illness, not the cause of it, and will help you get through it. If you're concerned about deep or prolonged feelings of sadness or helplessness, don't be afraid to tell your doctor about them, and to ask for a referral to a health psychologist or therapist or counselor. This article originally appeared in the Winter, 1997, issue of The CFIDS Chronicle. It has been reprinted with the permission of The CFIDS Association of America. For more information, call the Association at 800-442-3437 or visit www.cfids.org. Does ME Cluster In Schools? by Dr Elizabeth Dowsett, MB, ChB, Dip. Bact., Honorary Consultant Microbiologist South Essex Health Trust. Introduction by Jane Colby, Former headteacher, Executive Director of Tymes Trust, Member of the National Association of Educational Inspectors, Advisers and Consultants EDITOR'S NOTE: Jane Colby is also a member of the UK Government Chief Medical Officer's Working Group on CFS/ME. She can be contacted at Jane@jafc.demon.co.uk Introduction In the United States of America, where ME has now been listed as a Priority One disease, it is well accepted that ME clusters in schools, families and communities. But here in the UK that is not yet so. Yet this fact has been common knowledge amongst parents and teachers for some time, though perhaps not so obvious to doctors because they do not work in educational institutions. When, back in 1990, Dr Dowsett explained to me that "the problem is, we have no statistics showing the pattern of ME in schools," I said, "So let's get them." If I had realised then that it would take us five years, during which I was myself still ill with ME from a virus related to polio, I might not have considered it so readily. The exercise was hard, frustrating at times and involved accepting that due to political pressures on the education system (and the near chaos of its re-organisation into new types of institutions) schools were now overtly in competition with one another. Funds depended on pupil numbers, numbers depended on reputation and public image. They still do. This meant that there was extreme reluctance to reveal statistics to us in some quarters. ME had a controversial image back then, more even than now, and in addition there was a perception that if schools admitted to clusters of cases, there would be a scare that children with ME could transmit it to others. This would drive parents away. If anyone doubts this assertion, let them try, as many journalists have, to persuade the head of a school to admit - on air - to a cluster of ME and see what response you get. I for one cannot blame them. When our study was published we explained that this difficulty must indeed have reduced the numbers of returned questionnaires. Logically, however, if it did skew the balance of our statistics in any way, it should have inflated the "negative" reports we got; no school minds submitting a blank return showing no cases of ME at all! And indeed, these came in plentifully. In fact, we had taken some careful steps in the design of the study, in order to check the reliability of our results. One was to graph out other statistics which came in. Our statistical graph for leukaemia correlated with already known statistics for this disease, indicating that our results were taken from a representative sample. In addition, we checked that we had received a balanced response from schools in both secondary and primary sectors. The cases of illness on which our study was based were diagnosed cases of ME. No schools were involved in diagnosis, and were asked to list suspected cases separately. We were of course never allowed to see any documents, doctors' reports etc. on any child or member of staff, as these are confidential. However, it is part of each school's responsibilities under the 1991 attendance regulations to place any absence from school in one of two categories - authorised or unauthorised. In categorising absences the Head must be satisfied that the explanation is genuine, and will request documentation as proof. We naturally accept the professional competence of senior educational staff to carry out this duty. In the following paper, Dr Dowsett pays tribute to the education authorities who so kindly assisted us in the face of controversy. It may be that some of the areas which declined to help did so because they already knew they had large numbers of cases. Whether this was the case or not we shall never know, but our study had to take place in those areas we chose for their geographical location and which were also willing to reveal their ME statistics. I should here like to add my thanks to those of Dr Dowsett. Needless to say, we assured them of complete anonymity so that their areas would not be publicly identified with the statistics uncovered; without such assurances we would never have got the study off the ground, like others who have attempted to study schools and failed. The discoveries we made are many and various, and Dr Dowsett covers most of them below. There are two others, however, which I find particularly interesting - though perhaps not surprising. One is that where Local Education Authorities are ungenerous in their provision of special arrangements for the education of children with ME, parents are more likely to vote with their feet and take them off the school roll entirely. This is sad because it means they are ineligible for all the help which the state should be giving. This in turn means there is a danger of a two-tier education system for children with ME, one for those whose parents can pay for suitable education, another for those who can't. The other discovery, rather ironic in its way, is that although ME has often been regarded as, at least in part, a psychological or psychiatric condition, the LEAs we studied provided more home tuition for psychiatric illnesses than for ME. A case of heads you win, tails I lose. This is not entirely the fault of the LEAs, who have often been following the advice of the 1996 report of the Royal Colleges of Medicine (produced, they now explain, mainly by The Royal College of Psychiatrists). This report is not recommended by the Dept. of Health as a treatment protocol. The Chief Medical Officer's Working Group on CFS/ME (instigated by Sir Kenneth Calman in summer 1998) will make recommendations for suitable guidance. NB Action for ME, which helped to fund our study, is the ME charity listed in "Supporting pupils with medical needs in school - A Good Practice Guide", jointly issued by the Depts. for Health and Education. ME/CFS in the UK School Population 1 The Problem Do children and adolescents suffer from ME/CFS? Simple common sense tells most parents, teachers and doctors that they do and often more severely than adults. Yet there remains a sizeable proportion of professionals in Health Care, Education and Social Services who are still prepared to ascribe the numerous, disabling but seemingly unconnected symptoms of this illness in young people to anorexia, depression, school phobia or a dysfunctional family background. All are, at least, agreed that the illness presents a considerable economic, educational and social problem. 2 Market Research No hopeful salesman can, nowadays, expect to succeed without prior market research and no-one intending to raise a bank loan for such a purpose will be considered without a well-researched business plan. Unfortunately, when we turn our attention to human disease, conclusions are often reached without prior study of the WHO, WHAT, WHERE and WHEN which can lead to effective consideration of WHY. In medical terms, this is called EPIDEMIOLOGY, which means the study of human diseases in relation to their environment rather than, for example, the behaviour of small animals or tissue cultures in laboratory setting. 3 Historical Background Over 60 years ago, epidemiological studies of ME/CFS were commonplace and usually initiated by doctors who, lacking modern technology, simply used their eyes and ears. Of over 70 recorded epidemics of ME/CFS since that time, 13 clearly mention young people while one is entirely devoted to that age group. These old surveys were small and without government funding but they clearly delineated present day findings such as female predominance of cases, peak incidence at puberty, variable prevalence linked closely to seasonal and geographical features (summer/autumn onset, cool/temperate climates and rural or suburban settings) as well as the key role of schools and similar institutions in the spread of the illness, with clustering of cases within families and schools. The serious disabling potential and chronicity of ME/CFS leading to relapse is always noted. 4 Why did we embark on our school survey? To our knowledge, no government anywhere in the world has yet funded an epidemiological survey of this type and scale in schools. Ten years ago, a group of sufferers from ME/CFS who were also teaching professionals, parents or carers of young people similarly affected, conducted a postal survey of sufferers under the age of 25 years which stimulated a response from nearly 600 young people in the UK and abroad. The results were disheartening in what they revealed, but they supplied the initial impetus to seek further information about the WHO, WHAT, WHERE and WHEN with an unexpected bonus in relation to WHY? What did this survey disclose? There were 3 major findings:- a) Only 29% of respondents under the age of 25 were in full time education, training or work, while 34% were totally excluded from all three. The remaining 37% were making various compromises, few of which could be considered satisfactory. b) An enormous loss of independence and self-esteem was reported, with additional grief at the general disbelief expressed not only by the press and by various professionals but at the loss of support even from friends, colleagues, family members and those in a previously loving relationship. c) The bonus was a personal invitation to study, at close hand, the consequences (after 2 years) of a seemingly trivial respiratory/gastro-intestinal infection causing symptoms in 1/3 of a village school roll, leaving some 10% of children so affected, with chronic relapsing energy problems, musculo-skeletal pain and disabling prolonged cognitive disturbance. Early laboratory investigation was missed but relapses in the following year were significantly associated with the viruses then circulating in the school (Influenza A and parvovirus infection). 5 How did we set up the 1991-1995 Schools Survey? With a clear indication from the young people themselves of what to look for, we set out to find if such a cluster of ME/CFS cases in a single school was a unique event or if (as we already suspected) it would be duplicated elsewhere. We hoped that, if successful, the study might provide clear indication of a cause as well as some helpful pointers to diagnosis, management, treatment and prevention. Since we were not sanguine about the funding of our "business plan" by medical or charitable agencies, we were grateful for the prompt and unreserved backing of the educational profession, to whose understanding of the importance of this subject, all students and young sufferers from this illness must pay tribute. 6 What methods were used? A search was made in six English Local Authority (LEA) areas, chosen initially for their widely varied geographical, economic and social mix, for all causes of medically certified long term sickness absence in pupils and staff. Confidentiality was assured in the collection of these details which were handled only by senior educational staff and specifically excluded all personal identification. The information requested included geographical location, age, gender and school class location of sufferers, size of pupil and staff roll and education or management provision while sick, as well as the outcome. 7 What were the most important findings? Between 1991 and 1995 we were able to collect details of all types of long term medically certified sickness absence from schools with this problem as well as useful information from those without it, and from 63 private schools outside the LEA jurisdiction. Excluding (for cost reasons) the private sector, whose details did not differ significantly from the public one, our survey comprised 1098 schools, 333,024 pupils and 27,327 staff - the largest epidemiological survey of this type made to date. Significant findings included:- a) Prevalence of ME/CFS in Schools Over one third of the schools providing information reported long term sickness absence and of these, 2/3 had cases of ME/CFS (230 pupils and 142 staff) suggesting a prevalence in this population of 70/100,000 in pupils and 500/100,000 in staff - a rate two or three times that quoted in other adult population surveys. b) Types of Illness Which Cause Long Term Sickness Absence Among the 885 individual sickness records received in 6 LEAs, ME/CFS was by far the commonest cause (42% overall, 33% in staff and 51% in pupils) followed by: cancer and leukaemia (23%); general medical or surgical conditions (13%); musculo-skeletal problems (12%); psychiatric disturbance and virus infections (5% each). c) Clustering of Cases Using a definition of 3 or more cases with the same diagnosis in the same school, we looked at all illness falling within this category and found 54 clusters (36 due to ME./CFS, 7 to virus infections, 4 to psychiatric disturbance, 3 each to cancer/leukaemia and musculo-skeletal conditions and one to diabetes). 45 clusters, including all but one of virus infection, occurred in schools with ME/CFS, the exception being one in close geographical proximity. 4 small clusters (less than 6 cases) of cancer, musculo-skeletal conditions and psychiatric disturbance were noted in schools with no evidence of ME/CFS. This was a noticeable difference from the large clusters of viral infection (variously described as respiratory/gastro-intestinal, "flu" or "glandular fever" numbering up to 16 cases each) associated with ME/CFS. Of the 372 ME/CFS cases in pupils and staff, 149 cases (40%) were distributed as single cases, 78 (21%) as pairs and 145 (39%) as clusters of 3-9 cases, a remarkably high prevalence of coincident ME/CFS and viral infection in selected geographical areas. d) Geographical Prevalence of ME/CFS Though single instances were noted in all LEA areas but one, we were surprised to find that the majority of ME/CFS case clusters were associated with virus infection grouped in an LEA district which was by no means the largest, but which was characterised by its suburban growth after much recent population influx from the building and expansion of "New Towns" in green-field sites. It has always been a characteristic feature of certain epidemic infections (e.g. poliomyelitis) and of illnesses now suspected to be triggered by environmental factors including infection (e.g. childhood leukaemia) that, when isolated rural or suburban communities with an established and naturally acquired "herd immunity" are subjected to an influx of new population, the prevalence of such illnesses increases. e) The Effect of Age and Gender on the Prevalence of ME/CFS in Schools Of the 230 pupils certified as suffering from this illness, 157 were female and 73 were male, a F/M ratio of 2:1. The average age was 13 with peak prevalence at 15 years. Sex ratios below puberty were more even, indicating a hormonal influence upon the known frequency and chronicity of the illness in females during child-bearing years. The majority of these sufferers were located in senior schools. Of the 142 staff with ME/CFS, 111 were female and 31 male, a F/M ratio of 4:1, influenced by the fact that most staff employed in schools are female. This is especially noted in the primary sectors where female staff are more common and there is close personal contact with children suffering from various infections and where the majority of staff suffering from ME/CFS are located. f) Education and Work Modification for ME/CFS Although varying education and work management patterns were used in sequence or in combination in all LEAs surveyed, we consider the following to be key factors in reducing physical over-exertion and mental stress which, together with secondary infection, are the commonest causes of relapse in subjects with ME/CFS in the school environment:- Pupils Home Tuition for those too ill to attend school (but not generally suitable for the very sick, who make a better recovery if education is postponed until stabilisation of the illness). Modified Timetable which permits, for example, continued participation in selected school activities (excluding sport) and the taking of examinations sequentially over longer periods. School Withdrawal which, in the absence of such concessions, may oblige education "otherwise" at home. There is, however, no evidence that young people educated this way fare worse than in conventional school classes, while many sufferers achieve better results in the absence of school stress and repeated exposure to infection. In our survey, provision over the 6 LEAs for Home Tuition averaged 48% (range of averages 14-79%), Modified Timetables 57% (range of averages 50-86%) and School Withdrawal 6% (range of averages 0-17%). Though some other medical conditions enjoyed more generous provision, the majority of pupils with ME/CFS were not removed from the school roll. (NB Listing on a school roll does not necessarily imply attendance.) Staff: The average provision for Official Part-time work was 50% (range of averages 38-80%) but Early Retirement was taken up by 38% on average (range of averages 27-100%) representing a serious and probably avoidable loss of career potential compared with other illnesses. Commentary 1. Of all the symptoms associated with ME/CFS, disturbance of cognitive function is the most disabling and long lasting in both pupils and staff 3]. It induces prolonged difficulties in maintaining wakefulness and attention, in concentration and memory, in language and mathematical ability and in appreciation of shape and judgement of distance which, combined with motor dysfunction affecting balance and fine motor control interferes with practical tasks and independence. Funding for educational research 4] into the correct management and educational needs of those affected (such as that already received by individuals with other movement, speech or cognitive disorders) would be well repaid by preventing the loss of educational potential in pupils at an age when brain development is at its peak. 2. Our survey into the clustering of ME/CFS cases in schools and the wide variations in geographical prevalence disclosed suggests that it would not be difficult to identify specific infections which can trigger the onset or relapse of ME/CFS. Epidemiological research directed to the school population, where good records are kept and prolonged follow-up of physical and cognitive problems is possible, would undoubtedly be as economical in terms of diagnosis, management and prevention as it was of the understanding of the true prevalence and mode of transmission of poliomyelitis in the past. 3. The 1996 issue of CR54 5] and the guidelines relating to management of young people with ME/CFS (e.g. discouragement of Home Tuition, encouragement of early return to school, intervention with anti-depressant therapy and graded exercise) may well leave us with a generation of young people suffering from educational deficit and an inability to assist themselves back into work, to meet government targets. Note: Since the publication of our study, a community paediatrician unaware of our work and unknown to us at the time has, in subsequent years, completed a similar epidemiological survey in just two boroughs of one LEA mentioned in our study, with similar results. This work was published in 1998. References: 1. Bell DS Children with Myalgic encephalomyelitis/Chronic Fatigue Syndrome: overview and review of the literature. In: Hyde Bm, Goldstein J, Levine P eds. The Clinical and Scientific basis of Myalgic-encephalomyelitis/Chronic Fatigue Syndrome, Nightingale Research Foundation, Ottawa, Ontario, Canada. 1992: 209-216. 2. Dowsett E G, Colby J. Long term sickness absence due to ME/CFS in UK Schools Journal of Chronic Fatigue Syndrome. 1997; 3(2): 29-42 3. Bastien S. Patterns of neuropsychological abnormalities and cognitive impairment in adults and children. In Hyde Bm, Goldstein J, Levine P eds. The Clinical and Scientific basis of Myalgic-encephalomyelitis/Chronic Fatigue Syndrome, Nightingale Research Foundation, Ottawa, Ontario, Canada. 1992: 209-216. 4. Colby J. The School Child with ME: British Journal of Special Education. National Association of Special Educational Needs. 1994; 21: 9-11 5. Royal College Of Physicians. Chronic Fatigue Syndrome - Children and CFS. 1996; CR54 : 29-33 6. Arzomand Ml. Chronic Fatigue Syndrome among School Children and Their Special Needs - Journal of Chronic Fatigue Syndrome, Howarth Press Inc. New York. London 1998 ; 4(3) : 59-69 A Young Action Online document. You are welcome to redistribute or reprint "Does ME Cluster in Schools?" without seeking our permission provided: 1) you do not abbreviate or change the text in any way; 2) the authorship information is retained; and 3) www.youngactiononline.com is credited as the source.