B.L. Goldklang (1), S.S. Festa (1), and J.S. Hason (1,2).
Lyme Disease Coalition of New York and Connecticut, Katonah, NY, USA (1); Arthritis Foundation, Long Island, NY, USA (2).
Lyme encephalopathy has been defined in the medical literature as a “mild confusional state” characterized by subtle disturbances in memory, mood, and sleep.1’3 To patients with this syndrome, however, this mild confusional state has a disabling impact on all aspects of daily life: from the ability to maintain full-time employment, relationships, and independence, to the simple capacity for task performance, household maintenance, and the rudimentary abilities to plan, schedule, and organize. For many patients, the simplest activities become difficult, time consuming, or completely impossible to perform.` Other patients are unable to work or attend school for months to years, leading severely curtailed lifestyles and a significantly diminished quality of life.
Although people with Lyme encephalopathy may appear physically well, many are seriously disabled.’ The extent of disability is linked to a range of cognitive, sensory, and neuropsychiatric symptoms and syndromes, including but not limited to, short-term memory loss, language processing difficulties, attention deficits, ,extreme sensitivity to sound, light, and movement, insomnia, lability, mood disorder, major depression, anxiety and panic attacks, obsessive compulsive disorder, and extreme fatigue.4,842 Irritability escalating to violence has also been observed as a manifestation of Lyme encephalopathy.4’8’13 To put a human face on this complex and poorly understood aspect of Lyme disease, we interviewed adult and pediatric patients with Lyme encephalopathy, particularly focusing on its impact on the patient’s quality of life (QOL). For adults, we looked at QOL in terms of vocational functioning (ability to work at or maintain a job), social functioning (ability to sustain relationships, marriages, friendships), mental and physical independence (ability to pursue daily activities ranging from money management to transportation, or shopping without help of others), psychosocial functioning (ability to cope with behavioral and emotional limitations and deficits caused by dysfunctions in mood and personality, severe depression, sleep disturbance, and cognitive impairment), and recreational functioning (ability to maintain hobbies, interests).
For children with Lyme encephalopathy, we looked at QOL in age-appropriate areas of educational progress (ability to attend and complete school) and social functioning (ability to make and maintain friendships). Psychosocial and recreational functioning were also considered, as these areas likewise reflect critical aspects of childhood development. Not surprisingly, children with encephalopathy are hit hardest by its often dramatic (not subtle) mood and psychiatric changes–changes they neither understand nor are frequently able to express or articulate, as many may be too young and have nothing in their experience by which to compare, gauge, or comprehend such changes.14-16
While initial unstructured interviews of encephalopathic patients reveal complaints of memory loss and inability to focus or concentrate, mood swings, fatigue and sleep disturbance, the tremendous impact of these symptoms on daily functioning is not immediately clear. The complaints of patients with encephalopathy may seem “vague” in part because of the patients’ difficulties in recalling specific examples and organizing their histories or interviews. In patients presenting with memory and sleep problems, for example, symptoms which may appear vague, are often of profound duration and severity. While everyone from time to time may forget a word or a name, or lose their car keys, patients with Lyme encephalopathy exhibit these types of problems repetitively and with marked frequency many times in a single day.
It should be noted that a complaint remains vague only as long as the clinician allows it to remain vague.” And “… what often appears ‘vague’ to the doctor can be devastating to the patient. “18 Where signs of encephalopathy are persistent and disabling, neurologic, psychiatric, and neuropsychiatric evaluations are essential and invaluable adjunctive aids for the clinician.19-24
ADULTS WITH LYME ENCEPHALOPATHY
Adults with Lyme encephalopathy commonly present with memory and attention problems, language difficulties (including dyslexia, word-finding problems, word misuse and mispronunciation), impaired calculation and reasoning (with number reversal and inability to perform simple math problems, including inability to make change), impaired judgment and problem solving, major to severe depression (with suicidal ideation and attempts), and sleep disturbance (ranging from hypersomnolence to insomnia). Patients’ symptom descriptions are dramatic, and, to an attentive and alert clinician, should be readily recognizable. These difficulties can be measured objectively by neuropsychological tests, and studies have shown that significant impairment is not uncommon.’-5’24
Adults with Lyme encephalopathy describe their problems as an increase in the forgetting of names, conversations, phone numbers, directions, and appointments. Many are highly distractable and misplace objects, forget how to drive their own cars, frequently get lost driving in their own neighborhoods, cannot recall information they’ve just heard, seen on TV, or read in books or newspapers. Many rely heavily on reminder notes and some have been known to tape Post-it notes to themselves to remind them of tasks in progress. By the time a doctor sees these patients many are totally dependent on family members for assistance with daily errands and meals.
Commonly patients with Lyme encephalopathy are mentally overwhelmed by the simplest of tasks, like composing and mailing a letter or writing a check. Many patients require “lists” for everything from the important to the mundane, highlighting the fact that they are no longer able to multitask (or do more than one task at a time), and can neither plan, schedule, nor prioritize—the given executive functions in individuals with healthy frontal lobes.
Outside the doctor’s office, this breakdown in memory and attention translates into major vocational disability, resulting decline in financial status, and severe impact on quality of life.
Impact of Lyme encephalopathy on vocational functioning:
• A highly educated financial analyst who had sudden problems with math and dyslexic number reversals, fatigue and concentration, stopped work for fear of making major mistakes.
• A nurse quit her job because she was seriously afraid of giving patients the wrong medications or wrong doses of medication. She also feared giving wrong medications to the wrong patients.
• A building contractor nearly lost his business because he could no longer remember how to cost-estimate jobs, perform accurate measurements, or prepare building drafts.
• A controller stopped work and changed careers when simple bookkeeping became impossible and for the first time this accurate accountant was handing in reports with six-figure mistakes. With extensive training in math and accounting, this individual suddenly found it difficult to make simple change.
• Editors and teachers alike ended their careers when they could no longer remember basic spelling and grammar. The editor, who suddenly had problems distinguishing spellings of homophones like “pear” from “pair” or “there” from “their” or “they’re”, had a master’s degree in English from an ivy league school and was qualified to teach English on a college level.
• A 27-year-old landscaper who could no longer run his own business due to complete confusion and could no longer function independently committed suicide.
Impact of Lyme encephalopathy on social functioning:
Problems of memory loss, inattention, and confusion result not only in job loss and consequent decline in financial status but in isolation and social withdrawal that accompany these changes in mental status and standard of living. Numerous patients with Lyme encephalopathy also experience sensory abnormalities such as extreme sensitivity to light, sound, and motion.4’8 These symptoms further isolate patients by keeping them homebound and/or dependent on caregivers for any travel outside of the home. Patients with acute dizziness and sound sensitivity (where a collapsed tolerance to environmental noise can cause physical pain, total distraction from tasks at hand, or make individuals withdraw and retreat to dark, silent locations) particularly tend to lead very restricted lifestyles, seldom even leaving their homes, rarely going to social gatherings or public places.
Impact of Lyme encephalopathy on independence:
In addition, memory and attention disturbances may cause numerous driving accidents. Many encephalopathic adults have complained about car accidents, near accidents, and difficulty driving, particularly on highways (with missed
exits; inability to remember cars behind them or in their blind spots, etc), or at 4-way intersections where they have to remember instantaneous light signals or “go-ahead” signals of other drivers. One patient, an owner and enthusiast of SAAB automobiles for 20 years suddenly could no longer identify or locate the various switches, dials, and buttons on the car dashboard—a dashboard whose design has changed very little in 20 years. All of these hurdles add up so that ultimately travel is restricted and the Lyme encephalopathic patient is further cut-off from his/her premorbid independence.
With job loss, social withdrawal, an onslaught of unusual and unpredictable sensory abnormalities, and restricted transportation, comes a decline in ability to maintain one’s sense of self-esteem. And, as the person with this organic brain condition who “looks well” to the public, but privately continues to intellecutally deteriorate, grows increasingly dependent on others to accomplish the simplest routines and errands, breakdowns subsequently occur in family and social relationships.
Impact of Lyme encephalopathy on psychosocial functioning:
Severe depression with suicidal ideation and suicide attempts, part of the Lyme encephalopathic picture and not situational in nature, further exacerbate the adult’s overall decline in quality of life. Dramatic mood and personality changes ranging from lability and bipolar disorder to violence,g’21 obsessive compulsive disorder,g’11-12 psychosis, and Alzheimer’s- and schizophrenia-like dementia9’22’2s-3° (severe and extreme manifestations of neuropsychiatric Lyme encephalopathy which have been extensively documented in the international medical literature), lead to destruction of families and, in less severe cases, erode patients’ abilities to provide adequate and effective parenting.31 Fatigue and sleep disturbance (ranging from hypersomnolence to complete insomnia), again, independent of the depression and part of the constellation of Lyme encephalopathy, render patients, especially depressed patients, essentially nonfunctional.
The quality of life, then, for adults with Lyme encephalopathy is one marked by major declines in vocational, social, independent, psychosocial, and recreational functioning, and overall deterioration in cognitive and neuropsychiatric status. Patients who have lost their jobs, standard of living, ability to engage in activities with family and friends, physical and mental independence, and who are grossly immobilized by major depression or
bizarre behavioral changes and sleep disturbance, individuals who have little concentration to focus on very much or for very long periods of time, or who can no longer sustain interest in former goals or hobbies, are clearly living from day to day, from minute to minute, neurologically impaired, invisibly disabled, and leading extremely curtailed lives.24
CHILDREN AND ADOLESCENTS WITH LYME ENCEPHALOPATHY
For children and adolescents with Lyme encephalopathy the most frequent complaints, mainly attentional and behavioral in nature, ultimately affect QOL areas of education and social development. Children with Lyme encephalopathy are not able to concentrate or study in school and not able to learn and remember new information. Intense fatigue prohibits students of all ages from keeping up with the pace and volume of schoolwork, staying awake in classes, and making and maintaining friendships. Acute sensitivities to light, sound, and multistimuli also make concentration difficult and can exacerbate personality and mood changes. Behavioral symptoms can range from sudden fussiness and irritability in very young children,15’32 to anger, rage, violence, major depression,9 and obsessive compulsive ritualsl 1-12 in children and adolescents. For example, one premorbidly polite, easy-going 1.3-year-old boy with Lyme encephalopathy exhibited sudden, uncontrollable cursing which resolved soon after antibiotic therapy.
Educational hurdles dramatically affect college-bound adolescents with Lyme encephalopathy as well. This group frequently requires non-standard forms of testing like special time-extended PSATs and SATs. Once in college, these students may flounder due to, heavy work loads and short semester deadlines, or struggle to adapt by scheduling classes around daily rest periods. Numerous college students with this condition however, drop out of school or take many class extensions (in excess of the traditional four-year program) and, again, with the supportive aid of tutoring.
Impact of Lyme encephalopathy on education:
Dramatic increases in attention and behaviorial changes cause students to fall behind in school. Aside from the invisible multisystemic symptoms and pain of Lyme disease, these unseen and poorly understood neurologic components
cause children and adolescents to miss many school days, sometimes months to years at a time. Often, in-school tutoring is sufficient to carry them through difficult stages of their illness, but more commonly, at-home tutoring becomes necessary.” Home-tutoring, which is expensive and, according to one estimate, can cost anywhere between $50 to $65 an hour,33 (and which normally would be paid for by the student’s school district) is not universally accepted or implemented across school districts and from state to state.34 In fact, many schools do not cover tutoring for the “intermittently” chronically ill child—the Lyme disease student for example, who can be well one day and drastically sick the next.34 However, new and creative solutions are being experimented with in places like Suffern, New York, where attempts are being made to bring the homebound student into the classroom via video conferencing.3s
Impact of Lyme encephalopathy on social development:
The overall affect of Lyme encephalopathy on QOL for children and adolescents with this condition remains one characterized by disrupted education and resulting isolation, loneliness, and frustration. Unable to keep up with schoolwork or the daily activities of their friends, these individuals are soon forgotten by their peers. It is tragic to see formerly healthy children being raised as isolated invalids. As one Lyme disease children’s support group leader explains, “It’s as if these children are missing out on their own childhoods….There is a sense that the world is moving on without them.”16
Major depression, a frequent presentation of the encephalopathic state (and not primarily situational in nature) complicates the young person’s ability to develop normal, adequate coping skills. While adults with Lyme encephalopathy have already acquired years of education, and many years’ worth of memories and experiences from which to draw upon for coping with the unusual pain and confusion of this organic condition, young children with Lyme encephalopathy lack this advantage. They are clearly at a loss to articulate or explain to themselves, their parents and families, friends, and most importantly, doctors, what it is that they are going through—what it is that invisibly comes and goes with such ferocity and unpredictability, making their lives something that they cannot begin to explain to anyone who has not experienced this condition. How. do you explain that the teacher talks but it’s as if you don’t hear any of the information? Or how do you explain your need to wear sunglasses indoors or earplugs at your family dinner table?
Probably the most disturbing aspect of QOL for a child or adolescent with Lyme encephalopathy is that many have been hastily misdiagnosed as having learning disabilities or attention disorders, or worse, hypochondriasis. Their complaints have been repeatedly written off, outright dismissed, or mistakenly attributed to psychiatric or psychologic problems.15’32 Parents of children with this condition are told that their child’s problems are psychiatric even though tests have not been done to indicate such types of problems. Symptoms of Lyme encephalopathy may be frequently mistaken for multiple sclerosis, postinfectious encephalopathy of other etiologies, or psychiatric illness.37 This mislabeling can be emotionally devastating for the child and no doubt puts a tremendous strain on the entire family.
Children therefore suffer a similarly compromised quality of life—but a more tragic one in that they are in a situation where they are robbed of the ability to build essential self-esteem and confidence—core elements in normal human development., The impact of Lyme encephalopathy on children is one that strongly affects functional areas of education, interpersonal/social development, psychosocial development, and recreational functioning. A Centers for Disease Control study performed in 1992 in New Jersey revealed an astronomical social and economic impact of Lyme disease in children: Medical cost average $96,000; educational loss for students of at least half a year of school; decline in school performance and disruption of social activities.38 So, whereas adults with this illness, particularly with the more neurologically debilitating aspects of this disease, suffer a decline in vocational functioning and hence total professional QOL, children are most affected in their education, which is, in effect, a preparation and indicator for success in future vocational functioning.
It is now strikingly apparent that adults, children, and adolescents with Lyme encephalopathy share a severely compromised quality of life. Even on “good days” when they look and appear well and seem to be functioning up to speed, they continue to live on the fringes of mainstream society, some intensely confused but hiding it as they fight to cling to jobs or to keep up with their education; others quietly disoriented yet trying to remain selfsuff’icient; and the majority cognitively and/or psychiatrically impaired. While not visibly handicapped, these patients are challenged daily by malfunctioning cognitive, sensory, and neuropsychiatric systems which leave many of them living at home—many in their parents’ homes—unemployed, functionally dependent, isolated, depressed and suicidal, apathetic and increasingly unemployable.
Many have not been cognitively rehabilitated and continue to cost society millions of dollars in lost productivity and increasing insurance and disability claims. Others continue to cause an increased drain on special, expensive medical and educational resources needed to keep them functioning on a daily basis. Inevitably, a portion of these patients (ones who were originally inadequately treated, misdiagnosed, or simply undiagnosed) will likely pose a further burden to society via the phenomenal cost of long-term psychiatric and mental institutionalization and/or incarceration in jails.13’24 The cost of increased Lyme encephalopathic-related violence and crime is something that has been recently alluded to, yet requires further investigation.4,74,13’214’29 So, while the academic community continues to define Lyme encephalopathy as a “mild confusional state characterized by alterations of memory, mood, and sleep,” the patients’ experience is one of devastating, phenomenally expensive, invisible disability. Lyme encephalopathy affects all aspects of patients’ glnality of life by halting intellectual growth and development, derailing professional and scholarly achievement, and ending futures that might have been promising or full of contribution to the betterment of society. The patients’ experience of Lyme encephalopathy is that, in many cases, it is a long-lasting, damaging assault against an individual’s total well-being, and one that is ultimately debilitating and unquestionably disabling.
The problem of Lyme encephalopathy is enormous and critical, and something that affects us all. The medical and research community must recognize the significance of this brain condition, its impact on patients’ quality of life, and its future implications for society. It must encourage and support research which will advance our understanding and treatment of this serious, complicated, and frequent manifestation of Lyme disease. Special thanks to Cynthia Onorwto, Lyme Disease Patient Advocate, in the preparation of this manuscript. Special thanks to Marian Rissenberg, PhD, Neuropsychologist in private practice, Katonah, NY, for her suggestions. Additional thanks to Martha Agricola, FM’, Martha Kramer, Suzanne Sugar, Pat Walsh, and members of the Lynne Disease Coalition of New York and Connecticut for their support and encouragement.