Pediatric Lyme Disease

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							                                                                                                                                                                   10/3/2004




             Pediatric Lyme Disease

                             Dr. Ann F Corson




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                             Risk Factors                                                                                     Risk Factors
                                                                                                   • Having pets that come in and out, dogs or
•   Age 10-19
                                                                                                     cats
•   Rural (suburban) vs. urban, 3x risk
•   Single family homes                                                                            • Outdoor activities:
                                                                                                            • Horseback riding
•   Homes with yards +/- woods or attached land
                                                                                                            • Hunting, fishing
•   Homes within 100 feet of woodland
                                                                                                            • Any activity in the woods or open land or abutting
•   Tick hosts being seen on land: deer, mice                                                                 high grasses including field sports, golf
           Public Health Reports 2001, Volume 116, 146-156. Risk Factors for Lyme Disease in
           Chester Country, Pennsylvania
                                                                                                            • Activities in any outdoor area frequented by deer
• CDC: Any child under 9 yrs at risk with many
  new cases LD in children under 14 yrs                                                            Anyone can be infected on any
           MMWR 1991. 42; 557-558
                                                                                                    warm day of any month of the year!
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               Signs and Symptoms                                                                                  Signs and Symptoms
• Lyme disease is truly is the “great imitator” of our                                                   • All children
  time just as syphilis was for prior generations                                                             – Tick bite – less than 50% remember a bite,
• All organ systems of the body can be affected                                                                 even less remember an EM rash
• Onset of illness may be abrupt or indolent                                                                  – Flu-like illness at any time of year in 80%
• Symptoms are often vague and shift from day to                                                                (parents often claim the child was never
  day therefore many children are thought be                                                                    well again)
  maligning
                                                                                                              – Fatigue, often unrelieved by rest, in 100%
• Children often don’t understand what is wrong
  with their bodies                                                                                           – Unexplained cyclical fevers
                                                                                                              – Headaches occur in 90% to 100%

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                Signs and Symptoms                                                             Signs and Symptoms
                                                                               • All children, cont.
• All children, cont.
                                                                                     – Abdominal pain in about 50%, can mimic
      –   Dizziness
                                                                                       acute appendicitis, mesenteric adenitis,
      –   Neck pain and stiffness in almost 90%
                                                                                       Crohn’s disease, colitis, irritable bowel
      –   Sore throats
                                                                                       syndrome, sometimes vomiting, heartburn
      –   Swollen lymph nodes
      –   Excessive thirst                                                           – Urinary urgency and frequency, sudden lack
      –   Chest pains in at least 70%, some have palpitations
                                                                                       of control in toilet trained child, return to or
      –   Sense of air hunger or shortness of breath, dry cough
                                                                                       new onset enuresis.
                                                                                     – Rashes that come and go, malar rashes, new
                                                                                       psoriasis
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                Signs and Symptoms                                                             Signs and Symptoms
• All children, cont.
                                                                               • All children, cont.
     – Migratory arthralgias, joint pains in 50% to
       100% (arthritis in only 40%), myalgias in over                                – Neurological symptoms are protean and can
       80%, back ache, morning stiffness, pain at                                      appear at any time during the course of
       rest, muscle weakness                                                           infection (few weeks to over a year or more)
                                                                                          • Hypersensitivity of skin, scalp and hair
     – Frequent illnesses, dark circles under their                                       • Hypersensitivity to noise, light, smell
       eyes, intermittent red, hot pinnae of ears                                         • Alterations of taste
     – Sleep disturbance in over 80%                                                      • Poor balance and coordination
          • Trouble falling asleep                                                        • Uncharacteristic behavior outbursts, mood disturbances,
          • Frequent awakenings                                                             depression in over 90% with suicidal thoughts in over 40%
          • Excessive sleep                                                               • Social withdrawal, decreased participation
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                Signs and Symptoms                                                             Signs and Symptoms
• All children, cont.                                                          • All children, cont.
      – Neurological symptoms, cont.                                                 – Neurological symptoms, cont.
           • New onset phobias, anxiety disorders                                         • When measured with formal neuropsychiatric testing,
           • Oppositional behaviors                                                         children demonstrate defects in auditory and visual
           • Obsessive compulsive disorders                                                 sequential processing
           • Deterioration in school performance in over 90%                          less commonly:
           • Difficulty with concentration and attention in school with                  • Movement disorders – spasticity, ataxia, motor or vocal
             easy distractibility as well as “brain fog” in over 80%                       tics
           • Word finding problems in over 80%                                           • Cranial neuropathies, e.g. Bell’s Palsy or optic nerve
                                                                                           neuritis (can result in visual loss)
           • Short term memory difficulties in over 90%
                                                                                         • Peripheral neuropathies – numbness and tingling, distal
           • New onset ADD
                                                                                           parasthesias, subtle weakness
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                 Signs and Symptoms                                                            Signs and Symptoms
                                                                                • Adolescents
• All children, cont.
                                                                                      – Parents and teachers may think any unusual
      – Neurological symptoms, cont.                                                    behaviors are just “normal” adolescence or
           •   Peripheral motor weakness
                                                                                        problems such as illicit drug use or new onset
           •   Partial complex seizures
                                                                                        psychiatric disorder
           •   Apparent demyelinating disease (multiple sclerosis)
           •   Spinal cord involvement                                                – Mood swings, oppositional behaviors, anxiety,
           •   Pseudo tumor cerebri or increased intracranial pressure,                 depression
               papilledema
                                                                                      – Self mutilating behaviors
                                                                                      – Teenagers often do not report to or show
                                                                                        parents problems with their bodies
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                 Signs and Symptoms                                                            Signs and Symptoms
• Adolescents, cont.                                                           • Pre-schoolers and toddlers
      – Teens can also turn to alcohol and illicit drugs                             – Mood swings, sudden emotional outbursts
        as self medication                                                           – Irritability
      – Teenage girls may have pelvic pain or                                        – Personality changes
        menstrual problems, ovarian cysts, boys may                                  – Regression of motor and social skills
        have testicular pain                                                           (developmental milestones)
      – Teens need to be aware that Borrelia may be                                  – Changes in play behavior, tire easily, less
        sexually transmitted and that a fetus can                                      active
        acquire the infection from the mother during
        pregnancy
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                 Signs and Symptoms                                                       Congenital Lyme disease
• Pre-schoolers and toddlers, cont.                                            • Infants can be infected with Borrelia
      – Trouble falling asleep, frequent awakenings                              transplacentally in any stage of pregnancy
      – Nightmares, new phobias, recurrence of                                   and/or via mother’s breast milk.
        separation anxiety                                                     • The co-infections: Babesia, Bartonella,
      – Diaper rash unresponsive to normal treatment                             Mycoplasma and perhaps even the
      – Frequent URIs, ear and throat infections,                                Ehrlichias can be transmitted
        bronchitis, pneumonia                                                    transplacentally to the developing fetus.


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           Congenital Lyme disease                                     Congenital Lyme disease
• Gestational Borreliosis can be associated                 • Borrelia spirochetes have been found at autopsy
  with repeated miscarriages, fetal death in                  in fetal brain, liver, adrenal glands, spleen, bone
  utero, fetal death at term (stillbirths),                   marrow, heart and placenta
                                                                  – None of the infected tissues showed any sign of
  hydrocephalus, cardiovascular anomalies,                          inflammation
  intrauterine growth retardation, neonatal                 • Maternal antibiotic treatment during pregnancy
  respiratory distress, “sepsis” and death,                   does not guarantee that the fetus will be free of
  neonatal hyperbilirubinemia, cortical                       infection
  blindness, sudden infant death syndrome                   • Mothers with Lyme disease should be treated
  and maternal toxemia of pregnancy.                          throughout pregnancy
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           Congenital Lyme disease                                     Congenital Lyme disease
• Infants either infected congenitally or from              • Infants infected congenitally can have
  breast milk can have
                                                                  – Small windpipes (tracheomalacia)
      – Floppiness with poor muscle tone
      – Irritability                                              – Eye problems (cataracts)
      – Frequent fevers and illness early in life                 – Heart defects
      – Joint sensitivities and body pain                   • Infants bitten very early in life will have
      – Skin sensitivity                                      many of the same symptoms
      – Gastro esophageal reflux                                  – loss and decline in developmental milestones
      – Developmental delays
      – Learning disabilities and psychiatric problems
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                           Co-Infections                                               Co-Infections
• Bartonella henselae: abdominal pain,                      • Ehrlichiosis: high fevers, headaches,
  headache, visual problems, significant lymph                muscle pains, flu-like symptoms. Labs
  node enlargement (e.g. mesenteric adenitis),                can show low WBC, platelets and
  rashes, unusual “stretch marks”, resistant
  neurological deficits, new onset seizure disorder,
                                                              increased liver enzymes
  acute encephalitis                                        • Mycoplasma sp.: fatigue, abdominal pain
• Babesia microti (and other species): malarial             • Viruses: abdominal pain, mouth sores
  like illness inside red blood cells with intermittent
  fevers, chills, day and night sweats, abdominal
  pain, profound fatigue
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                           Co-Infections                                                        Evaluation
  Co-Infections are the rule, not the exception                     • Tick borne disease is a clinical diagnosis
• Co-Infections are often best diagnosed clinically                 • Laboratory testing can be very difficult as many
                                                                      patients are serologically negative for antibodies
• Co-Infected patients are:
                                                                      to Borrelia despite active infection
      – sicker
                                                                    • Routine labs are usually unremarkable
      – more likely to have failed prior treatment
                                                                    • Even the majority of spinal taps reveal normal
      – require longer treatment with multiple agents
                                                                      spinal fluid
• Co-Infections must be eradicated or Borrelia                      • Full evaluation at labs that specialize in TBD can
  infection will persist                                              be very helpful although negative results do not
                                                                      mean absence of disease
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                            Treatment                                                          School issues
• Treatment lasts as long as is necessary                           • Teachers, school administrators, school
      – Until children are completely symptom free for 2 to 3
        months
                                                                      health professionals, pediatricians, family
      – No more cyclical Herxheimer reactions                         practitioners and parents all need to be
      – No recurrence of Lyme symptoms with concomitant               aware of the protean manifestations of
        infections, illnesses or stresses, e.g. surgery, trauma,
        psychological stresses
                                                                      TBD
• Sickest children often need many months of                        • Mental health professionals and educators
  intravenous antibiotic therapy                                      in Lyme endemic areas need to recognize
• Children whose diagnosis and treatment are                          the possible infectious etiology of
  delayed may suffer considerable impairment
                                                                      neuropsychiatric disease in children.
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                           School issues                                                       Social impact
• It is imperative to identify children with persistent             • Isolation
  neuroborreliosis so they can receive appropriate
  medical, psychological and educational                            • Loss of peers and normal socialization
  assistance.                                                       • Loss of self-esteem
• Two Federal laws exists that protect students
  with Lyme disease and supercede state codes                       • Inability to participate in sports or
  and regulations:                                                    extracurricular activities
      – IDEA: Individuals with Disabilities Education Act           • Loss of academic work
        www.ideapractices.org
      – Section 504 of the 1973 Rehabilitation Act                  • Interruption of normal family life
        www.504idea.org

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                           Social impact                                                              Prevention
• Quote from Pat Smith, President of the Lyme                              • Avoid exposure to ticks
  Disease Association, from her address to                                 • Get the deer out of your yard and spray yard
  Congress:                                                                  with permethrin
    “The emotional damage these children suffer is                         • Clear away underbrush and cut back shrubbery
    tremendous and it follows them though out the most
                                                                           • Wear protective clothing and use appropriate
    impressionable stages of their lives. To get out of
    bed is an accomplishment, to shower is a miracle.
                                                                             insecticides while outdoors
    They have few or no friends, no regular school                         • Put out Damminix® for mice to use in nests
    attendance, no sports or activities, and no self-                      • Contact your local government regarding tick
    esteem. Some contemplate suicide, unfortunately,                         and deer control and elimination
    some are successful.”
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                             Caveats                                                                   Caveats
• Any child who becomes ill after a tick bite                              • Neurological signs and symptoms are often the
                                                                             most common indication of persistent infection
  needs a full evaluation for the presence of                                after inadequate treatment
  co-infections                                                            • In 1989, Dr. Pachner predicted that “If, as it now
• Any child who becomes ill after a tick bite                                seems, the Lyme spirochete is indeed highly
  who was treated with 3 to 4 weeks of oral                                  neurotrophic and able to remain dormant in the
                                                                             CNS for long periods, we may well see a sizable
  antibiotics has most likely been                                           number of individuals who currently have latent
  inadequately treated.                                                      neuroborreliosis presenting in the future with
• Initial inadequate treatment makes future                                  symptomatic infection.”
  treatment more difficult
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                  Selected references                                                        Selected references
Andrew Pachner. Neurological Manifestations of Lyme Desease, the
  New “Great Imitator”, Reviews of Infectious Deseases. Vol II,            MacDonald, MD. Gestational Lyme Borreliosis Implications for the
  Supplement 6. September-October 1989.                                      Fetus, Rheumatic Disease Clinics of North America, Volume 15,
                                                                             Number 4. November 1989.
Pietrucha, MD. Neurological Manisfestations of Lyme Disease in
   Children                                                                Lyme Disease and other Tick-Borne Diseases: A Two Day Discussion
   A review of over 300 children with LD. 1991                               of the Most Recent Developments in Research and Clinical
                                                                             Management, November 13-14,1999.
Bloom et al. Neurocognitive abnormalities in children after classic
   manifestations of Lyme disease, Pediatric Infectious Disease
   Journal 1998;17:189-96.                                                 Lyme & Other Tick-Borne Diseases: Focus on Children & Adolescents,
                                                                             A National Conference for Physicians & Allied Health Professionals,
                                                                             November 4, 2000.
Fallon et al. The Underdiagnosis of Neuropsychiatric Lyme Disease in
   Children and Adults, The Psychiatric Clinics of North America.
   Volume 21 Number 3 September 1998.                                      The Lyme Times: issues July-October 1999, Winter 2001/Spring 2002,
                                                                             Fall/Winter 2002/3.
Tager et al. A Controlled Study of Cognitive Deficits in Children With
  Chronic Lyme Disease, The Journal of Neuropsychiatry and Clinical        Personal communication, Dr. Charles Ray Jones 2003-2004.
  Neurosciences 2001: 13:500-507.
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                  Selected references
•   Web sites:
      – www.ilads.org
      – www.lymepa.org
      – www.lymediseaseassociation.org




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