Medical Concerns in Adults with Developmental Disabilities

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Medical Concerns in Adults with Developmental
Disabilities

• Sharon Witemeyer, MD
• Continuum of Care Project
• UNM-HSC

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Medical Concerns in Adults with Developmental
Disabilities Objectives

• Define Developmental Disabilities
• Become familiar with common co-morbidities
• medical conditions
• polypharmacy
• communication issues
• Become familiar with common medical concerns for
  women with developmental disabilities
• Know some ways to enhance the DD patients visit
  to the office

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Developmental DisabilityDefinition

• Injury to the developing brain before age 22
  years that impacts function in
• - mobility
• - communication
• - self help
• - independence
• - learning

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Individuals with Developmental Disabilities NM
Population

• 1-2 of the total population
• 20,000 with all types
• 1,000-1,500 severe
• All reside in the community
• Life expectancy in the 1930s was 18.5 yrs
• Life expectancy today is 66.2 yrs

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COMMON CO-MORBIDITIES

• Medical Conditions
• Polypharmacy
• Communication Issues

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MEDICAL CONDITIONS

• Some medical conditions commonly seen among
  individuals with developmental disabilities are
  intimately associated with the disability itself.

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Some Diagnoses Associated with Specific Medical
Conditions Include

• Cerebral Palsy
• Down Syndrome
• Spina Bifida

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MEDICAL CONDITIONS IN CEREBRAL PALSY

• Spasticity or Hypotonia
• Movement Disorder
• Contractures
• Limb and chest wall deformity
• Kyphoscoliosis
• Seizure disorder

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MEDICAL CONDITIONS IN DOWN SYNDROME

• In addition to the typical physical features,
  Down Syndrome may be associated with
• Congenital Heart Defects (40) AV communis,
  VSD, PDA, ASD, and aberrant subclavian artery
• GI Defects esophageal atresia, duodenal atresia
• GI problems GERD, increased triglycerides,
  decreased HDL cholesterol, apolipoprotein A1,
  HDLTG ratio, Hirschprungs, Celiac disease,
  obesity,constipation, neonatal jaundice

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MEDICAL CONDITIONS IN DOWN SYNDROME

• Developmental brain abnormalities delayed
  myelination, fewer neruons, decreased synaptic
  density, decreased acetycholine neurotransmitter
  receptors, mental retardation
• ADHD
• Hypotonia
• Seizures (5-10)
• Autism
• Sleep Apnea

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MEDICAL CONDITIONS IN DOWN SYNDROME

• Thyroid problems neonatal hypothyroidism (27 X
  normal), hypothyroidism, hyperthyroidism
• Atlanto-axial instability
• Abnormalities of immune function
• Vision and hearing impairments
• Dementia

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Things to consider before making the diagnosis of
Alzheimers

• Electrolyte imbalance Stroke
• Hydrocephalus Other Dementias
• Thyroid disease Other Psychiatric Problems
• Vitamin deficiency
• Infection
• Drug toxicity
• Depression
• Subdural hematoma
• Brain tumor

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MEDICAL CONDITIONS IN SPINA BIFIDA

• Hydrocephalus
• VP/AP shunt malfunction
• Sleep Apnea
• Tethered cord
• Chronic urinary tract infections
• Skin breakdown
• Obesity

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MEDICAL CONDITIONS

• Some medical conditions are found more frequently
  among individuals with any developmental
  disability than in the general population.

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MEDICAL CONDITION COMMON AMONG INDIVIDUALS WITH
DD REGARDLESS OF ETIOLOGY

• Endocrine especially thyroid problems
• GERD
• Osteoporosis
• Weight concerns
• Constipation

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MEDICAL CONDITIONS

• Among the medically fragile and developmentally
  disabled common concerns include
• Aspiration risk
• Marginal nutritional status
• GT/JT placement risks including bowel
  obstruction or perforation and diaphragmatic
  hernia
• Tracheotomy
• Pulmonary insufficiency due to scoliosis
• Marked osteoporosis, silent fractures

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MEDICAL CONDITIONS

• Of course specific syndromes are associated with
  a myriad of other abnormalities such as
• Congenital anomalies
• Renal/Metabolic disease
• Cardiac disease
• Vision/Hearing impairments

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MEDICAL CONDITIONS

• Individuals with DD are frequently brought to the
  doctor because of behaviors that can vary from
  mild to extremely severe. Before assuming it is
  just behavior or prescribing a psychotropic
  medication, consider some of the common medical
  conditions that may be manifested in this way.

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MEDICAL CONDITION OR BEHAVIOR PROBLEM?

• PAIN from
• Otitis
• Sinusitis
• Dental problem
• GERD
• Glaucoma
• Undiagnosed fracture

• Hidden abscesses
• Foreign bodies
• UTI
• Renal or gallstones
• Headache
• Constipation

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MEDICAL CONDITION OR BEHAVIOR PROBLEM?

• Thyroid disease
• Sleep apnea
• Spinal cord problems
• Increased ICP
• Vitamin deficiencies (especially the B vitamins)
• Drug side effects

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Review of 150 patients with DD and MI seen in DD
Clinic

• Number of Medications
  Number of Individuals
• 0 8
• 1 10
• 2 22
• 3 20
• 4 24
• 5 27
• 6 16
• 7 11
• 8 7
• 9 2
• 10 2
• 12 1
• 13
  1

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Review of 150 patients with DD and MI seen in DD
Clinic

• Number of medications per patient 1-13
• Mean number of medications per patient 4.45

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POLYPHARMACY

• Side Effects
• Neurologic paresthesias, seizures, psychiatric,
  movement disorders,headaches, delirium
• GI dry mouth, heartburn, pain, constipation,
  anorexia, bleeding
• Cardiac arrhythmias
• GU retention, incontinence
• Bone - osteoporosis

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POLYPHARMACY

• Drug-Drug Interactions
• SSRI
• Anticonvulsants
• Neuroleptics
• GI drugs acid blockers, proton pump inhibitors
• Antibiotics
• Birth control pills

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POLYPHARMACY

• Idiosyncratic and Allergic Reactions
• Benadryl
• Phenobarbital
• Benzodiazepines
• Neurontin

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COMMUNICATION

• Non-verbal
• Limited expressive language skills
• Limited receptive language skills
• Usually history must be obtained from a third
  party parent, guardian, or direct care provider

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Most women with developmental disabilities have
• normal female anatomy
• normal endocrine function
• normal breast development

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Some women with developmental disabilities do not
  have normal reproductive systems.
• Pituitary dysfunction may be seen in women with
  central nervous system malformations as the
  result of genetic, congenital, traumatic or post
  operative abnormalities.
• Thyroid dysfunction is more common in
  individuals with developmental disabilities

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Women with spina bifida frequently enter puberty
  early
• Reproductive endocrine disorders occur more
  frequently among men and women with epilepsy
• Anticonvulsant medications used in this
  population are associated with ovarian
  dysfunction (Polycystic Ovary Syndrome)

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Most women with developmental disabilities have
• normal female anatomy
• normal endocrine function
• normal breast development

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Many families and physicians are reluctant to
  provide routine gynecologic health maintenance to
  women with developmental disabilities because
  they think it is
• too traumatic
• takes too much time
• simply a waste of time

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Women with developmental disabilities need and
  deserve the highest standard of medical care
  available and this includes reproductive health
  surveillance

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REPRODUCTIVE HEALTH CARERECOMMENDATIONS

• Breast Examinations
• First breast examination at age 18 years
• Annual examination thereafter
• Many women with developmental disabilities are
  unable to perform breast self examination

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REPRODUCTIVE HEALTH RECOMMENDATIONS

• Mammograms
• First mammogram at age 50 years
• Annually thereafter
• If there is a family history of breast cancer in
  mother, sister, daughter or two close female
  relatives or known genetic risk (BRCA1 or BRCA2
  genes) then first mammogram should be at age 35
  years. Annual mammograms thereafter

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REPRODUCTIVE HEALTH RECOMMENDATIONS

• Pelvic Examinations and Pap Smears
• First pelvic examination and Pap smear when
  patient becomes sexually active or age 18 years
• Annual pelvic examination and Pap smear for 3
  years. If normal exams and 3 negative Pap smears
  the Pap smears can be done every 2-3 years

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REPRODUCTIVE HEALTH RECOMMENDATIONS

• Pelvic Examinations and Pap Smears
• Annual pelvic examinations
• Annual pelvic examinations and Pap smear after
  age 40 years
• The problem with increasing the interval between
  Pap smears is that many women will not return for
  a routine annual pelvic examination and ovarian
  cancer may be missed.

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Pelvic Examination
• Position may be limited by patient anxiety or
  physical deformities (flexion contractures,
  paralysis, spasticity)
• Visualization
• Bimanual
• Speculum Use a narrow speculum such as a
  Pederson for virginal patients
• Pap Smear may use a Q tip blindly to sample os

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Common Problems

• Dysmenorrhea and PMS
• Withdrawn behavior, rocking motions,
  vocalizations of a distressed nature, aggression
  toward caretakers or self-abuse use
  Prostaglandin inhibitors
• PMS violent, physically abusive to staff or
  selves use Prostaglandin inhibitors, OCPs or
  long-acting progestin

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REPRODUCTIVE HEALTH CARE FOR WOMEN WITH DD

• Women with developmental disabilities are more
  likely to have been sexually abused than women in
  the general population

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PREVENTATIVE HEALTH CARE

• Immunizations
• Obesity nutrition, exercise
• Tobacco
• Alcohol and Substance Abuse
• Responsible/Safe Sex
• Check our website (http//star.nm.org/coc) for
  syndrome specific health-watch recommendations

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COMMON PROBLEMS OSTEOPOROSIS

• Osteoporosis is usually defined as a reduction of
  bone mineral density per unit volume of bone. It
  is caused by reduced bone matrix.
• Osteomalacia is caused by reduced mineral
  deposition secondary to Vitamin D deficiency
• Osteopenia refers to an early form of osteoporosis

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COMMON PROBLEMSOSTEOPOROSIS

• Osteoporosis and the risk of fractures is a
  significant problem in people with developmental
  disabilities.
• There is little medical literature that
  addresses the problem. Those studies that do
  exist report increased prevalence of osteoporosis
  among female and male, young and old with
  developmental disabilities.

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COMMON PROBLEMSOSTEOPOROSIS

• Fracture rate for individuals with developmental
  disabilities is
• 1.7-3.5 times greater than the rate for the
  general population.

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COMMON PROBLEMSOSTEOPOROSIS

• Factors associated with osteoporosis in
  individuals with developmental disabilities
• 1. Immobility
• 2. Gonadal/Endocrine Dysfunction
• 3. Medications (i.e.ACM)
• 4. Small Body Size (i.e. Down Syndrome)

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COMMON PROBLEMSOSTEOPOROSIS

• Diagnosis
• DEXA determination of bone mineral
  density (BMD) is the gold standard but is not
  always possible in individuals with developmental
  disabilities because of deformity or inability to
  cooperate.
• Urinary peptides may provide an alternative in
  the future.

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COMMON PROBLEMSOSTEOPOROSIS

• Prevention
• 1.Calcium (1000-1500mg/day)
• 2.Vitamin D (400 units/day)
• 3.Weight bearing exercise
• 4.Avoid smoking and excess alcohol consumption

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COMMON PROBLEMSOSTEOPOROSIS

• Treatment
• 1. Fosamax
• 2. Didronel
• 3. Calcitonin
• 4. Estrogen in women
• 5. Androgen in men
• 6. Parathyroid hormone (Forteo) ?

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THE DD PATIENT IN THE OFFICE

• Preparation of the Patient
• Explain ahead of time
• Role play
• Define desired behavior
• Rewards and reinforcers
• A tour visit
• A familiar person to accompany the patient

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THE DD PATIENT IN THE OFFICE

• Preparation of the office staff
• Schedule on days that are not crowded
• Plan for a short waiting time
• Schedule a series of appointments
• Provide staff for continuity
• Explain procedure calmly and in a soft voice
• Know that the procedure may take longer
• Consider specifics of the disability
• Avoid restraints, be flexible
• Safety is a priority

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THE DD PATIENT IN THE OFFICE

• PATIENCE
• SENSITIVITY
• FLEXIBILITY
• CREATIVITY
• DIGNITY
• RESPECT

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