Primary Care for Adults with Developmental Disabilities

1. Primary Care for Adults with Developmental Disabilities Mary Ciccarelli, MD Associate Professor of Clinical Medicine - Pediatrics Indiana University School of Medicine Center for Youth and Adults with Conditions of Childhood (CYACC) IRHA Annual Meeting June 2009

2. Aging With a Developmental Disability Effects of the disability and its treatment Normal effects of aging Limited access to quality health care Lack of knowledge about aging for people with DD Inadequate funding for health care Person with a Disability Lifestyle effects Negative attitudes about people with disabilities Decreased Quality of Life

3. Crocker AC. Pediatrics,1991. “Our ability in current times to provide accurate health care for adults with mental retardation is improved but incomplete. These persons are of diverse personal and clinical background, and generalizations should be approached cautiously. For the majority, the medical needs can be expected to approximate those of average persons Consider influences of altered personal independence and self-care practices and from past experiences and supports. Others have special vulnerabilities deserving preventive and therapeutic assistance.”

4. Total Mental Retardation and/or Developmental Disability 18+ Years = 3.2% of population ~60-75% live with family ~ 25% have caregivers over age 60 Estimated Prevalence of Conditions in Non-Institutionalized U.S. Population Condition Prevalence Developmental disabilities 11.28 Mental retardation 7.80 Cerebral Palsy 4.02 Spina Bifida 0.62 Autism 0.40 Prevalence = Number of people per 1,000

5. Life expectancy comparison All Women Age 79 Women > age 40 with developmental disabilities (not Down syndrome) Age 67 Women > age 40 with Down syndrome Age 57

6. Chronic Conditions in the Adult vs. Aging DD Population Hypertension 24% 21% N-DS 9% DS Heart Disease 25% 35% N-DS 27% DS Visual Loss 0.2-0.4% 17% N-DS 70% DS Diabetes 7.5% 4.3%N-DS 9% DS Thyroid Abn. 0.2-2% 10% N-DS 45.5%DS

7. Topics • Informed consent • Adaptive function • Healthy weight • Physical activity • Vision & hearing • Dental care • Minimize medications • Risk for neglect/abuse • Advance care plans • Problem behaviors • Psychiatric illness • Cardiovascular screening • Thyroid screening • Bone health • Oromotor/swallowing problems • GE reflux • Constipation • Screen for STD’s • Evaluate for fall risk • Evaluate for seizure risk • Evaluate for changes

8. Patient and Family Centered • Respect patient’s and family’s knowledge of medical condition • Family and patient involved in decision making • Consent and Assent • Effect of patient’s condition on caregiver and family • Who is caregiver to carry out treatment plans?

9. Guardianship • In most states at age 18, person becomes responsible for health care decisions unless another adult applies for guardianship • Once patient is an adult, the level of continued involvement of previous guardian (parent) is determined by patient’s consent and need • Legal guardians • provide legal consent to care • serve as an advocate for the patient • include patient in both discussions and care as that person is able

10. Issues of health care financing • Changes in insurance coverage • Enroll in Medicaid? Medicaid Care Select? • What about Medicaid Waivers? • Autism • Developmental Disabilities • Traumatic Brain Injury • Aged and Disabled • Support Services

11. Information for the Visit • Current medications • Medicines that were recently changed and why • Problems with other medications in the past • Over the counter and herbal remedies • Recent visits to other doctors/contact information • Recent test results • Xray reports, MRI reports, etc. • Summary of past medical history • Diagnoses/ Hospitalizations/ Surgeries • Allergies • Immunizations • Family history • Dietary /toileting/sleep/activity routine • Symptoms of concern • What, when, where, how, why • Question list

12. Preparing the Patient • Anticipate the expected activities of the visit • Clothes that are easy to open/remove • Hygiene supplies • Eat or not prior to visit • Length of visit • Potential difficulties and possible solutions • Bring occupying activities for the wait • Accompany with trusted support person • Collect information from others who can not attend • Alert health caregivers to potential stressors or stressed behavior • Attempt to divert, alleviate or distract from stressors • Alternate hard activity with more pleasurable activity

13. Making the appointment What is best time of day? Does the patient need special accommodations at the office visit? Do office staff need to be prepared to accommodate needs?

14. Special visit activities • Checklist of activities • With photos • Countdown during visit • Social story preparation • Christine Sarkine Autism Treatment Center • www.handsinautism.org

15. ADA for health care facilities • Accessible parking spaces close to entrances • Accessible front entrance with ramp • Doors that are wide and easy to open • Accessible route throughout the facility • Clear floor space • Low counters, service windows or receptionist stations • Desk-height writing surfaces with knee space for use by seated person • Accessible toilet and dressing rooms large enough for a person using a wheelchair • Audible and visual alarm systems • Qualified sign language interpreters • Large printout capability of key papers for people with low vision • Raised lettering and Braille on signs such as rooms and elevators

16. Health Care Facility Suggestions • Personnel sensitivity training • Scales • standing while holding on and/or sitting in a wheelchair • Exam tables • Motorized, adjustable-height • Mammography machines • for woman in seated position • A portable, amplified communication system or device • Accessible toilet and/or dressing room • The Center for Universal Design and The North Carolina Office on Disability and Health

17. People with Cognitive and Speech Disabilities Cognitive Disabilities • Take the time necessary to assure clear understanding. • Use simpler words and add gestures while you talk. • Use precise language and try to employ words that relate to things you both can see. • You may need to write down information or draw a simple picture. • Consider use of yes/no questions for those with more limited expressive language. • Be prepared to give the person the same information more than once in different ways. Speech disabilities • Give whole, unhurried attention when you're talking to a person who has difficulty speaking. • Keep your manner encouraging rather than correcting • Be patient rather than speak for the person. • Never pretend to understand. • Repeat what you understand. The person's reaction will assist you and guide you to understanding.

18. People with Mobility Disabilities • Adaptive aids are part of that person's personal space. • Ask before you move a person in a wheelchair, • Do not move wheelchair/device beyond patient’s easy reach. • Lock wheelchairs before transfers. • Talk to patient at eye level when possible. • Don't pat patient on the head.

19. Unique components of the history • How does the patient communicate? • Is receptive language superior to expressive language skills? • How does the patient manifest pain? • Who observes functional skills, i.e. toileting habits? • Does the patient manage own health habits, i.e. eating, sleeping, etc.?

20. Disdat tool • Publisher: St. Oswold's HospiceDate: April 2008 • Distress assessment tool to help health professionals identify distress cues in people with cognitive impairment or physical illness which severely limits communication. • www.mencap.org.uk

21. Procedures and Testing • Evaluate potential difficulties with preparation for test • Evaluate ability to cooperate • Easy vs. potentially painful or immobilizing tasks • Consider options/alternatives • Consider potential difficulties with post-test needs

22. Encouraging self-care • Assess developmental/functional level • Encourage caregivers to promote learning and self-care • Learning should be attempted in small steps

24. General Health • Is Etiology of DD known? • What is level of adaptive function? • What is done to maintain healthy weight? • Encourage physical activity

25. Assessing Activities of Daily Living • Bathing • Dressing • Toileting • Transferring • Continence • Feeding • Katz ADL • www.annalsoflongtermcare.com/article/6412 • Index of Independence in ADL • www.chcr.brown.edu/pcoc/adl.pdf

26. Good principles of care • Minimize medications • Assess risk for non-adherence, neglect, abuse • Consider Advance Care Planning

27. Medication Principles to Remember • Monitor the condition for which drug is given (eg. seizures, constipation, depression) • Alert to effects when new drug added, drug stopped or tapered, or dosage altered. • Consider best method of administration • Pill, liquid, IM, PR, etc. • Set up a system to monitor behavior and side effects.

28. Illnesses of Adult Life • Cardiovascular screening • Hypertension, Cholesterol • Diabetes risks • Thyroid screening • Bone health • Oromotor/swallowing problems • GE reflux • Constipation • STD’s

29. Protecting the Heart • Seek Family History • Blood pressure and cholesterol checks • Encourage regular, moderate exercise • Avoid cigarette smoking • Healthy, low sodium diet • Decrease fat in diet • Watch for signs of decreased endurance - distress, dizziness, confusion • Teach signs and symptoms of a heart attack • Slow the pace of activities • Change position slowly to prevent dizziness

30. Protecting the Lungs • Avoid smoking and second-hand smoke. • Encourage deep breathing, physical activity. • For persons with difficulty, slow pace of activity, allow rest. • Help alleviate stress. • Proper diet and enough fluids. • Immunizations for lung diseases (flu and pneumonia). • Watch for signs of infection (increased coughing, shortness of breath, colored sputum, increased confusion).

31. Cancer prevention & screening • Mammogram • Colonoscopy • Smoking cessation • Alcohol in moderation • Pap smear • Testicular exam • Skin exam • Prostate screening

32. Increased Risks with DD • Evaluate for fall risk • Screen for bone health • Evaluate for seizure risk • Evaluate hydration • Screen for constipation • Screen for adequate sleep • Consider sleep apnea • Assess for sexual health issues

33. Aging and Developmental DisabilitiesOsteoporosis Fracture rate for individuals with developmental disabilities - 1.7-3.5 x increase Factors associated with osteoporosis in individuals with developmental disabilities: 1. Immobility 2. Gonadal/Endocrine Dysfunction 3. Medications (i.e. anticonvulsants) 4. Small Body Size (i.e. Down Syndrome)

34. Protecting theMusculoskeletal System • Encourage independent movement and self-care. • Promote regular exercise. • Implement safeguards, avoiding ill-fitting shoes, throw rugs, irregular surfaces. • Promote safe use of mobility aids. • Provide seating that is comfortable, firm, and not too deep. • Consider calcium and vitamin D intake and needs, weight-bearing opportunities.

35. Seizure Disorders • Frequency and description • Medication • Drug levels • Screening blood work • Drug interactions • Oral health • Bone health • Sedation • Falls prevention • Bathing & climbing

36. Hydration/ Constipation • Fluid intake • Urine concentration • Ease and frequency of stooling

37. Sleep • Well-rested • Sleep environment • Sources of disruption • Medication effects • Caffeine • Melatonin

38. Development Self-exploration Curiosity Gender identity Puberty Self-consciousness Exploration Intimacy Education Attitudes Information Decision-making skills Stranger-friend errors Private-public errors Shepperdson. Child: Care, Health & Devel. 21(5),1995. Sexuality

39. Gynecologic health • Access to pelvic examinations • Risks of breast and gyn cancer • 6% of all disabled women are virginal • 3% non-disabled • Increased risk sexual exploitation in women with disabilities

40. Congenital heart disease Adult valvular disease Hypothyroidism Early menopause Obesity Celiac disease Sleep apnea Osteoarthritis Hip dysplasia Gout Atlantoaxial instability Depression Autism, OCD Dementia Seizure disorder Solid tumors Testicular cancer Midface hypoplasia Sinusitis, otitis Xerosis Folliculitis Health surveillance: Down syndrome

41. Evaluation of cognitive function • Protocol for Recording Baseline Behavior Information for Persons With Down Syndrome • Walking • Coins in a jar • Open and close padlock • Conversation • Personal topics, work/social topics, personal preferences • www.uic.edu/orgs/rrtcamr/demrecordingprotocoltest.pdf • PCAD project - Preparing Community Agencies for Dementia

42. Spasticity Joint deformity Hip dislocation Worsening bowel & bladder function Osteoporosis Skin breakdown Worsening communication skills Oromotor dysfunction GE reflux Aspiration Delayed gastric emptying Constipation Health surveillance: Cerebral palsy

43. Aspiration Prevention • Changing diet: consistency, texture, temperature • Positioning: correct neck position, seating support, proper alignment • Adaptive equipment – spoons, cups, glasses • Assisted eating techniques • Non-oral eating methods – tubes • Good oral hygiene • Appropriate choice of medications

44. Screening and Prevention • Evaluate vision & hearing • Last evaluation • Signs of change in vision/hearing • Provide dental care • Cavities, loose teeth, grinding teeth, swollen/bleeding gums

45. Symptoms of a Vision Problem When a patient can’t tell you there is a vision problem? • Rubbing eyes • Squinting • Shutting or covering one eye • Tilting head or leaning forward

46. Potential Clues to Changes in Visual Function • Falling. Stumbling. • Hesitancy when stepping. • Sitting closer to TV. • Holding objects closer to eyes. • Change in desire to participate in activities.

47. Support Strategies for Vision Problems • Have regular eye exams. • Modify the environment: • Use high contrast colors, non-glare lighting and surfaces, large print. • Red, oranges, yellows better than blues, greens, violets • Provide increased lighting, use night lights. • Keep belongings in organized, consistent places. • Keep eyeglasses clean, well-fitted and try to prevent scratches.

48. People with Vision Disabilities Limited Vision • Allow the person to take your arm offered in assistance. • This will help you guide rather than propel or lead them. • Identify yourself and others who may be with you. • Speak in a normal tone of voice, indicate when you move from one place to another, and let it be known when the conversation is at an end.

49. Potential Clues to Hearing loss When a patient can’t tell you there is a hearing problem? • Turning TV up loud. • Speaking loudly. • Inappropriate response to questions. • Confusion in noisy situations. • Refusal to participate in previous activities.

50. Support Strategies for Hearing Problems • Have regular hearing screening by qualified professional. • Speak slower, and clearly. • Reduce background noise. • When speaking, face person with light on your face.