Families with Deafness: Providing a Pediatric Medical Home Rachel St. John, MD, CMHC Director: Kids Clinic for the Deaf, Georgetown University Hospital Visiting Professor: Gallaudet University, Dept. of Counseling

General Background Incidence of congenital hearing loss is 0.5-3/1000 live births 0.5-3/1000 live births 90% of deaf children born to hearing parents 90% of deaf parents have hearing children

Why a Medical Home? Large cultural communities tend to congregate in urban areas, especially if residential school/university in area if residential school/university in area * Washington DC metropolitan area deaf residents estimated in “hundreds of thousands”

Why a Medical Home? Legal Mandates: -Americans with Disabilities Act (1990) -Americans with Disabilities Act (1990) -DHHS Office for Civil rights policy -DHHS Office for Civil rights policy statement regarding Low English statement regarding Low English Proficiency patients (2000) Proficiency patients (2000) Parents are primary historians for young children – good communication and cultural awareness critical

Why a Medical Home? Parents often seek advice from health professionals related to areas outside medical arena – familiarity with community resources can be very helpful

Areas of Decision-Making LANGUAGE HEARING AMPLIFICATION CULTURE & PSYCHOSOCIAL MEDICAL CONSIDERATIONS

LANGUAGE

Communication/Education Modes SIGNED (ASL, SEE) ORAL (cued speech, speech) TOTAL COMMUNICATION BI-BI (No single “best-fit” answer – each family has unique set of circumstances)

HEARING AMPLIFICATION

Behind-The-Ear Aids

BAHA (Bone-Anchored Hearing Aid) Conductive hearing loss Previous surgery or malformation prevents use of conventional aid Screw implanted in mastoid bone connects to external processor

Cochlear Implant

FM SYSTEM

HEARING AMPLIFICATION Again, no single “best-fit” option - educated choices best made by evaluating child’s form of deafness, family context, and social environment

CULTURE & PSYCHOSOCIAL

FACTORS Access to Resources Presence of Deaf community Parental expectation

Family Milieu Deaf-of-Hearing: *may have guilt, grief, sense of loss *may have guilt, grief, sense of loss *potential unrealistic expectation/denial *potential unrealistic expectation/denial *variable accessibility to resources *variable accessibility to resources -geographic isolation -geographic isolation -SES -SES -education -education

Family Milieu Deaf-of-Deaf: *may be thrilled!! *may be thrilled!! *multi-generational: strong cultural *multi-generational: strong cultural heritage heritage *exposed to intact first language from *exposed to intact first language from birth – reduces sense of urgency for birth – reduces sense of urgency for educational decision making educational decision making

MEDICAL CONSIDERATIONS

Medical Considerations Initial considerations: *PMH: TORCH (CMV), anoxia, ECMO, ototoxic *PMH: TORCH (CMV), anoxia, ECMO, ototoxic drugs, hyperbili, etc. drugs, hyperbili, etc. *PE: dysmorphism, auricular or preauricular *PE: dysmorphism, auricular or preauricular distortion distortion Genetics referral *based on FH *based on FH *connexin 26 *connexin 26 Audiology referral *report to state *report to state *sedated BAER *sedated BAER *recommendations for amplification *recommendations for amplification and follow-up and follow-up

Medical Considerations (cont) Coordinating Subspecialists *syndrome-related deafness often requires multiple *syndrome-related deafness often requires multiple subspecialist care subspecialist care Specific Medical Considerations *often require increased ENT services *often require increased ENT services *preventative care for cochlear implant patients *preventative care for cochlear implant patients

KIDS CLINIC FOR THE DEAF

KCD – The Medical Home ENVIRONMENT : -pediatrician fluent in ASL: direct rapport -pediatrician fluent in ASL: direct rapport with families, preserves confidentiality with families, preserves confidentiality -medical interpreter present for non- -medical interpreter present for non- physician encounters (billing, check-in, physician encounters (billing, check-in, nursing screening) nursing screening) -dedicated TTY line -dedicated TTY line -staff exposure to Deaf culture via workshops -staff exposure to Deaf culture via workshops -HIPAA-compliant communication system -HIPAA-compliant communication system for non-medical issues for non-medical issues

KCD – The Medical Home CONSULTATION (often deaf children of hearing parents): *language choices *language choices *E.I. Part C state coordinators *E.I. Part C state coordinators *audiology support *audiology support *school options *school options *appropriate psychoeducational testing *appropriate psychoeducational testing resources resources

KCD – The Medical Home SPECIFIC MEDICAL CARE SITUATIONS: -cochlear implants: appropriate vaccines, -cochlear implants: appropriate vaccines, coordinating with audiology/ENT, coordinating with audiology/ENT, monitoring language milestones monitoring language milestones -syndromic deafness: coordinating -syndromic deafness: coordinating subspecialty care subspecialty care -normal development awareness for -normal development awareness for bilingual/trilingual households bilingual/trilingual households

KCD – The Medical Home EDUCATION: -Community workshops -Community workshops -Involvement at local deaf-education -Involvement at local deaf-education schools (i.e. Back-To-School night) schools (i.e. Back-To-School night) -Grand Rounds lectures at area hospitals -Grand Rounds lectures at area hospitals -Involvement of medical students and -Involvement of medical students and residents fluent in ASL in clinical residents fluent in ASL in clinical and educational activities and educational activities

KCD – The Medical Home RESOURCE COLLABORATION: -Baby Watch™: continuity of care -Baby Watch™: continuity of care -Georgetown Patient and Physician -Georgetown Patient and Physician Advocacy department: interpreter Advocacy department: interpreter services for hospital services for hospital -Gallaudet Interpreting Service – medical -Gallaudet Interpreting Service – medical interpreter interpreter -National Association of the Deaf -National Association of the Deaf -Center for Families in Transition -Center for Families in Transition

REFERENCES Acclaim Clipart : East Melbourne Hearing Research Group: Faulconbridge and Bowdler, Hearing Aids: Gallaudet University: “Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss”: ACMG statement, vol 4, no 3, May/June 2002 Hearing Loss”: ACMG statement, vol 4, no 3, May/June 2002 “What Is A Cochlear Implant”: m m m