Psychotherapy with Deaf Clients from Diverse Groups

7 Psychotherapy for People with Usher Syndrome

136 7 Psychotherapy for People with Usher Syndrome KAREN BAILEY AND ILENE D. MINER There are four basic categories of deaf-blindness: congenital deaf-blindness, acquired deafness and blindness, congenital or early deafness with acquired blindness, and congenital blindness with acquired deafness (Adler, 1987; Ingraham, Carey, Vernon, & Berry, 1994). Usher syndrome is the leading genetic cause of deaf-blindness. More than half of all deaf-blind adults have Usher syndrome (Helen Keller National Center, 2008; Smith et al., 1994; Vernon & Hicks, 1983). Some people with Usher syndrome communicate primarily or solely using visual or tactile sign language and are involved with the Deaf community, while others rely on spoken language and do not consider themselves to be culturally Deaf. Individuals in both groups may utilize hearing aids and/or cochlear implants. To complicate matters, progressive loss of vision and hearing over time leads many people to increasingly rely on tactile sign language. In addition , advances in both print-based and Braille-based technology have provided communication independence for many people who are deaf-blind (A. Bhattacharyya, personal communication, July 10, 2007). The broad subject of deaf-blindness contains many different repercussions, however, that one chapter could not cover the entire subject. We limit this chapter to issues presented by people with Usher syndrome. All people with Usher syndrome experience some level of hearing loss and have deteriorating vision as a result of retinitis pigmentosa (RP), a degenerative eye disease marked by night blindness in the early stages, retina pigment changes, loss of peripheral vision, legal blindness, and possibly eventual total blindness. Usher syndrome comprises approximately 10% of all cases of RP (Daiger, Bowne, & Sullivan, 2007). Numerous mutations in the genes associated with Usher syndrome have been identified and linked to varied clinical manifestations (Daiger, 2004). There are three types of Usher syndrome: 1. People with Type I are born profoundly deaf, have no internal sense of balance , and begin to show RP symptoms in early childhood, although it is not Karen Bailey: I would like to thank my community, including others who are deaf-blind and allies, for the support and modeling that made me believe that I could succeed as a Deaf-Blind professional. Ilene D. Miner: I would like to thank the many deaf-blind people who have been so willing to share parts of their lives. I also wish to thank McCay Vernon, PhD, for his support and guidance during all the time I have been working with and writing about people with Usher syndrome. He has been a source of help, clarification, and understanding, and has been one person consistently concerned with mental health access and service to deaf-blind people. Psychotherapy for People with Usher Syndrome 137 usually recognized until later (Kimberling & Möller, 1995; Smith et al., 1994). As children, they have delayed motor milestones (Boys Town National Research Hospital, n.d.) and experience night blindness in infancy or early childhood. They can be legally blind by adolescence (Miner & Cioffi, 1997). About 3–6% of children born deaf will have Type I (Kimberling & Möller, 1995). 2. People with Type II are born hard of hearing, have moderate stable hearing loss, and normal balance. They develop blind spots by late childhood or teens and can be legally blind by late adolescence to early adulthood (Miner & Cioffi, 1997). Their RP is usually diagnosed in adolescence, and sometimes not until early adulthood (Kimberling & Möller, 1995; Smith et al., 1994). About 3–6% of hard of hearing children are born with Type II (Boys Town National Research Hospital, n.d.). 3. Type III involves progressive hearing loss and RP. Hearing loss onset varies widely from adolescence to mid-adulthood. Difficulties with balance may occur later in life (National Institutes of Health [NIH], 2008). A person with Type III will experience night blindness in childhood or as a teenager, and can be legally blind by early- to mid-adulthood (Miner & Cioffi, 1997; Pakarinen et al., 1995). Parents of children with Usher syndrome often recount that their children seemed to be bumping into things or tripping frequently before being diagnosed with RP. Vision loss for any person changes every sphere of life and compromises membership in the person’s community of primary identification. In a person who is deaf or hard of hearing , however, vision loss can present unique challenges to communication and can increase isolation. Working effectively with people with Usher syndrome requires understanding this condition and its progression over the life span. Due to the variability in clinical symptoms , therapists must...