Community care for adults with Williams syndrome: how families cope and the availability of support networks

Abstract

A study of the adjustment difficulties and needs of 70 adults with Williams syndrome found that the majority continued to live at home and remained heavily dependent on their families for their self-care. Twenty-nine families (41.4%) had had no contact with a social worker in the preceding 2 years, and 34 out of the 48 families whose children still lived at home (70.8%) had no access to respite care. Advice regarding benefits, and appropriate living and occupational arrangements for the adults was also patchy. Despite progressive medical problems, and high rates of behavioural and emotional difficulties, only 20 adults (29%) were receiving regular health checks, while 21 (30%) had had some contact with a mental health service in the preceding 2 years. In the majority of cases, families continued to shoulder the main burden of care for their sons and daughters with Williams syndrome well into adulthood, with little support from statutory and voluntary agencies. The implications of these findings are considered with regard to the principles of community care.

Social Competence in Persons with Prader‐Willi, Williams and Down's Syndromes

“…1994; Mervis et al. 2001), and adults have particular difficulty coping with the demands of employment (Udwin et al. 1998).…”

O síndrome de Williams e o contexto família: um estudo piloto

“…Estas três famílias participaram num estudo piloto cujo objectivo era examinar a situação psicossocial de famílias portuguesas e alemãs com um membro com o síndrome de Williams. O ponto de partida relacionava-se com questões como: Com que exigências, expectativas e dificuldades têm os membros da família (incluindo o afectado pelo síndrome) de se confrontar, como lidam eles com estes problemas no quotidiano e que ajudas é que necessitam (Udwin et al, 1998)? A ter em linha de conta estavam as suas necessidades e questões, assim como os comportamentos íntimos à família, relações afectivas e reacções de cada um dos membros.…”

Anxiety in children with Williams syndrome : association with negative reactivity, self-regulation, and sensory modulation.

“…Self-regulation has been associated with the neurobiological substrates of the OFC, the dorsolateral prefrontal cortex, and the anterior cingulate cortex (Whittle et al, 2006). Difficulties with self-regulation, such as poor inhibition, poor adaptability, perseveration, and hyperactivity, have been documented as weaknesses for individuals with WS (Arnold et al, 1985;Davies et al, 1998;Dilts et al, 1990;Dykens & Rosner, 1999;Gosch & Pankau, 1994;Klein-Tasman & Mervis, 2003;Tomc et al, 1990;Udwin, 1990;Udwin et al, 1998;Udwin & Yule, 1991;Udwin, Yule, & Martin, 1987). Further, executive functioning abilities have been associated with anxiety, problem behaviors, and sensory modulation difficulties in children with WS, underscoring the significance of these regulatory skills (John & Mervis, 2010 Gioia et al, 2000) to investigate the association between aspects of executive functioning and the probability of having an anxiety disorder in children with WS.…”

“…Specifically, studies have shown that individuals with WS have difficulty with inhibitory control as indexed by measures of hyperactivity, approach, and attention seeking behaviors (Arnold et al, 1985;Dilts et al, 1990;Dykens & Rosner, 1999;Klein-Tasman & Mervis, 2003;Tomc et al, 1990;Udwin, 1990;Udwin et al, 1998;Udwin & Yule, 1991). Individuals with WS also have been shown to demonstrate difficulties with set-shifting and emotion regulation, as indexed by measures of low adaptability, high preoccupations, low persistence, high levels of stress reaction, and increased temper tantrums Klein-Tasman & Mervis, 2003;Tomc et al, 1990;Udwin, 1990;Udwin et al, 1987).…”

“…WS is associated with a characteristic set of phenotypic features which include mild to moderate intellectual disability, a distinctive cognitive profile, facial dysmorphology, common personality traits, cardiovascular problems such as supravalvar aortic stenosis (SVAS), and connective tissue disorders (Morris, 2010). In addition, anxiety symptoms (Leyfer, Woodruff-Borden, Klein-Tasman, Fricke, & Mervis, 2006;Leyfer, Woodruff-Borden, & Mervis, 2009), negative reactivity John & Mervis, 2010;Tomc, Williamson, & Pauli, 1990;Udwin, Howlin, Davies, & Mannion, 1998), problems with self-regulation (Arnold, Yule, & Martin, 1985;Davies et al, 1998;Dilts, Morris, & Leonard, 1990;Gosch & Pankau, 1994;Tomc et al, 1990;Udwin, 1990;, and sensory modulation difficulties (Dilts et al, 1990;Gallo, Klein-Tasman, Gaffrey, & Curran, 2008;John & Mervis, 2010) are common. As with most phenotypes, substantial variability exists in the expression of these characteristics (Morris, 2010).…”