Medication use by adolescents and adults with fragile X syndrome.

BACKGROUND: The behavioural challenges and medical conditions associated with fragile X syndrome (FXS) can lead to increased need for medications. METHOD: This longitudinal study examined the use of prescription medications for psychotropic and non-psychotropic purposes by adolescents and adults with FXS drawn from a North American community sample (N = 105). Odds and probabilities of continuing or discontinuing medication were calculated. Predictors of medication use were calculated. RESULTS: More than two-thirds took psychotropic medication, and about one-quarter took non-psychotropic medication. Over a 3-year period, those who initially took prescription medications were considerably more likely to remain on medications than to stop. Individuals with more autism symptoms, more behavioural problems, a mental health diagnosis, and greater family income were significantly more likely to use psychotropic medication 3 years later. Individuals who had more health problems, a mental health diagnosis, and were female were more likely to use non-psychotropic medication over this time period. CONCLUSIONS: Findings highlight the elevated and ongoing use of medication by individuals with FXS. Implications for social and behavioural research on FXS are discussed.

Behaviour problems, maternal internalising symptoms and family relations in families of adolescents and adults with fragile X syndrome.

BACKGROUND: Studies have linked the behaviour problems of children with fragile X syndrome (FXS) to maternal well-being, but less is known about how behaviour problems relate to important family factors such as marital satisfaction and family cohesion. METHODS: Married mothers of 115 adolescents and adults with FXS completed questionnaires and interviews, and maternal CGG repeat length was obtained by medical/laboratory records or by blood analysis. RESULTS: Indirect effects were present between behaviour problems and family variables in that behaviour problems were positively related to maternal internalising symptoms which were, in turn, negatively associated with both family cohesion and marital satisfaction. Direct associations between behaviour problems and family relationship variables were not significant. CONCLUSIONS: Findings suggest the importance of intervening with behaviour problems in individuals with FXS and identify maternal mental health as a potentially powerful conduit for the effects of child behaviour on relationships within these families. Implications for targeted interventions are discussed.

Factors predicting mortality in midlife adults with and without Down syndrome living with family.

BACKGROUND: Little is known about the mortality of individuals with Down syndrome who have lived at home with their families throughout their lives. The current study evaluates the predictors, causes and patterns of mortality among co-residing individuals in midlife with Down syndrome as compared with co-residing individuals with ID owing to other causes. METHOD: This paper examines mortality in 169 individuals with and 292 individuals without Down syndrome from 1988 to 2007. Dates and causes of death were obtained from maternal report, the Social Security Death Index and the National Death Index. Risk factors predicting mortality, including demographic variables, transition variables, and initial and change measures of health, functional abilities and behaviour problems, were obtained from maternal report. RESULTS: Having Down syndrome is a risk factor of mortality, net of other risk factors including older age, poorer functional abilities, worsening behaviour problems, residential relocation and parental death. The causes of death among individuals with and without Down syndrome who are in midlife and co-residing with their families are similar, and are most commonly due to cardiovascular or respiratory problems. CONCLUSIONS: The findings indicate that midlife adults with Down syndrome who co-reside with their families generally exhibit similar causes of mortality as do midlife adults with intellectual disability owing to other causes, but show an elevated risk of mortality in midlife net of other variables, such as age and changes in functional abilities and behaviour problems.

The role of coping in maintaining the psychological well-being of mothers of adults with intellectual disability and mental illness.

BACKGROUND: Mothers who have a child with intellectual disability (ID) or mental illness face a lifetime of caregiving responsibilities and challenges. The present study investigated changes over time in how mothers cope with the challenges of caring for an adult child with disabilities and the effects of changes in coping on maternal well-being. METHODS: A sample of 246 ageing mothers of adults with ID and 74 mothers of adults with mental illness was drawn from two parallel longitudinal studies of later-life caregiving. RESULTS: There was considerable variability at the individual level in the degree to which mothers changed over time in their use of problem-focused and emotion-focused coping strategies. For both groups, an increase in their use of emotion-focused coping led to declining levels of well-being. For the parents of adults with ID, an increase in their use of problem-focused coping resulted in a reduction in distress and an improvement in the quality of the relationship with their adult child. For the parents of adults with mental illness, an increase in the use of problem-focused coping had no effect on levels of distress, but led to an improved relationship with their adult child. CONCLUSIONS: The present study underscores the importance of coping in the lives of older mothers of adults with disabilities.

Extracapsular spread. A significant predictor of treatment failure in patients with squamous cell carcinoma of the tongue.

INTRODUCTION: The presence of nodal metastases remains the most significant predictive factor for regional recurrence and survival in patients treated for squamous cell carcinoma of the head and neck. Survival rates are further decreased in patients with nodal metastases that have spread beyond the lymph node capsule, or extracapsular spread (ECS). To the authors' knowledge, this is the first report on the impact of ECS in a large series of patients treated for squamous cell carcinoma of the oral tongue (SCCOT) at a single institution using surgery as the primary treatment modality. METHODS: The authors retrospectively reviewed the medical records of all patients treated for SCCOT with resection of the primary and neck dissection at a single institution between 1980 and1995. RESULTS: Two hundred sixty-six patients were included in the study. Of that number, 146 patients (55%) were pathologically node-negative (pN0), 75 patients (28%) were pathologically node-positive (pN+) without ECS (pN+/ECS-), and 45 patients (17%) were pN+ with ECS (pN+/ECS+). The 5-year disease-specific and overall survival rates for pN0 patients were 88% and 75%; for pN+/ECS- patients, 65% and 50%; and 48% and 30% for pN+/ECS+ patients. The patterns of failure for the pN0, pN+/ECS- and, pN+/ECS+ groups showed overall recurrence rates of 19.8%, 34.2%, and 51.1% with regional failure rates of 11.5%, 19.2%, and 28.9%, respectively, and distant metastases rates of 3.3%, 8.2%, and 24.4%. CONCLUSIONS: ECS is the most significant predictor of both regional recurrence and development of distant metastasis accounting for decreased survival of patients with SCCOT in the current study. Therefore, intensive regional and systemic adjuvant therapy may be indicated for patients with ECS. Future studies should focus on identifying molecular mediators involved in ECS to determine targets for adjuvant therapies in this subset of patients.

High incidence of complications encountered in chronic otitis media surgery in a U.S. metropolitan public hospital.

OBJECTIVES: To examine the incidence and nature of complicating factors in surgery for chronic otitis media (COM) in a metropolitan public hospital in the United States. METHODS: A retrospective review was performed over 2 years in a metropolitan public hospital to identify cases of surgery for chronic ear disease with the following complicating factors: intracranial or extracranial abscess, labyrinthine fistula, dural dehiscence with or without associated encephalocele or meningocele, or extensive involvement of the facial nerve by cholesteatoma with or without paralysis. RESULTS: Thirty-three of 90 consecutive operations for COM (37%) met criteria for complicated chronic otitis media (CCOM). The majority (85%) of patients had cholesteatoma involvement. Most patients (73%) were managed by an extended modified radical mastoidectomy. An additional 31 procedures were required in these 33 patients for control of complications. There was no iatrogenic sensorineural hearing loss or facial paralysis. CONCLUSIONS: The incidence of CCOM in this large, U.S. metropolitan public hospital is similar to that observed in developing nations. CCOM significantly complicates chronic ear surgery and substantially increases the use of resources. Maintaining functional hearing is possible, but difficult, and should be considered secondary to prevention of further complications and eradication of disease. Access to health care and proper follow-up are crucial in prevention of CCOM.

The role of postoperative chest radiography in pediatric tracheotomy.

A postoperative chest radiograph has traditionally been obtained after tracheotomies to evaluate for the presence of a pneumothorax and to assess tube position. Several recent studies in adults have questioned the usefulness of routine postoperative chest radiography in uncomplicated cases, but the role of post-operative chest radiography in pediatric patients has not been previously reviewed. We performed this study to examine the clinical utility of post-tracheotomy chest radiography in pediatric patients and determine if this routine practice impacts patient management enough to merit continued usage. A retrospective review was performed of 200 consecutive pediatric patients who underwent tracheotomies by the otolaryngology service in a tertiary care pediatric hospital from January 1994 to June 1999. All patients received postoperative chest radiographs. Five of 200 patients had a new postoperative radiographic finding, with three requiring interventions. Two patients required chest tube placement for pneumothorax, and one patient required tracheostomy tube change for repositioning. Fifty-one patients, including both pneumothoraces, exhibited clinical signs of pneumothorax (decreased breath sounds or oxygen saturation) in the immediate postoperative period. Chest X-ray ruled out a pneumothorax in the remaining 49 patients. The majority of these 51 patients were less than 2 years old (94%, P=0.002) or weighed less than 17 kg (89%, P=0.004). Postoperative chest X-rays yielded clinically relevant information in 168 patients that fell into one or more of four high risk categories: age less than 2, weight less than 17 kg, emergent procedures, or concomitant central line placement. Avoiding chest X-rays in the remaining 32 patients would have resulted in potential savings of $5000, which does not reflect the actuarial cost of a missed complication. Since the majority of our patients (84%) fell into a high-risk category, we feel it would be prudent to continue obtaining postoperative chest radiographs following all pediatric tracheotomies.

Life course impacts of parenting a child with a disability.

We contrasted parents who had a child with a developmental disability, a serious mental health problem, and a normative comparison group with respect to parental attainment and well-being at mid-life. Data are from the Wisconsin Longitudinal Study, collected when the respondents were 18, 36, and 53 or 54, on average. Although similar at age 18, group patterns of attainment and well-being diverged thereafter. Parents of a child with a developmental disability had lower rates of employment, larger families, and lower rates of social participation but were similar to parents without a child with a disability in educational and marital status, physical health, and psychological well-being. Parents whose child had a serious mental health problem had normative patterns of educational and occupational attainment and marriage, but elevated levels of physical symptoms, depression, and alcohol symptoms at mid-life.

Siblings of adults with mental illness or mental retardation: current involvement and expectation of future caregiving.

OBJECTIVE: The study examined the factors associated with the involvement of siblings in the life of a brother or sister who has mental illness or mental retardation. Involvement was defined as the current provision of instrumental and emotional support as well as the expectation of future caregiving responsibility. METHODS: A mailed questionnaire was used to collect data from 61 siblings of adults with serious mental illness and 119 siblings of adults with mental retardation. The sample was drawn from two ongoing longitudinal studies. RESULTS: The two groups of siblings showed striking differences in their expectations about their responsibility for future caregiving. Almost 60 percent of the siblings of adults with mental retardation expected to assume primary caregiving responsibility in the future, but only one-third of the siblings of adults with mental illness held this expectation. For both groups, competing family responsibilities limited the involvement of siblings, whereas closeness to the family of origin led to greater sibling involvement. CONCLUSIONS: The extent of current and future involvement by siblings of adults with disabilities is a function of the demands and constraints of midlife as well as the degree of closeness with the family of origin. The findings highlight the importance of clinicians' work to support and strengthen family relationships, which loom large in determining the extent to which siblings are involved in the care of a brother or sister with disabilities.

Change in depressive symptoms among daughter caregivers: an 18-month longitudinal study.

This longitudinal study investigates, over an 18-month period, the caregiving experience of a probability sample of 115 daughters who provided care to an aging parent. The levels of depressive symptoms manifested by these daughters were relatively low, with only 23.5% scoring in the clinical range during the study. Nevertheless, there was substantive change in depressive symptoms among the daughters during the 18 months. Daughters with higher levels of mastery were more likely to use problem-focused coping strategies, which led to reductions in depression, whereas daughters with lower levels of mastery were more likely to use emotion-focused coping, which led to increased levels of depression. Mastery was higher when the caregiving role was shared with a sibling: it was lower if the daughter had other caregiving responsibilities and if the parent care recipient had elevated levels of behavior problems.

Social support and depressive symptoms: differential patterns in wife and daughter caregivers.

This cross-sectional study examined how three types of social support-social participation, emotional support, and caregiving support-were related to depressive symptoms in wives caring for their elderly husband and daughters caring for their elderly parent. We investigated whether different dimensions of social support affect mental health via different mechanisms and whether the context in which the support is needed and received will temper its effects. We found that social participation had a main effect on depressive symptoms for daughters but not for wives. Emotional support buffered the stress emanating from the husband's behavior problems for wives. For daughters, emotional support buffered the stress emanating from both the behavior problems and the ADL/IADL limitations of the parent care recipient. Using caregiving as the example, our data suggested that social support does not have uniform effects; rather, the type of stressor, the type of social support, and the individual context interact to result in the specific effect of support.

Measuring quality of life: a new and practical survey instrument.

Despite increasing recognition of the importance of measuring the quality of life (QOL) of people with severe mental illness, such assessments are seldom carried out because of the lack of an efficient, easy-to-use, and valid measurement instrument. To facilitate the gathering of QOL information from clients in evaluation, program improvement, or other efforts, the authors present a new short (24-item) self-administered questionnaire called the Quality of Life Questionnaire (QLQ). The questionnaire assesses subjective QOL in seven areas. Evidence for the reliability and validity of the QLQ is based on data gathered from 971 clients with serious mental illness who were receiving publicly funded mental health services at the time of the study. The results of a confirmatory factor analysis using a random split-half procedure indicated that a seven-factor solution fit the data well. Scores on the QLQ also correlated significantly with the client's functioning and satisfaction with services, providing support for the validity of the QLQ. The advantages of the QLQ over existing measures include low-cost administration and some superior psychometric properties.

Factors associated with subjective burden in siblings of adults with severe mental illness.

Experiences of subjective burden were analyzed in a sample of 164 siblings of persons with serious mental illness. Findings indicated that the well sibling's experience of burden was consistently related to the symptomatology of the ill sibling. In addition, those who viewed the ill sibling's behavior as outside his or control exhibited lower levels of subjective burden than did those who viewed the behavior as within the sibling's control. Implications for research and clinical intervention are discussed.

The other side of caring: adult children with mental illness as supports to their mothers in later life.

This article discusses the role of adult children with serious mental illness as a source of support to their aging parents. The data come from a cross-sectional study of 105 mothers age 55 and older who were living with and caring for an adult child with a serious mental illness. The mothers participated in in-depth face-to-face interviews conducted in their homes and also completed a set of self-administered questionnaires. The majority of mothers in this study reported that their children provided at least some ongoing help with a range of daily living tasks. As hypothesized, the adult child's assistance and support were related significantly to lower levels of maternal subjective burden. By beginning to acknowledge the contributions that people with mental illness make to their families, social workers can help clients maintain and sustain self-esteem and improve the quality of their lives.

A comparison of coping strategies of aging mothers of adults with mental illness or mental retardation.

Differences in coping by 105 aging mothers of adults with mental illness and 389 similar mothers of adults with mental retardation were investigated. Although no differences in problem-focused coping were found, mothers of adults with mental illness used more emotion-focused coping, which predicted greater maternal depression. For mothers of adults with retardation, depressive symptoms were a function of their child's behavior problems, although this source of stress was buffered by coping. For mothers of adults with mental illness, depression was a function of caregiving demands, but coping did not buffer the effects of stress. Explanations for findings include maternal perceptions of the context of care, of her control over the disability, and her caregiving efficacy.

Contributions of persons with serious mental illness to their families.

OBJECTIVE: This study investigated the contributions that persons with serious mental illness make to their families according to both clients and family respondents. METHODS: The sample consisted of 725 clients with serious mental illness and 725 family respondents living in rural counties or counties with small urban areas in Wisconsin. Clients were in contact with family respondents at least three times a year; 23.7 percent of them lived with the respondent. Clients returned a questionnaire, and family respondents completed a telephone interview; both rated the amount of help the client gave in eight areas such as household chores, shopping, and companionship. RESULTS: Overall, the clients, especially those who lived with their families, provided substantial help. For the total sample, clients were most likely to contribute by providing companionship; family respondents reported that 59 percent of clients gave such help. For clients who lived with respondents, between 50 and 80 percent helped by doing household chores, shopping, listening to problems, providing companionship, and providing news about family and friends, according to family respondents. CONCLUSIONS: Many persons with severe mental illness play positive roles in their families. Recognition of clients' contributions by providers, researchers, and clients and families themselves could help reduce stigma and expand community opportunities for persons with severe mental illness.

Aging parents of adults with disabilities: the gratifications and frustrations of later-life caregiving.

Using a stress process model, we investigated the impact of later-life caregiving on 105 mothers of adult children with mental illness and 208 mothers of adult children with mental retardation. As hypothesized, mothers of persons with mental illness reported higher levels of frustrations and lower levels of gratifications. Whereas the adult child's behavior problems were the strongest predictor of maternal gratifications, the adult child's diagnosis was the strongest predictor of maternal frustrations once all other factors were controlled. In addition, the size of the mother's social network, the family social climate, and the child's participation in an out-of-home program were associated with the effect of caregiver stress.