Factors related to caregiver strain in ALS: a longitudinal study.

OBJECTIVE: To examine the longitudinal associations between caregiver strain and patients' clinical and psychosocial characteristics as well as caregivers' psychosocial characteristics. METHODS: At 4-month intervals during the 12 months study period, longitudinal data on caregiver strain and patient and caregiver factors potentially associated with caregiver strain were collected from 126 couples, who participated in a randomised controlled trial on the effectiveness of case management in amyotrophic lateral sclerosis (ALS). Caregiver strain was assessed with the Caregiver Strain Index (CSI). Patient and caregiver factors included sociodemographic characteristics, distress, coping style and perceived quality of care, as well as the patient's functional status and emotional functioning. Multilevel regression analyses were performed. RESULTS: Caregiver strain increased significantly during the study period (ß=0.315 points/months, p<0.001) and was significantly associated with patient time-dependent factors functional status (ß=-0.131 points/months, p<0.001) and emotional functioning (ß=0.022 points/months, p=0.03), and caregiver time-dependent factors passive coping style (ß=0.152 points/months, p=0.03), symptoms of anxiety (ß=0.186 points/months, p<0.001) and perceived quality of care for the caregiver (ß=-0.452 points/months, p<0.001). CONCLUSIONS: Our study has identified that apart from the patient's physical disability and emotional well-being, a passive coping style of the caregiver, increased symptoms of anxiety and feeling less supported by the ALS-team impact on caregiver strain. The multidisciplinary teams involved with the care of patients with ALS need to be aware of these factors and increase their attention for the caregiver. This will help guide the development of evidence-based supportive interventions that focus on caregiver's coping style and avoiding distress. TRIAL REGISTRATION NUMBER: Netherlands Trial Register, number NTR1270.

LucKi Birth Cohort Study: rationale and design.

BACKGROUND: Infancy and childhood are characterized by rapid growth and development, which largely determine health status and well-being across the lifespan. Identification of modifiable risk factors and prognostic factors in critical periods of life will contribute to the development of effective prevention and intervention strategies. The LucKi Birth Cohort Study was designed and started in 2006 to follow children from birth into adulthood on a wide range of determinants, disorders, and diseases. During preschool and school years, the primary focus is on the etiology and prognosis of atopic diseases (eczema, asthma, and hay fever) and overweight/obesity. METHODS/DESIGN: LucKi is an ongoing, dynamic, prospective birth cohort study, embedded in the Child and Youth Health Care (CYHC) practice of the 'Westelijke Mijnstreek' (a region in the southeast of the Netherlands). Recruitment (1-2 weeks after birth) and follow-up (until 19 years) coincide with routine CYHC contact moments, during which the child's physical and psychosocial development is closely monitored, and anthropometrics are measured repeatedly in a standardised way. Information gathered through CYHC is complemented with repeated parental questionnaires, and information from existing registries of pharmacy, hospital and/or general practice. Since the start already more than 5,000 children were included in LucKi shortly after birth, reaching an average participation rate of ~65 %. DISCUSSION: The LucKi Birth Cohort Study provides a framework in which children are followed from birth into adulthood. Embedding LucKi in CYHC simplifies implementation, leads to low maintenance costs and high participation rates, and facilitates direct implementation of study results into CYHC practice. Furthermore, LucKi provides opportunities to initiate new (experimental) studies and/or to establish biobanking in (part of) the cohort, and contributes relevant information on determinants and health outcomes to policy and decision makers. Cohort details can be found on www.birthcohorts.net .

Need and value of case management in multidisciplinary ALS care: A qualitative study on the perspectives of patients, spousal caregivers and professionals.

Our objective was to explore the needs and value of case management according to patients with amyotrophic lateral sclerosis (ALS), their spousal caregivers, and health care professionals in the context of multidisciplinary ALS care. We undertook semi-structured interviews with 10 patients with ALS, their caregivers (n = 10) and their ALS health care professionals (n = 10), and held a focus group (n = 20). We transcribed the audio-taped interviews and analysed all data thematically. Participants indicated that in certain circumstances case management can have an added value. They identified factors for receptiveness to case management: adequacy of usual care, rate of disease progression, and degree of social network support and personal factors of patients and spousal caregivers. Participants valued the time for consultation, house calls and proactive approach of the case manager. Patients with ALS and caregivers appreciated emotional support, whereas professionals did not mention the importance of emotional support by the case manager. In conclusion, ALS teams can consider implementation of valued aspects of case management (accessibility, ample time, proactive approach, emotional support) in the usual multidisciplinary ALS care. Additional support might be provided to patients with rapidly progressive disease course, passive coping style and small social network.

Prognostic factors for the course of functional status of patients with ALS: a systematic review.

The progressive course of amyotrophic lateral sclerosis (ALS) results in an ever-changing spectrum of the care needs of patients with ALS. Knowledge of prognostic factors for the functional course of ALS may enhance clinical prediction and improve the timing of appropriate interventions. Our objective was to systematically review the evidence regarding prognostic factors for the rate of functional decline of patients with ALS, assessed with versions of the ALS Functional Rating Scale (ALSFRS). Two reviewers independently assessed the methodological quality of the thirteen included studies using the Quality in Prognosis Studies (QUIPS) tool. The overall quality of evidence for each prognostic factor was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, considering risk of bias, imprecision, inconsistency, indirectness, and publication bias. The quality of evidence for the prognostic value of age at onset, site of onset, time from symptom onset to diagnosis, and ALSFRS-Revised baseline score was low, mainly due to the limited data and inconsistency of results in the small number of studies included. The prognostic value of initial rate of disease progression, age at diagnosis, forced vital capacity, frontotemporal dementia, body mass index, and comorbidity remains unclear. We conclude that the current evidence on prognostic factors for functional decline in ALS is insufficient to allow the development of a prediction tool that can support clinical decisions. Given the limited data, future prognostic studies may need to focus on factors that have a predictive value for a decline in ALSFRS(-R) domain scores, preferably based on internationally collected and shared data.

Trends in wheeze in Dutch school children and the role of medication use.

BACKGROUND: While the prevalence of childhood wheeze continues to increase in many countries, decreasing trends have also been reported. This may be explained by increased use of asthma medication, which effectively suppresses wheeze symptoms. In this study we investigated trends in wheeze in Dutch school children between 1989 and 2005, and their association with medication use. METHODS: In five repeated cross-sectional surveys between 1989 and 2005, parents of all 5- to 6-year-old and 8- to 11-year-old children eligible for a routine physical examination were asked to complete a questionnaire on their child's respiratory health. We identified all children for whom a questionnaire was completed in two successive surveys. Children were grouped according to birth year and classified into one out of four wheeze categories: "no wheeze," "discontinued wheeze," "continued wheeze," or "new-onset wheeze." RESULTS: In total, 3,339 children, born in 1983 (N = 670), 1988 (N = 607), 1992 (N = 980), and 1995 (N = 1,082), participated twice. Over the study period, the proportion of children with "no wheeze" increased from 73.8% to 86.1% (Ptrend < 0.001), while the proportion of children with "discontinued" and "continued" wheeze decreased from 13.2% to 6.3% (Ptrend < 0.001) and from 8.8% to 3.1% (Ptrend < 0.001), respectively. Medication use was consistently associated only with the presence of wheeze symptoms and this association did not change over time (Pbirth year × medication use > 0.05 for all wheeze categories). CONCLUSION: An increasing trend of Dutch school children with "no wheeze," and decreasing trends of children with "discontinued" and "continued" wheeze between 1989 and 2005 could not be explained by (increased) medication use. This suggests that wheeze prevalence is not masked by medication use, but is truly declining.

The provision of assistive devices and home adaptations to patients with ALS in the Netherlands: patients' perspectives.

The timely provision of assistive devices and home adaptations (ADHA) is crucial in the management of patients with amyotrophic lateral sclerosis (ALS) in order to maintain their independence and relieve their caregivers. Our objective was to study the experiences of patients with ALS during the process of procuring ADHA. We sent a cross-sectional questionnaire survey addressing issues concerning the application for and provision process of ADHA to 239 patients with ALS registered at one of the three tertiary academic diagnostic centres within the Netherlands ALS Centre. One hundred and fifty-nine (89%) of the 179 responding patients (response rate 75%) had experience with the procurement process and 93 (58%) of them indicated problems in obtaining ADHA. The most reported problems were delay (42%) and the authorities' lack of disease knowledge (24%). Patients viewed these issues as the most prominent requiring improvement. In conclusion, the main problems perceived by patients indicate that increasing awareness of ALS and promoting a proactive attitude among ALS care professionals towards the application for ADHA may contribute positively to the quality of ALS care.

Cluster RCT of case management on patients' quality of life and caregiver strain in ALS.

OBJECTIVES: To study the effect of case management on quality of life, caregiver strain, and perceived quality of care (QOC) in patients with amyotrophic lateral sclerosis (ALS) and their caregivers. METHODS: We conducted a multicenter cluster randomized controlled trial with the multidisciplinary ALS care team as the unit of randomization. During 12 months, patients with ALS and their caregivers received case management plus usual care or usual care alone. Outcome measures were the 40-item ALS Assessment Questionnaire (ALSAQ-40), Emotional Functioning domain (EF); the Caregiver Strain Index (CSI); and the QOC score. These measures were assessed at baseline and at 4, 8, and 12 months. RESULTS: Case management resulted in no changes in ALSAQ-40 EF, CSI, or QOC from baseline to 12 months. ALSAQ-40 EF scores in both groups were similar at baseline and did not change over time (p = 0.331). CSI scores in both groups increased significantly (p < 0.0001). Patients with ALS from both groups rated their perceived QOC at baseline with a median score of 8, which did not change significantly during follow-up. CONCLUSION: Within the context of multidisciplinary ALS care teams, case management appears to confer no benefit for patients with ALS or their caregivers. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that case management in addition to multidisciplinary ALS care does not significantly improve health-related quality of life of patients with ALS.

Estimating the effectiveness of screening for scoliosis: a case-control study.

OBJECTIVE: The aim of this study was to test the hypothesis that screening for scoliosis is effective in reducing the need for surgical treatment. METHODS: The study was a case-control study. A total of 125 consecutive patients who were treated surgically for idiopathic scoliosis between January 2001 and October 2004 and who were born on or after January 1, 1984, were invited; 108 agreed to participate. A total of 216 control subjects were selected randomly and anonymously, matched with respect to age and gender. For 279 adolescents, exact screening exposure and outcomes could be analyzed. Case subjects were recruited from 4 university and 6 nonuniversity Dutch hospitals; control subjects were recruited from all 37 municipal health services in The Netherlands. RESULTS: Screen-detected patients received diagnoses at a significantly younger age than did otherwise-detected patients (10.8 +/- 2.6 vs 13.4 +/- 1.7 years). In total, 32.8% of the surgically treated patients had been screened between 11 and 14 years of age, compared with 43.4% of the control subjects. The odds ratio for being exposed to screening was 0.64. In total, 28% of the patients were diagnosed as having scoliosis before 11 years of age. CONCLUSIONS: Our results showed no evidence that screening for scoliosis reduced the need for surgery. Abolishing screening seems justified, especially because the effectiveness of early treatment with bracing is still strongly debated. A randomized, controlled trial on the effectiveness of treating patients with idiopathic scoliosis with bracing is urgently needed.

Childhood infections and risk of wheezing and allergic sensitisation at age 7-8 years.

BACKGROUND: Childhood atopic disease has been associated with exposure to infections early in life. There is however considerable controversy on this issue, since evidence exists for both protective and adverse effects of infections. Aim of this study was to investigate the association between childhood infections and the occurrence of wheeze and allergic sensitisation in 7-8-year-old Dutch and German children. METHODS: A nested case-control study was conducted among German and Dutch children participating in a large longitudinal study on respiratory health. Parents of 510 7-8-year-old children with respiratory complaints and an equal number of children without respiratory complaints were asked to complete a questionnaire. Blood samples were collected for specific serum Immunoglobulin E (IgE) analysis. Data on childhood infections were collected through records of the participating Municipal Health Services. RESULTS: No association was found between recent wheeze and any of the childhood infections investigated. For scarlet fever infection an increased risk of allergic sensitisation (OR: 2.82; 95% CI: 1.40-5.72) and sensitisation against grasses (OR: 3.00; 95% CI: 1.35-6.69) at the age of 7-8 years was found. Varicella infection tended towards increased risk of sensitisation against grasses (OR: 1.88; 95% CI: 0.99-3.58). For allergic sensitisation and sensitisation against grasses the odds ratios increased with increasing number of infections. CONCLUSIONS: The results do not lend support to the suggestion that childhood infection protects against wheezing or allergic sensitisation at age 7-8 years. Scarlet fever or varicella infection presented an increased risk of allergic sensitisation.