Modification of the Paediatric Gastro-oesophageal Reflux Disease Symptom and Quality of Life Questionnaire (PGSQ) for children with cerebral palsy: a preliminary study.

OBJECTIVE: Gastro-oesophageal reflux disease (GORD) is a common condition affecting children, characterised by the passage of gastric contents into the oesophagus causing pain, vomiting and regurgitation. Children with neurodisability (such as cerebral palsy; CP) are predisposed to more severe GORD due to coexisting gut dysmotility and exclusive/supplementary liquid diet; however, there are no existing tools or outcome measures to assess the severity of GORD in this patient group. For children without CP, the 'Paediatric Gastro-oesophageal Symptom and Quality of Life Questionnaire' (PGSQ) assesses symptoms and response to treatment, but the questions are not suitable for children with significant cognitive impairment. We aimed to adapt the existing PGSQ assessment tool to enable use in evaluating children with CP and GORD. PATIENTS/INTERVENTIONS: Cognitive interviews were conducted by the research team with six parents/carers of children (aged 3-15) with CP (Gross Motor Function Classification System level V) who have current or past symptoms of reflux. They were asked to interpret the questionnaire using a 'think-aloud technique,' and offer suggestions on alterations to questions. Reasons for changing questions included confusing/difficult to understand questions, differing interpretations of questions and response choices not applying to the patient group. RESULTS: The PGSQ was modified iteratively following each interview. Overall, parents/carers reported that it was acceptable to recall information over the past 7 days. In the final version, it was felt the questions were relevant, useful and related to symptoms that they observed. It was easy to comprehend with no uncomfortable questions. Suggestions for future work included a section specifically focusing on the school day answered by school staff and home life answered by carers who assist them in the home. CONCLUSIONS: We have adapted the PGSQ to improve relevance and acceptability for families/carers of children with symptoms of GORD and neurodisability. Further work is needed to validate the questionnaire for this patient group.

Psychometric Properties and Predictive Value of a Screening Questionnaire for Obstructive Sleep Apnea in Young Children With Down Syndrome.

STUDY OBJECTIVES: Obstructive sleep apnea (OSA) is common in children with Down syndrome (DS) and is associated with adverse health and cognitive outcomes. Daytime clinical assessment is poorly predictive of OSA, so regular screening with sleep studies is recommended. However, sleep studies are costly and not available to all children worldwide. We aimed to evaluate the psychometric properties and predictive value of a newly developed screening questionnaire for OSA in this population. METHODS: 202 children aged 6 months to 6th birthday with DS were recruited, of whom 188 completed cardio-respiratory sleep studies to generate an obstructive apnea hypopnea index (OAHI). Parents completed the 14-item Down syndrome OSA screening questionnaire. Responses were screened, a factor analysis undertaken, internal consistency calculated and receiver operator characteristic (ROC) curves drawn to generate an area under the curve (AUC) to assess criterion related validity. RESULTS: Of 188 children who completed cardiorespiratory sleep studies; parents completed the screening questionnaire for 186. Of this study population 15.4% had moderate to severe OSA defined by an OAHI of ≥5/h. Sixty-three (33.9%) participants were excluded due to "unsure" responses or where questions were not answered. Using the remaining 123 questionnaires a four-factor solution was found, with the 1st factor representing breathing related symptoms, explaining a high proportion of the variance. Internal consistency was acceptable with a Cronbach alpha of 0.87. ROC curves for the total score generated an AUC statistic of 0.497 and for the breathing subscale an AUC of 0.603 for moderate to severe OSA. CONCLUSION: A well designed questionnaire with good psychometric properties had limited predictive value to screen for moderate to severe OSA in young children with DS. The use of a screening questionnaire is not recommended. Screening for OSA in this population requires objective sleep study measures.

Drooling Reduction Intervention randomised trial (DRI): comparing the efficacy and acceptability of hyoscine patches and glycopyrronium liquid on drooling in children with neurodisability.

OBJECTIVE: Investigate whether hyoscine patch or glycopyrronium liquid is more effective and acceptable to treat drooling in children with neurodisability. DESIGN: Multicentre, single-blind, randomised controlled trial. SETTING: Recruitment through neurodisability teams; treatment by parents. PARTICIPANTS: Ninety children with neurodisability who had never received medication for drooling (55 boys, 35 girls; median age 4 years). EXCLUSION CRITERIA: medication contraindicated; in a trial that could affect drooling or management. INTERVENTION: Children were randomised to receive a hyoscine skin patch or glycopyrronium liquid. Dose was increased over 4 weeks to achieve optimum symptom control with minimal side-effects; steady dose then continued to 12 weeks. PRIMARY AND SECONDARY OUTCOMES: Primary outcome: Drooling Impact Scale (DIS) score at week-4. SECONDARY OUTCOMES: change in DIS scores over 12 weeks, Drooling Severity and Frequency Scale and Treatment Satisfaction Questionnaire for Medication; adverse events; children's perception about treatment. RESULTS: Both medications yielded clinically and statistically significant reductions in mean DIS at week-4 (25.0 (SD 22.2) for hyoscine and 26.6 (SD 16) for glycopyrronium). There was no significant difference in change in DIS scores between treatment groups. By week-12, 26/47 (55%) children starting treatment were receiving hyoscine compared with 31/38 (82%) on glycopyrronium. There was a 42% increased chance of being on treatment at week-12 for children randomised to glycopyrronium relative to hyoscine (1.42, 95% CI 1.04 to 1.95). CONCLUSIONS: Hyoscine and glycopyrronium are clinically effective in treating drooling in children with neurodisability. Hyoscine produced more problematic side effects leading to a greater chance of treatment cessation. TRIAL REGISTRATION NUMBERS: ISRCTN 75287237; EUDRACT: 2013-000863-94; Medicines and Healthcare Products Regulatory Agency: 17136/0264/001-0003.

The Development of a Screening Questionnaire for Obstructive Sleep Apnea in Children with Down Syndrome.

Obstructive sleep apnea is a condition which affects an estimated 50% of children with Down syndrome, particularly in their early years. It can cause serious sequelae in affected children but may not be recognized by parents or health professionals. Routine screening has been recommended in some countries, but is not standard practice. There are no validated questionnaire-based tools available to screen this population of children for this particular sleep-related disorder. Using existing validated sleep questionnaire items, we have developed a questionnaire to screen children with Down syndrome up to 6 years of age for obstructive sleep apnea, which corresponds with the recommendations made in UK national guidelines. This paper describes these first steps in demonstrating content validity for a new questionnaire, which will be subject to further in-depth psychometric analysis. Relevance, clarity, and age appropriateness were rated for 33 items using a content review questionnaire by a group of 18 health professionals with expertise in respiratory pediatrics, neurodevelopmental pediatrics, and sleep physiology. The content validity index was calculated for individual items and contributed to decisions about item inclusion. Scale level content validity index for the modified questionnaire of 14 items was at an accepted level of 0.78. Two parents of children with Down syndrome took part in cognitive interviews after completing the modified questionnaire. We describe the development of this 14 item questionnaire to screen for OSA in children with DS from infancy to 6 years.

Adolescents with physical disability: seeing the individual in context.

Adolescence is a time of profound developmental change-socially, cognitively and psychologically. Although we know that many young people with a variety of long-term conditions negotiate adolescence with no more difficulty than other young people, those with physical disabilities can face barriers to their development in a number of areas. While many of these may not affect their physical health directly, it is important for health professionals to take a holistic view and remember that, as with other areas of child development, opportunities lost at critical periods may have later consequences. We need to anticipate potential difficulties early and discuss them with young people, parents and other professionals if we are to best improve outcomes for this group of patients.

The needs of children and their families at end of life: an analysis of community nursing practice.

BACKGROUND: there are few studies in the literature describing or evaluating the workload of children's community nurses prior to and following the death of a child with a non-oncological life-limiting disorder. METHOD: a documentary analysis of nursing records of all children under the care of the Lifetime Service who died during a 5-year period was carried out. RESULTS: the number of visits and telephone calls involving children's community nurses in the end of life and bereavement periods was analysed and the level of the input to families found to vary widely. In total, the number of visits range from 0 to 50 (median 8). The total number of telephone calls made or received for each child ranged from 0 to 127 (median 20). Calls and visits were with a wide range of people relating to diverse subject areas and continued even when children were in hospital. CONCLUSION: community children's nurses liase with a wide range of professionals and organisations and the nature of their workload at the end of life is often hidden.