A Dutch paediatric palliative care guideline: a systematic review and evidence-based recommendations for symptom treatment.

BACKGROUND: Children with life-threatening and life-limiting conditions can experience high levels of suffering due to multiple distressing symptoms that result in poor quality of life and increase risk of long-term distress in their family members. High quality symptom treatment is needed for all these children and their families, even more so at the end-of-life. In this paper, we provide evidence-based recommendations for symptom treatment in paediatric palliative patients to optimize care. METHODS: A multidisciplinary panel of 56 experts in paediatric palliative care and nine (bereaved) parents was established to develop recommendations on symptom treatment in paediatric palliative care including anxiety and depression, delirium, dyspnoea, haematological symptoms, coughing, skin complaints, nausea and vomiting, neurological symptoms, pain, death rattle, fatigue, paediatric palliative sedation and forgoing hydration and nutrition. Recommendations were based on evidence from a systematic literature search, additional literature sources (such as guidelines), clinical expertise, and patient and family values. We used the GRADE methodology for appraisal of evidence. Parents were included in the guideline panel to ensure the representation of patient and family values. RESULTS: We included a total of 18 studies that reported on the effects of specific (non) pharmacological interventions to treat symptoms in paediatric palliative care. A few of these interventions showed significant improvement in symptom relief. This evidence could only (partly) answer eight out of 27 clinical questions. We included 29 guidelines and two textbooks as additional literature to deal with lack of evidence. In total, we formulated 221 recommendations on symptom treatment in paediatric palliative care based on evidence, additional literature, clinical expertise, and patient and family values. CONCLUSION: Even though available evidence on symptom-related paediatric palliative care interventions has increased, there still is a paucity of evidence in paediatric palliative care. We urge for international multidisciplinary multi-institutional collaboration to perform high-quality research and contribute to the optimization of symptom relief in palliative care for all children worldwide.

Palliative care for children: methodology for the development of a national clinical practice guideline.

BACKGROUND: Provision of paediatric palliative care for children with life-threatening or life-limiting conditions and their families is often complex. Guidelines can support professionals to deliver high quality care. Stakeholders expressed the need to update the first Dutch paediatric palliative care guideline with new scientific literature and new topics. This paper provides an overview of the methodology that is used for the revision of the Dutch paediatric palliative care guideline and a brief presentation of the identified evidence. METHODS: The revised paediatric palliative care guideline was developed with a multidisciplinary guideline panel of 72 experts in paediatric palliative care and nine (bereaved) parents of children with life-threatening or life-limiting conditions. The guideline covered multiple topics related to (refractory) symptom treatment, advance care planning and shared-decision making, organisation of care, psychosocial care, and loss and bereavement. We established six main working groups that formulated 38 clinical questions for which we identified evidence by updating two existing systematic literature searches. The GRADE (CERQual) methodology was used for appraisal of evidence. Furthermore, we searched for additional literature such as existing guidelines and textbooks to deal with lack of evidence. RESULTS: The two systematic literature searches yielded a total of 29 RCTs or systematic reviews of RCTs on paediatric palliative care interventions and 22 qualitative studies on barriers and facilitators of advance care planning and shared decision-making. We identified evidence for 14 out of 38 clinical questions. Furthermore, we were able to select additional literature (29 guidelines, two textbooks, and 10 systematic reviews) to deal with lack of evidence. CONCLUSIONS: The revised Dutch paediatric palliative care guideline addresses many topics. However, there is limited evidence to base recommendations upon. Our methodology will combine the existing evidence in scientific literature, additional literature, expert knowledge, and perspectives of patients and their families to provide recommendations.

Translation and cross-cultural adaptation of the PEDI-CAT: Dutch version.

PURPOSE: The PEDI-CAT measures daily functioning of children and youth, aged 1 to 21 years, with a variety of physical, cognitive and/or behavioral disabilities. In order to use an instrument in another culture or language, translation and cross-cultural validation are important, particularly for end-users. This study describes the process of translation and cross-cultural adaptation of the Dutch version of the PEDI-CAT. METHODS: End-users were involved in all steps. First, the PEDI-CAT items were reviewed to determine whether the items were relevant and acceptable in the Dutch culture. Then, the PEDI-CAT was translated into Dutch using specific guidelines. Finally, the wording of the Dutch items and response options were reviewed and tested with 22 parents of children and adolescents with and without disabilities. RESULTS: All 267 items and response options of the original PEDI-CAT were assessed as relevant and translated into Dutch. A selection of 175 items was tested with Think Aloud interviews which revealed that the translation of 46 items could be improved. CONCLUSION: The role of end-users in the process of translation and cross-cultural adaptation was crucial. This collaborative process resulted in a Dutch version of the PEDI-CAT that has been optimally adapted to the Dutch language and culture.

Medical and psychosocial problems in middle-aged spina bifida patients: survey among members of the Dutch patients' association.

PURPOSE: To identify physical, emotional and psychosocial issues in the older person with spina bifida (SB). METHOD: Members of the Dutch patients' association aged >25 years (n = 184) were asked to complete a questionnaire. This survey contained questions on physical and psychosocial complaints, as well as problems regarding social participation. RESULTS: A total of 61 people (33.2%) with a median age of 45 years (IQR 33.5-60.0) years responded. During the previous 5 years, 86.9% experienced new physical complaints, whereas only 13.1% remained free of new complaints. 50.8% of the persons had new bladder problems and 44.1% had bowel complaints. Older persons more often had physical complaints. New musculoskeletal problems occurred in 75.4%. Psychological problems were common (78.7%). Overall psychological problems were not associated with gender, hydrocephalus or age. Social participation was good, with 90.2% of persons taking part in some type of social activity. CONCLUSIONS: In this study, a majority of adult persons with SB reported newly arising physical and psychological problems during the previous 5 years. Given the large number and diversity of the newly emerging problems after adolescence, regular and multidisciplinary surveillance of adult persons with SB is recommended. IMPLICATIONS FOR REHABILITATION: Adults with spina bifida often disappear from follow-up, or are forced to take care of their follow-up themselves. According to a survey carried out among adult SB-patients from the general community, these persons continued to struggle with many physical and psychosocial problems. Given the high number of psychosocial problems in this sample of patients, psychosocial counseling could be beneficial during follow-up. This paper adds to the body of evidence indicating that multidisciplinary follow-up for SB-patients could be beneficial, also when patients grow beyond the age of 18 years.

Health issues in young adults with cerebral palsy: towards a life-span perspective.

OBJECTIVE: To obtain better insight into the health issues of young adults with cerebral palsy. DESIGN: Cross-sectional. SUBJECTS: Two data sources were used: 54 adults with cerebral palsy (age range 25-36 years) and 48 physicians (members of the Netherlands Society of Physical and Rehabilitation Medicine). METHODS: Adults with cerebral palsy participated in a physical examination and a semi-structured interview assessing several health issues and utilization of healthcare. Rehabilitation physicians completed a questionnaire on impairments they recognized as being related to cerebral palsy. RESULTS: In the patient sample, pain (59%) and joint deformities (19-57%) were observed most frequently. Evidence of a decrease in the utilization of healthcare services at adult age emerged. Lower gross motor function and cognitive level appeared to be determinants of motor and speech impairments and of the utilization of 3 allied healthcare services. Rehabilitation physicians reported pain (88%), joint deformities (86%) and fatigue (76%) as being cerebral palsy-related health problems in adults. CONCLUSION: Based on the high prevalence of pain and joint deformities and the decrease in the utilization of healthcare services, systematic follow-up in adults with cerebral palsy seems warranted. Cerebral palsy needs to be considered as a life-long condition, requiring a life-span perspective in order to better organize optimal care.