When parents face the death of their child: a nationwide cross-sectional survey of parental perspectives on their child's end-of life care.

BACKGROUND: Parents facing the death of their child have a strong need for compassionate professional support. Care services should be based on empirical evidence, be sensitive to the needs of the families concerned, take into account the heterogeneity within the medical field of paediatrics, and fit into the local health care system. We need to better understand the perspectives of parents facing the death of their child in order to guide further development and evaluation of specialised paediatric palliative and end-of-life (EOL) care services. METHODS: Questionnaire survey to assess the EOL care perspectives of a Swiss population-based sample of bereaved parents who had lost a child due to a cardiac, neurological or oncological condition, or during the neonatal period in the years 2011 or 2012. The parental perspective was assessed with a newly developed and tested instrument that was structured according to six evidence-based quality domains. Responses regarding parental experiences and perceived satisfaction are described. Differences between the four diagnostic groups are analysed using a generalized estimation equation to account for the dyadic data structure. RESULTS: Of 307 eligible families, 267 could be contacted and 135 (51%) consented to participate in this questionnaire survey. Our findings show positive parental experiences of their child's EOL care and high perceived satisfaction with the care their child received. Parents of a child with cancer rated their experiences highest in most of the six quality domains and reported the highest satisfaction with care. The lowest scores were mainly reported by parents from the neurology group, with the exception of the shared decision making domain, where parents of neonates reported significantly less positive experiences. CONCLUSIONS: Although positive in general, our study results suggest some areas for improvement. The integration of specialised paediatric palliative care has the potential to minimise lost opportunities to support and assist parents

Long-term pulmonary disease among Swiss childhood cancer survivors.

BACKGROUND Pulmonary diseases are potentially severe late complications of childhood cancer treatment that increase mortality risk among survivors. This nationwide study assesses the prevalence and incidence of pulmonary diseases in long-term childhood cancer survivors (CCS) and their siblings, and quantifies treatment-related risks. METHODS As part of the Swiss Childhood Cancer Survivor Study, we studied CCS who were diagnosed between 1976 and 2005 and alive at least 5 years after diagnosis. We compared prevalence of self-reported pulmonary diseases (pneumonia, chest wall abnormalities, lung fibrosis, emphysema) between CCS and their siblings, calculated cumulative incidence of pulmonary diseases using the Kaplan-Meier method, and determined risk factors using multivariable logistic regression. RESULTS CCS reported more pneumonias (10% vs. 7%, P = 0.020) and chest wall abnormalities (2% vs. 0.4%, P = 0.003) than siblings. Treatment with busulfan was associated with prevalence of pneumonia (odds ratio [OR] 4.0, 95% confidence interval [CI] 1.1-14.9), and thoracic surgery was associated with chest wall abnormalities and lung fibrosis (OR 4.1, 95% CI 1.6-10.7 and OR 6.3, 95% CI 1.7-26.6). Cumulative incidence of any pulmonary disease after 35 years of follow-up was 21%. For pneumonia, the highest cumulative incidence was seen in CCS treated with both pulmotoxic chemotherapy and radiotherapy to the thorax (23%). CONCLUSION This nationwide study in CCS found an increased risk for pulmonary diseases, especially pneumonia, while still young, which indicates that CCS need long-term pulmonary follow-up

Pharmacogenetic aspect of busulfan based conditioning regimens prior to allogenic hematopoietic stem cell transplantation in children

Allogenic hematopoietic stem cell transplantation (HSCT) is a well-established but complex treatment option for malignant and non-malignant disorders in pediatrics. Most commonly used myeloablative and non-myeloablative conditioning regimens in children comprise alkylating agents, such as busulfan (BU) and cyclophosphamide (CY). There exists inter-individual variability in the pharmacokinetics of these alkylating agents resulting in either poor conditioning of the patient and therefore relapse or rejection due to under exposures or occurrence of toxicities due to over exposures. With the introduction of the intravenous formulation of BU, this variability has been reduced but still could not be fully predicted. Interestingly, inter and intra-individual variability of BU kinetics is more common in children compared to adults and toxicity of BU based regimens is still a concern. It has been hypothesized that some of this variability in treatment outcomes, especially the toxicity might be predicted by genetic variants of enzymes involved in the metabolism of BU and co-administered agent CY. This review intends to summarize the studies performed to date on the pharmacokinetics and mainly on the pharmacogenetics of BU myeloablative therapy, specifically in relation to children

Le cancer de l’enfant et adolescent

On a coutume de dire que les cancers des enfants sont des maladies du développement, alors que ceux des adultes sont des pathologies du vieillissement, les mutations de l’ADN s’accumulant au cours des ans. Les cancers pédiatriques sont en effet souvent très différents de ceux qui affectent les individus d’âge mûr. Ils sont beaucoup moins fréquents, puisqu’ils ne représentent que 1 % de tous les cancers répertoriés et, compte tenu de leur grande diversité, ils constituent un ensemble de maladies rares. Aux cours des dernières décennies, le diagnostic a été affiné et les thérapies sont de plus en plus individualisées à chaque patient. Grâce à ces avancées, la mortalité a considérablement diminué et en Suisse, globalement, plus de 80 % des enfants guérissent de leur maladie. Quels sont les principaux cancers qui affectent les enfants ? Comment les prend-on en charge ? Comment soutenir son enfant malade ? Que faire lorsqu’il rentre à la maison ? Cet ouvrage de la série « J’ai envie de comprendre… » répond aux questions que se posent les parents et leur donne quelques conseils pratiques pour les aider à faire face au cancer de leur fils ou de leur fille

Commentary: A myriad aberrations on information of ontogeny of drug metabolizing enzymes in the pediatric population: an obstacle for personalizing drug therapy in the pediatric population

Major lacunae exists in our understanding of how developmental changes in drug biotransformation influence drug's exposure and thus its efficacy and toxicity in children. It is not just about smaller weight in children, which modifies the pattern of the drug's exposure. There are developmental, functional changes in organ systems, liver to body mass ratios, changes in metabolism. Understanding these changes and conducting studies to obtain data on ontogeny of drug metabolizing enzymes is essential for implementation of personalized dosing schedules in the pediatric population

Hémato-oncologie pédiatrique : de la biologie cellulaire aux traitements ciblés et individualisés

Progresses in pediatric oncology over the last decades have been dramatic and allow current cure rates above 80%. There are mainly due to multicentre clinical trials aiming at optimizing chemotherapy protocols as well as local therapies in a stepwise approach. Most of the new anticancer drugs currently in development are based on targeted therapies, directed to specific targets present only in or on tumor cells, like growth factor receptors, mechanisms involved in proliferation, DNA repair, apoptosis, tumor invasion or angiogenesis. Concerning bone marrow transplantation also, new strategic approaches are in advanced development. They aim at reducing treatment induced toxicity and enhancing efficacy at the same time. This short paper would like to point out these new technologies, which should be known by the general practitioner