A multi-disciplinary, comprehensive approach to management of children with heterotaxy.

Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.

Fathers of children with cancer: involvement, coping, and adjustment.

INTRODUCTION: This study examined the role of fathers caring for children with cancer. Psychological adjustment, coping, and work patterns of mothers and fathers were described. METHOD: Twenty fathers of children with cancer were compared with 20 mothers of children with cancer and 20 control fathers of healthy children. Questionnaire data were collected regarding coping, parental adjustment, child adjustment, and family involvement. RESULTS: Fathers did not differ from mothers or control fathers in terms of psychological adjustment or coping. However, fathers of children with cancer spent more hours at work and more hours caring for children than did control fathers. Paternal adjustment was significantly related to child adjustment only when the child had cancer. Coping was related to work outside the home for fathers and adjustment for mothers. DISCUSSION: Models of family adaptation may be different for fathers and mothers. Treatment teams must attend to the unique needs of fathers.