Childhood CNS tumours : Health and functional outcomes in adult survivors, and follow-up needs of patients and parents

Sammanfattning: Childhood central nervous system (CNS) tumour patients represent a high-risk population regarding tumour- and treatment-related late effects. The overall purpose of the present thesis was to gain increased knowledge about the long-term consequences for survivors and their parents after a CNS tumour diagnosis in childhood. The thesis comprises four studies specifically addressing: health and functional status of adult survivors, health care needs of adult survivors, the influence of illness characteristics on parental distress, and the impact of illness on families of adult survivors. Study I covered 321 parents (182 mothers, 139 fathers) of children diagnosed with CNS tumours, bone tumours, acute lymphoblastic leukaemia (ALL), or acute myeloid leukaemia (AML), and who were treated at one of two Swedish sites. The Studies II-IV are population-based, and involve an entire nation-wide cohort of all Swedish survivors meeting the inclusion criteria. These studies were based on data from 531 ≥18 years old childhood CNS tumour survivors, for whom >5 years had passed since time of diagnosis, 556 of their parents, and 996 general population controls, stratified by sex and age. The studies used a cross-sectional design and are based on quantitative self-report data. Parental distress was assessed by the Parental Psychological Distress in Cancer, health and functional outcomes by the Health Utilities Index Mark2/3, and family impact by the Impact on Family Scale. Health care needs were assessed by a multidimensional questionnaire. When followed-up at a median time of 16 years after diagnosis, comparisons with control subjects confirm persistent disability in multiple functional domains in adult survivors of childhood CNS tumours. Specifically, survivors showed disability in domains of sensation, mobility, and cognition, whereas indices of emotion and pain were unaffected compared to control subjects. In comparison with male survivors, females showed poorer overall health and functional status. Furthermore, two fifths of survivors were found to have unmet health care needs in adult life, particularly regarding psychosocial services and illness education. Survivors with health-related disability had greater health care needs in adult life, and more unmet such needs. Findings on parental distress suggest that distress reactions are influenced by diagnosis-related factors such as great uncertainty about late effects, as parents of children treated for CNS and bone tumours showed heightened vulnerability to distress. The illness-related impact on families of adult CNS tumour survivors was subsequently addressed. At the group level, the conditions of families of adult survivors appeared to be mildly to moderately influenced by the child s past illness, although a subgroup of parents reported adverse family consequences even at this late stage of follow-up. In conclusion, outcomes show that the consequences of cancer extend into adult life by compromising survivors functional ability a finding that underscores the importance of extended long-term follow-up continuing into adulthood. Addressing identified unmet health care needs in extended follow-up increases the quality of comprehensive care for this patient population. Furthermore, parents of CNS tumour patients indicate exceptional needs for supportive measures and enhanced information, an issue that needs attention in order to reduce avoidable illness-related distress and long-term adverse family consequences.