These resources include the best available evidence about the course of Rett syndrome and its management as well as practical information about family associations, specialist clinical centres and links to relevant websites.
Family satisfaction following spinal fusion in Rett syndrome: a cohort study
Downs, J., Torode, I., Ellaway, C., Jacoby, P., Bunting, C., Wong, K., Christodoulou, J., & Leonard, H. (2016). Family satisfaction following spinal fusion in Rett syndrome. Developmental Neurorehabilitation, 19(1), 31-37. Download snapshot, see article abstract.
Spinal fusion is a complex surgical procedure and it can be challenging for families to decide whether or not to proceed with the surgery. This study evaluated families' satisfaction following spinal fusion in girls with Rett syndrome.
What we did
Sixty-one families participating in the population-based and longitudinal Australian Rett Syndrome Database whose daughter had undergone spinal fusion provided data. We assessed parents' overall satisfaction; care the girls received during hospital admission through to discharge; and changes in the girls' health and function after the spinal fusion (e.g. level of discomfort).
What we found
Following surgery, families were satisfied overall with their daughter having had a spinal fusion. Parents observed improvements in their daughters' spinal symmetry, wellbeing and function. Several parents were pleased with their daughters' greater ease of sitting, dressing and transfer activities, and perceived their daughters as being more comfortable and happier after undergoing surgery. Most parents felt less anxious about their daughter's future health following the procedure and felt that they would recommend spinal fusion to other families facing the same situation as they had.
Many families were very satisfied with the amount of support their daughters received in relation to their surgical and intensive care unit (ICU) care. Outstanding clinical care and the development of strong relationships with clinical staff were much appreciated by families. This was particularly true when parents saw staff as being very confident, experienced and proactive in delivering care to their daughters.
When families were not provided with regular, clear and consistent information from staff, parents had less confidence in the care team. Poor information exchange and inconsistent care were the primary causes of concern reported by parents. Long-term complications (e.g. return to surgery and recurrence of scoliosis) occurred infrequently but were a source of considerable dissatisfaction for families.
What does it mean
When spinal surgery is being considered, there is an ongoing need for more information to support the decision-making process of both clinicians and families. Several strategies are suggested to improve the care received by families in relation to spinal fusion for Rett syndrome: the importance of optimal timing of surgery by highly skilled staff; building effective relationships between clinicians and families; seamless pain management between ICU and ward settings; and ongoing orthopedic follow-ups over the longer term.
For further reports and findings, see our publications list.