Quality of life outcomes for children with neuromuscular scoliosis considering spinal surgery

Abstract

Cerebral palsy (CP) affects the child, their family and the wider community. Children with cerebral palsy Gross Motor Function level (GMFCS) IV/V often have severe scoliosis and while surgery is effective, the risks associated with surgery are high. Parents, carers and medical professionals struggle with choosing interventions, and there are only case series to provide information. That families will consider spinal fusion surgery, despite high complication rates, demonstrates the suffering for some children with scoliosis. Therefore, this thesis asked: 1. Does surgery for scoliosis improve quality of life (QoL) and clinical outcomes for children with CP (GMFCS level IV/V) at 6, 12 and 24 months compared to children with CP who declined spinal surgery? 2. What influences a family’s decision to proceed with surgery or not, and are they happy with their decision? A prospective observational study was conducted over two years, of families choosing (n=21) or declining (n=21) spinal fusion surgery for their child. The first study captured outcomes of spinal fusion surgery in children with CP and scoliosis in terms of QoL, pain, surgical complications, and improvement in Cobb angle at baseline, 6, 12 and 24 months after surgery. Children living with cerebral palsy at GMFCS level IV/V, including a surgical group (n=21, 10 female, 11 male), ages 14.4 (2.1) years (mean (SD)) and a non-surgical group (n=21, 12 female, 9 male), ages 13.8 (1.8) years). Quantitative outcomes collected included Caregiver Priorities and Child Health Index of Life with Disabilities (CP Child, primary outcome), the Cerebral Palsy Quality of Life Questionnaire (CPQOL), Non-Communicating Children’s Pain Checklist —v Revised (NCCPC-R), Canadian Occupational Performance Measure (COPM) and review of patient records. The CP Child overall score showed at 12 months the surgical group had higher scores, recording 95.2 (8.7) out of 100 compared to 44.8 (8.7) for the nonsurgical group, and after two years the effect was even greater 99.5 (2.8); 40.4 (2.8; p<0.001). NCCPC-R also improved after spinal surgery. At 12 months the surgical group had a pain score of 7.9 (5.6) compared to 34.1 (5.4) for the nonsurgical group and the effect was maintained at two years for the surgical group 4.1 (2.5), however the non-surgical group pain slightly worsened 34.7( 6.2) (p<0.000). Surgical correction of the scoliosis was demonstrated by improvements in Cobb angles and the improvements were maintained. There were 13 minor complications and two major complications, and the complication rate was in keeping with rates reported in the current literature. The second study served a dual purpose by allowing deeper understanding into the experience of the family living with a child with CP and scoliosis and greater insight into the process of decision-making regarding spinal surgery. The methodological approach used was interpretive phenomenology. The parents of the children were those who chose surgery (n=21, 20 female, 1 male) or decided against surgery (n=21, 19 female, 2 male). Semi-structured interviews were conducted in order to collect data. There were significant emotional, physical as well as financial costs of having a child with CP and scoliosis. The families of children who experienced frequent spinal pain and poor QoL described opting for surgical intervention and accepted the potential risks and complications of surgery to improve their child’s vi QoL. Families reported that scoliosis surgery helped to alleviate their child’s spinal pain and improve overall QoL for their child. Families were happy with their decision to have surgery. Some families did not feel the severity of pain was worth taking the risk of complications as they did not feel surgery would improve their child’s pain or QoL. Families were happy with their decision if their child’s conditions remained unchanged, but some families questioned their decision if their child’s pain was increasing and their QoL was deteriorating. Some families regretted their decision if they felt they left it too late to take up the surgery. Conclusion: QoL and pain influenced the family’s decision-making. Families of children with CP, GMFCS level IV and V with scoliosis elected to proceed with spinal surgery for their child if they had poor quality of life and severe pain, and were happy with their decision at 6, 12 and 24 months post operatively. Families of children declined surgical intervention for their child if they had good quality of life and low levels of pain, however if their child’s QoL decreased and their pain increased they became unhappy with their original decision. Emotional and physical costs to themselves and their child, social isolation as well as financial burdens caring for a child with scoliosis and cerebral palsy, were described. Clinical Recommendations: I recommend that research findings are shared with families in an accessible format to inform families of the benefits and risks of surgery in this population and assist in this difficult decision.

Date of Award	2021
Original language	English
Supervisor	Jennie SCARVELL (Supervisor), Marian Currie (Supervisor) & Carmel Mcquellin (Supervisor)