Disease activity and transition outcomes in a childhood-onset systemic lupus erythematosus cohort

Abstract

Objective The chronicity and severity of childhood onset Systemic Lupus Erythematosus (cSLE) necessitate effective transition from pediatric to adult providers. We studied transition outcomes in a cSLE cohort. Methods We identified patients at an adult Lupus clinic diagnosed with SLE ≤ 18 years who had been followed by a pediatric rheumatologist. Data extracted from the first 3 years in adult care (“post-transition period”) included: sociodemographics, depression, anxiety, SLE manifestations, SLE Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/ACR Damage Index for SLE (SLICC) scores, non-adherence, and gaps in care (no appointments in the recommended time frame). Multivariable logistic regression analyses for predictors of: (1) time between pediatric and adult providers, (2) gaps in care, (3) unscheduled utilization (Emergency Department visits and admissions) (4) depression and/or anxiety were performed, as was a multivariable Poisson regression analysis for number of missed appointments. Results In 50 patients, SLEDAI scores were stable (mean 5.7 ± 5.0 at start vs. 4.7 ± 4.8 at year 3, p=0.2), but SLICC scores increased (0.46 ± 0.84, vs. 0.78 ± 1.25, p=0.01). Depression and anxiety increased significantly (10% vs. 26%, p=0.02). Mean time from last pediatric to first adult provider visit was almost 9 months (253 ± 392 days). Nearly 75% of patients had ≥1 gap in care. White race, low education level and non-adherence were significantly associated with missed appointments. Conclusion Despite moderate disease activity in this cSLE transition cohort, prolonged time between pediatric and adult providers and gaps in care in the post-transition period occurred. Anxiety and depression were frequently reported. Future work should identify methods to improve transition.

Pediatric rheumatology: addressing the transition to adult-orientated health care

“… 21 In rheumatology-specific studies of transition outcomes in childhood onset of SLE, Son et al showed that lower educational level (less than a high school degree) was associated with increased missed appointments in the posttransition period, indicating that at-risk populations may benefit from additional guidance during the transition process. 12 Recently, a UK study investigated the clinical impact of the DON’T RETARD project (Devices for Optimization of Transfer and Transition of Adolescents with Rheumatic Disorders). The DON’T RETARD transition program included a transition coordinator and provided information about JIA and medication management as well as how to manage fatigue, friendships, and schoolwork.…”

“…For instance, youths with SLE have shown notable anxiety and depression in the post-transition period. 12 Last, many adolescents and young adults lack important self-management and health care utilization skills needed for the transfer to adult care. 13 – 15 …”

Transition and transfer of the patient with paediatric-onset lupus: a practical approach for paediatric and adult rheumatology practices

“… 52 Poor adherence in pSLE is further complicated by the neurocognitive and memory impacts of SLE, 53 as well as high rates of comorbid depression and anxiety. 20 54 Furthermore, myelination and pruning of the prefrontal lobes continues through the early 20s, such that AYA patients are still developing abstract and long-term reasoning skills, including delayed gratification and consideration of distant consequences. 55 56 In addition, children with chronic illness are often less mature than healthy peers due to the stagnation or slowing of social maturation that can occur at the time of a life-altering diagnosis 57–59 ; as such, a 21-year-old with pSLE onset at age 13 may have self-management skills more closely resembling a typical 13-year-old’s skills than a typical 21-year-old’s skills, and this can be perpetuated by parents who become overprotective when faced with their child’s critical illness.…”

“…It is important to consider the UK model and other approaches designed to improve transition in rheumatology 10 37 69–78 as well as several well-studied non-rheumatologic chronic conditions, 79–84 in light of the bleak data on post-transfer outcomes of patients with pSLE, including increased disease activity, 42 disease damage 20 and depression. 20 54 Given the vast resources spent on medical care for children and adolescents with pSLE, 15 incremental investment in modest transition services—such as the training of rheumatologists in transition best practices and the hiring of transition coordinators—is likely a cost-effective means to improve the percentage of successful transfers and thereby improve clinical outcomes for young adults with pSLE.…”

“…Son and colleagues 20 examined the transfer experience of 50 patients with pSLE aged 18–23years old as they transferred to adult rheumatology care at their institution The median transfer interval between the last appointment in paediatric rheumatology and the first appointment in adult rheumatology was 133days, while the mean transfer interval was 253days. It was not rare for the AYA to be out of care for several years after leaving paediatric rheumatology care, representing a potentially dangerous delay in care.…”