How Does Cognition Impact Healthcare Transition Skills, Engagement and Utilization in Young Adults Living with Sickle Cell Disease?
Authors: Georges Phalica , Marsolais Sophie , Charles Chrystelle , Hafsaoui Amer Yassine , Bergeron Jade , Fritz Barbara , Van Synghel Nancy , Fraile Valerie , Fournier Nathalie , Leblanc Melissa , Arena Lauren , Sultan Serge , Robitaille Nancy , Desjardins Leandra , Gaucher Nathalie , Pastore Yves D , Forte Stephanie 
Published: December 2024

Background
The adolescent and young adult period is a time of increased risk of acute complications and mortality in patients with sickle cell disease (SCD). Transfer of care from pediatric to adult healthcare setting presents several challenges. Attention difficulties, lower IQ and other cognitive deficits may negatively impact transfer success and adherence to adult health maintenance appointments. Cognitive screening is available since 2021 at the CHUM, an adult SCD centre. The main aims of this study are to 1) describe the results obtained by young adults (YA) with SCD in different cognitive screening tests, and 2) to explore if these results are associated with different measures of transition skills, healthcare engagement and utilization.
Methods
A retrospective study was conducted at the CHUM (Montréal, Canada), a tertiary care centre treating adults with SCD. Patients with SCD between the ages of 18-25 who had undergone cognitive screening between August 2021 and December 2023, and who had a follow-up period of at least 12 months were included. Cognitive screening was conducted using the Montreal Cognitive Assessment Test (MoCA), the Rowland Universal Dementia Assessment Scale (RUDAS) and a standard neurological examination. Electronic medical records were used to extract baseline characteristics including demographics, pre-existing intellectual disability (ID) or attention disorder (AD), as well as variables pertaining to transition skills (Transition Readiness Assessment Questionnaire [TRAQ] score), health care engagement (appointment attendance, interactions with clinic nurses), and acute care utilization (ED visits, hospitalizations). Patients were identified as having a cognitive comorbidity (CC) if they had a documented ID or AD, a RUDAS score of &lt;28 or a MoCA score of &lt;27. Mann-Whitney U test was used for comparison of transition success measures between those with and without CC and Spearman's ρ to find correlations between those same measures.
Results
Thirty patients fulfilling the inclusion criteria were identified. The median age at the time of cognitive assessment was 20 years [range: 18-23]. Nineteen (63%) patients were female and 20 (67%) had the SS/Sβ0 genotype. Most patients (28, 93%) were followed at a pediatric centre with expertise in SCD prior to the transfer. Three (10%) patients had a previous diagnosis of stroke, 8 (27%) were diagnosed with AD and 1 (3%) patient had a diagnosis of ID.
As part of the cognitive screening, 28 (93%) patients completed the MoCA and 28 (93%) the RUDAS. The median RUDAS score was 30 (23-30) and the median MoCA score was 27 (22-30). RUDAS and MoCA scores were suggestive of a mild cognitive disorder in 7 (23%) and 10 (33%) patients, respectively, and overall, 15 (50%) patients were identified as having a CC.
With regards to transition success, mean TRAQ scores were similar between patients with and without a CC (74±8 vs. 72±7, p=0.69).
No differences were noted in attendance of ophthalmology (72±32% vs 68±29, p=0.69) or imaging appointments (86±22% vs 87±22%, p=0.85) between those with and without CC, however attendance of hematology appointments was noted to be higher in patients with a CC (88±10% vs. 81±13%, p=0.04). Numbers of formal communications per year in the form of emails or phone calls between patients and the care team were similar between groups, regardless if initiated by the patient's family or the patient themselves.
No significant differences were noted in the mean number of ED visits per year (1.0±1.1 vs. 0.8±0.6, p=0.95), day hospital visits per year (0.3±0.7 vs. 0.3±0.4, p=0.37) nor hospitalizations per year (0.4±0.5 vs. 0.5±0.6, p=0.54) between those with and without CC.
Conclusion
In our study, YA with SCD and cognitive comorbidity demonstrated similarly favourable transition skills, good healthcare engagement and limited acute care utilization when compared to their peers. While this study is limited in size, we hypothesize that the potentially negative effect of cognitive vulnerabilities on health outcomes in youth with SCD could be mitigated with appropriate support throughout the transition period.