Assessment of transition readiness to predict health care utilization during transition to adult care in sickle cell disease

ABSTRACT

Background

Transition-age patients with sickle cell disease (SCD) are at risk for poor outcomes associated with incomplete transition readiness and neurocognitive deficits. Study objectives were to: 1) test if a SCD-specific measure of self-management skills was associated with transition outcomes and 2) evaluate if caregiver-reported executive functioning was associated with self-management skills and transition outcomes among youth with SCD.

Research design and methods

Youth/caregivers were selected from a longitudinal cohort study. Caregivers completed the Behavior Rating Inventory of Executive Function (BRIEF); caregivers and youth completed the Self-Management Skills Checklist (SMSC) at a median age of 16.8 ± 0.6 years. Non-parametric tests compared SMSC and transition outcomes. Regression assessed the incremental validity of SMSC in predicting transition outcomes.

Results

In total, 95 participants (54% male, 55% severe genotype) completed the SMSC assessment. Most participants (87%) transferred to adult care within six months and 87% were retained for at least 12 months. BRIEF and caregiver-reported SMSC assessments were weakly, negatively correlated (ρ = −0.25, p = 0.0392) but were not significant in predicting transition outcomes (p > 0.05).

Conclusions

The SMSC and executive function did not predict adult care engagement. Development of readiness assessments that predict care engagement and reflect self-efficacy is important for monitoring transition-aged patients with SCD.

KEYWORDS:

• Executive functioning
• self-management skills
• Health Care Transition
• sickle cell disease
• transition readiness

Abbreviations

Table

Author contributions

KE Howell, AM Heitzer, JS Hankins, and JS Porter substantially contributed to the conception and design of the study, analyzing the data, drafted and revising the manuscript. JN Longoria, B Potter, W Wang, S Anderson, and G Kang substantially contributed to revising the manuscript for intellectual content. All authors have agreed on the journal to which the article has been submitted, reviewed and agreed on all versions of the article, and agree to take responsibility and be accountable for the contents of the article.

Declaration of interests

JS Hankins receives consultancy fees from Global Blood Therapeutics, CVS Health and Forma Therapeutics and received funding from U01HL133996 during the conduct of this study. AM Heitzer provides consulting for Global Blood Therapeutics. JS Porter receives consultancy fees from Forma Therapeutics Inc and receiving funding from K01HL125495 during the conduct of this study. American Lebanese Syrian Associated Charities (ALSAC) supports research conducted by St. Jude Children’s Research Hospital.

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/17474086.2022.2144216

Additional information

Funding

St. Jude Children’s Research Hospital is funded by American Lebanese Syrian Associated Charities. JS Hankins received funding from U01HL133996, and JS Porter received funding from K01HL125495.