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Updated: Jul 12, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Shared decision-making between paediatric haematologists, children with sickle cell disease and their parents: an
Ricardo Wijngaarde1,2, Mijra Koning3, Karin Fijnvandraat4
1Department of Surgery, Amsterdam University Medical Centers, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. r.o.wijngaarde@amsterdamumc.nl.
Insights
Shared decision-making (SDM) in pediatric sickle cell disease (SCD) care shows potential for improvement. Objective assessments reveal lower SDM levels than subjective reports, highlighting a need for enhanced interventions and training.
Area of Science:
- Pediatric Hematology
- Patient-Centered Care
- Health Outcomes Research
Background:
- Children with sickle cell disease (SCD) are vulnerable to suboptimal care and health outcomes.
- Shared decision-making (SDM) can enhance patient participation and improve outcomes in pediatric SCD care.
- Existing literature suggests room for improvement in SDM for SCD patients and families, particularly concerning intercultural challenges and risk communication.
Purpose of the Study:
- To assess the extent to which pediatricians engage children with SCD and their parents in shared decision-making.
- To objectively measure the level of patient involvement in treatment decisions for children with SCD.
- To compare objective SDM scores with subjective perceptions of SDM from patients, parents, and pediatricians.
Main Methods:
- Observational, cross-sectional study involving pediatric SCD patients and parents at a university hospital outpatient clinic.
- Audio-recordings of consultations were independently analyzed by two evaluators using the OPTION-5 instrument to objectively score SDM.
- Patient, parent, and pediatrician perceptions of SDM were assessed using the SDM-Q-9 and SDM-Q-Doc questionnaires.
Main Results:
- Median OPTION-5 scores (objective SDM) were 25.0% (range 0-55%), indicating significant room for improvement.
- Median perceived SDM scores were 56.7% for patients/parents (SDM-Q-9) and 68.9% for pediatricians (SDM-Q-Doc).
- Twenty-four consultations involving treatment decisions for pediatric SCD patients (mean age 9.4 years) were analyzed.
Conclusions:
- While subjective perceptions of SDM were fair, objective measurements indicate a need for enhancement in pediatric SCD care.
- Interventions should focus on increasing awareness of SDM benefits, implementing child-centered SDM strategies, and providing SDM training for pediatricians.
- Addressing intercultural complexities and improving risk communication are crucial for optimizing SDM in pediatric SCD management.
Abstract:
Children with sickle cell disease (SCD) face various healthcare choices to be made during the disease process that may impact their lives. Shared decision-making (SDM) could improve their health outcomes. We assessed if, and to what extent, paediatricians engage children with SCD and/or their parents in the decision-making process. In this observational cross-sectional study, paediatric SCD patients and their parents visiting the outpatient paediatrics clinic of a university hospital participated in a SDM baseline measurement. Two evaluators independently and objectively analysed the level of patient involvement in decision-making from the audio-recordings of the consultations using the OPTION-5 instrument, a 0-20-point scale from which scores are usually expressed as a percentage of ideal SDM. The level of SDM, as perceived by patients, parents and paediatricians, was appreciated using the SDM-Q-9 and SDM-Q-Doc questionnaires, respectively. Scores could range from 0% (no SDM) to 100% (exemplary SDM). Twenty-four consultations in which a decision needed to be made about SCD treatment were audiotaped and analysed; six were from each paediatrician. The group consisted of 17 male and 7 female patients from various cultural backgrounds between 2 and 17 years old, with a mean age of 9.4 years (SD 4.2). Median OPTION-5 scores were 25.0% [IQR] 20.0-40.0%; range 0-55%). Median SDM-Q-9 and SDM-Q-Doc scores were 56.7% (IQR 39.4-88.9%) and 68.9% (IQR 57.8-77.8%), respectively.
Conclusion:
Although subjective scores of SDM were fair, the objectively scored level of SDM among children suffering from SCD leaves room for improvement. This may be realized by increasing knowledge about the benefits of SDM, child-centred SDM interventions and SDM-training for paediatricians that takes into account the complexity of intercultural challenges and risk communication between stakeholders.
What Is Known:
• Children that suffer from sickle cell disease (SCD) are more vulnerable to factors that negatively impact the care that they receive as well as suboptimal health outcomes. • Shared decision-making (SDM) can help children participate in a collaborative decision-making process about their preferred treatment options and improve their health outcomes.
What Is New:
• The level of participation in the decision-making process for patients suffering from SCD and the families that they belong to leaves room for improvement. The impact of intercultural challenges and the quality and consistency of risk-communication between stakeholders in paediatric SDM needs further exploration. • Paediatricians are more confident about their ability to involve the child and parents compared to how children and their parents experience their level of involvement in a shared decision-making process.
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