Research Offers Improved Stroke-Risk Detection for Children with Sickle Cell Anaemia

A new study on stroke risk among Jamaican children living with sickle cell anaemia is offering hope for improved clinical outcomes.

The study, done by Paediatrician Dr. Angela Rankine-Mullings, provides an expanded pathway for early detection and prevention, which could reshape how stroke risk is identified among the vulnerable population.

It introduces arterial stiffness measurement as a promising complementary tool to Transcranial Doppler (TCD) screening in identifying children who are at risk of a stroke.

“Arterial stiffness has the potential to expand screening in children with sickle cell anaemia. It offers another pathway to saving lives and preventing disability,” Dr. Angela Rankine-Mullings said.

Titled ‘Arterial Stiffness and Stroke Risk Classification in Jamaican Children with Sickle Cell Anaemia: A Completed Cross-Sectional Study,’ the study was presented at the Ministry of Health and Wellness’ recent 16th Annual National Research Conference held at The Jamaica Pegasus hotel in New Kingston.

Dr. Rankine-Mullings, in sharing the research findings, noted that stroke in children with sickle cell anaemia is a critical issue often caused by blockages in the brain’s arteries.

“This can be prevented by screening to diagnose children who are high risk for stroke and treating them early,” she pointed out.

Central to current stroke-prevention strategies is TCD screening, which is a non-invasive technique used to measure blood flow velocity in the brain.

Dr. Rankine-Mullings said that while the method is effective, it is not without limitations.

She noted that the procedure relies on access to specific “bony windows” in the skull, which are not always available in every patient. Additionally, it requires specialised training to perform and interpret accurately.

These barriers, she argued, can limit widespread access to screening, particularly in resource-constrained settings.

Pointing to arterial stiffness measurement as a complementary tool, she said that unlike TCD, which focuses solely on cerebral arteries, arterial stiffness assesses the condition of the body’s larger arteries, particularly the aorta.

Using an arteriograph, a device that operates with a simple brachial cuff, clinicians can evaluate how rigid or flexible arterial walls are, without the need for highly specialised training.

“Healthy arteries move freely, allowing smooth blood flow, but when arteries become stiff, it reflects damage that may be associated with increased stroke risk,” she points out.

While arterial stiffness is widely used in adult populations, there has been little to no data on its application in children with sickle cell disease, particularly in relation to stroke risk.

To address this gap, Dr. Rankine-Mullings conducted a cross-sectional study involving 34 children between the ages of four and 16 years. Participants were divided into high- and low-stroke-risk groups based on existing TCD data.

The study measured three key indicators of arterial stiffness – aortic pulse wave velocity, aortic pulse pressure, and aortic augmentation index.

The findings revealed significant differences between the two groups. Children classified as high stroke risk demonstrated higher aortic pulse wave velocity and pulse pressure, suggesting increased arterial stiffness.

Of the three indicators, pulse wave velocity emerged as the most consistent predictor, remaining significantly different even after adjusting for hydroxyurea use, a common treatment in sickle cell management.

Interestingly, the study also identified variations in certain clinical and biochemical markers between the groups, including hydroxyurea use and levels of gamma glutamyl transferase, a liver enzyme.

While most demographic and physical characteristics were similar, these differences provided additional insight into factors that may influence stroke risk.

Dr. Rankine-Mullings emphasised that her statistical approach accounted for potential confounders by introducing variables sequentially into regression models, rather than constructing a full model with all variables at once.

This decision, influenced by the modest sample size, was acknowledged as a limitation but also a deliberate effort to maintain analytical precision.

She also noted that the study’s cross-sectional design and the absence of imaging data limited the ability to establish causation or visualise structural changes in the arteries.

Nevertheless, the results align with existing international research while contributing new knowledge specific to paediatric populations in Jamaica.

Beyond the data, the implications of the findings are far-reaching. By incorporating arterial stiffness measurements into routine screening, healthcare providers may be able to identify high-risk children earlier and initiate preventive treatment more effectively. This could significantly reduce the incidence of stroke-related disability and mortality among children with sickle cell disease.

Dr. Rankine-Mullings said that the research provides “a practical, scalable solution that could transform stroke prevention strategies in Jamaica and beyond”.

With more than 30 years of medical experience and more than a decade dedicated to sickle cell research, Dr. Rankine-Mullings brings both expertise and passion to her work.

As a Senior Research Fellow at the Sickle Cell Unit within the Caribbean Institute for Health Research (CAIHR), she has been instrumental in advancing clinical trials, particularly in the use of hydroxyurea as a disease-modifying therapy.

Her contributions extend to major international collaborations, including National Institutes of Health (NIH)-sponsored trials such as SCATE and EXTEND, conducted in partnership with Cincinnati Children’s Hospital Medical Centre.

These studies have played a critical role in expanding treatment options and improving quality of life for children with sickle cell disease.

Looking ahead, Dr. Rankine-Mullings and her team are already planning further research to build on these findings, including studies that incorporate imaging and larger sample sizes.

Her PhD work continues to explore the vascular biology underlying neurological injury in sickle cell disease, with a focus on integrating arterial stiffness and TCD measurements.