An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective

Abstract

Background: Effective stroke prevention in sickle cell disease (SCD) is recommended for children with sickle cell anaemia. Effective implementation relies on the correct stratification of stroke risk using Transcranial Doppler Ultrasound (TCD), prior to committing children to long-term treatment with transfusion. Nevertheless, less than 50% of children with SCD in Europe receive annual TCD—one of the reasons being a lack of trained personnel. The present European multi-centre study was designed to promote the standardisation and delivery of effective screening. Methods: Fifty-five practitioners from differing professional backgrounds were recruited to the TCD training program. The impact of the training programme was evaluated in three European haematology clinics by comparing stroke risk classification and middle cerebral artery time-averaged maximum velocity (TAMMV) obtained from a cohort of 555 patients, before and after training. Results: 42% (23/55) of trainees successfully completed the program. The TAMMV, used to predict stroke risk at each Centre, demonstrated the highest values in Centre 3 (p < 0.0001) before training. The imaging-TCD TAMMV was also higher in Centre 3 (p < 0.001). Following training, the TAMMV showed closer agreement between centres for both imaging-TCD and non-imaging TCD. The stroke risk distribution of children at each centre varied significantly before training (p < 0.001), but improved after training (Fisher’s Exact: no treatment = 5.6, p = 0.41, treatment = 13.8, p < 0.01). The same consistency in stroke risk distribution following training was demonstrated with both non-imaging and imaging-TCD data. Conclusion: The attainment of competency in stroke screening using transcranial Doppler scanning (TCD) in sickle cell disease is more feasible for professionals with an ultrasound imaging background. A quality assurance (QA) system is required to ensure that standards are maintained. Further work is in progress to develop an achievable and reproducible QA program.

Transcranial doppler as screening method for sickling crises in children with sickle cell anemia: a latin America cohort study

“…We used a shared frailty survival model with a gamma distribution, in which individuals were the random effects component of the model [ 12 ]. The decision to use this approach for regression analyses was based on the following arguments.…”

“…Although very efficient in preventing stroke, the TCD screening protocol is poorly implemented, even in resourceful countries. Less than 50% of children in Europe receive annual TCD examinations [ 12 ]. In a survey conducted at 28 sites using annual TCD screenings, differences in the stroke prevention practices demonstrated the real-world challenges from translation of trials into clinical practice [ 13 ].…”

Sickle Cell Disease (SCD): Usefulness of Transcranial Doppler (TCD/TCCS) Monitoring

“…This affirmation, however, posts several points to consider. First, there is evidence of low adherence to periodic TCD, especially for patients seen first at the emergency department [37], therefore there is an opportunity for increased screening as to whether new efforts point toward recruiting new patients and continue educating personnel at these specific clinical settings [38].…”

Relationship between hemoglobin, hemolysis, and transcranial Doppler velocities in children with sickle cell disease: Results from a long‐term natural history study in Italy in the era of multimodal therapy

“…Over the past decade, the optimization of comprehensive care and treatment has led to some advances in the management of pediatric SCD 5,6 . Of note, the effectiveness of transcranial Doppler (TCD) ultrasonography in stratifying stroke risk in patients with SCD has been confirmed by randomized controlled trials, which has established the use of TCD screening and monitoring for stroke prevention in children with SCD 2,7,8 . Generally, abnormal (≥200 cm/s) or persistently high conditional (≥170 to <200 cm/s) cerebral artery blood flow velocities, as assessed by TCD, are an indication to start a chronic transfusion regimen and to consider disease‐curative options 2,4,8,9 .…”

“…5,6 Of note, the effectiveness of transcranial Doppler (TCD) ultrasonography in stratifying stroke risk in patients with SCD has been confirmed by randomized controlled trials, which has established the use of TCD screening and monitoring for stroke prevention in children with SCD. 2,7,8 Generally, abnormal (≥200 cm/s) or persistently high conditional (≥170 to <200 cm/s) cerebral artery blood flow velocities, as assessed by TCD, are an indication to start a chronic transfusion regimen and to consider disease-curative options. 2,4,8,9 In case of stable normalization of TCD velocities after 1 year of transfusions, switching to hydroxyurea (HU) treatment can be considered in the absence of stenosis with magnetic resonance angiography (MRA).…”