Sickle Cell Study Explores Hydroxyurea Non-Adherence, mHealth App Potential

A proposed smartphone-based intervention to address hydroxyurea nonadherence was welcomed by a majority of surveyed individuals with sickle cell disease.

sickle cell disease

Helen Fogarty, MB, MSc, MRCPI

A new study conducted in Ireland noted that recall was a significant barrier to hydroxyurea adherence among patients with sickle cell disease (SCD).

Even more, a majority of the overall patient population expressed enthusiasm for or interest in a smartphone-based application to help increase or maintain adherence.

The survey study was led by Helen Fogarty, MB, MSc, MRCPI, Royal College of Surgeons in Ireland (RCSI), and represented more than 10% of the country’s SCD population.

Previous research has demonstrated that nonadherence to hydroxyurea continues to be a significant challenge for patients and their providers. As noted by the evolving literature, this barrier can stem from unintentional factors (forgetfulness, etc.) or intentional ones (fear, mistrust, etc.)

“Understanding and overcoming the barriers to hydroxyurea adherence in adults and young adolescents could substantially improve health-related quality of life (HRQOL) and other health outcomes,” Fogarty's team wrote.

The Survey

They assessed a total of 63 patients, a majority (63%) of which were female. The mean age of the population was 19 years, and all were of African ethnicity.

Fogarty and her team thus administered a survey, which was largely inspired by the modified Morinsky adherence 8 items (MMAS-8) and Brief Medication Questionnaire (BMQ). Using 28 multiple choice or yes/no questions, as well as a visual analogue scale, the survey evaluated adherence rates and barriers over the past month.

The investigators also incorporated elements from the Patient Reported Outcomes Measurement Information System (PROMIS) in order to assess patient HRQOL. The survey gauged smartphone app preferences using questions informed from the current literature regarding app-based interventions.

“The final survey content and questions were reflective of multidisciplinary input from experts in the field of SCD including paediatric and adult Haematology, Nursing and Psychology,” the team wrote.

Assessing Nonadherence

Thus, they reported that the average monthly self-reported adherence was 76% according to the visual analogue scale. Furthermore, 38% of the participants indicated that hydroxyurea worked “very well,” while 35% indicated it worked “reasonably well.”

Additionally, there was an association between high self-reported adherence (>80%) and perceived high benefits from the medication.

Issues of forgetfulness were noted in 62% of respondents, with 67% of them indicating a reliance on family members to remain adherent.

Another 21% of overall participants reported other reasons for nonadherence. Further, 11% said they discontinue medication when they believe their SCD is under control, and 6% discontinue due to adverse side effects.

Access barriers—such as inability to obtain a prescription or running out of medication—were evident in 33% of survey respondents.

Quality of Life

In terms of HRQOL assessments, 36.5% of patients felt they have been physically able to participate in recreational activities—while 51% reported disruptions in school or work due to pain, and 69% due to inability to concentrate.

As many as 81% of the participants experienced anxiety about health, which they indicated largely interfered with their lives. 

Additionally, 35% expressed worry about others knowing they take hydroxyurea, and 54% were concerned about the long-term effects of the medication.

There was a notable correlation between non-adherence and poorer HRQOL outcomes.

A Mobile Intervention

Of the entire population, 87% expressed interest in a smartphone app designed to improve medication adherence.

The most popular proposed features were daily medication reminders (73%), adherence progress tracking (77%), and facts about SCD (72%)—as compared to features like sharing adherence data with data (55%), rewards for app use (48%), and incentives (43%).

Although these findings were consistent across age groups, discussion forums were more preferable among young adults (18-35 years)‚ versus adolescents (12-17 years; P = .01).


“With recent National Institute of Health (NIH) and British Society of Haematology (BSH) guidelines recommending the use of hydroxyurea in SCD patients from 9 months of age, optimizing adherence across the lifespan is more essential than ever and understanding barriers to adherence may provide valuable and actionable insights,” the Fogarty and colleagues wrote.

They stressed the importance of soliciting and utilizing patient input at the outset of designing future mHealth applications. Furthermore, they noted cost analyses of such technology-based interventions are warranted.

Access to smartphone technologies and socio-economic status should also be considered in future research and implementation, factors which were not explored in the present study.

The study, “Adherence to hydroxyurea, health-related quality of life domains and attitudes towards a smartphone app among Irish adolescents and young adults with sickle cell disease,” was published online in Irish Journal of Medical Science.

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