A new study has determined that, in addition to pain, disease-related symptoms negatively impact children and adolescents with sickle cell disease (SCD). The research, published in the Journal of Pain Symptom Management, noted that the frequency of the symptoms reduced the health-related quality of life (HRQOL) of young patients, particularly as they became older.

“The current study aims to identify whether pain and SCD-related symptom frequency separately predict the HRQOL of children diagnosed with SCD, after controlling for sociodemographic variables,” the authors wrote. “Although pain is considered the hallmark symptom associated with SCD, this population also experiences a plethora of other SCD-related symptoms that may impact their wellbeing, which warrants this type of investigation.”

The research team conducted a cross-sectional study on 150 patients with SCD aged between 8 and 17 years at a single hospital between July 2018 and February 2019. Information on the patients’ HRQOL was collected using the PedsQL 3.0 SCD module, a 9-domain self-assessment questionnaire. Parents were also surveyed regarding the frequency of pain and SCD-related symptoms in their children.

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The results demonstrated that the frequency of pain and of SCD-related symptoms both reduced HRQOL in this population. SCD-related symptoms include infection, acute chest syndrome, and acute hemolytic crises. Unexpectedly, age emerged as the only significant predictor of worsening HRQOL and pain among the patients in the study.

The authors had expected, as occurs with adults, that genotype, income, and sex would be associated with worsening pain and HRQOL, but this was not the case. The authors hypothesize that one explanation for the age connection with pain could be that adolescents and young adults may transition from acute pain to chronic pain due to avascular necrosis, and it is possible that mental health issues including stress tend to worsen as children become adolescents, which could exacerbate pain and lower HRQOL.


Moody KL. Association of sickle cell pain & symptoms on health-related quality of life among pediatric patients. J Pain Symptom Manage. Published online May 9, 2022. doi: 10.1016/j.jpainsymman.2022.05.003