Hematology

Sickle Cell Disease

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Psychosocial Stressors and Pain in Sickle Cell Disease

patient care perspectives by Stella T. Chou, MD

Overview

Patients with sickle cell disease (SCD) and acute vaso-occlusive crises experience pain that can be debilitating. A multidisciplinary approach may be helpful in differentiating between SCD-related pain and other forms of pain.

Expert Commentary

Stella T. Chou, MD

Associate Professor of Pediatrics
Chief, Division of Transfusion Medicine
Children’s Hospital of Philadelphia
Perelman School of Medicine at the University of Pennsylvania
Philadelphia, PA

“Adolescents are at a stage in life when they face a large number of stressors in addition to their SCD, making it very important to understand those stressors and to help manage them.”

Stella T. Chou, MD

Being a parent and trying to help with the medical management of your own child is difficult, but as clinicians we do rely on parents to help us in this regard (ie, to provide insights into their children with SCD, especially younger children). For example, parents can be very insightful as to whether their children are experiencing acute pain from a vaso-occlusive event or something else that is manifesting as pain. For young children, it can be difficult to pinpoint a particular stressor; however, most parents will likely tell you that their children might experience a stomachache or a headache at certain times (eg, perhaps around certain stressors), so there is familiarity with that type of pain vs pain that is due to their SCD. Parents are also usually aware of issues that may be going on with their child psychosocially or physically. Parental input helps us recognize whether this is typical childhood pain or whether it is SCD-related pain.

Adolescents with SCD are a special group of patients, and we have to take care and be cautious when treating those who are experiencing pain. Adolescents are at a stage in life when they face a large number of stressors in addition to their SCD, making it very important to understand those stressors and to help manage them. This may involve a multidisciplinary approach with our colleagues in behavioral health, psychologists, or social workers. Relationships with the patient that are forged on the outpatient side can often be helpful when they present with a vaso-occlusive crisis. For example, we may be able to identify whether the adolescent is experiencing difficulties at school or at home that are then manifesting as pain.

With any type of stressor, there is the potential to have somatic symptoms, and this applies to adults and children. It is important to recognize that we cannot effectively address somatic symptoms in a 15- or 20-minute visit. When we identify patients who are in significant pain, I think that we need to take the time to determine whether there are psychosocial reasons for that pain, and then we should think about ways that we can help our patients overcome those psychosocial stressors, if present.

References

Brandow AM, DeBaun MR. Key components of pain management for children and adults with sickle cell disease. Hematol Oncol Clin North Am. 2018;32(3):535-550. doi:10.1016/j.hoc.2018.01.014

Osunkwo I, O’Connor HF, Saah E. Optimizing the management of chronic pain in sickle cell disease. Hematology Am Soc Hematol Educ Program. 2020;2020 (1):562-569. doi:10.1182/hematology.2020000143

Sagi V, Mittal A, Tran H, Gupta K. Pain in sickle cell disease: current and potential translational therapies. Transl Res. 2021;234:141-158. doi:10.1016/j.trsl.2021.03.007

Telfer P, Kaya B. Optimizing the care model for an uncomplicated acute pain episode in sickle cell disease. Hematology Am Soc Hematol Educ Program. 2017;2017(1):525-533. doi:10.1182/asheducation-2017.1.525

Stella T. Chou, MD

Associate Professor of Pediatrics
Chief, Division of Transfusion Medicine
Children’s Hospital of Philadelphia
Perelman School of Medicine at the University of Pennsylvania
Philadelphia, PA

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