Patterns of Pain Scores, Health Care Use in Adolescents With Sickle Cell Disease

Sickle cell anaemia. Artwork showing normal red blood cells (round), and red blood cells affected by sickle cell anaemia (crescent shaped). This is a disease in which the red blood cells contain an abnormal form of haemoglobin (bloods oxygen-carrying pigment) that causes the blood cells to become sickle-shaped, rather than round. Sickle cells cannot move through small blood vessels as easily as normal cells and so can cause blockages (right). This prevents oxygen from reaching the tissues, causing severe pain and organ damage.
Investigators assessed the prevalence of neuropathic pain in adolescents with sickle cell disease.

Adolescents with sickle cell disease (SCD) are likely not sufficiently evaluated for neuropathic pain (NP), according to a questionnaire-based study published in Pediatric Blood & Cancer.

Researchers enrolled patients (N=83) aged 12 to 18 years with SCD for the study at St Jude Children’s Research Hospital in June to July 2019. All participants were evaluated by the painDETECT questionnaire and were retrospectively reviewed for pain-related health care during the previous 12 months.

Patients had a mean age of 15.14±2.01 years, 50.6% were girls, 49.4% received hydroxyurea therapy, 15.66% received chronic transfusion therapy, and 78.3% had fewer than 3 pain events during the previous 12 months. The patient population was a mixture of HbSS (48.19%), HbSC (26.51%), HbSβ+ (12.05%), HbSβ0 thalassemia (7.23%), and other (6.02%) genotypes.

Among patients who were receiving hydroxyurea therapy, 19.5% had painDETECT scores indicating NP compared with 16.7% of patients not receiving hydroxyurea therapy (P =.18). No patient receiving chronic transfusion therapy had scores indicative of NP. Only 3 patients were receiving NP-directed therapy.

Most patients (n=81) responded to the painDETECT questionnaire as an outpatient. The average score was 7.72±6.52 points. Stratified by patient status, inpatients reported significantly higher painDETECT scores (mean, 18.14 vs 6.81 points; P =.00025).

Stratified by age, patients who were older (≥15 years: n=57) reported significantly higher painDETECT scores (mean, 9.16 vs 5.06 points; P =.0092). When removing inpatients, whom were all aged 15 years or older, older patients tended to report higher scores (mean, 7.9 vs 5.06 points; P =.055).

The high-pain group (≥3 pain events: n=20) reported significantly higher painDETECT scores overall (mean, 12.43 vs 6.24 points; P =.00026) and as outpatients (mean, 9.94 vs 6.05 points; P =.0092), but not as inpatients (mean, 20.4 vs 12.5 points; P =.079).

No trends were observed on the basis of gender or therapy.

In the multivariate analysis, higher painDETECT scores associated with inpatient status (P =.0098) and the number of pain events (P =.013).

This study may have been limited by using the painDETECT instrument, which has no physical examination component; however, it was chosen because it has been validated for the pediatric population.

This study detected patterns of pain among pediatric patients with SCD. The study authors found that few patients were receiving treatment for NP despite a number of children meeting the criteria, which may indicate an unmet need.

“Our study noted that patients who were hospitalized for acute pain had high painDETECT scores, suggesting a neuropathic mechanism for acute vaso-occlusive crisis (VOC),” the study authors wrote. “Furthermore, we found that patients identified as being in the ‘high-pain group’ experience significantly higher NP scores than patients in the ‘low-pain group.’ These findings add to the current knowledge on the specific characteristics of NP in patients with SCD.”


Cregan M, Puri L, Kang G, Anghelescu D. Prevalence of neuropathic pain in adolescents with sickle cell disease: A single-center experience. Pediatr Blood Cancer. Published online February 11, 2022. doi:10.1002/pbc.29583