Introduction: Sickle-cell disease (SCD) is characterised by episodes of acute self-limiting pain due to vaso-occlusion. Around two-thirds of SCD patients are HbSS homozygotes whilst most of the remainder have HbSC disease. The HbSC phenotype is typically milder and patients experience fewer pain episodes. Whilst oral and maxillofacial pain and altered facial sensation have been reported in SCD, no genotype-specific data has been published.
Methods: 208 SCD patients were surveyed via post, telephone or face-to-face interview on oral and maxillofacial sequelae of sickle-cell crises. Patients that did not have HbSS or HbSC SCD were excluded from the study.
Results: 179 patients met the inclusion criteria; 101 had HbSS disease and 78 HbSC. 66% of HbSS patients experienced sickle bone pain once a month or more, compared to 49% of HbSC patients ( P < 0.05 Chi-Square). 40% of HbSS sufferers had experienced painful crises associated with the jaw and/or face, compared to 24% of HbSC patients ( P < 0.05 Chi-Squared). 36% of HbSS patients had experienced altered facial sensation compared to 8% of HbSC patients ( P < 0.05 Chi Square).
Conclusion: These data further delineate the HbSS and HbSC phenotypes. Altered facial sensation, and sickle crises associated with oral and maxillofacial pain are more common in patients with HbSS-SCD than HbSC-SCD. The most common trigger for SCD patients to access medical services is due to painful crises; Medical and Dental professionals must be aware of the potential involvement of the face and jaw, especially in HbSS patients.
Conflict of interest: None declared.