Sickle cell disease (SCD) is the most common severe monogenic condition in the world. The central pathophysiology of this disease is related to hemoglobin polymerization, leading to erythrocyte rigidity and vaso-occlusion. Vaso-occlusion and ischemia are responsible for acute systemic painful vaso-occlusive crisis, leading to pain being the most common morbidity in these patients. These individuals are more likely to experience frequent work/school absences, depression, impaired peer relationships and lower quality of life when compared to those without pain crisis.Therefore, identifying the characteristics of the most vulnerable patients in this population towards painful episodes can assist in clinical investigation and screening.

Identify characteristics of the most vulnerable patients towards pain and amplify the search for them in the clinical practice. Methods: Cross-sectional study with 59 patients of a SCD cohort at HemominasJF/MG, aged 18-60 from all social classes. Pain was assessed with 2 questions from the SF-36 questionnaire, each contributing 50% to the score, which were:“How much bodily pain have you had during the past 4 weeks? (1) none, (2) very mild, (3) mild, (4) moderate, (5) severe or (6) very severe”, and “During the past 4 weeks, how much pain interferes with your normal work (including both work outside home and housework)? (1) not at all, (2) a little bit, (3) moderately, (4) quite a bit and (5) extremely”. The individual started with 100 points and the higher the pain the lower the score, losing 10 points for each higher degree of pain in the 1st question and 12,5 in the 2nd. The answers none or not at all did not retract any points.Scores were calculated, considering answer combinations, leading to the categories: no pain (100), mild pain (90-80), moderate pain (70-45),moderate to severe pain (45-22.5), and severe or very severe pain (below 22.5).

In a cohort of 62 patients, 47 patients used hydroxyurea (HU) and, genotypically, 43 were HbSS, 10 HbSC, 8 HbSBeta, and 1 HbSAlfa.In 28 pain-free patients (mean age 31.4 ± 12.4 years), 82.1% used HU (1054.2 ± 471.4 mg/day), and 75% had HbSS. Nine reported mild pain (average age 30.9 ± 12.2 years, all black), 66.7% had HbSS and 66.7% used HU (1327.2 ± 718.9 mg/day).In the moderate pain group (12 patients, average age 30 ± 9.7 years), 75% had HbSS, with 75% using HU (1147.4 ± 616.5mg). Out of 6 patients with moderate to severe pain (average age 37 ± 12.7 years, 83.3% black, 66.7% female, 50% having HbSS), 4 used HU (910.5 ± 473.2 mg).Seven had severe to very severe pain (mean age 27.1 ± 5.7 years, 57.1% black, 57.1% female), 57.1% had HbSS, 71.4% used HU (1164 ± 563.8 mg). These findings underscore the varied impact of sickle cell disease, highlighting variations in pain experience, medication usage, and genotype distribution among distinct pain severity groups. Discussion:The demographic characteristics and the use of HU were similar between the groups with and without pain. However, it is noteworthy that the frequency of pain in sickle cell anemia is high, with 54.8% (n = 34) of patients experiencing pain. Among those with pain, 73.5% (n = 25) reported moderate to very severe pain.

Understanding the frequency and intensity of pain in Sickle Cell Disease highlights the need for a more effective approach in pain management for this population.