Background Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin S, leading to red blood cell deformities, chronic hemolysis, and frequent vaso-occlusive crises (VOC). While advancements in medical care have improved survival rates, adults with SCD continue to face substantial challenges in their quality of life (QoL) due to chronic pain, recurrent VOCs, and various complications. This study aimed to evaluate the health-related quality of life (HRQoL) in adult patients aged 14 years and above with SCD and identify key factors influencing patient outcomes using the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me). Methods A cross-sectional study was conducted at the Prince Sultan Oncology Center, King Salman Northwest Armed Forces Hospital, Tabuk, Saudi Arabia, between December 2019 and May 2020. The study population comprised adult SCD patients attending outpatient clinics. The QoL was assessed using the ASCQ-Me Short Form, which evaluates five domains: emotional impact, social functioning, pain impact, sleep impact, and stiffness impact. Additionally, a nine-item SCD Medical History Checklist was used to evaluate complications and treatments. Data were collected through structured one-on-one interviews. Scores were transformed into T-scores (mean = 50, standard deviation (SD) = ±10) based on standardized guidelines. Inferential analysis was conducted to compare QoL domains between groups stratified by VOC frequency and severity using the Mann-Whitney U test, with a significance threshold of p ≤ 0.05. Results A total of 53 adult SCD patients were surveyed, with the majority aged 25-34 years (50%). Gender distribution was nearly equal, with 50.9% male participants. The prevalence of complications was notable, with 31% reporting chronic pain, 26% reporting gallstones, and 9% reporting avascular necrosis. Laboratory findings revealed a mean hemoglobin level of 8.63 g/dL (SD: 1.59) and an average fetal hemoglobin (HbF) level of 10.59% (SD: 5.64). The frequency of VOCs varied, with 30% of patients reporting no VOC in the past year, while 57% experienced two to three VOCs. Patients with higher VOC frequency (≥4 per year) reported significantly lower scores across all QoL domains except stiffness (p < 0.05). Higher VOC severity was associated with poorer sleep quality and social functioning (p < 0.05). Pain severity was also a critical determinant, with more than 55% of patients rating their last pain episode as severe or extreme. Conclusion The study highlights the significant burden of pain and VOCs on the quality of life in adult SCD patients. Frequent VOCs and chronic complications such as avascular necrosis and gallstones were major contributors to reduced HRQoL. Findings emphasize the need for comprehensive, multidisciplinary care approaches targeting pain management, psychological support, and functional independence. The ASCQ-Me tool proved valuable for identifying specific domains of impairment and guiding patient-centered interventions. Future research should focus on longitudinal studies to explore the efficacy of tailored interventions and address the ongoing challenges faced by this vulnerable population.

This chapter delves into uncommon wounds including pyoderma gangrenosum, sickle cell disease ulcers, vasculitic wounds, Martorell hypertensive ischemic leg ulcers, and malignant ulcers. Emphasizing a multidisciplinary approach, it covers diagnostics, treatments, and challenges, with case studies illustrating complexities in managing these conditions. The discussion extends to radiation-related wounds, underscoring the need for patient-centered care, interdisciplinary collaboration, and realistic goal setting. Overall, the chapter navigates the intricacies of uncommon wounds, emphasizing the importance of tailored approaches for improved outcomes in patients with diverse underlying conditions.

Introduction: Different mechanisms contribute to myocardial dysfunction in sickle cell disease [SCD] and beta thalassaemia major [TM]. TM mainly involves the highly vascular subepicardium by iron load and SCD mainly operates by inducing ischaemia in the relatively ischaemic subendocardium. The aim of this article was to determine if pattern of left ventricular [LV] dysfunction differ among the two groups of patients.Methods: Forty TM and 40 SCD patients and 40 age- and surface area-matched controls were subjected to conventional echocardiography, 2D Speckle tracking myocardial layer strain discriminating echocardiography (MLSD-STE) which is able to discriminate if myocardial dysfunction is predominantly subepicardial or subendocardial and 3D echocardiography for ejection fraction assessment as well as haemoglobin, ferritin, and lactate dehydrogenase levels.Results: TM patients had a deeper subepicardial dysfunction while SCD had prevalent subendocardial dysfunction, epicardial GLS (TM: -10.9 ± 2 vs. SCD: 19.9 ± 1.7; p value < 0.01); endocardial GLS (TM: -19.9 ± 1.7 vs. SCD: -10.6 ± 1.6, p value < 0.01).Conclusion: This study points towards divergent microcirculatory mechanisms in the pathogenesis of myocardial dysfunction in haemoglobinopathies. It shows predominant subendocardial dysfunction with underlying ischaemia of SCD and prevalent subepicardial iron-induced injury in cases of TM.

Sensorineural hearing loss (SNHL) has been reported to occur at increased frequency in the pediatric sickle cell disease (SCD) population, likely secondary to ototoxic medication regimens and repeat sickling events that lead to end organ damage. Risk and protective factors of SNHL in this population are not fully characterized. The objective of this study was to describe audiology results in children with SCD and the prevalence and sequelae of SNHL.

A comprehensive clinical database of 2600 pediatric SCD patients treated at 1 institution from 2010-16 was retrospectively reviewed to identify all patients who were referred for audiologic testing. Audiologic test results, patient characteristics, and SCD treatments were reviewed.

181 SCD children (97 male, 153 HbSS) underwent audiologic testing, with 276 total audiology encounters, ranging 1-9 per patient. Mean age at first audiogram was 8.9 ± 5.2 years. 29.8% had prior cerebrovascular infarct and an additional 25.4% had prior abnormal transcranial Doppler screens documented at time of first audiogram. Overall, 13.3% had documented hearing loss, with 6.6% SNHL. Mean pure tone average (PTA) among patients with SNHL ranged from mild to profound hearing loss (Right: 43.3 ± 28.9, Left: 40.8 ± 29.7), sloping to more severe hearing loss at higher frequencies.

Hearing loss was identified in a significant subset of children with SCD and the hearing loss ranged from normal to profound. Though the overall prevalence of SNHL in SCD patients was low, baseline audiology screening should be considered.