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World Sickle Cell Disease Day

World Sickle Cell Disease Day

19 June 2024

The Global Action Network for Sickle Cell & Other Inherited Blood Disorders (GANSID) hopes for uniformity in awareness campaigns as #WorldSickleCellDay is celebrate on June 19, 2024. This year’s theme is “Hope Through Progress: Advancing Global Sickle Cell Care & Treatment”. The Global Action Network for Sickle Cell & Other Inherited Blood Disorders (GANSID)

Sickle cell disease

Sickle cell disease is a group of blood disorders that affect the haemoglobin. Haemoglobin is in red blood cells and its responsible for carrying oxygen around the body.  
Sickle cell disease is an inherited condition. To have sickle cell anaemia, both parents must have a faulty gene.

The parents may not:

  • have sickle cell anaemia
  • know that they carry the gene for sickle cell anaemia

The genes for sickle cell disease are most often found in people with any of the following ethnic backgrounds:

  • African
  • Caribbean
  • Indian
  • Middle Eastern
  • Pakistani
  • South American
  • Southern European

People with sickle cell anaemia may have these symptoms:

  • be tired
  • have pale skin
  • Children with sickle cell anaemia may have delayed growth and development.

The sickle (crescent) shape of the red blood cells can cause them to get caught in small blood vessels. This can cause:

  • pain in hands and feet
  • bone and chest pain
  • bacterial infections
  • organ damage

Health direct

Patient resources







Cochrane Library

Clinical practice guidelines


Mental health



Treatment and therapies





Mental health

Psychiatric comorbidities in adults with sickle cell disease: A narrative review
Although descriptions of quality of life and patient reports of mood in sickle cell disease (SCD) have become more common in the literature, less is known about psychiatric illness prevalence, presentation, and treatment, particularly for adults. We provide a narrative review of what is known about common and debilitating psychiatric conditions such as depression, anxiety, and cognitive impairment, specifically for adults with SCD. We discuss the limitations of the current evidence, make provisional recommendations, and identify opportunities for research and improved care. British journal of haematology 16 July 2023

Impact of hospitalization for vaso-occlusive crisis on health-related quality of life in children with sickle cell disease
The adverse effects of hospitalization for vaso-occlusive crises (VOCs) in children with Sickle cell disease (SCD) persist up to 12 months after hospitalization. After hospitalization for VOC, extra attention and support for its negative impact on health-related quality of life (HRQoL) are recommended. This study also underlines the importance of systematically measuring HRQoL, allowing clinicians to intervene accordingly. Pediatric blood and cancer 25 September 2023

Mental health, pain and likelihood of opioid misuse among adults with sickle cell disease
Depressive symptoms are prevalent in individuals living with sickle cell disease (SCD) and may exacerbate pain. This study examines whether higher depressive symptoms are associated with pain outcomes, pain catastrophizing, interference and potential opioid misuse in a large cohort of adults with SCD. British journal of haematology 3 January 2024

The effect of an adapted digital mental health intervention for sickle cell disease on engagement: a pilot randomized controlled trial
Target enrollment for this study sought to enroll 40 participants. However, after difficulties locating qualified participants, enrollment criteria were adjusted to expand the population pool. Regardless of these efforts, the sample size for this study was still smaller than anticipated (n = 21). Additionally, irrespective of group approximately 40% of participants did not engage with the app. However, despite a small sample size and poor engagement, this study 1) demonstrated the feasibility of implementing socially relevant changes into a mental health app and 2) indicated that participants in the intervention group displayed better outcomes and showed trends for greater app interaction. BMC digital health 18 December 2023

Psychosocial challenges of persons with sickle cell anemia: A narrative review
Sickle cell anemia (SCA) is a severe form of sickle cell disease that primarily affects black populations and individuals in tropical countries. This condition causes significant morbidity and mortality and leads to a range of psychosocial challenges. Mental disorders, sleep disturbances, interpersonal relationship challenges, stigmatization, and workplace discrimination were identified as significant contributors to the psychosocial distress experienced by individuals with SCA and their families. Depression and anxiety were prevalent among individuals with SCA, leading to poor treatment adherence, increased pain, and disruptions in various aspects of life. Sleep disturbances, including sleep-disordered breathing and sleepwalking, were also identified as significant contributors to poor sleep quality in SCA patients. Families of individuals with SCA also face challenges, including psychological stress, financial strain, and social disruption. Stigmatization is common, leading to misconceptions and discrimination. Workplace discrimination is prevalent, with a high unemployment rate among adult SCA patients. Comprehensive care is crucial to address these psychosocial issues. Early identification and intervention, comprehensive support programs, patient and family education, enhanced pain management strategies, and integration of mental health into clinical care are recommended. School-based support, research and advocacy, and community support groups are also important. By addressing these challenges through comprehensive care and support, healthcare professionals, policymakers, and society can reduce psychosocial distress and improve the lives of individuals with SCA. Medicine 24 November 2023

Sickle Cell virtual support group program for adults: An implementation evaluation
Sickle cell disease (SCD) is associated with medical and psychosocial challenges. SCD in adulthood is characterized by increased morbidity and mortality, vulnerability, inadequate self-management preparation, and limited social support. This study evaluated the implementation of a virtual support group during the COVID-19 pandemic using an intervention parameters framework evaluation. Issues in mental health nursing 7 August 2023



Sickle cell disease in Australia: a snapshot from the Australian Haemoglobinopathy Registry
This study provides the first national picture of sickle cell disease (SCD) in Australia, describing the characteristics and needs of SCD patients, elucidating demand for current and novel therapy and facilitating the planning of services for this vulnerable population. Internal medicine journal 8 December 2023

Predictive factors for 30-Day readmission and increased healthcare utilization in Sickle Cell Disease patients: A single-center comparative retrospective study
This study identified factors associated with 30-day readmission rate and high healthcare utilization among sickle cell disease (SCD)  patients. Strategies to reduce readmissions may include specialized SCD clinics, educational programs for patients, improved physician awareness of mental health screening, and further research on the impact of opioid use. Limitations include retrospective nature, single-center design, reliance on self-reported data, and exclusion of critically ill patients. However, despite the limitations, this study could lay a foundation for future projects aiming to optimize care and outcomes for patients living with SCD. International journal of general medicine 11 May 2024

Investigating home-based opioid use among youth with sickle cell disease using ecological momentary assessment
Youth with sickle cell disease (SCD) take opioids appropriately in response to their pain, based on daily self-report. Beyond daily pain severity, age, and daily variation in positive affect were related to home-based opioid use. This suggests that behavioral interventions that enhance positive affect may promote reduced opioid use among youth with SCD. Pediatric blood and cancer 27 May 2024

Association of elevated tricuspid regurgitation velocity with cerebrovascular and kidney disease in children with sickle cell disease
This cross-sectional, multicenter study identifies associations between surrogate markers of pulmonary hypertension with kidney disease and cerebrovascular disease. A prospective study should be performed to evaluate the longitudinal outcomes for patients with multiple surrogate markers of end-organ disease. Pediatric blood and cancer 21 April 2024

Promise of Composite Pain Index as a single pain outcome for sickle cell disease across the lifespan
The initial validation of Composite Pain Index (CPI) as a single pain outcome measure represents a significant advancement in pain assessment for sickle cell disease (SCD). Further validation is warranted for the CPI as a measure is for both clinicians and researchers to enable longitudinal pain assessment from age 8 years across the lifespan as children age into adult care. Pediatric blood and cancer 4 April 2024

Assessment of menstrual health in adolescent and young adults with sickle cell disease
The prevalence of heavy menstrual bleeding (HMB) and dysmenorrhea is high among adolescents and young women with Sickle cell disease (SCD). Strategies that incorporate menstrual health assessment into routine medical care in this population would help address this important area of pediatric health. Pediatric blood and cancer 16 October 2024

Gastrointestinal symptoms, diagnostic evaluations, and abdominal pathology in children with sickle cell disease
Children with sickle cell disease (SCD)  frequently present with abdominal pain and other GI symptoms, with limited gastrointestinal (GI) evaluations performed. GI-specific evaluation may increase diagnosis of GI pathologies, rule out GI pathologies, and contribute to the limited knowledge of the abdomen as a primary site of SCD pain. Pediatric blood and cancer 3 October 2024

Assessment of fatigue in adult patients with sickle cell disease: Use of the functional assessment of chronic illness therapy—Fatigue (FACIT-fatigue) questionnaire
Most adult patients with sickle cell disease (SCD) experience significant and sometimes intense fatigue; this is probably due to several factors, including disease activity. Fatigue should be evaluated systematically during consultations and in patient education programmes and as an end-point in therapeutic trials. British journal of haematology 27 May 2024



Sustained increase in annual transcranial Doppler screening rates in children with sickle cell disease: A quality improvement project
Although other QI initiatives had demonstrated ability to increase adherence to transcranial Doppler (TCD) screening for patients with sickle cell disease (SCD), this is the first quality improvement (QI) project to collect data over such a prolonged period of time to demonstrate a sustained increase in screening rates throughout the intervention (an 8-year period). Pediatric blood and cancer 28 May 2024

“Everyone screens to some extent”: Barriers and facilitators of developmental screening among children with sickle cell disease: A mixed methods study
Developmental screening is inconsistent and insufficient for young children with SCD. Providers are interested in supporting children with SCD, but report a lack of standardized measures and consistent guidance as barriers. Pediatric blood and cancer 17 May 2024


Treatment and therapies

Current state of gene therapy in sickle cell disease

  • Gene therapy is potentially a curative treatment for sickle cell disease.
  • Early findings show lower occurrence of vaso-occlusive crisis and reduced haemolysis, although longer follow-up is still necessary to evaluate adverse effects.
  • The cost of gene therapy will be a significant barrier to its accessibility.

Vox Sanguinis 15 March 2024

Hematopoietic stem cell transplantation in Sickle Cell Disease: A multidimensional review
This review encompasses information on the use of hematopoietic stem cell transplant (HSCT) in patients with SCD, including the indications for HSCT, conditioning regimens, alternative donors, and posttransplant outcomes. Cell transplantation 28 April 2024



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