Sickle Cell Disease: Understanding the Condition, Recognizing Complications, and Advancing Care
Recognizing National Sickle Cell Awareness Month
By Rodrico Gordon, DNP-C, AGPCNP-BC, NE-BC
Founder and Clinical Director, Gordon Wellness Service
To Learn more about Sickle Cell Disease, click to watch the video above.
Every September, National Sickle Cell Awareness Month provides an opportunity to increase understanding of sickle cell disease, support individuals and families affected by the condition, and highlight continued advances in treatment and research (National Heart, Lung, and Blood Institute [NHLBI], 2026).
Sickle cell disease (SCD) is a group of inherited blood disorders affecting hemoglobin, the protein within red blood cells responsible for carrying oxygen throughout the body. Approximately 100,000 people in the United States are living with sickle cell disease. Although SCD occurs in people from many racial and ethnic backgrounds, it disproportionately affects individuals of African ancestry in the United States and is also seen among people with ancestry from parts of the Mediterranean, Middle East, India, the Caribbean, and Central and South America (Centers for Disease Control and Prevention [CDC], 2024).
For adults living with sickle cell disease, appropriate preventive care, recognition of complications, and coordination between primary care and hematology are important components of long-term health (American Society of Hematology [ASH], 2020).
What Is Sickle Cell Disease?
Sickle cell disease results from inherited changes affecting hemoglobin. The best-known form, sickle cell anemia, is associated with hemoglobin S (NHLBI, 2024).
Under certain conditions, hemoglobin S can polymerize within red blood cells. Instead of maintaining their usual flexible, disc-like shape, affected red blood cells can become rigid and develop the characteristic crescent or “sickle” shape (Kato et al., 2018).
These abnormal cells can break down prematurely, contributing to chronic hemolytic anemia. They can also obstruct small blood vessels, reducing blood and oxygen delivery to tissues. This process, known as vaso-occlusion, contributes to many of the acute and chronic complications associated with the disease (Kato et al., 2018).
Sickle Cell Disease vs. Sickle Cell Trait
Sickle cell disease and sickle cell trait are not the same condition.
A person with sickle cell trait has inherited one sickle hemoglobin gene and one normal hemoglobin gene. Most people with sickle cell trait do not experience the clinical manifestations associated with sickle cell disease (CDC, 2024).
Sickle cell disease occurs when a person inherits genetic variants that result in clinically significant sickling disorders, such as hemoglobin SS disease or certain combinations involving hemoglobin S and another abnormal hemoglobin gene (NHLBI, 2024).
Understanding carrier status can be particularly important when planning a family. Adults who do not know their hemoglobin status can speak with a healthcare professional about whether hemoglobin testing or genetic counseling is appropriate (CDC, 2024).
Sickle Cell Disease Is More Than a Pain Disorder
Pain is one of the most recognizable manifestations of SCD, but the disease can affect virtually every organ system (Kato et al., 2018).
A vaso-occlusive crisis occurs when sickled cells and other disease processes interfere with normal blood flow. These episodes may produce severe pain and sometimes require urgent medical treatment or hospitalization (ASH, 2020).
However, SCD can also be associated with:
Acute and chronic anemia
Acute chest syndrome
Stroke and other neurologic complications
Kidney disease
Pulmonary and cardiovascular complications
Eye disease
Bone and joint complications
Increased susceptibility to certain infections
Chronic pain
Priapism
Liver and gallbladder complications
Progressive organ damage
These complications reflect the systemic nature of SCD and the effects of recurrent vaso-occlusion, hemolysis, inflammation, and vascular injury (Kato et al., 2018). Because some complications develop gradually, routine medical follow-up remains important even when a person feels relatively well.
Recognizing Symptoms That Require Immediate Medical Attention
People living with sickle cell disease should have an individualized plan for managing symptoms and knowing when urgent medical evaluation is necessary (ASH, 2020).
Symptoms such as chest pain, difficulty breathing, fever, sudden neurologic changes, severe or unusual pain, or prolonged painful erections (priapism) warrant prompt medical assessment (NHLBI, 2024).
Acute chest syndrome is an especially serious complication of SCD and can present with symptoms including chest pain, fever, cough, shortness of breath, or low oxygen levels (Kato et al., 2018).
Sudden weakness, numbness, difficulty speaking, facial drooping, confusion, or other symptoms concerning for stroke require emergency evaluation.
Managing Sickle Cell Disease Across Adulthood
Modern sickle cell management extends well beyond treating individual pain episodes.
Long-term care may involve hematology, primary care, pain management, nephrology, cardiology, ophthalmology, behavioral health, and other specialties depending upon the individual's needs and complications (ASH, 2020).
Preventive and chronic disease care remain particularly important as individuals with SCD move through adulthood. Routine care may include monitoring blood pressure, kidney function, blood counts, medication use, immunizations, cardiovascular risk factors, and other age-appropriate preventive health needs (ASH, 2019, 2020).
The American Society of Hematology has developed evidence-based guidelines addressing several major areas of SCD care, including acute and chronic pain, kidney and cardiopulmonary complications, transfusion therapy, cerebrovascular disease, and stem-cell transplantation (ASH, 2019, 2020).
Hydroxyurea and Disease-Modifying Treatment
One of the most established disease-modifying treatments for sickle cell disease is hydroxyurea.
Hydroxyurea increases fetal hemoglobin and can reduce red blood cell sickling. Clinical evidence has demonstrated reductions in complications such as vaso-occlusive pain episodes and acute chest syndrome (Charache et al., 1995). Long-term studies have also supported its important role in reducing morbidity and mortality among adults with sickle cell anemia (Steinberg et al., 2003).
Treatment decisions are individualized. Medication selection depends on factors including the specific type of SCD, previous complications, age, other health conditions, reproductive considerations, treatment response, and patient preferences.
Blood transfusion therapy also remains an important component of care for selected patients and clinical situations, including prevention or management of certain severe complications (Chou et al., 2020).
A New Era: Gene Therapy and Potentially Curative Treatment
Perhaps one of the most significant developments in modern sickle cell treatment has been the emergence of gene therapy.
In 2023, the U.S. Food and Drug Administration approved the first cell-based gene therapies for sickle cell disease. One of these treatments, exagamglogene autotemcel (Casgevy), became the first FDA-approved therapy utilizing CRISPR/Cas9 genome-editing technology (U.S. Food and Drug Administration [FDA], 2023).
Clinical trial evidence published in The New England Journal of Medicine demonstrated that exagamglogene autotemcel could eliminate severe vaso-occlusive crises for extended periods in many treated patients (Frangoul et al., 2024).
Treatment has continued to evolve. In July 2026, the FDA expanded the indication for Casgevy to include eligible patients 2 years of age and older with sickle cell disease and recurrent vaso-occlusive crises (FDA, 2026).
These therapies are not appropriate for every person with SCD. They require specialized treatment centers, collection and modification of a patient's blood-forming stem cells, intensive conditioning therapy, and careful short- and long-term follow-up (Frangoul et al., 2024).
Stem-cell transplantation may also offer a potential cure for selected individuals with SCD, although the risks and benefits must be carefully considered for each patient (ASH, 2021).
Everyday Health Still Matters
Specialized treatment does not replace the importance of everyday health maintenance.
People living with sickle cell disease should work with their healthcare team on an individualized plan that may include maintaining adequate hydration, staying current with recommended vaccinations, taking prescribed medications consistently, attending routine medical appointments, avoiding known personal triggers for vaso-occlusive episodes when possible, and addressing other chronic health conditions (NHLBI, 2024).
Regular primary care is also important. Adults with SCD still require routine preventive healthcare, including appropriate cancer screening, cardiovascular risk assessment, sexual and reproductive healthcare, mental health support, and management of conditions such as hypertension, diabetes, or obesity when present.
Awareness Includes Understanding Sickle Cell Trait
National Sickle Cell Awareness Month is also an opportunity for people to learn more about their own hemoglobin status.
Because sickle cell trait usually does not cause the symptoms associated with sickle cell disease, some adults may be unaware that they carry the trait (CDC, 2024).
Knowing your status can provide valuable information for reproductive planning. When both prospective parents carry hemoglobin variants, genetic counseling can help them better understand the probability of passing sickle cell disease or another hemoglobin disorder to their children (CDC, 2024).
Moving Toward Better Sickle Cell Care
The story of sickle cell disease is changing.
What was once managed primarily through supportive treatment now has multiple disease-modifying approaches, increasingly sophisticated multidisciplinary care, stem-cell transplantation, and gene-based therapies (ASH, 2020; FDA, 2023; Frangoul et al., 2024).
Yet awareness remains essential. Individuals living with sickle cell disease can still encounter barriers to comprehensive care, particularly during the transition from pediatric to adult healthcare. Improving access to knowledgeable clinicians, evidence-based pain management, preventive healthcare, hematology services, and emerging therapies remains an important public health priority.
This September, National Sickle Cell Awareness Month is an opportunity not only to recognize the challenges associated with SCD, but also to encourage education, informed conversations, and continued progress in the care of people living with the disease.
When to Speak With a Healthcare Professional
Consider discussing sickle cell disease or sickle cell trait with a healthcare professional if you:
Have sickle cell disease and need ongoing adult preventive or chronic disease care
Are unsure whether you carry sickle cell trait
Have a family history of sickle cell disease or another hemoglobin disorder
Are planning a family and have questions about carrier status
Have SCD and are experiencing new or changing symptoms
Need help coordinating primary care with hematology or other specialists
Gordon Wellness Service provides adult primary care, preventive care, chronic disease management, and health education, with referral and coordination with hematology and other specialists when appropriate.
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Medical Disclaimer
This article is provided for general educational and informational purposes only and is not intended to replace professional medical advice, diagnosis, or treatment. The information presented should not be used to diagnose or manage a medical condition without consultation with a qualified healthcare professional. Individual medical needs and treatment recommendations may vary. Always consult your healthcare provider regarding questions about your health, symptoms, medications, testing, or treatment options. If you are experiencing a medical emergency or symptoms such as severe chest pain, difficulty breathing, signs of a stroke, or another potentially life-threatening condition, call 911 or seek emergency medical care immediately.
References
American Society of Hematology. (2019). ASH clinical practice guidelines on sickle cell disease: Cardiopulmonary and kidney disease. American Society of Hematology.
American Society of Hematology. (2020). ASH clinical practice guidelines on sickle cell disease. American Society of Hematology.
American Society of Hematology. (2021). ASH clinical practice guidelines on sickle cell disease: Stem cell transplantation. American Society of Hematology.
Centers for Disease Control and Prevention. (2024). About sickle cell disease. U.S. Department of Health and Human Services.
Charache, S., Terrin, M. L., Moore, R. D., Dover, G. J., Barton, F. B., Eckert, S. V., McMahon, R. P., & Bonds, D. R. (1995). Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. The New England Journal of Medicine, 332(20), 1317–1322. https://doi.org/10.1056/NEJM199505183322001
Chou, S. T., Alsawas, M., Fasano, R. M., Field, J. J., Hendrickson, J. E., Howard, J., Kameka, M., Kwiatkowski, J. L., Pirenne, F., Shi, P. A., Stowell, S. R., Thein, S. L., Westhoff, C. M., Wong, T. E., & Akl, E. A. (2020). American Society of Hematology 2020 guidelines for sickle cell disease: Transfusion support. Blood Advances, 4(2), 327–355. https://doi.org/10.1182/bloodadvances.2019001143
Frangoul, H., Locatelli, F., Sharma, A., et al. (2024). Exagamglogene autotemcel for severe sickle cell disease. The New England Journal of Medicine, 390, 1649–1662. https://doi.org/10.1056/NEJMoa2309676
Kato, G. J., Piel, F. B., Reid, C. D., Gaston, M. H., Ohene-Frempong, K., Krishnamurti, L., Smith, W. R., Panepinto, J. A., Weatherall, D. J., Costa, F. F., & Vichinsky, E. P. (2018). Sickle cell disease. Nature Reviews Disease Primers, 4, Article 18010. https://doi.org/10.1038/nrdp.2018.10
National Heart, Lung, and Blood Institute. (2024). Sickle cell disease. National Institutes of Health.
National Heart, Lung, and Blood Institute. (2026). Sickle Cell Awareness Month. National Institutes of Health.
Steinberg, M. H., Barton, F., Castro, O., Pegelow, C. H., Ballas, S. K., Kutlar, A., Orringer, E., Bellevue, R., Olivieri, N., Eckman, J., Varma, M., Ramirez, G., Adler, B., Smith, W., Carlos, T., Ataga, K., DeCastro, L., Bigelow, C., Saunthararajah, Y., ... Terrin, M. (2003). Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: Risks and benefits up to 9 years of treatment. JAMA, 289(13), 1645–1651. https://doi.org/10.1001/jama.289.13.1645
U.S. Food and Drug Administration. (2023). FDA approves first gene therapies to treat patients with sickle cell disease.
U.S. Food and Drug Administration. (2026). FDA approves first gene therapy for young children with sickle cell disease.
