Sickle Cell Disease Quiz
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Frequent body pain
Fatigue and weakness
Pale skin
Yellow skin and eyes
Painful and swollen hands
Painful and swollen feet
Pain in arms and legs
Stiff and swollen fingers
Unexplained abdominal pain
Dizziness
Back pain
Joint pain
Not seeing your symptoms? No worries!
What is Sickle Cell Disease?
Sickle cell anemia is a genetic blood disorder where the body produces abnormal hemoglobin, causing red blood cells to take on a sickle shape instead of their normal round form. These distorted cells can block blood flow, leading to pain, organ damage, and an increased risk of infection.
Typical Symptoms of Sickle Cell Disease
- Whole body pain
- Weakness or paralysis on one side of your body
- Yellowish eyes or skin
- Dizziness
- Headache
- Pain in my penis
- Finger pain
- Bluish or pale skin
Diagnostic Questions for Sickle Cell Disease
Your doctor may ask these questions to check for this disease:
- Do you have pain all over your body?
- Do you have weakness or paralysis on one side of your body?
- Have you noticed any yellowing of your eyes or skin?
- Do you feel dizzy?
- Do you have a headache?
Treatment of Sickle Cell Disease
Treatment for sickle cell disease focuses on managing symptoms and preventing complications. Pain management, proper hydration, and blood transfusions are used to address severe anemia or blockages. Medications like hydroxyurea may be prescribed to reduce the frequency of painful episodes, and newer therapies aim to address the underlying cause of the disease.
Reviewed By:
Eisaku Kamakura, MD (Pulmonology)
Dr. Kamakura graduated from the Tokyo Medical and Dental University, School of Dentistry, and the Niigata University School of Medicine. He trained at Yokosuka Kyosai Hospital and held positions in the Respiratory Medicine departments at Yokosuka Kyosai Hospital, Tokyo Medical and Dental University, Ome City General Hospital, and Musashino Red Cross Hospital. In 2021, he became the specially appointed assistant professor at the Department of General Medicine, Niigata University School of Medicine.
Kenji Taylor, MD, MSc (Family Medicine, Primary Care)
Dr. Taylor is a Japanese-African American physician who grew up and was educated in the United States but spent a considerable amount of time in Japan as a college student, working professional and now father of three. After graduating from Brown, he worked in finance first before attending medical school at Penn. He then completed a fellowship with the Centers for Disease Control before going on to specialize in Family and Community Medicine at the University of California, San Francisco (UCSF) where he was also a chief resident. After a faculty position at Stanford, he moved with his family to Japan where he continues to see families on a military base outside of Tokyo, teach Japanese residents and serve remotely as a medical director for Roots Community Health Center. He also enjoys editing and writing podcast summaries for Hippo Education.
Content updated on Jan 23, 2025
Following the Medical Content Editorial Policy
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- My whole body aches and I feel weak
- Body aches and chills
- White of the eyes are yellow
- Joint pain and stiffness all over body
- Whole body aches no fever
- Have a headache
- My whole body hurts
- Icterus
- Whole body pain
- Dizziness multiple times
- Pulsating headache
- Off-balance like floating
- Headache on the side of my head
- Spinning feeling
- Headache over the entire head
See full list
Symptoms Related to Sickle Cell Disease
Diseases Related to Sickle Cell Disease
FAQs
Q.
Is the Pain Unending? Why Sickle Cell Disease Hurts & Your Medical Next Steps
A.
Sickle cell disease pain happens when sickled red blood cells block blood flow and damage tissues, triggering sudden pain crises and sometimes chronic pain; it is not hopeless and can often be reduced with hydroxyurea, newer disease modifying medicines, transfusions, and in select cases transplant or gene therapy. There are several factors to consider; see below to understand more. Your next steps include partnering with a hematologist, following a prevention and pain plan, and seeking urgent care for chest pain, trouble breathing, high fever, new weakness or speech changes, severe abdominal swelling, or a painful erection lasting over 4 hours, with more details that could change your decisions outlined below.
References:
* Darbari DS, Kasteleijn-Nolst Trenité D, Piel FB. Pathophysiology and management of pain in sickle cell disease. Nat Rev Rheumatol. 2020 Apr;16(4):213-228. doi: 10.1038/s41584-020-0381-8. PMID: 32170154; PMCID: PMC7464082.
* Brandow AM, Dampier CD. Mechanisms of Pain in Sickle Cell Disease. Hematol Oncol Clin North Am. 2020 Jun;34(3):365-381. doi: 10.1016/j.hoc.2020.01.006. Epub 2020 Apr 14. PMID: 32360334.
* Ezenwaji AK, Dike AN, Dike AN, Ezenwaji AI, Ihegihu CG. Clinical management of painful episodes in sickle cell disease: a review of the evidence. Ther Clin Risk Manag. 2023 Jan 24;19:75-87. doi: 10.2147/TCRM.S394209. PMID: 36710775; PMCID: PMC9884581.
* Scurlock C, Barakat LP. Management of chronic pain in adult sickle cell disease: a systematic review. J Pain Symptom Manage. 2020 Dec;60(6):1276-1290.e2. doi: 10.1016/j.jpainsymman.2020.06.014. Epub 2020 Jun 22. PMID: 32585640; PMCID: PMC7708688.
* Yawn BP, Sun S, Golding J, Abah I, Eguakun A, Adedokun B, Iheanacho N. Advances in the treatment of sickle cell disease: from novel drug development to gene therapy. BMC Med. 2021 Jul 26;19(1):164. doi: 10.1186/s12916-021-02031-6. PMID: 34311890; PMCID: PMC8313437.
Q.
Worried About Warfarin? Why Your Blood Is Reacting and Medically Approved Next Steps
A.
If you are on warfarin and notice bruising, bleeding, or off-target INR results, it usually means the balance needs fine-tuning, not that the drug is failing. Triggers often include changes in vitamin K intake, new medications or supplements, illness, alcohol, or missed or extra doses, and the approved next steps are to avoid self adjustments, get your INR checked promptly, review all meds and diet with your clinician, and seek urgent care for red flag bleeding or clot symptoms. There are several factors to consider, including how clinicians adjust dosing, when vitamin K may be used, and whether a DOAC alternative fits your condition; see the complete guidance below to understand more and choose the safest next steps.
References:
* Patel P, Gandhi B, Gandhi A, Gandhi J. Warfarin Therapy: A Review of its Mechanism of Action, Indications, and Adverse Effects. Cureus. 2023 Dec 13;15(12):e49999.
* Witt DM, Nieuwlaat R, Clark NP, Hylek EM, Crowther MA, Douketis JD, Ferreira MJ, Garcia DA, Greenblatt KL, Kahn SR, Lane DA, Lee AY, Linkins LA, MacGregor EA, McBane RD, McLeod AJ, Mullins A, Nafziger AN, Sansom BI, Schunemann HJ, Thornton LM, Van Cott EM, Waite LH, Wortman B, Bui CJ, Guyatt G. American Society of Hematology 2024 guidelines for the management of venous thromboembolism: Antithrombotic therapy for VTE disease. Blood Adv. 2024 Mar 12;9(5):989-1065.
* Zhou Y, Deng Z, Li S, He C, Zhang M, Deng H, Zheng T. Anticoagulant-Associated Bleeding: Classification, Prevention, and Management. J Thromb Thrombolysis. 2021 May;51(4):1043-1055.
* Rask-Andersen M, Olsson B, Eriksson M, Larsson J, Fredriksson R, Schiöth HB. Pharmacogenetics of Warfarin: Current Status and Future Perspectives. Trends Pharmacol Sci. 2018 Sep;39(9):839-851.
* Holbrook AM, Haire B, Hagemann T, Dager WE. Update on Anticoagulation for the Generalist. J Hosp Med. 2018 Jun 1;13(6):408-417.
Q.
Blood Too Thick? Why Polycythemia Vera Happens & Medical Next Steps
A.
Polycythemia vera is a myeloproliferative blood cancer, usually driven by a JAK2 mutation, that makes too many red blood cells so blood becomes thick and more likely to clot, raising risks of stroke and heart attack. Diagnosis is confirmed with targeted tests like JAK2, EPO, and sometimes bone marrow, and treatment typically includes phlebotomy, low-dose aspirin, and cytoreductive medicines, with urgent evaluation for sudden chest pain, weakness, or speech or vision changes. There are several factors to consider, including other causes of high red counts and how your clotting risk shapes therapy; see important details below to guide your next steps.
References:
* Spivak JL. Polycythemia Vera: 2023 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 Jun;98(6):1001-1015. doi: 10.1002/ajh.26888. Epub 2023 Feb 15. PMID: 36792942.
* Vainchenker W, Constantinescu SN, Bourrillou R, Villeval JL. The molecular pathogenesis of polycythemia vera and its therapeutic implications. Haematologica. 2020 Jan;105(1):15-28. doi: 10.3324/haematol.2019.231264. Epub 2019 Sep 19. PMID: 31537617.
* Barbui T, Barosi G, Cervantes F, Vannucchi AM, Tefferi A. Management of polycythemia vera: a practical approach. Blood. 2021 Jul 15;138(2):98-111. doi: 10.1182/blood.2020008061. PMID: 33735740.
* Nangalia V, Eder V, Kent DG, Vannucchi AM, Harrison CN. Diagnosis, Risk Stratification and Management of Polycythemia Vera: An Update on the Evolving Landscape. J Clin Med. 2023 Apr 19;12(8):3017. doi: 10.3390/jcm12083017. PMID: 37108928; PMCID: PMC10142835.
* Sridharan N, Bilen M, Mesa R, Mascarenhas J. Therapeutic Approaches for Polycythemia Vera: An Overview of Current and Emerging Options. Cancers (Basel). 2023 Feb 1;15(3):853. doi: 10.3390/cancers15030853. PMID: 36765799; PMCID: PMC9913495.
Q.
Sickle Cell Pain? Why Your Blood Is Clumping & Medical Next Steps
A.
Sickle cell pain happens when stiff, sickle shaped red blood cells stick together and block small blood vessels, cutting off oxygen to tissues and causing a vaso occlusive crisis. Seek urgent care for chest pain, trouble breathing, fever over 101 F, severe headache, weakness, confusion, abdominal swelling, or a prolonged erection, and work with a hematologist on hydration, avoiding triggers, prompt infection treatment, pain control, disease modifying medicines, transfusions, and transplant options. There are several factors to consider for your next steps, and key details that can change what you do next, so see the complete guidance below.
References:
* Gauthier, S. M., & Hémond, B. C. (2017). Pathophysiology of Vaso-Occlusion in Sickle Cell Disease and New Therapeutic Approaches. *Frontiers in Physiology*, *8*, 178.
* Olanrewaju, T., & Fasakin, O. O. (2021). Management of sickle cell disease: a review of the latest evidence. *Therapeutic Advances in Hematology*, *12*, 2040620721990497.
* Glassberg, J., & Smith, W. R. (2018). Acute Pain Management in Sickle Cell Disease. *Current Pain and Headache Reports*, *22*(8), 53.
* Ma, L., & Grosse-Wilde, P. E. (2020). Emerging Therapies for the Management of Vaso-Occlusive Crisis in Sickle Cell Disease. *Therapeutic Advances in Hematology*, *11*, 2040620720942503.
* Dampier, C., Palermo, T. M., & Smith, W. R. (2016). Chronic Pain in Sickle Cell Disease: A Clinical and Research Update. *The Journal of Pain*, *17*(11), 1133–1145.
Q.
Confused by Blood Types? Why Your Biology Matters & Medically Approved Next Steps
A.
Your blood type affects safe transfusions, pregnancy via the Rh factor, emergency care, and small differences in risks like bleeding or clots. There are several factors to consider, so see below to understand more. Next steps: confirm your type through your doctor, medical records, or a blood donation; tell your prenatal team if pregnant; consider a sickle cell disease symptom check if relevant to your background or symptoms; and seek care promptly for red flags like unexplained fatigue, pain crises, unusual bleeding, shortness of breath, or jaundice.
References:
* Franchini, M., & Favaloro, E. J. (2018). ABO Blood Group: A Review of the Genetics, Pathophysiology, and Clinical Implications. *Seminars in Thrombosis and Hemostasis*, *44*(4), 319–326. doi: 10.1055/s-0037-1607310
* Wagner, T. (2022). Rh Blood Group System: A Comprehensive Review. *Journal of Clinical Laboratory Analysis*, *36*(7), e24584. doi: 10.1002/jcla.24584
* Roback, J. D., & Grossman, B. J. (2020). Transfusion Medicine. *New England Journal of Medicine*, *383*(15), 1475–1485. doi: 10.1056/NEJMra1816301
* Lomas-Francis, C., & Zantek, N. D. (2022). Update in Transfusion Medicine: Recent Advancements and Best Practices. *Clinics in Laboratory Medicine*, *42*(1), 1–17. doi: 10.1016/j.cll.2021.10.001
* Storry, J. R., & Reid, M. E. (2020). The blood group antigen factsbook: A comprehensive reference book for clinicians, scientists, and students. *Transfusion Medicine Reviews*, *34*(2), 116–123. doi: 10.1016/j.tmrv.2020.01.006
Q.
Sickle Cell Pain? Why Your Blood is Sickling & Medically Approved Next Steps
A.
Sickle cell pain happens when inherited hemoglobin S makes red blood cells stiff and crescent shaped, so they block tiny blood vessels and starve tissues of oxygen, causing sudden, severe crises; triggers often include dehydration, cold, infection, stress, high altitude, and overexertion. Medically approved next steps include working with a hematologist, considering proven medicines like hydroxyurea, L-glutamine, crizanlizumab, and voxelotor, preventing crises with hydration and vaccines, managing pain safely, and knowing emergency signs such as chest pain or stroke symptoms. There are several factors to consider, so see the complete guidance below for important details that can shape your personal plan, including transfusions, transplant, and emerging gene therapy.
References:
* Stuart M. C. (2018). Pathophysiology of Vaso-Occlusion in Sickle Cell Disease. *Blood*, 132(11), 1157-1166.
* Manwani D, Quinn CT. (2014). Vaso-occlusive pain in sickle cell disease: biology, genetics, and therapy. *Current Opinion in Hematology*, 21(3), 220-226.
* Brandow AM, et al. (2020). American Society of Hematology 2020 guidelines for the management of sickle cell disease: vaso-occlusive episodes. *Blood Advances*, 4(13), 3044-3079.
* Tisdale JF, et al. (2023). New and emerging treatments for sickle cell disease. *American Journal of Hematology*, 98(7), E205-E209.
* Smith WR, et al. (2020). Management of acute pain in sickle cell disease. *American Journal of Hematology*, 95(5), 570-580.
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Siddiqi AE, Jordan LB, Parker CS. Sickle cell disease--the American saga. Ethn Dis. 2013 Spring;23(2):245-8. PMID: 23530308; PMCID: PMC4517422.
https://pmc.ncbi.nlm.nih.gov/articles/PMC4517422/Elendu C, Amaechi DC, Alakwe-Ojimba CE, Elendu TC, Elendu RC, Ayabazu CP, Aina TO, Aborisade O, Adenikinju JS. Understanding Sickle cell disease: Causes, symptoms, and treatment options. Medicine (Baltimore). 2023 Sep 22;102(38):e35237. doi: 10.1097/MD.0000000000035237. PMID: 37746969; PMCID: PMC10519513.
https://pmc.ncbi.nlm.nih.gov/articles/PMC10519513/Brandow, A.M., Liem, R.I. Advances in the diagnosis and treatment of sickle cell disease. J Hematol Oncol 15, 20 (2022).
https://jhoonline.biomedcentral.com/articles/10.1186/s13045-022-01237-z