Sickle Cell Disease Due To Hb S S Cn381300
An inherited blood disorder causing abnormal red blood cells and chronic complications. Affects people of African and Mediterranean descent. Management includes pain control and preventing complications.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Vaso-occlusive pain crisis
Acute chest syndrome
Splenic sequestration
Priapism
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hydroxyurea Therapy
Hydroxyurea increases fetal hemoglobin production, reducing sickling events and complications. It is a cornerstone disease-modifying treatment for eligible patients.
Chronic Red Blood Cell Exchange Transfusion
Regular blood transfusions reduce HbS percentage and prevent complications in high-risk patients or those with specific indications like stroke prevention.
Additional treatment options
Hydration and Temperature Management
Maintaining adequate hydration and avoiding temperature extremes reduces vaso-occlusive crisis risk. Environmental modifications and fluid intake protocols are essential daily management strategies.
lifestyleFolic Acid Supplementation
Daily folic acid supports increased erythropoiesis demand from chronic hemolysis. Adequate nutrition with micronutrient support is important for overall health.
dietaryPain Crisis Management and Support
Comprehensive pain management protocols address acute vaso-occlusive crises while psychological support helps manage chronic pain burden and disease-related stress.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Sickle Cell Disease Due To Hb S S Cn381300?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
National Heart, Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2020. NHLBI Guidelines. 2021.
Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031.
Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376(5):429-439.
Martinez RM, O'Brien J, Bhatnagar P, et al. Hydroxyurea therapy for sickle cell disease: A systematic review and meta-analysis. Blood. 2020;135(18):1554-1565.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy