Sickle Cell Anemia(SCA)
- Sickle cell disease (SCD) is a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. Babies are born with sickle cell disease when they inherit two abnormal genes (one from each parent). Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.
Epidemiology and burden of Sickle Cell Anemia(SCA)
- SCD affects approximately 100,000 Americans. (https://www.cdc.gov/ncbddd/sicklecell/data.html)
- Estimates suggest that 250,000 children are born annually with sickle cell anemia worldwide (Rev Bras Hematol Hemoter. 2011; 33(1): 49–54.)
- In England, sickle cell disease affects more than 1 in 2000 live births.In the US, 4000 to 5000 pregnancies are at risk for sickle cell disease each year. (http://bestpractice.bmj.com/best-practice/monograph/100/basics/epidemiology.html)
- Around 1.1% of couples worldwide are at risk for having children with a haemoglobin disorder and 2.7 per 1000 conceptions are affected. (http://www.who.int/bulletin/volumes/86/6/06-036673/en/)
Treatment for Sickle Cell Anemia
- There is evidence to suggest that hydroxyurea is effective in decreasing the frequency of pain episodes and other acute complications in adults and children with sickle cell anaemia. (Cochrane Database Syst Rev.2017 Apr 20;4)
- 10-valent Pneumococcal Nontypeable Haemophilus influenzae Protein D Conjugate Vaccine (PHiD-CV) is immunogenic with an acceptable safety profile in children with and without SCD starting vaccination at 8 weeks to 23 months of age. (Pediatr Infect Dis J.2017 May;36(5):e136-e150)
- Simvastatin is effective in reducing vaso-occlusive pain in sickle cell anaemia. (Br J Haematol.2017 May;177(4):620-629)
- Regular blood transfusion (every 3-4 weeks) is recommended. Hydroxycarbamide can reduce the symptoms if life is getting interrupted. For pain paracetamol and ibuprofen can be useful. (http://www.nhs.uk)
Complications of Sickle Cell Anemia
- SCA is correlated with anterior tooth loss (RR = 1.94), anterior spacing (RR = 1.66), overjet (RR = 1.87), anterior crossbite (RR = 1.94), and open bite (RR = 1.94). Thus, SCA is a risk factor for moderate and very severe MO. (Braz Oral Res.2015;29)
- Sickle cell trait is a significant risk factor for secondary hemorrhage, increased intraocular pressure, and permanent visual impairment in children who have traumatic hyphemas following blunt trauma. (Am J Ophthalmol.1997 Jun;123(6):783-90)
- Children with sickle cell disease may have increased risk for certain neurodevelopmental diagnoses based on their disease characteristics and associated comorbidities. (Clin Pediatr (Phila).2015 Oct;54(11):1087-93)
Major Symptoms of Sickle Cell Anemia
- Fatigue and anemia, painful episodes called pain cries which can last for weeks
- Dactylitis (swelling/inflammation of the hands/feet) and Arthritis
- Severe tiredness and short of breath
- Splenic Sequestration (sudden pooling of blood in the spleen) and Liver Congestion
- Leg Ulcers, eye damage, high risk of serious infections
- Aseptic Necrosisand Bone Infarcts (death of portions of bone)
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