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Sickle cell anemia

Also known as Sickle Cell Disorder

Sickle-cell disease (SCD), or sickle-cell anaemia (SCA) or drepanocytosis, is a hereditary blood disorder, characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the haemoglobin gene. Individuals with one copy of the defunct gene display both normal and abnormal haemoglobin. This is an example of codominance.

Source: Wikipedia

What are the symptoms?

Within all the people who go to their doctor with sickle cell anemia, 42% report having ache all over, 37% report having leg pain, and 29% report having fever. The symptoms that are highly suggestive of sickle cell anemia are dry lips, although you may still have sickle cell anemia without those symptoms.


What might my doctor prescribe?

Common Tests and Procedures

Patients with sickle cell anemia often receive hematologic tests, complete blood count, urinalysis, glucose measurement, other diagnostic procedures (interview; evaluation; consultation), hemoglobin a1c measurement, pelvis exam and ultrasonography .

Common Medications

The most commonly prescribed drugs for patients with sickle cell anemia include folic acid, hydroxyurea, penicillin, deferasirox (exjade), deferoxamine, nelfinavir (viracept), pirbuterol (maxair), benzylpenicilloyl polylysine (pre-pen), zidovudine, lamivudine, factor viii, mesna and collagen topical .

Who is at risk?

Groups of people at highest risk for sickle cell anemia include age 5-14 years, age 1-4 years, race/ethnicity = black, age 15-29 years and age < 1 years. On the other hand, age 75+ years and race/ethnicity = white almost never get sickle cell anemia.

Age

< 1 years
2.0x
1-4 years
1.7x
5-14 years
2.8x
15-29 years
1.8x
30-44 years
0.8x
45-59 years
0.3x
60-74 years
0.1x
75+ years
0.0x

Sex

Male
0.9x
Female
1.0x

Race/Ethnicity

Black
4.6x
Hispanic
0.5x
White
0.0x
Other
0.3x
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