About
Sickle cell disease (Hemoglobin SS Disease) is a group of inherited red blood cell disorders. In a healthy person, red blood cells are a round and they move through the body’s blood vessels carrying oxygen to all parts of the body. In someone with Sickle Cell Disease, the blood cells become sticky and are shaped like the letter C; called a crescent or sickle shape. These sickle shaped cells die early, causing a constant shortage of blood cells. When they travel through small blood vessels, they can get stuck and clog the blood flow. This can cause severe pain, infection, and other serious problems.
Most Common Types of Sickle Cell Disease:
Hemoglobin SS
People who have this type inherit two sickle cell genes (S), one from each parent. This is usually the most severe form of this disease.
Hemoglobin SC
People who have this type inherit an (S) gene from one parent, and one gene for hemoglobin (C) from the other parent. This is usually a milder form of the disease.
Hemoglobin S beta thalassemia
People who have this type inherit one sickle cell (S) gene from one parent and one gene for beta thalassemia from the other parent. There are two types of thalassemia: “zero” and “plus”. The “zero” type of thalassemia is usually a more severe form of the disease.
Condition Type:
Core Conditions
Frequency:
Sickle cell disease is the most common inherited blood disorder in the world. It affects approximately 100,000 Americans. It commonly affects people whose families come from Africa; Spanish-speaking regions in South America, Central America and parts of the Caribbean; the Arabian Peninsula; India; and the Mediterranean countries, such as Turkey, Greece, and Italy.
More Information for Parents:
Also known as:
- Hemoglobin SS Disease
- Hemoglobin SC disease
- SCD
- Sickle cell disorders
- Sickle Cell Anemia
- Sickle beta thalassemia
- Beta Thalassemia
- FS, FSC or FSA
Core Conditions
- Propionic Acidemia (PROP)
- Methylmalonic Acidemia (Methylmalonyl-CoA Mutase Deficiency) (MUT)
- Methylmalonic Acidemia (Cobalamin Conditions)
- Isovaleric Acidemia (IVA)
- 3-Methylcrotonyl-CoA Carboxylase Deficiency (3-MCC)
- 3-Hydroxy-3-Methylglutaric Aciduria (HMG)
- Holocarboxylase Synthetase Deficiency (MCD)
- Beta-Ketothiolase Deficiency (BKT)
- Glutaric Acidemia, Type I (GA-1)
- Mucopolysaccharidosis, Type II (MPS II)
- Biotinidase Deficiency (BIOT)
- Critical Congenital Heart Disease (CCHD)
- Cystic Fibrosis (CF)
- Classic Galactosemia (GALT)
- Hearing Loss or Varying Hearing Levels
- Severe Combined Immunodeficiency (SCID)
- X-Linked Adrenoleukodystrophy (X-ALD)
- Pompe
- Spinal Muscular Atrophy (SMA)
- Mucopolysaccharidosis, Type I (MPS I)
Secondary
- Citrullinemia, Type II (CIT II)
- Hypermethioninemia (MET)
- Benign Hyperphenylalaninemia (H-PHE)
- Biopterin Defect in Cofactor Biosynthesis (BIOPT-BS)
- Biopterin Defect in Cofactor Regeneration (BIOPT-REG)
- Ornithine Transcarbamylase Deficiency (OTC)
- Carbamoyl Phosphate Synthetase Deficiency (CPS)
- Tyrosinemia, Type II (TYR II)
- Tyrosinemia, Type III (TYR III)