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Sickle-cell anemia?

What is Sickle Cell Disease?

Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.

When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for sickle cell disease. Hemoglobin – is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body. Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them. People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S (S stands for sickle). Red blood cells containing mostly hemoglobin S do not live as long as normal red blood cells (normally about 16 days). They also become stiff, distorted in shape and have difficulty passing through the body’s small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease.

Types of Sickle Cell Disease.

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC)
Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

Causes of Sickle Cell Anemia

Cause of Sickle Cell Anemia: Chronic hemolytic anemia resulting from destruction of red blood cells (RBCs) because of a genetic defect in these RBCs. What causes Sickle Cell Anemia?: Related information for Sickle Cell Anemia causes, as with all medical conditions, there may be many causal factors. Further relevant information on causes of Sickle Cell Anemia may be found in the risk factors for Sickle Cell Anemia, medications that may cause Sickle Cell Anemia, contagiousness for Sickle Cell Anemia, genetics of Sickle Cell Anemia, and underlying causes of Sickle Cell Anemia.

Affected Regions

	Very high risk regions: Africa-Tropical area- and India.
	High risk regions: West Indies, South America-Brazil.
	Medium risk regions: North America, Greece.
	Low risk regions: Portugal, Turkey, Israel.

Symptoms of Sickle Cell Anemia

General information about signs and symptoms of Sickle Cell Anemia: The symptom information on this page attempts to provide a list of some possible signs and symptoms of Sickle Cell Anemia. This signs and symptom information for Sickle Cell Anemia has been gathered from various sources, may not be fully accurate, and may not be the full list of Sickle Cell Anemia signs or Sickle Cell Anemia symptoms. Furthermore, signs and symptoms of Sickle Cell Anemia may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed Sickle Cell Anemia symptoms.

List of symptoms of Sickle Cell Anemia: The list of signs and symptoms mentioned in various sources for Sickle Cell Anemia includes those listed below. Note that Sickle Cell Anemia symptoms usually refers to various symptoms known to a patient, but the phrase Sickle Cell Anemia signs may refer to those signs only noticable by a doctor:

- Common early symptoms : Anemia; Pallor; Shortness of breath; Failure to thrive; Recurrent infections; Pneumococcal infections; Fever; Jaundice - see also symptoms of jaundice ; Yellow skin; Yellow eyes;Delayed growth...

- Chronic anemia: Pain; Painful crisis - a sudden attack of pain, usually in joints or bones; Joint pain; Bone pain; Foot pain; Hand pain; Chest pain; Abdominal pain; Vision problems; Retina damage; Delayed puberty; Small stature; Vomiting; Skin ulcers; Ankle ulcers; Enlarged liver; Enlarged spleen; Resistance to malaria - people with SCA are resistent to malaria, which is believed to be an evoluationary advantage in Africa, possibly explaining the higher prevalence of SCA in people of African descent.

Symptoms of Sickle Cell Anemia: A painful crisis is a sudden attack of pain, which may be mild or extremely severe, often occurring in bones and joints in adults. For this study, which was limited to patients with moderately severe disease (at least 3 attacks/year), a crisis was a visit to a medical facility for pain not due to another disease, lasting at least 4 hours, and treated with injections of a narcotic pain-killer.

Treatments for Sickle-Cell Anemia

Treatment list for Sickle Cell Anemia: The list of treatments mentioned in various sources for Sickle Cell Anemia includes the following list. Always seek professional medical advice about any treatment or change in treatment plans.

1-Preventive antibiotics: The use of antibiotics to prevent bacterial infection or complications

Count: a Preventive antibiotic is listed as a: treatment for 11 conditions; alternative treatment for 11 conditions; preventive treatment for 6 conditions; research treatment for 0 conditions.

Preventive antibiotics are a type of: Antibiotics (190)

Treatments: all treatments.

Preventive antibiotics as a treatment: The following list of conditions has 'Preventive antibiotics' or similar listed as a treatment in our database:

A- Acute rheumatic fever = preventive antibiotics

B- Burns = preventive antibiotics

C- Chronic Granulomatous Disease = preventive oral antibiotics

F- Frostbite = preventive antibiotics

H- Human bite = preventive antibiotics

L- Lyme disease = preventive antibiotics after tick bite

M- Mitral regurgitation = preventive antibiotics

· Mitral-valve prolapse = preventive antibiotics

R- Rheumatic fever = preventive antibiotics

· Rheumatic heart disease = preventive antibiotics.

Sickle-Cell Anemia = preventive antibiotics.

Preventive penicillin - especially for newborns, infants and children with SCA; Pain crisis treatments; Pain-killing drugs; Fluids; Blood transfusions; Various treatments for complications of SCA ; Eye surgery; Laser coagulation; Gallstone removal; Hip joint replacement; Lifestyle treatments; Avoiding infections; Good hygiene; Healthy diet; Bed rest; Genetic counselling - for affected patients starting a family or parents of infected children wishing to have more children.

Treatments of Sickle Cell Anemia discussion

In 1995, an NHLBI clinical trial of hydroxyurea in adults over the age of 18 demonstrated, for the first time, an effective therapy for reducing painful episodes in severely affected adults with sickle cell anemia. Although hydroxyurea has been approved by the FDA for adults with sickle cell disease, there is concern about the potential toxicity of hydroxyurea in children, and its effects on growth and development. Therefore, hydroxyurea is not recommended for children at this time. However, clinicians are interested in determining its benefits, if any, in children particularly in preventing organ damage and improving long-term survival. Sickle cell anemia patients treated with HU: had about half as many painful crises; had longer periods between crises; had fewer attacks of acute chest syndrome (a pneumonia-like problem); had to be hospitalized less often; had to be transfused less often; and had no significant bad effects from treatment. Most HU treated patients did continue to have some crises and all had to get their blood counts checked every 2-4 weeks. Taking capsules during an acute attack did not stop the attacks. HU works slowly, and a decrease in the frequency of crises was not seen until about 6-8 weeks after starting. Good effects of treatment only last while patients continue to take their capsules.

Cure Research for Sickle Cell Anemia

Cure Research list for Sickle Cell Anemia: The list of research areas and treatments under analysis mentioned in various sources for Sickle Cell Anemia includes:

- Fetal hemoglobin stimulants; Hydroxyurea (HU) - a promising treatment for SCA ; Hydroxyurea and erythropoietin combination; Butyrate; Clotrimazole; Bone marrow transplantation; Gene therapy.

Cure Research discussion for Sickle Cell Anemia

A Phase I/II study ( PED-HUG) was supported by the NHLBI to determine the therapeutic dose of hydroxyurea in children with 3 or more painful episodes per year and to identify any short-term toxicities.

Several other drugs under investigation (erythropoietin, butyrate and related compounds) may work together with hydroxyurea to make it more effective, but those studies are still in progress. Bone marrow transplantation can really cure the disease, but the risks are high, and only a small number of young patients are good candidates for that form of experimental therapy. Some day gene therapy may provide a safe and effective cure, and that will be some years in the future.

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