Sickle Cell Anemiais a recessive autosomatic genetic blood disorder which involves the abnormality of certain red blood cells to adopt a rigid sickle shape. The abnormality is classified as a disease when it leads to the development of pathological complications that could possibly result in death and other serious complications. The greatest concern for people suffering from sickle cel
Sickle cell disease
l anemia is the reduction of their life span and the high mortality rate in complications.
However, contrary to the widely prevalent notion, not all incidents of sickle-cell disorder are lethal or even dangerous, due to the genetic polymorphism. A person having a sickle-cell gene or a sickle-cell trait may not necessarily be suffering from the disorder.
Sickle cell disease
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Sickle Cell Anemia Cause
Sickle-Cell Anemia is caused by a mutation in the coding for the protein called hemoglobin (HbS). Hemoglobin is a protein that is central to the production of the red blood cells and structurally binds those cells together and is even responsible for their red pigmentation. The red blood cells are usually biconcave in shape and the genetic mutation causes them to be shaped like a sickle or a crescent.
Sickle-Cell Anemia only appears in a child if he or she carries a recessive HbS or sickle cell anemia trait from either parent. The occurrence of that would be one out of four among the children of parents with sickle cell trait due to genetic recombination.
The development of sickle cells complicates the regular blood function of the body as the sickle shaped red blood cells have a shorter life span, which ultimately leads to deficiency in red blood cell count. This condition further leads to anemia or the acidic concentration of the blood.
This change causes the sickling of red blood cells. The red blood cells may become sticky at the ends and start sticking together and dying prematurely. This could lead to several complications.
There are several causes that induce the sickling of red blood cells.
- Deficiency of folic acid
- Acidosis
- Low blood oxygen level
- Dehydration
- Loss of bodily fluids
- Alcohol consumption
- Emotional stress
- Physical exertion
- Toxicity
Sickle Cell Anemia Symptoms
- Pain
- Swollen Hands and Feet
- Necrosis due to Poor Blood Oxygen Supply
- Joint Pain due to necrosis
Sickle Cell Anemia Risk Factors & Occurrence
The occurrence of sickle-cell anemia shows a certain genetic pattern. The incidence of the disease is more common among Africans than any other ethnic community in the world, as well as among the African American community. Africans from the Central African descent have the highest occurrence of sickle-cell anemia.
The condition is also prevalent among Indians, Middle Eastern and East Mediterranean populations. However, an interesting fact in this context is that people with sickle-cell anemia trait were found to more resistant to the deadly malarial parasite, which coincidentally is common in the parts of the world where the genes for this disorder are mostly found.
Sickle Cell Anemia Diagnosis
Sickle cell anemia is diagnosed by carrying out quite a few tests which include:
- Physical examination checking for obvious symptoms and bone deformities
- Reduction in red blood cell count than normal levels
- Presence of the HbS gene
- Full Blood Count Test
- Hemoglobin Electrophoresis
- Test for sickling of red blood cells
- Serum Creatinine
- Serum Potassium
Sickle Cell Anemia Complications
Sickle cell-anemia can give rise to several complications. Some of the most important ones are as follows.
- Stroke
- Silent Stroke
- Dactylitis
- Acute Chest Syndrome
- OPSI (Overwhelming Post-AutoSplenctomy Infection)
- Osteomyelitis or Bone Infection
- Gall Bladder Infection
- Lung Infection
- Urinary Tract Infection
- Hyposplenism
- Pulmonary Hypertension
- Avascular Necrosis
- Acute Papillary Necrosis
- Eye Disorders
- Renal Failure
- Heart Failure
- Organ Damage
- Increased Risk of Severe Bacterial Infections
- Psychological and Cognitive Problems
- Loss of Vision
- Retarded Body Growth
- Delayed Puberty
- Chronic Pain
- Leg Ulcer
- Joint Pain
- Possible death
Sickle Cell Anemia Treatment & Management
The treatment for sickle cell anemia patients is an ongoing process whether or not they are suffering from a serious anemia crisis. Consistent check up and proper healthcare is essential for the general good health of the patient, prevention of crises, curbing the symptoms of the condition and prolonging the life span. Due to recent advances in the treatment of sickle cell anemia, the probability of the lethality of the condition has been greatly reduced.
The precise treatment of sickle cell anemia depends on the particular condition of the patient. If the patient has developed some complication, the treatment is made accordingly. However, there are several measures which can be taken to improve the general health of the patient suffering from sickle cell anemia and help avoid complications.
- Folic Acid Supplements Intake
- Supplementary oxygen supply
- Over the Counter Pain-relief medicine
- Adequate hydration
- Increased fluid intake
- Avoiding physical exertion
- Avoiding mental stress
Sickle Cell Anemia Medication
Medication for sickle cell anemia precisely depends on the condition of the patient. However, the following are the most frequent medication prescribed for sickle cell anemia.
Antibiotics: Antibiotics are administered for the treatment of bacterial infections.
Vaccination: Vaccinations may be administered to prevent or treat infections. Some of the frequently used vaccines for anemia patients include Hib, PCV and PPV.
Hydroxyurea (Hydrea): It helps the production of fetal red blood cells which reduce the sickling of red blood cells and reduces the periodic bouts of pain. However, prolonged administration of this drug could lead to conditions like infections and leukemia.
Deferasirox: The drug helps reduce the clogging of blood vessels with iron build up to prevent necrosis.
Sickle Cell Anemia Transfusion
In cases of severe anemia and loss of red blood cells, blood transfusion may become necessary. However, blood transfusion is not frequently required since anemia can be prevented in most cases.
Bone Marrow Transplant
Successful Bone Marrow Transplant from a healthy donor can help effectively treat sickle cell anemia in children. However, the process is very complicated, potentially dangerous and difficult to carry out due to the unavailability of donors. Furthermore, the patient’s body may not accept the bone marrow transplant.
