What Do You Understand by Sickle Cell Anemia?
Sickle cell anemia is a severe hereditary type of anemia in which mutated form of hemoglobin alters the individual red blood cells to form a crescent shape at low oxygen levels. Normal blood cells are round and flexible enough to move easily across our body through the veins and arteries. When one has sickle cell anemia the red blood cells are sticky, rigid and crescent-shaped, these features make them get stuck in the blood vessels or block the small capillaries in various organs. This condition has no cure for most people. Owing to this reason there are a variety of medications to avoid sickle cell anemia.
Research conducted shows that this condition is more common in those people of African descents. Since it is common in African descents, people from these regions should go for a frequent blood test to check for hemoglobins.
Symptoms of Sickle Cell Anemia
The symptoms and signs of this condition, vary from one individual to individual and may change over time. These signs usually show up at a very young age as early as 4 months old. These warning signs include:
- Babies may show fussiness
- Bedwetting in babies due to kidney problems
- An individual may experience irritability and excessive fatigue
- Jaundice, this condition individual may have yellowing of the eyes and skin
- An individual may experience chest, back, legs and arm pain
- Individual hands and feet may swell
- Frequents infection is common for an individual with this condition
Types of Sickle Cell Disease
There are four main types of sickle cell anemia caused by a different mutation in these genes. It should be recalled that Hemoglobin is a protein found in the red blood cell that is responsible in a carriage of oxygen across the body. It has two beta chains and two alpha chains. These types include:
Hemoglobin SS Disease
This is the most common type of sickle cell anemia. It occurs when individual inherit copies of the hemoglobin S gene from the parent (both) Individual with this type of sickle cell anemia experience the worst symptoms.
Hemoglobin SC Disease
This type occurs when individual inherit hemoglobin C gene from one parent and the other parent contribute hemoglobin s gene. An individual with a type of sickle cell disease will experience the same symptoms as an individual with hemoglobin SS though it’s less severe.
Hemoglobin SB+ (Beta) Thalassemia
This one affects beta-globin gene production. The individual with these type will have reduced size of red blood cell because less beta protein is produced. If this condition happens to be inherited with hemoglobin S gene the symptoms will be less severe
Hemoglobin SB 0 Thalassemia
This type is similar to hemoglobin SS anemia in terms of symptoms experienced. This type is more severe.
Sickle Cell Trait
An individual with these type only inherits hemoglobin S (mutated gene) from one parent. They don’t show any symptoms.
Sickle Cell Anemia Diagnosis
In developed countries like the United States, all newborns are screened for sickle cell disease, they go as far as testing the fetus. They use amniotic fluid to test for this condition.
For children and adults, these procedures may be used in the diagnosis of sickle cell anemia.
The doctor may test for sickle cell anemia if an individual portrays these symptoms, for example:
● Ulcers of the legs
● Heart problems
● Respiratory infections
● Severe pain in the bones
● Painful enlargement of the spleen
Several blood tests can be carried to check for sickle cell anemia. Blood counts can be done to check the level of abnormal hemoglobin in the blood in the range of 6 to 8 grams per deciliter. Also, sickle solubility test can be done to check the presence of hemoglobin in the blood
After one is suspected to have sickle cell disease Hb electrophoresis can be done to confirm this. This test measures the different types of Hb in the individual blood.
Sickle Cell Anemia Medications
Sickle cell anemia is inherited condition this means that an individual is born already with this condition. The only thing which can be done is to prevent infection, relieve pain, prevent organ damage, treat anemia and lastly control complication accompanied by this condition.
|Treating Pain||Narcotics, Acetaminophen and Nonsteroidal Anti-inflammatory Drugs (NSAIDs)|
|Preventing Infection||For Children – Antibiotics
For Adult – Annual Flu Shots and Vaccination
|Other Medicines||Acetaminophen or Paracetamol, Vitamin E Systemic, Glutamine Systemic|
An individual may experience either mild or severe pain. Mild pain can be treated with over the counter medicine and heating pad while for a severe case the individual can be taken to a hospital. Common medicines used in treating pain include narcotics, acetaminophen, and nonsteroidal anti-inflammatory drugs (NSAIDs). Mild pain can be treated by mere use of acetaminophen while severe case narcotics are used.
For people who experience painful sickle cell anemia, hydroxyurea can be used to reduce the pain crises. It should be noted that this drug is used to prevent pain not to treat it when it occurs. Hydroxyurea works by stimulating the production of fetal hemoglobin which helps to prevent the formation of sickle cells. (Fetal hemoglobin is a type of hemoglobin found especially in a newborn)
The disadvantage of this drug is that it increases the risk of infection, long-term uses are likely to cause a problem in the user in later life. Pregnant women are discouraged from taking this drug.
People with this condition often get a bacterial infection, which can be treated or prevented. In babies and young children, theses treatment can be applied. A daily dose of penicillin for the children between 2 months of age to 5 years old
They can also get vaccination flu shots and vaccinations against streptococcus pneumonia. Taking antibiotic by children helps to prevent infections like pneumonia which can be life-threatening to an infant with sickle cell anemia.
For adults with this condition, they can be given annual flu shots and vaccination against pneumococcal infections. Also, adults whose spleen are removed required to take penicillin throughout their life.
People with sickle cell anemia have a variety of complications, including acute chest syndrome (lung crises), eye damage, gallstones, pulmonary hypertension and leg ulcers that don’t heal. Blood transfusions are the most common treatment used to manage sickle cell anemia. Hydroxyurea can also be used in the treatment of painful crises before transfusion is conducted.
Sometimes red blood transfusion can be done, through this, normal blood cells in circulation is increased. The red blood cells are always extracted from donated blood then it’s intravenously given to the patient with this condition.
This method comes with its own risks like excess iron buildup and infection. Excess iron in the blood can damage the liver, heart and other organs. Patients who undergo frequent red blood transfusion may require medication to reduce the level of iron in the blood.
Bone Marrow Transplant(Stem Cell Transplant)
This is the replacement of bone marrow affected with sickle cell anemia with those of healthy bone marrow. For one to undergo this process they are supposed to be subjected to radiation destroy their bone marrow stem cells then healthy stem cells are injected intravenously into the patient bloodstream.
Other Medicines Used to Treat Sickle Cell Anemia Include
Acetaminophen or paracetamol
Vitamin e systemic
Apart from taking medicine, there are other lifestyle and home remedies patient may follow to help avoid complication of this condition. One can choose to take folic acid supplements daily and also prefer a healthy diet.
Who is at Risk for Sickle Cell Anemia?
Children are more prone to this condition if both parents are carriers of sickle cell trait. A blood test known as hemoglobin electrophoresis can be done on children to determine which type of sickle cell anemia they have.
Regions in which endemic malaria is common, people in those areas are like carriers. This region includes:
- The Mediterranean
- Saudi Arabia
Why is Sickle Cell Anemia Classified as A Recessive Disorder?
Sickle cell anemia is inherited in an incomplete dominance fashion. The disease is transmitted through a recessive gene that leads to the synthesis of abnormal hemoglobin. A normal adult human contains hemoglobin in their red blood cells and have the gene type HBA HBA in sickle cell anemia patients, hemoglobin A is entirely replaced by hemoglobin S. Such patients have genotype HBS HBS and this why this condition is classified as a recessive disorder.
Research on gene therapy, bone marrow transplants and new medicines for this condition is ongoing. It is hoped that this will provide better medications to avoid sickle cell anemia effects. However, when taking the available medicine the sickle cell person can still lead relatively normal life.
Medically Reviewed By