This article is for information purposes only. It is not a substitute for medical advice or treatment. Seek medical care for your treatment.
What is sickle cell disease?
Sickle cell anemia is a genetic blood condition called sickle cell disease. Your red blood cells are affected by SCD which mutates them from pliable round discs to rigid, gooey sickled cells. Red blood cells are prevented from fulfilling their duty of transporting oxygen throughout your body by sickled cells. Healthy red blood cells have a longer lifespan than sickled cells. Therefore, you experience anemia and it is called sickle cell anemia in which you don’t have plenty of healthy red blood cells.
Sickled cells can clump and obstruct blood flow, which makes it difficult for blood to carry oxygen throughout your body which can leave you fatigued, breathless, in pain and can make you vulnerable to infections. Your different parts and organs of your body are affected by sickle cell anemia.
Causes of sickle cell disease:
Sickle cell anemia is a genetic condition that is passed down from one’s biological parents. The gene that aids in producing healthy red blood cells mutates or changes in sickle cell anemia. Sickle cell anemia affects people who receive the defective hemoglobin protein gene from both biological parents. The sickle cell trait is present in people who receive the mutant gene from one biological parent.
Who is affected by SCD?
If both parents have the sickle cell trait, the risk of sickle cell illness in the child is higher. A blood test called hemoglobin electrophoresis can determine the specific kind you have.
The majority of the 100,000 Americans with sickle cell disease are African Americans. People with endemic malaria, including those with Mediterranean, African, Southern European, Middle Eastern, or Asian Indian ancestry, may also experience problems.
Symptoms of sickle cell disease:
Symptoms of sickle cell disease develop around the 6 months of age and they differ from person to person. Some of the symptoms are:
- Pain crises
- Frequent infections
- Inflammation of hands and feet
- Slow growth or puberty
- Vision problems
Treatment for sickle cell disease:
SCD requires prolong treatment. Treatment of sickle cell anemia is typically to prevent pain crisis, relieve symptoms and avert any dire consequences. Blood transfusions and medicines are possible forms of treatment. A stem cell transplant may be able to reverse the condition in certain children and teenagers.
- Hydroxyurea: Daily hydroxyurea usage lowers the frequency of uncomfortable crises and may lessen the requirement for hospital stays and blood transfusions. But it might also make infections more likely. If you are pregnant, avoid taking the medication.
- L-glutamine oral powder: This medication has just got FDA approval to treat sickle cell anemia. It helps to lessen how often people experience pain crises.
- Crizanlizumab: This medication, administered intravenously, can assist in lowering the frequency of pain crises in both adults and children older than 16. Nausea, joint discomfort, back pain, and fever are possible side effects.
- Voxelotor: This medication is used to treat adults and children over the age of 12 who have sickle cell disease. This medication, when taken orally, can enhance blood flow throughout the body and reduce the risk of anemia. Headache, nausea, diarrhea, exhaustion, rash, and fever are examples of potential side effects.
- Pain relieving medicines: during sickle cell pain crises your doctor might suggest some narcotics to avert pain and discomfort.
- Averting infections:
Penicillin may be administered to kids with sickle cell anemia between the ages of 2 months and at least 5 years old. By doing this, illnesses like pneumonia that can be fatal to kids with sickle cell anemia are reduced.
If they’ve undergone pneumonia or spleen surgery, adults with sickle cell anemia may need to take penicillin for the rest of their lives.
All children should receive their first round of vaccines to help avoid sickness. Due to the potential severity of their infections, they are particularly more crucial for children with sickle cell anemia. Your kid’s doctor should make sure that your child obtains the full complement of recommended childhood immunizations, including those for hepatitis B, pneumonia, meningitis, and meningococcal disease, as well as an annual flu shot. Vaccinations are crucial for people with sickle cell anemia.
- Surgical and other procedures:
- Blood transfusions: These are utilised by those who have sickle cell disease to treat and avoid problems including stroke. Red blood cells are taken from donor blood and supplied through a vein to a patient with sickle cell anemia during a red blood cell transfusion. This raises the quantity of healthy red blood cells, which helps lessen signs and side effects. Risks include infection, an immunological reaction to the donor blood, which may make it difficult to identify new donors, and an excess of iron accumulating in your body. If you receive regular transfusions, you might require treatment to lower iron levels because too much iron can harm your heart, liver, and other organs.
- Stem cell transplant: This surgery, also known as a bone marrow transplant, involves replacing sickle cell-affected bone marrow with healthy bone marrow from a donor. Typically, a sibling or other matched donor who is free of sickle cell anemia is used in the surgery. A bone marrow transplant is only advised for persons, mainly youngsters, who have severe symptoms and consequences of sickle cell anemia because of the risks involved with the treatment, which include death. The only known treatment for sickle cell anemia is a stem cell transplant. Gene treatments and adult stem cell transplantation is currently the subject of clinical trials.
How to avoid painful episodes?
The significant thing that you can do to minimize your chances of having painful episodes is to avert possible triggers.
- Drink ample fluids to prevent dehydration
- To avoid cold, wear warm clothes
- Try to evade sudden changes in temperature, such as swimming in cold water
- Regularly take folic supplements and choose a healthy diet
- Quit smoking
Currently, a bone marrow or stem cell transplant is the only approved treatment for SCA, but there is a chance that these procedures could have life-threatening side effects, doctors rarely advises them.
Clinical trials for other potential therapies are lately being conducted.