Sickle cell anemia is a genetic disorder which is hereditary in nature , a condition in which there are not sufficient healthy red blood cells to carry enough oxygen all through the body.
Sickle cell anemia is caused by a mutation of gene. Hemoglobin carries oxygen from the lungs to all other parts of the body. In sickle cell anemia, hemoglobin will be abnormally shaped like sickles or Crescent moon, which causes red blood cells to become rigid, sticky and misshapen. These irregularly formed cells can get stuck in small blood vessels, which can gradually slow down blood flow and oxygen to parts of the body.
The sickle cell gene is passed from generation to generation in a pattern of inheritance known as autosomal recessive inheritance. This means that both the mother and the father should pass by on the defective form of the gene for a baby to be affected.
Signs and symptoms of sickle cell anemia, which differ from person to person and change over time, include:
Sickle cells break apart easily and die, leaving you without sufficient red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells die in 10 to 20 days, leaving a scarcity of red blood cells (anemia). Without enough red blood cells, the body cannot get the oxygen it wants to feel energized, causing fatigue.
Episodes of pain. Periodic episodes of pain, called crises, are an important symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow thru tiny blood vessels to the chest, abdomen and joints. Pain can also occur in the bones.
Painful swelling of arms and feet. The swelling is triggered by sickle-shaped red blood cells blocking blood waft to the arms and feet.
Frequent infections. Sickle cells can damage an organ that fights infection (spleen), leaving you greater vulnerable to infections. Doctors normally give infants and teenagers with sickle cell anemia vaccinations and antibiotics to stop potentially life-threatening infections, such as pneumonia.
Delayed growth. Red blood cells provide the oxygen and nutrients the body growth. A shortage of healthy red blood cells can gradual the normal growth cycle and delays puberty in teenagers.
Vision problems. Tiny blood vessels that supply the eyes may also become plugged with sickle cells. This can harm the retina — the portion of the eye that processes visual images, leading to vision problems.
Sickle cell anemia can lead to a host of complications, including Stroke, Acute chest syndrome, pulmonary hypertension. Organ damage, Blindness, Leg ulcers, Gallstones, Priapism etc.
Medications used is not to treat sickle cell anemia but to avoid complications arise due to the disorder.
Antibiotics. Children with sickle cell anemia may start taking the antibiotic penicillin when they’re about 2 months old and proceed taking it till they’re at least 5 years old. Doing so helps stop infections, such as pneumonia, which can be life-threatening to a child with sickle cell anemia.
As an adult, if the spleen is removed or had pneumonia, you may want to take penicillin throughout the life.
Pain-relieving medications. To relieve pain during a sickle cell crisis, the health practitioner may prescribe pain medications.
Hydroxyurea (Droxia, Hydrea). When taken daily, hydroxyurea reduces the frequency of painful crises and may reduce the need for blood transfusions and hospitalizations. Hydroxyurea seems to work by stimulating production of fetal hemoglobin — a type of hemoglobin found in newborns that helps stop the formation of sickle cells.
Hydroxyurea will increase the risk of infections, and there is some situation that long-term use of this drug might cause problems later in life for people who take it for many years. More study is needed.
The physician can help you determine if this drug may be really useful for you or the child. Don’t take the drug if you are pregnant.
Vaccinations to prevent infections
Childhood vaccinations are necessary for preventing disease in all children. They’re even more important for kids with sickle cell anemia due to the fact their infections can be severe.
The physician will make sure the child receives all of the recommended childhood vaccinations. Vaccinations, such as the pneumococcal vaccine and the annual flu shot, are additionally important for adults with sickle cell anemia.
In blood transfusion, red blood cells are separated from the donated blood, then given intravenously to a person with sickle cell anemia.
Blood transfusions increase the normal red blood cells in circulation, helping to relieve anemia. In adolescents with sickle cell anemia at high risk of stroke, normal blood transfusions can limit the risk. Transfusions can also be used to treat other issues of sickle cell anemia, or they can be given to prevent complications.
Blood transfusions elevate some risk, including infection and extra iron buildup in the body. Because excess iron can harm the heart, liver and other organs, people who endure regular transfusions may want remedy to reduce iron levels.
Bone marrow transplant, additionally known as stem cell transplant, gives the solely possible treatment for sickle cell anemia. It’s typically reserved for people younger than age sixteen because the risks extend for people older than 16 finding a donor is difficult, and the procedure has serious risks associated with it, inclusive of death.
Treatments may include medicines to reduce pain and stop complications, and blood transfusions, as well as a bone marrow transplant.
Bone marrow transplant
A bone marrow transplant, also known as a stem cell transplant, involves replacing bone marrow affected by sickle cell anemia with healthy bone marrow from a donor. The procedure generally makes use of a matched donor, such as a sibling, who doesn’t have sickle cell anemia. For many, donors aren’t available. But stem cells from umbilical cord blood may be an option.
Because of the risks associated with a bone marrow transplant, the technique is recommended only for people, usually children, who have significant symptoms and issues from sickle cell anemia.
If a donor is found, the individual with sickle cell anemia receives radiation or chemotherapy to destroy or reduce his or her bone marrow stem cells. Healthy stem cells from the donor are injected intravenously into the bloodstream of the person with sickle cell anemia, where they migrate to the bone marrow and start producing new blood cells.
The technique requires a lengthy hospital stay. After the transplant, you’ll receive drugs to help prevent rejection of the donated stem cells. Even so, the body may reject the transplant, leading to life-threatening complications.
Do you have questions after reading this article? Please let us know! Leave a comment below, we would love to give you some answers! For the best Healthcare Facilitator Services Contact the number below.
Have Questions? Enquire Now
Call +91 9600195686