Sickle Cell Impact
Normally, your red blood cells are flexible and round, moving easily through your blood vessels. In people with Sickle Cell, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons.
These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body. There’s no universal cure for Sickle Cell Anemia. But treatments and medication can relieve pain and help prevent problems associated with the disease.
Commonly Known
Symptoms & Treatments
Symptoms
Anemia
Because sickled cells are short-lived or destroyed, there are less red blood cells available in the body. This results in anemia. Severe anemia can make you feel dizzy, short of breath, and tired.
Pain crisis (Vaso-occlusive)
This occurs when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel. The pain can occur anywhere, but most often occurs in the chest, arms, and legs. Infants and young children may have painful swelling of the fingers and toes. Interruption in blood flow may also cause tissue death.
Acute chest syndrome
This occurs when sickling occurs in the chest. This can be life-threatening. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough.
Splenic sequestration (pooling)
Crises are a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly. The spleen can also become enlarged and painful from the increase in blood volume. After repeated episodes, the spleen becomes scarred, and permanently damaged. Most children, by age 8, do not have a working spleen either from surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a working spleen. Infection is the major cause of death in children younger than age 5 in this population.
Stroke
This is another sudden and severe complication of people with sickle cell disease. The misshapen cells can block the major blood vessels that supply the brain with oxygen. Any interruption in the flow of blood and oxygen to the brain can result in severe brain damage. If you have one stroke from sickle cell anemia, you are more likely to have a second and third stroke.
Priapism
This is a painful obstruction of the blood vessels in the penis by sickle cells. If not promptly treated, it can result in impotence.
Avascular necrosis (AVN)
The death of bone tissue due to a loss of blood supply. You might also hear it called osteonecrosis, aseptic necrosis, or ischemic bone necrosis. If it isn’t treated, AVN can cause the bone to collapse. AVN most often affects your hip.
Sickle Cell Bone Disease (SBD)
This is a chronic and invalidating complication of SCD. About 80% of SCD young adults have low bone mineral density (BMD), osteopenia, osteoporosis, increased risk of fractures, vertebral collapse, and bone pain [10,11,12]. The reduced BMD in SCD subjects is independent of risk factors typical for the general population such as age, sex, and menopause. Bone loss could partially depend on different factors such as vitamin D insufficiency, low physical activity, malnutrition, and release of inflammatory cytokines (Figure 1) [13].
Treatments
Pain medications
Help to relieve pain during sickle cell crises.
Blood transfusions
Help to treat anemia and prevent stroke. They are also used to dilute the sickled hemoglobin with normal hemoglobin to treat chronic pain, acute chest syndrome, splenic sequestration, and other emergencies.
Drinking plenty of water daily (8 to 10 glasses)
Helps to prevent and treat pain crises. In some situations, intravenous (IV) fluids may be required.
Vaccinations and antibiotics
Used to prevent infections.
Folic acid
Helps to prevent severe anemia.
Hydroxyurea (Chemotherapy)
Increases fetal hemoglobin and may helps reduce the frequency of pain crises and acute chest syndrome. It may also help decrease the need for blood transfusions.
Regular eye exams
These are done to screen for retinopathy.
Oxbryta
Helps to reduce the amount of sickled red blood cells and may help reduce pain crises, jaundice and other sickle cell related complications
Bone marrow transplant
Bone marrow transplants can cure some people with sickle cell disease. The decision to have this procedure is based on the severity of the disease and ability to find a suitable bone marrow donor. These decisions need to be discussed with your doctor and are only done at specialized medical centers.