Saving Yourself From Sickle Cell Anemia

Your five-month-old baby is incredibly fussy. His hands and feet look swollen and he has jaundice. This is not a surprise because he has sickle cell anemia.

According to the Cleveland Clinic, sickle cell anemia is a form of the inherited blood disorder, sickle cell disease. Sickle cell anemia affects your red blood cells, transforming them from round, flexible discs into stiff and sticky sickled cells. The sickled cells prevent oxygen from being carried out to parts of your body. Sickled cells don’t live as long as regular red blood cells. As a result, you don’t have enough red blood cells and you develop anemia.

In the past, babies with sickle cell anemia rarely lived to be adults. Now, with early detection and newer treatments, about half of those diagnosed with the condition can live into their 50s. Those with sickle cell anemia still face challenges due to the condition. But healthcare providers have treatments that reduce the risk of complications and ease symptoms when they happen. There are still some places in the world where people don’t have access to effective medical treatment for sickle cell anemia.

Sickle cell anemia is rare in the United States. It mostly affects people whose ancestry links back to parts of the world where people have malaria and carry a gene that provides partial protection against anemia. The gene also causes sickle cell anemia. Blacks are more vulnerable to the disease but it also affects people from Southern European, Middle Eastern or Asian Indian ancestry.

Babies may not have symptoms of sickle cell anemia for several months. And when they do, symptoms include extreme tiredness or fussiness from anemia, painfully swollen hands and feet, and jaundice. Babies may be vulnerable to infections and spleen damage that affects their immune system. As people with sickle cell anemia age, they will develop different and more serious health problems that happen as a result of organ tissues not receiving enough oxygen. Those with the disease are at increased risk for stroke and lung, kidney, spleen and liver damage.

Those with sickle cell anemia inherit the disease from their parents. In sickle cell anemia, the gene that helps make normal red blood cells mutates, or changes. People who inherit the mutated hemoglobin protein gene from both biological parents have sickle cell anemia. Those that inherit the mutated gene from one biological parent have the sickle cell trait.

Normal red blood cells contain hemoglobin. Hemoglobin is a protein and the main part of red blood cells. When the hemoglobin gene mutates, it creates sickled cells that have difficulty traveling through the network of blood cells that carry oxygen, nutrients and hormones throughout your body. The reason why is:

§  Normal hemoglobin is soluble, meaning it dissolves in fluid. Abnormal hemoglobin isn’t as soluble and forms solid clumps in your red blood cells.

§  Red blood cells need to be flexible to squeeze and slide their way through narrow blood vessels. Red blood cells carrying abnormal hemoglobin can’t do that. Instead, blood cells with abnormal hemoglobin end up blocking blood vessels and blood flow.

§  Regular blood vessels live about 120 days. Sickled cells last within 10 to 20 days. Normally, your bone marrow makes enough red blood cells to replace dying cells. When cells die earlier than usual, your bone marrow struggles to match supply with demand. When the bone marrow can’t keep up, you don’t have enough red blood cells.

Sickle cell anemia symptoms usually start around 5 to 6 months old. As they age, most people with sickle cell anemia have an increased risk of developing new medical conditions. Some of these conditions can be life-threatening. But by educating yourself on conditions and symptoms, people with sickle cell anemia can seek help at the first sign of trouble, allowing healthcare providers to treat the condition.

One condition is vaso-inclusive crisis (VOC) or acute pain crisis which consists of a sudden intense pain or sharp, stabbing pain that affects your abdomen, lower back, arms and legs. Living with VOC can be one of the more difficult aspects of having sickle cell anemia. This can be remedied with opioid painkillers but often there is a stigma attached to this need. Some studies show that members of racial minorities receive less pain meds and have to wait longer than people who are white.

Acute chest syndrome is the most common complication of sickle cell anemia. It is also the most common cause of death and the second most common cause of hospital admission. It occurs when sickled cells clog and clump blood vessels in your lungs. You have sudden chest pain, cough, fever and trouble breathing.

People with sickle cell anemia may have mild, moderate or severe forms of anemia. You have skin color that is more pale than usual, fatigue and trouble breathing.

Babies with anemia can be unusually fussy or irritable and may grow at a slower rate than other children the same age or may enter puberty at a later age than other children.

People with sickle cell anemia are vulnerable to strokes, including babies. Stroke symptoms include severe headache, sudden weakness on one side of your or your child’s body, change of alertness, trouble speaking, trouble seeing and trouble walking.

Splenic sequestration often causes acute anemia. This occurs when sickled cells become stuck in your spleen, which forces your spleen to get larger. You can experience pain in your upper left belly (abdomen) and sometimes, children’s spleen are visible and felt through the skin.

Other symptoms are bacterial infections, priapism (long-lasting erections), leg ulcers, pulmonary hypertension, chronic kidney disease and detached retina.

Healthcare providers diagnose sickle cell anemia by taking blood samples. They may use one of two techniques, hemoglobin electrophoresis or high-performance liquid chromatography. Since 2007, all babies in the United States have been tested for sickle cell anemia. Early diagnosis and treatment are why fewer babies or children die of this disease.

Right now, allogenic stem cell transplantation is the only way providers can cure sickle cell anemia and other forms of sickle cell disease. This procedure is recommended for patients with severe complications such as stroke, acute chest syndrome, and recurring VOC/acute pain crises.

Otherwise, providers usually treat sickle cell anemia with blood transfusions, antibiotics that treat infections and medicine which reduces symptoms caused by complications of the disease.

Sickle cell anemia is a chronic illness that can’t be cured. There are medications that make managing sickle cell anemia a little easier. Now the average patient with the disease can live into their 50s. Those with sickle cell anemia can now have a better quality of life.