Hope Through Progress: Advancing Sickle Cell Care Globally

The Mayo Clinic states that sickle cell anemia is one of a group of inherited disorders known as sickle cell disease. It affects the shape of red blood cells, which carry oxygen to all parts of the body. Red blood cells are usually round and flexible, so they move easily through blood vessels. In sickle cell anemia, some red blood cells are shaped like sickles or crescent moons. These sickle cells also become rigid and sticky, which can slow or block blood flow. World Sickle Cell Day is an international awareness day observed annually on June 19th. The day is set aside to alert the global public about sickle cell disease. The theme for this year's observance is "Hope Through Progress: Advancing Sickle Cell Care Globally." The Centers for Disease Control and Prevention (CDC) estimates 100,0000 people in the United States of America have sickle cell, and it’s more common among Black people about 1 in every 365 Black newborns in the U.S. have it. The Sickle Cell Unit (SCU) indicates that 1 in every 150 Jamaicans are born with Sickle Cell Disease (SCD). Symptoms. About 35% of all people who are assigned male at birth (AMAB) who have sickle cell anemia develop priapism, or painful erections, that last four hours or more. Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body cannot get enough oxygen. This causes fatigue. Symptoms can include: Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay. Some people with sickle cell anemia also have chronic pain from bone and joint damage, ulcers, and other causes. Swelling of hands and feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell. Delayed growth or puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers. Frequent infections. The spleen is important for protecting against infections. Sickle cells can damage the spleen, raising the risk of developing infections. Babies and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia. Vision problems. Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells. This can damage the portion of the eye that processes visual images, called the retina, and lead to vision problems. 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. Life Expectancy. There was a time when babies born with sickle cell anemia rarely lived past age 5. Now, healthcare providers are able to diagnose sickle cell and begin treatment that eases symptoms and complications. People are living into their 50s with sickle cell anemia. Even so, people who have sickle cell anemia live 20 to 30 years less than people who don’t have the condition. Treatments . While the current approach to treatment is rooted in relieving pain and the prevention of complications, exciting and emerging treatments are on the horizon. The fight to cure Sickle Cell Disease has been boosted recently. Gene therapy appears very promising in this regard. The U.S. Food and Drug Administration approved two milestone treatments, Casgevy and Lyfgenia, representing the first cell-based gene therapies for the treatment of sickle cell disease (SCD) in patients 12 years and older. Additionally, one of these therapies, Casgevy, is the first FDA-approved treatment to utilize a type of novel genome editing technology, signaling an innovative advancement in the field of gene therapy. Healthcare providers typically treat sickle cell anemia with blood transfusions, antibiotics to treat infections and medications that reduce symptoms caused by sickle cell anemia complications. Regular health checkups with your primary provider care may prevent serious medical complications. Having a relationship with your healthcare provider who understands your situation may make it easier to get help if you have an acute pain crisis. It is very important that persons diagnosed with Sickle Cell disease pay attention to their emotional health. Unfortunately, there are stigmas associated with sickle cell anemia. Sometimes, these stigmas cause people to have depression or anxiety. Talk to your healthcare provider if you are struggling with emotions related to feeling stigmatized. They can recommend resources to help you. More than that, they may be able to advocate for you by educating peers about sickle cell anemia complications and symptoms. On this World Sickle Cell Day let us stand with all those who continue this challenging journey. In the words of Kahlil Gibran, out of the suffering have emerged the strongest souls. Wayne Campbell is an educator and social commentator with an interest in development policies as they affect culture and or gender issues. waykam@yahoo.com @WayneCamo © #WorldSickleCellDay