Sickle Cell Disease
Sickle Cell disease was once the rally cry for Black suffering and the need for better health care for patients of this disease characterized by one word: pain. In the past 30 years, the main treatment for sickle cell crisis: opioids, has been contentious for the medical community and maddening for the patients who need drugs like morphine and demerol to live. Amid our national “war on drugs", we have to continue to educate the medical community on this disease that largely affects people of African descent in order to prevent unjust suffering and death. These patients are often stigmatized and have to fight to be recognized.
What is Sickle Cell Disease?
Sickle cell disease is a group of inherited red blood cell disorders that affects hemoglobin, the protein that carries oxygen through the body. Normally, red blood cells are disc shaped and flexible to move easily through the blood vessels. If you have sickle cell disease, your red blood cells are crescent or “sickle” shaped. These cells do not bend and move easily and can block blood flow to the rest of your body.
The blocked blood flow through the body can lead to serious problems, including stroke, eye problems, infections and episodes of pain, called pain crises. Having sickle cell disease also raises your risk for severe illness from COVID-19.
It is estimated that:
SCD affects approximately 100,000 Americans.
SCD occurs among about 1 out of every 365 Black or African-American births.
SCD occurs among about 1 out of every 16,300 Hispanic-American births.
About 1 in 13 Black or African-American babies is born with sickle cell trait (SCT).
Most infants with SCD born in the United States are identified by routine neonatal screening. As of 2016 all 50 states include screening for sickle cell disease as part of their newborn screen. The newborn's blood is sampled through a heel-prick and is sent to a lab for testing. The baby must have been eating for a minimum of 24 hours before the heel-prick test can be done. Some states also require a second blood test to be done when the baby is two weeks old to ensure the results.
What Health Problems Does Sickle Cell Disease Cause?
“Pain Episode” or “Crisis”: Sickle cells don’t move easily through small blood vessels and can get stuck and clog blood flow. This causes pain that can start suddenly, be mild to severe, and last for any length of time.
Infection: People with SCD, especially infants and children, are more likely to experience harmful infections such as flu, meningitis, and hepatitis.
Hand-Foot Syndrome: Swelling in the hands and feet, often along with a fever, is caused by the sickle cells getting stuck in the blood vessels and blocking the blood from flowing freely through the hands and feet.
Eye Disease: SCD can affect the blood vessels in the eye and lead to long term damage.
Acute Chest Syndrome (ACS): Blockage of the flow of blood to the lungs can cause acute chest syndrome. ACS is similar to pneumonia; symptoms include chest pain, coughing, difficulty breathing, and fever. It can be life threatening and should be treated in a hospital.
Stroke: Sickle cells can clog blood flow to the brain and cause a stroke. A stroke can result in lifelong disabilities and learning problems.
How is Sickle Cell Disease Treated?
For Infants: Infants need to see a pediatrician regularly and frequently. Every 3 to 12 months depending on their age.
For Adults: Regular vaccines, including the influenza and flu shots are encouraged. Two pneumococcal vaccines as part of their immunizations. The goal is to prevent infections. Some patients are on prophylactic antibiotic therapy
With Medication: For severe cases of SCD, the medicine hydroxyurea is an option. Research suggests that hydroxyurea can reduce the number of pain episodes, need for blood transfusions and acute chest syndrome.
Surgery: The only cure is a bone marrow or stem cell transplant. This is extremely risky and the bone marrow has to be a close match to increase the likelihood of a successful operation.