September is Sickle Cell Disease Awareness Month. Sickle cell disease is a genetic condition that make it hard for the red blood cells to carry oxygen to tissues and organs.
Patients can experience episodes of severe pain, along with anemia, leg sores, frequent infections and more.
Regional One Health’s Diggs Kraus Sickle Cell Center provides comprehensive care to help reduce symptoms and allow patients to live a longer, healthier life.
During Sickle Cell Disease Awareness Month, outreach about this chronic genetic condition is especially important in the Memphis area.
Memphis has one of the highest rates of sickle cell disease in the country and is also home to the oldest facility dedicated to treating the condition: Regional One Health’s Diggs Kraus Sickle Cell Center. Medical Director Ugochi Ogu, MD leads a team that provides exceptional clinical care and research to help patients achieve longer, healthier lives.
“We take care of our patients as a whole with an amazing team of doctors, nurse practitioners, nurses, a dedicated pharmacist, social workers, care coordinators and more,” she said. “We also engage in cutting-edge research to advance the field. There is more we can do, and we’re hopeful for the future.”
Here’s what Dr. Ogu wants patients to know about living with sickle cell disease:
What is sickle cell disease?
It is an inherited disease in which a gene mutation causes abnormal hemoglobin in the red blood cells. “Usually red blood cells are round like a donut, but the mutation causes them to be sickle shaped,” Dr. Ogu explained. “They aren’t as pliable and can’t squeeze through blood vessels. If they get stuck, it prevents oxygen from reaching tissues and organs.”
That can lead to episodes of extreme pain. “Sickle cell crisis occurs when the organs and tissues are starved of oxygen, and pain is how your body tells you that’s happening,” she said.
Dr. Ugochi Ogu leads a team that provides comprehensive care for patients. “We take care of our patients as a whole with an amazing team of doctors, nurse practitioners, nurses, a dedicated pharmacist, social workers, care coordinators and more,” she said.
In the long-term, it can cause organ and tissue damage. The average life expectancy for a patient with sickle cell disease is in the mid-40s.
Who is at risk?
Most patients are of African descent. The mutation persists as it provided protection against severe forms of malaria, which is more prevalent in Sub-Saharan African nations.
In the United States, there are about 100,000 people living with sickle cell disease. It occurs in 1 of 365 births to black or African American parents.
A person gets sickle cell disease if both parents pass on the mutation. If only one parent passes on the mutation, the person carries the sickle cell trait but does not have symptomatic disease.
“If both parents have the sickle cell trait, with each pregnancy there’s a 25 percent chance they’ll have a child with sickle cell disease, 25 percent chance the baby will be normal, and 50 percent chance the baby will have sickle cell trait,” Dr. Ogu said.
How do I know if I have sickle cell disease?
Babies are routinely screened at birth, so most patients know from a young age. For those who weren’t screened, there are symptoms to watch for, and a routine blood test is used for diagnosis.
Crisis episodes are the signature symptom.
Sickle cell disease can be treated with medication, lifestyle changes and blood transfusions. Patients who receive regular care tend to have fewer symptoms and overall better health.
“People describe the pain in different ways,” Dr. Ogu said. “Some say they would rather have a baby without an epidural. Some say it feels like someone in their bones stabbing them with knives. It’s excruciating pain.”
Other symptoms include anemia, leg sores or ulcers, swelling in the hands or feet, frequent infections, and vision problems. Avascular necrosis, which is most common in the hips, causes joint pain when the bone starts dying due to lack of oxygen.
“You have blood running from head to toe, so any organ system can be affected,” Dr. Ogu said.
What are the treatment options?
The Diggs Kraus Center offers comprehensive treatment and the latest therapies. The clinic and infusion suite are in the same space, making it easy for patients to get all their care in one place.
For some, that means frequent blood transfusions. “You give them ‘good’ blood to dilute out the sickle cell hemoglobin and provide healthy red blood cells to deliver oxygen,” Dr. Ogu said.
There are four medications approved for sickle cell disease, one given as an infusion and the others as oral medications. Currently, the only established cure is a stem cell transplant, and trials are underway for a genetic therapy cure.
Dr. Ogu develops individualized treatment plans based on a patient’s symptoms, how much their symptoms impact their life, their overall health, and their needs and goals.
What can patients do to manage the disease?
Sickle cell disease is a genetic disorder. Most babies are screened at birth, so patients know they have the condition from a young age.
“Number one is to take your medicine!” Dr. Ogu said. “Number two is to lead a healthy lifestyle with diet and exercise, and number three is to know your triggers and try to avoid them.”
Common sickle cell triggers include extreme heat or cold, dehydration and stress.
Why is proper care so important?
Treatment helps patients have fewer crisis episodes and manage long-term risks like stroke, organ failure, and acute chest syndrome, a type of pneumonia that is the leading cause of death for sickle cell patients.
Dr. Ogu’s team encourages patients to access regular care.
“We make our patients feel like family – some of our nurses have been here over 20 years,” she said. “That contributes to a high show rate for patients, which helps us work toward that common goal of getting you living to your best health outcome.”