Here's everything you need to know about sickle cell disease

What is sickle cell disease, who does it affect and what is the proper care

HOUSTON – Did you know, sickle cell disease affects approximately 100,000 people in the United States?

Symptoms can range from severe anemia to heart and lung problems.  

But what exactly is sickle cell disease and who does it affect? 

Q: What is sickle cell disease? How do you get sickle cell disease?
A: Sickle cell disease is an inherited group of blood disorders that causes red blood cells to become mis-shapen or sickle-shaped.  A red blood cell's normal shape makes it flexible and allows it to easily move through blood vessels to deliver oxygen. When cells are sickle-shaped, they stick to vessel walls preventing blood and oxygen flow, which ultimately causes severe pain. These episodes of pain, known as "pain crises" can last a week or longer in some patients. 

Q: What is the incidence of sickle cell disease?
A: Sickle cell disease affects approximately 100,000 people in the United States, according to the Centers for Disease Control. According to published research, sickle cell disease affects about 1 out of every 365 African American newborns and about 1 out of every 16,300 Hispanic-American newborns. Additionally, about 1 in 13 African American infants are born with the sickle cell trait.

Q: How do you know if you have sickle cell disease?
A: A blood test can check for hemoglobin S — the defective form of hemoglobin that underlies sickle cell anemia. In the United States, this blood test is part of routine newborn screening done at the hospital. But older children and adults can be tested, too.
In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where it's screened for hemoglobin S.
If the screening test is negative, there is no sickle cell gene present. If the screening test is positive, further tests will be done to determine whether one or two sickle cell genes are present. 

Q: What are some of the signs and symptoms of sickle cell disease? 
A: Acute pain crisis, severe anemia, stroke, bone infarct, splenic infarct, risks for infections, retinal damage, priapism, leg ulcers, heart problems, lung problems (acute chest syndrome), kidney disease, liver complication and multiple organ dysfunction or even multiple organ failure.

Q: How is sickle cell disease treated?
A: Bone marrow transplant, also known as stem cell transplant, offers the only potential cure for sickle cell anemia. It's typically reserved for people younger than age 16 because the risks increase for people older than 16. Finding a donor is difficult, and the procedure has serious risks associated with it, including death. As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. Babies and children age 2 and younger with sickle cell anemia should make frequent visits to a doctor. Children older than 2 and adults with sickle cell anemia should see a doctor at least once a year, according to the Centers for Disease Control and Prevention. However, since patients actually have progressive organ damage as they grow older, adult SCD specialists recommend that patients should continue to see a doctor at least every 3 to 6 months. Treatments might include medications to reduce pain and prevent complications, and blood transfusions, as well as a bone marrow transplant.

Q: What makes the UT Physicians Comprehensive Sickle Cell Center different from other clinics that treat patients with sickle cell disease? 
A: The clinic is located at 1200 Binz Street, Suite 850, and is the only family comprehensive sickle cell center in Houston. Subspecialists, social services and behavioral health services have all been added to the center for a holistic approach to patient care. What makes the Comprehensive Sickle Cell Center unique is that it provides lifelong care for patients. The center focuses on providing a wide range of care and resources for its patients. Having the facility and care available at one place is key. Sickle cell patients need a multidisciplinary team that can care for their unique needs and prevent visits to the emergency room. Thanks to the medical professionals at the center, not only do patients have access to quality care, but there are also resources available to assist them in managing the pain. Infusion rooms are designed to help keep patients comfortable and relaxed. The rooms come equipped with televisions, treatment recliners, privacy curtains, pillows, heated blankets, and IV therapy used to keep patients hydrated while doctors monitor their pain.

Watch the full clip above for an explanation from Medical Director, Dr. Idowu, at UT Physicians Comprehensive Sickle Cell Center, along with patient, Hoxi Jones who shares details on her diagnoses for sickle cell disease since the age of 3. 

For more information or to schedule a consultation at the location nearest you, just visit utphysicians.com or give them a call at 888-4UT-DOCS. 
 

Sponsored by UT Physicians


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