Sickle Cell Management
Novant Health Blume Pediatric Hematology & Oncology is the only practice in Charlotte offering sickle cell research opportunities to our patients. All of our sickle cell patients benefit from the Presbyterian Comprehensive Sickle Cell Disease Management Program. This is a collaboration between our office, Presbyterian Hospital and community agencies. The program offers health screenings, strategies to manage pain and complications of the disease, laboratory tests, referrals, individualized and group counseling, coordination with community resources, nutritional evaluation, and inpatient disease management. The program also provides advocacy for school-related issues, future planning and help with transitioning to the adult program.
What is Sickle Cell?
Sickle cell disease is a blood disorder caused by defective hemoglobin. Hemoglobin is a protein in red blood cells that allows those cells to carry oxygen to tissues throughout the body. Defective hemoglobin causes sickle cells to be shaped liked “C”s, or sickles, instead of “O”s like normal red blood cells. Instead of carrying oxygen to tissues, those sickle cells get stuck, causing pain in joints and fatigue throughout the body.
Sickle cells do not live for as long as normal blood cells. And, sickle cells are often destroyed by the body because of their shape and stiffness. This reduces the amount of hemoglobin and proteins in the blood, causing anemia.
There are a few variations of the sickle cell gene. You can read more about those by clicking here. One of the most common forms is sickle cell anemia.
What Causes Sickle Cell?
Sickle cell is caused by a gene mutation. Sickle cell disorder is hereditary, meaning it is passed down from parent to child. A parent can be a carrier of the sickle cell gene without knowing it.
Sickle Cell Anemia Symptoms
Symptoms of sickle cell anemia include:
- A pain crisis, or sickle cell crisis, happens when sickle-shaped cells get stuck in the blood vessels and block the flow of blood to an area. These events can also include swelling and possible death of tissue not receiving adequate blood and oxygen supply.
- Jaundice, or yellowing of the skin, occurs in sickle cell sufferers because blood cells are dying faster than the liver can filter them out.
- Acute chest syndrome happens when cells get blocked up in the chest. This can cause fever, pain and violent cough. This can be life-threatening if blood and oxygen do not get to the lungs.
- Splenic sequestration occurs when sickle cells pool in the spleen, which can cause the spleen to become large and painful.
- Priapism is when sickle cells block blood flow to the penis. This can cause impotence if not treated promptly.
- Stroke can happen if sickle cells block blood flow to the brain. Our clinic offers exchange transfusions and apheresis services for patients who have suffered strokes.
Sickle Cell Anemia Treatment
Treatment of sickle cell anemia can include:
- Pain medications or medications to prevent infections.
- Drinking plenty of water to prevent and treat pain crises.
- Blood transfusions to replace sickle cells with healthy cells. We are able to offer exchange transfusions manually and by pheresis. We are aware of the side effects of blood transfusions, and are skilled at managing patients who have an iron overload after multiple transfusions.
- Bone marrow transplants. In some severe cases, these procedures have reduced or eliminated sickle cell symptoms.
- Encouragement. We understand it is important to recognize successes and encourage accomplishment. Our Report Card for Success program encourages students to perform up to their potential, and our entire staff recognizes those achievements. We also recognize health successes, and urge our patients to take an active role in their treatment. We know that attitude and outlook are an important part of any treatment plan!