Depending on the severity of a patient’s disease manifestation, treatment options range from medications like hydroxyurea and other newly licensed products to blood transfusions, red blood cell exchanges and, in some cases, hematopoietic stem cell transplantation. At City of Hope, we tailor our treatment plans specifically to each patient’s unique needs. Treatment for sickle cell disease consists mostly of symptom management and prevention.
Our sickle cell disease program offers several unique features that allow our patients to receive treatment in a more efficient and less invasive manner. Because we have our own donor apheresis center blood bank, our patients don't have to travel to another hospital for transfusions and, most times, the process can be completed in one day. In addition to this ease of treatment, our donor apheresis nurses have a high level of expertise that allows them to perform this procedure without implanting a port-a-cath.
When a child with sickle cell disease begins treatment, we are there to provide support and treatment until he or she reaches 21 years of age, at which time we help transition his or her care to an adult treatment center to continue care throughout life.
Symptoms
Major symptoms of sickle cell disease include pain, anemia, infections and stroke. Pain can be managed with a home treatment regimen. Acute pain crises can result in sudden pain in the abdomen, arms, chest, lower back and legs. They can be caused by dehydration, other illnesses, stress and change in altitude and environment. For acute pain, doctors recommend drinking lots of water and taking some over-the-counter pain medicine like ibuprofen or acetaminophen. Combining medicine with massage or a heating pad can give some patients relief, but others might need stronger medicine or need to receive IV fluids. Chronic pain can result from complications like ulcers and necrosis. Most chronic pain cases are managed with prescribed medication.
Anemia is also common in sickle cell patients. While most sickle cell patients have mild anemia, some can develop severe anemia, which is particularly harmful in young children and infants. Severe anemia can be caused by aplastic crisis and splenic sequestration in children with sickle cell disease. An aplastic crisis occurs when a sickle cell is combined with a common virus called parvovirus B19. The two combined can cause bone marrow to stop producing new red cells, leading to anemia. Splenic sequestration occurs when red blood cells get trapped in the spleen, which usually filters the blood. Red blood cells stop circulating, causing severe anemia. Both are treated with blood transfusions, and sometimes with L-glutamine oral powder, although that medication is still being researched.
The spleen is also responsible for protecting against infection. Sickle cells weaken spleen function, making sickle cell patients more susceptible to infection. Those who have damaged spleens are at much higher risk to bacterial infections like chlamydia, meningococcus and salmonella. Anyone with sickle cell who gets a fever about 101°F must see a doctor because of the risk of these infections.
Acute chest syndrome can affect sickle cell patients when blood vessels in the lungs are damaged by sickle cells. This cuts off some oxygen from the lungs, and causes chest pain. Acute chest syndrome can begin a few days after the pain begins. Make sure you see a doctor if you experience chest pain, cough, fever, rapid breathing or shortness of breath. Treatment for acute chest syndrome can include antibiotics, oxygen therapy and blood transfusion.
Stroke and brain complications can also occur. A stroke occurs when blood flow to the brain is blocked and brain tissue is damaged or dies. Stroke is more common in sickle cell patients because of the blood abnormalities caused by sickle cells. Symptoms of stroke include loss of balance, severe headache, trouble speaking, and weakness in the arm or leg on one side of the body. If you suspect someone is having a stroke, call 9-1-1 right away. They will need immediate medical attention. Sometimes, silent stroke can also occur. This means there will be no outward sign of stroke, but there is a brain injury that can cause difficulty learning and making decisions. A doctor will be able to see the silent stroke on an image of the brain.
Searching for a cure for sickle cell disease
For children and adults with sickle cell disease, usual care involves lowering the number and severity of complications, but it does not get rid of sickle cell disease. With more children and adults looking to City of Hope for sickle cell disease treatment, we are committed to providing you or your child with a cure. We want to provide treatment that goes beyond just managing symptoms. New treatments that we are actively researching involve less toxic transplants from a half-related donor and more effective options for gene therapy. An important part of this new effort is the collaboration across multiple disciplines. Our multidisciplinary team of doctors, nurses and other health professionals has decades of experience in treating inherited blood disorders such as sickle cell disease. Our expertise in finding a cure for sickle cell disease includes the following research: