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Friday, March 7, 2014
The treatment of sickle cell disease involves preventative measures (health maintenance), medicines to treat the symptoms and complications of sickle cell, as well as medicines and blood transfusions to treat sickle cell directly. While all patients with sickle cell disease should receive preventative care, the use of medicines and transfusions should be individualized based on the specific problems that occur. This NetWellness feature contains information about:
Routine follow-up appointments with a hematologist or physician familiar with sickle cell disease every 3 months under the age of two years and every 6 months over the age of two years.
Children with sickle cell anemia need to start taking the antibiotic penicillin or amoxicillin when they reach 2 to 4 months of age and continue until they're 5 years old. Doing so helps prevent serious blood infections which can be life-threatening. Both children and adults with sickle cell disease require extra folic acid to help their body make red blood cells.
Vaccinations for blood infections (bacteremia) pneumonia, meningitis, influenza (the flu), and hepatitis. Children with sickle cell disease are at increased risk for getting infections including life threatening blood infections.
Education about sicke cell complications is extremely important for patients and families with sickle cell disease. Knowing when to seek medical attention can potentially be life-saving. Listed below are descriptions of some important conditions:
Fever: Patients with sickle cell have an increased risk of getting severe bacterial infections. All patients with fever need to be seen by a doctor within 24 hours or immediately if they seem very ill and/or if the fever persists. A physical exam, blood work and IV antibiotics need to be given and, in some circumstances, patients will require admission to the hospital.
Enlarged spleen (splenic sequestration): In sickle cell disease, blood can suddenly become trapped in the spleen and this can lead to severe anemia that requires a blood transfusion and splenic enlargement and pain. Patients and their families need to recognize symptoms of an enlarged spleen, which include lethargy (very tired), pale appearance, and pain on the left side of the belly. Patients can be taught to feel for the spleen as well.
Pain management: Patients and their families need to be educated with a plan on how to treat sickle pain at home so they can avoid numerous trips to the clinic and emergency room.
Acute chest syndrome: Patients with sickle cell disease are at risk to develop a severe lung injury. This may be due to a lung infection or fat released from the bone marrow and transmitted to the lung. Patients who develop fever, chest pain, cough or difficulty breathing should be seen by a doctor to look for this syndrome.
Avoiding dehydration: Sickling can be triggered by dehydration, extreme cold or heat (especially swimming in cold water), and vigorous physical activity. Patients should do their best to avoid these triggers and always drink plenty of non-caffeinated fluids.
The use of medicines and transfusions for treatment of symptoms and complications of sickle cell should be individualized based on the specific problems that occur. Listed below are some treatment options:
Pain medications: Over-the-counter pain medicines such as acetaminophen (Tylenol) and ibuprofen (Advil or Motrin) as well as the application of warm heat are helpful for mild pain. For moderate pain, prescription painkillers such as naproxen (Naprosyn) or opioids such as codeine, morphine, or oxycodone may be necessary to control sickle pain. For severe pain, patients will require admission to the hospital to receive IV (in the vein) opioids medicines and close monitoring.
IV Fluids: IV fluids may help reduce blockage of blood vessels by diluting out the sickled red blood cells.
Oxygen: Breathing in supplemental oxygen through a breathing mask adds oxygen to your blood and helps you breathe easier. It may be necessary if you have a low oxygen blood level, acute chest syndrome, or a sickle cell crisis.
Antibiotics: Antibiotics are important for patients with sickle cell disease when they develop fever, pneumonia, acute chest syndrome or other infections.
Blood transfusions: In a red blood cell transfusion, red blood cells are removed from a supply of donated blood. These donated cells are then given through an IV to a person with sickle cell anemia. Blood transfusions increase the number of normal red blood cells in circulation, helping to correct the anemia. Blood transfusions can be life-saving for patients with sickle cell disease especially if the number of red blood cells in circulation becomes very low (severe anemia).
Hydroxyurea (Droxia, Hydrea): This prescription drug, originally used to treat cancer, may be helpful for children and adults with sickle cell disease. When taken daily, it reduces the frequency of painful crises and acute chest syndrome and also reduces the need for blood transfusions. It works by causing an increase in fetal hemoglobin (a type of hemoglobin found in newborn babies) that prevents the formation of sickle cells. This is a preventative medicine only. It does not relieve pain once a patient has already developed a pain crisis.
Blood Transfusions: Simple blood transfusions can also be given at a regular schedule (every 4-6 weeks) to prevent sickle cell problems. In some instances, patients are placed on a special machine so they can receive exchange transfusions where 6 to 10 units or a total of 1500 cc are replaced with normal blood cells. The most common reason that children receive scheduled transfusions is for the prevention of stroke. Blood transfusions are very effective at preventing stroke as well as preventing pain crises, acute chest syndrome, enlarged spleen (splenic sequestration) and allowing leg ulcers to heal.
Blood transfusions carry some risk. There is still a risk of catching a virus through a blood transfusion, although the risk is now very low because of better testing. For example, the risk of getting HIV (the AIDS virus) is 1 in 2.5 million. Sometimes, if there is minor mismatch between the donor blood and the patient's blood, the patient can have a reaction to the blood. These are usually mild and easily treated. Blood contains the mineral iron. Riegular blood transfusions cause an extra iron to build up in your body. Because excess iron can damage your heart, liver and other organs, people who undergo regular transfusions must often receive a medicine to reduce iron levels.
Bone Marrow Transplant: Because red blood cells are made in the bone marrow, a bone marrow transplant is a potential cure for sickle cell disease. In a bone marrow transplant, a patient is given high dose chemotherapy and usually radiation to the entire body to wipe out the bone marrow. Then, bone marrow from a donor (usually a sibling) is given through an IV to the patient. The donor bone marrow cells repopulate the patient's bone marrow and start to make red blood cells, which are normal red blood cells that do not sickle.
One problem with transplant is that it is often difficult to find a bone marrow donor who is a match. Also, because of the high dose chemotherapy and radiation, patients can experience many side effects and there is a 5-10% risk of death. Recently, doctors have been performing bone marrow transplants using less intense chemotherapy and radiation to try and reduce the side effects and the risk of death.
Gene therapy: Because sickle cell anemia is caused by a defective gene, researchers are exploring whether correcting this gene and inserting it into the bone marrow of people with sickle cell anemia will result in the production of normal hemoglobin. Scientists are also exploring the possibility of turning off the defective gene while reactivating another gene responsible for the production of fetal hemoglobin — a type of hemoglobin found in newborns that prevents sickle cells from forming. Thus far gene therapy has only been performed in animal experiments.
Decitabine is a new drug that may work better than hydroxyurea. It also works to increase the amount of hemoglobin F in the body, thus reducing the ability of the red cells to form sickle cells.
Butyric acid: Early studies show that this commonly used food additive may increase the amount of fetal hemoglobin in the blood.
Clotrimazole: Normally used to treat fungal infections, this over-the-counter medication helps prevent a loss of water from red blood cells, which may reduce the number of sickle cells that form.
Nitric oxide: People with sickle cell anemia have low levels of nitric oxide, a gas that helps keep blood vessels open and reduces the stickiness of red blood cells. Treatment with nitric oxide may prevent sickle cells from forming.
This article is a NetWellness exclusive.
Last Reviewed: Jul 10, 2009
Eric H Kraut, MD
Professor of Hematology
College of Medicine
The Ohio State University
Anthony D Villella, MD
Formerly, Assistant Professor of Pediatrics
School of Medicine
Case Western Reserve University