Sickle cell anemia requires ongoing, lifelong treatment to improve quality of life and prevent complications. Treatment of sickle cell anemia includes taking measures to treat anemia, prevent sickle cell pain crises and other complications, and treat any crises or complications that do occur. This may be accomplished with the help of medications, surgery, or other procedures, and complementary therapies.
Talk with a healthcare professional about all of the available treatment options for sickle cell anemia, as well as the pros and cons for each method.
Medication
Currently, there are two main types of medications used to treat sickle cell anemia. All options work to help reduce pain and improve overall quality of life.
Hydroxyurea
The drug hydroxyurea (Droxia, Hydrea, Mylocel, and Siklos) is approved for use in adults with sickle cell disease, and is sometimes used in children as young as 9 months old. This oral medication is considered a first-line treatment for sickle cell disease, and can help reduce pain crises.
Hydroxyurea causes the body to produce fetal hemoglobin, a type of hemoglobin that is normally produced only before and shortly after birth. The presence of fetal hemoglobin lengthens the life span of red blood cells and makes them less likely to sickle. It takes several months for hydroxyurea to start having any benefit.
People taking hydroxyurea should have their blood checked monthly to assess the effect of the drug and to help determine the correct dose. Possible short-term side effects of hydroxyurea include gastrointestinal complaints. On rare occasions darkening of the skin and nails may also occur. Little is known about the long-term side effects of hydroxyurea. Also, it is not recommended for use during pregnancy.
Pain Medications
If hydroxyurea doesn’t improve sickle cell anemia-related pain, a doctor may also recommend pain medications. These may include:
Acetaminophen (Tylenol) A doctor may recommend this over-the-counter pain reliever to manage mild pain on a short term basis.
Crizanlizumab (Adakveo) This injectable medication aims to decrease the number of pain crises in people ages 16 and older. Possible side effects include flu-like symptoms, such as fever, joint pain, nausea, and back pain.
L-glutamine (Endari) Like crizanlizumab, L-glutamine may decrease the number of pain crise you experience. This is a prescription version that comes in an oral powder formula.
Voxelotor (Oxbryta) Prescribed to people ages 12 and older, this oral medication may improve blood flow in your body while also decreasing anemia risk. Possible side effects include rashes and flu-like symptoms.
Surgery and Other Procedures
In addition to pain-alleviating drugs, a doctor may consider several types of surgeries and other procedures for sickle cell anemia.
Some people need blood transfusions only occasionally, while others need them regularly.
Blood transfusions have their own complications, including transfusion-induced iron overload.
Because the body does not have a way to get rid of iron received from blood transfusions, excess iron can build up in and damage vital organs.
Iron overload is prevented with a class of medications called iron chelators, drugs that attach to iron in the body so that it can be excreted in feces.
Stem Cell Transplants
Blood and bone marrow stem cell transplants may be used to treat sickle cell anemia and may offer a cure for a small number of people. They are performed mainly in children with sickle cell anemia, although they may be feasible in adults as well.
Stem cells are found in bone marrow and, in smaller quantities, in blood and in the umbilical cord. Stem cells can develop into red blood cells, white blood cells, and platelets. The stem cells used for a transplant must come from a closely matched donor — usually a family member who does not have sickle cell anemia.
Stem Cell Gene Addition Therapy
Unlike a stem cell transplant, this procedure involves the use of a person’s own stem cells. After a number of stem cells are removed, a doctor injects a hemoglobin-containing gene before adding the stem cells back into your body.
While not as common as a stem cell transplant, stem cell gene addition therapy may be an alternative for people who lack closely matched donors.
Gene Editing Therapy
This is a newer therapy approved for people ages 12 and older that also involves removing stem cells from your body. Once this takes place, a doctor “edits” the sickle gene so your body can then produce healthy red blood cells once again. After gene editing, the stem cells are returned to your body via transfusion. Researchers don’t know the long-term side effects of gene editing therapy just yet.
Lifestyle Changes
Maintaining a healthy lifestyle with sickle cell anemia can help with symptoms and includes:
Eating a heart-healthy diet
Exercising — but talk to your doctor about an appropriate amount
Talking to your doctor about how much alcohol, if any, is safe to drink
People with sickle cell anemia can also help to prevent sickle cell crises and complications by following these precautions:
Don't take decongestants, which tend to tighten blood vessels.
Avoid very hot or cold temperatures, both of which can trigger a crisis.
Be cautious at high altitudes — you may need extra oxygen.
Avoid choosing a job that requires a lot of physical labor, exposes you to extreme temperatures, or involves long work hours.
Don't travel in unpressurized airplanes (speak to your doctor if you must fly in such a plane).
Children with sickle cell anemia are at high risk of infection, so much of their routine care is aimed at preventing infection by the following:
Daily penicillin
Folic acid supplements to boost red blood cell production
All routine vaccinations, including an annual flu shot
The pneumococcal vaccine, which protects against infection with pneumococcal bacteria
Children should also be up to date with the meningitis vaccine.
Complementary and Integrative Approaches
Complementary treatments for sickle cell anemia primarily involve techniques to help reduce pain, when used with other treatments. Common options include:
Gentle exercises, such as yoga
Meditation
Breathing exercises
Acupuncture
Massage
Cognitive behavioral therapy (CBT), a type of talk therapy
Research also supports complementary pain management options for sickle cell disease, but more studies need to be conducted to determine whether these techniques have any significant impacts on reducing missed days at school or work, as well as hospital visits.
Pain Management
Aside from complementary treatments, a doctor may also recommend certain home treatments as part of your pain management plan. Consider the following:
Pain relievers, such as acetaminophen
Drinking fluids
Using heating pads
Cold compresses
Rest
In a hospital, sickle cell crises are generally treated with:
Fluids, given by mouth or intravenously
Pain relievers, including those used at home or, for severe pain, opioids
Oxygen therapy if oxygen levels are low
Rest
The Takeaway
Sickle cell anemia requires ongoing treatment to help reduce symptoms, such as pain, as well as prevent potentially life-threatening complications.
A doctor may recommend a combination of medications, medical procedures, and complementary therapies to help achieve these goals.
Consider reaching out to a healthcare professional regarding the best options for your own individual case.
