Alpha-thalassemia

Treatment

Is there a treatment for alpha-thalassemia?

Treatment for alpha-thalassemia depends on the specific type an individual has. Hemoglobin Bart is the most severe. Hemoglobin H can range from mild to severe. Carriers may experience mild anemia or no symptoms at all.

Fetuses with hemoglobin Bart typically do not survive beyond the newborn period. A few babies have received transfusions while in the uterus or immediately after delivery and have survived to five years of age. These fetuses are monitored before birth by ultrasonography and, as children, require regular transfusions. They usually have serious symptoms including birth defects of the heart, limbs and genitals, and delays in development. The mothers of these infants often experience maternal complications including severe anemia, preeclampsia, an abnormal amount of amniotic fluid, and delivery complications. Because of these serious complications, prenatal diagnosis and termination of affected pregnancies can be considered.

For individuals with hemoglobin H disease, supplementation of folic acid is generally recommended, especially in children, pregnant women, and women who may become pregnant. Because affected individuals can have low levels of certain vitamins, a non-iron containing multivitamin is usually recommended. Patients should avoid oxidative compounds and medications, and a high iron diet. Any infections should be treated promptly. There should be ongoing monitoring of growth, bone health, spleen size, leg ulcers, fatigue, and inflammation of the gallbladder.

During pregnancy, women with Hemoglobin H disease should be monitored for potential preeclampsia, congestive heart failure, threatened miscarriage and worsening anemia. Children should be monitored for growth and development every 6-12 months.

Usually blood transfusions are not necessary for individuals with hemoglobin H disease. However, over time symptoms such as poor cardiac function, fatigue, anemia, poor growth, poor weight gain and bone changes, such as enlargement of the upper jaw, around the eyes and forehead, may worsen and indicate the need for a blood transfusion. The ages at which these symptoms worsen, if at all, depends on the severity of disease in each individual.

People affected with alpha-thalassemia experience anemia. Patients should be watched for anemia especially if they have an infection, a fever, an enlarged spleen, or are pregnant. Patients can experience a hemolytic crisis, which is a rapid destruction of red blood cells. During a hemolytic crisis, patients require immediate medical treatment, possibly including a blood transfusion. During a hemolytic crisis and transfusion, hydration and fever control is very important. The cause of infection should be identified and treated. Patients who receive blood transfusions, and even those who do not, may develop iron overload, as determined by measurements of liver iron. These patients may require chelation therapy, treatments to rid the body of excess iron. Patients should be monitored for iron overload and hemoglobin levels at least annually.

For those with an enlarged spleen, having the spleen removed may improve symptoms. Risks associated with this include blood infection and blood clots. Aspirin is recommended for those who undergo this surgery to control clotting. For those with persistent gallstones that do not respond to antibiotics or surgical removal of the stones, removal of the gallbladder may be indicated.

Carriers of alpha-thalassemia may have mild anemia, which would need monitoring and potentially treatment.

References
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Are there organ specific treatments to treat alpha-thalassemia?

I have alpha-thalassemia hemoglobin H disease. How do I know if I should have my spleen removed?

I have hemoglobin H disease. How do I know if I should start having transfusions?

If I have alpha-thalassemia, what should my doctor know about managing the disease?

Are there organ specific treatments to treat alpha-thalassemia?

Treatment for alpha-thalassemia depends on the specific type an individual has. Hemoglobin Bart is the most severe. Hemoglobin H can range from mild to severe. Carriers may experience mild anemia or no symptoms at all.

Fetuses with hemoglobin Bart typically do not survive beyond the newborn period. A few have received transfusions while in the uterus or immediately after delivery and have survived to five years of age. These fetuses are monitored prenatally by ultrasonography and as children require regular transfusions. They usually have serious symptoms including birth defects of the heart, limbs and genitals, and delays in development. The mothers of these infants often experience maternal complications including severe anemia, preeclampsia, an abnormal amount of amniotic fluid, and a difficult delivery. Because of these serious complications, parental diagnosis and termination of affected pregnancies are usually considered.

For those with hemoglobin H disease, supplementation of folic acid is generally recommended, especially in children, pregnant women, and women who may become pregnant. Because affected individuals can develop vitamin deficiencies (having too little of a specific vitamin), a non-iron containing multivitamin is usually recommended. Patients should avoid oxidative compounds and medications, and a high iron diet. Any infections should be treated promptly. There should be ongoing monitoring of growth, bone health, spleen size, leg ulcers, fatigue, and inflammation of the gallbladder.

During pregnancy, women with Hemoglobin H disease should be monitored for potential preeclampsia, congestive heart failure, threatened miscarriage and worsening anemia. Children should be monitored for growth and development every 6-12 months.

Usually blood transfusions are not necessary for individuals with Hemoglobin H disease. However, over time symptoms such as poor cardiac function, fatigue, anemia, poor growth, poor weight gain and bone changes, such as enlargement of the upper jaw, around the eyes and forehead, may worsen and indicate the need for a blood transfusion. The ages at which these symptoms worsen, if at all, depends on the severity of disease in each individual.

Individuals with alpha-thalassemia experience anemia. Patients should be watched for anemia particularly closely if they have an infection, a fever, an enlarged spleen, or are pregnant. Patients can experience a hemolytic crisis, which is a rapid destruction of red blood cells. During a hemolytic crisis, patients require immediate medical treatment, possibly including a blood transfusion. During a hemolytic crisis and transfusion adequate hydration and fever control is important. The cause of infection should be identified and treated. Patients who receive blood transfusions, and even those who do not, may develop iron overload, as determined by measurements of liver iron. These patients may require chelation therapy, treatments to rid the body of excess iron. Patients should be monitored for iron overload and hemoglobin levels at least annually.

For those with an enlarged spleen, having the spleen removed may improve symptoms. Risks associated with this include blood infection and blood clots. Aspirin is recommended for those who undergo this surgery to control clotting. For those with persistent gallstones that do not respond to antibiotics or surgical removal of the stones, removal of the gallbladder may be indicated.

Those who are carriers of alpha-thalassemia may have mild anemia, which would need monitoring and potentially treatment.

References
I have alpha-thalassemia hemoglobin H disease. How do I know if I should have my spleen removed?

Patients with hemoglobin H disease frequently have an enlarged spleen. Fewer individuals with hemoglobin H disease are choosing to have their spleen removed because of potential blood clotting complications. Having the spleen removed is considered if the patient has an increased need for blood transfusions and medication can no longer regulate iron levels. Having an enlarged spleen does not necessarily mean it must be removed, but it should be monitored.

References
I have hemoglobin H disease. How do I know if I should start having transfusions?

The decision to have transfusions depends on signs that the body can no longer tolerate low levels of hemoglobin. These symptoms include heart exams showing increased cardiac effort, fast heart rate, sweating, poor eating and poor growth. Less commonly, the shape of the bones of the face can change or the spleen is enlarged. Deciding to have regular transfusions is a decision to be made between doctor and patient and is based on symptoms, hemoglobin levels and quality of life with and without regular transfusions. Patients can try having regular transfusions on a trial basis to help decide if that is the right decision for them.

References
If I have alpha-thalassemia, what should my doctor know about managing the disease?

Treatment for alpha-thalassemia depends on the specific type an individual has. Hemoglobin Bart is the most severe. Hemoglobin H can range from mild to severe. Carriers may experience mild anemia or no symptoms at all.

Genetics professional, such as a geneticist or genetic counselor, can coordinate testing, interpret results, and provide information about treatment and risk to other family members. Genetic counselors can refer their patients with alpha-thalassemia to the appropriate specialists and for appropriate testing.

Fetuses with hemoglobin Bart typically do not survive beyond the newborn period. A few have received transfusions while in the uterus or immediately after delivery and have survived to five years of age. These fetuses are monitored by ultrasonography and as children require regular transfusions. They usually have serious symptoms including birth defects of the heart, limbs and genitals, and delays in development. The mothers of these infants often experience maternal complications including severe anemia, preeclampsia, an abnormal amount of amniotic fluid, and a difficult delivery. Because of these serious complications, prenatal diagnosis and termination of affected pregnancies may be considered. A genetic counselor can help with this decision. A maternal fetal medicine specialist should follow these pregnancies to manage complications.

For those with hemoglobin H disease, supplementation of folic acid is generally recommended, especially in kids, pregnant women, and women who may become pregnant. Because affected individuals can become low on certain vitamins, a non-iron containing multivitamin is usually recommended. Patients should avoid oxidative compounds and medications, and a high iron diet. Any infections should be treated promptly. There should be ongoing monitoring of growth, bone health, spleen size, inflammation of the gallbladder, leg ulcers and fatigue. Internal medicine specialists should be consulted for any organ damage. A physician can help ensure patients do not take medications they should avoid. A dietician can help with avoiding iron overload and getting proper nutrients.

During pregnancy, women with Hemoglobin H disease should be monitored for potential preeclampsia, congestive heart failure, threatened miscarriage and worsening anemia. These women should consult with a maternal fetal medicine specialist for potential complications. Children should be monitored for growth and development every 6-12 months.

Usually blood transfusions are not necessary. However, over time symptoms such as poor cardiac function, fatigue, anemia, poor growth, poor weight gain and bone changes, such as enlargement of the upper jaw, around the eyes and forehead, may worsen and indicate the need for a blood transfusion. The ages at which these symptoms worsen, if at all, depends on the severity of disease in each individual.

Those affected with alpha-thalassemia experience anemia. Patients should be watched for anemia particularly closely if they have an infection, a fever, an enlarged spleen, or are pregnant. Patients can experience a hemolytic crisis, which is a rapid destruction of red blood cells. During a hemolytic crisis, patients require immediate medical treatment, possibly including a blood transfusion. During a hemolytic crisis and transfusion it is important patients receive adequate hydration and fever is controlled. The cause of infection should be identified and treated. Patients who receive blood transfusions, and even those who do not, may develop iron overload, as determined by measurements of liver iron. These patients may require chelation therapy, treatments to rid the body of excess iron. Patients should be monitored for iron overload and hemoglobin levels at least annually. A hematologist should be consulted to manage blood health.

For those with an enlarged spleen, having a splenectomy may improve symptoms. Risks associated with this include blood infection and blood clots. Aspirin is recommended for those who undergo this surgery to control clotting. For those with persistent gallstones that do not respond to antibiotics or surgical removal of the stones, removal of the gallbladder may be indicated. An internal medicine specialist should be consulted for organ damage.

People who are carriers of alpha-thalassemia may have mild anemia, which would need monitoring and potentially treatment.

References
  • Vichinsky, E. (2012). Advances in the treatment of alpha-thalassemia. Blood Reviews, S31-S34. http://www.sciencedirect.com/science/article/pii/S0268960X12700103
  • Origa, R., Moi, P., Galanello, R., & Cao, A. (2013). Alpha-Thalassemia. GeneReviews at GeneTests Medical Genetics Information Resource (database online). Copyright, University of Washington, Seattle. 1997-2013. Available at https://www.ncbi.nlm.nih.gov/pubmed/20301608
  • Piel, F. & Weatherall, D. (2014). The alpha-thalassemias. The New England Journal of Medicine, 1908-1916. doi: 10.1056/NEJMra1404415

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