Thalassemia and pregnancy with risk factors explained 2020

Thalassemia and pregnancy with risk factors explained 2020

Thalassemia and pregnancy with risk factors explained 2020

 

Thalassemia and pregnancy

Although female fertility is reduced in transfusion-dependent thalassemia, pregnancy is possible in some cases. Some women have been reported in the medical literature, most of whom have beta-thalassemia intermedia and some of whom have severe beta-thalassemia.

With the availability of assisted reproductive techniques and as advances in medicine further increase the quality of life and life expectancy of women with beta-thalassemia, the number of successful pregnancies in these women continues to increase. Given the condition of the mother, there are important considerations for both maternal and fetal health during pregnancy.

This section focuses on women with beta-thalassemia, with a particular focus on transfusion-dependent women. Some of the aspects listed below may also be important in women with alpha-thalassemia, especially those with hemoglobin H-permanent spring disease and requiring transfusion.
A woman with transfusion-dependent thalassemia who becomes pregnant should consult a doctor, including her hematologist, perinatologist, genetic counselor, and other professionals.

General considerations

Medical care for patients with beta-thalassemia will further improve over time, as will the approach to assisted reproduction. As these trends continue, more and more women with this condition will be able to consider getting pregnant. Maternal and fetal health issues should be considered and discussed prior to conception.

Other important aspects for some families included the availability of a support system for caring for the infant, given their mother’s medical needs. The life expectancy of mothers with potential thalassemia is also an important consideration for some families in deciding whether or not they want a child.

This is also related to the availability of social benefits for some families. Overall, with the support of the family and the comprehensive medical team, an increasing number of women with thalassemia will continue to have healthy pregnancies and babies.

Thalassemia and pregnancy: Maternal Health

It is important to note that physiological stress in pregnancy can potentially exacerbate thalassemia symptoms in pregnant mothers. The heart, liver, and endocrine system are particularly vulnerable in pregnant women with thalassemia. It is important to evaluate the functioning of each system before and during pregnancy.

Therefore, continuous care by a hematologist is required. A hematologist is essential to monitor the transfusion schedule, which tends to increase pregnancy, and the administration and administration of iron chelating agents and other drugs. A very vulnerable midwife or perinatologist also needs to check the health of the pregnant mother and her fetus.

Iron chelation

Removal of excess iron using chelating drugs is critical to the health and longevity of a thalassemic woman. The increased need for transfusion during pregnancy may increase the need for chelation therapy. However, the safety of desferrioxamine (Desferal) has not been established during pregnancy; it is not clear whether this drug poses a risk to the developing fetus.

Depending in part on the amount of iron-loaded in the liver, temporary discontinuation of desferrioxamine during pregnancy may be considered. Some suggest that pregnancy itself may also serve as a chelating agent for iron, as the developing fetus uses free iron uptake.

Although data on the use of Desferal during pregnancy are minimal, CHO has experienced two successful pregnancies in one major patient with beta-thalassemia. Because she had a severe iron burden, she received a heavy pregnancy Desferal through one port during both pregnancies.
Both infants have not previously experienced adverse effects as a result of chelation therapy.

Pregnant women with thalassemia should discuss the benefits and potential risks of continuing or discontinuing desferrioxamine during pregnancy with their physicians.

Organ function and transfusion requirements

Cardiac function and transfusion requirements

During pregnancy, the fluid component of the blood usually increases. This can increase the degree of anemia, leading to a more frequent need for blood transfusions. Increased anemia can also result in the heart having to work harder to deliver adequate oxygen to all tissues in the body.

Increased blood volume can also cause stress in the heart. In thalassemia, the heart may already be under stress due to the harmful effects of iron overload. Therefore, it is important to monitor heart function before and during pregnancy. Regular attendance at scheduled transfusion meetings is also critical to reduce anemia and reduce the work to be done by the heart.

Liver function

Before pregnancy, a liver biopsy may be performed to determine the extent of iron overload. This information can be useful in deciding whether to stop chelating iron. A liver biopsy may help determine if there has been damage due to iron deposits or previous hepatitis infection. Blood tests during pregnancy can also assess liver function.

Endocrine function

Patients with thalassemia have an increased chance of developing insulin-dependent diabetes due to iron overload. Pregnancy stress can worsen this condition, which can be detrimental to the health of the mother and the developing baby. It is important to stabilize diabetes before pregnancy and to maintain appropriate treatment during pregnancy. Thyroid function can be detrimental due to iron burden in women with thalassemia.

Splenic function

The spleen expels anomalous red platelets from the circulation and performs significant immune functions. People with thalassemia have an unusual amount of red blood cells. The spleen makes these cells very active. This activity increases the spleen and makes the removal of even more cells even more effective and can cause hemolytic anemia.

During pregnancy, there is a greater need for hemoglobin for the normal growth and development of the fetus and due to the fact that the mother’s blood volume increases dramatically. During this time, the need for a transfusion for a pregnant woman increases, especially during the last trimester of pregnancy.

If the transfusion is adequate, the bone marrow will be suppressed and the work of the spleen may be reduced. Sometimes this leads to some reduction in spleen size and activity.

Nutritional needs

Pregnant women regularly receive prenatal vitamin supplements with OB / GYN. Many of these accessories contain iron. Pregnant women with thalassemia should avoid iron-containing prenatal vitamins for which iron overload is a concern.

Folate: Folate (folic acid) is important for cell growth and division. Therefore, the demand for folic acid increases during pregnancy. Folate supplementation is recommended within one month of conception and at least 8 weeks of pregnancy. It is thought to help prevent megaloblastic anemia (anemia marked by immature red blood cells) in women with thalassemia.

Vitamin C: Vitamin C (ascorbic acid) is given during iron chelation (usually 100 to 250 mg at each stage of chelation) to increase iron removal. High doses of ascorbic acid can release large amounts of ionized iron and can actually cause tissue damage, especially in the heart. Ascorbic acid should be continued by chelation during therapy. Higher doses should be avoided.

The health of a mother with thalassemia is related to the health of the developing baby. Therefore, the importance of comprehensive thalassemia treatment during pregnancy cannot be emphasized. In addition, a perinatologist and genetic counselor are available to address issues specifically related to the baby’s health.

Fetal risk due to thalassemia

Women with beta-thalassemia or hemoglobin E / beta-thalassemia may be at risk of inheriting thalassemia or other inherited blood diseases due to the hereditary nature of the fetus. This depends on the type of hemoglobin in the baby’s father. The fetus can be endangered if the father himself suffers from thalassemia or sickle cell disease. More generally, the fetus is at risk if the father is a carrier of the beta-globin property.

These properties may include the beta-thalassemia property, the hemoglobin E property, the sickle cell property, or others. The genetic counselor can arrange for the baby’s father to be examined and explain the inheritance of hemoglobin types.

If a couple is found to have a child with thalassemia or other inherited blood disorders, a genetic counselor can explain this risk as well as try out the options before the baby is born. The tests are available as early as 10 weeks of pregnancy. These tests can often show with high accuracy whether an infant has thalassemia. Because these tests are invasive in nature, they are not risk-free.

The genetic counselor can explain the risks and benefits of the above tests. Rejecting or rejecting prenatal testing is a very personal decision that depends on each couple’s beliefs and values. A genetic counselor can help a couple identify their own issues that may be important in the decision-making process both before and after prenatal tests.

Risks associated with maternal thalassemia

Thalassemia and pregnancy

According to some studies, maternal chronic anemia can lead to a lack of oxygen supply to the fetus. Such hypoxia may be associated with an increased incidence of fetal slow growth in the uterus, loss of a pregnancy, and preterm birth and childbirth.

Other studies in which maternal anemia was well treated have not provided evidence of an increased risk of these complications. This again emphasizes the importance of continuous thalassemia treatment during pregnancy.

Women with thalassemia tend to be smaller in stature than their siblings who do not have thalassemia. In women with thalassemia, adequate pelvic bones may contribute to an increased chance of imperial childbirth, which was considered the only labor complication in these women.

Although any woman can develop diabetes, women with thalassemia are more likely to do so due to iron overload. Maternal diabetes, especially the insulin-dependent type, is known to increase the risk of birth defects.

Maternal diabetes increases the risk of prenatal and neonatal complications, including perinatal loss. Proper blood sugar control, especially in early pregnancy, and close monitoring by a perinatologist during pregnancy are known to help reduce these risks.

Risks associated with medicines

Genetic counseling is an important source of information about the harmful effects of maternal medications on the developing fetus. It is important that all pregnant women consult their doctors before taking any medication.

The potential risks to the fetus depend on the type of medicine, the dose and the period of pregnancy in which the exposure occurs. Certain medications may also be present in breast milk and pose a risk to the newborn. A genetic counselor or perinatologist can provide information about the risks to each woman.

A woman with thalassemia may also take one or more medications, including iron-chelating agents or antiviral medications, to treat previous infections. The known or theoretical risks of these drugs should be taken into account when determining whether to continue them during pregnancy or to change the dose.

Desferal is the most commonly used drug among these women because it plays an important role in reducing iron overload and its consequences. The potential for adverse effects is suggested by animal studies in which skeletal abnormalities are observed at doses comparable to human doses.

Less than 40 cases have been published among pregnant women describing the outcome of pregnancy in women receiving chronic deferral treatment. None of the women who were pregnant took Desferal after the first trimester.

There was no evidence in these infants to suggest adverse effects of Desferal. Other cases have been reported in which treatment with Desferal was initiated in late pregnancy after an iron overdose. At least one of the infants has been reported to have iron deficiency due to maternal Desferal treatment. Overall, the number of cases reported in women is insufficient to establish the safety of Desferal during pregnancy.

As far as the safety of breast-feeding is concerned, Desferal is unlikely to have any adverse effects as it is poorly absorbed in the adult gut. However, no information is available on the use of Desferal during breast-feeding. Therefore, the expected benefits and risks of therapy during pregnancy or lactation should be discussed with your doctor for each woman. This is true of any additional medication given to pregnant women with thalassemia.

Risks related to infectious agents

Screening for infectious pathogens is an important part of prenatal care for all pregnant women. Certain types of maternal infection carry the risk of spreading to the fetus or may have other adverse health effects on the fetus.

These effects, as well as the chance of fetal or perinatal infection, depending on a number of factors: the type of pathogen, the severity of the maternal infection, the amount of virus or other infectious substance, the stage of pregnancy, and the method of delivery. Information on the special risks for women may be sought from a genetic counselor and/or perinatologist.

Although the safety of our nation’s blood supply has increased dramatically in recent years, blood transfusions remain a risk factor in acquiring certain infections. Women with thalassemia who are planning to become pregnant should be tested for all forms of hepatitis and HIV infection before conception.

If one of these infections is identified, specific information is available on the health risks and chances of transmission to the developing fetus. In some cases, preventive treatment or alternatives to childbirth may be considered to reduce the risk of the baby spreading.

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