Pregnancy and Sickle Cell Anemia


Sickle cell disease is an transmissibled blood disorder which affects red blood cells. Sickle-shaped RBCs are quickly hemolyzed and have a life span of only 10-20 days (vs the normal 120 d).
People having sickle cell disease have red blood cells which contain mainly hemoglobin* S, an abnormal type of hemoglobin. It is featurised by pain episodes, anemia (shortage of red blood cells), serious infections and damage to vital organs. Occassionally these red blood cells become sickle-shaped (crescent shaped) and have trouble passing through small blood vessels. Deoxygenation of the heme moiety of HbS leads to hydrophobic interactions between proximate HbS molecules, that then cumulate into larger polymers, distorting the red blood cell (RBC) into the classic sickle shape. Hemoglobin – is the main component of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body.

Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are soft and round and can embosom through tiny blood tubes (vessels). Normally, red blood cells survive for about 120 days before new ones replace them. Patients who are homozygous for the HbS gene have full-blown sickle cell anemia. Patients who are heterozygous for the HbS gene are carriers of the condition. Under stressful conditions, carriers may display some clinical manifestations (eg, severe hypoxia). If both members of a couple are carriers, they have a 25% chances of producing a child who is homozygous for the HbS gene.

Sickle-shaped red blood cells also die and break down more rapidly than normal red blood cells, resulting in anemia. The effects of sickle cell disease differ eminently from one person to the next. People who have sickle cell anemia are born with it. It is a lifelong disease. Most people who have sickle cell anemia now outlive into their forties or fifties, or longer. A significant number of patients with the disease have few admittance and live productive and relatively healthy lives. Infectious exigences are due to underlying functional asplenia in most adults with sickle cell anemia. This is the most common symptom of sickle cell disease.

Some affected individuals have one or fewer pain. Pain episodes usually last a few hours to a few days, but they may betimes last for weeks. Hands and feet may swell when small blood vessels become blocked. This may be the first symptom of sickle cell disease in babies. If sickle-shaped cells block a blood vessel in the brain, a stroke can result. Around 10 percent of children with sickle cell disease have a stroke.

Sickle cell disease chiefly affects those of African descent and Hispanics of Caribbean ancestry, but the trait has also been found in those with Middle Eastern, Indian, Latin American, Native American, and Mediterranean heritage. In the US, it has been evaluated that 1,000 children are born each year having sickle cell disease. One in twelve African-Americans has sickle cell trait.

Women having sickle cell disease are at increased risk of difficulties which can influence their health and that of their babies. The pregnancies are at higher risk of unceremonious abortion, premature labor, pseudo-toxemia and cesarean section. During pregnancy, the disease may become more acute and pain occurrences may happen more frequently. There are significant fetal and maternal complications in pregnancies experienced by mothers with sickle cell disease (SCD) [1-8]. The increased metabolic demands, hypercoagulable state, and vascular stasis associated with pregnancy predispose to these complications.

Women who are pregnant with sickle cell have more frequent sickle cell crises due to the extra stress of pregnancy. A sickle crisis will occur in about a third of pregnancies. Pregnancy is an intense burden on a woman's body, and this incredible strain can easily exacerbate the sickling of red blood cells. When these cells cluster together, they can build up in different organs throughout the body, leading to intense pain. After the birth, all women are more likely to have infections and blood clots, but the risk for sickle cell mothers is even greater.

Treatment during pregnancy with Sickle Cell Anemia

Most Treatment available of pregnancy with sickle cell anemia. First is taking hydroxyurea. Second is folic acid is given routinely. Third is Cord blood transplant is a more widely available option for couples with a child who is severely affected by sickle cell disease. Other treatment is Early and regular prenatal care is important for pregnant women with sickle cell disease. General pregnancy care comprise a healthy diet, prenatal vitamins, and preventing dehydration. Some women may benefit from blood transfusions to replace the sickled cells with fresh blood.

These may be done several times during the pregnancy to help increase the blood's ability to carry oxygen and decrease the number of sickled cells. It is vital for women who receive blood transfusions to be screened for antibodies that may have been transferred in the blood and that may affect her fetus. During labor, intravenous (IV) fluids are given to help prevent dehydration. Most women will receive extra oxygen through a mask during labor and a fetal heart rate monitor is usually used to watch for changes in heart rate and signs of fetal distress. There are no special prescribtions for the type of delivery for women having sickle cell disease and most women can deliver unless there are other complications.

 

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