Sickle cell disease also called sickle cell anemia is an autosomal recessive disorder. It is more likely to be a blood disorder passed from mother to child. This kind of blood disorder is very painful and intolerable for pregnant women with SCD(Sickle cell disease).
Why is sickle cell disease autosomal recessive?
It is called recessive. Because this disease is inherited from the parents. And the parents are responsible for the transfer of sickle cell disease to the child. This type of blood disorder is either SCD(sickle cell disease) or SCT( sickle cell traits).
Normally, the pregnant mother who has SCT delivers a healthy baby. And the mother with SCD needs to consult a genetic counselor.
Why does it occur?
The normal red blood cell contains globin protein. These are called hemoglobin.it carries oxygen to the body.
The healthy and normal red blood cells are round in shape. They are also very smooth and flexible. It looks like a full moon and circulates easily through the blood vessels.
But the sickle cell contains abnormal hemoglobin. They are like sickle shape. It’s like the letter c’.These abnormal cells are impacted on the capillaries and cause hemolysis with local anoxemia. This causes more pain and lack of oxygen supply to the tissues.
Causes of sickle cell disease
It is a genetically inherited disease that comes from the parents to the child through the gene. Genes basically come in pairs( one from father and one from mother). These are responsible for the growth and development of the baby.
But, sometimes the gene function becomes altered. It is called gene mutation. This is mainly responsible for birth defects and other health problems in the baby.
How do you know SCD or SCT will be passed to your child or not?
If the mother and the father both have sickle cell disease then the baby will surely suffer from it. but, if the mother and father have sickle cell traits then the results are quite different.
- There will be 75 percent chances that the baby won’t have sickle cell disease.
- 50 percent chances are that the baby has sickle cell traits.
- 25 percent chances that your baby has sickle cell disease.
- Another 25 percent chances are like, your baby has neither SCD nor SCT.
Effects of sickle cell disease in pregnancy
- Increased risk of abortion
- Risk of infections
- Preterm deliveries
- Preeclampsia and hypertension
- The risk for maternal mortality
- Intrauterine growth retardation
- Increased cardiac output
What are the normal symptoms of SCD?
The symptoms of this disease appear at 5 months of age. But, in some cases, it may vary from individual to individual.
So, in these cases the normal sign and symptoms are ;
- Recurrent pain
- Delayed growth and puberty
- There may be swelling of hands and feet
- Frequent infections
- Vision problems
- Non-healing ulcer
A complication of sickle cell disease during pregnancy
As it affects the body organs, there are several complications related to it.
The complications in pregnant women are like;
- Infection in the urinary tract, lungs, and kidney
The complications seen in a developing baby are like;
- Severe anemia
- Poor fetal growth
- Preterm birth
- Low birth weight
How to find out you are having sickle cell disease or sickle cell traits?
The tests that are very essential to diagnose SCD and SCT are very safe and helpful during pregnancy.
A swab is inserted into the mother’s mouth. And gently rub against the inside of the cheek. It helps to collect some cells. This technique is very useful to find out SCD.
Family history collection
It is a screening test of fetal cells in the maternal blood.
Black and Hispanic
In the United States, black people mostly suffer from sickle cell anemia. 1 in 500 live births and Hispanics 1 in 36,000 live births are having this. Also, the people having their ancestors from Saudi, Africa, India, the Caribbean or Central America carry SCD.
Treatment of Sickle cell disease during pregnancy
- Early diagnosis and regular checkups are very important for the pregnant mother having this disease.
- If the sickle cell trait is present in the baby’s father then amniocentesis is needed to check if the baby has the trait or not.
- If the mother has sickle cell trait then the doctor advises the father to do the prenatal screening.
- In sickle cell disease, some people need a blood transfusion to replace their abnormal cells with normal cells. but, in the case of pregnant women, it may affect the baby.
- During pregnancy, the physician does not advise taking hydroxyurea. a lower dose may be recommended.
- During labor, the doctor gives intravenous fluid therapy and oxygen therapy and also regularly checks the fetal signs.
Management of SCD
- Aspirin 100 mg/day
- Folic acid 5mg/day
- Vitamin supplementation
- Routine urine culture
- Parenteral fluid therapy