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Management of Sickle Cell Disease in Pregnancy

A group of disorders that affect hemoglobin is called Sickle Cell Disease. The hemoglobin present in red blood cells carry oxygen to the entire body. The red blood cells are round and flexible move easily through blood vessels in healthy individuals. However, in Sickle cell disease, these red blood cells become hard, sticky, and C-shaped (Sickle )  thus blocking the blood flow to the entire body. 

Sickle cell disease is a group of blood disorders that is a lifelong illness. It is an inherited blood disorder. When the healthy red blood cells with oxygen don’t flow through the body become anemic and the risk of infections, organ damage also increases. It is the most common blood disorder that is inherited and it commonly affects the tribal population in India.

Sickle Cell Disease in Pregnancy 

Women with sickle cell disease need extra care before and during pregnancy. Sickle cell disease leads to various complications during pregnancy but these days due to improvement in healthcare facilities the outcomes are much better than before. 

Pregnancy with sickle cell disease could worsen the health of both mother and unborn baby. Thus regular screening and careful monitoring of mother and baby during pregnancy by healthcare providers are important for a better outcome. 

Sickle cell disease is a lifelong and prolonged form of illness thus women trying to conceive should take medical help before planning to conceive. 

During pregnancy after the second month, compulsory tests are advised by healthcare professionals to find out if the baby would have sickle cell disease or not. 

Causes 

Sickle cell disease is an inherited disorder and is caused genetically that is it is passed from parent to child. Sickle cell disease is passed genetically to a child when both the parents pass the gene change to the child. 

If the father is not having the sickle cell trait but the mother is having sickle cell disease, the disease is not passed to the baby. However, various tests are conducted after the second month of pregnancy to confirm the diagnosis. 

Symptoms of Sickle Cell Disease in Pregnancy 

  • Pregnant ladies with sickle cell disease present one or more symptoms – 
  • Anemia is the most common symptom that could lead to weakness, fatigue.
  • Pain in joints increases and becomes more frequent.
  • Dull Pain in organs like the stomach occur more frequently 
  • Pain could last from a few hours to days.
  • Pain could also occur in the arms, legs.
  • Yellowing of the skin, eyes  (Jaundice) is also one of the common symptoms of sickle cell disease.
  • The frequency of infections often increases during pregnancy 
  • Some pregnant moms also face vision problems.

Diagnosis of Sickle Cell Disease During Pregnancy 

Screening of pregnant mothers with sickle cell disease is important to look at if the baby is at risk for sickle cell disease.

The Diagnosis is Done By –

  • Taking complete Family history  for sickle cell disease 
  • A blood test is important to identify defective hemoglobin in the blood and to determine if the baby would bear with sickle cell disease. 
  • Hemoglobin electrophoresis a newborn screening test is done to identify sickle cell disease in a baby. 
  • Newborn screening is done immediately after birth by heel prick test in which blood sample is drawn from heels of infant, the sample is sent to a laboratory to confirm the diagnosis.
  • By using the placenta tissue sample the baby’s genetic abnormalities are identified. 
  • Kidney function and liver function tests 
  • Retina screening to identify issues with vision 

Risk Factors For Sickle Cell Disease 

The risk for having sickle cell disease increases if there is an incidence of family history for this disease. 

Thus if you are planning for pregnancy and you have a family history of sickle cell disease careful screening and monitoring during pregnancy is important to prevent complications and to achieve a better outcome. 

Complications in Pregnancy with Sickle Cell Disease 

There are various associated risks and complications for mother and unborn baby both during pregnancy with sickle cell disease.  The complications observed are there is increased risk of miscarriage, the incidence of having low birth weight baby, severe pain to mother, infections, fetal growth is not proper and maternal mortality. 

In most pregnant ladies with Sickle cell Disease pain the duration and severity of pain in joints and organs increases. 

Management of  Sickle Cell Disease in Pregnancy 

  • Careful screening, monitoring, and management of sickle cell disease in pregnancy are important. Early diagnosis and the right treatment is the key to getting better results.
  • The management is done by a team of specialists who are well aware of dealing with sickle cell disease. The team includes obstetricians, hematologists, and skilled nurses. 
  • Women with sickle cell disease should be well informed about how sickle cell disease could affect their pregnancy, what the possible complications that could arise both for mother and baby.
  • Regular visits to your specialist are important if you have sickle cell disease during pregnancy. 
  • A daily dose of folic acid tablets is usually advised by a specialist to maintain the red blood cells. 
  • Iron tablets are also advised if the mother is having severe iron deficiency anemia. 
  • It is important to prevent the mother from dehydration (lack of fluid ) thus maintaining fluid balance is important. 
  • A healthy, nutritious, and well-balanced diet plays a very important role in pregnant ladies with sickle cell disease. 
  • Overexertion should be avoided to prevent fatigue and weakness.
  • Blood transfusion is required in pregnant ladies with sickle cell disease, although it is not recommended on a regular basis, it should be done under proper supervision. 
  • Pregnant ladies with sickle cell disease often experience severe and recurrent pain in the legs, arms, joints, or organs. Analgesics (pain killers ) are advised under moderate doses under the proper supervision of doctors. 
  • A team approach with proper care should be followed at the time of delivery of a pregnant lady with sickle cell disease. 

Know More – Bone Marrow Transplant and Sickle Cell Anemia Treatment In India

Final note – Although, most pregnancies with sickle cell disease often lead to many complications in the mother, child to be born, or both. But careful screening and management under the knowledgeable team of specialists could surely prevent the risks, complications associated with sickle cell disease. 

The hospitals should follow all the necessary protocols and national guidelines in pregnancies with sickle cell disease.  

 

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