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Hello Friends!! Happy September. This month in honor of sickle cell awareness month, we are diving into the topic of the sickle cell disease.  What it is? How to manage it? How to prevent it in children and much more.

Now what exactly is the Sickle Cell disease?

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Sickle Cell is an inherited blood disorder where red blood cells become sickle-shaped and harden. To be born with sickle cell disease or trait, both parents have to be carriers of the trait or have the disease. Currently, there is no current cure for sickle cell disease. About one in ten African Americans are carriers for the trait, but other ethnicities carry the trait, like Caribbeans, Hispanics, and South and Central Americans.

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What is the Sickle Cell Trait?

Sickle Cell Trait is when the A and S hemoglobins are produced in the red blood cells. Having the trait does not mean that you have the disease. Living with the trait, you can live a healthy, normal life, and most people do not even know they are carriers of the trait until they try to have children. To determine if you have the trait or the disease, you can take a blood test called Hemoglobin Electrophoresis, which looks at the different types of hemoglobin (blood) in your body.

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What are the symptoms of Sickle Cell Disease?

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The symptoms of sickle cell can differ from person to person. One of the most common symptoms people experience is pain. The pain comes from the blood flow blocked by the blood vessels' sickle cells. There are various reasons for the pain, but mostly, it is caused by oxygen being blocked by the sickle cell.  Main organs and tissues are not getting oxygen and causing pain. Anemia which is low iron causes a person to feel extremely tired and weak. Sickle cells can cause damage to other organs in the body like kidneys, liver, spleen, heart, and lungs. 

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Can I have Kids if I have Sickle Cell Disease?

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Can I have Kids if I have Sickle Cell Disease? This can be a difficult question to answer because it depends on the person. You can still have children, but it can come with difficulties. Males who have sickle cell disease can have lower sperm count and or testicular dysfunction. Women who have the disease might have a lower chance of conceiving and might have complications during pregnancy. With the advancement of medicine, many types of fertility treatment can help get pregnant. Women with sickle cell disease can still get pregnant, but there are different risks while pregnant; please make sure to check with your doctor for a healthy and safe pregnancy.

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Would my Children inherit the disease or the trait?

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Sickle Cell disease/ trait can be inherited to your children, like hair texture or color, blood type, physical traits, and eye color. Hemoglobin genes are inherited from both parents, and two sets are needed, one from each of the parents.

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Here is an example of the different outcomes:

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Couple 1: Mom has the trait, and Dad has the trait as well. There is a 50% chance their child can have the trait and pass it to their children.

Couple 2: Dad has the trait, and Mom does not. There is a 50% chance that one child will have the trait and a 50% chance the child will not.

Couple 3: Mom and dad both have the trait there is a 25% chance that one of their children will have sickle cell disease.

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What can I do if myself and m partner have the sickle cell trait to prevent the disease in my children?

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If you and your partner both have sickle cell trait, your child could have the disease or the sickle cell trait. Knowing that you and the person you plan to marry have the sickle cell trait can be a huge challenge because of all the risks. However, It's important to know if you and your partner have sickle cell traits. If you do, there's a 25% chance of having a child with SCD with each pregnancy. There's also a 25% chance of having a child with no trait or disease (normal genes) and a 50% chance of having a child with sickle cell trait.

If both of you are carriers and you plan to have a baby, talk to your GP about getting a referral to a genetic counselor, who can explain the risks to your children and your options.

Some of the options in this case include:

• having genetic tests during pregnancy to see if your baby will have sickle cell disease
• adopting a child
• trying IVF with you and your partner's egg and sperm to genetically select embryos without the full-blown sickle cell disease. AA - 25 % chance of 2 normal genes with normal red blood cells.AS - 50% chance of 1 normal gene and 1 sickle gene.
• trying IVF with a donor egg or sperm
• trying pre-implantation genetic diagnosis (PGD)

PGD is a treatment used in IVF where the resulting embryos are tested to check that they do not have sickle cell disease before they are implanted in the womb.

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Can Genetic Testing and IVF really help prevent Sickle Cell Disease in Children?

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Yes, genetic testing and IVF can help minimize the risk of your children having sickle cell disease/trait. Genetic testing is the most important step to see if either or both parents have the trait or the disease. IVF is used to help prevent passing the disease or the trait to your children. IVF is in vitro fertilization, where the eggs are removed from the ovaries and fertilized by injecting sperm into the egg. After six days, Preimplantation Genetic Diagnosis is completed, which is the embryo analysis to see if the genetic markers exist. For this step to be complete, a sample of both parents and family members is needed to help track the chromosomes of sickle cell in the family. Once the results are available, it will state which embryos are or are not affected by sickle cell. IVF helps with having a healthy pregnancy and prevents your children's chances of having sickle cell or sickle cell trait. 

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I have the disease? How can I properly manage it?

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Managing sickle cell means learning about the realities of living with sickle cell. These realities include the symptoms, the situations, and the feelings those living with sickle cell often face. Learn how to manage sickle cell with these tips and resources proactively.

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Building a foundation of healthy habits can help you live your life! People with sickle cell can have full lives and enjoy many activities. You can not only survive, but thrive, with sickle cell!

Living Well With Sickle Cell Disease

• Find good medical care.
  Sickle cell is a complex condition that can be difficult to understand. Good medical care from doctors and nurses familiar with the situation can prevent serious problems. Including a hematologist (a blood specialist) in your care plan is a good idea to ask your current healthcare provider about routine checkups. Always talk to your healthcare professionals about your symptoms and care plan.
• Prevent infections
  Common illnesses like the flu can be dangerous when you have sickle cell. Practice daily good hygiene with hand-washing and food safety.
• Develop healthy habits
  Hydration and nutrition are essential for people with sickle cell. Staying well hydrated may help prevent a pain crisis, so drink at least 8 to 10 glasses of water daily. Eat a well-balanced diet with plenty of calcium-rich foods like low-fat milk, yogurt, cheese, leafy green vegetables, and calcium-fortified foods like soy milk, orange juice, and tofu. It can also help add nutrient-rich, high-calorie foods like dried fruit, nuts, and smoothies.
• Maintain a balanced body temperature.
  Try not to get too hot or too cold. Physical activity should be part of your life but don’t overdo it.
• Get support
  Your family and friends can help you check your health and be there to listen to you. You should find a support group or community organization to provide information and support. Talking with people who know what you’re going through can make all the difference. It can provide a network of people to learn from.
• Talk with your healthcare provider about clinical studies that might suit you.
  New clinical studies about sickle cell are starting all the time. Ask your healthcare provider whether any studies might be suitable for you.

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