Sickle Cell Awareness Month: How it Affects Children and What You Need to Know About This Disease

September marks the start of Sickle Cell Awareness Month. Sickle cell disease is an inherited blood disorder that can cause serious medical issues for children. This is why it's important to know what this disease is and when you should take your child to their doctor if you suspect that they have sickle cell.

Children with this blood disorder need an early diagnosis to prevent life-threatening medical complications. Not every parent realizes the risk of this disease, this is why Sickle Cell Awareness Month was created. This month is all about spreading information about sickle cell, gathering funds for research and treatment, and telling stories about the children and parents that are dealing with sickle cell disease.

I found out that I had the sickle trait right after my 2nd son was born. During his newborn screening, they were able to test for rare health conditions. About a month after coming home from the hospital, I received a letter in the mail stating that blood tests indicated that he had sickle cell trait. I immediately panicked and called my doctor. He was then able to test me and it was proven that I also had sickle cell trait.

Honestly, I kind of figured that I was the one that carried the trait and not my husband because my cousin passed from the disease years ago. This is still a difficult subject to talk about, but I want to raise awareness because there are so many people that could have the trait and don’t even know it. Besides myself, I know people personally whose spouses didn’t even know they had the trait when their children were born. Needless to say, the children ended up having sickle cell disease. No one should have to deal with this childhood disease alone.

So, I’m kicking off Sickle Cell Awareness Month to help parents across the country…

My blog will discuss sickle cell disease in detail this September to help raise awareness and provide you with all the information you need to know about this disease. Stay tuned to find out the underlying causes of sickle cell, who exactly is affected by sickle cell, and what you can do to help improve sickle cell awareness, treatment, and research efforts.

What is Sickle Cell and What Causes the Disease?

Sickle cell disease is an inherited disease. What this means is that sickle cell is passed down to children from their parents. Specifically, this genetic condition changes the shape of red blood cells and causes other serious health complications.

You can’t catch sickle cell from someone else. People with this disease are born with it. It’s like other heritable traits such as eye color, hair color, and height. But sickle cell can be dangerous and even deadly when left untreated. What is the exact mechanism of SCD?

The specific cause of sickle cell comes in the form of genes. Children must inherit two sickle cell genes. One from their mom and one from their dad. When both genes are inherited, the red blood cells in the body begin to change. Kids with the disease develop red blood cells that are sticky, hardened, and C-shaped. The shape is where the name sickle cell comes from, as red blood cells look similar to a sickle.

Normal red blood cells are more flexible and circular and can live up to 120 days. This helps blood, oxygen, and other nutrients move around the body more easily and keeps kids healthy. With sickle cell though, red blood cells are less robust and only live up to 10 to 20 days.

The crescent shape of the cells and their lowered lifespan of them makes it harder for the body to get vital nutrients and oxygen. Over time, if left untreated, sickle cell can lead to blocked blood flow, damaged arteries and veins, and a major decline in health.

What is Sickle Cell Trait?

Sickle cell trait is not the same as sickle cell disease. I was a little worried about this diagnosis when I got my blood results back. My doctor said that while this is an inherited condition where kids and adults carry certain gene markers, the effects of sickle cell trait are less pronounced and not as deadly. More specifically, people with SCT only have one sickle cell gene. The other gene marker is normal which prevents them from having full-blown sickle cells. You should know that:

• You don’t have sickle cell if you only have one sickle cell gene

• With SCT you can still pass down the gene to your children in the future

• SCT is less debilitating as most people don’t have any symptoms of sickle cell, but there are some rare cases where some symptoms are present

• Anyone can get sickle cell or sickle cell disease, but this is most common in people with African American heritage

Since people with SCT are considered carriers of the disease, it's important to consult your doctor before you have children. If you pass on one gene of sickle cell to your child, and your partner also has the SCT, then your kids can get sickle cell disease. I suggest that anyone with a history of sickle cell in their family should consult a genetic counselor.

Who is Affected by Sickle Cell?

You might be wondering who has Sickle Cell. I was curious about this too and did a little digging. I found out that in the United States alone, around 100,000 people suffer from sickle cell disease. Most often, individuals with African American heritage will inherit sickle cell. In fact, around 90% of people with sickle cell in the U.S. are of African American descent. People of other ethnicities can still get sickle cell. Though it’s rarer, kids with South Asian, Southern European, Hispanic, and Middle Eastern backgrounds can get the disease.

Sickle cell genes began showing up in regions with malaria. Scientists say that having this gene helped fight malaria and gave people an evolutionary advantage. Unfortunately, sickle cells can cause other health issues, even though it protects people from malaria.

What Are the Complications of Sickle Cell?

If you are a parent that has a child with sickle cell you need to take this diagnosis seriously. This disease can be managed with medication and the right lifestyle. But if your child does not get an early diagnosis or these issues are ignored their condition can worsen and the following health complications can occur:

1. Acute Chest Syndrome

Acute chest syndrome is one of the most common complications associated with sickle cell. This is a life-threatening condition that causes infection, fever, chest pain, and other issues. The red blood cells clump together in the chest making it hard to breathe and blocking off oxygen flow.

2. Stroke

Stroke is another serious complication of sickle cell. The blood flow in the brain can get blocked because of the abnormal shape of the blood cells. Even children can get a stroke. So look out for the signs which can include numbness in the arms and legs, speech issues, paralysis, and difficulty walking. Strokes are deadly and can result in brain damage so don’t wait to see a doctor.

3. Pulmonary Hypertension

This effect is usually seen in adults with sickle cell. Pulmonary hypertension, also known as high blood pressure, can lead to an increased risk of heart disease and blood vessel damage over time.

4. Splenic Sequestration

When sickle cells get trapped in the spleen, this can cause spleen enlargement and serious bouts of pain. Parents should check their child's spleen and schedule regular visits. By the age of eight, most kids don’t have a functioning spleen or they need to have their spleen removed. Spleen enlargement is a major risk for infection in children so don’t ignore this complication.

5. Organ Damage

One of the most dangerous complications associated with SCD is organ damage. Since the body isn’t getting enough oxygen, various organs in the body can become oxygen-deprived and become damaged. This includes the kidneys, brain, lungs, and heart.

6. Permanent Blindness

Sickle cell disease affects the eyes as well. Without proper oxygen flow, the eyes can get damaged which may lead to blindness.

7. Leg Ulcers

Children with SCD can also develop open sores and painful ulcers. Which makes pain management a must.

8. Deep Vein Thrombosis (DVT)

Finally, the risk of DVT, also known as deep vein thrombosis is increased with this disease. Red blood cells stick together and create dangerous clots that can break off and block blood flow. This can lead to sudden death.

Is There A Cure For Sickle Cell?

It pains me as a parent to say, but at this moment, there is no cure for sickle cell disease. Researchers and scientists are still searching for ways to cure sickle cell and return red blood cell production to normal. Considering this, research and funding for the disease are crucial!

Treatment For Sickle Cell/Daily Maintenance

While there is no cure for sickle cell yet, your child’s doctor can help alleviate symptoms and keep your child healthy. Again, diagnosing sickle cell is crucial with this disease.

You want to prevent complications by going to the doctor regularly and looking out for dangerous health issues. Treatment options do vary, but for the most part, they include a healthy lifestyle and medication prescribed by a medical professional and other approaches. I suggest doing the following things:

1. Keeping a Healthy Diet and Taking Supplements

Folic acid, multivitamins, and other supplements can help the body create more blood cells and keep bone marrow healthy. It's best to talk to your child’s doctor about any supplements you plan on giving your kid. Definitely do some research on folic acid and other supplements. In addition, your child should eat a healthy well-balanced diet to prevent serious sickle cell complications.

2. Drink Water

When the body is dehydrated this can also increase the risk of sickle cell-related complications. Make sure your child is drinking at least eight glasses of water a day. If they are involved in after-school activities or sports, they should drink more water.

3. Stay in Temperate Areas

Extreme temperatures can also lead to further issues with sickle cell. Children shouldn’t be in extreme heat or cold weather.

4. Moderate Amount of Exercise.

Your child should exercise regularly to keep their blood flowing and their body in shape, but they should not overdo it. Talk to their doctor about what level of activity is appropriate for them.

5. Over-the-Counter Medication

The use of over-the-counter pain medications, like Ibuprofen and Aleve, can be helpful. But they should be used rarely as they can worsen kidney issues.

6. Prescribed Medication

Finally, prescribed medication will be given to your child. Usually, between the ages of 2 months and five years old, penicillin will be prescribed. This helps prevent infections and other serious complications. In some cases, children may stay on penicillin their whole life.

Hydroxyurea is another common medication used for this disease, as well. Doctors use this to prevent painful flare-ups of sickle cells. Hydroxyurea can also reduce the need for blood transfusions.

Life Expectancy For Children With Sickle Cell?

Sickle cell disease manifests differently for each person. Some people don’t have symptoms until they are adults and can end up living longer. However, other people with sickle cell can have symptoms immediately and might not live past childhood.

On average, people with sickle cell have a life expectancy of 42 to 50 years. This number will change depending on the individual and their symptoms.

How Can You Help?

Look out for the signs of sickle cell early on. The symptoms of sickle cell disease can appear as early as five months. Common signs include:

• Extreme pain

• Fatigue, muscle weakness, dizziness, fever

• Swelling in the joints

• Poor circulation and blood blockage

• Development of Jaundice

Final Thoughts

As a mother with sickle cell trait, I know just how important it is to educate yourself on this topic. My post provided a lot of the information you need to know about sickle cell. Parents who are dealing with sickle cell diseases or sickle cell traits need to stay in contact with their doctors.

Also, if you know that your family has a history of sickle cell disease, don’t wait for symptoms to appear. Early detection is important for this disease and you want your child to get the best possible care. This way they can live a more high-quality life!