Joseph is a sweet and rambunctious almost-three-year-old who loves watching Curious George on repeat, sneaking into our bed in the middle of the night, and spending hours playing in the sandbox.
He also happens to have Sickle Cell Disease (SCD), a blood disorder he was born with.
Living with Sickle Cell is just part of Joseph’s life. It’s something we try not to dwell on. Sometimes it’s even easy to forget that he has it. Other times, I feel myself going down that rabbit hole of worry and what-if’s.
Today is World Sickle Cell Day, and I’ve been inspired by other mamas with kiddos who have SCD to share a little bit about what our kids bodies are going through on the inside, even though they might look super healthy from the outside. I didn’t know much about SCD until becoming Joseph’s mom. When Joseph came home, we knew that Sickle Cell was a possibility for Joseph, but the first doctor he saw in the U.S. took one look at him and said he didn’t need to test Joseph because he “looked healthy.” Still, I advocated for a blood test, and that day I went home with a giant binder — “What to Know When Your Child Has Sickle Cell Anemia.”
So…What is Sickle Cell? Healthy red blood cells are round — these move through blood vessels carrying oxygen to all of our body parts. In SCD, these cells become hard, sticky, and look like a C-shaped farm tool called a “sickle”. Sickle cells don’t last long, which causes a constant shortage of red blood cells. Sickle cells can get stuck in small blood vessels and block the flow of blood and oxygen to organs in the body. These blockages cause serious pain episodes, organ damage, serious infections, or even stroke. SCD affects everyone differently — some people only experience a few symptoms, others suffer daily.
This is sweet Emmy, who also has Sickle Cell and was adopted at three weeks old. I met her mom online, and we now have a group for adoptive moms of kids with sickle cell. They also all happen to love Jesus. It has been such a blessing to have them as a community. Here, Emmy is on her way to get a blood transfusion. She is so brave! Emmy has had a rough time with SCD. Her first admission was 5 weeks old, and she has had over 50 admissions in 3 years. I pray for this little lady a lot!
What Causes Sickle Cell? SCD is an inherited, lifelong blood disorder that people are born with. (It’s not contagious in any way.) It’s inherited in the same way that people get the color of their eyes, skin, and hair. Because of the type of SCD Joseph has, we know that he inherited two genes for sickle hemoglobin— one from each birth parent. SCD is a genetic adaptation that actually prevents malaria, so it makes sense that SCD is most common in people whose families come from areas where malaria would be prevalent: Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries, India, and Saudi Arabia. In the U.S., SCD affects between 70,000–100,000 people, mainly African Americans. It occurs in about 1 out of every 500 African American births. In Nigeria, where Joseph was born, it affects 2 out of every 100 births.
What are Common Complications of Sickle Cell? A “crisis” happens when sickle cells get stuck and clog blood flow. This causes pain that can start suddenly, be mild to severe, and last for any length of time. When Joseph recently had his first pain crisis, his knees hurt so badly that he couldn’t put weight on them and wanted to be carried up the stairs. For those of you who know Joseph, you know that he must have been really hurting, because he’s an independent dude who loves running and jumping and climbing! A few calls to his pediatric hematologist (blood specialist) and some prescription Tylenol helped get him back on track. It is so hard to see your kid hurting!
Infants and children with SCD are more likely to experience harmful infections such as flu, meningitis, and hepatitis. A tiny cold hits Joseph a lot harder than it might hit you or me. If Joseph’s temp reaches 101, it’s considered a “medical emergency” and he has to be seen in the E.R. immediately. People who have SCD may get infections easily and have a hard time fighting them because SCD can damage the spleen, an organ that helps fight infections. Young children who have damaged spleens are more likely to get serious infections that can be life-threatening within hours or days.
It can be really scary for us when we’re in the E.R. — can you imagine how terrifying it is for him? We are so thankful that Joseph has only had to be admitted to the hospital once from these E.R. visits. (Seriously, PTL!) Since things can get really serious from something as simple as a cough, we are cautious when it comes to being around people who are sick. I think we’ve had some family members roll their eyes when we’ve declined a visit because one of them was sick, thinking we were being over-protective parents. We can’t live in a bubble, but I can’t tell you enough how important it is to let us know if you are under the weather! It’s not that we don’t want to see you — we do — it’s that we want to keep our son out of the emergency room.
At Joseph’s hematology appointment last week, a Child Life Specialist used a teddy bear to demonstrate what it looks like to get blood drawn.
What Can Be Done to Help Sickle Cell? Until age 5, daily penicillin is given to most kids (including Joseph) who have SCD. He also takes a vitamin called folic acid (folate) to help boost red blood cell production. He gets all of his vaccines and has regular check-ups with his pediatric hematologist, too. (He had his most recent visit this week, where we learned he has gained about five pounds since November –he weighs a whopping 39 pounds now, putting him solidly in the 98th percentile. Seriously a miracle — usually kids with SCD have delayed growth.)
There are prescriptions available to help prevent pain, the sickling of the cells, and anemia, but Joseph’s symptoms haven’t been serious enough to need those, something we are beyond thankful for. It’s important for him to stay super hydrated and have a healthy diet (but it’s so hard to resist giving him a Pop-Tart with those puppy dog eyes!). We avoid high elevations, which can trigger a pain crisis, and try to avoid extreme temperatures. There’s no cure, but there’s hope.
Since SCD varies greatly, there are a lot of unknowns, but we take it one day at a time. I remind myself that God loves Joseph so much more than I do — and I love Joseph more than words can say. We feel honored and humbled to have the privileged to take care of Joseph — being his mommy is a blessing beyond words.
