SCD, RBC, and Everything in Between

Adrian Daniel “EHHH DEEEEE” alexander,

How is it that this is my first letter addressed to you? One of the reasons is clear. I haven’t done a ton of writing since you were born. I’ve started 2 letters and finished another. You have received a mention but not a letter of your own. The other reason, and the most powerful one, is that I haven’t been sure what to say. As I’m writing now, I’m still not completely sure. But I have a Guide, and I believe He will get us to where we need to go.

So, you were born with a blood disorder called Sickle Cell Disease. In a nutshell, that means some of your red blood cells (RBCs) are shaped differently. Red blood cells are typically circular in shape, like a full moon. And you have some RBCs like that, but you also have some that are shaped like a crescent moon aka the “sickle moon”. Think about your toenail clippings, smooth and thin in the middle but coming to curved, sharp points on each end.

Now, the job of a red blood cell is to carry oxygen from the lungs throughout the body. Oxygen in blood has the same purpose as oxygen in the air; it facilitates breathing. We breathe oxygen into our lungs, that oxygen hitches a ride on RBCs to all areas of our bodies so that our cells can breathe too. Every organ in the body relies on oxygen to do their job. Without it, they don’t function properly and can be damaged. 

This is a sickled red blood cell.

The shape of RBCs has an impact on how our blood moves through the body. Sickled cells can restrict the flow of non-sickled cells because of their shape. Think of a lazy river at a waterpark. The intertubes are circles. That allows for a mostly smooth flow. Circles don’t grab onto others circles, they bump and keep it moving. Circles can however get stuck on shapes with points like a sickle, causing a traffic jam. An RBC traffic jam can be painful as it stops the flow of blood and oxygen to where it is needed. That is called a pain crisis. To our knowledge, you've never had a pain crisis. Praise God! But your sickled cells impair your immune system. That leads to easier contraction of illnesses and a tougher time dealing with them when they occur.

You've been through a lot for a 3 year old. For about your first 18 (or so) months on the planet, we had to take you to the hospital every time you ran a temperature of 100 degrees or higher. You had more IVs in your first year of life than I've had 38. There were times where I've had to tell medical staff (with the best intentions) that you're not a pin cushion. It got to the point where mommy and I wouldn't allow an IV to be placed without an ultrasound team. They have this cool machine that can see through your skin to your veins. Technology is really dope, all things considered. The day before your very first birthday, you were admitted to the hospital. We were there for a 4-day hospital stay due to a battle with acute chest syndrome.

That’s an explanation of what sickle cell disease is and some of the health concerns and consequences that come with it. But it was the combination of that knowledge plus how one inherits the disease that really flipped my world upside down. Mommy carries the sickle cell trait, which means she can pass it on to babies we make together. Your grandparents on Mommy’s side also carry the trait. Both your brother and sister carry the trait. But I was under the impression that I did not. Your diagnosis was the news that informed me of otherwise. It sent me down a genealogical spiral that still blows by to spin my mind periodically.

For my first 30 years of life, I only knew of sickle cell anemia as something that T-Boz from TLC had. Completely detached, no real concern. Then, your brother was born with the trait. I started paying a little more attention, but not a ton. 17 months later, your sister was born with the trait. I start to dig in a bit. I researched what it means to have the trait, what concerns I should I have about my kids, what precautions are helpful and/or needed? Then, you were born…

You had been breathing outside air for less than 24 hours before the doctor told us you had sickle cell disease, SS to be specific. I had done enough research at this point to know this was a really big deal! BUT I also knew the diagnosis was impossible. “I don't have the trait. What are the odds of the test being a false positive?” The doctors advised it to be very slim, but possible. So I schedule a blood test. After the longest 3 weeks of my life, BOOM, positive test result for sickle cell trait. 

I'm devastated at this point. What had I brought upon you? How did I let this happen? How did I not know? Imagine living 35 years without knowing you had a genetic trait with catastrophic potential. Your diagnosis was exclusively my fault. No one else was to blame but me. I would carry the burden of your condition and the genesis of so many questions about my own genetic make up ALONE for awhile. Then, I started playing the blame game with others. I didn't know because I wasn't told! Who did the gene come from? Did they not know? Your mother was educated about her trait before she was in double digits. Who failed me, and in turn, failed you? 

My anger set in and I harbored it! I allowed it to grow and grow and grow. Every time we had to rush off to the hospital in the middle of the night, every time you had to be poked with a needle, every time the nurse would miss your little baby veins and have to “try again” to get the IV in, that anger would grow. I was mad at myself, mad at family, and mad at the rest of the world.

I was a little mad at God Himself but in all honesty, my anger could and would still be growing today if I had not given your sickle cell disease over to Him. I wasn't doing anything with that anger. Bottled and shelved. No time, no desire, no energy.  Life was busy enough that it just numbed my emotions. I didn't do much exploring of my genetics. I didn't “confront” anyone or request blood tests. But, I was at church one morning and a member of the worship team reminded me that God is in control. She said “sickle cell disease will bow” between worship songs. That was my wake up call. And in short, I've been awake ever since.

Part 2 coming soon!