Okay I know alot of you are probably wondering, what the flip is sickle cell? Here’s my shortened personal version that should give you the skinny.
First of all, if you have the sickle cell trait, it means you are a carrier of the gene but don’t have the disease. Good for you. Now just do your future kids a favor and don’t get knocked up by someone who either has the disease or the trait and you won’t pass it on.
Sickle cell disease is a inherited blood disorder that affects about 300,000 people in the world with 80,000 of those people in the US. About 200,000 children are born annually with it—so yeah, folks, it’s not going anywhere. It’s the mutation of the hemoglobin gene, the body’s natural response combat malaria. When a mosquito shoots you full of plasmodium, sickle cells don’t hang on to that bacteria, so you don’t get malaria…that’s good thing right? Wrong!
Normal red blood cells (RBCs) are shaped like perfect little donuts that fit into the blood cells swimmingly. Sickle cells are shaped like sickles…little inverted C’s. When the body is under any sort of stress the heart pumps blood faster into the veins and so then the sickle cells clog up the tiny capillaries, venuoles and arterioles in the body. It’s like one big ass traffic jam, the sickle cells are the ones causing the pill up and barely any cells are getting through.
When blood isn’t moving sufficiently to the body cells & tissues, it isn’t getting Oxygen. Oxygen is essential for life, so the affected region starts screaming like a whiny bitch…HELP! I NEED O2 STAT! The screaming for oxygen—that’s when pain kicks in. Pain is the body’s response saying that something is really wrong and you need to fix it. Okay, so that’s what happens when just one capillary clogs up—now imagine that, in every single joint, capillary, arteriole and venuole in your body. Can you say immense pain? Think about the worse physical pain you’ve ever had in your life. Now multiply that by 100 and that probably still won’t encompass the pain of a sickle cell crises.
Main triggers of a crises are:
- Infection (which causes dehydration)
- Extreme temperatures of cold & heat
- Pressurized vehicles like airplanes —remind me to tell you of the crises I had on a plane, that shit was no joke!
- Any situation that compromises the level of oxygen in your body, like swimming, rigorous sports, labor & delivery etc.
So pretty much you have to avoid all those things. Like I said, it’s a fine balance all the time.
Sickle cells live an average of 10 days. Normal RBCs live 120 days. So of course someone with the disease is going to be anemic…your bone marrow is constantly producing mostly jacked up cells and your spleen is working overtime to recycle those wimpy sickle cells. That’s where the whole enlarged spleen comes from, super work=super muscle. A regular person has a hemoglobin count of 12-16..but someone with SSA hangs around the 8-10mg/dl on a good day and drops anything below 7 on a bad day.
When you drop low like that, the main solution they have right now is blood transfusions. And blood transfusions come with a whole plethora of their own problems. There is the fear of catching another shitty disease like HIV or Hepatitis (yeah, they say they check the blood but all it takes is one person who doesn’t know they have something, or one girl having a bitchy day at work and bam, your ass is full of another disease!)
In addition to that, blood transfusions fill you up with weird ass proteins, antibodies and cells that don’t play nice with each other and your body. The more you have, the harder it becomes to get a good blood match. The harder it is, the greater chance you have of having a severe blood rejection episode, throwing you into shock, coma and worse. In addition, these new proteins from the transfusions tend to stick in your capillaries with the sickle cells in your chest well causing a pesky huge complication called acute chest syndrome that gives you severe difficulty with breathing until you need to get intubated or croak.
Moving right along, pain therapy is a huge boon to SSA patients, thank whoever created painkillers! However, the drawback here is that with the high doses of narcotics you are on usually on a regular basis, you have a greater chance of getting addicted or killing your kidneys & liver. So we try to chill out on the pain meds, which means that we are in pain alot more than we let on.
Hmmm, what else? We are at risk for Pulmonary embolism, blood clots, strokes and pretty much any blood related complication you could think of. The complications are pretty much what eventually does us in. Life expectancy in the States is 43-48 (which for some reason people think is good. Oh Hell fuckin’ no!) In Jamaica its in the 50s for some reason. I’ve always known about this life expectancy thing and lived my life like I was going to die in my 40s even though I hope to God I don’t.
So…can someone please find a fuckin cure already???