03.01 Sickle Cell Anemia

Hey guys, in this lesson we are going to be talking about Sickle Cell Anemia.

So, Sickle Cell Anemia is a hereditary disorder that most often affects people of African or Mediterranean descent. It’s passed on as a recessive trait. So both parents have to have the trait for one of their kids to have it.

What’s happening with this disease is that some of the red blood cells have hemoglobin that have the sickle cell trait. When this hemoglobin is exposed to stress (like extreme temps or a fever or extreme exercise) they begin to harden and change shape or sickle. So you get red blood cells that are misshapen and rigid. These sickled cells aren’t able to carry oxygen as well and they are likely to get stuck in small vessels in the body. When this happens it’s called a micro-occlusion and these micro-occlusions are super painful.

When a lot of cells become sickled you get what’s called a Sickle Cell Crisis. There are few different types of these crises that can occur - so let’s take second to look at those.

So the first kind of crisis is a vaso-occlusive crisis. This is when the sickled cells are getting stuck in small vessels. This results in decreased blood flow to that particular area which means that tissue isn’t getting oxygen and can lead to cellular death. The most common place for this to occur is in the extremities and joints, but they can also happen in organs. One really serious complication that can occur with a occlusive crisis is called an Acute Chest Syndrome. It is the most common complication and can have really poor outcomes. So you’ve got to be aware of it and on the lookout for it! It’s usually associated with a fever, chest pain, hypoxia and increased respiratory effort. It kinda looks like a pneumonia. So keep a close eye out for these symptoms.

The second kind of crisis is when blood pools or gets trapped in a certain part of the body because of the sickling. This is called a Sequestration crisis. The most common locations for this are in the spleen and in the liver. For the patient this looks like decreased circulating blood volume and shock. Again - something not to miss!

We’ve already gone over most of the symptoms, but let’s summarize them here. The most obvious symptom is pain. Remember, the micro-occlusions and lack of oxygen makes them really hurt. The pain often feels like it’s “all over” but definitely pay attention to their description of the pain because it may give you a clue about where their sickled cells are clumping up. They will also have symptoms that are expected with any anemia, like hypoxia, fatigue and pallor.

Last but not least - keep those complications we mentioned earlier in mind when you are assessing. So be on the lookout for fever, chest pain, difficulty breathing and signs of hypovolemia and shock.

Management of an acute crisis is pretty straight forward. You can use the mnemonic HOP to help you remember what to do! So if you see a patient with symptoms of a sickle cell crisis you need to HOP to it and get them some fluids! So yeah the first thing we need to do is get those sickled blood cells re-hydrated and moving again. The second thing to consider is oxygen. If they are hypoxic they should be placed on O2. Then you need to get them some pain meds. This is so so important guys because the pain is severe during a crisis and this pain is on top of the chronic pain they likely live with every day. I know the opioid crisis is on everyone’s mind right now, but we have to remember that pain is what the patient says it is and these kids are hurting a lot all of the time. So work with the patients - discuss what helps them feel better. Always utilize non-pharmacological methods to help with pain like heat packs (not ice packs - these make the occulussions worse!) but know that they will likely need both NSAIDS and opioids to truly manage the pain. Remember - as with any patient you see who lives with chronic pain- find out from them what number on the 0-10 pain scale is something they can live with. 0 probably isn’t realistic.

The last two things listed here antibiotics and hydroxyurea. These are two drugs that are really important for long term management and hopefully preventing acute sickle cell crisis. The sickle cell trait makes people more prone to infection and remember stress and infection can cause a crisis so prophylactic antibiotics are used to help prevent this. Hydroxyurea is a newer drug that has been approved for use adolescent and adult patients with sickle cell anemia. Basically it increases the number of fetal hemoglobin in the body and fetal hemoglobin or HbF doesn’t sickle like normal hemoglobin does. So increasing fetal hemoglobin decreases the number of hemoglobin that can actually sickle! So be on the lookout for those meds during your clinicals.

Your priority nursing concepts for the pediatric patient with Sickle Cell Anemia are clotting, oxygenation and comfort.
Okay guys your key points for this lesson are: 1) Sickle Cell Anemia is a recessive hereditary disorder that causes misshapen red blood cells. These misshapen red blood cells can’t carry oxygen as well and can get stuck in small vessels. 2) When the cells get stressed and a crisis occurs the patient experiences pain and hypoxia. The pain mostly comes from the cells sticking together which is called a vaso occlusive crisis. 3) When this happens we treat with fluids, oxygen and pain medications! And 4) monitor the patient closely for complications like Acute Chest Syndrome or a stroke.

That’s it for our lesson on Sickle Cell Anemia. Make sure you check out all the resources attached to this lesson, especially the video on what it’s like to live with this disease! Now, go out and be your best self today. Happy Nursing!