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Sickle Cell Anemia

Sickle Cell 101

Back to the basics – a clear explanation to better understand sickle cell anemia and how it is passed on.

Understanding how a disease can be passed on is extremely important so you can plan accordingly when it comes to having kids. This is even more important for patients who are carriers of a disease, and may not be aware of their carrier state since most have minimal symptoms.

Sickle cell disease is quickly picked at birth since there is a mandatory newborn screening within the US (national recommendation since 1987). In order to have sickle cell disease, you need to receive an abnormal hemoglobin gene from each parent. When each parent has sickle cell trait, there is a 25% chance of having a child with the disease, 50% chance of having trait and 25% chance of nothing. It’s important to remember the probability of having an affected child is the same with each pregnancy, just because you have one child with the disease doesn’t mean your next offspring won’t be affected.

Categories
Sickle Cell Anemia

Sickle Cell Medications

Many have requested information on currently approved medications for sickle cell. Hydroxyurea is still the gold standard for treatment and is the only medication we can say will make you live longer with sickle cell. BABY-HUG studied the effectiveness of hydroxyurea on babies, specifically looking at reducing pain episodes and organ damage. It does cause various side effects (which is why we need to monitor your labs to make sure it’s having the effect we want) and can’t be used for all patients, so it is exciting to have newers options available!

Endari was the first medication approved for sickle cell in almost 20 years! Oxbryta and Adakveo were both approved at the end of 2019 and address different aspects of sickle cell. Many think it is only the sickled cells that cause the occlusion in the vessel, leading to pain, however that is only the first problem. After the cells sickle, we have white blood cells, platelets and other inflammatory cells attaching to the blocked vessel, which adds more insult to injury, making the entire problem worse. Adakveo helps address the inflammatory component of sickle cell while Oxbrya helps prevent hemolysis (red cell breakdown) in the first place.

Let me know what questions you may have!

Categories
Sickle Cell Anemia

Managing Sickle Cell Pain


Pain is always a hot topic – and many underestimate the power of NSAIDs (non-steroidal anti-inflammatories), my personal fav being naproxen. When you are having a sickle cell crisis, your body is in a pro-inflammatory state, so taking medications like naproxen actually address one of the causes of your pain. Opioids are definitely stronger analgesics (aka pain medication) however they just put a bandaid on your pain and don’t actually treat the cause. You can also consider medications that address neuropathic (nerve) pain. Speak to your provider to come up with the best pain management regimen for you!