Pathophysiology: A mutation in causes the red blood cells to form in a sickle shape. The sickle cell shape does not travel the way it should throughout the body.
- Hereditary disorder that primarily affects African Americans by recessive trait
- Genetic mutation leads to rigid, misshapen RBCs
- Affects the hemoglobin’s ability to carry oxygen
- Can cause micro-occlusions in small vessels
- Can lead to Sickle Cell Crisis
- Acute Exacerbation
- Caused by hypoxia, exercise, high altitude, fever, temperature extremes
- Severe Pain
- Due to micro-occlusions
- Dilute blood to ‘wash out’ sickled cells
- Give IV Fluids for hydration
- Blood transfusions – to give properly shaped/functioning RBCs
- Oxygen Supplementation
- Increase oxygen delivery to the tissues
- Pain Relief
- This pain is severe, they may require opioid analgesics to find relief
- Ensure 20g or larger IV in place for blood transfusions
- Administer PRBC’s per protocol
- Give IV Fluid boluses as ordered
- Administer supplemental oxygen via nasal cannula
- Assess SpO2 levels
- Assess pain (OLDCARTS)
- Administer analgesics as ordered
- Avoid extreme temperatures
- Stay hydrated, especially when ill or exercising
- Report illness with fever to primary care provider
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This lesson is going to explore sickle cell anemia.
Sickle cell anemia is a hereditary disorder of the red blood cells that primarily affects African Americans. Ultimately it’s a genetic mutation that makes some red blood cells become rigid and misshapen to the point that they look like little sickles, hence the name. Normally oxygen would bind to hemoglobin on the red blood cells to get carried to the body. On these sickled cells, oxygen can’t bind because the hemoglobin is misshapen. So that decreased oxygen carrying capacity is the anemia. The other problem sickle cell patients have is that these rigid red blood cells can actually clump together and block small blood vessels. So patients get these micro-occlusions in tiny vessels. As with most diseases, there’s a possibility for exacerbation, in this case it’s called Sickle Cell Crisis, where the decreased oxygenation and micro-occlusions are severe and cause a lot of issues for the patient. It can be caused by extreme temperatures, exercise, or illness and fever.
The assessment of a sickle cell patient is pretty straight forward – they’ll have some signs of anemia like pallor and fatigue, but because of those micro-occlusions and the lack of oxygenation to the tissues – the biggest symptom of sickle cell is severe pain all over. This is the biggest clue. If you have a young African American male come in with weakness and severe generalized pain, chances are it’s a sickle cell crisis.
Management of sickle cell anemia is also very straight forward. You can remember the mnemonic HOP. As in, if you feel signs of sickle cell crisis, HOP to the Hospital! H stands for hemodilution – we know they’re at risk for these micro-occlusions that can be very painful – so our goal is to dilute out the blood so there isn’t such a high concentration of these sickled cells in one area. We can do that with basic IV fluids like Normal Saline or Lactated Ringers, we want to keep them very hydrated. We also encourage them to hydrate really well before exercise or if they feel sick. We can also dilute by giving blood transfusions. Not only does it add some volume, but it also gives them more normal, functional red blood cells to dilute out the sickled ones. Remember that blood type matching is required for red blood cell transfusions, so check out the cheat sheet attached to this lesson to see a compatibility chart. So, that’s hemodilution. The O stands for Oxygen – they will likely need supplemental oxygen to improve their overall oxygen delivery to the tissues. And finally the P stands for pain relief. These patients will require pretty significant pain control. Many of them require strong doses of opiates. This is a chronic pain issue, so sometimes we find they require stronger and stronger doses as time goes on. I usually try to encourage my patients that they won’t likely get to a score of 0/10, so we want to just get them to a tolerable level.
So our priority nursing concepts for sickle cell anemia are clotting, oxygenation, and comfort. Check out the care plan attached to this lesson to see more detailed nursing interventions and rationales.
So sickle cell is pretty straight forward, but let’s just recap. A genetic disorder causes red blood cells to be misshapen and rigid, meaning they can’t carry oxygen like they should. Patients have signs of anemia like pallor and fatigue, and they also get severe generalized pain because of these micro-occlusions in small vessels. We manage by hemodiluting with fluids and blood transfusions and we give supplemental oxygen and focus on pain relief. Check out the outline attached to this lesson to see patient education topics to help them avoid sickle cell crisis.
Those are the basics of sickle cell, don’t miss the resources attached to this lesson to see the big picture for these patients. Now, go out and be your best self today. And, as always, happy nursing!