Sickle Cell Anemia NCLEX Review

This is an NCLEX review on sickle cell anemia.

Sickle cell anemia is a genetic blood disorder that affects the shape of the red blood cell. As the nurse, you will want to be familiar with the pathophysiology, signs and symptoms, sickle cell crisis, treatment, and nursing interventions.

Don’t forget to take the free quiz after reading this review: sickle cell anemia nclex questions

Lecture on Sickle Cell Anemia

Sickle Cell Anemia NCLEX Review

What is Sickle Cell Anemia? It is where a person has abnormal hemoglobin S (normal hemoglobin is hemoglobin A) on their red blood cell. This type of hemoglobin is very sensitive to oxygen changes, which causes the red blood cell to change it’s shape. This can lead to many complications (sickle cell crisis).

Sickle cell anemia is the most common and severe form of sickle cell disease.

How do people get sickle cell anemia?

They’re born with it! The person inherited two hemoglobin S genes from EACH parent. Therefore, it is an autosomal recessive disorder.

Again, this means in order for sickle cell disease to occur in a person they must have received TWO abnormal hemoglobin S genes for each of their parents (one from each). This disease is HOMOGYZOUS (Hemoglobin SS).

The parents normally don’t show signs and symptoms of the disease. They will each possess Hemoglobin A and S (one NORMAL hemoglobin and one ABNORMAL hemoglobin). This is known as SICKLE CELL TRAIT. It is rare for people who have sickle cell trait to show signs and symptoms of the disease because they usually have just enough normal hemoglobin to prevent sickling of the red blood cells. However, if two people with sickle cell trait have a child, there is a 25% chance the child will develop sickle cell disease.

Which patient population is most affected by sickle cell anemia?

Sickle cell anemia is most common in African-Americans.

WHY? According to the CDC.gov, it’s because 1 in 12 African-Americans in the US have the sickle cell trait, so it can easily be passed to their offspring.  Other patient populations affected by this genetic disorder include: Middle Eastern, Asian, Caribbean, and Eastern Mediterranean.

How is sickle cell trait diagnosed?

This disorder tends to be diagnosed in the very young pediatric populations…..like at 6 to 8 months of age. Many times (usually around 6 to 7 months) a parent will notice that their baby is all of the sudden extremely fussy (like the child is in constant pain), feverish, and has unusual swelling in the hands and feet (dactylitis…hand-foot syndrome). These signs and symptoms present because the baby’s RBCs have started to sickle and cause extreme pain. The sickling decreases blood flow to the extremities (hence hands and feet) and organs.

Now WHY is this occurring now and not at birth or around 2 to 3 months? Because in utero and during the first 6 months of age, the baby has fetal hemoglobin (remember this because we talk about this type of hemoglobin for the treatment of this condition with the medication Hydroxyurea) in their system that is the main carrier of oxygen. However, around the age of 6 months this type of hemoglobin diminishes and the type of hemoglobin their genetic code gave them takes over…..which is unfortunately hemoglobin SS.

Sickle cell anemia testing is part of newborn screening in most hospitals. Tests used include:

Dithionite (Sickledex): a blood test that assesses for abnormal hemoglobin S…it doesn’t differentiate between if the person has sickle cell trait or sickle cell disease.

Therefore, a hemoglobin electrophoresis will need to be performed. This is a blood test that assesses for various types of hemoglobin.

Pathophysiology of Sickle Cell Anemia

How should a red blood cell NORMALLY look? It should be round, smooth, concave with flat centers on both sides and contain no nucleus. Because the RBC doesn’t contain a nucleus it can easily squeeze through our vessels to accomplish its job.

What’s one of the jobs of the RBC? To carry oxygen…..and it does this with the help of HEMOGLOBIN! For a RBC to achieve this, it must have hemoglobin A present so the shape of the red blood cell won’t change.

However, people with sickle cell anemia don’t have this type of hemoglobin. Yes, their red blood cells have hemoglobin, but it’s a different type called hemoglobin S, which causes the red blood cell to be stiff, sticky, and form a “sickle” type shaped, especially when there is a low amount of oxygen in the body causing the patient to experience a sickling episode (sickle cell crisis).

These RBCs can stick together so well they will cause circulation problems, AND this is when signs and symptoms will manifest themselves in a sickle cell patient. It is important to remember that most patients with sickle cell anemia will usually just have anemia and they won’t have major signs and symptoms until a crisis episode.

When the RBCs sickle, this abnormal shape causes the red blood cell to become stuck in vessels (blocking blood flow)…so organs and tissue suffer….which leads to many signs and symptoms, especially pain.

Where does the ANEMIA part come in? Red blood cells with hemoglobin S are very weak and easily rupture. They only live 20 days compared to the normal 120 days. The bone marrow cannot keep up with producing RBCs, so the patient is anemic.

Factors that can cause Sickle Cell Crisis: remember these

There are various types of sickle cell crisis, which occur when there is something causing the body to increase its demands for oxygen OR there is something affecting how oxygen is transported.

“Sickle”

Significant blood loss…surgery, trauma etc.

Illness (at risk for this due to spleen function being affected)

Climbing or flying to high altitudes

Keeping continued stress (physical or mental)

Low fluid intake (dehydrated)

Elevated temperature…..fever, strenuous exercise (extreme temperature changes like cold weather or cold water….like swimming in cold can lead cells to sickle)

Types of Sickle Cell Crisis:

• Vaso-occlusive: fever, pain, edema in the hand and foot (seen in babies)….RBCs sticking together blocking blood flow which can lead to decreased circulation to organs, tissues along with infarction (stroke, renal issues).
• Hyperhemolytic: destruction of RBCs at an accelerated rate….remember these RBCs are weak. When RBCs rupture they release bilirubin. Normally, the body (gallbladder and spleen) can keep up with the recycling of the RBCs and clean-up of bilirubin. However, this is not the case here in SCA…the patient can experience jaundice, gallstones, anemia etc.
• Aplastic: the halt of red blood cell production….bone marrow cannot keep up with producing red blood cells so the patient will cease in making RBCs….the patient will have anemia.
• Non-functional spleen: the spleen helps recycle old RBCs and filters the blood to kill foreign invaders. In SCA, the spleen can become congested with sickled RBCs, which leads it to swell and not work properly. The patient is at risk for infection and many patients will need their spleen removed.

Crises vary among patients….some can have them randomly, while others have them frequently.

Signs and Symptoms of Crisis in Sickle Cell Anemia

Dactylitis (early sign seen in babies at 6 months): hand-foot syndrome….blood flow being blocked to hands and feet…will be swollen and fever present

Pain (very severe)….back, joints, chest etc. comes in episodes

Anemic: RBC dying too soon: fussiness, tired, tachycardia, jaundice or pale (for dark skinned patients assess mucous membranes),  delayed growth, shortness of breath

Infection risk: spleen recycles old RBCs and helps us fight infection by filtering out foreign invaders….sickled RBCs become trapped in spleen and this leads  it to swell and not work properly…..at risk for infection, especially pneumonia (needs pneumococcal vaccine and flu, meningococcal)….some patients need a splenectomy

Gallstones: WHY? sickled RBCs are constantly breaking down and releasing bilirubin, which leads to gallstones because there is too much bilirubin for the gallbladder to manage

Stroke: if sickled RBCs stick together and block blood flow to the brain…blood supply is limited to the brain leading to a decrease in brain blood supply….can cause mental and physical disabilities….NEURO checks very important.

Eye problems: vision issues due to the blockage of blood flow to eye vessels from sickled cells (needs eyes checked regularly)

Risk for abnormal clotting due to an increase in blood coagulation: at risk for DVT or PE

Leg ulcers (older children and adults): lack of blood flow…painful and very slow healing

Acute chest syndrome (happens due to infection like pneumonia or embolism or sickled cells blocking perfusion to lung tissue): chest pain, cough, fever, low oxygen saturation, new chest x-ray infiltrate….very deadly…monitor respiratory status

Also, damage to the organs that depend on high blood flow: kidneys, liver, heart…blood flow limited

Nursing Interventions for Sickle Cell Anemia

Sickling of RBCs occuring: focus hydration, oxygen, pain, at risk for infection, monitor respiratory status, neuro checks, at risk for acute chest syndrome, prevention of future crisis episodes, medications, blood transfusions

Pain: opioids around the clock (PRN not the best at this time until crisis over)

IV fluids and oral fluid: dilutes blood and helps kidney function (blood flow is being limited because RBCs are sticking together)

Oxygen: remember RBCs are stressed because of the lack of oxygen….this helps with alleviating the sickling

Penicillin: prevents infection or other antibiotics to treat (some MDs will order some patients to take prophylactic doses of PCN to prevent infection because their risk)…. if patient develops acute chest syndrome due to pneumonia may be ordered antibiotics.

Blood transfusion to replace RBCs and helps with anemia and increases oxygen levels in the body…….for multiple transfusions watch for iron overload

Bed rest

Educate about preventing infection (needs vaccines to be up-to-date, hand hygiene, avoid extreme weather and physical activities, mental/physical stress, staying hydrated, avoiding smoking, high altitudes)

Keep extremities elevated and extended to prevent swelling and helps blood flow

Remove restrictive clothing because it decreases perfusion

Warm compresses (not cold leads to sickling) for painful areas

Folic acid administration: helps make RBCs…..NOT iron….this doesn’t help with this type of anemia but can actually build up in the body causing toxicity and harm to organs

Medication for Sickle Cell Anemia:

Hydroxyurea: this helps actually treat cancer, but it will help with SCA in that it will help create fetal hemoglobin (hgb F). This helps decrease sickling episodes and this protein is present at first til 5-6 months of age, which is why babies don’t start showing signs and symptoms until after then. It will also help with anemia (decreasing the need for blood transfusions)….Side effect: lowers WBC count

Stem cell transplant: to cure….bone marrow will make healthy RBCs…needs a matched donor

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