Sickle Cell Anemia Nursing | Symptoms, Pathophysiology, Sickle Cell Crisis & Trait

Summary

In this educational video, Sarah French Turner discusses sickle cell anemia, a genetic blood disorder characterized by abnormal hemoglobin S, which causes red blood cells to become rigid and sickle-shaped under low oxygen conditions. This leads to various complications, including anemia and increased risk of infections. The video covers the pathophysiology, types of crises, and nursing interventions for management. It also highlights the importance of hydration, avoiding triggers, and the potential use of hydroxyurea to increase fetal hemoglobin. Although stem cell transplants offer a cure, they are rare due to donor matching requirements.

Takeaways

• 😷 Sickle cell anemia is a genetic condition characterized by abnormal hemoglobin (hemoglobin S) in red blood cells, leading to a sickle shape and reduced oxygen transport.
• 🩸 Hemoglobin S is sensitive to low oxygen levels, causing red blood cells to become rigid, sticky, and sickle-shaped, which can obstruct blood flow and lead to crises.
• 🌡️ Triggers for sickle cell crises include high altitude, infection, dehydration, and extreme temperatures, all of which can increase the body's demand for oxygen.
• 🧬 Sickle cell anemia is an autosomal recessive disease, requiring inheritance of two abnormal hemoglobin S genes, one from each parent.
• 👶 The condition often presents in infancy, typically around 6-8 months when fetal hemoglobin levels decrease, and hemoglobin S starts to dominate.
• 🏥 Newborn screening and prenatal testing, such as amniotic fluid testing, are available to detect sickle cell anemia early.
• 🧪 The thionite test and hemoglobin electrophoresis are common diagnostic tests to identify and differentiate sickle cell anemia from the sickle cell trait.
• 🩹 Symptoms of a sickle cell crisis include pain, dactylitis (swelling of hands and feet), anemia, and potential organ damage due to blocked blood flow.
• 💊 Hydroxyurea is a medication that can increase fetal hemoglobin and reduce the severity of sickle cell anemia, but it requires monitoring due to its potential to lower white blood cell count.
• 🏥 Management of sickle cell anemia includes hydration, pain control, blood transfusions, and avoiding triggers to prevent crises, with a stem cell transplant being a potential cure in specific cases.

Q & A

What is sickle cell anemia?

Sickle cell anemia is a condition where a patient has abnormal hemoglobin, specifically hemoglobin S, on their red blood cells. This type of hemoglobin is sensitive to low oxygen levels, causing red blood cells to change shape into a sickle or C-shape, becoming stiff and sticky, which can block blood flow to organs and tissues.

Why are red blood cells with hemoglobin S problematic?

Red blood cells with hemoglobin S are problematic because they become stiff and sticky under low oxygen conditions, leading to a sickle shape. This shape causes them to clump together and block blood flow, potentially leading to organ damage and pain.

What is the difference between hemoglobin A and hemoglobin S?

Hemoglobin A is the normal type of hemoglobin that allows red blood cells to function properly, carrying oxygen throughout the body. Hemoglobin S, on the other hand, is abnormal and causes red blood cells to become sickle-shaped under low oxygen conditions, leading to complications.

How does the spleen become affected in sickle cell anemia?

The spleen becomes affected in sickle cell anemia because it is overworked trying to recycle the rapidly breaking down red blood cells. Additionally, blood flow to the spleen can be blocked by the clumping of sickle-shaped red blood cells, potentially leading to splenomegaly and an increased risk of infection.

What causes sickle cell anemia?

Sickle cell anemia is caused by inheriting abnormal hemoglobin S genes from both parents, resulting in a homozygous condition where the individual has hemoglobin SS on their red blood cells.

Why do symptoms of sickle cell anemia often present around 6 to 8 months of age?

Symptoms of sickle cell anemia often present around 6 to 8 months of age because that is when the fetal hemoglobin, which is less prone to sickling, is replaced by hemoglobin S as the child grows. This switch makes the red blood cells more sensitive to low oxygen levels, leading to the onset of symptoms.

What is the significance of the thionite test in diagnosing sickle cell anemia?

The thionite test, also known as sickle solubility test, is significant in diagnosing sickle cell anemia because it can detect the presence of abnormal hemoglobin. However, it cannot differentiate between sickle cell trait and full sickle cell disease, requiring a follow-up hemoglobin electrophoresis test for confirmation.

How does hydroxyurea help in treating sickle cell anemia?

Hydroxyurea helps in treating sickle cell anemia by increasing the production of fetal hemoglobin, which is less likely to sickle and thus reduces the severity of the disease. It also helps with anemia by decreasing the need for blood transfusions.

What is the role of the thionite test in newborn screening for sickle cell anemia?

The thionite test is often used as part of newborn screening for sickle cell anemia to identify the presence of abnormal hemoglobin early. Early detection allows for prompt intervention and management of the condition.

What is the potential cure for sickle cell anemia mentioned in the script?

A stem cell transplant is mentioned as a potential cure for sickle cell anemia. This procedure involves replacing the bone marrow to produce new, healthy red blood cells without the abnormal hemoglobin. However, it requires a matched donor and is not commonly performed.