General Knowledge
What is the primary genetic cause of sickle cell disease?
A mutation in the HbS gene, which encodes the beta-globin subunit of hemoglobin.
What medication is commonly used to manage pain in patients with sickle cell disease during vaso-occlusive crises?
Analgesics such as opioids (e.g., morphine, hydromorphone) are commonly used.
What is the hallmark symptom of sickle cell disease?
Pain, often due to vaso-occlusive crises.
What is the recommended daily fluid intake for patients with sickle cell disease?
At least 8 to 10 glasses of water per day.
Provides the costs, types of treatment, and the importance behind it
The Sickle Cell Disease Association of Canada
What is the typical lifespan of a sickle cell red blood cell compared to a normal red blood cell?
Sickle cell red blood cells have a lifespan of around 10 to 20 days, compared to the typical 120-day lifespan of normal red blood cells.
What nursing intervention is crucial for preventing complications related to dehydration in patients with sickle cell disease?
Ensuring adequate hydration through oral or intravenous fluids. Treating the underlying cause as well
What term describes the obstruction of blood vessels by sickled red blood cells, causing tissue ischemia and pain?
Vaso-occlusion.
Name one strategy for pain management that patients with sickle cell disease can use at home.
Applying heat or cold packs to painful areas.
What online platform provides comprehensive resources for individuals with sickle cell disease, including webinars, lectures, and conferences, emphasizing up-to-date information?
Sickle Cell Association of BC
Which ethnic group is most commonly affected by sickle cell disease?
Individuals of African descent are most commonly affected by sickle cell disease. More in areas that are endemic to malaria
Name one nursing intervention aimed at preventing infections in patients with sickle cell disease.
(I hope you got this one) :)
Name one potential complication of sickle cell disease involving the bones.
Avascular necrosis (osteonecrosis).
Why is it important for patients with sickle cell disease to avoid extreme temperatures?
Extreme temperatures can trigger vaso-occlusive crises. (can trigger sickle cells can work too)
What organization provides education on current legislation, clinical trials, and other relevant information surrounding sickle cell disease?
OneSCDvoice
What is the name of the hemoglobin variant that causes sickle cell disease?
Hemoglobin S (HbS) is the abnormal hemoglobin variant responsible for sickle cell disease.
What nursing action is essential for preventing complications in patients receiving chronic transfusions for sickle cell disease?
Monitoring for signs of iron overload and managing it with chelation therapy if necessary.
What organ is most commonly affected by acute chest syndrome in patients with sickle cell disease?
The lungs.
What should patients with sickle cell disease do to reduce the risk of infection?
Practice good hygiene, receive recommended vaccinations, and avoid exposure to sick individuals.
Which institution offers a Hemoglobinopathy program that includes screening, manages chronic transfusion programs, supervises iron chelation therapy, and may offer options such as bone marrow transplantation or participation in clinical trials?
BC Children's Hospital.
What are the two main types of sickle cell disease, and how do they differ?
The two main types are sickle cell anemia (HbSS), where both genes are HbS, and sickle beta thalassemia (HbC), where one gene is HbS and the other is beta thalassemia. Sickle cell anemia tends to be more severe.
How do nurses assess and manage the risk of iron overload in patients with sickle cell disease receiving chronic transfusions?
Nurses monitor serum ferritin levels and may perform liver iron concentration assessments. Chelation therapy with agents like deferoxamine or deferasirox may be used to manage iron overload.
What are the signs and symptoms of a splenic sequestration crisis in patients with sickle cell disease?
Signs include sudden enlargement of the spleen, abdominal pain, and signs of hypovolemic shock such as tachycardia and pallor.
Why is it important for patients with sickle cell disease to receive regular vaccinations?
Vaccinations help prevent infections, which can be particularly severe in individuals with sickle cell disease due to their compromised immune function.
What organization provides information on self-care, basic understanding of sickle cell disease, and current research on its conditions?
Health Link