Sickle Cell Anemia Report

Multiple choice questions

Question no. 1) Comprehension: These questions test the students’ ability to summarize and describe in their own words without necessarily relating it to anything.
A nurse suspects that the case she is handling must be a case of sickle cell disease. What symptom should the nurse try to observe in the patient to confirm the diagnosis?

Fatigue

Pallor
Pain in limbs
Breathlessness
Answer and Rationale: C, Pain in limbs. All the other symptoms will also be present in the same patient. But these symptoms can be seen otherwise also. Pain in limbs is a sure sign of the disease. Manifestation of pain will occur as the sickle shaped cells causes vaso- occlusion leading to increased pressure in the limbs along with pain.
Question no. 2) Knowledge: These types of questions test the students’ ability to memorize and to recall terms, facts and details without necessarily understanding the concept.
An eight weeks pregnant woman reports to the nurse and asks her that she wants to undergo prenatal testing to detect any hemoglobinopathy of the unborn. When should the nurse ask her to undergo the test?

Immediately

After 10 weeks
After 24 weeks
After 32 weeks
Answer and Rationale: B, after 10 weeks. It takes around 10 weeks for sickle cell disease to be detected in the test properly.
Question 3: Application: Application questions encourage students to apply or transfer learning to their own life or to a context different than one in which it was learned. 
A 4 year old child is diagnosed with sickle cell anemia. What treatment should the nurse suggest his parents keeping in mind his age?

Hydroxyurea

Blood transfusion
Bone marrow transplant
Gene therapy
Answer and Rationale: A, hydroxyurea. Hydroxyurea is a treatment in which medicines are given which promote the formation of fetal hemoglobin. This fetal hemoglobin has the capacity to transform into normal adult hemoglobin, thus helping in combating the symptoms of sickle cell anemia.
Question 4: Synthesis: These questions encourage students create something new by using a combination of ideas from different sources to form a new whole.
A patient of Sickle cell disease presents with acute pain in the limbs. On proper case taking it is revealed that he had a history of frequent seizures. Which drug out of the following should the nurse avoid administering him?

Clotrimazole

Penicillin
Morphine
Meperidine
Answer and Rationale: D, Meperidine. It should be avoided as its metabolism leads to the formation of normeperidine which has the potential to develop seizures. Already the patient has a history of seizures, thus this drug should be avoided (Hick, Nelson, Hick and Nwaneri, 2006).
Question 5: Evaluation: Evaluation questions encourage students to develop opinions and make value decisions about issues based on specific criteria (Academy of Art University, 2015)
A young patient of 5 years presents with severe body pain occurring on and off since few days, his parents are unable to recall how many days exactly. He often suffers from fatigue and dyspnoea. On observation he appears to be pale and hyperventilating. On further questioning they admit that the mother is suffering from sickle cell disease and she had to undergo blood transfusion every few months. As an attending nurse, what test would you recommend to be done first?

Routine blood test

Hemoglobin electrophoresis
Serum potassium levels
Erythrocyte sedimentation rate test
Answer and Rationale: The patient should go for Hemoglobin electrophoresis test first. The case is suggestive of some blood related genetic disorder. Thus undergoing this test will help in prompt diagnosis and early treatment commencement.

Question 6: Knowledge

A 5 year child is diagnosed with sickle cell anemia. Name the drug which the nurse should administer daily to a patient of sickle cell anemia.

Clotrimazole

Penicillin
Metronidazole
Alprazolam
Answer and Rationale: B, penicillin. Daily dose of penicillin is required to be given to the patient of sickle cell anemia, mainly children. It prevents them from pneumococcal infections (Cober and Phelps, 2010).

Question 7: Comprehension

A patient is newly diagnosed with sickle cell disease. During his counseling sessions he was asked to abstain from hard work, not to perform too much of physical labor and avoid exposure to too much of cold and getting wet. He understood the cause for all these, except the fact that why he should not expose to cold and wet. What should the nurse explain to him regarding the matter?

He has a predisposition to take cold easily

Sickle cell anemia leads to easy catching of cold and flu
His immune mechanism is weak
His already occluded arteries will further constrict on exposure to cold.
Answer and Rationale: D, His already occluded arteries will further constrict on exposure to cold. As he is having sickle cell disease his arteries are already obliterated due to abnormal shapes of the RBCs. On exposure to cold or being wet his blood vessels will undergo further vasoconstriction. This will lead to decreased blood flow to the peripheries and hence may become problematic for the patient.

Question 8: Evaluation

A 70 year old patient is admitted to the emergency department of the hospital, he was diagnosed with stroke. His attendant informed that he is having sickle cell disease. What other signs should the nurse look for in the unconscious patient to believe the diagnosis?

Leg ulcers

Jaundice
Reduced pulse
Increased perspiration
Answer and Rationale: A, leg ulcers. Elderly sickle cell disease patients have a tendency to develop leg ulcers due to stasis of blood in the lower limbs.

MATCHING

For each given procedure match them with their appropriate explanation
Column A Column B
Hydroxyurea - Introducing healthy gene
Electrophoresis - Promoting fetal hemoglobin
formation

Gene therapy - Separating different forms of Hb

Blood transfusion - Giving healthy blood
Answer and Rationale:
Hydroxyurea- promoting fetal hemoglobin formation. In this method medicine s are given to the patient which helps in synthesizing fetal hemoglobin which are capable of replacing the sickle cell hemoglobin by forming normal healthy adult hemoglobin.
Electrophoresis- separating different forms of hemoglobin. This method helps in separating different forms of Hb depending on their density.
Gene therapy: Introducing healthy gene. In this method, healthy genes are introduced in the bone marrow which helps in forming normal RBCs.
Blood transfusion: Giving healthy blood. In this the patient is given healthy blood to replace the abnormal blood and thus helping the patient to carry out with a normal life.

SHORT ANSWER QUESTION (Knowledge, Analysis, Critical thinking)

How would you manage a patient with acute symptoms of sickle cell anemia?
Answer and Rationale: Immediate blood transfusion will be required to combat the effects. If the symptoms are of acute pain, then analgesics may be required. Higher doses of analgesic may be needed in certain patients. If the symptoms are related to respiration, that is, acute chest syndrome, then antibiotics may be required. The patient should abstain from intake of much liquid as the liquid may lead to pneumothorax as a consequence. The action required to be taken, depends on the system affected, symptoms and their severity.

Which test would you recommend to a pregnant lady who is a carrier of sickle cell trait?

Answer and Rationale: Amniocentesis should be recommended to ensure if the fetus is having the disease.
ESSAY QUESTION (Evaluation)
A couple is informed that their unborn baby is having sickle cell disease. They are still willing to continue with the pregnancy. How would you, as a nurse, convince them for the contrary?
Answer and Rationale: On emotional grounds the parents are willing to keep the baby. But they should be explained the implications of such decision. It is not only going to be a costly affair to keep the baby as he has to remain under medications all his life, he has to undergo repeated blood transfusion life long, also it would be a pain for the baby to bear the pain of the acute symptoms every now and then. He will be suffering from a lifelong disease. So on moral grounds it will be better to terminate the pregnancy.

References

Academy of Art University (2015). Different Types of Questions based on Bloom's Taxonomy. Retrieved 25 March 2015, from http://faculty.academyart.edu/resource/question_types.html
Cober, M., & Phelps, S. (2010). Penicillin Prophylaxis in Children with Sickle Cell Disease. The Journal Of Pediatric Pharmacology And Theraputics, 15(3), 152-159. Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3018247/
Hick, J., Nelson, S., Hick, K., & Nwaneri, M. (2006). Emergency Management of Sickel Cell Disease Complications. Minnesota Medicine. Retrieved 25 March 2015, from http://www.minnesotamedicine.com/Past-Issues/Past-Issues-2006/February-2006/Clinical-February-2006