- What does the peripheral blood smear show?
Examination of the peripheral smear shows marked poikilocytosis and anisocytosis with numerous sickled erythrocytes.
- What is the diagnosis from these findings?
Sickle cell anemia (Hgb SS).
- Which of the following tests would be most useful to determine the
etiology:
- A. Hemoglobin electrophoresis
- B. Reticulocyte count
- C. Stool for occult blood
- D. Vitamin B12 assay
- E. Bone marrow biopsy
Answer: A
- A "crisis" in this patient is most likely to be initiated by which of the following?
- A. Quinacrine ingestion
- B. Parvovirus infection
- C. Decreased oxygen tension
- D. Exposure to cold
- E. Transfusion therapy
Answer: C This is why people with Hgb SS disease do not live at high elevation.
- Discuss the demographics of this disease
Through human history, the driving force behind the appearance of sickle cell anemia has been malaria, particularly falciparum malaria, which can have a high mortality rate when untreated. Persons who are heterozygous for the sickle cell gene (Hgb AS, or sickle cell trait) have less severe infection with P. falciparum. Persons with sickle cell anemia are likely to trace their ancestry to parts of the world where mosquitos harboring P. falciparum are present, such as West Africa or Eastern Arabia or India.
What is your response when this patient demands a large amount of pain medications?
Sickle cell disease and its complications can be very painful, not to mention life threatening. Yet it is not uncommon for some medical professionals to view 'sicklers' in crisis who want pain medications as representing drug-seeking behavior, i.e. addicts, drug abusers, or those just seeking recreational drugs. It is hard not to consider this lack of empathy has some racist presuppositions. Persons most likely to be affected are of West African, Arab, or Indian descent.
The worst examples involve physicians giving placebos to 'prove' their point. This is ethically questionable for a number of reasons. Placebos usually mean lying to the patient (giving a saline injection, but telling the patient it's the opioid requested). Furthermore, a placebo effect doesn't prove there was no real pain, it only proves that the placebo effect (thanks to faith in medicines and doctors) is so strong it can reduce pain (something already well studied). Lastly, the pain that remains can be doing long-term damage.
Physicians are not free from all the prejudice that runs through the society in which they live. There will be some (hopefully, very few) physicians who secretly think (even if they would never say it out loud) that some patients belonging to some race, social status, or religion complain more than others. Perhaps they think such patients have a sense of entitlement because they feel they've been deprived for so long, that now they somehow want to get it all back by getting everything they want now. Well, such views probably increase the chance that a doctor will minimize the importance of the disease.
In the U.S., many studies have shown differences in quality of care for blacks versus whites, even when adjusted for the same level of insurance, income, and education. Sickle cell anemia, of course, is the classic disease that "only" blacks get. So it can be the ultimate test of whether white physicians can empathize with black patients.
