Understanding Thalassemia

Questions:

1) A 68-year-old male is admitted to the hospital for emphysema. He is hypoxic and his labs reveal low oxygen levels. His hematocrit is 65%. The physician has told him that he has a type of polycythemia in which he has an increased number of erythrocytes circulating in his bloodstream. The patient tells the nurse that he does not understand what that means. How would the nurse explain this in terms the patient would understand?

2) A 52-year-old woman was diagnosed with leukemia and has been receiving chemotherapy as an outpatient. She tells the RN that she hasn’t been feeling well. The patient’s skin is warm to touch and she has a low-grade fever of 100.2 F. The neutrophil blood count is less than 1000/ul. The nurse is concerned about the possibility of infection because of the neutrapenia and low-grade fever. Explain why.

3) A man of Mediterranean ancestry goes to his doctor with the following symptoms. He is very tired all of the time. He has difficulty catching his breath after even mild exercise. His doctor orders the following tests: complete blood count (CBC), hematocrit, differential WBC count. The tests show immature erythrocytes, fragile erythrocytes, and less than 2 million RBCs per cubic millimeter. What would be a tentative diagnosis and suggested treatment?

4) Mariam, a blood type Rh’ is married to an Rh F” husband. She was pregnant for the first time and their child has been determined to be Rh’ The first pregnancy does not usually cause any problems, but the child was born blue and cyanotic.

a) What is the Rh incompatibility called?
b) Why is the baby cyanotic?
c) Since this was Mariam’s first pregnancy, how can you explain for the baby’s problem?

5) A 17-year-old black male is admitted to the hospital in sickle-cell crisis. Pain management is a top priority for clients in sickle-cell crisis. Explain why.

1. The nurse should tell the patient that as he has a reduced level of oxygen within the blood, his system has responded by generating more red blood cells and this caused polycythemia (ÄÂogaš). Reduced oxygen level fuels the production of erythropoietin within the kidneys to produce more RBCs.  

2. Low grade fever along with neutropenia represents a major concern for a patient’s survival. It is an important matter of concern because neutrophil’s function in phagocytosis (Wolach, Shpilberg and Lahav). This patient suffers from decreased capacity to fight against infection.

3. The tentative diagnosis would be Thalassemia and suggested treatment would be blood transfusion (Aydinok).

4. a) The Rh incompatibility is said to be a condition which develops when a pregnant female has Rh negative blood but her baby has Rh positive blood inside her womb.

b) The baby is cyanotic may be because the blood inside the baby’s system is not bearing adequate oxygen.   

c) The mother needs to be informed about the baby’s complication, which means during pregnancy Mariam should get a RHO GAM shot and as her baby is Rh positive she should be administered with another shot. This helps the mother for further births and manages post padrum delivery complications.

5. Red blood cells with sickle shape live almost 10-12days where normal red blood cells can survive for almost 120days within the system. The destroyed sickle shaped red blood cells clump together and fasten to the blood vessel walls, which blocks the flow of the blood. This incident can cause pain and permanent harm to the organs like liver, kidneys, heart, brain, lungs, spleen and bones. Therefore, pain management is a top priority for clients with sickle cell crisis.

References

Aydinok,. ‘Thalassemia’. Hematology (2012): n. pag. Web.

ÄÂogaš, Zoran. ‘Sensomotor Axonal Peripheral Neuropathy As A First Complication Of Polycythemia Rubra Vera: A Report Of 3 Cases’. American Journal of Case Reports 14 (2013): 385-387. Web.

Wolach, Ofir, Ofer Shpilberg, and Meir Lahav. ‘Neutropenia After Rituximab Treatment’. Current Opinion in Hematology 19.1 (2012): 32-38. Web.