Question

Case Study
You are caring for a young adult patient who has had repeated hospitalizations for sickle cell crisis.
Ms. Soha was diagnosed with sickle cell anemia and has had multiple crises requiring hospitalization. Ms. Soha states that the pain in her chest is an 8 on a 0-10 scale. She describes the pain as a constant burning pain. Her vital signs are temperature of 38.5, blood pressure 120/76, pulse 96, and respiration rate of 22. Her oxygen saturation on room air is 94%. She is having some difficulty breathing and is placed on 2 liters of oxygen by nasal cannula. She explains that in the past she has been given morphine for the pain and prefers to use.
Her blood work reveals the following values: white blood cell count (WBC) 18,000, Red Blood Cell count (RBC) 3/106, hemoglobin (Hgb or hg) 7.5 g/dL, hematocrit 21.8%, and reticulocyte count 23%. Ms. Soha is admitted for pain management antibiotic treatment and respiratory support.
1. What factors should be assessed to determine the patient’s education.
2. Identify the causes of sickle cell anemia
3. Discuss the characteristic signs and symptoms of sickle cell anemia.
4. Discuss the potential complications associated with sickle cell anemia.
5. Describe the use of transfusion therapy for management of sickle cell anemia.

Answer 1

1) Assessment data for a sickle cell anemia patient should include:

• Factors causing previous crisis. The patient is asked to identify factors that precipitated previous crisis and measures the patient uses to prevent and manage the crisis.

• Pain levels.

• Characteristics of pain.

• Infection.

2) Causes:

Sickle cell anemia is caused by a mutation in the gene that tells your body to make the iron-rich compound that makes blood red and enables red blood cells to carry oxygen from your lungs throughout your body (hemoglobin). In sickle cell anemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky and misshapen.

3) Signs and symptoms can include:

• Anemia. Sickle cells break apart easily and die, leaving you with too few red blood cells.

• Episodes of pain.

• Swelling of hands and feet.

• Frequent infections.

• Delayed growth or puberty.

• Vision problems

4) Complications:

Sickle cell anemia can lead to a host of complications, including:

• Stroke. Sickle cells can block blood flow to an area of your brain. Signs of stroke include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness. If your child has any of these signs and symptoms, seek medical treatment immediately. A stroke can be fatal.

• Acute chest syndrome. A lung infection or sickle cells blocking blood vessels in your lungs can cause this life-threatening complication, resulting in chest pain, fever and difficulty breathing. It might require emergency medical treatment.

• Pulmonary hypertension. People with sickle cell anemia can develop high blood pressure in their lungs. This complication usually affects adults. Shortness of breath and fatigue are common symptoms of this condition, which can be fatal.

• Organ damage. Sickle cells that block blood flow to organs deprive the affected organs of blood and oxygen. In sickle cell anemia, blood is also chronically low in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including your kidneys, liver and spleen, and can be fatal.

• Blindness. Sickle cells can block tiny blood vessels that supply your eyes. Over time, this can damage your eye and lead to blindness.

• Leg ulcers. Sickle cell anemia can cause open sores on your legs.

• Gallstones. The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in your body can lead to gallstones.

• Priapism. In this condition, men with sickle cell anemia can have painful, long-lasting erections. Sickle cells can block the blood vessels in the penis, which can lead to impotence over time.

• Pregnancy complications. Sickle cell anemia can increase the risk of high blood pressure and blood clots during pregnancy. It can also increase the risk of miscarriage, premature birth and having low birth weight babies.

5) Transfusion therapy is a key intervention in decreasing morbidity and mortality in patients with sickle cell disease (SCD). Current indications for acute and chronic transfusion therapy have significantly increased the number of RBC units transfused to patients with SCD worldwide.

This review summarizes transfusion management for the treatment or prevention of neurologic and perioperative complications, acute chest syndrome, and acute anemia associated with SCD. Despite the recognized benefits of transfusion therapy, it is not without the risks of iron overload, alloimmunization, and delayed hemolytic transfusion reactions. Transfusional iron overload management includes automated RBC exchange, noninvasive imaging to monitor iron burden, and iron chelation with parenteral or oral agents.