Question

illoul VIU.40 PIV Case Study, Chapter 13, Disorders of Red Blood Cells S is a 12-year-old African American girl who is brought to the emergency department by her day camp counselor. She is in excruciating pain and keeps saying something about a crisis situation. No one has any knowledge of her previous medical history, and no one has been able to make contact with any family members. The counselor shared that she became short of breath and started reporting pain when they got to the top of the mountain they were climbing. The nurse immediately suspects a sickle cell crisis. 1. What is sickle cell disorder? 2. The nurse finally made contact with S's mother, who confirmed that S did indeed have sickle cell anemia. What factors in the scenario prompted the crisis at this time? 3. What are other risk factors for a sickle cell crisis? S's mother begins to cry and says, “If only I had known, I would have never had children. When she had her first crisis at 2 weeks of age, I decided then I would never have any more children since they would all have sickle cell disease." 4. How would the nurse respond? 5. Why did S not have a crisis at birth? 6. What are some possible complications associated with repeated sickle cell crises?

Answer 1

1.sickle cell disorder is a group of inherited, autosomal recessive disorders that characterized by an abnormal form of haemoglobin in the RBC.

2.The sickle cell crisis is triggered by low oxygen tension in the blood. Hypoxia can be caused by following factors.

• Viral or bacterial infection
• High altitude
• Emotional or physical stress
• Blood loss.

3.

• Dehydration
• Acidosis
• Increased plasma osmolality
• Decreased plasma volume
• Hypothermia

4. Positive reinforcement provided to the mother be calm at present pain management can done to minimize the crisis. If both parents have the trait, there is a 1 in 4 chances that their baby will have sickle cell disease. If one parent have the trait some of chances are to develop they usually not shows symptoms. Screening test can be performed to detect.

5. The disease is inherited and recessive in nature so does not show any symptoms. Here inherited haemoglobin S from one parent and normal haemoglobin( Hb A) from other parent sickle cell trait is mild to asymptomatic condition.

6.

• Pneumonia
• Acute chest syndrome
• Infection Is a major cause of morbidity and mortality.
• Autosplenectomy( the spleen becomes small because of the repeated scarring)
• Pulmonary embolism
• Osteoporosis and osteo sclerosis.
• Chronic leg ulcers
• Renal failure
• Retinal detachment