Sickle Cell Scenario

CASE STUDY Sickle Cell Scenario V.M. is a 29-year-old African American married man who has sickle cell disease (SCD) marked by frequent episodes of severe pain. His anemia has been managed with multiple transfusions. Six months ago, he started showing signs of chronic renal failure. His regular medications are acetaminophen, hydroxyurea (Hydrea), and folic acid. In the hematology clinic this morning, V.M.’s hemoglobin (Hgb) measured 6.7 g/dL (67 g/L). He received 2 units of packed red blood cells (PRBCs) over 3 hours and then went home. He developed dyspnea and shortness of breath about 1 hour later, and his wife called 911. The emergency medical system crew started O2 at 8 L per nasal cannula and transported V.M. to the emergency department (ED). 1.What is SCD? A group of disorders that cause red blood cells to become misshapen and break down. Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues. 2. Evaluate each of the following statements about SCD. Enter “T” for true or “F” for false. __F___1. Only African Americans get SCD. __F___2. There is currently no cure for SCD. __F___3. Those with SCD should not receive childhood vaccinations. __F___4. SCD cannot be diagnosed before an infant is 3 to 4 months old. __T___5. An ophthalmologist should perform an eye examination every 1 to 2 years. ___F__6. Those with SCD should fly only on airplanes with pressurized cabins. 3. Which statement is true about the inheritance pattern of SCD? a. If V.M.’s wife has sickle cell trait, each child will either have SCD or be a carrier. b. If V.M.’s wife does not have sickle cell trait, each child has a 50% risk for having SCD. c. If V.M.’s wife has sickle cell trait, each child can either have SCD or be normal. d. If V.M. has children, each child will automatically have SCD regardless of his wife’s status. 4. V.M.’s Hgb measured 6.7 g/dL (67 g/L). Why is anemia common in patients with SCD? Ans- Sickled cells keep red blood cells from doing their job, which is carrying oxygen throughout your body. As a result, V.M don’t have enough healthy red blood cells and would develop anemia 5. Why is it difficult to crossmatch blood to transfuse V.M.? Ans- His blood type is a little harder to match and the blood type much be the same and compatible to prevent the body from flighting or rejection the blood. 6. What role does hydroxyurea (Hydrea) play in managing V.M.’s SCD? Ans- makes his red blood cells bigger. It helps them stay rounder and more flexible Case study progress When V.M. arrives at the ED, you perform a quick assessment and note a grade III systolic murmur and crackles in V.M.’s bases bilaterally. Vital signs are BP 176/102, P 94, R 28, T 97.8° F (36.6° C), and Spo2 78%. Peripheral pulses are equal and 3 +. He is alert and oriented; he tells you he is nauseated. His abdomen is nontender, with hypoactive bowel sounds in × 4 quadrants. Acting according to the standing orders for your institution, you start an intravenous (IV) line, obtain arterial blood gas (ABG) values, and draw blood for complete blood count with differential and basal metabolic panel. 7. The ED resident asks him whether he is in pain and whether he needs pain medication. V.M. answers “no” to both questions. Why did the ED resident ask these questions? Ans- V.M could be having a pain crisis in which the sickle blood cell is clustered and occluding blood flow to any area of the body resulting in pain, swelling, also based on his vital its evident that he is not feeling normal, his blood pressure and heart rate is high which is sometime sigh of pain. V.M may have extremely high tolerance for pain. 8. V.M.’s ABGs on 8 L O2 by simple face mask show Pao2 74 mm Hg. Is V.M. being adequately oxygenated? Ans- No, normal Pao2 is 80-100 on room air, while V.M is receiving additional oxygen 8 l. His Poa2 should be much higher. 9. Are your assessment findings consistent with fluid overload or deficit? What findings led you to your conclusion? Ans- Dyspnea and increased respiratory rate of 28, systolic murmur, crackles in V.M. base bilaterally and his blood pressure of 176/102 and pulse rate of 94. 10. Your institution uses electronic charting. Based on the assessment described, document your findings by providing a brief narrative note. □ Neurologic- the irregular shaped sickle cell can interrupt blood flow to the brain. Complete clogging of the blood flow to the brain can lead to an obvious stroke. □ Respiratory – A sickled RBC block the capillaries in the lungs resulting in decreased supply and exchanged of oxygen. Which can lead to high blood pressure □ Cardiovascular – SCD increase cardiac output with minimal increase in heart rate □ Gastrointestinal – this is rare but usually occur in the form of ischemic colitis □ Psychosocial-Affected in a negative way, patients are frequently in pain and medical problem which eventually they miss out on social events. Chart View Laboratory Test Values Sodium 137 mEq/L (137 mmol/L) Potassium 4.9 mEq/L (4.9 mmol/L) Chloride 110 mEq/L (110 mmol/L) CO2 16 mEq/L (16 mmol/L) BUN 27 mg/dL (9.6 mmol/L) Creatinine 2.7 mg/dL (239 mcmol/L) WBC 4300/mm3 (4.3 x 109/L) Hgb 7.8 g/dL (78 g/L) Hct 20.9% Platelets 208,000/mm3 (208 x 109/L) 11. Interpret V.M.’s lab results. Ans- V.M Co2 level is low, creatine level is high, hemoglobin level is low and hematocrit is low 12. V.M. complains of being short of breath. Do you believe the low Hgb level is responsible for his complaints? Ans-Low levels of hemoglobin prevent adequate oxygen from reaching the brain. Blood vessels swell, blood pressure drops, and it can result in headaches, neurological issues, and shortness of breath or fainting spells 13. What action will you expect the ED resident to take next and why? Ans- administer diuretic, corticosteroid and restrict sodium and fluid intake Case study progress The ED resident prescribes furosemide (Lasix) 40 mg IV push now, methylprednisolone (Solu-Medrol) 75 mg IV push now, and ceftriaxone 1 g IV piggyback after the furosemide (Lasix). 14. Indicate the expected outcome for V.M. associated with each medication he is receiving. AnsFurosemide is a loop diuretic that is used to treat fluid retention or in this case fluid overload, this will help V.M take the pressure of the fluid of his heart and lung. Methylprednisolone is used to alleviate V. M’s severe pain. Ceftriaxone is used to prevent infection. 15. The methylprednisolone 75 mg IV is supplied as a 125 mg/2 mL solution. Shade in the dose to be given on the syringe. 16. Identify 3 outcomes that you expect for V.M. as a result of your interventions. Ans- lower his chance of an infection, blood pressure will decrease and pain level, the fluid overload will decrease around his lung and heart. Case study progress V.M. voids 1900 mL within 2 hours of giving the furosemide. As V.M.’s dyspnea is relieved, he shakes the ED resident’s hand and thanks him for asking about the presence of pain and the need for pain medication. V.M. states, “One of my biggest fears is that I’ll come in here in crisis and the doctor won’t treat my pain aggressively enough. I don’t want to be labeled as a drug seeker or an emergency room abuser.” 17. Why would V.M. be concerned about obtaining adequate pain control in the ED? Ans- Because his pain crisis can eventually lead to death if not treated. 18. What issues will you discuss with V.M. before discharge? Ans- Any signs of infection to report to the ER immediately, ensure that vaccinations are up to date, good hand washing and avoid fluid loss and any sign of dehydration. Case study outcome V.M. is discharged from the ED after being observed for 6 hours. He is instructed to follow up with his primary care provider about the episode and discuss with the hematology clinic staff longer transfusion times for any future transfusions.