Module 4 Chapter 11 Irie case study

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University of Cincinnati, Main Campus *

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NSBN2002C

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Medicine

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Dec 6, 2023

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docx

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2

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Name: Case Study: Irie Part 1: Irie is a 9-day old female, born to immigrant parents Claude and Arlene. She was delivered vaginally at 39 weeks and 2 days gestation. Claude and Arlene are new to the area, having immigrated from the West Indes just 2 months prior to delivery. Arlene had not established prenatal care in the US prior to delivery, but had a midwife in the West Indes. Irie is their third born child. Her older brother, CJ is 12 and older sister, Marley is 10. CJ and Marley were born in the West Indes. Arlene used the services of a midwife for their deliveries and delivered both at home. Irie was discharged home from the hospital on day of life 2. Heel stick sampling to obtain the Ohio Newborn Screening test was done prior to Irie’s discharge from the hospital, the newborn was examined by a neonatal hospitalist and was determined to be healthy. You have just received referral information to establish Irie in your pediatric clinic and a phone call from the ODH lab indicating that Irie’s newborn screening has an “out-of-range" result for Hb SS. What is the Ohio Newborn Screening Test? What does an “out-of-range" result for Hb SS indicate? Part 2: You notify your pediatrician on call about Irie’s referral and newborn screening results. The pediatrician asks you to reach out to the family to inform them of the screening result, and schedule Irie to be seen as soon as possible in clinic for an exam and lab work. What lab work can you expect the pediatrician to order? Who will the pediatrician likely consult with to discuss Irie? What would you like to know about Irie’s family history?
Part 3: You have completed blood samples on Irie and sent them to the lab for stat processing and the pediatrician is examining Irie for splenomegaly. Claude and Arlene state there are no known family members with sickle cell disease or sickle cell trait, but they do not recall ever being screened, or having CJ or Marley screened. Claude remembers a cousin that died young that was very sickly, and in constant pain, but it was in an undeveloped village in the West Indes many years ago, and no one was able to explain what happened. The pediatrician has prescribed penicillin prophylaxis for Irie until confirmatory testing is complete. You must teach Claude and Arlene about the most serious complications of sickle cell disease to watch for. What early but serious complications of sickle cell disease should Claude and Arlene watch for while waiting for confirmatory tests? How will you explain sickle cell disease to Claude and Irene? Part 4: Irie’s lab work confirms that she has sickle cell anemia. She is going to be seen by a hematologist next week and because CJ and Marley have never been tested or screened, the family has been referred for genetic counseling and testing. How is sickle cell disease inherited? What is the difference between sickle cell trait and sickle cell disease? What are the odds that CJ has sickle cell trait? What are the odds that he has sickle cell disease? What other complications do patients with sickle cell face beyond sepsis and splenic sequestration? What types of treatment do sickle cell patients often need?
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