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PATIENT PRESENTATION

Chief Complaint

“I’m glad I was able to be discharged from the hospital last week, but I’m tired of being here every month for a pain crisis!”

History of Present Illness

CH is a 14-year-old male with sickle cell disease (HbSS) who has had 3 admissions for pain crisis over the last six months. He was hospitalized last year with an acute chest syndrome episode.

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Missing Information?

Evaluate:

Patient Database

Drug Therapy Problems

Care Plan (by Problem)

TARGETED QUESTIONS

  1. What disease-related concerns do you have for CH at this time?

    Hint: See Treatment in PPP

  2. What factors in CH’s medical history do we need to consider when deciding upon medication therapy?

    Hint: See Treatment in PPP

  3. How should you initiate hydroxyurea therapy?

    Hint: See Treatment in PPP

  4. How will you monitor CH for safety and effectiveness of hydroxyurea?

    Hint: See Treatment and Table 71-2 in PPP

  5. What risks and adverse effects of therapy would you discuss with CH?

    Hint: See Treatment in PPP

FOLLOW-UP

One year later, CH’s has had 2 episodes of severe myelosuppression that recurred despite dose decreases and the decision has been made to discontinue hydroxyurea. What other pharmacotherapy options should be considered?

Hint: See Treatment in PPP

CASE SUMMARY

Global Perspective

Sickle cell disease (SCD) is an inherited group of red blood cell disorders that affects millions worldwide and is most common among those whose ancestry includes sub-Saharan Africa, regions in the Western Hemisphere (South America, the Caribbean, and Central America), Saudi Arabia, India, and Mediterranean countries such as Turkey. Because of population migration, SCD is present in most countries. SCD is a significant cause of morbidity and mortality. The World Health Organization estimates that SCD contributes to 5% of the deaths of children younger than 5 years of age in some African countries. Many young adults with SCD are at risk for premature death. Each year, more than 300,000 babies with severe forms of SCD are born worldwide, with the majority in low- and middle-income countries. The most cost-effective strategy for reducing the burden of SCD worldwide is to utilize both disease management with prevention programs, such as genetic counseling. Barriers include inequitable access to health services, lack of research initiatives and lack of awareness of the international community of the global burden of SCD and related disorders.

Key References

1. +
World Health Organization. https://www.afro.who.int/publications/sickle-cell-disease Accessed June 29, 2022.
2. +
Centers for Disease Control and Prevention. Sickle Cell Disease Clinical Guidelines. https://www.cdc.gov/ncbddd/sicklecell/recommendations.html Accessed June 29, ...

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