World Sickle Cell Day: The Pharmacist’s Role in Medication Management

In 2008, the United Nations recognized June 19 as World Sickle Cell Disease Day with the goal of increasing awareness of a prevalent genetic disease at the international level, sickle cell disease (SCD). Although millions of people worldwide have SCD, there are approximately 100,000 Americans currently diagnosed and roughly 1 in 13 African Americans carry the sickle cell trait.^1,2

Notably, the role of the pharmacist in SCD management revolves around appropriate pain management, because the disease is known for its episodes of pain, as well as preventative therapy against sickle cell crisis. SCD manifests due to a single base substitution found in the beta-globin gene of chromosome 11, which results in valine replacing glutamic acid producing sickle shaped hemoglobin S instead of the typical disk-shaped hemoglobin A.³

The altered shape subsequently triggers the vaso-occlusive events (sickle cell crisis) that lead to the acute and chronic pain of these patients. Because pain is so subjective and variable among these patients, it makes management difficult in this population, allowing pharmacists to step-in to optimize treatment regimens.

In acute management, pharmacists can ensure adequate dosing, medication, and administration routes because these patients typically have developed drug tolerance requiring higher doses of IV pure opioids, and their hypoxemic state, combined with poor venous access, suggests these patients may get more benefit from oral or subcutaneous administration.³

Following acute treatment, pharmacists can be essential in managing these patients’ chronic pain medications by ensuring they effectively treat the pain while using the safest long-term option. A few examples include making sure patients are tapered off opioid treatment following acute attacks to avoid withdrawal symptoms, completing frequent monitoring of hepatic and kidney function due to potential weakening from compensating for hematologic abnormalities to ensure appropriate dosing, use of adjuvants for additional symptom relief, and management of long-term use of opioids, such as constipation.³

Furthermore, with the opioid crisis making access to these necessary medications much more difficult for this patient population, pharmacists should serve as advocates for improved access as well as educators to prescribers that are hesitant.⁴ Management of SCD generally should also include measures to prevent sickle cell crisis.

Hydroxyurea has been the mainstay treatment option for the prevention of pain episodes, acute chest syndrome hospitalization, and blood transfusions, due to its ability to prevent vaso-occlusive events.³ Patients who have 3 or greater pain episodes in a year should be considered for initiation of hydroxyurea.

The pharmacist should confirm doses are optimized based on weight, age, hemoglobin F levels, and level of myelosuppression.³ Adherence with hydroxyurea may be a challenge in some patients.³

Pharmacists can help improve adherence through several traditional methods. Routine counseling sessions following hospital initiation of hydroxyurea on the importance of medication adherence for optimal results and the education on what to practically expect based on the medication’s adverse effect profile can increase understand expectations of the treatment to hopefully improve adherence rates.

In turn, this may help reduce sickle cell crisis and subsequent hospitalizations and/or opioid use. Because hydroxyurea is classified as pregnancy category D, all patients initiated on it, other health care providers, and potentially pregnant women should be counseled on the risks of this medication to fetal development and address other treatment options following discontinuation of hydroxyurea.

Considering the age demographics of SCD include more patients of a younger age than many common comorbidities, the recognition of the risk in pregnancy is vital. Although there is some literature that provides more optimistic data on hydroxyurea in pregnancy, the severity of the teratogenic effects, particularly in early pregnancy, are profound enough that some experts recommend stopping the medication before the patient becomes pregnant.⁵

On the other hand, hydroxyurea therapy may be considered in rare cases during the second and third trimesters in which the risk of not being on the medication is thought to substantially outweigh the risk to the baby.⁴

More recently in 2017, the FDA approved an additional prevention medication, Endari (L-glutamine oral powder), for SCD patients older than 5 years of age, which can be added on to hydroxyurea to prevent sickle cell crises.³ Although pharmacist counseling points and management is similar to hydroxyurea, administration counseling is more important because the medication must be taken twice a day and dissolved in a liquid.

Endari is much more expensive than hydroxyurea, making access to this medication significantly difficult. Because SCD patients may pay significant out-of-pocket costs for their treatment even with private insurance, pharmacists can play a huge role in advocating for improved affordability of care.⁶

While pharmacologic management is essential in SCD pain management and preventative options, nonpharmacologic management is a key role pharmacists play in this population as well. Some examples of this include cognitive behavioral therapy, massage therapy, acupuncture, participation in social support groups.⁷

In all patient care settings, pharmacists can discuss and connect patients with resources to improve and prevent severe pain episodes. Furthermore, pharmacists are optimally placed to provide patient-specific counseling to parents of young individuals diagnosed with SCD because management can be difficult.

SCD is one of the most common genetic diseases in the world. With treatment options being minimal and medication adherence rates typically being low, there is a clear need for intervention and improvement in the management of this patient population.

Pharmacists can address these gaps by increasing patient counseling on preventative medications, managing acute and chronic pain regimens, and arguably the most important, providing thorough education to patient, caregivers, and other health care team members.

References

1. World sickle cell day - friday 19th june 2020 // Sickle Cell Society. Sickle Cell Society. (2021, October 20). Retrieved May 15, 2022, from https://www.sicklecellsociety.org/wscd/
2. Home. Sickle Cell Disease Association of America Inc. (n.d.). Retrieved May 15, 2022, from https://www.sicklecelldisease.org/
3. Ikechukwu Okwerekwu, P. D. C. (2018, August 17). Sickle cell disease pain management. U.S. Pharmacist – The Leading Journal in Pharmacy. Retrieved May 15, 2022, from https://www.uspharmacist.com/article/sickle-cell-disease-pain-management
4. Opioid crisis adds to pain of sickle cell patients. National Heart, Lung, and Blood Institute. (2017, September 15). Retrieved May 20, 2022, from https://www.nhlbi.nih.gov/news/2017/opioid-crisis-adds-pain-sickle-cell-patients
5. MontironiR, Cupaiolo R, Kadji C et al. Management of sickle cell disease during pregnancy: experience in a third-level hospital and future recommendations. J Matern Fetal Neonatal Med.2020:1-3
6. The Cost of Living with Sickle Cell Disease. American Society of Hematology. (2022, May 16). Retrieved May 20, 2022, from https://www.hematology.org/newsroom/press-releases/2022/the-cost-of-living-with-sickle-cell-disease
7. Williams PhD, H. (n.d.). Sickle Cell Disease: A Review of Nonpharmacological Approaches for Pain: Review Article . Journal of Pain and Symptom Management . Retrieved May 15, 2022, from https://www.jpsmjournal.com/article/S0885-3924(15)00606-5/pdf