The role of the pharmacist in the management of sickle cell disease revolves around appropriate pain management.
In 2008, the United Nations recognized June 19 as World Sickle Cell Day in an effort to raise awareness of an internationally prevalent genetic disease, sickle cell disease (SCD). Although millions of people worldwide have SCD, approximately 100,000 Americans are currently diagnosed, and approximately 1 in 13 African Americans carry the sickle cell trait.1.2
In particular, the role of the pharmacist in the management of sickle cell disease revolves around appropriate pain management, as the disease is known for its painful episodes, as well as preventive treatment against sickle cell crisis. . SCD manifests due to a single base substitution found in the beta-globin gene on chromosome 11, which results in valine replacing glutamic acid producing sickle-shaped hemoglobin S instead of l typical disc-shaped hemoglobin A.3
The altered form then triggers the vaso-occlusive events (sickle cell crisis) that lead to acute and chronic pain in these patients. Because pain is so subjective and variable in these patients, it makes management difficult in this population, allowing pharmacists to intervene to optimize treatment regimens.
In acute management, pharmacists can ensure adequate dosage, medications, and routes of administration, as these patients have typically developed tolerance to medications requiring higher doses of IV pure opioids, and their hypoxemic state, combined with poor venous access, suggests that these patients may derive more benefit from oral or subcutaneous administration.3
After acute treatment, pharmacists can play a vital role in the chronic pain management of these patients by ensuring they are effectively treating pain while using the safest long-term option. Some examples include ensuring patients are off opioid therapy after acute attacks to avoid withdrawal symptoms, performing frequent monitoring of liver and kidney function due to potential impairment due to compensation of hematological abnormalities to ensure proper dosing, the use of adjuvants for additional symptom relief, and the management of long-term opioid use, such as constipation.3
Additionally, with the opioid crisis making access to these necessary medications much more difficult for this patient population, pharmacists should serve as advocates for better access as well as educators to hesitant prescribers.4 Management of sickle cell disease should generally also include measures to prevent sickle cell crisis.
Hydroxyurea has been the mainstay treatment option for the prevention of painful episodes, acute chest syndrome hospitalizations, and blood transfusions, due to its ability to prevent vaso-occlusive events.3 Patients who have 3 or more episodes of pain in a year should be considered for initiation of hydroxyurea.
The pharmacist should confirm that doses are optimized based on weight, age, hemoglobin F levels and level of myelosuppression.3 Adherence to hydroxyurea can be a challenge in some patients.3
Pharmacists can help improve adherence through several traditional methods. Routine counseling sessions after in-hospital initiation of hydroxyurea on the importance of medication adherence for optimal results and education on what to practically expect based on the profile of adverse drug reactions can better understand treatment expectations to hopefully improve adherence rates.
This, in turn, can help reduce the 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 informed of the risks of this drug to fetal development and consider using it. other treatment options after stopping hydroxyurea.
Since the age demographics of sickle cell disease include more younger patients than many common comorbidities, recognition of risk during pregnancy is vital. Although some publications provide more optimistic data on hydroxyurea during pregnancy, the severity of teratogenic effects, especially in early pregnancy, is profound enough that some experts recommend stopping the drug before the patient becomes pregnant. .5
On the other hand, treatment with hydroxyurea may be considered in rare cases during the second and third trimesters when the risk of not taking the drug is thought to greatly outweigh the risk to the baby.4
More recently, in 2017, the FDA approved an additional preventative medication, Endari (L-glutamine oral powder), for SCD patients over the age of 5, which can be added to hydroxyurea to prevent sickle cell crises.3 Although the points of advice and the management of the pharmacist are similar to those of hydroxyurea, the advice of administration is more important because the drug must be taken twice a day and dissolved in liquid.
Endari is much more expensive than hydroxyurea, which makes access to this drug considerably difficult. Since patients with sickle cell disease can pay significant fees for their treatment, even with private insurance, pharmacists can play a huge role in advocating for better accessibility of care.6
While pharmacological management is essential in SCD pain management and preventive options, non-pharmacological management is also a key role that pharmacists play in this population. Some examples of this include cognitive behavioral therapy, massage therapy, acupuncture, attending social support groups.seven
In all patient care settings, pharmacists can chat and connect patients with resources to improve and prevent episodes of severe pain. Additionally, pharmacists are ideally placed to provide specific patient advice to parents of young people diagnosed with SCD, as management can be challenging.
SCD is one of the most common genetic diseases in the world. With minimal treatment options and generally low medication adherence rates, there is a clear need for intervention and improved management of this patient population.
Pharmacists can fill these gaps by increasing patient counseling on preventative medications, managing acute and chronic pain regimens and arguably most importantly, providing in-depth education to patients, caregivers and other members of the health care team.
- World Sickle Cell Day – Friday, June 19, 2020 // Sickle Cell Society. Sickle Cell Society. (2021, October 20). Retrieved May 15, 2022, from https://www.sicklecellsociety.org/wscd/
- House. Sickle Cell Association of America Inc. (nd). Retrieved May 15, 2022, from https://www.sicklecelldisease.org/
- Ikechukwu Okwerekwu, PDC (August 17, 2018). Management of sickle cell pain. American Pharmacist – The leading pharmacy journal. Retrieved May 15, 2022, from https://www.uspharmacist.com/article/sickle-cell-disease-pain-management
- Opioid crisis adds to pain for 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
- Montironi R, Cupaiolo R, Kadji C et al. Management of sickle cell disease in pregnancy: experience in a third level hospital and future recommendations. J Matern Fetal Neonatal Med.2020:1-3
- 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
- Williams PhD, H. (nd). Sickle cell disease: review of non-pharmacological approaches to pain: review article . Diary of Pain and Symptom Management. Retrieved May 15, 2022, from https://www.jpsmjournal.com/article/S0885-3924(15)00606-5/pdf