Sickle-Cell Disease (SCD) and Related Pain
By Clarence Herring, Jr., CPh, Pharm.D.
Assistant Directorof Pharmacy Services
Tallahassee Memorial HealthCare
Posted: 4:49p.m .est, October 08, 2006
Clarence Herring, Jr., Pharm. D.
Tallahassee Memorial HealthCare
Assistant Director Inpatient Pharmacy Services
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Sickle-Cell Disease is a term that encompasses a group of inherited blood disorders for which Hemoglobin S is the dominant hemoglobin. The featured hallmark of this disease is pain. Although Sickle-Cell Disease can be found in various parts of the world in persons of African, Mediterranean, Middle-Eastern, Asian and East Indian heritage, it predominantly affects African American, African/Hispanic, Caribbean, and South American people in the United States.
Pain in the leading cause of emergency department visits and hospitalization, and is the major focus of home management for people with Sickle-Cell Disease. Severe pain can be experienced as early as six months of age, and can occur unpredictably at varying intervals throughout the course of a person’s life. How pain is discussed and managed during childhood affects an individual’s reaction and ability to cope with the pain as an adult. Older children and adults are faced not only with their present pain, but also with the memory of past episodes of pain, and future pain episodes.
It is probably best to first recognize the need for adequate assessment and management of Sickle-Cell pain. There are, however, some barriers that affect the understanding of this disease such as
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Clinicians’ disbelief of patient’s reports of intense pain and the amount of medication required for treatment. (Patients get inadequate dosing of pain reducing medications.)
The variability and unpredictability of the acute episodes of pain. (Because of the different states of pain associated with Sickle-Cell Disease and underlying causes, it is very difficult to predict pain.)
Biases based upon inadequate knowledge of opioid use (e.g. patients’, families’, and clinicians’
fears of addiction).
Commonly occurring racial, ethnic, and socioeconomic factors that affect communication between clinician
and patient.
Limited access to care for some patients. |
Fortunately, with concerted effort and education, some of these barriers can be overcome. Although Sickle-Cell Disease is not yet curable, except possibly by bone marrow transplantation in selected cases, pain can be effectively managed in a vast majority of patients by using the currently available therapies. The focus will be on pharmacological management.
The pharmacological management of pain, including pain associated with SCD, consists of the use of NSAIDs (Non-Steroidal Anti-Inflammatory Drugs), opioids, and adjuvant medications. NSAIDs form the backbone of the management of mild-to-moderate pain. These agents have ceiling doses not resulting in increased analgesia. All NSAIDs are administered orally, except for ketorolac, which can be used orally or parenterally. Some of the over-the-counter NSAIDs include: Aspirin, Ibuprofen, Ketoprofen and Naproxen Sodium. There are prescription NSAIDs which include: Choline Magnesium, Diclofenac, Etodolac, and Fenoprofen Calcium. These product are not with out problems in themselves sometimes casuing or increasing problems associated with liver and kidney function. These drugs should be used with caution or only on the advice of your physician, if you suffer from gastric, peptic ulcers, or have a bleeding disorder (coagulopathies), and renal failure.
Opioids are used to treat moderate-to-severe pain. Opioid maintenance therapy for patients with Sickle-Cell Disease should be considered only after all other reasonable attempts to achieve pain relief have been made. Most commonly used opioids include: Hydromorphone, Codeine, Oxycodone, Hydrocodone, Methadone, Levorphanol and Fentanyl. This group of drugs requires a balance between maximizing pain relief and minimizing side side effects. Some side effects include sedation, nausea, constipation and respiratory depression to name a few.
The intent of this article is not to minimize the treatment of SCD, but bring to mind drugs that are used in some instances to treat the symptoms of this disease.
