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Sickle Cell Anemia and the Management of the Adult Patient with Frequent Pain
(James R. Eckman) |
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A complete medical evaluation should be performed including physical examination, laboratory assessment, and imaging to define causes of the pain, exclude other medical conditions, and characterize sickle complications. Radiographic imaging of painful areas followed by an MRI may be useful in diagnosing bone marrow infarctions and avascular necrosis.
Thorough psychosocial assessment will define stressor, co-morbid conditions such as depression, coping strategies, and support systems. The individual's developmental stage, cognitive functioning, emotional stressors, and mood should be defined. Mood disturbances occur with pain and are predictors of level of pain. Control of pain improves mood, and treatment of underlying depression may improve pain coping. Cognitive coping strategies and negative thought patterns in patients and family members correlate with pain reporting. Influence of the pain on social functioning in the family, work or school performance, interactions with significant others, self-maintenance, and parental functioning should be assessed. Detailed vocational assessment is required for patients whose pain interferes with their ability to function at their job. |
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Etiologies of Chronic and Frequent Pain |
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Pain is a life long experience in patients with sickle cell disease. Management of pain is the overriding concern of the patient and the most common reason for seeking medical attention. Acute pain episodes are recurrent, unpredictable events that vary in frequency, severity, and duration within and between individuals with sickle disease.Episodes increase in frequence in early adulthood and individuals who average greater than six episodes per year have a reduced survival compared to those with less frequent events. A small minority of patients have very frequent pain episodes causing a significant burden ont the patient and their families. In these individuals, pain episodes impact negatively on comfort, daily activities, work and school performance, mood, quality of life, parenting, social interactions, and recreation. This group is also responsible for the vast majority of emergency room visits and hospital admissions. Appropiate management of this population can have major impact on their quality of life and their utilization of medical resources.
In addition to a subset of patients who have frequent acute pain episodes, adults with sickle cell disease can develop chronic pain syndromes. Patients with avascular necrosis and leg ulcers may have constant pain that causes severe discomfort, disrupting social and vocational functioning. Some patients have constant pain without clear etiology; however, bone films and MRI imaging often will identify multiple or diffuse bone infarctions. Patients with Hb SC and sickle-B+ thalassemia may actually be predisposed to bone complications as they become older. |
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Therapy of Frequent Acute and Chronic Pain in Sickle Disease |
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Frequently recurrent or chronic pain poses a very significant management problem for the patient and health care provider. Frequent use of narcotics result in tolerance, and higher doses of narcotics are frequently required to control acute pain episodes. For the patient who is well known to the physician, previous regiments that have been successful should be initiated promptly. Morphine-based therapy is generally preferred because sustained release, intermediate release oral, and rapid acting parenteral preparations are available allowing flexible dosing. Antihistamines should always be administered to prevent the almost universal itching that occurs from histamine release. Hydromorphone also comes in three dosing forms; however, it is more expensive in most settings. Long acting and intermediate acting combinations of oxycodone are available for patients that use meperidine parenterally.
Patient-controlled analgesia (PCA) utilizing a basal infusion of morphine sulfate from 1 to 3 mg with demand doses of 1 to 3 mg with a 5 to 8 minute lock out can also be used in paitents who are treated frequently. Demand doses alone should be used in the patients who are treated infrequently for acute pain. Hydromorphone can also be utilized for PCA in patients who are morphine intolerant; however, the doses must be 1/8 those used for morphine. Meperidine (demerol) is not recommended because of the potential for accumulation of normeperidine which causes significant CNS stimulation including seizures. |
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Evaluation of the Patient with Frequent Acute or Chronic Pain |
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Patients with frequent acute and chronic pain need periodic complete assessment to establish appropiate diagnostic and therapeutic intervention plans. Such evaluations should be obtained at a time when the patient is not being treated for acute pain. Evaluation requires a multidiscplinary team that can address the patient's medical, psychologic, social, and pharamacologic needs. Patient self-report should be the primary source of information; however, formal evaluation utilizing instruments like the McGill Pain Questionnaire, Brief Pain Inventory, Multidimentional Pain Inventory, and Pain Diary may be sueful in quantifying pain, determining mood, and assessing impact on functioning. Patient's report may also be supplemented by review of hospital and pharmacy records and family interviews.
Characterization of the pain and previous therapy includes location, severity and frequency of pain, precipitants and associated symptomatology. History defines frequency of home therapy, number of ER visits, hospitalization numbers and duration, and pattern of medication usage. The paitent's past methods of communicating pain quality and intensity are explored. A complete history of pharmacologic and non-pharmacologic interventions determines pain relief strategies that have worked best to relieve and prevent pain. Medication use, response, side effects, allergies ( including type of reaction ), and changes in treatment and response over time are detailed. |
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