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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A questionnaire was sent to principal investigators of NIH-sponsored clinical research in sickle cell disease. Twenty of 21 respondents indicated they used parenteral narcotic analgesics for pain episodes sufficiently severe to warrant hospitalization. Eleven used meperidine; seven, morphine; and one each, nalbuphine, hydromorphone, and acetaminophen with codeine. They gave the agents at frequent, regular intervals or by continuous infusion. A total of 41 of more than 3,500 patients required chronic transfusion for pain control. Complications included meperidine-associated convulsions reported by nine respondents and addiction by six. This information indicates that vigorous pain-control methods are used at institutions having a special interest in providing medical care for children with sickle cell disease.
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PMID:Survey of pain management therapy provided for children with sickle cell disease. 137 56

Pain is the most common problem encountered by patients with sickle cell disease (SCD). We report the results of a survey sent to hematologists and emergency department (ED) physicians regarding their perceptions and practices concerning pain and its management. Hematologists and ED physicians differed considerably in their perceptions about the natural history of the pain, and about the percentage of patients who are addicted to analgesics. Fifty-three percent of the ED physicians and 23% of the hematologists thought that more than 20% of patients are addicted. These beliefs and perceptions about SCD-related pain and the prevalence of addiction must be addressed if clinical care is to be changed substantively.
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PMID:Sickle cell-related pain: perceptions of medical practitioners. 967 Jun 33

The vaso-occlusive crisis, or sickle cell crisis, is a common painful complication of sickle cell disease in adolescents and adults. Acute episodes of severe pain (crises) are the primary reason that these patients seek medical care in hospital emergency departments. Frequently, however, the pain is incompletely treated. Despite advances in pain management, physicians are often reluctant to give patients adequate dosages of narcotic analgesics because of concerns about addiction, tolerance and side effects. It is important to recognize a pain crisis early, correct the inciting causes, control pain, maintain euvolemia and, when necessary, administer adequate hemoglobin to decrease the hemoglobin S level. The family physician and the hematologist must work together to treat acute pain episodes promptly and effectively, manage the long-term sequelae of chronic pain and prevent future vaso-occlusive crises.
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PMID:Approach to the vaso-occlusive crisis in adults with sickle cell disease. 1073 42

Professional objectivity should be the primary focus of patient care. Health care professionals are at times reluctant to give opioids out of fear that patients may become addicted, which would result in the undertreatment of pain. The influence of nurses' attitudes on the management of sickle cell pain was studied. The variables of age, education, area of practice, and years of active experience were considered. Of the respondents, 63% believed addiction was prevalent, and 30% were hesitant to administer high-dose opioids. Study findings suggest that nurses would benefit from additional education on sickle cell disease, pain assessment and management, and addiction. Educational recommendations are discussed.
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PMID:Nurses' attitudes and practices in sickle cell pain management. 1169 21

The unpredictable, recurrent, intense, and frequently persistent nature of pain associated with sickle cell disease poses a difficult challenge in terms of management. A wide variability exists in the way painful episodes are managed. Variations in practice reflect different views about the suitability of opioids, the efficacy of parenteral administration, and the risk of dependence on opioids. Consequently, the acute and chronic pain associated with sickle cell disease often is undertreated or inappropriately managed. Although medical staff fear that patients might abuse pain medication and become psychologically dependent, patients are more concerned about the side effects associated with analgesics. Some patients may persuade staff to give them more analgesics, engage in clock-watching, and request specific medications or dosages; these patients often are perceived as manipulative or demanding. However, these patients are knowledgeable about their medications and doses that have worked in the past. Requests for specific medications and dosages should not be interpreted as indications of drug-seeking behavior, but clinicians should communicate with these patients, make accurate assessments, and provide adequate doses of opioid analgesics. The American Pain Society recognized that the undertreatment of pain and inappropriate management of pain in sickle cell disease seem to be common. A Clinical Practice Guideline was developed to provide evidence-based recommendations that could potentially improve pain management. The purpose of this report is to describe the pharmacologic strategies used to manage pain associated with sickle cell disease, examine issues and challenges related to pain management as well as concerns and fears related to addiction, and explain the administration of opioids as recommended by the American Pain Society.
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PMID:Pain management in sickle cell disease. 1174 47

Sickle cell disease affects 70,000 Americans who experience an average of 0.8 painful episodes each year. The pathophysiology of sickle cell pain is not completely understood. The disease is characterized by both acute and chronic pain syndromes. Patients with sickle cell pain often encounter barriers to receiving appropriate care, including lack of continuity of care and perceived opiate addiction. Studies describing pharmacotherapy for sickle cell pain have been primarily retrospective and uncontrolled. In analyzing the available literature regarding pathophysiology, assessment, and treatment of sickle cell pain, we found a need for increased practitioner education and intervention to improve the level of care provided to patients with this disease.
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PMID:Treatment of sickle cell pain. 1193 83

There has been widespread speculation that patients with sickle cell disease (SCD) may become drug dependent if their painful crisis is treated with narcotics. However, there has been no scientific evidence to support this assertion. Paradoxically, individuals suffering from sickle cell disease who are not adequately treated may develop an addiction to narcotics due to self-medication to treat their pain. In this article, we describe a 38-year-old African American woman who became addicted to cocaine due to self-medication of her sickle cell pain with cocaine.
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PMID:Drug addiction in sickle cell disease: case report. 1290 47

Many physicians believe that patients with sickle cell disease (SCD) are more likely to become addicted to pain medication than are other patient populations. This study hypothesizes that physicians' attitudes towards addiction in patients with SCD affects pain management practices. The Physician Attitudes Survey was sent to 286 physicians at seven National Institutes of Health-funded university-based comprehensive sickle cell centres. The survey assessed demographic information; and physician's attitudes toward and knowledge of pain, pain treatment, and drug addiction and abuse. Significant Pearson product-moment correlations were found between attitudes towards pain and beliefs regarding addiction to prescribed opioids. Physicians reported varied pain management strategies, however, many believe that attitudes toward addiction and to patients in pain crises may result in undertreatment of pain. These results indicate that physicians might benefit from additional education regarding sickle cell disease, addiction to pain medication, the pharmacology of opioids, and the assessment and treatment of pain.
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PMID:Physicians' attitude and practices in sickle cell disease pain management. 1648 93

Judgments about people with pain are influenced by contextual factors that can lead to stigmatization of patients who present in certain ways. Misplaced staff perceptions of addiction may contribute to this, because certain pain behaviors superficially resemble symptoms of analgesic addiction. We used a vignette study to examine hospital staff judgments about patients with genuine symptoms of analgesic addiction and those with pain behaviors that merely resemble those symptoms. Nurses and doctors at hospitals in London, UK, judged the level of pain, the likelihood of addiction, and the analgesic needs of fictitious sickle cell disease patients. The patient descriptions included systematic variations to test the effects of genuine addiction, pain behaviors resembling addiction, and disputes with staff, which all significantly increased estimates of addiction likelihood and significantly decreased estimates of analgesic needs. Participants differentiated genuine addiction from pain behaviors resembling addiction when making judgments about addiction likelihood but not when making judgments about analgesic needs. The treatment by staff of certain pain behaviors as symptoms of analgesic addiction is therefore a likely contributory cause of inadequate or problematic hospital pain management. The findings also show what a complex task it is for hospital staff to make sensitive judgments that incorporate multiple aspects of patients and their pain. There are implications for staff training, patient education, and further research.
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PMID:Factors affecting hospital staff judgments about sickle cell disease pain. 1649 11

We report a case of bone pain associated with primary hyperparathyroidism in a patient with sickle cell disease. A 17-year-old girl with sickle cell disease (SS phenotype) was seen for bilateral knee and back pain. She had had recurrent severe vaso-occlusive crises and acute chest syndrome in the course of her disease. In the last 2 years, she had frequent visits to the emergency department for severe bone pain. She complained of long-standing fatigue and lethargy. Her physical examination was normal. Hydroxyurea treatment, as well as and long- and short-acting narcotics were given, with little improvement in symptoms. Poor compliance with medication, family dysfunction, and potential narcotic addiction were felt to be significant contributors to the patient's symptoms. She was incidentally found to have an extremely elevated total calcium level of 3.19 mmol/L (range: 2.25-2.76) with an ionized calcium level of 1.9 mmol/L (range: 1.15-1.35). Phosphorus level was 0.82 mmol/L (range: 0.90-1.50), alkaline phosphatase level was elevated at 519 U/L (range: 10-170), and parathyroid hormone level was extremely high at 1645 pg/mL (range: 10-60). Her renal function was normal. Ultrasonography of the neck and a Sestamibi scan revealed a single left inferior parathyroid adenoma adjacent to the thyroid lobe. There was no evidence of an underlying multiple endocrine neoplasia. The patient was diagnosed with primary hyperparathyroidism. Fluid hydration, hydrocortisone, calcitonin, and bisphosphonates were initiated for acute hypercalcemia management before surgical excision of the left parathyroid adenoma. On review of previous blood work, a borderline calcium level of 2.72 was present 18 months before this admission. Two years postsurgery, she has normal renal function, calcium, and parathyroid hormone levels. The weekly visits to the emergency department for pain episodes decreased to 1 every 2 months within the first few months after her surgery. The decrease in pain episodes, even if it coincided with the treatment of primary hyperparathyroidism, may still reflect the natural evolution of sickle cell disease in this patient. However, the high morbidity associated with primary hyperparathyroidism was successfully prevented in this patient. Primary hyperparathyroidism is rare in childhood. In a recent study, it occurred more commonly in female adolescents and was because of a single adenoma, as in our patient. Significant morbidity, mainly secondary to renal dysfunction, was because of the delay in diagnosis after the onset of symptoms (2.0-4.2 years), emphasizing the need for a rapid diagnosis. Sickle cell disease affects approximately 1 of every 600 blacks in North America. Acute episodes of severe vaso-occlusive crisis account for > 90% of sickle cell-related hospitalizations and are a significant cause of morbidity in patients. There is no known association between sickle cell disease and primary hyperparathyroidism, and this case is most probably a random occurrence. However, as emphasized by this case report, pain may also be a harbinger of other disease processes in sickle cell disease. Because management may vary, we suggest that care providers consider the diagnosis of vaso-occlusive crisis as the diagnosis of exclusion and that other etiologies for pain be envisaged in this patient population, especially in the presence of prolonged pain or unusual clinical, radiologic, or biological findings.
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PMID:Primary hyperparathyroidism mimicking vaso-occlusive crises in sickle cell disease. 1688 90


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