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Three Part Question

In [sickle cell disease] is [there a higher incidence of opioid abuse] than [in the general population]?

Clinical Scenario

A 27 year old male with sickle cell disease presents to the Emergency Department with pain in the arms and legs with onset over the last 24hrs.He claims the pain is in keeping with his usual painful crises. The nurse tells you she is suspicious as he is requesting a specific dose of morphine. You wonder to yourself if there is a higher incidence of morphine abuse in people with sickle cell compared to the general population.

Search Strategy

MEDLINE (1966 to present)
EMBASE (1988 to present)
OVID: [sickl$.mp.] AND [exp Anemia/ or anaemia$.mp] OR [exp Anemia, Sickle Cell/ or sickle cell crisis.mp.] AND [painful.mp. and crisis.mp.] AND [drug seeking behaviour.mp.] AND [exp Narcotics]

Search Outcome

This search yielded 110 articles. Bibliographic references found in these articles were also examined to identify related literature. Only original research articles were included. Three articles directly addressed the question. The Cochrane library was also searched but no relevant results were found. Other mesh terms were also searched such as "addiction" but yielded no more relevant results.

Relevant Paper(s)

Author, date and country	Patient group	Study type (level of evidence)	Outcomes	Key results	Study Weaknesses
Elander J 2003 UK	Interviews were conducted with 51 patients with sickle cell disease	Survey	Substance abuse prevalence	When pain-related symptoms were included, 31% of the sample met DSM-IV criteria for substance dependence and 37% met the criteria for substance abuse.	Small study size Data depended on participants' 'self reports' therefore liable to bias.
Elander J 2004 Uk	Interviews were conducted with 51 patients with sickle cell disease	Survey	Substance abuse prevalence	No predictor variables were associated with pseudo-addiction playing a more important role than genuine analgesic dependence.	Small study size Data depended on participants' 'self reports' therefore liable to bias.
Waldrop RD 1995 USA	Surveys were mailed to all internal medicine, family practice, paediatric and ED (emergency department) attendings, residents and nurses who staff those services on October 1, 1993	Survey, Review	substance abuse prevalence	Staff physicians perceived a significantly smaller percentage of patients with pain to be opioid dependent in both groups. A significantly higher percentage of sickle cell patients were perceived to be opioid dependent in all health professional categories.	This study was relying on the memories of the physicians and comparing it to hospital logs so the accuracy may come into question

Comment(s)

A sickle cell crisis is a common, painful complication of sickle cell disease. The crisis which often presents with severe episodes of pain is the primary reason why these patients attend the emergency department. However all too frequently the pain is undertreated. The undertreating of pain is often down to the reluctance of physicians to give these patients adequate dosages of narcotic analgesia due to concerns about addiction, tolerance and side effects.(2) Physicians tend to overestimate the prevalence of opioid dependence in patients with sickle cell crises. Yet the incidence of opioid analgesic addiction in patients with sickle cell disease has been reported as being no higher than three percent.(2) It has been found that undertreatment of pain can lead to 'pseudoaddiction' where reports of pain by the patient are not acknowledged so the patients resort to exaggerated or manipulative pain behaviours which often reinforce the staffs' perceptions of substance dependence.(3) The evidence suggests that a high percentage of sickle cell patients are perceived to be opioid dependant when in fact the percentage of sickle cell patients who are opioid dependant is no higher than the general population.(1) The impression of addiction may be given as sickle cell patients may be very knowledgeable about the dose and type of medication to treat their pain. They may also show signs of withdrawal as most patients with sickle cell crisis require prolonged treatment with opioids and so will develop some form of tolerance. It is therefore important to be able to distinguish this type of tolerance and withdrawal from addiction.

Clinical Bottom Line

The incidence of addiction, though hard to measure, seems to be no greater in sickle cell patients that in the general population. Therefore clinicians should be encouraged to treat the pain experienced by the patient fully. Evidence suggests that undertreating the pain can lead to pseudoaddiction, early readmission and increased fear of future crisis episodes.

References

1. Elander J et al Pain management and symptoms of substance dependence among patients with sickle cell disease Social science and medicine 2003;1683-166
2. Elander J et al Understanding the causes of problematic pain management in sickle cell disease: Evidence that pseudoaddiction plays a more important role than genuine analgesic dependence. Journal of pain and symptom 2004; vol 27
3. Waldrop, RD et al Health Professional perceptions of opioid dependence among patients with pain American journal of emergency medicine 1995; vol 13
4. Steven H et al Approach to the Vaso-occlusive Crisis in Adults with Sickle Cell Disease American Family Physician 2000;1349-56
5. Waldrop and Mandry et al Opioid pseudoaddiction: An iatrogenic syndrome.Pain 1989;363-366
6. Brozovic, M et al Pain relief in sickle cell crises Lancet 1986;624-625