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Pain Management for Rare Diseases

1. What is the decision or change (e.g. clinical topic, practice guideline, system design, delivery of care) you are facing or struggling with where a summary of the evidence would be helpful?

Pain management for patients with rare diseases including sickle cell disease has been adversely affected by guidelines limiting the use opioid that name only a few common conditions like cancer. The unanticipated consequence of response to the "opioid epidemic" has discarded evidence-informed practice in rare diseases and causing great stigma, suffering, and unnecessary barriers as many people in the healthcare system block them from legitimate pain control. This misapplication of guidelines "only affects a few people" but these vulnerable people are being severely harmed because the providers do not trust anybody.

2. Why are you struggling with this issue?

There is variation in what healthcare providers are doing in practice, such as ED's that proudly claim they are opioid-free when there are patients whose best-practices care needs opioids—this is akin to making an ED free of antibiotics as a way to demonstrate a response to multidrug resistant bacteria. Patients are suffering from misapplication of guidelines that are too simplistic, from people who are meeting them for the first time, disregarding the plans from long-term care providers.

3. What do you want to see changed?

How will you know that your issue is improving or has been addressed? Build individualized pain management plans into every ED and electronic medical record system and pharmacy order plan. An Individualized pain management plans for chronic disease that is constructed and approved by the continuity doctor should be accepted by unfamiliar personnel as a legitimate authorization to have opioids for pain control. The guidelines should recognize that there are exceptions and reward medical personnel for higher quality care when they recognize and act correctly on this personalized approach to medicine, rather than considering these exceptions to be signs of poor quality.

4. When do you need the evidence report?

Fri, 11/01/2019

5. What will you do with the evidence report?

Bring it to policy-makers, administrators of EDs and hospitals, pharmacy managers, professional societies practice guidelines, training for physicians and nurses and pharmacists. Persuade patients that building these plans is another reason to maintain continuity of care for chronic disease.

==Supporting Document==

NHBLI Evidence-Based Guidelines for Sickle Cell Management 2014 (PDF, 260 KB)

Page last reviewed June 2019
Page originally created May 2019

Internet Citation: Pain Management for Rare Diseases. Content last reviewed June 2019. Effective Health Care Program, Agency for Healthcare Research and Quality, Rockville, MD.

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