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Guideline For The Management Of Acute And Chronic Pain In Sickle Cell Disease.

This practice guideline was developed by an interdisciplinary committee of experts in the management of sickle cell pain that was assembled by the American Pain Society. A formalized strategy was used for literature search and review, and evidence was graded on a 5-class scale (I-V) according to predefined criteria. Strength of recommendation was also divided into 5 levels, based on quality of evidence: A, B, C, D and Panel Consensus, with A being the strongest recommendation and Panel Consensus being the weakest. The target audience for the guidelines is all healthcare professionals who work with patients with SCD. Panel members were required to disclose all potential conflicts of interest; no relationships were deemed significant. The manuscript does not identify a funding source.

Recommendation Level B
  • In the assessment of an acute painful event, the clinician should choose a simple measurement of pain intensity, reassess frequently, and record the measurement for treatment evaluation.
  • Meperidine should not be used if frequent or large doses or long treatment durations are anticipated.

Recommendation Level Panel Consensus
  • When a patient with severe pain presents to an emergency department (ED), opioids should be used immediately.
  • If the patient's pain is of short duration (less than 24 hours), opioids or opioid formulations with short duration of action are appropriate and have the additional advantage of quicker onset of action.
  • Intramuscular administration of medication should be avoided, if possible. For severe pain, intravenous administration is the route of choice, and opioids can be administered subcutaneously in patients with poor venous access.
  • Adjuvant medications (anti-inflammatory agents, antihistamines, and anxiolytics) can be used in combination with opioids to enhance efficacy and improve side effect profile.
  • Sickle cell pain is sufficiently intense that patient-controlled analgesia should be started only after satisfactory pain relief has been achieved with an aggressive opioid titration schedule.
  • In the management of acute vaso-occlusive pain crises, intravenous fluids should not exceed 1.5 times maintenance. The fluid of choice is 5% dextrose in half normal saline. (Note: this recommendation is abstracted from the National Heart, Lung, and Blood Institute document, introduced on page 4).

Source: Part III: Pain Management and Part IV: Treatment of Pain in Sickle Cell Disease, from the "Guideline for the Management of Acute and Chronic Pain in Sickle Cell Disease" copyright 1999 by the American Pain Society. Used with permission.

Editorial Commentary:

The vaso-occlusive pain crisis is the most common reason for ED visits related to SCD.2,3 It has been well documented that patients often receive inadequate treatment for their painful crises in the ED; this may be related to lack of familiarity with SCD or ED physician biases regarding opioid tolerance, physical dependence, and confusion with addiction.4 Improving the ED care of patients with acute pain has both ethical and economic value. In addition to alleviating suffering, evidence suggests that when patients with SCD receive high-quality, protocolized treatment from practitioners familiar with SCD, hospital admission rates and length of stay can be reduced. 

Because of the renal abnormalities prevalent in patients with SCD, NSAIDs and meperidine should be used with caution. Almost all patients with SCD will have some degree of renal insufficiency. Patients with SCD have supranormal proximal tubule function, which causes increased creatinine secretion. Consequently, serum creatinine underestimates the degree of renal dysfunction so that a patient with a creatinine level of 1.0 mg/dL may have significant renal dysfunction with decreased glomerular filtration rate (GFR). Compared to other opioids, meperidine is a relatively poor analgesic; however it is often requested because of its euphoric effects. The metabolite of meperidine, normeperidine, is a renally cleared cerebral irritant, and if it accumulates in the blood, adverse effects ranging from dysphoria to seizures can result. Because renal dysfunction in SCD is so common, meperidine should therefore be reserved for very brief treatment courses (no more than 48 hours at doses no higher than 600 mg/24 hours) for patients who have allergies or uncorrectable intolerances to other opioids. 

NSAIDs have been shown to hasten the decline of renal function in patients with SCD, but they are not contraindicated in the management of acute pain unless the patient has acute renal failure.6 Short courses may be helpful, but clinicians should not discharge patients with prescriptions for an extended regimen. Other adjuvant medications, such as acetaminophen and diphenhydramine, are safer than NSAIDs and have opiate-sparing effects. 

Patients who are admitted for vaso-occlusive crises are often placed on patient-controlled analgesia (PCA) pumps. Literature supports the use of PCA for the treatment of postoperative pain; however, acute SCD pain is typically more intense and long-lasting than postoperative pain, and using PCA as the initial analgesic modality may not be effective.7 In order for PCA to be effective in SCD vaso-occlusive crises, pain must first be controlled by an aggressive initial opioid titration; once relief (or significant improvement) is achieved, the patient can be placed on a PCA pump and admitted. 

Anecdotal and case series data suggest that too much IV fluid can precipitate acute chest syndrome when patients present with painful crises.8 Patients with acute pain crises are dehydrated and in need of intravenous hydration; however, bolus isotonic crystalloid is not recommended unless the patient is hypovolemic. The fluid of choice is 5% dextrose in half normal saline and infusion rates should not exceed 1.5 times maintenance. 

While vaso-occlusive crises dominate the sickle cell landscape for emergency clinicians, SCD patients are prone to numerous more sinister causes of pain. Pain should be treated aggressively and reassessed frequently at the same time that dangerous sources and precipitants of pain are excluded.

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Last Modified: 07/23/2017
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