- In sickle cell disease, a mutation in the beta globin gene leads to production of sickle cell hemoglobin. A prominent clinical feature is vaso-occlusion by sickle hemoglobin, resulting in tissue ischemia.
- The term “sickle cell crisis” is frequently used to refer to an acute painful episode, although this term is not ideal, as it implies aggressive pain management, including with opioids, should be brought forth only when a patient is in “crisis.” There may also be an unintentional associate of catastrophizing or emotional instability associated with patients when using the term “crisis.”
- Extremities, back, chest, and abdomen are common locations for vaso-occlusive pain
Challenges
- A decrease in hemoglobin and increase in reticulocyte count accompanies an acute sickling event. A patient may present with acute on chronic sickle cell-related pain and not have significant abnormalities in their laboratory studies.
- Many of the same challenges that are present with other chronic pain syndromes are present with sickle cell pain: patient report is a key component of pain assessment.
- In general, analgesic efforts should proceed for a patient presenting with severe pain, before associated laboratory studies have been completed
- Chronic pain in SCD may stem not from acute vaso-occlusion but rather chronic bone and joint injury and central sensitization with opioid hyperalgesia. Many patients may have associated depression (greater than 25% by some reports), anxiety, and feelings of despair about their lifelong, hereditary condition. As such, a multi-disciplinary approach is needed –including, e.g., attention to mental health and social well-being.
Treatment recommendations
- Acute pain management should be multi-modal: acetaminophen, NSAIDs (if renal function is acceptable), and opioids. PCAs are frequently and reasonably employed.
- Ketamine infusions should be considered for patients with sickle cell pain, in particular if opioid-tolerance is present
Special considerations
- Indications of SCD complications other than vaso-occlusive pain include abnormal vital signs, major changes in CBC, abdominal distention, jaundice, hematuria, cough, and pain that for a given patient is atypical.
- Given the young age when opioid analgesia begins for patients with SCD and the lifelong nature of this condition, the development of opioid use disorder is a possibility –as is the case for other painful conditions. Be aware there may be unwarranted bias that SCD patients are at increased risk of opioid misuse or abuse. As a result of the racial and socio-economic demographics of patients with SCD, inappropriate withholding of pain care and negative attitudes toward this group may result.
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