Pain in sickle cell disease (SCD) is more often chronic than previously thought, though acute pain is still the hallmark of the disease. Both are undertreated. In adults and even in children, the quantity and severity of SCD pain may be vastly underestimated, because most of the “iceberg” of chronic SCD pain is “submerged” at home. The implications of this iceberg phenomenon are significant for pain treatment. Earlier, clinicians focused on adequate treatment of acute pain in SCD. It is still a challenge. Appropriate acute care triage of SCD crises to a high rather than low priority, coupled with individualized pain plans, can ensure timely, more effective acute therapy, and likely save hospitalizations. But a comprehensive focus requires remittive and palliative therapies for chronic pain treated at home. Three issues dominate the chronic pain debate. First, should opioids be the mainstay of chronic palliative SCD therapy? Second, does preventing sickling, preventing vasculopathy from SCD, preventing organ failure, or even curing SCD with bone marrow transplant, result in improvement of acute or chronic subjective pain? Third, do we have enough information to justify use of therapies intended for neuropathic or central pain, present in diseases as diverse as irritable bowel syndrome, temporo-mandibular joint syndrome, fibromyalgia, chronic pelvic pain, and rheumatoid arthritis?
Transformative SCD pain therapy would feature an array of interventions for acute and chronic SCD pain, and a full classification of pain phenotypes which qualified patients for acute and chronic pain palliative and remittive interventions.