Algorithm for reducing acute pain episodes in individuals with SCD

In addition to medical therapy, all individuals with SCD-associated pain should have access to the following:

A good therapeutic relationship with their provider
An individualized care plan
Opioids and other analgesics for acute pain when needed
Assistance with managing other causes of pain such as orthopedic or menstrual pain
General health maintenance including adequate sleep, assistance with social and psychiatric supports, and healthy nutrition

Refer to UpToDate for additional information regarding an approach to management and prevention of pain.

SCD: sickle cell disease, including HbSS, HbS-beta⁰-thalassemia, and occasionally other variant sickle syndromes such as HbSC disease or sickle-beta⁺-thalassemia; MTD: maximum tolerated dose based on hematologic parameters; CBC: complete blood count; HbF: fetal hemoglobin.
* Hydroxyurea is recommended for the following individuals:

Infants >9 months regardless of whether they have pain episodes (possibly infants 6 to 9 months with acute pain or dactylitis as well)
Older children, adolescents, and adults with >1 vaso-occlusive episode (acute pain episodes, acute chest syndrome)

Hydroxyurea should generally be avoided in individuals planning conception (male or female) and during pregnancy and breastfeeding, although some centers continue hydroxyurea or reinstitute it after the first trimester when embryogenesis is complete. These decisions require close discussions between the individual and their clinician with shared decision making.
¶ Assessment of adequate pain prevention is individualized with shared decision making. Ideally, pain episodes do not recur. Some individuals who have mild to moderate pain episodes once or twice a year or less may find pain control to be adequate; those with 3 or more episodes per year requiring medical intervention or hospital admission are likely to derive the greatest benefit from additional therapies. Likewise, drug tolerability may differ among different individuals. Some individuals may not tolerate hydroxyurea due to hematologic toxicity, hair loss, gastrointestinal symptoms, or concerns about fertility and pregnancy.
Δ Hydroxyurea should be continued for at least 3 to 6 months to determine if it is effective. Other agents include L-glutamine, voxelotor, and crizanlizumab. In most cases, the other agents are added to hydroxyurea. However, in selected cases in which hydroxyurea is not well tolerated, other agents may be used instead. The decision to add another agent and the choice among them is individualized according to the patient’s age, predominant symptoms, comorbidities, and values and preferences regarding the differing burdens of each therapy. The advantages and disadvantages of these other agents are summarized in a table in UpToDate.
◊ Monitoring depends on dose titration and response:

Every 4 weeks during titration to MTD
Every 2 to 3 months once MTD is reached
Every 3 to 6 months once stable values for the CBC and HbF level are established

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Algorithm for reducing acute pain episodes in individuals with SCD