sickle haemoglobin polymerises to cause vaso-occlusive crisis. Chronic complications can also set in.

It goes without doubt that the vaso-occlusive crisis (hereafter termed VOC) is the most common acute complication of sickle cell disease. It manifests as acute severe pain. 

Much as a few people report a gradual onset of VOC, this typically presents as sudden onset excruciating pain, and almost all people living with sickle cell disease will experience a vaso-occlusive crisis during their lifetime. 

VOCs can manifest as early as six months in the form of dactylitis. In children and adults, extremities, chest, and back are the most common regions for VOCs. When pain occurs anywhere, think about the other possible causes of this pain, for example, headache for an impending stroke, pain in the flanks for papillary necrosis, and abdominal pain for either hepatic or splenic sequestration or constipation due to opioid toxicity.

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Health Management for People with Sickle Cell Disease

 

We outline the recommendations for the management of the vaso-occlusive crisis. We have adapted them from the expert panel report on evidence-based management of sickle cell disease, and they work in all settings where patients present with VOC.

  1. In adults and children with SCD and pain, when indicated, initiate a diagnostic evaluation of the causes of pain other than a VOC while beginning to treat pain.
  2. In adults and children with SCD and a VOC, it is crucial to determine characteristics, associated symptoms, location, and intensity of pain based on patient self-report and observation. If the VOC pain is atypical, investigate other possible causes of pain.
  • Rapidly assess the recent analgesic usage, both opioids, and nonopioids.
  • Rapidly initiate analgesic therapy within 30 minutes of triage or 60 minutes of registration.
  • Base the analgesic selection should on pain assessment, associated symptoms, outpatient analgesic use, patient knowledge of effective agents and doses, and experience with side effects.
  1. In adults and children with SCD and a VOC, use an individualized prescribing and monitoring protocol or an SCD-specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of the VOC.
  2. In adults and children with SCD and a VOC associated with mild to moderate pain who report relief with NSAIDs in the absence of contraindications to the use of NSAIDs, continue treatment with NSAIDs.
  3. In adults and children with SCD and a VOC associated with severe pain, rapidly initiate treatment with parenteral opioids.
  4. In adults and children with SCD and a VOC associated with severe pain,
  • Calculate the parenteral (IV or subcutaneous) opioid dose based on total daily short-acting opioid dose currently being taken at home to manage the VOC.
  • Administer parenteral opioids using the subcutaneous route when intravenous access is difficult.
  • Reassess pain and re-administer opioids using if necessary, for continued severe pain every 15-30 minutes until it is under control per patient report.
  • Maintain or consider the escalation of the dose by 25% until you control the pain.
  • Reassess after each dose for pain relief and side effects.
  • Initiate around-the-clock opioid administration by patient-controlled analgesia (PCA) or frequently scheduled doses versus as requested (PRN) administration.
  1. If ordering around-the-clock, continuous infusion of opioids via the PCA, carefully consider whether there is a need to withhold long-acting oral opioids to prevent over-sedation. If demand dosing only is ordered via PCA, continue the use of long-acting opioids. At discharge, evaluate inpatient analgesic requirements, wean parenteral opioids before conversion to oral opioids, and adjust the home dose of long- and short-acting prescriptions to prevent opioid withdrawal after discharge.
  2. In adults and children with SCD and a VOC, do not use meperidine unless it is the only effective opioid for an individual patient.
  3. In adults and children with a VOC, administer oral NSAIDs as an adjuvant analgesic in the absence of contraindications.
  4. In adults and children with a VOC who require antihistamines for itching secondary to opioid administration, prescribe agents orally, and do not re-administer with each dose of opioid in the acute VOC management phase. Re-administer every 4 to 6 hours if needed.
  5. Encourage using incentive spirometry while awake, and ambulation and activity as soon as possible. It will reduce the risk of acute chest syndrome in patients with a VOC.
  6. Use adjunctive nonpharmacologic approaches to treat pain like local heat application and distraction.
  7. In euvolemic adults and children with SCD and a VOC who are unable to drink fluids, provide intravenous hydration at no more than maintenance rate to avoid over-hydration.
  8. In adults and children with SCD and a VOC that receive opioids, monitor for excessive sedation by measuring sedation with an objective measurement sedation scale and oxygenation levels.
  9. Gradually titrate down parenteral opioids as VOC resolves.
  10. In adults and children with SCD and a VOC, do not administer a blood transfusion unless there are other indications for transfusion.
  11. In VOC and the patient has an oxygen saturation <95% on room air, administer oxygen.

We base these recommendations on the most recent evidence. Make the Uganda Clinical guidelines your companion.

Do not forget to share with your fellow clinicians.

By IAmDrSsekandi

I am a medical officer interested in maternal and child health. I am a content creator, author and founder of https://ssekandima.com. I do private practice with a public touch. I am a certified digital marketer. I earned certificates in Understanding Clinical Research and Writing in Sciences from the University of Cape Town and Stanford University respectively.

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