Event 3: Meet Tylee as a Young Adult

Tylee's Health History

  • Social
    • Lives with grandparents
    • Attends college for fashion marketing 3 days/week
    • Works part-time in a warehouse 2 days/week
  • Health
    • Treatment facility/hematologist remains the same as when he was a child
  • Hospitalizations
    • Cannot remember last hospital visit
  • Medication
    • Prevention of Sickle Cell Disease (SCD) Symptoms:
      • Penicillin and folic acid
    • Treatment of Sickle Cell Disease (SCD) Pain:
      • Ibuprofen
      • Then acetaminophen with codeine if needed
      • IV morphine if needed (only in hospital)
  • Self-Care Management
    • Warm baths, showers, eats well, and gets plenty of rest

How Tylee Deals with Sickle Cell Disease Pain as a Young Adult


  • Tylee must speak up and request the care he believes is necessary for his pain
  • In order to receive proper pain management, Tylee must educate the interdisciplinary team about his sickle cell disease

20-Year-Old Tylee Advocates for His Health


  • Opioid analgesics titrated to pain relief with observation for side effecs
  • NSAID: oral ibuprofen, intravenous ketorolac
  • Stay hydrated
  • Avoid infection (immunizations)
  • Heat
  • Massage

How Doctors Decide on a Proper Treatment for Tylee

Impact of Opioids

Opioid Use and Sickle Cell Disease (SCD)

  • Some patients have described sickle cell disease related pain as "the worst pain one can suffer," worse than post-operative and cancer-related pain.
  • This pain is the leading cause of emergency room visits and hospitalizations among sickle cell patients.
  • Long-term therapy with opioids can be safe and effective and in selected patients, the benefits outweigh the risks.
  • Reducing access to prescription opioids for sickle cell disease patients:
    • Increases emergency room visits
    • Stigmatizes Sickle Cell Disease (SCD) patients as engaging in drug-seeking behaviors
    • Denies patients an effective way to manage their severe pain crisis


Opioid crisis adds to pain of sickle cell patients. Available at: https://www.nhlbi.nih.gov/news/2017/opioid-crisis-adds-pain-sickle-cell-patients Accessed May 15, 2019

Risks of Opioid Use

  • Risks of opioid use and potential for abuse with sickle cell disease patients are relatively low
  • Fear those diagnosed with Sickle Cell Disease (SCD) are more likely to become addicted to opioids, which means patients with SCD may be stigmatized as "drug-seeking."
    • 63% of nurses believed patients with SCD were opioid abusers
    • Clinicians assume opioid addiction is more likely in patients with SCD than other chronic pain syndromes
  • Sickle Cell opioid deaths remain relatively consistent while those in the general population have increased, nearly quadrupling.


Ruta NS Ballas SK., 2016


  • Severe Sickle Cell Disease (SCD) Pain/Vaso-Occlusive Crisis (VOC) Pain
  • Low back pain and headache
  • Leg pain (bone pain)
  • Chronic joint pain

New Strategies for Managing Vaso-Occlusive (VOC) Pain


  • NMDA receptor antagonist with strong analgesic properties
  • NMDA receptor effects may modulate central sensitization and hyperalgesia that are associate with recurrent Vaso-Occlusive (VOC) and therefore reduce opioid requirements
  • Low dose infusion associated with lower pain scores and reduced opioid usage
  • Trial of intranasal ketamine is underway
  • Oral ketamine may also be effective
  • Only given in the hospital (IV administered)

Hagedorn JM Monico EC., 2019; Manwani D Frenette PS., 2013; Young JR., et al., 2017; Jennings CA, Bobb Bt, Noreika DM Coyne PJ., 2013


  • Dexmedetomidine: a specific α2-adrenoreceptor agonist with analgesic and mild sedative properties without respiratory depression
  • Dexmedetomidine infusion associated with reduced pain scores, decreased opioid requirement in patients with pain unresponsive to escalating opioid dosage and ketamine
  • Only given in the hospital (IV administered)

Hagedorn JM Monico EC., 2019; Manwani D Frenette PS., 2013; Young JR., et al., 2017; Jennings CA, Bobb Bt, Noreika DM Coyne PJ., 2013

Vaso-Occlusive Crisis Prevention Methods

Vaso-occlusion in sickle cell disease
Vaso-occlusion in sickle cell disease


  • Monoclonal antibody that binds to P-selectin, blocking interaction with PSGL-1, decreasing leukocyte adhesion to vascular endothelium
  • Administered monthly by infusion
  • RCT 198 patients 16-65 yrs for 52 weeks
  • 1.63 vs. 2.98 VOC per year
  • No use in childhood yet


Atage KI, Kutlar A Kanter J., 2017


Reduces oxidative stress of sickled RBC

Decreases endothelial adhesion

RCT 230 patients 5-58 yrs in 2:1 ratio

25% reduction in crises over 48 weeks

Decreased ED visits and hospitalizations

FDA approved


Niihara Y, e al., 2018

Test Your Knowledge

Tylee is your patient. He is complaining of sickle cell pain in his legs and knees. Which non-pharmacologic approach would you recommend in combination with an appropriate analgesic based on his pain score?


Tylee's Discharge Plan

A follow-up appointment is arranged with the hematologist in 3-5 days unless symptoms worsen.

Next: Tylee's Follow Up Treatment and Outcomes

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