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Sickle Cell Disease Pain Management
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Kathleen Broglio discusses Sickle Cell Disease Pain Management at ONS Congress 2017. For the ability to view on your mobile phone please visit us at

This transcript is software driven, please understand there may be errors.  Should any inaccuracies or omissions be found, please notify for correction.

Hi. I'm Kathleen Broglio. I'm a nurse practitioner in palliative care at Dartmouth Hitchcock Medical Center in Lebanon, New Hampshire. I'm going to talk to you today about sickle cell disease and pain management.

Let's talk about Jane. She's a 26-year-old woman with sickle cell disease. She's been in the emergency department seven times in the last year, and during that time she had six admissions secondary to vaso-occlusive crisis. In addition, she has chronic pain secondary to avascular necrosis and is on chronic opioid therapy. The day clinic where she previously received her care closed to lack of funding. This is a typical person that we'll see with sickle cell disease.

When we think about sickle cell disease, the hallmark of sickle cell disease is vaso-occlusive crisis. Some individuals have acute exacerbations of disease, which is characterized by bone pain and fatigue several times a year, but many of these patients also experience chronic pain daily.

The long-term sequelae of sickle cell disease not only is chronic anemia, but are complications, including cardiac, pulmonary, hepatobiliary, splenic, renal, musculoskeletal, ophthalmic, and endocrine changes. We have to remember that people with sickle cell disease have a life expectancy of only about 48 years, although it's improved a lot over the last few decades.

The most common cause of emergency department and hospital visits for people with sickle cell disease is the acute vaso-occlusive crisis. What happens is when the red blood cell sickles, this leads to hypoxia, ischemia, and tissue damage, and the person starts to experience excruciating pain usually in the extremities and the joints, and it worsens with each occurrence. What happens is that the person is then set up to have chronic pain through a process called central sensitization where they have hyperalgesia or pain beyond the area of original pain and may have sensitivity even to touch.

The consequences of this acute painful crisis can be acute chest syndrome, multi-organ failure, and sudden death. For up to 50% of these people that are admitted with a vaso-occlusive crisis, they are readmitted within one month, usually due to acute pain or pain that's not controlled once they go home.

People with sickle cell disease also develop chronic pain syndromes from avascular necrosis, usually of the humerus and femur, from disc disease, from chronic osteomyelitis, from chronic wounds, and again, because they develop central sensitization from recurrent vaso-occlusive crisis’s. Up to 80% of patients will use home analgesics part of the time.

We also think that people with sickle cell disease develop neuropathic pain syndromes because of tissue damage and end up with multiple neuropathies, but this is not well understood and has been studied only in the sickle mouse. It could be from the tissue damage of vaso-occlusive crisis; however, more research is needed in this area.

When we think about acute pain management, we need to intervene during the prodromal stage. This is when the person starts feeling some paresthesia, some numbness in the extremities, and this is before the pain really escalates. Hydration is the number one thing we do to try to hydrate that person, so that the sickle cells do not attach to the endothelium, causing more pain. Multi-modal analgesia, which is your non-steroidal anti-inflammatories and your opioids, are a mainstay of treatment.

If a patient's admitted and the pain is severe, you might want to consider using patient-controlled analgesia versus intermittent boluses with opioids. Obviously, the most important thing is on preventive measures. Diet, sleep, hydration, and hydroxyurea use can prevent these vaso-occlusive crises.

When we think about people that come to the emergency room, if vaso-occlusive crisis is suspected, the pain management should not be delayed while waiting for laboratory values. Many times patients wait for hours to get their first dose of analgesics, and really clinicians need to respect this patient's report of pain and manage it regardless of whether you have the laboratory results. In one interesting study, the use of intranasal fentanyl was used if there was no IV access, and it provided good results.

We need to think about what we need to do if the pain becomes chronic. Is this a nociceptive pain versus a neuropathic versus an inflammatory pain, and we always need to consider multi-modal therapy. Many of these patients may have been using opioid therapy since very young in life and may be centrally sensitized and may have high tolerance to opioids, and that's why a multi-modal approach is always important.

We need to think about when we look at multi-modals, acetaminophen nonsteroidal anti-inflammatories, anticonvulsants like your gabapentinoids ... thinking about some NMDA receptor antagonist, such as ketamine. All of these are used to try to better manage the pain through treating the pain from multiple pathways to decrease the use of opioids.

If we are treating chronic pain, we need to think about using universal precautions if we're going to put patients on chronic opioid therapy. These universal precautions were originally developed for use in chronic pain management, and are effective strategy because all patients are treated as if they are at risk for addiction ... because this is important, because clinicians may not be able to identify an at-risk individual. It also reduces the stigma associated with labeling an individual as at-risk.

When we use universal precautions, we need to look at doing a comprehensive pain assessment. What type of pain does this person have? Establishing a differential diagnosis for the pain. Conducting an opioid risk assessment and using informed consent and a treatment agreement and a plan about what are the goals for opioid therapy as well as the ongoing reassessment of the analgesic effect. We may want to be using urine drug screening; this is part of universal precautions. We want to make sure that that patient is taking the medications as prescribed and avoiding illicit use of medications.

When we think about managing pain, we have to think about aggressively managing acute pain in a timely manner, trying to decrease the recurrence of vaso-occlusive crises because this can lead to central sensitization and chronic pain. We want to manage pain using a comprehensive approach with multi-modal analgesia.

Thanks so much for attending this presentation.