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  • Writer's pictureTracy Harrison

Manage Your Sickle Cell Pain Care Skills

Updated: Jan 15

From Pedia Pain Focus podcase dated October 29, 2018 with Anjana Kundu MD

In this episode, Dr Kundu discusses sickle cell disease (SCD) pain with William Zempsky MD from Connecticut Children's, a pediatric emergency physician with extensive experience with sickle cell disease. We will discuss the challenges of assessment and treatment of this disease that has a significant impact on quality of life and provide some information that you can apply immediately with your interactions with this vulnerable population.

Dr Kundu: Welcome Dr Zempsky to the podcast. How did you become involve in pediatric pain and specifically in sickle cell disease?

Dr Zempsky: 2010 was the 100th year anniversary recognition of sickle cell disease. However not much advancement has been made regarding the treatment of pain in this disease process; this remains a significant challenge to those individuals with sickle cell and their families.

Dr Kundu: What are some of the common challenges that providers face?

Dr Zempsky: Pain is driven by a vaso-occlusive event that occurs with sludging of blood cells within the vasculature and resultant ischemia when the erythrocytes sickle in the bloodstream. Additionally, a host of inflammatory events occur that escalate the process of ischemia and pain. These crises can last 3 to 9 days and can be extremely painful and disabling. We provide supportive care with fluids and NSAIDs that address the inflammation. In contrast to postoperative pain that improves each day after surgery, pain associated with sickle cell disease may continue to escalate for 4-5 days after the onset, which creates a great challenge to pain management and keeping patients functioning during this pain. We are now seeing an uptick in the development of chronic pain in these individuals aside from the acute vaso-occlusive crises events that alone have a significant impact on quality of life. Unfortunately there might not be much that we can do to affect the duration of the crisis event but rather we are supporting them during this natural process. Often we need to just let the crisis run its course, so to speak.

The challenge is that most clinicians do not realize this transition happens and many do not understand how to differentiate acute pain symptoms from chronic pain symptoms.

Dr Kundu: That is a great point. What can health care providers know to distinguish between acute and chronic pain in SCD? Can you clarify this for those health care providers who treat these individuals?

Dr Zempsky: The pain during the acute process escalates over 24 to 48 hours and has not responded to their typical treatment. Many providers feel 'these patients don't look like they're in pain. Why should I treat with opioids now?' The truth is that early treatment of these acute events have a significant positive impact on the SCD process itself. There has been a real disparity in treatment realizing that opioids may be used appropriately in this setting. Effective pain management in itself might actually change the pathophysiology of the crisis and hasten the resolution.

In chronic pain of SCD, pain becomes more widespread, rather than associated with only one or two locations typical of the more acute events. The classic vaso-occlusive pain often begins in long bones, such as the legs or arms; as one ages and the disease progresses, pain often is focused in the back, abdomen, chest. In research studies, there were two types of pain identified, local and widespread. Those with widespread pain required more opioids, longer hospitalizations and were thought to be on the verge or at risk of developing a chronic pain syndrome. The prudent advice is to go with what the patient describes after all, this is the mantra of the pain physician.

Pain is 'whatever the experiencing person says it is, existing whenever the experiencing person says it does'

Dr Kundu: Exactly. And this is no different than other types of acute or chronic pain scenarios. (The challenge is that when there is little objective evidence of 'injury' such as a broken bone, scrutinizing providers can sometimes feel challenged as to how aggressively to treat the patient's reported pain. Note: Italicized text is my comment.)

Dr Zempsky: We need better definitions for chronic pain in SCD. We published a paper last year to develop a standard evidence-based classification system for chronic SCD pain (AAPT) Check out the article here. This pdf describes the most current recommendations for the management of acute pain crises in those with SCD. These are the first steps in providing guidelines for clinicians to manage the disparity in the treatment in SCD. As experts, we have differing opinions regarding treatment and we need consensus guidelines to better educate those providers on the frontlines who see these individuals.

There are three modifiers for those individuals with chronic pain in SCD:

- pain with no identifiable cause (widespread pain)

- pain with identifiable cause (such as avascular necrosis of the hip, leg ulcers, or objective findings on physical exam or imaging of a pain generator)

- the presence of both discreet physiological entities and widespread pain without identifiable cause

Dr Kundu: How do you differentiate how you will treat those with vaso-occlusive pain vs those with chronic pain?

Dr Zempsky: We know that these individuals have a 30 - 50% chance of developing chronic pain during their lifetime. We have the responsibility to provide access to the non-pharmacologic strategies that can be very effective for pain and maintaining functioning. These include self-hypnosis, meditation, relaxation, distraction and emphasizing the notion that physical activity and mobility is important despite the presence of pain. We have not done a particularly good job in emphasizing these strategies (and also providing pharmacologic strategies when appropriate, weighing the risks and the benefits).

Dr Kundu: Absolutely. I am a big proponent of multimodal pain management from the beginning of all pain syndromes. Can you describe the evidence for utility in SCD?

Dr Zempsky: Yes, all of the above-described modalities have robust evidence in other pain syndromes but there admittedly is a lack of large studies to describe efficacy specifically in SCD. However, there is no reason to believe that what is true for other pain syndromes cannot be true for sickle cell disease pain. There are initiatives to use web-based and mobile devices for individuals to manage their pain regardless of their location. This an exciting area of development, seeking to treat individuals that are further afield of major medical centers.

Dr Kundu: What do you think is the role of supplements in SCD?

Dr Zempsky: There is a real lack of examining the effect of nutrition and supplements in SCD. Certainly vitamin D supplementation is important for many of the body's processes and pain and attention to consumption of fish (or in the form of fish oil supplements) can have a positive impact on inflammation. Furthermore, glutamine may decrease pain in SCD so there is much opportunity for more investigation in how diet can affect pain. (My recommendation would be for all individuals to assure that they do not skip meals, therefore eating breakfast, lunch and dinner at those times and assuring 2 - 3 snacks during the day, eating a variety of foods, and being wary of restrictive diets).

Dr Kundu: Zinc can also have some benefits in pain management and therefore we need to really look at nutrition as just as important as medications for disease and pain. What else might you recommend a provider discuss with their patients with SCD?

Dr Zempsky: The older school of thought was to restrict physical activity in individuals with SCD to minimize the metabolic stress. However, we are now recognizing that all individuals need a certain amount of physical activity, albeit in moderation with attention to the risks and the benefits, with the original disease process in mind. (We need to move away from the notion that we will only be physically active if the pain is eliminated).

Dr Kundu: Regarding physical activity, are there guidelines during the acute pain process or chronic pain scenario?

Dr Zempsky: This is the problem: there are no guidelines. However, we can take some guidance from the acute post-operative setting. We have moved away from prolonged bedrest after surgery and we need to translate this practice to those individuals who are hospitalized or at home suffering from an acute vaso-occlusive crisis or pain. For the first day, you may be allowed to rest but very soon we encourage avoiding prolonged bed rest and being excessively sedentary. We recommend slow and gradual return to moving around more during the day, despite the presence of pain. Some adolescents may enjoy resting and sleeping during the day and being awake at night but particularly in all pain conditions, this can be in complete opposition of a sound healthy pain management plan. Our goal is to get them back to daily living and we need to model that in the hospital, providing these suggestions to parents and individuals with SCD. We set that expectation as part of the treatment plan. It is not punitive but may also have positive effects with regard to mood, self-esteem, sleep, and other psychological effects.

Dr Kundu: You published one of many great articles on sickle cell disease. Could you briefly summarize the steps in assessment and treatment of patients presenting with pain in the setting of SCD?

Dr Zempsky: The Emergency Severity Index is a five-level ED triage algorithm that provides stratification of individuals presenting for emergency care. The highest level of need is identified as 1, those with major trauma and others requiring emergent, urgent treatment. Level 2 is fulfilled by individuals with SCD presenting with acute pain, possibly at risk for suffering a vaso-occlusive process. This is an emergency and should be viewed as such. We need to provide rapid administration of opioids as appropriate within 60 - 90 minutes from presentation, weighing the risks and benefits. By aggressively treating these patients in the emergency department (ED) we can decrease their length of stay, decreasing transition to chronic pain, and improve their quality of life.

This article provides some excellent guidelines towards a more systematic approach. Not unlike guidelines for treating acute myocardial infarction in the ED, guidelines for SCD are needed to level the playing field and provide the best care possible to individuals suffering with SCD.

Individualized care plans that are readily available in the electronic medical record ideally should be developed for each patient at risk for vaso-occlusive crisis. The problem arises when these at-risk patients encounter different ED providers who may have idiosyncratic approaches to SCD thus putting these individuals at risk for under-treatment of pain, prolonging their time to appropriate treatment and their hospital stay. There may be a higher level of opioid tolerance that might be concerning to providers if they do not understand each individual patient's needs.

Dr Kundu: Thank you to Dr Zempsky for his invaluable discussion about the challenges of assessing and treating pain in sickle cell disease, both acute and chronic pain situations. I would summarize with three specific points:

1 - one needs to make an astute observation whether this is acute pain, acute-on-chronic pain, or chronic pain experience

2 - continue to assess functional status and provide recommendations for how to return or maintain daily performance of activities of daily living (ADLs) and physical activity. Do not leave this point out. If you do not verbalize that this is just as important as medications, your patients may falsely assume that being sedentary and resting is permissible and advised.

3 - promote the use of multimodal analgesia, be increasingly receptive to the patient's reports of pain, and have patients involved in decision-making regarding their pain management.

You can listen to the full podcast and see the shownotes here.

Are you an individual with sickle cell disease? Can you comment on some of the strategies your provider recommended to you that were the most helpful? Misunderstanding of the disease process and undertreatment of pain in SCD is widespread but we can change that together.

And as always, if you have a comment of what you would like to see on the PreEmpt Pain website, I would love to hear from you!

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