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Goals and Guidelines of Sickle Cell Treatment


Ifeyinwa Osunkwo, MD: There are people who listen to this and who ask themselves: “I am a general practitioner, I am a [hematologist/oncologist], or I have my own private practice, and I don’t have a case manager, I don’t have a social worker. How does this happen in real life? And how do you prevent certain anecdotes from happening, as Dr. Smith shared?

Nirmish Ramesh Shah, MD: It’s about having open conversations and expectations to try to find out what the patient’s goals are. When I first see them, I try to do a lot of my conversations with patients, not just about pain, and that’s to try to understand the context of what’s going on in that patient’s life. That’s when I start hearing stories like, “I just broke up with this person, I don’t have a home, I’m homeless. I don’t have a job or insurance, that’s why I don’t refill my medicine. I know clinics can be busy, but it’s an essential part of the conversation, so I spend some time understanding where the patient is coming from. Then you dive a bit into pain expectations, and for patients who have chronic pain, that’s a very difficult situation. When we give painkillers, it is only a palliative. Now let’s get into these conversations about these disease-modifying drugs and some approved sickle cell drugs. [cell disease] it can help relieve pain, but from a pain point of view, chronic pain is there every day. Patients have pain more days than not, and it interferes with their lives. I have to try to figure out what success is for them. I know you suffer every day; how can i make you functional? In a lot of our chronic pain patients it’s not going to go down to 0, it’s not going to go away completely, so how can I make you productive in your goals with a medication plan of some kind and a non-pharmacological management plan too.

When I talk about non-pharmacological management, these are strategies that also take a little time but are very valuable because you don’t just put a bandage on it with painkillers; we try to think about how patients are going to deal with this pain on a daily basis. Cognitive-behavioral therapy, mindfulness, breathing, relaxation, distraction; these are things we need to get patients to understand, and you need to get them to understand that this is a valuable strategy that works. They may have to go to the pain specialist to get some of this, you may have to refer them to a psychologist to start some of this, but you have to give them those tools. I appreciate you bringing this up because it’s a conversation where a provider who doesn’t treat sickle cell disease: a primary care provider, a senior oncologist, a bit of hematology; they fear that the pain is all it is about. That’s true to some extent, but if you think about the context, it’s something where you can come up with a plan and get others to try to help this patient through it.

Ifeyinwa Osunkwo, MD: Dr. Desai, before we get to the factors that impact the treatment options we offer our patients, can you tell us about the guidelines that exist for the patients we just described? Acute and chronic pain, sickle cell disease, superimposed on chronic pain as a disease. I know there are a few guidelines that have come out recently. Can you tell where people can find these resources to guide their treatment decision, and also help build trust with patients, that they don’t do this as a punitive measure but as a recommendation because that’s the best practice?

Payal Desai, MD: Let me first address the guidelines. The American Society of Hematology has excellent guidelines on acute and chronic pain management. Now I will say that there are differing opinions on how best to implement them. Also, in general, there is a shift in focus on how we manage chronic pain across the country; how does sickle cell disease [disease] fits into this image of these guidelines is really important. Some of the opioid guidelines explicitly excluded sickle cell disease. We need to think about where this fits, but again, as Dr. Shah mentioned these options, framing the pain management context simply within the opioid framework is a failing on our part. We need to talk about pain management as a holistic approach, and each center does this slightly differently. As Dr. Smith said, when you saw a sickle cell center, you only saw one sickle cell center; I will quote it back, that each center does it slightly differently. Is it the hematologist who writes the prescriptions for the analgesics? Is it the primary care center? How do you coordinate this? What if you have 1 or 2 patients and not an entire center? I think it’s important to have a clear approach to how you’re going to manage chronic pain in your practice, whether it’s how often patients are supposed to be seen or what your reasonable expectations are with regard to the patient. For example, you can’t bring the patient in for a visit every week if they don’t have transportation; you can’t have unreasonable expectations, but you must set expectations.

I don’t think having sickle cell disease or chronic pain means you don’t have expectations of the patient on the other side either. It’s really a tightrope, this balance exercise that works for every patient in terms of chronic pain management, and opioids are usually part of that discussion. But again, you can’t let the conversation stop there, which means you need more time. If you have 2 patients with sickle cell disease they will sometimes take that extra time and you will have to talk about these things if they have a more complicated disease. You’re going to have to take the time to say, “Here are the expectations, here are the limits, here are the things that we can reasonably offer that allow me to do this legally, but let me also take care of you in the best way possible” , so expectations are known on both sides. Again, the cookie-cutter approach of, if you miss early, you’re breaking a pain contract, doesn’t always work. But neither does telling your patients that ‘they can take their long-acting painkillers for 1 week and then take them off for 3 weeks; that doesn’t work either. You have to find some kind of balance between that everyone can live with and that they can manage their long and short acting pain, which varies from day to day. How do you do that? What they’re doing, having these open and honest conversations so they can share what they’re doing, leads to the greater long-term success.

Ifeyinwa Osunkwo, MD: To sum up, what I have heard is that sickle cell pain is unpredictable and irregular in its presentation. You don’t have acute pain every day, you have chronic pain all the time with intermittent flare-ups of acute exacerbation, so you don’t use your short-acting painkillers the same way every day of the month. Both the provider and the patient need to understand that there will be bursts, seizures, and discontinuations of use. Also, what I’ve heard is that it has to be a shared decision between patient and provider. There must be limits that everyone understands from the start, and it must be multimodal, it must not rely solely on opioids. You have to think about adjuvant therapy, you have to think about non-pharmacological therapies, and it may need to be tailored to each patient. It always takes more time; we need to establish that time is the biggest currency a provider struggles with when it comes to caring for patients with sickle cell disease. You should know from the start that this will not be a 15 minute visit; it’s going to take half an hour, maybe an hour, maybe several half-hour visits, to come up with a plan that works for every patient.

Transcript edited for clarity.