Treatment Goals and Guidelines in Sickle Cell Disease


Focusing on disease-modifying therapeutics, expert panelists discuss foundational treatment goals and guidelines for sickle cell disease.


Ifeyinwa Osunkwo, MD: There are people who are listening to this and wondering, “I am a primary care doctor, I am a [hematologist/oncologist], or I have my own private practice, and I don’t have a care manager, I don’t have a social worker. How does this get done in real life?” And how do we avoid some of the anecdotes from happening, like Dr Smith shared?

Nirmish Ramesh Shah, MD: It’s all about having open conversations and expectations to try to find out what the goals of that patient are. When I first see them, I do try to make a lot of my conversations with patients not just about pain, and that’s to try to get context of what is going on in that patient’s life. That’s when I start to hear the stories, like, “I just broke up with this person, I don’t have a house anymore, I am homeless. I don’t have a job and insurance, so that’s why I am not filling my medication.” I know clinics can be busy, but that’s a critical part of the conversation, so I spend a bit of time to understand where the patient is coming from. Then you dive a little into the expectation about pain, and for those patients who have chronic pain, it’s a very difficult situation. When we give pain medications, it’s just palliative. Now, we will get into these conversations about these disease-modifying drugs and some medications that are approved for sickle [cell disease] that can help with pain, but from the standpoint of pain, chronic pain is there every single day. Patients are having pain on more days than not, and it’s interfering with their life. I need to try to figure out what is success for them. I know you have pain every day; how do I get you to be functional? In many of our patients with chronic pain, it’s not going to go to 0, it’s not going to go away completely, therefore how do I make you be productive in what your goals are with a medication plan of some sort, and a nonpharmacologic management plan as well.

When I say nonpharmacologic management, these are the strategies that also take a little time but are very valuable because we are not just putting a Band-Aid on it with some pain medications; we are trying to think how the patients are going to cope with this pain on a daily basis. Cognitive behavior therapy, mindfulness, breathing, relaxation, distraction; these are things that we need to get patients to understand, and you need to give that insight to the patient that that is a valuable strategy that works. It may be that they have to go see the pain specialist to get some of it, it may be that you need to refer them to a psychologist to get some of that started, but you need to get them those tools. I do appreciate you bringing this up because this is a conversation where a provider who doesn’t treat sickle cell: a primary care provider, a primary oncologist, a little bit of hematology; they are concerned that the pain is all it’s about. That is true to certain extent, but if you think about the context, it is something where you can come up with plan and get others to try to help that patient through that.

Ifeyinwa Osunkwo, MD: Dr Desai, before we go to factors that impact what treatment options we offer our patient, can you speak to what guidelines are out there for patients we just described? Acute and chronic pain, sickle cell disease, superimposed on chronic pain as a disease. I know that there are a couple of guidelines that have been published recently. Can you speak to where people can find these resources to guide their treatment decision, and also help build trust with the patients, that they are doing this not as a punitive measure but as a recommendation because this is the best practice?

Payal Desai, MD: First let me address the guidelines. The American Society of Hematology has some great acute and chronic pain management guidelines. Now, I will say there are differing opinions on how to best implement those. Also, in general there is a shift in focus on how we manage chronic pain across the country; how sickle cell [disease] fits into that picture of those guidelines is really important. Some of the opioid guidelines explicitly excluded sickle cell disease. We need to think about where that fits in, but again, as Dr Shah mentioned those options, framing the context of pain management simply in the setting of opioids is a failure on our part. We must talk about pain management as a comprehensive approach, and every single center does that slightly differently. As Dr Smith said, when you have seen one sickle cell center, you’ve only seen one sickle cell center; I will quote it back, that every single center does it slightly differently. Is it the hematologist who writes prescriptions for the pain medication? Is it the primary care center? How do you coordinate that? What if you have the 1 or 2 patients and not an entire center? I think having a clear approach of how you are going to manage chronic pain in your practice is important, whether that’s how frequently the patients are supposed to be seen, or what your expectations of the patient that are reasonable. For example, you can’t have the patient come in every week for a visit if they have no transportation; you can’t have unreasonable expectations, but you need to set expectations.

I don’t think that having sickle cell or chronic pain means you don’t have expectations of the patient on the other side either. It’s a very much that tight rope, that balancing act that fits for every patient in terms of managing chronic pain, and opioids are usually part of that discussion. But again, you can’t let the conversation be only that, which means that you require extra time. If you have 2 patients with sickle cell disease, sometimes they are going to take that extra time, and you’re going to have to talk about these things if they have more complicated disease. You’re going to have to take the time to say, “Here are the expectations, here are the boundaries, here are the things that we can come up reasonably that let me do this legally, but also let me take care of you in the best way possible,” therefore the expectations are known on both sides. Again, the cookie-cutter approach of, if you run out early, you violated a pain contract, doesn’t always work. But neither does telling your patients that they can take their long-acting pain medications all in 1 week, and then they are withdrawing for 3 weeks; that doesn’t work either. You have to find some sort of balance in between that everybody 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 are doing, having those open, honest conversations so they can share what they are doing, leads to the most success long term.

Ifeyinwa Osunkwo, MD: To summarize, what I heard is that sickle cell pain is unpredictable and irregular in how it presents. You are not in acute pain every day, you have chronic pain all the time with intermittent bursts of acute exacerbation, so you don’t use your short-acting pain medicines the same way every day of the month. The understanding of the provider and the patient should be that there are going to be bursts, fits, and stops of use. Also what I heard is that it needs to be a shared decision between the patient and the provider. There need to be some boundaries that everybody understands up front, and it has to be multimodal, it shouldn’t rely solely on opioids. You need to think about adjuvant treatment, you need to think about nonpharmacological therapies, and it may have to be customized for each patient. It always requires more time; we need to establish that time is the biggest currency a provider struggles with when it comes of taking care of patients with sickle cell disease. You have to know up front that this is not going to be a 15-minute visit; this is going to be half an hour, maybe an hour, may be multiple half an hour visits, to come up with a plan that works for the individual patient.

Transcript edited for clarity.

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