Quality of Life of Patients with GPP

Aaron Farberg, MD, and Mark G. Lebwohl, MD, review the biggest unmet needs of patients with generalized pustular psoriasis (GPP) and the impact GPP has on their quality of life.

Ryan Haumschild, PharmD, MS, MBA: Now that we’ve talked a bit about the background of generalized pustular psoriasis [GPP], let’s talk a little more about the implications of managing GPP from a patient’s perspective. How do we explore the unmet needs and future opportunities for treatments of this disease? Dr Farberg, we’ll start with you. What do you think are the biggest areas of unmet needs right now for patients with GPP?

Aaron Farberg, MD: I think the largest unmet need is simply awareness, both from the public and from clinicians. We realize now that we have treatments available for this disease. If we can identify it early and correctly, at the earliest moment possible, we know we can have a significant impact on these patients. Again, this is a dangerous and deadly disease. Often during those moments of panic and urgency, we don’t always follow the protocol, and perhaps we don’t get the dermatologist at the bedside as soon as possible because rightfully so, we’re worried about other organ involvement, as we discussed previously. That’s again where it’s important to get all the minds at the bedside so that all the doctors can get together and figure out that diagnostic odyssey as Dr Lopes discussed earlier. We [need to] get that early diagnosis because it really isn’t a difficult diagnosis for dermatologists. I don’t expect other clinicians such as emergency department staff or family practice staff to be able to know GPP off the back of their hand, but getting a dermatologist to the bedside and getting more awareness to other clinicians, colleagues, and the public, I think is the largest unmet need.

The second unmet need is medication. We now finally have spesolimab, which does have a significant impact on these patients. If you asked me about the biggest unmet need a few months ago before the medication was approved, I would say an approved medication because there were none until recently that actually treated these patients. Now we’re still trying to gain the clinical experience to really fine-tune and understand the best way to utilize this medication for our patients with GPP.

Ryan Haumschild, PharmD, MS, MBA: Dr Farberg, [I have a] question for you. If we had a patient who was experiencing GPP and they had a GPP flare, what would that feel like, and what do they describe when you’re meeting with them for the first time in experiencing that?

Aaron Farberg, MD: I know Dr Lebwohl was involved in studies at [Icahn School of Medicine at] Mount Sinai, and I know pain was definitely one of the objective measurements for the studies. You realize that these patients are sick, they’re miserable, they’re in significant pain, and they’re in trouble. It’s important to get them treatment as soon as possible. These patients overall do not look well, and that’s one of the more important differential signs and symptoms that you can see in these types of patients.

Ryan Haumschild, PharmD, MS, MBA: I appreciate you describing the flare and the pain that patients feel. As you said, it’s life-threatening. I think that’s one of the biggest takeaways so far in this discussion. Dr Lebwohl, because you’re so experienced with these flares, what do you think the impact is on the quality of life for patients suffering from GPP? What are some of the important limitations that you’ve seen from these patients, and how does that affect absenteeism, their quality of life, and being productive members of the workforce?

Mark G. Lebwohl, MD: This is one of those things where my children would say… they’re covered in pus, they’re febrile tachycardic, and have swollen legs. They can’t walk. They’re shivering because they’re losing temperature. You’re worried they’re going to die, and it affects their quality of life? “Duh,” as they would say. It has a huge impact on quality of life. No, they cannot go to work. They can’t do anything. It is an enormously devastating disease for patients who have it severely. Some patients have a chronic, low-grade presence of pustules on limited parts of their body surface area, even sometimes areas that become confluent, and they’re very uncomfortable. It also has a majorly negative impact on their quality of life. Years ago, when we didn’t have many drugs, we would use methotrexate. That’s even before retinoids. We would control the disease a little so that they could function and go to work, for those patients who did not have the life-threatening form of GPP. The ones with the life-threatening form were always in the hospital, often for many weeks.

We do fortunately have better drugs today, and it’s no longer that kind of problem, but you would never question the need for it. I’ll tell you a story that happened to 2 patients in the last few weeks. Fortunately, they found superb doctors. One was Boni Elewski, [MD,] who’s the chair of dermatology at the University of Alabama at Birmingham, who called me. I gave her the phone number of someone to get her the drug that day. She showed me pictures from one day to the next. You have a patient who looks like they’re going to die, and then the next day looks almost normal. The second patient was described to me. Again, the patient was lucky, they found Erin E. Boh, [MD, PhD, FAAD,] who’s the chair of dermatology at Tulane [University School of Medicine]. She called and told me about the patient, and the patient sounded like they were going to die. The patient had all kinds of comorbidities, diabetes, and obesity, and was in completely frail health, and now had generalized pustular psoriasis. I said this patient’s going to die, and you have to get the patient spesolimab. She had to fight the institutions, all the insurers. She had to fight to be able to get it, but she did it. In fact, she called me late at night. I think she got it that night. She had to go all the way to the top of the chain to get it, but she got it approved, and the patient was better the next day.

Ryan Haumschild, PharmD, MS, MBA: Wow. Those are impactful stories because like you said, it’s a debilitating and rare disease, where if patients don’t get the right treatment, they are not showing up to work and are not productive. So when you think about employer groups and insurers, they’re considering that total cost of care.

Mark G. Lebwohl, MD: They’re dying.

Ryan Haumschild, PharmD, MS, MBA: Exactly, and I feel like that’s significant. Creating awareness, and now having this new IL-36 [interleukin-36] pathway, and providing that drastic improvement, not only in survival, but also in the quality of life for that patient, is really important.

Transcript edited for clarity.

Related Videos
View All
Related Content
© 2023 MJH Life Sciences
AJMC®
All rights reserved.