Before Rodney and his wife Debbie arrived at an emergency room in April 2021, they had never heard of ANCA-associated vasculitis (AAV). Rodney's organs were failing and his blood oxygen level was dangerously low due to hemolytic anemia. After several transfusions, dialysis rounds and doses of steroids, his condition stabilized. The cause, however, remained a mystery and specialists from cardiology, nephrology, oncology and rheumatology reviewed his case looking for answers.
In hindsight, the variety of symptoms Rodney experienced in the months prior to his hospitalization suggested vasculitis, but the puzzle pieces didn't come together until the situation became life-threatening and a rheumatologist diagnosed him with severe active granulomatosis with polyangiitis (GPA), a type of ANCA-associated vasculitis (AAV). This rare autoimmune disease is characterized by inflammation of the blood vessel walls that mainly affects small-to-medium blood vessels. While Rodney and his wife Debbie were relieved to have a diagnosis, it was the beginning of a much longer journey. To gain a deeper understanding of their journey with AAV, Amgen recently sat down with Rodney and Debbie to hear their story and what advice they have for others.
How do you reflect on your journey to diagnosis, knowing what you know now?
Rodney: In a way we got the luck of the draw. Because I showed up to the hospital presenting in a life-threatening state, my diagnosis process was considered expedited compared to others. Now I know that the mobility issues, anemia and other symptoms I was previously experiencing were all related, but nothing was adding up at the time because AAV can impact essentially every part of your body. And because so many systems are impacted, it wasn't even clear what specialists I should have seen. The doctors I saw never mentioned vasculitis and I didn't see a rheumatologist until I was at the hospital, which I learned would've been the best place to start.
Debbie: Speaking to other members of our local support group, it can sometimes take up to a year and a half for most people with a form of vasculitis to be diagnosed. In a way, we're grateful our answer came sooner, but wish we had been more aware of vasculitis or even heard of it before Rodney's condition escalated to the stage that it did.
What advice do you have for someone who's been newly diagnosed with AAV? What about someone who thinks they may have a form of AAV?
Rodney: Expect this to be a long journey, read as much as you can and make sure you have a strong support system. You can't do it alone. Vasculitis impacts the blood vessels, which means it can affect every inch of your body, so you're going to need a team of specialists to manage your treatments. When choosing your care team, make sure to ask if they have experience treating vasculitis, not just if they've heard of it. Once you find specialists that work for you, make sure they're willing to collaborate with the rest of your care team, so if one makes a change in your care plan, it's shared with everyone.
Debbie: The Vasculitis Foundation has been a helpful source for information and finding support groups. They can help direct you to specialists in your area and explain the different tests you may need. They created a set of guidelines for the management of ANCA-associated vasculitis that we've found immensely helpful.
Debbie, what is your role as a caregiver and loved one of someone living with ANCA-associated vasculitis?
Debbie: Beyond being a source of emotional support, I act as a problem solver and doer. The level of responsibility a vasculitis patient takes on is overwhelming. There are frequent appointments with many specialists that can be challenging to keep track of. I don't see how anyone could manage it on their own; it really takes two people. One of the most important decisions we made was for me to always be present during Rodney's appointments. There is a massive amount of information and questions, and living with a rare disease requires you to be a strong self-advocate, which can be difficult – but not impossible – to take on when you're actively managing your disease. So, it's helpful to have someone on your team there to be your ears, take notes and speak up, whether that's a family member, friend or caretaker.
What do you wish others understood about the condition and its impact?
Rodney: I wish others knew how debilitating this disease is. Even if I look healthy on the outside, it's still incredibly difficult to manage each day. This was one of the most challenging aspects when I was diagnosed. Doctors and nurses would ask if I was in pain, to which my response would be no. This isn't the case with every form of vasculitis, so it was challenging to vocalize the true impact my symptoms were having on me until I was in an emergency situation.
Debbie: Even years later I'm still working on my "elevator pitch" for vasculitis. Often when I try to explain it to friends, you can tell it's not all clicking despite how much they care and want to understand, because it's a rare and complicated disease. But it's still important to educate others, partially because you never know who the information may help. I created a Facebook group for our friends and family to help educate them and it's also a great source of moral support for us.
What brings you joy during challenging times?
Rodney: My joy comes from Debbie. We've been together more than 30 years and our joy still comes from spending time with each other. This past winter we tapped maple trees and tried making maple syrup, which we've done for many years but haven't been able to since my diagnosis. It may not have been the best, but we accomplished something together and had fun doing it.
Debbie: It's important that we both keep doing the things we love. For me it's a trip to the bookstore and for Rodney it's fishing. We know from experience that you can get really sick, really fast, so it's important to cherish even the small moments when things are good. After all, a glass of wine tastes much better when it's shared.
To learn more about ANCA-associated vasculitis and find support, please visit the Vasculitis Foundation.