I’m almost three months into my pemphigus diagnosis. I’ve completed my initial treatment plan. And now, I’m just sitting and waiting the six to eight weeks to see how (if!) it works for me. The treatments destroyed a significant part of my immune system, so I’m in a bit of a medical isolation. I can’t see a lot of people or go most places. I have lots of alone time to sit and think.

This time has allowed the awful reality of having a rare, incurable, life-threatening disease to fully set in to my psyche. I’m currently in a very dark place. I’ve had a full week of tearful mornings now. I wake up each day wishing there was some way out. I don’t want this life. I don’t want to exist trapped in this broken, traitorous body.

Physically, I feel better than I have in a year and a half (thanks, prednisone!) – but mentally, I am absolutely drowning in grief. I feel worse emotionally than I did during any point during my medical mystery. I thought getting through the ‘big unknown’ would be the hardest part and that once someone definitively identified my illness, I’d feel more in control because I’d know what I was dealing with and be able to make plans and decisions. Unfortunately, it hasn’t worked out that way.

I’ve been trying to analyze why I’m having these intensely grim feelings and try to figure a way past them. I would love to reclaim some of the hope and positivity I felt after I was first diagnosed. Here is what I’ve come up with…

Reality is Fluid

When I first got my pemphigus diagnosis, my doctor said something along the lines of, ‘I have good news and bad news. You have a serious, rare autoimmune disease – and that’s bad – we don’t want that for you. But the good news is there is an FDA approved treatment that’s been very effective and I’ve seen fantastic results with my patients.’

My doctor is experienced, warm, empathetic, and persistent. She is everything you want in a doctor, especially when you get a scary diagnosis. She makes me feel as safe and hopeful as possible – but she also can’t change reality.

When she and I first discussed my upcoming rituximab treatments, she shared that the clinical study data showed that 89% of patients reached complete remission after an initial two rounds of treatment. That was great news and I was immediately filled with hope. I could see remission and a normal life on my horizon for 2025.

Then, a few weeks later, I attended the annual IPPF patient education conference and learned that, yes – most patients reach remission, but it doesn’t last. The reality is that 50-75% of patients relapse within 18 months of completing their rituximab infusions.

I also learned that the longer it takes a patient to be diagnosed and treated, the harder it becomes for them to reach remission. The average pemphigus patient takes ten months from symptom onset to diagnosis. It took me sixteen months to get my diagnosis, and then two more months to secure an appointment for my infusions. That means my disease was allowed to fester and grow stronger for a full year and a half.

All of these realities diminish my ability to feel hopeful. But, the harshest blow to my positivity has been hearing from other patients.

Shared Trauma is Overwhelming

Now that I’m an active member of the pemphigus community, I’ve heard directly from many other patients at conferences/webinars, through online communication, and in support groups.

Very few of the patients that are sharing their experiences are successfully reaching remission. They’re doing many rounds of rituximab, and living their lives in immune suppression limbo. They’re adding IVIg treatments and secondary therapies like azathioprine and mycophenolate. Some of them are also experiencing spread of their symptoms in brutal, disfiguring ways. They’re dealing with the terrible side effects of our short-list of treatment options.

Earlier this year, the IPPF published a report called Voice of the Patient Report: Understanding the Unmet Needs of the Pemphigus and Pemphigoid Community. It was full of despairing patient quotes, traumatizing photos, and data that I found incredibly overwhelming and grim. I was shown the report just this week, and it has fed into my downward spiral.

I wish there were more people sharing their great outcomes, but these people either don’t exist or they don’t share their success stories. In the interest of disclosure, there were a few people who presented at the annual conference that are in long-term remission, but they presented other topics rather than the story of how they reached remission. People struggling with the disease have seemed far more common than the success stories.

I am starting to have mixed feelings about whether or not these educational and support offerings are helpful for me. I’m an anxious person with PTSD, so my takeaway experience might differ from someone else.

I’m Fatigued and Burned Out From my Journey

I’ve been sick for nineteen months now. It’s taken a toll on me physically and mentally. I’m tired of waiting for results. I fear and despise the vast abyss of the unknown that still sprawls before me.

I fought for sixteen months to get to my diagnosis – doctor after doctor, test after test, misdiagnosis after misdiagnosis. It was a harrowing trail of challenges, failures, and suffering.

Finding my specialist at UNC and getting an accurate diagnosis felt like reaching a summit. After all that climbing, it seemed like I found a high vista where I could sort of see everything behind me and ahead of me.

The diagnosis felt like a relief, and I guess I expected an easier downhill walk. But, I’m finding the descent to be just as grueling and littered with threats. I told my therapist the other day that this health crisis is a good analogy for successfully climbing Mount Everest, only to find that you’re way more likely to die on the way down.

Descenders die from fatigue. They die because their bodies are too broken down. They die because their exhausted brains make mistakes.

I think being stuck home, alone and immunocompromised, has fueled my tendency to ruminate. The fact that it’s all happening in the dark and cold of winter doesn’t help either. Having to miss the social festivities and gatherings of the holidays is just an additional sorrow. I feel disassociated from the human experience.

If I’d had my infusions in the summer or spring, I’d still be meeting friends to hike, meeting up for a drink on a bar’s outdoor patio, or inviting people over for dinner on the deck. Winter pushes everyone indoors to socialize, and I just can’t risk that right now. It’s very lonely to have a chronic disease, and adding physical aloneness on top of that is tough.

What Can I Do to Make Things Better?

1. I’m going to experiment with limiting how involved I am in support groups and education. I already know my illness is serious – potentially fatal. I have a good doctor I can turn to if things go badly or if I have questions. I already know many patients are struggling and not getting better. I probably don’t need to hear it repeated over and over. I think my mental well-being might improve if I can push the darker aspects of my reality to the perimeter, and focus on things in my life that are not related to pemphigus. This will be a challenge because my personality is built to seek information and connection with peers, but I’m going to try.
   
2. I’m going to work on implementing more positive affirmations and self-talk. I’ve read a lot about neuroplasticity and how we can train our brains to believe things. I’m going to try telling myself that the positive things are equally likely to be true (even if the scientific data doesn’t support that.) I did a Yin yoga practice the other day that included two positive affirmations – ‘All is well in my world and I am safe’ and ‘I trust that my life is unfolding exactly as it should.’ I found these affirmations more triggering than helpful, so I definitely have some work to do. I think I need to choose smaller affirmations to start with… baby steps!
   
3. I’m going to seek out hobbies that fill the long, lonely winter days. I already read, work out, cook, and watch a lot of shows. I feel like I need to find something that is a more fulfilling creative outlet. I enjoy writing, but I don’t feel the same connection with art or music. I’ll continue to experiment and see if I can find something that lights me up. I think a distraction would be good.