26 Aug One in a Million –
My Ongoing Battle with Pemphigus Vulgaris
On February 14, 2022, my husband Andy tested positive for COVID. I was pissed because I was scheduled to travel to Florida and visit my friend in 2 weeks’ time and now, selfishly, I had to worry whether or not I would be able to go. Responsibly, I took a rapid test, and thankfully, it was negative. Little did I know getting COVID would have been a cake walk compared to what subsequently occurred.
It started as a rash. First, I noticed that I was itchy on my left forearm. That’s strange, I thought. What’s up with that? The itch spread to the other arm and became rather uncomfortable. I started to pop Benadryl pills and still thought nothing of it. When I left for Florida two weeks later, the rash had moved from my arms to my thighs and became bumpy, uncomfortable, and incredibly itchy but manageable. I resolved that I would simply enjoy my time in Florida despite having this bizarre unexplained rash. That said, given the fact that it wasn’t going away, I decided to do the responsible thing and schedule a doctor’s appointment with my GP. Therefore, when I returned to Montreal, I met with my doctor on March 9, 2022. The doctor didn’t seem too concerned; however, she was unable to render any diagnosis. She wrote a prescription to help manage the itch and wrote out a referral suggesting I further investigate this with a dermatologist.
In the meantime, the rash continued to move about my body, never relenting. Then one day it just stopped. However, with my body no longer itchy, I realized I had neglected to pay attention to the fact that my mouth was bothering me. I noticed that it felt as though I had cut the inside of my cheek. My gums felt extremely sensitive. Yet, I went about my day, my life, my schedule as if nothing were different.
On April 30th I attended a weekend training for a travel company that I work for. It was then that I started to notice it was becoming increasingly more and more uncomfortable to eat. Upon getting into bed on the first evening, I remember thinking that perhaps I had a tooth infection and that I might need an implant. Seeing that I had just spent the past year dealing with a tooth extraction and implant, it seemed to be the logical conclusion. My ability to consume any food became increasingly difficult. Therefore, as soon as I returned from the training weekend, I contacted my dentist for an emergency appointment.
On May 3rdmy dentist managed to fit me into his schedule. I sat in the chair, he took one look at my mouth and said, “Hold on, I’ll be right back.” When he returned he said that he thought that I had Lichen planus, a condition that can cause swelling and irritation in the skin, hair, nails and mucous membranes. His suggestion was that I apply an oral cortisone gel and if the condition did not improve in 10 days, he advised me to see an oral surgeon who could do a biopsy (a biopsy??? Now this is getting serious!) to determine what type of virus it was, and what type of treatment I would require. While I wasn’t happy to hear this, I was not terribly concerned either.
Being a good patient, I did as my dentist suggested, however 10 days later I was not feeling better. In fact, it occurred to me that I was developing unexplained blisters on my torso – both front and back. These blisters also appeared under my arm, and one on my forehead. As the days progressed others appeared on my back. Then, as if that wasn’t enough, I noticed some bumps on my head – one, then two, then three, then more. By the time I spoke to the dentist again, I had managed to make an appointment with a dermatologist.
The dermatologist confirmed that something was amiss, and sent a referral to an oral surgeon who contacted me a week later. As soon as the oral surgeon (Dr. Peter Chauvin – who, unbeknownst to me, would be the first of many incredible specialists I would encounter) looked into my mouth he said, “It appears to be Pemphigus Vulgaris.” I’m like, “Pempha what?” He advised me that he had to do a biopsy to confirm this diagnosis, and that he was going to refer me to the Montreal General Hospital for treatment with the dermatology clinic immediately. I told the doctor that I was scheduled to be working in Europe at the end of June. He said, “No you’re not!” And that’s when this sh-t got real!
*Pemphigus Vulgaris (PV) is a rare long-term condition caused by a problem with the immune system. It causes blisters in the mouth and on the skin. It can be very serious, but treatment can help control it. According to the Mayo Clinic (2022) an estimated 1 to 5 out of every 1 million people receive a pemphigus diagnosis throughout the world each year. I was now one in a million. To my knowledge, there is no known cause, and no known cure.
I was in COMPLETE and TOTAL SHOCK. Me? I’m in great shape, I eat well, I take care of myself. How? Why? What? What did this mean? A part of me knew that my life/lifestyle was about to change, but I really didn’t know exactly how.
On June 6th I met with the most professional, compassionate dermatologist, Dr. Therese El-Helou (Montreal General Hospital), who literally walked me through the process of what this potential diagnosis might mean, what the process would be, and what potential treatment options would be.
And so began the process…Blood tests (12 vials of blood), gastroscopy, every vaccine known to mankind (I had 7 different vaccines to take because this condition makes one susceptible to infections.). I had to protect myself in every way I could. My new normal was a mixture of meds/vitamins, prednisone tablets, calcium, vitamin D and approx. 5 different creams & two rinses to help mitigate the lesions on my body, (head, trunk, mouth, eyes, throat, and other areas that shall remain nameless). This treatment was done to help keep my condition stable while I waited for approval of a well-researched infusion treatment called Rituxan. Rituxan is an infusion treatment given to individuals to target certain parts of one’s immune system and to help fight PV. In order to receive this treatment, I needed two very important things to occur: 1) All tests and vaccines had to be up to date, and 2) I needed my insurance to approve the request, given that it is a costly medication.
On July 20th I received the approval from my insurance and a request was made to the clinic to set up the procedure. Due to a series of miscommunications, I still had to wait 3 weeks to schedule my first 6 hour infusion. The first infusion happened on August 17th, thankfully, with virtually no side effects. My second infusion is scheduled for August 31st. Once I have the medication in my system, I am hoping to be able to be weaned off the steroids. I am determined to remain optimistic that this is what will put me into remission..
What have I learned from all this? Your attitude is everything, and it determines how you experience every aspect of your life (G. Jampolsky). I have never been one to engage in pity parties, but this one somehow felt different. I had to put my whole life on hold – I could not travel, I could not eat many of my favorite foods, in fact, I could not eat anything solid for quite some time (and still currently cannot eat many foods), I could not take care of my own body (my husband had to tend to my open blisters). I could not be in the sun, I could not drink alcohol. But most notably, I could not be more scared! This condition has a life of its own and I could not know what or how I would look as I woke up each day, and still don’t! THIS uncertainty can truly wreak havoc with your thoughts. I had to learn to live in the moment and appreciate what I could do – I could still work with the travel company, (just not in the way I had planned). I could still facilitate groups. I could plan, organize and facilitate Myra’s Kids Foundation weekend Camp 2022. I could create my own version of a healthy smoothie to ensure I was getting the nutrients I needed. I could reach out to a former social work student who has been living with PV for the past 7 years (who turned out to be an incredible source of information, support and guidance). I could join the Pemphigus Vulgaris Facebook group and meet incredible, supportive warriors who offer kindness, care, advice and compassion. I could get support and guidance from the International Pemphigus and Pemphigoid Foundation (IPPFI). I could appreciate the overwhelming outpouring of support from friends and family offering anything from soup delivery, lifts to appointments, a silver ring inscribed with the words “this too shall pass” to the daily, weekly, check ins or ‘I’m thinking of you’ texts. I could attend my nephew’s wedding and my best friend’s daughter’s wedding. I could celebrate my husband’s 60th birthday party (even if it was in our backyard rather than on an Island in Greece as planned). I could laugh (and laugh I still do). I could acknowledge and appreciate the incredible support from health care providers (such as Dr. Michael Starr) who took the time to listen, to explain and to advocate for me.
I could still meet and counsel clients. Ironically, I have never felt closer to my clients than these past few months. As a grief counsellor I am acutely aware of how fragile life is – how one minute your life is one way and then in the blink of an eye so much can change. I felt I could empathize with each and every one of them more than I had ever done before,
(despite having experienced the death of my brother 34 years ago which brought me into this field ). I never have nor would I ever compare my experience to those who are bereaved, but I do know about unwelcome change and the challenges that we all experience. If I have learned anything from this experience, it is that the only way to navigate challenges is to reach out and to lean on the people around you. There is no fixing grief; there is also no fixing autoimmune conditions (with no known cause and no known cure ). And yet, there are many ways to help/support people by accompanying them on their journey.
All to say, I remain eternally grateful to the plethora of people who have played that role in my life. For without them, I have no idea where I would currently be. It is very true that the best thing in life to hold onto is each other! I am constantly reminded of a statement one of my colleagues said about me. “That disease picked the wrong person to mess with!” So yeah, I still have no idea what the future has in store for me, but then again, does anyone ever really?