Why I'm Taking a Medication That Could Cause Life-Threatening Issues
A week ago, I just took my first dose of a medication called hydroxychloroquine, also known as Plaquenil. It is commonly used to treat autoimmune diseases and has been used to treat others with the same rare condition I have that causes me to be allergic to ultraviolet light, solar urticaria. This is the first day on a journey to hopefully better control this condition. It brings me hope — hope of a better quality of life with the experiences that I have missed out on since I was 12. I haven’t been able to have the blinds up and my window open, and things like camping, road trips, nature, outdoor pools, geocaching, and even freely experiencing fresh air have been a challenge. I’d love to even be able to visit a beach. I’ve dreamed of having these experiences for years with no hives or pain from ultraviolet light. There is no guarantee this particular medication will work, but I am choosing to stay optimistic.
However, the hope for a better quality of life comes at a price. I was born with another rare disease, an inherited heart condition called long QT syndrome that can cause sudden cardiac arrest and/or death. This risk is increased by hundreds of medications that make up a list known colloquially as the Drugs to Avoid List. Plaquenil and other similar medications are on this list in a class that makes this prescription high-risk for me to take. It is known to prolong the QT interval, even in healthy people (let alone those already living with long QT). It will raise my risk of sudden cardiac arrest even further as it is in one of the highest risk categories.
It has been a long, tiring journey that has led to this point. I have lived in the dark, so to speak, since I was 12, having to do everything in my power to avoid ultraviolet light, whether from the sun, black lights, or artificial lighting. I’ve gone through years filled with many hospitalizations and trials of various treatments with none of them working. To go outside for even a few seconds during daylight means covering up head to toe in UV protective clothing (hat, sunglasses, buff that is pulled up over my face, long-sleeved shirt, long pants, socks, gloves, and closed-toed shoes). Under the clothing is specialized sunscreen. Without these, the 30 seconds it takes me to walk to the mailbox is all it takes for me to be covered in hives. Despite the sunscreen and clothing, I still have reactions.
Through the years, every option that could have helped that did not pose a risk to my long QT was tried as a potential treatment. Steroids (oral and creams), ultraviolet light phototherapy to desensitize my skin, antihistamines and allergy medications, niacin, beta-carotene, and vitamin D were all attempts to alleviate the solar urticaria. Even an ultra-specialized orphan drug called Xolair was trialed. However, that did not work and left me with severe side effects. I even tried six months of IVIG (intravenous immunoglobulin) infusions, which made me sick afterward and left me with aseptic meningitis three times.
Plaquenil could change this, allowing me to feel the warmth of the sun on my skin without excruciating pain. I cannot remember what that feels like. I cannot remember a time when the slightest bit of ultraviolet light on my skin didn’t feel like I was being lit on fire. I cannot remember a time where UV exposure did not lead to extremely itchy hives covering my body, nausea, headaches, dizziness, and irritability. The reaction ends with what my mom has named my “histamine hangover,” where I am so extremely exhausted that I can sleep for well over 24 hours after a reaction when I normally struggle to sleep a few hours a night.
When I was 15, I was told by doctors to move into my parents’ basement, cover the windows with plywood, and live down there forever. As it stands, I react even through windows. Therefore, even being inside by a window is not possible. When I was at a conference a few years ago, a room was rented for the youth forum I was a member of to do our work in for the weekend. The room had a huge window that completely covered one of the walls. There was nowhere for me to hide from the sun in that room. So, my group ended up working in our hotel rooms all weekend. This even eliminates going to a regular brick and mortar school or university as an option. I also react in the shade and through clouds. Neither offers me any protection.
When I do have to go out during the day and I am all covered, I see people turn around and stare. People are afraid of me because of my UV protective buff that covers my face. I have been told that they assume when I enter a store that I am someone with bad intentions, there to do something nefarious. When I was invited to my friend’s graduation in June, I wanted nothing more than to be able to wear a nice dress, but I had to wear full pants and a UV protective long-sleeve shirt instead. Traveling to see my specialists that are located three hours away means crouching down in the back seat with a heavy blanket completely covering my head and body trying to hide from the sun.
To give me this chance at a better quality of life, every precaution available by modern medicine has been taken to reduce my risk of sudden cardiac arrest from long QT. I take a preventative medication called beta blockers, and an additional medication that can offer some protection called Spironolactone. I traveled to the Mayo Clinic in the United States to have an extremely specialized heart surgery performed called left cardiac sympathetic denervation in June 2018, and also have an internal defibrillator/pacemaker implanted. With these treatments on board and close monitoring, my local heart specialist and the world’s top specialist on long QT gave me the go ahead. Despite this, there is still a significant risk that Plaquenil could cause serious, life-threatening arrhythmias and destabilize heart condition. My defibrillator is there, should this happen, to deliver shocks to my heart and save me from any arrhythmias. I have been cautioned that even with my defibrillator, it may not be able to save me if my heart became too unstable.
This is a risk that I have to take. There are no other safe options available.
I don’t want to keep missing out on the things that I used to love.
I want to be able to attend university. I want to be able to be more active and pursue meaningful opportunities. I want to be able to just go outside because I can. I live in an extremely beautiful province in Canada where I can drive a few hours and be surrounded by mountains, national parks, waterfalls, campgrounds, hoodoos, and more. I haven’t been to see any of these things in years and I miss them so much. As someone living with rare, chronic illnesses, I should not be forced to choose between quantity of life and quality of life. However, I am hopeful that the risks I take will be worth it.