Can you tell us, in one sentence, how primary immunodeficiency has impacted your daily life?
I am a university professor, writer, and mother and unknowingly, sneakily, and for many years, PI circled me closer and closer, taking me down more and more until it threatened to take me down completely.
What’s one misconception about primary immunodeficiencies that you wish more people understood?
PI may be a challenge, but it is not a personal deficit. We have a unique perspective and so much to contribute to the world because of our experience with PI.
What’s one thing you’ve achieved this year that you are proud of?
I am completing my third novel and a screenplay and am currently developing a course about disability and creative writing at the University of British Columbia’s School of Creative Writing where I am Associate Professor.
If you could share one message with the world about living with a primary immunodeficiency, what would it be?
We with PI have much to offer the world, but we are deeply concerned about a world where health care is not prioritized for many populations, world-wide.
Please take as much space as you would like to tell us about your experiences.
A segment of the following response is taken from my essay “The Golden Circles,” published in the anthology Against Death: 35 Essays on Living, edited by Elee Kraljii Gardiner, Anvil Press, September 20, 2019
I also read a portion for Immunity Canada’s British Columbia Education & Awareness Day, November 2024.
Storytelling has conflated with disease to galvanize me into the person I am. Through creating my fiction, plays, essays, and screenplays, I have used the writing all my life to make sense of my PI. Now, with growing insight into my condition, the writing and the PI have become intrinsically linked and hard-wired into my body and my mind. One cannot be separated from the other.
When she was five, my daughter realized she will die. She looked at me with stark betrayal as if I’d slapped her. I told her, “It won’t happen for a really long time, and by then you will become part of the flowers and the trees.”
She cried, “I don’t want to become part of the flowers and the trees.”
I could hardly tolerate the cut of her pain.
Years ago, I had seen a little black lamb leap across an open field. My daughter was once like that lamb, but the inevitability of death diminished her joy.
“Maybe when you’re older,” I told her, “they will discover a cure for death.”
Her face brightened as the power of this lie seeped in.
But was it a lie? Science was making progress.
I have experienced many near deaths, complicated, varied, and innumerable. By threatening me all my life, these deaths have taught me how to live. I call these threats, these near deaths, my golden circles.
As a young child I had mumps, infections, bronchitis, ear aches. But don’t most kids, the doctors had said.
Then the tonsils came out at three.
Then as a young teen, I got chicken pox. I was far sicker than the others because I was older, the doctors said.
That led a few years later to a strange undiagnosed illness. I couldn’t walk for months. The doctors never understood why. A virus they said.
I couldn’t imagine anything more serious than a virus.
Then I did get a virus. Mononucleosis. The kissing disease. I missed months of school.
Then super enlarged lymph nodes. Cancer, they whispered. Biopsies came back benign.
During the pre-internet years, I thought death only came for the old, stupid, and unlucky.
Death! What was that?
I had grown up in the 70’s and 80’s. Magazine rock stars glittered in our otherwise sterile suburban imaginations. I had applied myself with a girl guide’s focus to studying tiny knots where individuality could be picked open and unravelled. For years, illnesses manifested and disappeared like the cigarette smoke that curled and lingered around every corner. I didn’t think about how sick I kept getting, allowing intense experiences to fill up the spaces between this circle and the next. I wanted to taste every part of the world. To throw myself against the light and burn.
Then graduation. College. London. Then Montreal. Berlin. Paros. Foufounes. CBGB’S. The Cat Club. Words and warnings surfaced inside me like a magic 8-ball, but I pushed them down. I lived fast and hard and the future burned far away off the edges of the horizon. I felt horrible. Sick. Exhausted. But, you know, I thought, normal.
Then one day I returned home for Xmas. My head sagged into the turkey and gravy. Immediately, whisked off to hospital, placed in isolation, robots attended to me. I realize now they were probably Hazmat suits.
“Your hemoglobin is plummeting,” they told me. “We don’t know if you are a risk to us or we are a risk to you. If you’d waited one more day to get here you’d be dead.”
I was only 26.
Dying in a day seemed dramatic.
My parents stood at my bed when the doctor popped in. There’s a new disease he said. It’s called AIDS.
I wondered if it was even possible to contract a disease I’d only read about in newspapers. It was like a science fiction movie. Then I thought, shit, whatever I’d done to get this totally wasn’t worth it.
I’d grown up in the suburbs. A new house. Bay windows. My father sold insurance. I’d dated a high school football player. I’d once had a perm. As my parents stared in stunned silence I knew this isn’t what they’d had in mind when they conceived me.
But it turned out to be a disease called Auto-Immune Hemolytic anemia. I required high doses of Prednisone.
A splenectomy.
“Fingers crossed this works,” they told me.
“What happens if it doesn’t work?” I asked them.
“Let’s cross that bridge later,” they said.
I’d hidden from the world and threw myself into my writing. Something that I didn’t really think would amount to anything significant, but miles away from all this sickness and death talk.
No travelling to other continents, they warned me, where any mosquitoes carry illness. We can’t save you from those. Oh, goody. That was everywhere I wanted to go.
But the medical problems kept mounting.
Enlarge lymph nodes. More biopsies.
The cervical dysplasia. More biopsies.
Circles upon circles.
Then the pneumonia, the hospital visits where I had the empress suite overlooking Vancouver at St. Paul’s Hospital.
Then pneumonia again: same diagnosis, same suite. Every cloud, even the pneumatic ones, have a silver lining – real estate and views.
Then glass in the eye, excruciating pain, which turned out to be shingles, which is connected to chicken pox.
More circles.
Then I fell in love.
“Do you want a baby?” my husband asked.
“I guess,” I said.
It didn’t matter. As I suspected, I was broken. My body and mind rejected every pregnancy. Nothing in my life had prepared me to hold onto life.
Don’t worry, the doctors told me, you won’t have a baby. You’re too old.
After a few years and a number of losses they finally sent me to an endocrinologist who confirmed that i had Hashimoto’s and could not have a successful pregnancy until I lowered my FSH.
Then almost immediately after beginning synthroid, in my mid-forties, a little girl surfaced within me. Stunned by fear, wonder, and amazement, I wanted to protect that tiny pulsing life.
But as my daughter’s hand curled around my finger, the circles increased, deepened and tightened their grip, replacing that of my daughter.
Every day I walked through a thick fog, exhaustion sheared me to my bones. Baffling illnesses. Pneumonia, shingles, infections, cellulitis, strange rapidly multiplying cells that held knives against my throat.
It’s normal. You have a young child, the doctors told me, what do you expect.
When my daughter was two another circle arrived. My husband just became diagnosed with a serious heart defect and needed open heart surgery within the next few months. Immediately, my back seized, my head seared with pain. I trembled and shook on the floor. My daughter clung to me, reaching, sobbing as my husband pulled her away. My daughter and I were once intrinsically linked, but now my illness separated us.
When I arrived, I was emaciated and could hardly speak. The doctors, convinced I was a drug addict, living on the streets, told me I was fine and released me from the hospital.
“Please,” I begged them, “don’t send me away.”
The next morning my husband roused me from bed. The hospital had called.
Septic shock, the doctors told me. You’re the sickest person at St. Paul’s.
As I slanted on a gurney, they pumped gallons of water into my body to maintain my blood pressure as they gave me drugs to fight the infection in my blood.
The idea that I was dying flashed repeatedly as I stared at a computer monitor that instructed on proper handwashing protocol.
Even with my daughter, knowing that I may never see her again. That she would not remember me if I were to die.
The doctor injected me with morphine and I floated away.
When I woke up, they told me I’d made it but all the medication scarred my veins.
To feed me antibiotics, they inserted a pic line from my arm into a vein near the heart.
My daughter!
I was still breastfeeding before I was admitted to hospital. Where my daughter and I had once been intrinsically linked, the machine they’d attached to me to pump the drugs into my veins had turned me into a creature both human and humanoid. My two-year old daughter in her sweet pea dress stood in the living room and turned to me warily and without recognition.
Then – guess what? – I got sepsis again: sepsis shock once more; though, to be fair, not quite as bad because I caught it earlier, had become a bit (excuse my arrogance) of a master detective at this point.
Before I’d left the hospital, I had been given a radioactive blood tracer to find the bacteria. The same bacteria that had lodged itself inside me and waited like a monster to kill me. They couldn’t find the bacteria, but they had a warning. You can no longer breastfeed, they’d told me. It’s time to quit anyway, don’t you think? My daughter was two. I hadn’t planned to quit until we were ready, I said, quoting the La Leche brochures, via a slow weaning process. But, although I searched for a loophole, I knew that the connection between me and my daughter was now finally severed. I lay on the floor next to what would soon be my daughter’s new bedroom and her new double bed. I wept as the hot milk drained out of me and dried away that tiny fragile bond between us forever.
I was still on the PICC and my husband repeatedly had to postpone his heart surgery, so one of us could care for our two-year old. The city sent a social worker who met with my husband and I to get our affairs in order for our daughter.
“You don’t want to put her in public care, believe me,” she said.
Eventually, I recovered and my husband got his heart surgery. And even though I had weaned off the antibiotics after two bouts of sepsis, my brain still felt as though it was clotted with damp newspaper. I was back at work and wandered the halls of the university like a ghost. Nobody seemed to look in my direction. Maybe I was already dead, I thought.
I went in and out of emergency throughout the next handful of years. But after another bad infection, my infectious disease doctor sent me to an immunologist.
The immunologist did his tests. You don’t have a spleen, he said. The scar with its decorative perforations left by the surgical staples reminded me every day his assessment was correct. Here, the immunologist warned me of the hazards of a splenectomy, making me incapable of fighting certain bacterial infections, especially those that affect the lung. But he also told me I had [Monoclonal Gammopathy of Undetermined Significance (MGUS)].
Yay! Another disease. Another circle. I had never heard of half of these diseases, so why was I getting them?
But now I was lucky enough to know all about MGUS. I knew that the MGUS lowered and permanently impacted my antibodies. Making it so that the protein produced by the MGUS altered the readability of my antibodies. I would thus never know if my antibodies were increasing nor would I ever effectively respond to vaccines – so far as anyone could determine. These left me absolutely vulnerable, in some cases untreatable, and not fully diagnosable.
The immunologist sent me to a hematologist. The MGUS is a precursor to Multiple Myeloma. “We will need to test you for this every six months in case you develop this bone cancer or its smoldering precursor.”
Then UTI’s. Infections that wouldn’t quit. Antibiotics. No swimming, they told me in water that isn’t salt. No lakes. No pools. No hot tubs. Then soon, no baths at all.
Another circle.
Then, wow, I was so weakened that when that bus ran a red light I fell back and saw stars. The next few years I spent in dark rooms away from light and sound. Caution: don’t go on a child’s fair ride even a year post-concussion
The Boy in the Plastic Bubble was a movie in the 1970’s starring John Travolta based on the life of David Vetter and Ted Devita who had [Severe Combined Immunodeficiency (SCID)]. I had a milder subset of primary immunodeficiency called hypogammaglobulinemia. I don’t live in a bubble, but as of yet there is no cure and infections could be life-threatening.
“Wow!” said my Hematologist, “Thirty years ago you were diagnosed with Autoimmune Hemolytic Anemia. It was a mystery then but now we know probably much of what you’ve had is connected to the condition you have now.”
A constellation of symptoms. GP, endocrinologists, Infectious disease, hematologists, immunologists, internal medicine people.
Science’s tempo was a frenzied primordial dance whose strange offerings seem to spark off at random.
Maybe a cure for death was around the corner.
Then early cataract surgery because of all the prednisone I’d taken for the Hemolytic Anemia. Well, what’s the big deal, I thought, I’d probably need the surgery eventually, just got the early bird special.
Then, as if this wasn’t enough. When is it ever enough? The pandemic hit.
I won’t leave the house until it’s over, I told everyone I knew.
But due to my condition my daughter and husband could also no longer leave the house.
This is Vancouver where every square foot is at a premium. We’d just bought our tiny condo to finally get a toe into the market. But the apartment was about 940 square feet and we three lived in 2.5 rooms.. I’ll work in cafes when I wasn’t at UBC, I’d convinced my husband when we bought the place. But now here we were. Prisoners of our own real estate, isolated from even our neighbors, now excited only when the lights of a car grew and shrank past our windows.
“I feel like a tiger in a cage,” my daughter lamented, “staring through the bars of our balcony.”
At the beginning of the pandemic, we didn’t even go outside as doing so meant we’d need to go through the shared halls and my building wouldn’t commit to mask wearing. You’re the only one who is asking for it, they said.
I have a medical condition. Does my life mean nothing to you? I lamented over Zoom, but my neighbors all stared back at me wearily.
It’s not fair to your daughter, a doctor friend said. But what’s worse, losing a mother- a major trigger for depression- or do I keep the kid locked up, so that my twelve year old loses two years of social development? (How long would this last? We all wondered.)
Here ImmUnity Canada made all the difference. Those meetings during the bleakest of times when I had no interaction with others, except on Zoom, when I lost friends and couldn’t go into work or even see family except my immediate, when I did talk to people on those Zooms they often didn’t grasp the terror of someone who believed they could possibly die if I stepped outside those 2.5 rooms. Remember: this is before we had vaccines, Paxlovid, Evusheld. We’d heard stories on the news of gross numbers of people dropping dead in the corridors of hospitals. Young, healthy people. What would happen to us? To our families? My mother had a serious medical crisis in another city. I talked her through months of evaluation and treatment only by the thread of my landline. But ImmUnity Canada and my friends were there. They got it. We’d discussed how to keep ourselves and our families safe. How we could get access to vaccinations and drugs. How we could keep our jobs and make them understand our plight. And we asked the single question that seemed too horrible to even fully grasp: what if this pandemic never ends? Especially as weeks turned into months and then several years. Eventually we’d learn to evaluate our chances and to take well considered risks. But not without the support and encouragement of our ImmUnity Canada friends, solidifying friendships that would last a lifetime.
Then after all this, and the treatments and protocols, I picked up the phone. Nothing. I moved the receiver from my left ear to my right. I could hear my friend talking into the phone as clear as day.
Out of the depths of the tiny hole of my apartment I crawled to a hearing specialist.
“You have a brain tumor. It’s the only cause of this kind of hearing loss.”
“But surely not the only cause.”
“Yes,” they said. “The only cause.”
Another circle.
Later that same year, I noticed I had two ankles on my left leg. I’ve never noticed this before, I thought, examining the two bony protrusions.
Another circle. Another tumor.
“Prepare for cancer,” the surgeon said. But it wasn’t cancer. It was a benign tumor, the same one in my head. They removed the leg tumor.
Then the head tumor grew fast and hard. I went from single-sided deafness into neurosurgery. I could no longer walk without falling over. I couldn’t see out of the left eye as I no longer produced tears on that side and it hurt to keep it open. I was slumped over when I walked, couldn’t see, couldn’t hear. My face became numb and food had no flavour.
Another circle.
No neurosurgery. I’m scared to have permanent facial paralysis!
It didn’t matter that I was 60 years old. That I had been with my partner for more than 20 years, that nobody saw me because I rarely ever left the house. My face was def not my money-maker.
Well, we can’t control what happens to your face as the tumor is adjacent to the facial nerve. But we can try. We want to see if we need the hearing nerve.
“Okay,” I told them, “Permanently kill my hearing but please save the face.”
“We’ll try to save the face,” they said, “but the hearing is already dead.”
The three of us still live together; but we are wary, apprehensive, and walk around highly alert as we take each step and wonder if it will be our last.
The brain surgery worked. But the risk of the tumor returning is about 10%. I was warned I might not be so lucky next time.
Today I wait for the next circle. The one that will finally take me when I can no longer fight. Just as the oryx is lifted to be licked, evaluated, and devoured by the lion. But I am not yet the oryx. I want to be the little black lamb, racing through that open field.
My daughter still sometimes asks if she’s going to die, if I’m going to die, and each time she asks the spaces between grow longer, and I know soon, she won’t need to ask anymore.
But I was alive, we were all alive. I had to accept my limitations and make a choice. Was I going to feel badly or embrace life? I reached deep inside myself and within the tiny scope of my life I found joy.
As each circle embraces me, I need the superficialities of the physical world less. By reducing the world, I have found meaning in the tiniest details.
The circles live inside me. We are part of the same organic fabric. The same fabric that made my daughter, the flowers and the trees.
The fog that has descended on me all my life has lifted. Today I am a writer, a novelist, a screenwriter, on faculty at UBC, currently developing research that centers the disability experience as a force of truth and creativity. I have discovered that my experiences have given me an unusual POV on living that has made me a more interesting person and writer than I would otherwise be. This sounds like a cliché, but it may be true in my case. Would I be other than I am if I could? The answer now would be probably not. The myriad of conditions over the years has alchemized me into exactly who I was always meant, and dreamed, to be.
The pandemic may be over for now, for today, but I don’t believe it won’t return in some other form. Maybe tomorrow. Maybe in a year or ten. I urge you all to continue taking care of yourselves. To be your own best advocate. We have learned our lessons. We need someone to galvanize and speak on our behalf as we now know that in our highly politicized world with increasing economic disparity and stresses on the medical system that we do matter, that we are not disposable and we each make an invaluable contribution to our communities – but that numbers talk. I encourage all of you to continue your involvement and support of ImmUnity Canada – our patient organization – who is our home and our greatest support and advocate and disseminator of knowledge for the needs of our very special group.
Thank you to Immunity Canada whose support, advocacy, and information meant so much during all these times. At those meetings I finally felt validated, not crazy, and very much sane through the darkest times and now, finally, back into the light.
What emotions and revelations have you encountered throughout your journey with primary immunodeficiency?
Aside from my aforementioned response, I would underscore that having PI has ripped the rug from under me time and again. Or, more consistent with my aforementioned symbol, a golden circle. As I tried to explain earlier in this questionnaire, each time I’ve become ill, I’ve learned so much about life and death – the circle of life. I have a deeper understanding of the human experience and much more to offer the people in my personal and professional life as well as my friends within the PI community. Make no mistake: I love life. But I also know I no longer need to fear death. This fearlessness makes life more precious, but it also makes me more courageous to fight for what is important and to take calculated risks. I love life so much, and place such a value on it, that I know I’m very fortunate to be alive right now in this complex world with its many crises. And that many others who may or may not share my diagnosis have not been as fortunate. I know that I share my experience with people who are suffering and those who are surviving throughout the world.
My shared sorrow has galvanized within me a strong desire to educate myself, to advocate and to protect.
What challenges did you face during diagnosis and treatment?
The doctors I have spoken to and encountered through in the years I’ve been ill (since the 1970’s and beyond until today) were not aware of this condition as far as I knew. While each doctor helped me recover from the manifestation of the condition I had at that time, any further exploration appeared to be dismissed – even though I was obviously very ill. What the most cognizant and alert doctors did understand is whatever condition I was manifesting with each given illness, but they did not understand that all of these conditions were obviously connected like the roots of a tree, feeding and being fed by the same conditions. Only once I received my diagnosis of PI did the separate threads connect to form an understanding of my special unique physiological system – a system that strikes me with awe at my strength and survival and the deepening understanding of both the medical system and the PI community.
If you could send a singular, powerful message to the world about your condition, what would it be and why?
Storytelling allows us to be seen, we can be strong, we can support one another, we can build a stronger community of patients across Canada and demand our rights as a united, powerful force for good to improve the lives of all future patients who are affected by primary immunodeficiencies.
People with PI are uniquely positioned and their stories have weight and value that transcends the specificity of our disease.
Please do not disregard and underestimate the power and privilege of living with and surviving a rare disease. Those of us who have these conditions have so much to offer the world in understanding the power and potential of the human body and spirit.
Here we are, the zebras, the symbol of rare diseases, inviting all of you to tell your stories, experiences and hopes for the future. Historically medical professionals were told, “when you hear hoofbeats, think horses, not zebras.” But we with PI are the zebras. We are the different. The unusual. Beyond horses and zebras, we, the diagnosed, are the scapegoats of history and archetypes. The scapegoats are often targeted for society’s ills, not because of our differences. We are often punished and driven out of our communities. Even exterminated. The one in a million. In this world of growing us v them, where the number crunchers are now making cutbacks to science and medicine, we cannot afford to be silent. We must have agency. To speak up. To educate. Disease is our humanity, our interconnectedness, and to place a value on those differences that do not weaken humanity but strengthens it through diversification.
We are not disposable. We are your parents, children, students, bosses, workers etc. We count and we need to be protected by those in positions of power worldwide. We need the protection of leaders who value human differences, the contributions of the vulnerable to this complex living system, and we cannot be turned away as costly, disturbing, and/or difficult. Eventually, no matter how much we buy and what we use to distract ourselves, everyone will grow sick and even you – you will deserve our protection. As you are one of us and we are one of you.
You can find Maureen’s full essay in Against Death: 35 Essays on Living HERE.
You can also listen to Maureen share her story at our BC Education & Awareness Day on our YouTube channel HERE.