Stem-cell therapy
My story begins at 3 p.m. on February 25, 2015. I’m referring to my crisis, a Greek word that connotes the “turning point” of a disease, when an important change takes place indicating either recovery or death. We’ve all had such instances, and this was the most significant of mine – a curveball that became a moment of enlightenment.
While attending a conference in Boston, I was shivery and cold. But that week in New England, 239 centimetres of snow had tumbled from the sky, and the downtown streets were freakishly lined with three-metre walls of snow. I convinced myself I was cold because of the weather. I was shaking so dramatically, I left the conference hall for my hotel room, and filled the bathtub with hot water. The hot bath seemed to do the trick, except that as I was dressing to return to the conference, my body broke into a profound sweat. My ankles, thighs, hands, and forearms were sweating. I was the Trevi Fountain of sweat. And then I was fine, as though something nefarious had simply breezed through my body and left.
Unbeknownst to me, it was my first case of rigors, an extreme reflexive response to exaggerated shivering. It was what the soldiers felt in the wretched wet trenches of the First World War when they had contracted tuberculosis. Sweating is the body’s attempt to cool itself.
A long 10 days
After 10 straight days of rigors and night sweats, I went to a walk-in emergency clinic. The doctor asked questions about my personal life, then said, “There is nothing wrong with you. It’s anxiety.” I danced away, feeling as light as a bag of cotton candy. I had ways to deal with stress, I told myself. The engine of my life continued to run smoothly, except that for five months and nine days I had profuse, mattress-soaking night sweats.
During that stretch I also had enlarged lymph nodes under my chin, as though a string of pearls had been embedded beneath my skin. I saw three specialists, and had an emergency CAT scan and a bone-marrow biopsy, all of which turned up nothing conclusive. I saw an oncologist who ordered a neck biopsy of the golf-ball sized lymph node under my jaw. Trust me when I say a neck biopsy is not as good as it sounds. Seven needles were inserted into an area extremely close to the carotid artery, causing excruciating pain. Oddly, the pathology report showed no cancer, no leukemia, no lymphoma. It did show complete and total cell death – necrosis – but no diagnosis.
This entire time, I was living my life in every normal way: I was about to publish my first novel, and teaching spin classes at 6 a.m., working at the Douglas Hospital in Montreal with patients suffering symptoms of schizophrenia, and caring for my husband, who was battling depression. In hindsight, I was hurtling down a black tunnel, but I had every intention of launching my book, A Secret Music. That single-mindedness has given me great pause. We are able to compensate when we need to, even when the body is sending out a multitude of distress signals. It was as though I had run through a dozen red lights.
Gradually, then suddenly
In May, June and July, I did a book tour, ending every evening with four to five hours of extreme night sweats. I had a fever of 103°F at each event, but I was ridiculously happy. The mind is a powerful tool.
In August, at our beach house in Kennebunk, I got sick the way Ernest Hemingway says people go broke: gradually, then suddenly. My chills and fever occurred around the clock, instead of only at night, and then, in case I needed a final wake-up call, the right side of my face went numb. I couldn’t feel my gums or my nose. I could no longer wish myself well, or pretend it was stress. My husband and I drove 470 kilometres from Maine to Montreal. I had a fever of 105°F, saw clouds in the shape of angels, and prayed I wouldn’t have a stroke before we got to the hospital. At the border we were the only car, and in triage at the Jewish General Hospital I was the only patient. I had the distinct impression I was travelling first class.
I spent the next five days undergoing every conceivable medical test, including bone-marrow biopsies, lumbar puncture, lung scans, MRIs of the body and brain, CAT scans, PET scans, and blood tests. I was taking 4,000 mg of Tylenol a day to combat the fevers. I had visions. My father, who passed away in 2006, appeared several times. Scrawled across his face was the message: Do not come. It’s not your time. Stay and fight.
Through process of elimination, I was diagnosed with HLH, a rare autoimmune disease with a death rate of 70 per cent. Hemophagocytic lymphohistiocytosis. It’s a good Scrabble word. It affects one in a million adults. Without treatment, the patient dies of multi-organ failure within 60 days of onset. Nobody knew where I was on that trajectory.
The body’s security detail
Your immune system is your security detail. It’s hard-wired to tell the difference between what belongs in your body and what doesn’t. When it spies a villain like a virus, bacteria or a parasite, it finds it, surrounds it, kills it. Your immune system turns on if you have a cold or sore throat, as it’s supposed to, but then it turns off. With HLH, it never turns off. Think of a driverless and powerful John Deere lawnmower in your backyard destroying everything in sight.
Once HLH was identified, I was hurried into a chemotherapy protocol that very night.
My children flew in from New York. I had months of regular chemo, as well as intrathecal brain chemo (in which medication is injected into the fluid-filled space between the layers of tissue covering the brain), 32 transfusions of platelets, and a dozen types of intravenous treatments through a catheter that had been sewn into a vein in my neck. I also took high doses of Decadron (dexamethasone), the type of steroid Lance Armstrong used to win the Tour de France. I needed it to win the Tour de Vie.
Thankfully, I responded to the HLH protocol, and left the hospital on a sunny day in late October, ready to resume my book events. It was a euphoric time in my life. I enjoyed 77 days of wellness – but then the wheels fell off the tracks. HLH came back with a vengeance, and my organs were threatened. I would need a bone-marrow transplant if I had any hope of survival. Did I have any siblings? Yes, I answered, five. Good, the doctors said, siblings are the best possible match.
A surreal feeling
After all that was swirling around me, I had the surreal feeling that I was about to jump through a one-way portal, that I would never be the same again, forever changed by the people I would meet, the endurance test, the lessons I would learn. I was to put my arms around this experience without fear. Don’t they say the steeper the climb, the better the view from the top?
I entered the Royal Victoria Hospital in Montreal on May 15, my sister’s birthday, an auspicious date. Two days after I set up shop in my isolation room – with my weights, my yoga mat, my orange running shoes, and a calendar painted with cardinals so I could strike off each day – I went to pieces. It would be disingenuous to say there weren’t some terrible days. I lay on the floor of the bathroom so nobody would hear me crying as all the seams of my body opened up to let out my distress. Could I run a marathon without any practice? Did I have the stamina to cross the finish line before my body broke down? The doubt, fear, and psychological pain were much harder than the physical pain.
Extraordinary properties
Inside your bones is a hollow passage, filled with soft spongy tissue, where your blood is made. That tissue is called bone marrow. The marrow is composed of red blood cells that oxygenate your body, white blood cells that fight infection, platelets that help clot a wound, and immature cells known as stem cells. The body makes stem cells every minute. Stem cells have two extraordinary properties: They multiply like crazy, and they have the ability to become any type of cells in the body, as needed.
The stem-cell team removed all my bone marrow with chemotherapy. Blood cancers like lymphoma and leukemia are treated the same way. The side effects are profound: My hair fell out, my muscles wasted from the steroids, there was a cardboard hat to collect black diarrhea, I had lesions along the digestive tract, I sucked ice cubes to counteract mouth sores. I had near-daily blood transfusions.
Let me tell you, I felt ugly. But my husband arrived every single morning with a cinnamon latte that made me feel beautiful inside.
With chemotherapy, my immune system was eliminated, a process that took several weeks. I had four eerie days when I had zero white blood cells, and no ability to fight infection. Germs of every kind are your fatal enemy, hence the strict isolation.
Finding a match
One of my brothers had matched but for genetic reasons was eliminated from contention as a donor. That devastating cancellation required me to be listed on the International Donor Registry to try to find another stem-cell match. That was a terrible time. The clock was ticking. The 60-day window was shrinking.
For all bone-marrow transplants, matching occurs through HLA (human leukocyte antigen) typing. HLA are proteins, or markers, found on most cells in your body. Your immune system uses these markers to recognize which cells belong in your body and which do not. The best possible outcome for transplant is when the patient and the donor have 12 identical isomers, a certain type of chemical compound. William Ashby-Hall, a 23-year-old gay British man, was a 12/12 perfect match. There is no payment to the donor; it is done altruistically, out of sheer compassion. I mention his sexual orientation only because the rules for gay donors are highly restrictive in Canada and the United States. The same day William’s HLA typing was entered into the computer system, he was called and asked to save the life of an anonymous Canadian woman. He jumped.
On the day of the stem-cell retrieval, William sat in a recliner in a hospital in London, and over a period of eight hours his blood was drawn from one arm into a machine via a plastic tube. The stem cells from his blood were extracted via centrifuge and the blood, minus the stem cells, was returned to his other arm. It’s a procedure called apheresis, and replaces the old way of obtaining stem cells, which was done by inserting numerous large needles into the hip bones and withdrawing bone marrow. Apheresis is pain-free.
A Canadian policeman waited in the hallway. After the stem cells, salmon pink in colour, were collected, the officer flew to Canada with the fresh cells, which he brought to the hospital lab for verification. (William’s HLA typing was reconfirmed! Thank goodness.) I waited on the stem-cell unit with an unbridled sense of hope, tinged with dread. Would my body accept his cells? Graft versus host disease (GvHD) occurs when the patient’s organs reject the new stem cells. I’m here to say, it was a success.
First patient in Quebec
I was the first patient in the province of Quebec to receive a stem-cell transplant for HLH. The statistic is even more stark, given that most people die before they receive a diagnosis and a chance to be treated.
People have asked me if the ordeal has changed me. I’m still me, of course, but near-death can be an amazing teacher. Every detail about what one desires comes sharply into focus. What has changed is my attempt to preserve the sanctity of all that I hold dear. I guard my human possessions. People have also cited my resilience. No. A thousand hands lifted me back to where I am now.
I began to write thank-you letters to my donor, despite the fact that no contact can happen for two years; the death rate is too high. The first letter simply said, “It’s been 100 days, the first hurdle. I’m alive.” The second letter said, “It’s been one year, thank you for the miraculous gift of your stem cells.” The third letter said, “I would love to look you in the eye and thank you for giving me a second chance at living my life.”
William contacted me 27 months after my transplant. I met him in London on January 17, 2019, surrounded by my family. It was one of the truly transcendent moments of my life.
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