When the World Health Organization announced a global pandemic of the H1N1 virus in June 2009, it was anticipated that the new strain of Swine Flu would target the young and potentially claim as many people as the Spanish Influenza of 1918—millions. The British Government responded to this warning by supplying a vaccine, one that they claimed was both safe and effective, but one that had not been tested on children. General practitioners were financially incentivized to vaccinate as many of the population as possible. As with all parents of children under five, we received a letter strongly recommending the vaccine.
What emerged in 2010 was the realization that the new strain of H1N1 was not the threat predicted; no more lives were claimed than in any other flu season. Yet for the thousands of vaccinated children across Europe, 1500 already had begun to display bizarre neurological symptoms. For them it was too late. By the time each of those children had been diagnosed with narcolepsy, an incurable sleep disorder, the 70,000 neurons they needed to regulate sleep and wakefulness had been destroyed.
I have written this memoir as a mother who lost and regained a daughter; as a mother who, in the quest of waking Mathilda, has sought to illuminate the myths surrounding a condition that afflicts the mind by night and body by day—one that is under-diagnosed, under-recognized and under-reported. Mathilda’s descent into narcolepsy, the hospital admissions and battle for treatment within the confines of socialized health care in England are memories so raw, it is as though they happened yesterday.
Yet six years into caring for a young child with narcolepsy, I am only too aware of the impact of my own sleep deprivation. Throughout this journey, I have turned to the pages and pages of her medical notes, letters between doctors and a journal I kept at the time: a small leather-bound notebook once used for scribbling ideas, magazine cuttings of pretty backyards and academic resources for our older two children. Those entries dried up and were replaced with hospital phone numbers, prayers and lists of new symptoms as they transpired over the months. Friends and family who observed Mathilda’s decline in England offered helpful insights into both the baffling display of symptoms and the impact of her deterioration on all five members of my family. I have also relied on video footage and interviews with doctors who played a significant role during the time Mathilda remained undiagnosed.
What follows, then, is a recounting, to the best of my abilities, of the last year and a half we lived in England. I have not, could not, in any way attempt to understand or rationalize Mathilda’s journey into narcolepsy. Making sense of the why, to what end is anyone’s suffering, I leave to the theologians and philosophers out there, my husband Oliver among them.
In the pages of this book you will not find answers to such questions. What you will read is the story of an unremarkable family who took incredible risks to ensure their survival. I want people to understand what happens in the lives of those whose children suffer with an incurable and debilitating condition. I want people to realize that although living with a child that has narcolepsy is an extraordinary experience, we are not extraordinary. And I want people to grasp that when adversity strikes at the heart of your family and your own heart, it is possible to light a candle in the desperate darkness of each night.
Narcolepsy has added another chapter to our lives. One that I have despised on more days than I care to remember. But one that I trust will resonate with all those that have endured the same crushing heartbreak of loving a child for whom you wish you could just take it all away. That my daughter suffers more than I do still seems wholly wrong to me, and yet deep within her soul, she is the same child I bore ten years ago, my child. Therefore it is incumbent upon me to tell her story as her mother—a story that we together share with millions of others.
With the exception of Dr. Siddarth Shah and Professor Emmanuel Mignot, I have changed the names of doctors, not wishing to expose those whom I believe did what they thought best for us as a family and for Mathilda—mighty in battle.
Early in the evening of April 15, 2010, Mathilda sways in the doorway of our bedroom, then locks her knees. With several slow blinks comes a sharp flick of her head, as if to wake herself before stumbling in our direction and promptly falling back to sleep between Oliver and me. Unconsciously she wets the bed. Her drowsiness has gripped her so tightly that she does not wake until the three of us are halfway through an assessment in the ER room at Bristol Children’s Hospital. We tell the consultant about her excessive sleepiness during the day, the miserable waking and crying out through the night and the dramatic change in her personality. I explain she is also ataxic, has low tone, which she describes as feeling floppy and that, as an experienced physical therapist, I believe my daughter is neurologically sick on a fundamental level.
With one hand I wipe my face, then think over what I just said. I was a physical therapist, not more than being her mother, yet I know how the examination works and gently coax her to walk across the room for the “nice lady doctor.” Mathilda slowly grumps away, staggering like a drunkard, holding out her arms to steady herself. Her steps, though intentional, are interrupted by a level of concentration she cannot hold. Once on my lap, the cold disc of the stethoscope against her chest jolts her back into the aseptic world of the ER room; she begins to cry and turns inward, nestling under my arm. I manage to keep Mathilda awake long enough for the doctor to shine a light into her eyes, test her reflexes and scrape the underside of her foot several times with the pointed end of the patella hammer. Moments later, her three-year old body is asleep again, and I feel a rising panic with the slow release of pee all over my lap. Yes, this is new. No, she never struggled with going to the bathroom independently. She had in fact been potty-trained for over a year. Oliver turns the doctor’s attention from the wet patch spreading down my legs and fills the silence. Mathilda was always a bright and breezy child—sweet, funny, articulate, a real chatterbox with a joie de vivre. She had begun to read, but now was slurring her words and dropping off mid-sentence. Having returned home from a conference that weekend, Oliver could see the changes in his youngest child. He knows Mathilda isn’t right—that these symptoms are hitting her hard and fast and that he, we, are both at a loss.
The doctor fails to wake her with tickles and the promise of new toys and friends in the play center upstairs. Gradually the tenor of our conversation changes, and I recognize that fixed look when doctors start to choose their words more carefully. We were “right to bring her in,” and, “admission to the Neurology ward will be arranged immediately. The consultant neurologist and head of pediatric services may want to do an MRI on her brain. Tonight.” I want to shake the doctor’s sympathetic hand off my arm, aching from supporting Mathilda’s seemingly lifeless body. I am so distracted by the singularity of meaning behind the doctors’ words that, as if in a trance, I carry my daughter, floppy as a rag doll, and follow a young nurse to a bedded area behind the ER room. Distraught, I wait for admission and a bed on Ward 36.
Before our children were born, I worked only very briefly with pediatric patients when I was first qualified as a physical therapist. At West Middlesex Hospital in London, one of my duties was to reposition the ankles of club-footed babies and teach parents how to maintain the realignment I had achieved with strapping tape and stretches. Whatever their age or problem, I rarely had any trouble talking to patients. It was part of my training, part of my job, and part of me. The only time I had to reach deep down was when I rotated onto the intensive care unit as part of our post-graduate training, suctioning phlegm out of breathing tubes and stretching the limbs of the unconscious and those who reluctantly rapped at death’s door. There is evidence to show that those near death can hear everything, yet on those days I felt self-conscious talking to monitors and never receiving a word back. I did what I could to promote the hope that belongs to every living soul, beginning my monologue and rhetoric over again most mornings on a unit full of new patients. But by the end of each long week, the turnover of patients was high enough to leave me emotionally wrung out and turning down offers for a night out on the town with friends. I was not cut out for it. After six months of crying at the end of each shift on intensive care and smiling at the beginning of the next one, I decided to move to orthopedics.
Mathilda was now lying on a hospital bed two miles away from our home in an overflow ward with half a dozen other children waiting to be transferred upstairs onto a ward. In a bed next to hers, a tube-fed older girl was either groaning or talking, I couldn’t tell, whilst her mum ran a large brush through her thinning hair on the pillow. Her forearms were spindly and twisted across her chest, her fists contracted into tight balls under her chin.
“What’s wrong with your little girl?” she asked me, a question that felt like a probe into something deeply personal.
“We don’t know yet,” I murmured, hoping it would close her down. She told me they lived somewhere between home and a range of hospitals, including Great Ormond Street in London and Bristol Children’s, all the while trying to bring Rosie her daughter into the conversation, as if she would suddenly sit up and join in. Fearing she would draw comparisons between our daughters, I found myself struggling to articulate a response to her ongoing questions. But she was garrulous enough that I could get away with listening and throwing out the occasional platitude. She also had a very jolly disposition, despite her daughter’s severe disability and normal results for every test imaginable. “How could all her brain scans be clear when she is like this?” she asked me. Rosie had seen specialists from all over the world and still needed 24-hour care.
Now, doctors were being flown in from Paris to assess her. They still had hope. Hope of understanding why their perfectly normal child had gradually regressed to a bed-bound infant with a feeding tube and no intelligible language whilst her body passed through puberty. The only time I detected grief in the mother’s voice was when she told me 40% of pediatric neurological cases remain undiagnosed in England, even after years of extensive testing. In my own mind, I trusted her figures were exaggerated and tried blowing them off; to have a declining child and no idea why was close to my worst nightmare. Caught somewhere between my past and this absurd present, I was suddenly alarmed by the thought that I was supposed to suction Rosie’s tubes out and unbend her contracted knees. I imagined I would readjust her head, put her hands in splints to prevent further contractures and walk away. I could so easily have done that. But then I remembered I was on the other side of the fence now. And what a change that was. To go from knowledgeable to vulnerable, from clinician to patient, from happy to heartbroken. I wanted to switch back. And I wanted to take my baby home.
It was almost midnight when Dr. Anthony Jensen, consultant pediatric neurologist, and his team swept on to Ward 36. Mathilda was sleeping at the far end of a six-bedded bay beside a wall of glass, facing a city burning with streetlights and rattling with the constant peal of sirens. Oliver and I sat on either side of her bed, looking exhausted by the time the five doctors, hovering for a moment by the nurse’s station, looked our way. Despite the late hour, there was an easiness about their presence as their hushed jokes belied the purpose of their visit. It seemed as though, after a long day, they had saved this new, straightforward case until last, and I felt myself relax into thinking things were not so serious. Doctor Jensen, a balding man in his forties, led his party towards the foot of the bed, dropping down by her feet covered by a green counterpane.
“This must be Mathilda. I’m Dr. Jensen, head of Pediatric Neurology. How’s she doing?”
The open question led to vague answers about Mathilda, who was sleeping so soundly, one could have thought nothing was wrong with her. All she offered them was the shallow rise and fall of her chest and the skating of her eyeballs, like pendulums grafted beneath translucent eyelids.
During this exchange of questions and answers, Dr. Jensen ascertained that Oliver was a Reader in Philosophical Theology at the University of Bristol. They engaged in a conversation, which led to the realization that they were in fact colleagues. Dr. Jensen taught part-time at Bristol University Medical School, and their offices were just a few blocks away from each other. It was as though they had just been introduced at the university club over lunch, somewhere else, a million miles away from a sick child. And yet, I was not entirely unsympathetic to their effortless chumminess. A few moments’ escape was intensely appealing—if only I could lift my eyes from the child between them.
With the four junior members of the team largely observing, Dr. Ellen Dimlock, the intern, stood some distance away, stroking the ear pieces of the stethoscope that she wore round her neck like a necklace. She was the only doctor in a white coat, which did little to hide her immaculate preppy clothes. I rose to shake her hand, sensing she wanted to nail specifics with me whilst Oliver and Dr. Jensen went on with their professional pleasantries. I listed Mathilda’s symptoms for the second time that day: uncoordinated muscle movements (ataxia), slurring of sentences, pronounced daytime exhaustion, and the inability to sustain sleep either side of nightmares. I also relayed how she kept falling down whenever she tried to walk or play, that her personality was shifting, and that her latest symptom appeared to be incontinence. All this began, I said, around the same time as her collapse several weeks back, when a doctor at an ER Room on the South Coast diagnosed Mathilda with a urinary tract infection.
One of the young house doctors, who I later came to know as Rafael, went off to find a patella hammer, a basic piece of medical equipment for any neurologist in any hospital. He returned several minutes later with a nurse fumbling an apology. There did not seem to be one in the ward. Since none of the doctors carried one either, Dr. Jensen made light of resources being scarce in the National Health Service, a comment that sent further ripples of laughter amongst the team. Then, cutting in as if to reign in an unruly pack, he said:
“I’ve had a chat with the ER doctor who admitted Mathilda this evening. I don’t think we need to worry about waking her and performing another neurological exam now. The clinical picture here seems pretty clear. She’s an excellent doctor and I have no reason to doubt her judgment.” Then, just as the screeching of sirens outside stilled, Dr. Jensen dropped a bomb. “I am not going to bullshit you,” he said, lifting his head, his hands clasped together. “We’re looking at a cerebellar tumor.”
Brain tumors that lie between the brain stem and cerebrum typically afflict a person with drowsiness, unsteady gait, ataxia and personality changes. This was partly consistent with Mathilda’s symptoms, and the immediate MRI under anesthesia was expected to confirm the diagnosis of a malignant lesion. Dr. Jensen, so confident in his colleague’s earlier assessment, asked the on-call radiologist to let him know, at home in the small hours, the precise locale of Mathilda’s tumor. Standing there shaking, I simultaneously tried to process what he had just said whilst grasping on to key words he threw out, like biopsy, chemotherapy and treatment options down the road. Yet by the time I had ordered my train of thoughts to form anything like a coherent question, he and his team were gone.
Oliver drew the curtains around Mathilda’s bed and held me very close for a long time, saying nothing. His towering height and strong arms went some way to assure me that, even in the event of malignancy, together, in one piece, as one unit, we would get through the impending threat to our child’s brain. Screening out the commotion in the ward, the nurses talking in the drug room, and the lively conversations issuing from several TV sets, he finally drew back and raised the safe issue of practical arrangements. It was a given that I would stay, the one who spoke the language of hospitals, and that he, terrified of them, would go back to our older children, Liberty and Elliot. Yet even the thought of our regular lives at home, the kids sleeping in their bunk beds with toys strewn across the room, couldn't reel me in from what lay ahead. And his love for us, however deep, would reach only so far. It could not actually change the facts, certainly would not alter what showed up on the MRI. Neither of us, as parents, professionals, or anything else, could erase her symptoms or lessen her suffering. This realization of utter powerlessness left me feeling as if we were failing our child, giving rise to a sense that the entire situation was unnatural, perhaps even theatrical. There must be some explanation for the aberrance in what was unfolding. It was not right. Any of it.
Despite the surreality of the situation, I could not reconcile my sense of injustice with the fact that every ward at Bristol Children’s Hospital was full wall-to-wall. A 160 bedded regional unit, that meant 160 sick children. And what of the others? The dozens in the ER room and the overflow ward? The children in different hospitals, different countries? The war I began to wage on myself that night against my own rationalization of suffering left me oscillating between our neurological demise and the simple question—why not us? Never did I expect to dodge all the bullets of ill health. But when they came, it seemed like a firing squad had let rip on my heart.
I understood that Mathilda, like every other child in the hospital, was at the mercy of God, who, if He so desired, could wipe the brain scan clear and return her to us by breakfast. She was also at the mercy of the doctors who would go on caring for her long after the MRI was done. In my own my mind, I had begun to acknowledge that whatever was wrong with her was here to stay. It was an intuition that came at me with a maternal gravity that I had, up until that point, never before encountered. Then, facing the most frightening few hours of my life, I watched Oliver bend over Mathilda, whisper a prayer of his own, and just as the team had done half an hour earlier, walk away.
Leaving Mathilda asleep, I went in search of a phone. My parents have always maintained an etiquette regarding the appropriate use of the landline, stipulating that, under normal circumstances, nobody should make or receive a call after 9 p.m. Ringing later than that signified something terrible—an impending disaster or a death. This courtesy I upheld even when I went into labor. A number of babies are prone to arrive in the small hours; mine were no exception. Not wishing to deprive my parents of a night’s sleep we left the good news until morning and called after 9 a.m. Even now unable to break the familial protocol, I rang my sister Amanda instead, and as soon as she picked up, I broke down. Standing at the nurse’s station and sobbing into the handset, I listened to the steadiness in her voice.
“Claire, you don’t know that yet. You don’t know what’s wrong with her until the scan comes back. Stay with the facts. And right now the facts are that Mathilda is in good hands, and as far as you know, she doesn’t have a brain tumor.”
Stay with the facts. Stay with the facts. I replayed her words like a chant. Crawling near the cot-bed between Mathilda and the cold wall, I offered up my own petition: that God would spare her, that there would be no brain tumor, no disease at all. My words blurred into one long plaintive plea. I knew better than to bargain with the Deity, but I did it anyway. If He would loosen the grip of neurological disaster, I would no longer doubt Him. There would not be a cell in my body that would question His hand on her life. From that moment on I would have absolute, unwavering confidence in His mercy. I would do a better job at everything, be a better parent, a better person. I lay on my side, looking across at Mathilda in agonizing self-examination. My mind cast all the way back to the many mistakes I had made, trying to rationalize which misdemeanors would add up to her being there. I knew that was not really how it worked, that I was not thinking along straight lines, but I could not help myself. Trawling through my childhood, teens and early adulthood, I realized that it was not so much that I had done something terribly wrong; I had simply asked for too much. Three healthy children. That was it. Mathilda, our third child, had tipped some kind of theological balance.
Down in the empty basement of the hospital at 2 a.m., I continued stroking her face until she was wheeled off past the markings on the floor strictly stating, “Beyond this point, no access to parents.” I found the one empty waiting area and sat on a black plastic chair, staring at cinder blocks and a shelf of beat-up children’s books. Nothing marked the minutes except the drum beat of a headache pounding in time with the shrill beeping sounds coming from the scanning machine working over Mathilda’s head a few rooms away. I pressed my thighs down firmly on my hands to stop them trembling. Stay with the facts. Stay with the facts until they tell you otherwise. The truth lay somewhere between the long small hours of the night, my own exhaustion, and the threat of losing her.
It was right then and there that I had the strong sense that the two of us were being dangled over a gaping abyss. It may have been a vision, although by then I was already deep into months of sleep deprivation; I could not be sure. Yet the image was crystal clear, and it would return to me in varying degrees over the months that followed.
During the forty or so minutes that Mathilda underwent the MRI, I could see myself, as if observing from a distance, holding onto a rope above with one hand and firmly gripping Mathilda’s forearm with the other. Below us was a black cavern, empty and threatening; high above us was a cliff top that was too far up to be reached. I was exposed, fighting a fear that shook me so violently I wondered if God Himself was pulling me, testing the grip of my hands. If I let go, we would both fall. I would lose it all. I would question everything I believed in—goodness, purpose, meaning. He was the hurricane that blew around me, dangling there that night, and He became the tempest that, over the next few years, would threaten our survival. I was alone and terrified.
In the isolation and bareness of that space, staring at the bleak uncertainties ahead, I stopped bargaining and spared myself the burden of trying to make sense of it all. And with Mathilda clinging to my arm, I made a calculated decision: To not let go.