‘With the Project. Stupid fingers are numb. I can’t sew. I can’t feel the needle. I can’t hold the fabric straight. Stupid. Stupid, stupid, stupid.’ She bites her lip.
‘Poop,’ I agree. She looks a bit startled. ‘Medical jargon,’ I assure her.
‘Yeah, right.’
‘What’s the plan then?’ I ask.
Silly question: I am the plan. Within seconds she has produced the workbag, scissors, pins, a tape measure. Sitting with me at the huge kitchen table, she directs while I pin and sew small squares of fabric together. She surveys, changes, tilts her head sideways while she considers, sucks in her cheeks, shakes her head, moves fabric squares. Intermittently our attention is diverted into the observations of pulse, blood pressure, temperature, and then we are back on task.
While we work she chats about her family, her music, her friends, being bald, body image, legacy. Legacy is such an unusual word for a teenager to use, yet she really means it. Her school has run fund-raising events for leukaemia research, mainly concerts to which Sylvie has contributed either in person as a drummer, or remotely by editing tapes that were sold at the events. These tapes will be in circulation after she is dead, and she finds the idea fascinating, compelling, sad yet consoling. Being bald is ‘a nuisance in the winter, brrrr!’, but on the other hand, ‘It’s quite a cool look for a girl on stage.’ Her body-image issue is ‘hamster cheeks’ induced by steroids. Her changed facial appearance continues to startle her–‘I share the bathroom mirror with a girl I don’t know.’
She moves back to legacy. ‘There’s two parts to that. There’s living on in my music, and that’s kinda easy to get. Like, other people have already done that, haven’t they? John Lennon, John Bonham, Keith Moon… They made that noise in real time, and it’s like they’re here now when I play those tracks. But the other bit of legacy’s harder. It’s so sad for Mum and Dad. Dad has his work, he stays busy. He’s keeping it out of his head. I’m quite like him, really–I use drumming to stay sane. Mum, though, she’s different. She’ll be tough, but it will be so hard for her, being on her own while Dad stays busy. We sit here by the stove in the evenings, Mum and I. Just snuggle down, a chair each, a cup of tea. Just chatting, or thinking. That’s when she’ll miss me most, I reckon.
‘That’s what made me think of a cushion for her rocking chair. It’s my way of saying, when I’m not here any more, “Come and sit on my knee, Mum.” And I can rock her, and she can feel my arms around her, as we rock together in front of the stove. It’s genius. I hope she’ll like it.’ I cannot look at her, and the sewing is becoming blurred. I concentrate on not dripping tears onto the fabric. These tears I gotta hide… needles and pins…
By the end of the transfusion the patchwork is assembled under her direction, and I have stabbed my own fingers multiple times. Pins and needles spike my fingertips as I gather my bags; they have less in them than when I arrived, but somehow they feel heavier. They are filled with admiration and awe for this almost-woman, this great-hearted human, who has lived and loved so fully in a lifetime cut short, whose cup is half full–no, in fact, so full it is running over.
Lullaby
Offering a palliative care service to strangers is an intellectually stimulating challenge, filled with job satisfaction. It’s very different to be walking a palliative care route alongside our own dear friends and family, especially when the disease is stripping away the delights of childhood from a much-loved baby. This story is about the wonderful resilience of families in the most heartbreaking of circumstances, and a legacy that preserves a beloved child’s name as a word associated with comfort and care.
It’s hard to believe what I’m hearing my friend say. The evening light is skimming the hedges outside the lounge window and shafting into my eyes as I stand beside the telephone. I am blinded by the brightness, and all my focus is on the words she is saying in a calm voice–deliberate, carefully rehearsed phrases, cautious care in her words, measuring her ability to communicate such dreadful news.
‘Do you understand what I’m saying?’ Lil keeps repeating, and I realise that I am failing to communicate the horror that is creeping through me.
She is a paediatrician. She understands baby and child development, and she notices tiny details the rest of us might not. Now on maternity leave with her gorgeous twin girls, she has been relishing watching them change and develop, step by step; interpreting their gurgles and chuckles, delighting in their pleasure as they discover their fingers, their toes, their voices; and sharing, within our little friendship triad, the joy of becoming a mum and of learning the ropes of parenthood with her husband.
But she noticed a tiny detail that I certainly would not have seen. One of the twins, Helena, had developed very fine muscular twitching in her tongue. In a bright, happy and beloved baby, this was a shocking sign to her knowledgeable mum: a muscle disease that is progressive, debilitating and fatal. By the time my friend was ready to tell us her terrible news, Helena’s diagnosis had been confirmed by a specialist. Spinal muscular atrophy (SMA), Type 1–the most rapidly progressive form of all. Helena was unlikely to reach her second birthday.
‘Do you understand what this means?’ Lil asks again, and I am nodding–not helpful on the phone–because I am utterly lost for words. This is cruel, and dreadful, and these are twins: a horrible thought shoots into my awareness just as she is saying, ‘We’re so thankful that they’re non-identical. Saskia doesn’t have the gene.’ I cannot fathom how they can be thankful for anything at all as they ponder what lies ahead.
We have been friends since medical school, Lil, Jane and me. Lil specialises in child protection, which sounds unbelievably sad and traumatic to me. She will find it harder now she is a mum. Jane is a children’s anaesthetist: she manages tiny people through huge operations, and often works alongside colleagues in a children’s intensive care unit, another unspeakably difficult and stressful job. Yet they think my choice to work in palliative care is equally challenging. So between the three of us, Jane points out to me on the phone when we talk the next day, we have all the knowledge that will be required for Helena’s best supportive care for her short lifetime. How ironic.
Jane is looking ahead. She knows the progress of SMA from her work in intensive care. As the child’s throat muscles fail, they become unable to swallow safely or to clear phlegm from their airway, so their lungs are prone to infection. This is compounded by progressive weakness of the muscles in their chest, so they cannot cough or take deep breaths. Simple colds can become overwhelming chest infections. Early on in the condition, admission to intensive care and temporary use of a ventilator to support their breathing enables them to return to live at home, while their muscle failure slowly progresses to reverse the milestones of rolling, sitting, crawling and standing that they have so proudly achieved. Further on in the condition, the child may be incapable of more than the slightest movement. Fed by a tube for safety, and requiring constant attention to remove saliva that they cannot swallow from their mouths, yet fully mentally aware and attached to their families, there may come a time when admission to ICU is simply prolonging dying rather than restoring health. They may no longer have the muscle strength to breathe without the ventilator. Many families cannot manage a ventilator at home, and the child is tethered to life by a ventilator and to hospital by the need for ventilator management. Jane has seen families unable to recognise when promoting living has segued into a purgatorial extension of dying without hope of improvement. She is horizon-scanning for problems–that’s why she is good at her job.
My small contribution is regular telephone conversations, using my CBT knowledge to help Lil separate out her realistic, sad thoughts about the future from catastrophic imaginings that turn every day into a minefield. Out and about with both girls in their twin buggy, she is used to a mixture of admiring glances and inane or intrusive questions like ‘Are they twins?’ ‘So they’re the same age, then?’ ‘Did you have IVF?’ Now, these same villagers appear to
her to be avoiding her, crossing the road as she approaches or hurrying away towards bus stop or car park. Her world is suddenly full of twin buggies–mums and grannies with healthy twins, normal life expectancy, ‘just taking it for granted’ and fuelling her bitter anger and despair. Much of this is simply her own reinterpretation of events. Helena doesn’t look unwell, and as yet there is no reason for any passer-by to suspect that there is anything out of the ordinary about the threesome as Lil strolls around the shops with the buggy or attends nursery singing with the girls. Unhappiness grows like a weed that takes root in imperceptible cracks in our psyche. Lil and I examine her experiences, identify the weeds, and she vigilantly uproots them as she feels her mood slipping.
The presence of a healthy twin means that some kind of balanced family life must go on. While they are small enough, each twin sits in a backpack when their parents take them on wilderness walks in the Pennines or their beloved Scotland. Initially at least, both achieve their motor milestones, Helena sometimes a little later than her sister, but within the bounds of normal development. Both love music, both love bathtime, both love each other. Like many twins, they can be content in each other’s company for long periods of time, chuckling at a shared joke or admiring each other’s hand movements, mesmerised by such a companionable soulmate. They ‘talk’ to each other for hours: Lil phones us so we can listen in and marvel at these deeply meaningful, utterly unintelligible conversations.
Helena’s first serious chest infection arises suddenly, and she is in hospital, in an isolation room with oxygen to support her breathing, very quickly. Her parents take turns to stay with her while the other tries to keep life as normal as possible for her sister. And yet, for Saskia, how can life be normal without Helena? Jane stays in closest touch over this period, her anaesthetist’s brain vigilant for signs that ventilation may be necessary, and Lil finds her contact comforting. I manage a flying visit, chatting to Helena through the transparent tent that maintains a higher oxygen level for her breathing, and joining Lil in a nursery-rhymes medley. Helena is panting and her ribs shiver with the effort, but she still manages to laugh at us. Priceless. She gets home a week later without the need for a ventilator. This time.
Aged two, the twins are beautiful and aware of it, reeling me in with smiles, and responding with a clear ‘No!’ to most maternal instructions. Ah, I smile to myself, the terrible twos have arrived. While the instruction-resistant behaviour is to be expected, what is unexpected is that both twins are still here. As if to illustrate their evolving disparity, Saskia runs across the room to where Helena lies on the sofa; she clambers onto the sofa (nice mantelshelf manoeuvre–you can tell her parents are mountaineers) and launches herself over the back, while Helena lies motionless apart from her eyes, which follow every move of her agile and entertaining sister.
Helena can now only continue to breathe because other people observe her constantly, ready to suck strands of mucus and dribbles of saliva from the back of her throat where they pool, gurglingly obstructing the flow of air in and out of her lungs. This can mean using a little suction device thirty times an hour; deeper suction with a miniature vacuum cleaner is needed at less frequent intervals. She tolerates this intrusion, which she has no power to resist, with remarkable equanimity, occasionally furrowing her eyebrows and flaring her nostrils in protest, but smiling again as the tube is withdrawn from her mouth or nose.
The exuberant development of new skills and tricks by one twin mirrors her sister’s inexorable decline. The depth of Helena’s loss is illustrated by Saskia’s motor agility and emerging speech. And yet, Helena smiles and gazes, and enquires, and flirts, using every piece of her might to be part of the quadrangle of love between twins and parents. Her parents contemplate the expected death of this indefatigable daughter, and wonder at her daring and strength to have so outlived all expectations, every day a precious, fragile burden. Sleepless and exhausted, carried by willpower and dread of her distress, how much longer can they dare to hope to have her? How much longer can they bear to hope? I watch my weary, courageous friend with awe, while Helena bathes her family in the grace of her happy presence for a little while longer.
The phone rings around lunchtime while I am writing teaching materials at home. It is Lil. Is she crying? My heart lurches. No, she is laughing. She can barely speak. ‘I’m calling on behalf of Heli, who has something important to tell you. Here she is…’
I can hear noisy breaths. Helena’s facial weakness makes her speech hard to decipher, but what I hear is ‘Ka? Ka?’
It’s my name. ‘Hello, Heli, here I am.’
‘Ka! Monna nor-ee tep!’ Giggle. Fast breathing. Giggle. Lil provides translation–I’m on the naughty step.
‘Goodness! On the naughty step! What have you been up to?’ I ask, intrigued that she is capable of such wickedness, and delighted at her delight at her own mischief.
‘Ontid orter!’ (Wanted water) she trills merrily.
I can hear Lil laughing, then she says, ‘No, don’t look at me, I’m only here to hold you. This is the naughty step. No chatting with Mummy for two whole minutes!’ Heli giggles again.
‘Kath, this little lady is supposed to be having her nap while Saskia is asleep and Mummy does some work. But she wants to play with water and bubbles. She keeps shouting from the sofa across to me. And she has been asked to wait. She has been warned to give Mummy ten minutes’ peace. She has been warned that one more shout and she will have to sit on the naughty step. So here she is!’ I hear the sound of the saliva sucker in use, along with more toddler chuckling.
Heli comes back on the line. ‘Nor-ee tep!’ she splutters. ‘Like Ash-ya!’ Naughty step! Like Saskia!
‘Yes, Saskia is a regular attender here,’ laughs Lil. ‘But this is Heli’s first ever time, and she’s so pleased with herself.’
Two hilarious minutes are up, and Lil carries Helena back to the sofa to bask in her triumph. I assume the phone is tucked under Lil’s chin; no wonder she is getting a bad back. She sucks more saliva out of Heli’s mouth while telling me how wide Heli’s eyes are with smiling–now more indicative than her mouth. We say goodbye for now. As Lil rings off, I hear Heli demanding, ‘Orter.’
My dear friend. Keeping it ‘normal’. Disciplining her daughter, because that’s what normal looks like. The love between them almost palpable as they share this precious moment. I am so delighted that they shared it with me.
The family were active in seeking advice to help Helena to remain as well as possible. They were disappointed to discover that, although palliative care services for children with cancer were fairly well developed, there was almost no provision for children with other lethal diseases. Lil’s ruthless exclusion from the house of anyone with a cough, a cold or a sneezing child protected Helena from chest infections, and by networking, online research and sheer determination, she and her husband found specialists to help reduce Helena’s symptoms. They visited a clinic in Scotland, where Botox injections into her salivary glands reduced Helena’s saliva, drooling and need for suction; they found physiotherapy experts who advised them about optimising her muscle function and keeping her chest clear; they assembled a trusted team to sit and watch over her whenever they could not be present, enabling some snatched hours of sleep at night knowing that her saliva would be suctioned and her posture changed regularly to keep her comfortable. Her survival now demanded constant supervision to ensure saliva did not block her airway.
The twins enjoyed three birthdays together. This was a stunning survival time for a child with Type 1 SMA. They spent family holidays in Scotland, went hill walking, and Helena’s chairs and bed were adapted to enable her to sit and participate as fully as possible in family life. Helena focused her keen intelligence on a laptop computer (a novelty at that time), and used it to create colourful computer-generated animations to accompany her favourite music.
Although Helena’s horizons were shrinking, her three-year-old quality of life was lovingly maintained by her family,
who had helped her to outlive her original two-year prognosis and who continued to find inventive ways to enhance her life. When Saskia began to attend nursery, coming home with new stories about friends unknown to Helena, Lil was able to have one-to-one time with Heli so that she too would have news to tell Saskia when they were reunited. The girls were bridesmaids at their cousin’s wedding, clad in ‘princess dresses’ and very pleased with themselves, Helena in her chair and Saskia firmly on guard beside her. They were particularly proud of their silk ballet slippers: Saskia’s were quickly worn ragged, while her sister’s remained poignantly pristine.
Jane was very much afraid that Helena’s next chest infection would only be survivable by using a ventilator, and that her chest function was now too poor to escape the ventilator afterwards. She expressed concern that Lil and her husband would find it too hard to turn down the chance to extend Helena’s days by any means. But not knowing how to broach this discussion, we simply waited and wondered, marvelling at Helena’s life of utter bodily stillness and intense mental activity.
And then Helena became hot and breathless. The diagnosis of a chest infection was apparent to her mum immediately, and a decision needed to be made. Heli loved being at home, surrounded by her family and her familiar team, her own bedroom and her beloved toys, and she disliked the noise and unfamiliarity of hospital. So it was decided that she would stay at home, with fans and cool sponging, drugs to relieve breathlessness, extra oxygen as needed, and no ventilation. This meant that she could be cuddled by the family, and feel them close. She was unlikely to survive, but her parents’ love and forethought, as in all the other aspects of her short life, had prepared them for this decision.
We need not have worried. As Heli became drowsier and less aware, Lil rang Jane. Jane rang me. We wept and we waited.
With the End in Mind Page 25
