The Upside of Down (9 page)

‘Physio is the key' the hospital physiotherapist reminds us repeatedly, ‘which helps keep the destructive cycle at bay.'

So, I normally lay Aidan across my lap while I do the clapping, often with a book, toy or the TV to distract him—a mix of bribery and coercion to get through the twice daily ritual. Darryl is generally more patient about this than I am. When I'm in charge the process usually begins with something cheery and upbeat like: ‘If we finish physio quickly we can go to the park.' But by the ten-minute mark I'm frustrated, clenching my teeth and about to threaten my young son with: ‘If you don't stop wriggling you'll never see the park again.'

The women in my mothers group stress about the need to brush their toddlers' dainty new teeth, but that's just not on my radar.

In fact, my radar is irregular. At times it's cluttered with all sorts of panicky chatter—coughs, viruses, tummy troubles—but other times it stares blankly back at me as I coast happily along like any other mother in any other family, loving my son, content in my life. Aidan is generally so well that at times it's tempting to think that the whole thing is just a case of mistaken lab results.

One obvious change in my life, though, is that even when he's a hundred per cent healthy we are still enmeshed in the healthcare system. Aidan has been adopted by a foreign group of acquaintances: the GPs, respiratory physicians, gastroenterologists, dieticians, pharmacists, physiotherapists, social workers and appointment-takers. It's like having twenty new names show up in your address book but you can't put a face to any of them.

Though we are managing to keep Aidan out of the hospital, we are back regularly to see his main doctor. Archie, the paediatric respiratory physician who keeps watch over my son's tender breathing equipment, is soft-spoken and helpful, in his early forties, with a slightly scruffy beard, gently receding hairline and quick smile. Darryl and I regularly roll out a string of questions to which he replies willingly and with plenty of detail.

‘Although the lungs are generally the biggest worry, the destruction of the pancreas begins at a very early age. In fact, when the condition was first identified in the 1940s children died in infancy or early childhood, normally of malnutrition. Through autopsies it was discovered that their pancreas was full of fibrous cysts—hence the origin of the name—which kept the body from excreting enzymes.'

Looking at Aidan's bulky frame it's hard to remember how emaciated he was in the early days. On this particular visit to Archie, Aidan runs back and forth between my lap and the corner of the office where a few old toys congregate in a large plastic box. He's carrying them over to me one by one, holding out each object as he tries to identify it. His vocabulary is still limited; he gets ‘ball' and ‘car' right but then fixates on the two words and everything else gets one of those labels.

‘Do all your patients take enzymes?' I ask.

‘Nearly. About 95% of patients are pancreatic insufficient and thus unable to digest their food without taking enzymes when they eat.' For Aidan, missing enzymes can translate into stomach aches.

After completing his transfer of all the toys to our side of the office Aidan, still restless, toddles over to the spare chair and climbs on it, smooshing his face against the nearby window. I try to concentrate on the discussion, difficult without Darryl here today to help harness our toddler.

‘So, since his weight is now pretty steadily in the 90
^th
percentile, does that mean the enzymes are working just like his natural ones might have?'

‘Very close anyway. He still won't adequately digest all of the food he eats, either because he takes the enzymes at the wrong time or not enough of them or because they're just not a perfect substitute. But the development of more effective enzymes has been critical for improving people's wellbeing by allowing them to achieve and maintain a normal weight, nearly impossible even twenty years ago.'

With the conversation dragging on, Aidan's attention has shifted from the window and he's now devoting his energy to climbing higher. I pluck him off the top of the chair, pull him into my lap and go digging in my bag for snacks. Rather than raisins and apples, his morning tea includes a small pot of full-cream yoghurt and little chocolate biscuits. Noticing Aidan's snacks prompts Archie to shift our conversation, to the unusual—high fat, high salt—diet Aidan has been prescribed.

‘How are you going with Aidan's food?'

‘It's an adjustment for sure. We never thought we'd be encouraging our toddler to eat fish and chips.'

‘Most families find it a challenge initially. But both the extra calories and the salt are really important, particularly salt in the summer.'

‘You mean the two days a year that the weather is hot in Wellington?'

‘Yeah, okay, so the heat's not a big issue here, but he still needs extra salt because he loses five times more salt than normal through his sweat.'

That explains why his forehead tastes so salty when I kiss him on a hot day.

‘What does Aidan drink, apart from milk?'

‘Water or maybe juice.'

‘It's probably preferable to give him something with calories rather than water.'

‘No water? That requires a bit of mental modification. And is it really true that white bread is better for him?'

‘Although I wouldn't make a big deal of it, yes, it's probably preferable.'

‘Seriously?'

‘Yes. He doesn't need so much fibre right now. And white bread is less filling than whole wheat and he will require plenty of food.'

‘How much is plenty?'

‘Around 150% of the average recommended fat and calorie intake.'

I toss out my low-fat cookbooks and march through the grocery store, stocking up on full-cream everything and searching out high fat and high calorie snacks, determined to ignore the peering eyes who judge my grocery cart. My expectations of motherhood, and those of others, are meaningless in the face of reality.

***

When the wind settles and the sun finally shines in Wellington, it shines big. It's as if the elements want to apologise for all of the rough and blustery weather they heap upon this small city. As the nasty weather retreats, it ushers in the magnificent.

This magnificence is upon us one winter day when Darryl suggests a trip to the gliding field where Aidan was almost born. Despite the great weather, my own personal depressive low pressure system—that has been showing up somewhat regularly over the past year—is currently camped directly on top of me. Nothing is worth doing once this arrives.

‘I'm sorry, but I'm really not interested in sitting on the ground, taking care of Aidan and watching you fly all day.' Our tug-of-war over free time and hobbies (what Darryl prefers to call ‘interests') has multiplied in intensity since Aidan's arrival, giving us fertile ground for marital disagreements.

‘It's a beautiful day. We'll make a nice picnic. Come on. You'll feel better for getting out in the country.'

Grudgingly, I agree. We pack up and retrace our path along the winding Rimutaka road. The route is much more relaxing today with the sun shining and no baby begging to be born. We haven't been at the field long when an empty spot pops up unexpectedly in the back seat of a glider. Roger, the pilot, offers to take me up with him.

‘What do you think—should I go?' I ask, turning to Darryl.

Without hesitation he reaches over and takes Aidan from my arms. ‘Absolutely. You've got to do it.' As I walk away I wonder about the wisdom of this, throwing a worried glance back at my little family. ‘He'll be fine, don't worry,' Darryl calls out after me. But it's not Aidan I'm worried about, it's me.

Roger is a friendly fellow who I know from previous visits; he's probably in his late seventies and has been flying for decades. In that typically understated New Zealand way, the runway is actually a very long pasture with the gliders winch-launched rather than towed into the air. The winch is an odd contraption with an enormous engine and a thick steel cable about a kilometre long. Once the cable is attached to the glider, the engine pulls at about ninety kilometres per hour. The glider climbs like a kite. Pinned to the seat, it's how I imagine taking-off in a rocket might feel. All you can see is sky as the ground falls away behind.

We are halfway into our launch—at about five hundred feet—when there's a ferocious bang. I cry out in fear. Roger responds with an act of utter lunacy, thrusting the nose of the glider directly at the ground.

‘What are you doing? We're going to crash! You can't do that.'

I stop my hollering momentarily to shove my stomach back down where it belongs, though I know it won't stay there for long. I press my hands frantically against the sides of the glider in a desperate attempt to halt our decline. It feels as though we're nearly vertical and heading straight for the ground.

I begin yelling at Roger again. Later I learn that, in his own fright, he had accidentally been holding the mike open for everyone on the field to overhear my screaming.

‘We're going in too steep. Can you see the ground? Because, oh no, it's right there!'

Then I remember Aidan.

In that split-second I know I cannot die and leave my son behind. Despite the emotional upheaval brought on by my little bundle of boy, at this moment I can't imagine any better reason to stay alive.

Thankfully for Roger I'm sufficiently distracted with my worries about my motherless son that I barely notice as he puts the glider down safely at the top end of the field, stopping just a few feet short of the boundary fence. The ensuing explanation clears my pilot of either suicidal tendencies or dementia. We had suffered a break in the launch cable at the worst possible point in the launch—midway—when we were too low to fly on and nearly too high to be able to land safely on the field.

For the rest of the day I wander the field snuggling Aidan firmly against my breast and gripping Darryl's hand, smiling and giddy, chatty and warm, as though I'm in a drug-affected happy place. It's fascinating how a brush with death sweeps the mind clear of one's gripes, replacing it with intense gratitude for life.

***

Our GP, David, continues to provide steady ground for me, listening and facilitating help in practical ways. It takes a conscious effort not to cling to him like he's some kind of lifeboat. I'm beginning to understand why doctors can develop ego problems.

Shortly after Aidan was diagnosed I had been sitting in one of the consulting rooms at David's clinic when he mentioned his only other patient with CF.

‘She's a wonderful woman, very bubbly and upbeat.'

‘How old is she?'

‘Twenty-eight. Married. A really amazing person. You need to meet her.'

‘How is she doing … with her health? Is she okay?' I ventured the question hesitantly, not sure if I really wanted to know the answer.

‘She's getting by, though she takes a list of drugs the length of your arm.'

Several times in that first year David had encouraged me to meet her. Once, when I referred to a medical conference I had recently attended, he commented that I would learn more from talking to her than going to ten conferences. And yet, I couldn't bring myself to do it. Maybe it was fear of encountering this illness face-to-face after it had done its damage. Or possibly I worried that hearing her real-life experiences would make my optimism unsustainable. And given my growing attachment to my soothing friend ‘optimism', I wasn't sure I could cope without it. Until I laid eyes on her, the statistics about life expectancy were just numbers, numbers that we were intending to defeat. It was too soon to personalise those statistics.

Then one day when Aidan's about eighteen months old I make an appointment with David for some trivial ailment. He is subdued. After he addresses my medical concern he sits down in the chair opposite me and begins to write a prescription.

‘Is everything okay?' I ask him.

Looking up, he pauses before speaking. ‘My other patient with CF, the one I have told you about, died last week. I went to her funeral yesterday.'

I am stunned and say nothing.

He continues. ‘She had such a great sense of humour. Her husband spoke at the funeral, saying that she had left specific instructions with him about which of her friends he could and couldn't date once she was gone …'

For David and the family her death, though devastating, was probably somewhat expected. For me, it's like being sideswiped without warning by oncoming traffic. This woman I have never met, whose life I only ever experienced second-hand, yet whose illness my son shares. It's a reminder that while we're trying to live our lives as normally as possible, time is marching on towards some predetermined life expectancy figure, which this young and vibrant woman never even reached.

Her death raises a question which is becoming more significant in my mind: How do we live optimistically with a potentially fatal condition?

In entering this new world, we are tossed in with the many other families whose lives have also been changed unexpectedly. I quickly discover that not all of them have chosen, or been able to choose, optimism. During these few years Darryl and I have attended several CF conferences. It has become clear that conferences are crucial opportunities for learning. But they also attract a ragtag collection of people at varying stages of fighting their battle, from those with newly diagnosed babies to adults with end-stage lung disease awaiting transplant.

One mother of a twenty-two-year-old son, noting our positive outlook, informs us that she also had been confident when her son was born, but she ‘had since learned that it was futile … and we would too.' A retired nurse who has been in the business for thirty years—and probably watched many people succumb to the condition—warns us against ‘getting our hopes up too high'.