The Upside of Down (28 page)

Darryl, Ellis and Oliver are driving in front of me in a truck we've borrowed from some generous friends. In fact, so generous that half of what's packed in it once belonged to them: furniture, crockery, pots and pans, even a juicer and fruit-drying machine. Darryl's brother Grant has also donated the remnants of his former Sydney apartment, including beds, blankets and two fabulous ocean kayaks. All are essentials for our thrilling new beach house venture—if we ever make it there, the way Darryl's driving that truck. Eventually, via a message through Ellis, I demand to drive in front. If they're going to end up in a ditch I refuse to watch it happen.

‘We're here! We're at our bach!' Oliver's running around flapping his arms as though trying to get airborne. Meanwhile, Ellis is still obsessed with the new toy, disappearing into the shadows a few houses away, radio pressed tightly to his lips.

‘Mum, can you still hear me now?' And, about five steps further, ‘What about now? Can you still hear me?' Unfortunately, Aidan is missing the excitement as he's in New Zealand visiting Raewyn for the week.

In our typically ambitious manner we've decided to christen our house late on a Friday night, leaving home about seven and arriving after nine o'clock. Before I have located the key in the dark and opened the front door we hit the first snag.

‘I've turned on the mains power and the gas for the stove, but I can't find the tap for the water,' Darryl reports. We search outside in the dark for thirty minutes before giving up. Lack of water for the nightly cup of tea is bad enough, but even worse is the unprecedented popularity of the now unflushable toilet. Then we discover problem number two.

‘Susan, where are the sheets and duvets?' Darryl calls out from the back of the truck.

‘Uh, the last time I saw them they were on the front porch.'

‘Here?'

‘No, at home. Didn't you pick them up?'

‘No. I thought you were dealing with that pile of stuff.'

‘At least we have plenty of mattresses,' calls out Oliver helpfully, scrambling over six single beds to pop his head out of the truck. Eventually we find two child-size duvets and a couple of towels. Ellis and Oliver burrow in close to each other on one mattress while Darryl and I do the same on another.

In the morning, our new neighbours kindly locate the water tap and some friends arrive to help us unpack. By the end of the weekend the boxes have been emptied, a triple bunk bed built from scratch and the beginnings of a cubby house organised.

Our bach is an old cottage, renovated a decade earlier to include huge windows and just enough space to pack six kids and four adults into its three bedrooms. It sits on a road paved with bricks which, in summer, will be lined with street cricket matches. The beach is long and softly curving with uninterrupted views south and only a three-minute walk from the house, even when cautiously picking one's way, barefoot.

This becomes our escape from the realities of day-to-day life. The most thorny decisions are between the surfboard and the boogie board, fishing rod or crab net and Speed Scrabble or ‘Scum' after dinner. The kids roam the dunes, build forts, wander shoeless in years-old ripped pants, blackberry stained hands and a permanent grin on their faces. We use the outdoor shower all year, delighted by the sheer pleasure of showering under the open sky, even in the rain.

Of course, some realities we can't afford to escape. Rather than trying to block CF from the bach, we make space for it, hoping to manage its demands rather than letting it manage us. We do physio and now nebulisers, knocking them out of the way early in the day, though not without the occasional foot-dragging. This time it's Oliver.

‘I don't understand why we have to do nebs every day now. I'm not even sick.'

The kids have recently started using a relatively new drug that is taken twice a day via a nebuliser for about fifteen minutes.

‘Because they help you get any gunk out of your lungs. If it stays in there it becomes infected. I know you're not sick, but it's to keep you from getting sick.'

The ‘new drug' is called hypertonic saline and is just like it sounds—super salty water. The ocean is about 3% salt but the kids inhale 6%. In fact, it was thanks to the sea that hypertonic was discovered as a treatment for CF. A respiratory physician in Sydney noticed that his patients who surfed reported feeling much better and more able to cough after a surf. Research found that adding salt into the CF lung actually increases the airway surface liquid, allowing the little hair-like organisms called cilia (whose job it is to sweep the lungs clean) to more easily clear mucus and other foreign bodies from the lungs.

This isn't the first time the ocean has been credited with helping people with CF. We recall Dr Cropp from California and his story of the crusty old sailor who lived into his seventies. Researchers have long pondered the potential benefits of a salty environment. We had hoped the sea would make a difference for our kids physically (in addition to what it might do for all of us mentally) and it does. Aidan's sinus problems, at times debilitating, clear up after several days of dunking and somersaults in the salty waves. Thick coughs seem to move on more quickly when helped by the ocean. We shop around for second-hand wetsuits for everyone, encouraging family swims even in midwinter. When the weather is good we plan family triathlons, ocean kayaking and snorkelling, tipping ourselves into bed each night, worn out but elated.

Within a very short time the bach has become our own healing place.

16

RUNNING MATTERS

‘What are you doing, Oliver?' I ask, watching him on his third trip out the back door in ten minutes, the fly-screen slamming noisily behind him each time.

‘Nothing.'

It is school holidays and until half an hour ago he was suffering from a shocking case of boredom. It was the kind of boredom that begins with a quiet whinny of ‘I'm bored', rapidly ascending to high-pitched wailing and despair as the tedium sets in. The problem is that Oliver devours books faster than I eat my breakfast and, sadly for him, he has finished all of his library books and the library isn't open until this afternoon. For thirty minutes or so he writhed on the couch, desperate for a book, a game, a playmate, anything to distract him from the blanket of nothingness smothering him. But then, several minutes later he abruptly jumped up and took off down the hall to his bedroom.

And now he is focused. Carrying several large hardback books and a microscope, he bumps the fly-screen open again, heading for the back shed. Delighted to have the moaning and gnashing of teeth left behind, I don't press him for answers. However, three hours later when he fails to appear for lunch, I investigate.

‘What's up, honey?' I ask, poking my head in through the door of the shed. Inside he has set up an entire miniature laboratory, complete with a rudimentary lab bench and homemade Petri dishes.

‘I'm working,' comes the reply.

‘Oh. Would you like to take a break from your work to have some lunch?'

‘Maybe. I guess so. I haven't made much progress yet.'

‘Oh, really?'

‘I'm trying to find the cure for CF, but I haven't done it yet.'

‘That's a great idea but it's a big challenge. At least you still have the afternoon, so you can keep trying.'

‘Uh, huh. I will.'

True to his word, Oliver not only works the rest of that afternoon, but he continues for at least two hours a day throughout the following week of the holidays. Several weeks later he finally decides to dismantle his lab, accepting defeat.

At times Darryl and I feel comfortable with the progress of the very smart people who are trying to find a cure. But at other times we find ourselves twitchy and fretful about the future, particularly since reading Gawande's article in
The New Yorker
. As Gawande pointed out, differences in quality of care can account for differences in life expectancy of more than fourteen years. That's a big chunk of living to miss out on because of being at the ‘wrong' hospital. And, at this point anyway, none of us know which hospital is the best place to be.

We decide it's time to become more proactive.

Over the years we have generally gone for the ‘more is better' approach to the kids' care. More questions, more suggestions, more ideas, more sputum samples, more educational conferences, more physio. The questions, suggestions and ideas have probably made us aggravating to our healthcare team, not to mention time-consuming. Our binders stuffed full of queries and quirky ideas have at least helped us stay involved in the kids' treatment decisions—essential for such a hands-on, everyday-demanding condition. However, as much as we respect our team in Melbourne and feel involved, it's becoming clear that the ‘more is better' participation on an individual level is not enough.

Working alone, we might keep Aidan and Oliver healthy for longer but we're never going to influence policies at our hospital—either the significant or the more trivial ones. For example, we know that coming regularly to CF clinic is critically important. But if the hospital parking lot is a disaster and finding a space requires a GPS, 13 laps of the parking lot and 28 minutes of grumbling, even the most committed families may be tempted to stay home and steam-clean the carpets on clinic day. Or what about appointment overload? Patients often have many appointments with specialists, and one-off tests: could these be lumped together on the same day? Not yet. Last year our family had seventeen extra visits to the hospital for these appointments. Couldn't patients and families help the hospital to find a better way of doing things?

It's becoming obvious that I can squawk and grouse until I'm pink all over and nothing will change, apart from my becoming increasingly unpopular at the hospital and developing high blood-pressure. I find myself reflecting on Dr Cropp's words to us in San Francisco so many years ago. ‘I have seen enormous changes in quality of care—most of them were pushed through by parents and adults with CF. Remember, you have the power to make a real difference.'

But we can't do it alone. One problem is that Darryl and I don't know anyone else with CF. Because of concerns about cross-infection between patients, there's very little contact between families. We are not a community; we're people who look vaguely familiar and smile nervously at one another across the waiting room at two-monthly clinics. This is clearly our first obstacle because, jeez, this boat's going nowhere if we don't even know the names of the people rowing in front of and behind us.

So we decide to start a monthly CF support group. I begin casually approaching other parents across the clinic, sidling up alongside them like a lonely heart in the local pub.

‘We're starting a kind of support and advocacy group if you'd like to come along … We'll probably discuss, uh whatever anyone wants to discuss and then maybe think about how we can improve things for people with CF … It's just me and my husband right now …' Darryl and I decide to make the get-together at a local cafe that we both like, thinking that if nobody else shows then at least we will have a pleasant little date night.

But, thank goodness, they do show.

Lisa, the conscientious occupational therapist, leans over her decaf latte describing her five-year-old son's unhelpfully low-fat eating habits. ‘No matter what we try he seems to end up eating apples. We ordered a pile of fish and chips with friends on the weekend and all the other kids were tucking in but he wanted fruit!' Despite the cajoling, her son isn't interested in food and is just dangling on to the bottom of the growth chart as a result.

Then the conversation turns to Sue. Her knowledge is far-reaching and her commitment to her 13-year-old son's health is impressive. But the family's clearly going through a tough patch. ‘We have tried everything this past year—every test, antibiotic, treatment change but his lung function just keeps on slipping.' She pauses, running her fingers up and down the side of the berry smoothie in front of her. ‘It's incredibly frustrating.' The eight others around the table nod in acknowledgement, her pain physically present around the table—it could be any of us tomorrow. Any of us.

Jon is sitting on the other side of Sue, alongside his partner Jane. He's a forty-year-old adult living with CF, who has for the first time had to drop to part-time work in finance because of becoming unwell. ‘I stuffed my knee playing soccer about six months ago. I needed surgery to repair it—'

‘And that's when everything changed,' interrupts Jane passionately. ‘It has been like entering a whole new world since then, one that is completely different. One day he was working, playing soccer, running, and we were going out at the weekends and taking holidays. Then, practically overnight—after Jon got really bad pneumonia following his knee surgery—everything that we had known started falling apart. And now it's hard to know where we're headed …' she says, leaving her thoughts hanging, like their future.

The next month others come. Each time the discussion is a little different depending on who is there, how everyone's feeling, who has been in the hospital. Slowly we begin to know names and learn each other's stories. We find ourselves sharing details of minor victories and biggish defeats. A different Lisa, another mum, explains what it felt like to learn on one day that both of her two beautiful young children have CF. We watch as she struggles not to burn out in caring for them. One couple is conflicted as their eldest son, now in his twenties, thrives despite his illness while their youngest loses ground.

After six months a community has begun. Sometimes we pass opinions about what ingredients make the best high-fat milkshake (cream, yoghurt, ice cream, fruit) or how to make homemade salt tablets, while other nights we discuss drug trials or new medical articles that someone has read. There are times when we encourage each other to persist despite the tediousness of daily nebuliser sterilisation—which, it must be said, drives me loopy. There are celebrations and there is shared pain.