31 Days of Cystic Fibrosis: Part 5 May 21st – 25th

May is Cystic Fibrosis awareness month, and this year I’m doing 31 Days of on Facebook and Instagram. Please feel free to follow me.

May 21st – May 25th

31 Days of Cystic Fibrosis. May 21
Setting Off on the Road to Acceptance

I went home from hospital with an oxygen concentrator and a referral to the transplant team at Royal Perth. At that moment, an official sleep study hadn’t been done to meet the requirements for state-provided oxygen therapy, so the hospital provided me with a concentrator so I could continue to sleep with supplemental oxygen.

Although I had worked hard to maintain my health before, I never worked harder than I did in the second half of 2010. Because with time and effort I had ALWAYS bounced back and this time would be no different. After work I walked around the block, initially taking a stroll with Monkey. Monkey was 16 and her health failing, all too soon I had to walk alone because even my slow pace was too fast for her.

I did mini bouts of physio up to six times a day and nebulisers galore, trying to clear the gunk that was clogging my lungs. This extra airway clearance seemed to be working, as my sputum production decreased, but still, my lungs could not breathe.

All the while, I continued working, attending clinics at both Charlies and Royal Perth as well as transplant workup appointments.

Bonus Fact: The transplant team at Royal Perth (now Fiona Stanley) has the official name of Advanced Lung Disease Clinic. I like to joke that this meant that the team themselves were ‘advanced’ in treating Lung Disease, because the alternative wasn’t worth contemplating.

31 Days of Cystic Fibrosis. May 22
Koh Samui

In the midst of my transplant workup, Grant and I decided to take a quick
trip to Koh Samui. Once I was officially listed for transplant, I would be limited to the Perth Metro area, so this was a last chance getaway.

It turned out to be the trip I needed to prepare myself emotionally for transplant. Koh Samui experienced heavy flooding right before our arrival, and it meant there was only one street we could walk. I’ve often joked that it was lucky because I was only capable of walking one street. But it’s a truth that hit close to home.

Out of my own environment, it was an eye-opener to realise how much I was struggling to do everyday tasks. Then, on Grant’s 40th birthday, we walking up Chaweng Beach heading for a restaurant to have lunch and not only did I have to stop and rest regularly, but I was also overtaken by two elderly people with walking sticks.

It was the moment that I realised I was out of options. This wasn’t a nasty infection that had lingered way too long. This was my body losing the war against Cystic Fibrosis.

Bonus Fact: I returned from Koh Samui emotionally ready to get listed for transplant. It was fortunate timing, my workup tests for transplant had revealed why my lungs had declined so dramatically and also that recovery was out of the question. I had a chronic case of silent aspiration, meaning stomach acid entering my lungs was the cause of the inflammation that was making it so hard for me to breathe. Both the transplant team and Uncle Gerry agreed, I may survive the actual surgery to correct the silent aspiration, but my chances of making it through the first three weeks post op, sat squarely on 0%.

Extra Bonus Fact: I returned to Chaweng Beach in April 2017, and repeated that walk with new lungs. This time I didn’t need to stop and rest, and I talked while I walked. Simple things have far more meaning post-transplant.

31 Days of Cystic Fibrosis. May 23
Working Towards Transplant

An official placement on the transplant list also meant the acquisition of a shiny new pager that I had to carry everywhere. Working days were a blur of treatments, followed by work, dropping Jarryn at Soccer practice, home for treatments, then back to collect Jarryn before bed. There wasn’t time for anything else.

My hair grew progressively shorter. I struggled to breathe in the shower, and shorter hair was easier to care for. In amongst my daily work routines, I tried to write detailed instructions for my replacement, but these were limited to policy, procedures and standards because I didn’t know how long I could keep working, or when a call might come.

As 2010 came to an end, I started using oxygen full-time. It meant that I was officially smack bang in the middle of Respiratory Failure Stage 1. I wanted to keep working as long as possible because I knew that being active would be a key component if I was to survive. Despite my willpower, in 2011 after only being back at school for just over a week I ended up in hospital.

My stint in Respiratory Failure Stage 1 was short. At night, supplemental oxygen was no longer enough as my lungs could no longer rid themselves of carbon monoxide. I started to sleep on BiPap. Clinically my diagnosis was Respiratory Failure Stage 2, and my medical team told me that if I wanted, I could stay in hospital while I waited for the call.

Bonus Fact: During this time Jarryn had to make a decision. Did he want to stay here in Perth with me, or move to Sydney to live with his Dad? A word from me would have seen him stay. Perhaps a bit of my mother’s parenting rubbed off on me because I reminded him that this was my disease and it was not his job to be my carer. There was nothing he could do to change the outcome, and an offer of donated lungs was not dependant on his physical location. Maybe, just maybe there was more than a touch of selfishness in my words. I knew how this was going to end if an offer for lungs did not eventuate in time, and it wasn’t going to be pretty. I didn’t want Jarryn to have to watch me die.

31 Days of Cystic Fibrosis. May 24
Staying at Home

After four weeks in hospital, I went home. Physically, it would have been easier to stay in hospital, but I knew if I didn’t keep challenging myself, I would go downhill quicker.

The days merged into weeks, then months as I waited for the call that would change everything. I spent my mornings in bed, Bi-pap assisting me to breathe, as I read with Monkey at my side. At mid-day, I’d switch over to the oxygen concentrator with its 20-meter cord, and we’d slowly make our way to the kitchen for brunch. Next, Monkey would stretch out on the bathroom tiles while I showered. After that, we’d head to the lounge-room. Either we’d share the couch, or she’d lay on the floor beside me. This routine was punctuated by attending various medical appointments and visits from Dale, my Cystic Fibrosis WA homecare worker. Each day was physically harder than the last until it was no longer safe for me to drive any distance and my Mum had to chauffeur me to my clinic appointments and the gym.

Bonus Fact: Although the technical term for Bi-Pap is non-invasive ventilation, Monkey found this treatment rather invasive. Even under the best of circumstances, she didn’t like me to breathe near her face. Now, with my ragged breathing sounding not dissimilar to Darth Vader with a head cold, she’d found something she could not tolerate. But it was her duty to ‘look after’ me and she refused to let this get in her way. Her solution, as I lay in bed reading was to snuggle her backside under my armpit, so the steady stream of air emitting from the Bi-Pap mask was well away from her face.

31 Days of Cystic Fibrosis. May 25 (It’s 65 Roses Day today!)
The Mystery of the Sixty-Five Roses

In my days as Library Manager for the Swan Education Resource Centre, inclusivity became a topical buzzword. As I gathered books and other resources for a special section to house together, it quickly became apparent that Cystic Fibrosis was woefully underrepresented in the collection. Next, I searched high and low for books that both explained CF and could be used in an education setting that were available to purchase. Resources were either outdated or Americanised, So I decided to write my own.

The obvious organisation to approach was CFWA. Despite the fact that CFWA wasn’t a publishing house and that I was an unknown and unproven writer who didn’t have an idea let alone a single word written on paper, I walked away from a meeting with an agreement that CFWA would publish my book. It was a journey that would take six years and a new set of lungs to complete.

In May 2011 I didn’t know if I’d live long enough to see this book in print, but I knew that if I died, the project was advanced enough to continue without me.

Bonus Fact: The dedication in The Mystery of the Sixty-Five Roses reads as follows: “Dedicated to Dr Gerard Ryan, MB BS, RRACP (Uncle Gerry) and Sue Morey, OAM, FRCNA (Aunty Sue) for over twenty years of outstanding health care, even when faced with a (sometimes) difficult patient.”

I know they’d both say they were just doing their job, but when “just doing your job” means the difference between people living and dying, a book dedication does not even come close to an adequate way to say thank you.

 

31 Days of Cystic Fibrosis: Part 6 May 26th – 31st

31 Days of Cystic Fibrosis: Part 4 May 16th – 20th

May is Cystic Fibrosis awareness month, and this year I’m doing 31 Days of on Facebook and Instagram. Please feel free to follow me.

May 16th – May 20th

31 Days of Cystic Fibrosis. May 16
Hemoptysis

Years ago, I had watched Julie cough mouthfuls of blood and casually spit into the sink as if it was no big deal. So when I first saw bloody streaks in my sputum, I wasn’t overly concerned. At times there was more than streaks, and sometimes those episodes lingered longer than I would have liked.

One night I went to bed feeling fine. I woke from a deep sleep in a coughing fit. In my dream, someone was spraying the back of my throat with a water pistol (everyone else has strange dreams too, right?). Eventually, I woke up enough to realise that there was no water pistol and that I had become a bleeder.

In the morning, I took Jarryn to school and drove myself to the hospital. Once there it appeared I was out of options and HITH was off the table. Hospitals are very particular about measurements, preferring the use of plastic cups over tissues. My first bleed was over a thirty hours period, and included six separate incidents. Luckily my Mum took care of Jarryn while my fabulous neighbours, Wim & Lisa looked after my dogs.

I was a bleeder for the next three years. When awake, the commencement of a bleed was easy to identify. There is a particular sensation, hard to describe, but a hybrid feeling that manages to both push and pull at the same time. After that, you can both feel and hear the crackle of blood pooling in your lungs. So I call it, ‘snap, crackle and pop’, It would strike without warning, seemingly when I was feeling fine, however, an infection had been bubbling away in the background. Once, Jarryn had to pass me tissues to spit into after ‘snap, crackle and pop’ struck while I was driving.

Three years after my bleeds started, they stopped.

Bonus Fact: As it turns out, it’s possible to cough up over 800ml of blood in thirty hours, and like the energiser bunny, keep on going.

31 Days of Cystic Fibrosis. May 17
Travelling with CF

My first bleed was a trigger to change some things in my life. A reminder that the reasonably good health I had enjoyed up to this point would not continue forever. It was during this period I had my vision corrected with laser surgery, I began to write ‘The Mystery of the Sixty-Five Roses’, and I started to travel overseas.

Although I’d love to be able to be a spontaneous traveller and go on a holiday at the drop of a hat, as with everything else when it comes to CF, travelling requires careful planning. Letters from my doctor need to be organised (The first contains a list of all the medications I’m carrying for customs, and the second details my medical history for any doctors that may need to treat me while I am overseas). In addition to my regular medications, I always carried strong oral antibiotics and tranexamic acid (used to stop excessive bleeding). My small pharmacy always travelled with me as carry on luggage.

Between 2005 and 2008 I visited Thailand twice, Bali and Singapore. The difference in environment and pollution levels always saw my lungs struggle for the first few days as they adjusted. Ventolin was my best friend on these trips.

Bonus Fact: Travel Insurance companies have strange ideas regarding CF and travel. They place a lot of weight on hospitalisations or the administration of IV antibiotics in the last 12 months. A tick in this box is highly likely to result in a rejection for cover of CF as a pre-existing condition. Ironically, right after a course of IV antibiotics is when a person with CF is most fit to travel.

 

31 Days of Cystic Fibrosis. May 18
2009. The Year of Swine Flu

I started 2009 with a lung function that was 75%. During winter I dropped to 30%, and by the end of the year, I managed to raise it to 45%. (Predicted lung function is determined by your height and age and helps your medical team assess what percentage of your lungs are working.)

Despite having had the flu vaccination, I contracted the flu three separate times that winter. One on top of the other, with no break in between. They gave my lungs a beating.

I spent nearly the entire winter on IV antibiotics and ended up spending two weeks as an inpatient so that the team could do additional testing. Nothing conclusive came of these tests, although my CAT scan showed significant inflammation in my lungs. As there were no additional areas with scarring to indicate the damage to my lungs was permanent, this was good news. Both my team and I chalked it up as a bad winter that with time and effort I should be able to recover from.

In the November we took a family trip to Malaysia, this time I found it more difficult to cope with the air quality, but I still managed to enjoy the holiday.

Bonus Fact: The Swine Flu was rampant that winter, but as it turned out, none of the three flus I contracted turned out to be the Swine Flu.

Extra Bonus Fact: This is the holiday that Grant and I remember as the one that Jarryn bitched and moaned the whole way through. Apparently, it was hot, there was strange food and he had to walk everywhere (truly tragic stuff!) Jarryn, however, recalls this trip ‘fondly’.

 

31 Days of Cystic Fibrosis. May 19
Egypt

I kicked off 2010 with a holiday in Egypt. It was something that had been on my bucket list since I had been little. Given my scare the previous year, Grant had decided it was time to make this a reality instead of an item on a wish list.
I was determined to go everywhere and do everything that was ‘allowed’ and since climbing the outside of the pyramid was off limits; I joined the queue to use the Robber’s Tunnel and to climb inside the Great Pyramid of Giza to see the King’s Chamber. The tunnel was small, hot and crowded. The air quality was virtually nonexistent, making that climb was hard, but there was no way I was giving up. I couldn’t catch my breath the entire way and had to stop to try to gasp more air into my lungs every few metres. But I made it.

My second challenge was the at the Valley of the Kings, once again hot and crowded, and as most of the tombs had fans whirling to increase the air circulation, it was also dusty. Not being prepared for all the dust whirling around in an enclosed space, I had to explore the tombs with my nose and mouth covered, making it even harder to breathe.

Bonus Fact: It took me a few minutes a regain my breath when I finally reached the King’s Chamber in the Great Pyramid. When I wandered over to have a look in the sarcophagus, a man who had been laying down inside it unexpectedly popped up and yelled “Boo”, making me jump, which in turn, made all his friends laugh.

 

 

31 Days of Cystic Fibrosis. May 20
The chat you never want to have with your doctor

After my Egypt trip, I managed to raise my lung function to 50% and then winter arrived, bringing with it another bout of infections. The hard work I had done was wiped away, and my lung function dropped right back to 30%. This time HITH didn’t work at all, and after four weeks, I was admitted to Charlies. I spend the first few days on oxygen, then only required oxygen at night as I slept. For the first time, the team got me to try prednisone which proved extremely effective in reducing the inflammation in my lungs. My veins would no longer cope with a PICC line, so it was time for a more permanent solution and get a port-a-cath inserted near my left clavicle.

Nearly the whole admission past without me seeing my specialist (affectionately known as Uncle Gerry). I knew he’d been making the decisions for my treatment plan, but I hadn’t laid eyes on him myself. I joked with the staff that perhaps I should head on down to the clinic and make an appointment time.

The day before I was discharged, ward rounds came and went as usual, then that afternoon Uncle Gerry popped in unexpectedly to have a chat with me. It was, he said, the chat he never thought he would have to have with me. He thought he’d retire before I reached this point. And my heart stopped beating for a moment. Because it was the chat you never want to have with your doctor. The one where he tells you that there’s nothing more he can do. There are no other treatment options to try. That’s it time to make a decision and be referred to the transplant team.

Bonus Fact: When Jarryn was two there were rumours going around clinic that Uncle Gerry was about to retire. I asked him if they were true, and he replied he hadn’t decided yet. So I said, “I’ll make you a deal. If you promise not to retire, I promise not to die on your watch.” He just laughed at me and didn’t commit to anything.
Six weeks after my transplant I went back to say goodbye to my old medical team, I was planning to remind Uncle Gerry that I’d kept my promise (by the skin of my teeth perhaps) and hadn’t died on his watch. But he beat me to it. The first thing he asked was, “Can I retire now?”

31 Days of Cystic Fibrosis: Part 5 May 21st – 25th

31 Days of Cystic Fibrosis: Part 3 May 11th – 15th

May is Cystic Fibrosis awareness month, and this year I’m doing 31 Days of on Facebook and Instagram. Please feel free to follow me.

May 11th – May 15th

31 Days of Cystic Fibrosis. May 11
Julie

Sometimes, best friends have no qualifying factor. I can’t tell you why Julie was my best friend. Only that she was.

We grew up going to CF camp together, attending clinic together and in between times we wrote to letters one another. Letters filled with oh-so-important growing up stuff. Even as housemates, we continued to write to one another although we’d graduated to exercise books by this time. Often we’d sit at the same table, pens frantically writing away. I once wrote six pages on how she rejected the bread, and in return, I received four pages complaining about my six pages.

We went on stealth missions together; we danced, we painted our faces like Kiss to pick up pizza, there was the mysterious case of the higha-woofas, urtundidi, the occasional “There’s not something wrong with the hammer” and drawings. And so, so much laughter (which always ended in coughing fits).

Through everything, Julie always called me Bird or Birdie. Biiirrrrdiiiie when she was especially chipper. I wanted her to know that I’d carry the nickname she had given me forever, so I had a ladybird tattooed on my stomach a few weeks before she died.

Julie dealt with everything that CF threw at her with grace and a dignity that was well beyond her years. Of all the friends that CF has taken from me, the loss of Julie cuts the deepest.

Bonus Fact: I once went to bed and left Julie in charge of a party.
Bad, bad, bad move.
When I woke the next day, all our garden furniture was on the roof.

31 Days of Cystic Fibrosis. May 12
Growing Up When Your Mum has CF

Having a baby, I was told, is hard. As time passed it would be inevitable that my mothering instincts would kick in, I would sacrifice my medical needs to care for my child, and my health would decline.

I ensured my health did not decline by continuing to be an avid reader. Would I have been a reader if I did not have CF? Most likely. But perhaps reading would have been something I could have sacrificed as life got busy.

As Jarryn grew, I continued to read. I read a lot. Because reading has always been my way of pacing myself. To slow down, to rest, to be selfish and look after me. When Jarryn was a baby, I’d often drive to my Gran’s house, and let her look after Jarryn while I lay on her bed and read.

As he got older, I read while he played. On the days I didn’t feel I had the energy to keep up with Jarryn and his friends, I’d let them ride their bikes, play on the trampoline or climb the tree while I sat on the curb and read.

I often wondered what Jarryn thought of his childhood, and if he felt he missed out on things because I had CF, so I asked him.

“I was always busy, riding bikes or my Green Machine, jumping on the trampoline, playing with the dogs or games with my friends. Mum was always there. Sometimes she rode the Green Machine or jumped on the trampoline with me, and other times she would read while she watched me play.
Looking back now, I realise that the times she read instead of joining in were the times she was sick and needed to rest more. Back then, however, I had no clue. She was just Mum who liked to read a lot.”

Extract from ‘Growing Up When Your Mum Has CF Part 1’
Read Jarryn’s Full interview 

Bonus fact: A 31-year-old competitively racing Green Machines against eight-year-olds may not be very dignified, but it’s a great way of doing physio.

31 Days of Cystic Fibrosis. May 13
Mum (Mother’s Day)

This post is well out of chronological order, but I thought it best saved for this day. At eight, my mum made me responsible for taking my medications. At nine she handed over responsibility of drawing up my nebs. At twelve I took over my physio. At fourteen, my team at PMH suggested a transition period. The first appointment went smoothly, I went in first, told the team what had happened since I had last seen them, then Mum was called in, and they told her what I had told them. The team envisioned this ‘transition’ would take several years, but they didn’t factor my Mum into the equation.

On my next clinic visit, I caught the bus to Subiaco, then walked to the hospital. I was called when it was my turn, then they asked for Mum. To say they were gobsmacked when I replied I’d caught the bus is an understatement.

So they rang my Mum.

She replied that this was my disease to manage, not hers. And she didn’t attend another clinic appointment for 24 years, and then it was only because I was on oxygen and needed a chauffeur.

Mum’s reasoning was based on her statement to the doctors about CF being my disease to manage. She had witnessed friends children with CF go rapidly downhill when they hit adolescence, mostly due to rebellion and non-compliance. She hoped that by handing me control, I wouldn’t rebel quite so much.

The added bonus was that when I left home at 18, she knew I’d been managing my health for four years, so my health management wasn’t something she needed to worry about.

The result is that I’ve never been shy about advocating for my own health.

It wasn’t until I was a mother myself that I realised how difficult this must have been for her. So thanks, Mum.

Bonus Fact: I’m not going to lie and say I was perfectly compliant all the time. At the time the CF diet was still low fat, and I had a rather large dislike to the powdered milk I was supposed to drink, so I would wait until nobody was home to hop into the full cream milk. Some mornings I would skip my physio and say I hadn’t or just lay on my physio table and read. My first few admissions were a direct result of taking these shortcuts, and the lesson was quickly learnt.

 

31 Days of Cystic Fibrosis. May 14
Hospital in the Home
Jarryn was three when a sinus infection spread to my lungs and tipped my health upside down. It was an infection that ultimately cost me 10% of my lung function. For the first time in years, everything else failed, and I needed IV antibiotics. Fortunately, by this time, the Hospital in the Home (HITH) program was up and running. It meant that a person with CF could spend the first few days in hospital, and then continue the IV antibiotic course at home. Cystic Fibrosis WA organised nurse and physio visits at home.
I always liked to do things differently, and I had Jarryn to look after, so I always skipped the first three days in hospital, had my PICC line placed as an outpatient and went straight home. A PICC line has advantages over IV cannulas as they last for the entire course of IV’s. The line is inserted into a vein near the elbow and threaded through to end in an artery near your heart.

HITH meant that I was responsible for both drawing up my IV medications and then giving myself the push dose through the PICC line. Dependant on the antibiotics, dose schedules could be twice daily, every eight hours and occasionally every six hours.

This flexibility allowed me to continue to work, look after Jarryn and go to the occasional wedding. (As far as I know this is the only picture I have where my PICC line is still visible. This was normal life for me, so we never saw a reason to capture it on film.)

Bonus Fact: I’d always taught Jarryn that when having a new playmate over, he should show them around the house, so they know where important things like bathrooms are. We had recently moved, and Jarryn had started a new school, and his first friend had arrived. I was pre-preparing my IV medications for the next dose, as Jarryn took his friend through the house saying, “This is my room, this is the bathroom, here’s the fridge and that’s my mum shooting up her drugs.”

31 Days of Cystic Fibrosis. May 15
Disability Discrimination

Disability discrimination comes in many forms. Often, people create barriers because of what they perceive to be the problem rather than the actual barriers created by disabilities. I’ve experienced disability discrimination in many forms, and I’ve always felt more disabled by society than from my actual limitations.

Possibly the earliest episode I can remember is from my year seven teacher. He was ‘kind’ enough to inform the class that they should be nice to me because I was going to die when I was in hospital, yet also comfortable enough to tell me to, “Go and put your head in a bucket three times and pull it out twice,” when my coughing annoyed him.

The frenemy in high school who blamed all of my achievements on the fact that I had CF, and as a result, I was receiving ‘special treatment’, rather than putting in the hard work.

The job application I passed with flying colours, only to be failed by the company doctor who told me. “I don’t know how you’re still standing, but CF is a childhood disease. You can’t possibly have long left. Why would you want to work anyway?”

Another job interview, “Work and study? That’s a bit ambitious for you. A normal person perhaps, but not you. Study first, work later.” This privileged male, hired his ‘mate’ for the job I had applied for, assuming I would continue to stay on as a volunteer. I did not.

From a medical receptionist at CF clinic. “Thailand? Oh well, I suppose they can always medivac you out. I’ll watch for you on the news.”

Sadly, some of the more recent discrimination I have experienced has come from within the CF community as the cross-infections guidelines tighten.

These are but snapshots, it’s my experience that true equality is a myth, and on more than one occasion, I’ve had to perform to a superior standard in order to be treated as equal to the able bodied. However, there are times when being disabled can be an advantage – I am tough, stubborn and used to fighting to stay alive, I’ve conquered worse foes than discrimination.

Bonus Fact: Despite the fact that CF is not contagious, during flu epidemics like the Swine Flu or the Bird Flu, my superpower was the ability to clear entire supermarket aisles with a single cough.

31 Days of Cystic Fibrosis: Part 4 May 16th – 21th