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

31 Days of Cystic Fibrosis: Part 2 May 6th – 10th

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 6th – May 10th

31 Days of Cystic Fibrosis. May 6
Hospitalisations AKA Tune-ups

When prevention — the physio, the nebs, and exercise — is not enough to keep infections either at bay or under control, and an infection takes hold, antibiotics are required. Depending on the level of infection, they could be in tablet from, inhaled via nebuliser or intravenously (IV). Before the mid 90’s IV antibiotics required a trip to hospital. Usually for two weeks. Sometimes longer.

In the days before cross-infection reared its ugly head, people with CF would share rooms, and in B Block at Charlies we had a common room. This was a place where we could cook our own food, eat meals together, play board games or watch movies.

For me, the most frustrating thing about having a two week tune-up, is that at some point, the antibiotics would kick in, and I’d start to feel well — but still have to finish the course. In the second week it was common to be allowed a ‘day pass’ and I used mine to return to work, giving myself my own antibiotics at lunchtime in the staff room, returning to the hospital each night.

This photo of me was taken by Sonia Sears, who was my roomie one admission. She was a fabulous and passionate photographer, and this shot is a symbolic representation of “Going Nowhere” although the truth is, hospital food being what it is was we’d often be going on KFC or McDonalds runs – when we couldn’t convince the pizza guys to deliver directly to our rooms that is.

Bonus Fact: Most people with CF sound distinctly different when they cough. My friends and I could identify each other by the sound of our coughing alone.

31 Days of Cystic Fibrosis. May 7
The Last Walk
What happens when you have people with CF, creative nursing staff, and lots of free time?
Shenanigans!
I set my short story, ‘The Last Walk’, in the early 90s. It’s a celebration of the special friendships that people with CF share. The character of Saanvi is a nod to all those fabulous nurses who worked on B12. Not only did they take care of our medical needs, but they also took care of our emotional health, and participated in the shenanigans that made hospitals stays far more lively than they should have been.
50ml syringe water fight anyone?
Truth is way more fascinating than fiction, and I had to leave out far more than I included as I’m not sure anyone who didn’t live through the experience would have believed it!

Photo by Sonia Sears.

Bonus Fact: I once woke up on the balcony pictured. Tom, found it highly amusing to come into my room while I was sleeping, and wheel my bed – while I still slumbered – up the corridor, past the nurse’s station and parked me out there in the open. I’m reasonably confident; he didn’t act alone. But he was the only one I caught, and by caught I mean he hung about to laugh at me.

That’s all I can say about B Block Balconies because what happened on those balconies stays on B Block …

Read ‘The Last Walk’ here

31 Days of Cystic Fibrosis. May 8
Cross Infection 
For me, the first hint that life was changing in the CF community was having to provide sputum samples before going to the Adult CF camp in Queensland. I distinctly remember being amused that Mel and I were ‘not allowed’ to share bathroom facilities as we were both culturing very different bacteria despite the fact that we had been housemates for the few years.Then Burkholderia Cepacia reared its ugly head and ripped our community in two. People culturing Cepacia were not allowed to participate in any official events, and the rest of us were informed that we should have no contact with our segregated friends. Essentially we were told to treat them as if they had Leprosy.I am one of those who did not follow these instructions to the letter. I still saw my friends that had cultured Cepacia, because they were my friends and I wasn’t going to abandon them, but at the same time I wasn’t reckless – we met in the open, we no longer shared food or drink, and most importantly the hugs and kisses goodbye ceased.Cross infection is a minefield. Every year the ‘rules’ seem to tighten and now its normal for a person with CF to grow up isolated from other people with CF. Contact via technology is only the approved method, but it’s not the answer.I can’t say that cross-infection doesn’t exist, because I have friends who cross-infected. On the other hand, I’ve lived with, and dated people with CF. I shared food, drink even physio tables. It wasn’t uncommon for us to drag all our mattresses into the living room and have a slumber party. We had a communal Cotazyme bottle that all who visited dipped into – and I never cross-infected with any of my friends, including those with Cepacia. What I can say is that I don’t believe we have all the answers about cross-infection and what the actual risks are.Bonus Fact: My Mum thought the name Cepacia was unusual/pretty and so she hijacked it and used it as the ‘Official’ (or racing) name for one of her horses.

Happy snaps from the adult CF Camp, Queensland 1993

31 Days of Cystic Fibrosis. May 9
Pregnancy

I grew up believing that I wasn’t going to grow up. And if I did actually reach an age to be considered an adult, children were certainly out of the question – because people with CF were infertile, or so we were told.

But neither of those things turned out to be true.

In 1995 only a handful of women with Cystic Fibrosis attending my clinic had experienced pregnancy, and there was no conclusive data on outcomes. Despite this, I was confident that pregnancy was something my body could handle.

Everyone had an opinion, and no one had any hesitation to share theirs.

It turned out my intuition that my body could handle a pregnancy was correct. I am so very, very proud that during my pregnancy, the only medication I took included enzymes, vitamins and the occasional panadol.

I had one admission to hospital while I was pregnant. It was under the pretence of having additional physio, due to having had a nasty cold a week before. Once there, I discovered some discrepancies. There was only one physio on staff at King Edward who was trained to give percussion, and while she could come twice a day, I was only allocated 20 minutes of her time. My medical team started pushing hard for me to consider a caesarean. Soon. Very soon.

My suspicions on the nature of my stay were confirmed when my not-so-special specialist, informed me that he did not believe a woman with CF could endure labour.

When we got to the weekend, it turned out that despite being an inpatient, there was no one available to give me physio until the following Monday. So I discharged myself.

Bonus fact: Babies are not fond of being yelled at while in the womb.
Let me explain.
I had Julie on one side of my stomach, murmuring nice things, while Melinda was on the other side, bellowing “Hello Baby!” at the top of her voice.
My entire stomach lurched as my baby tried to get as far away from Melinda as possible, which incidentally was right into Julie’s hands.

31 Days of Cystic Fibrosis. May 10
Jarryn’s Birth
The Monday after I discharged myself, my water broke, and I returned to hospital and gave birth a few hours later without medical intervention. Unless you count having two panadiene that is.
Being a few weeks early, Jarryn was taken to the special care nursery, and I was sent to a new ward. This new ward had no record that I had been in hospital the week before, or that I was under a high-risk specialist. I asked for the physio who had done my treatments the previous week by name, but instead of paging her, they wrote me up on the board as being a hysterical new mum who was worried about her kegel exercises. (Fortunately, my physio was on the ball and found me!)Two days after giving birth, my not-so-special specialist came to see me. He had no idea I’d spent the weekend at home, or that I’d given birth. He wanted to discuss his plans to ‘book in’ my caesarean. You can imagine my delight as I started to explain to him that I was not under any circumstances having a caesarean. He started to bluster but didn’t have a leg to stand when I pointed out that he should perhaps read my chart.Bonus Fact: I was placed in a room with three mothers who had their babies with them. It was a rather uncomfortable feeling. There were whispered questions from their various visitors asking if my baby had died, or if my baby had been removed from my care. It went on ALL afternoon.
That night, after being woken multiple times by them and their babies, I went downstairs and spent the night with Jarryn in the special care nursery.
When I returned to the ward, I found out I had been evicted from the room. Turns out, the other women had gotten together and complained that my coughing was terribly disruptive to them.
Which meant I got to enjoy a private room for the rest of my stay.

 

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