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

31 Days of Cystic Fibrosis: Part 1 May 1st – 5th

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 1st – May 5th

31 Days of Cystic Fibrosis. May 1
Helen’s Story
After months of being told she was ‘just a nervous new mum’, my mum finally received confirmation that something was indeed wrong with her child.
A diagnosis that came with initial relief and hope for answers rapidly turned to being told her daughter only had a 50% chance of making it to 13.

31 Days of Cystic Fibrosis. May 2
Enzymes

I take 112 Creon 10,000U per week – that’s if I don’t eat any snacks. Every snack requires an extra Creon or two. (And not many days go by that I don’t eat snacks!!)
I’ve taken enzymes all my life, and without them, I can’t digest food. I can still remember the transition from the enzymes I took as a small child, a powder that needed to be sprinkled on my food, to tablet form. Each enzyme was in its own foil wrapped packaging. It took longer to open them than it did to swallow them!
As a teenager, I used Cotazyme, which was in a capsule form and contains hundreds of beads, like Creon is today. I took 20 with a standard meal and 25 if I was eating fast food.
Now with the advancements in medical technology, I only need 4 Creon for each meal. If you’re a math-based person and do the calculations, you’ll realise that 4 Creon taken with three meals a day, for seven days falls short of the 112 total. The extra tablets are because I take Creon with my immunosuppressant medications to aid their absorption.

Bonus Fact: I can take up to 27 tablets with 1 sip of water. It’s a CF Camp record that I’m rather proud of and can still achieve 33 years later.

31 Days of Cystic Fibrosis. May 3
Percussion
Until my transplant in 2011, physio was a constant in my life. A twice-daily grind that often felt like punishment. When your lungs are filled with gunk, and your ribs & head ache from coughing all day, it’s the last thing you feel like doing, but physio is a necessary evil.
As a child, percussion was the method of choice, and as an adult, I used a mix of the percussion technique along with a handheld device called a Flutter.
The day my Gran was accused of child abuse has made two appearances in my writing to date. It was fictionalised in my picture book, ‘The Mystery of the Sixty-Five Roses’ (published by Cystic Fibrosis WA and it also starts off my essay ‘Breathing Life into a Dream’ which was published in the Serenity Press anthology ‘Writing the Dream’.

Bonus fact: Always one to be polite, I would always thank Flame (my CFWA Homecare Worker) or the hospital Physios with “Thanks for beating me,” after a percussion session. Which was all fun and games until Jarryn told his teacher that he had to go straight home that afternoon because Flame was coming around to beat his Mum.

31 Days of Cystic Fibrosis. May 4
Cystic Fibrosis WA Part 1 Camps.
CF Camps run by CFWA were a highlight of my childhood. It was a place where I was no longer different. I wasn’t the only child taking handfuls of tablets, nor was I the child who had to go home early to have physio, or the only skinny child with a pot belly (the enzymes weren’t always the best back then!)
These things that marked us a different, at camp we all did together.
I forged lifelong friendships at these camps, friendships that spilt out of camp, out of the hospital and into our regular lives.
Apart from the friendships, the socialisation I had with other people with CF gave me something else immensely valuable. I saw all the stages of CF progression, and although my CF stagnated without progression for many many years, I was never under any delusion that it would stay that way. I knew that at some point I too would progress. CF didn’t throw me any surprises (except for how quickly I declined during end-stage respiratory failure) because I had watched my friends go through everything before me.
I knew about PICC lines, and ports well before I needed them. I knew about all about hemoptysis before I had to deal with it and I was very aware of the possibility of a pneumothorax (although fortunately I never had one). I held my friend’s hands as they were dying and I’ve been to more funerals than I care to remember. But rather than feeling anxious or scared, this knowledge was powerful, it educated me on the steps I need to take with my health and the things I would need to fight for. It made me a stronger person.

Bonus Fact: Mitch – I know I gave you terrible trouble during FLOB (Flat on Back – which was occasionally enforced between 1pm and 2pm so the adults could have some rest time to compensate for us staying up talking most of the night) time. However, now, nothing beats a good FLOB in the middle of the day

Horrible Fact: Due to cross-infection guidelines, people with CF are not encouraged to meet in person, there are no more camps or social activities. Some people with CF have never met another person who also has CF.

Picture from The West Australian, 28th April 1980. Front to Back: Julie, Melinda, Sandi and Shannon.

 31 Days of Cystic Fibrosis. May 5
Salt, salt, glorious salt.

People with CF have 5 x more salt in their sweat than people who don’t have CF. (Not to be confused with sweating 5 x more!!) Essentially, the salt travels to the skin surface with the sweat but is not reabsorbed.
In summer you can see, and brush off, the salt crystals on my skin.
When salt stores are low, symptoms can include fatigue, fever, muscle cramps, stomach pain, vomiting, dehydration and heatstroke. As a child, I had several admissions to hospital due to low salt and dehydration. Then the fun started. Salt tablets.
Nasty, nasty things!
To be clear, I’ve taken various and numerous medications over the years, and there is NOTHING worse than salt tablets.
Most days I have vegemite for breakfast, and in my early twenties, after a little experimentation, I was able to stop taking salt tablets. Now, my first warning that my salt is getting low starts before all the symptoms mentioned above, instead, I start to crave salted potato chips and salted nuts. By crave, I mean think about them constantly. And if my salt starts getting extra low, I start thinking about vegemite on cheese.

It’s a snack I’ve named for obvious reasons, “Would you like some cheese with your vegemite?”

Bonus Fact: All this extra salt makes me extremely attractive to the animal population. The horses used to pin me to the rails and use my legs as a personal salt lick. Pepsi, who enjoys licking without any incentive, has the same blissful look on her face when I’m salty as she does when she has to clean up spilt food.

 

 

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