31 Days of Cystic Fibrosis 2019: Part 4 May 14th – 18th

May is Cystic Fibrosis awareness month, and once again I’ve done 31 Days of Cystic Fibrosis on Facebook and Instagram.
In 2019 I concentrated on a hidden side of CF and profiled people who are impacted by CF.

May 14th – May 18th 2019

31 Days of Cystic Fibrosis. May 14 – 16
Merle Crawford: A Mother’s Story

For four and a half years, Merle Crawford took her daughter Carolyn back and forth to doctors. Despite a hospital admission for pneumonia and Gastroenteritis when Carolyn was 16 months, doctors dismissed Merle’s concerns. “One doctor thought I was fruit loop and another told me I was having babies too quickly.”

“The doctors at PMH didn’t think there was anything wrong with her because she didn’t look sick. They decided to put her on a fat-free diet, and when I went back, I told them it hadn’t made any difference.” Merle said. The next step was a sweat test, and Merle was told while they were not expecting a positive result, that someone would be in contact if there did prove to be something wrong.

That call did come, and at the resulting clinic appointment with Dr David Formby, Merle was told that Carolyn had CF. “Then David Formby turned to me and said to me ‘You realise she may only live a maximum of 16 years.’ I nearly fell off my chair.”

“They decided to take my two boys for the sweat test on them, but they were both clear. It was a relief, but the shock of Carolyn’s diagnosis remained. I’d never heard of CF.”

The next step was learning how to do postural drainage in hospital. “Carolyn got upset wanting to know why I was hitting her. She asked why I was doing this when she hadn’t been naughty. I explained to her that it was a problem with her chest. Explained that it was to help her, but in her mind, I was doing something awful to her.”

Carolyn’s diagnosis affected the whole family unit. “It was a complete change of lifestyle; you had physiotherapy in the morning and at night.
We got into a routine, the only thing I didn’t like was mixing up the powder, the stinking rotten enzyme powder. It smelt like horse pee.”


Merle asked David Formby if enzymes were available in tablet form, but he brushed her off stating that Carolyn was too young. “Let me try,” Merle said, “I gave her a demo and showed her how to take a tablet. I said hold your head back, and this big drink of water and they’ll all run down your throat. And she mastered it. I went back and said I don’t need this disgusting powder, can I have a script for the tablets?”

With the removal of the enzyme powder, the household went from one foul smell to another. Carolyn needed to inhale mucomyst by nebuliser before every airway clearance treatment. “It was pretty rank,” Merle said. “It stunk the whole house out.”

As Carolyn became a teen then an adult, the challenges became more confronting. “There were lung haemorrhages, and collapses. It was pretty frightening. I knew Carolyn was going downhill.”

When Carolyn was 26, Dr Gerard Ryan, from the CF Clinic at Sir Charles Gairdner Hospital, suggested she should meet with the transplant teams from either Sydney or Melbourne. As the team from St Vincent’s in Sydney, led by Dr Allan Granville was in Perth, it was an ideal starting point.

Allan Granville told Carolyn “I estimate you have about two years left to live.” “It was like a bowling ball coming across the desk whacking you fair in the chest,” Merel said. “And as it turned out, he was spot on.”

Instead of meeting with the Melbourne team for a second opinion, Carolyn put her faith in the team from Sydney. Both Merle and Carolyn moved to Sydney to start the long wait.

Merle couldn’t speak highly enough of the team from Sydney, the only hiccup being the need to navigate between two hospitals. The CF Clinic was held at RPA while St Vincent’s was responsible for both pre and post lung transplant.

After Carolyn had been on the waiting list for two years, Merle was given information that no parent wants to hear. Unless a transplant came through that weekend, the doctors would need to move Carolyn to ICU and intubate her. That would be the point of no return, for the team at St Vincent’s would no longer consider Carolyn a viable candidate for transplantation.
“I was freaking, but I didn’t let her know.”

On Sunday Carolyn and Merle received the news they had been waiting for. The next period was frantic as they prepped Carolyn for surgery. Merle said, “Carolyn asked for the room, and she said, ‘No matter what happens Mum you’ve been my rock. If I don’t come back from this, don’t be sad, because it will be a relief. But if I do – watch out!’”

“Saying goodbye to let her go to the theatre was hard. Waiting to see her again was hard. But when I did, Carolyn held up her pink fingernails and pointed to her pink lips. She was as happy as Larry.”

Allan Granville warned Merle that as Carolyn’s heart and other organs had worked so hard to keep her alive, one of the consequences would be a longer than normal recovery. “Yet sixteen days later, we were out. And I was in big trouble – Carolyn told me off for walking too slow. It was the first time she’d ever told me to hurry up.”

In November this year, Carolyn will celebrate her silver anniversary – 25 years with her new lungs.

31 Days of Cystic Fibrosis. May 17
The Financial Burden of Cystic Fibrosis

Sales usually invoke images of shopping bargains. The sale I went to this week wasn’t nearly as exciting – it was at the Chemist. Instead of a bag full of fancy clothing, I spent $362 in exchange for a bag full of vitamins; usual RRP is $624, that will only cover my needs for the next three months.

I reached the threshold for the Safety Net last week. The Safety Net protects families from the high cost of medications, and for me, it means a reduced price for medications covered by the PBS until the end of the year.
Vitamins, and other over the counter medications, while not covered by the PBS are not only prescribed by my medical team, but essential given my issues with malabsorption due to CF.

For me, the alternative would be regular admissions as a day patient for infusions of both iron and magnesium. Taking calcium every day has helped my bones stabilise, putting a hip replacement on the back burner – for now.
My twice daily dose of zinc and Insolar have reduced the number of skin cancers I’ve needed removed.

These costs make having a Health Care Card essential – but many people with CF don’t have one. Currently, parents of children with CF can access the Carers pension, which provides a Health Care Card, until their child is 16. IIf studying, a child with CF may have access up until the day they turn 25.

Adults with CF can access a Health Care Card via Centrelink only as a low-income earner, or via the Mobility Allowance.

We’re lucky to live in Australia with the subsidies provided by the PBS. However, the cost of medications, both on script and over the counter, can be a crippling burden for some families living with CF.

I’m lucky to be in a position where I don’t have to make choices about paying the rent or paying for my medications – for others; tough choices are made daily.

31 Days of Cystic Fibrosis. May 18
Maggie Cunnington – Resource Development and Training Officer Cystic Fibrosis WA

Maggie has to hit the ground running at the beginning of the year, as her time is consumed with presenting education sessions on CF to school staff. This year, Maggie presented her sessions to over 400 teaching staff – discussing CF, how it impacts the child, their family and what the subsequent impact may be on the classroom, including how to adapt the classroom environment for a child with CF.

Additionally, Maggie presents education sessions to students. After an overview of CF, Maggie presents a lesson – either on genetics or how to stop the spread of germs via hand-washing and proper hygiene.

Recently CFWA purchased a product that is visible under UV light, and in her introduction, Maggie asks all the students to introduce themselves while holding a pre-prepared tennis ball. “They’re always excited to say their name and favourite colour. Once we’ve got around the room, I tell them I’ve tricked them, that the ball has fake germs, so we turn off the lights, and use a UV flashlight. They hold out their hands so they can see all the ‘germs’ on their hands.”

This visual demonstration is a very effective tool to show children how germs spread rather telling them. The next step is for the children to wash and dry their hands using a variety of techniques. Then the UV light is again used to demonstrate how effective, or not, different hand washing techniques can be.

Another aspect of the education component of Maggie’s job is continuing to develop and promote CF Smart. CF Smart aims to improve the understanding of CF in a school-based environment and has sections for Teachers (including eLearning), Parents, Students and Health Professionals. The website is packed full of fact sheets, tips and checklists. The eLearning components of CF Smart, which allows teachers to access a Professional Development certificate, are unique.

With CFWA’s vision, “Lives Unaffected by CF” written on her whiteboard, Maggie has a constant reminder of how her work to help teachers understand the impact of CF on the family, can subsequently make school less of a struggle for students with CF.


Missed the start?
31 Days of Cystic Fibrosis Part 1 2019 starts here
Part 1 2018 starts here

31 Days of Cystic Fibrosis 2019: Part 1 May 1st – 5th

May is Cystic Fibrosis awareness month, and once again I’ve done 31 Days of Cystic Fibrosis on Facebook and Instagram.
In 2019 I concentrated on a hidden side of CF and profiled people who are impacted by CF.

May 1st – May 5th 2019

31 Days of Cystic Fibrosis. May 1
Helen Price: A Mother’s Story

A diagnosis of Cystic Fibrosis in the early ’70s was devastating for many parents, but the initial reaction for Helen Price was a relief. Finally, Helen had a diagnosis for her daughter, Sandi, along with a script for enzymes and more importantly a treatment plan.

However, the diagnosis of Cystic Fibrosis was delivered without explanation, and Helen knew she required more information. At the library, a one-line answer revealed that CF was a childhood disease. At the next clinic visit, she asked carefully considered questions. However, the answers continued to be spoon-fed with the information imparted over several months.

Eventually. after months of questions, she was told the grim news – her daughter only had a 50% chance or reaching 13. With all the pieces of information in place, Helen now knew that the initial prognosis was bleak. 

The entire family was involved in the daily regimen to keep Sandi as healthy as possible. Endless enzymes to digest food, nebulisers and physiotherapy sessions. There was no alternative other than to adapt to this new way of life. This daily regimen came hand in hand with learning to be patient, tolerant and brought with it a greater depth of understanding and sympathy to people who were ill. 

Everything was day-to-day – the future was never under consideration. Because according to the information, the statistics, there wasn’t supposed to be a future for a child born with CF in the early ’70s. The improvements in life expectancy, the advances in treatment – everything that has happened around the treatment of Cystic Fibrosis since Sandi was diagnosed has been ground-breaking.

Helen and Sandi 1973

31 Days of Cystic Fibrosis. May 2
Kathryn Pekin – Services Manager at Cystic Fibrosis Western Australia 
As Services Manager, part of Kathryn’s role is the provision of counselling support to both people with CF and anyone with a connection to CF. Cystic Fibrosis Western Australia have adopted a family-centric approach, as the importance of supporting a person’s entire network is backed up by extensive research. Therefore, Kathryn provides support to a complete network including family members, teachers and work colleagues – as CF impacts more than the individual.

Supporting people with CF and their network is a partnership which allows Kathryn and the wonderful team she works with at CFWA to work creatively to find the best solutions.
The toughest issue faced by CFWA, and by extension, the CF community is that of cross-infection. As an organisation, CFWA has to be sensitive to the needs of the people with CF, while still maintaining best practice with hospital guidelines. It’s a fine line. 

During Kathryn’s 13 years with CFWA cross-infection has increasingly become a tougher issue to navigate. For families that are newly diagnosed, cross-infection can cause a lot of angst. In response, Kathryn and CFWA are looking more and more towards digital technologies, and in particular, videos featuring people with CF sharing their lived experiences to help overcome this hurdle.
While digital technology is part of the solution, Kathryn knows that it’s not the complete answer to solving the isolation people with CF can feel. Providing support services focused on mental health will go a long way towards combatting the challenge of isolation. Kathryn is excited to be involved as CFWA instigates more services in this area. 

Despite her wonderful working team, the job does have its downfalls – even with all the measures and services that CFWA has in place there are still many gaps, and this knowledge can, at times, be overwhelming. 

Editing Note: I told Kathryn I hoped putting up with my son at the CFWA siblings and offspring camps was NOT the worst part of her job and this notion was met with way too much laughter. 

Kathryn Pekin – Services Manager at Cystic Fibrosis Western Australia

31 Days of Cystic Fibrosis. May 3
Katya Price: A Mother’s Story

Before Jordan’s diagnosis via the Guthrie test, the only exposure Katya Price had to CF was knowledge of a friend’s child who died young. Her first thoughts focused on the fear that her child was going to die.

Jordan’s diagnosis impacted the entire family with emotions from denial to devastation. Katya herself found the process overwhelming, and later on when she had had enough time to process everything properly she found herself depressed. Eternal sadness – the wording borrowed, but Katya feels it is so reflective of how she felt. 

As a coping mechanism, Katya immersed herself in the CF community to learn everything she could to help Jordan; she surrounded herself with people who could teach her, look after her and tell her what to do, including working at Cystic Fibrosis Western Australia. But with time, it became so consuming that Katya realised she no longer had a life outside the world of CF and it was time to step back.
Reflecting, Katya wishes she’d known the realities of life with a child with CF – that It was more than living with an expiry date. Knowing the various stages of CF would have empowered her, given her the knowledge that CF was manageable and Jordan’s life would be like any other kids – however there would be extra stuff he needed to do to stay healthy. 

CF made Katya stop and think. In the time before Jordan’s diagnosis, she had lived for tomorrow, working hard for a future without a mortgage. Now Katya strives to live everyday more meaningfully; she spends more time with her family and has a better quality of day-to-day life. Katya changed her life to give Jordan the best possible life she could, and if she could change one thing about CF, she’d remove the psychological impact of living with a cloud over your head. 

Katya has the following advice for parents of newly diagnosed children, ‘Don’t compare your child to other children with CF. Endless comparisons can see you end up with either a false sense of security or insecurity – neither of which help in the long term.’ 

Katya and Jordan

31 Days of Cystic Fibrosis. May 4
Jarryn Bowie: A Son’s Story
Having a mum with Cystic Fibrosis was Jarryn’s normal.
Over time he came to realise that other parents didn’t take handfuls of tablets every time they ate.
Other parents didn’t do physio.
Other parents didn’t go to the hospital to see specialist doctors regularly.
Other parents didn’t have friends in hospital that they would visit every other week.

Gradually the realisation dawned on him that having CF meant his mum was different from the other mums. However, that realisation had little impact until his mum’s health started to decline.
At first, the only change was that his mum started to cough more. Jarryn knew a major CF coughing fit would always pass, but when out in public he’d find that people would blatantly stare with a morbid curiosity. Never once did anyone ask or think to check if his mum was alright.When his mum came home from hospital on oxygen, Jarryn started to worry. Oxygen therapy was different from the regular maintenance regimen that he was familiar with. Of more concern, was that along with the oxygen came talk of a lung transplant. Previously he could help out, doing little things to help his mum. The transplant wait list was a different ball game – there was nothing he could do except wait and hope that a lung transplant would happen in time.
Post-transplant Jarryn says his mum has changed. She has more energy and is more like the mum he remembers from when he was younger.
As a carrier of the CF gene, Jarryn is aware of the possibility that his child might have CF. However, he doesn’t see it as a big deal. He’s aware of the advancements in treating CF, in particular in the last five years, and while these advancements haven’t provided a cure for CF, they have taken a huge leap in that direction. More importantly, he knows that if he does have a child with CF, there is hope and a future.

Editing Note: Jarryn mentioned that one of his favourite CF related memories was visiting me in hospital because the nurses would give him choc milk and lollies, then he’d hijack my jelly. I couldn’t help but burst his bubble. There was no hijacking – I always saved the jelly for him.

Sandi and Jarryn on Jarryn’s 21st Birthday

31 Days of Cystic Fibrosis. May 5
Sue Morey: Nurse Practitioner, Respiratory Medicine

Sue Morey is in a position to make a difference, and she takes advantage of her position to short-circuit the system and achieve the best possible outcomes for CF patients.

Working with CF patients differs from other respiratory patients due to the demanding nature of the disease and the requirement of a life-long continuous commitment. Additionally, CF patients are usually younger, occasionally stubborn**,and need more guidance to be compliant with treatment.

Sue says she was ‘lucky enough’ to be able to work with Dr Gerald Ryan to develop a model of care for CF patients, that not only works well but is in the best interests of the patients. An open-door policy allows patients to call Sue whenever they have a problem. Problems can range from the simple – a new medication script – to urgent access to a hospital bed.

For Sue, the best part of her job comes via the positive reinforcement from her CF patients. Her input is appreciated, as is the ease with which she can navigate the hospital system making dealing with CF a little easier. (My freddo bribery system pales in comparison to the way Sue operates.)

The flip side to the job is the reality that after years of building relationships and working with people with CF, many do deteriorate and die.

Bonus Fact: I’ve seen Sue on more than one occasion short-circuiting the system, and she is a force to be reckoned with. After nineteen years of refusing to be admitted to hospital unless it was necessary, I woke one morning knowing I needed to be admitted immediately. As Grant drove me, I rang Sue and asked her to arrange a hospital bed. Without question, she agreed. Ten minutes later as we pulled into the carpark, Sue was waiting for me with a wheelchair and oxygen. As she bustled about arranging a bed, I overheard one of the staff say, “You don’t ever want to get between Sue and one of her CF Patients.”

**NO! Sue was not referring solely to me. I know because I checked!!

Sue Morey


Missed last years 31 Days of Cystic Fibrosis? Part 1 2018 starts here

31 Days of Cystic Fibrosis 2018: Part 6 May 26th – 31st

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 26th – May 31st

31 Days of Cystic Fibrosis. May 26
Survival is a team effort
My decision to stay at home did not mean I was the sole person responsible for my health. I was surrounded by a team of people that were working together to keep me alive.

On a practical level, Mum was chauffeuring me to multiple appointments each week. Grant was preparing food, in charge of the hygiene requirements to keep my various medical equipment sterile, and in the last few weeks he took on the task of sorting out my IV medications and pre-preparing them for me. (All the while, both of them still had to attend their day jobs.)
Dale, my homecare worker from Cystic Fibrosis WA, came twice a week to take care of light housework and assist with physio treatments. John, my homecare nurse from CFWA, came once a week to change the dressing on my port.Physio staff from both hospitals compiled exercise and muscle strengthening programs for me – which I refer to as “pilates in bed”. In addition, Jamie did my lung function at clinic until it dropped that low that measurement pointless. (My last official measurement indicated that only 10% of my lungs were working.) Also at clinic, Paul the dietician kept an eye on my calorie intake and weight loss while Dr Joey kept an eye on my blood sugar levels.

Sue, or Aunty Sue as she is affectionately known, our Clinical Nurse Specialist, looked after my various scripts (including getting them filled) along with arranging my weekly tub of supplies full of syringes, saline, alcohol wipes etc and had them ready so that each Monday Mum could exchange an empty tub for a fresh one. Sue would also take blood from my port, and collect sputum samples to save me the walk to Path West. (Sue is an incredible woman and has an uncanny ability to read her patients. The less attention she pays to you the better, when she starts doing all of your fetching and carrying you know things are getting serious!)Uncle Gerry saw me in clinic once a week and would then liaise with Dr Mike to keep Royal Perth updated with my status (important for your ranking on the transplant list.)Behind the scenes were people I never saw. The pharmacist checking my various medications to make sure there were no contradictions, pathology staff checking all my various samples and various researchers over the world who developed new medications and technologies that had gotten me to this point (the new improved enzymes, ciprofloxacin, azithromycin and Pulmozyme).

Bonus Fact: During this time, I had one job. All I had to do was keep breathing. It may seem easy, but the effort required to lounge around in bed all day and continue to breathe was causing me to lose ½ kg a week despite my calorie intake being three times the daily recommendation. I was clinging to life by my fingernails.

31 Days of Cystic Fibrosis. May 27
Friday, May 27th 2011 – Transplant
Grant had gone to work, and I was reading in bed, Monkey tucked in my armpit. Our house phone rang. It rarely rings, most people knew to call my mobile. Normally I wouldn’t have worried, but I had a feeling this phone call was important.

By the time I’d unhooked my Bi-Pap, hooked up my oxygen and made my way to the phone it stopped ringing. I shuffled back to bed and sat for a moment staring at my mobile. As I weighed up the effort it would take to unhook my Bi-Pap versus waiting a few more moments to see if my phone would ring, it did.
Lungs had been offered. Lungs that had been matched to me.
When Grant and I got to the hospital, there was a sobering truth to face. In ‘normal’ circumstances, I would be prepared for surgery while under general anaesthetic, then ventilated while the surgeon checked the lungs to confirm he was happy for the surgery to proceed. Dr Mike told me my lung function was at the point that if I were to be ventilated, and the donated lungs did not pass the surgeon’s inspection, I would need to live in ICU while I waited for another offer. The compromise was I would go through and have all the various lines installed and primed, and would only be ventilated if the surgery was to proceed.
It is said that your life flashes through your mind as you’re about to die. When you’re primed for surgery and waiting on your lonesome, your life doesn’t just flash, you have time for reflection. I’d had a good life. I knew I was rolling the dice for a chance to have a future, knowing that I might not be strong enough to survive the surgery. I also knew that these lungs were my only hope.

Bonus Fact: On the 27th May 2011, it was 65 Roses Day, and my Aunty Mary had been planning to take me to have lunch with my Gran. I sent her a text declining her invitation due to having a “better offer”.

Extra Bonus Fact: I wanted to have a Facebook status of “Hi Ho, Hi Ho – it’s off for lungs I go..” posted after the phone call to confirm the surgery was going ahead. “He Who Was in Charge of the Communication Device” vetoed it.

Extra Extra Bonus Fact: My expert updo (pictured below) was skillfully arranged by my ICU nurse who used a rubber glove because we had no hair lacky available.

Extra Extra Extra Bonus Fact: Dr Mike had I had a conversation about what to expect in the ICU, and he asked if there was anything in particular I was worried about.
“Yes,” I said, “I’m going to need that ventilator tube out ASAP.”
“Why the tube phobia?” he asked.
“No phobia, but I think it’d be easier to complain without it.”
He didn’t look amused.

31 Days of Cystic Fibrosis. May 28
At home, Monkey was slowly deteriorating. Grant asked my team when I would be allowed to see her, as he was worried she wouldn’t make it to see me come home. The clearance, he was told, would be given when I had all tubes and drains removed to minimise the infection risk. It was a goal to work towards.

8 days after my transplant, I had my final chest drain removed and on the 9th day I was cleared to leave my room. On the 10th day, Grant took me down to the carpark in a wheelchair where Monkey was waiting.
My beautiful girl, who had stuck with me through thick and thin, who over the years had spent many nights in the bathroom sitting next to me when my enzymes hadn’t worked quite how they should, who had stayed up late to sit with me while I injected my last dose of IVs’ for the evening, and who had been my constant companion ‘looking after’ me while I’d been waiting for the call that would change my life, was now too weak to jump out of the car yet at the same time was practically jumping out of her skin with pleasure to see me.
So, I gingerly climbed in the back seat and sat with her. My Monkey, who had never ever liked anybody to breathe near her, sat thumping her tail and sniffing my breath. Because my breath was carrying a message, it told her I was no longer dying, and because of this, she knew that she didn’t have to hang on to look after me any longer.
Bonus Fact: My oxygen cord had a limit of 20 metres. In the beginning, I could walk a little faster than Monkey could, so she’d sit on my oxygen cord to make me wait for her. Towards the end, as she was sleeping more and more, she started sleeping with my oxygen cord wrapped around her paws, so she could wake up if I moved.

31 Days of Cystic Fibrosis. May 29
Life Post Transplant

On the 9th day post-transplant, I had my first shower standing up in seven months. Afterwards, I remember looking at my battered body in the mirror. I was covered in scars, dressings and bruises and all I could think about was how great life was. Because I could breathe.
I was filled with a simplistic pleasure in knowing the enjoyment of something that most people take for granted. I could breathe, walk, and talk, all at the same time again.

These lungs are magnificent. They do what lungs are supposed to do, and they do it naturally and without effort.

That’s not to say that my recovery wasn’t without effort. I put in hours at the gym, followed by hours of walking. On the 3 month anniversary of my transplant, I walked 4kms in the City to Surf. It took me 56 minutes, which may not seem like much, but before my transplant, I couldn’t walk the full 20 metres on my oxygen cord without stopping.

Full recovery including adjusting to life post-transplant and the various side-effects of my new medications took 12 months. But every second of that recovery was worth it because even now there is still so much joy in simply breathing.

Bonus Fact: I have done so much in the last seven years, I’ve travelled, received industry awards for my library work, published two books and seen Jarryn turn 21. All of this possible because a family said yes to organ donation.

31 Days of Cystic Fibrosis. May 30
A Transplant is a Trade Not a Cure

On the surface, it looks like my transplant provided a cure, but that’s not the case. I will always be a person who has chronic health issues; I traded one set of health problems for another, the bonus being I am still breathing, still alive and ready to continue the fight.

My first complication had minor symptoms, my left hip ached, something that is seemingly innocuous. Investigations revealed I had avascular necrosis in my hip. Essentially the medication keeping me alive was also strangulating the small vessel that supplies blood to the head of my femur, causing that bone to start cracking. My team managed to stabilise the problem, but eventually, I will need a hip replacement.

Towards the end of 2013, I was displaying all the symptoms of post-transplant lymphoma – all my lymph nodes on the left side of my body had enlarged. However, it turned out I had a funky fungi instead, one that has a reputation not only for mimicking lymphoma but also for being fatal. It saw me embark on two years of antifungals — a treatment that caused massive damage to my skin and both precancerous and cancerous growths develop on my face, chest, arms and hands.

Bonus Fact: As far as my team are aware, I am the first person in Australia to contract this fungi after a lung transplant. There is no data on surviving this fungi on a long-term basis. Because of this, they don’t know if my funky fungi has been eradicated or if it’s dormant and may resurface again. The guys in the white lab coats were all very excited to have an opportunity to study something they had never seen before and nerded out over the results of my bloodwork, establishing that I contracted the airborne version of the funky fungi, and it then migrated to my blood, and not the other way around.

Extra Bonus Fact: Post-transplant lymphoma is caused by the very medications that keep transplant patients alive. Everything is a trade, always.

31 Days of Cystic Fibrosis. May 31
The Future

I hope that by sharing my story, I’ve raised a little awareness about what it’s like to live with Cystic Fibrosis. Now’s the part where I ask for a little help.

Recently, the first medication to treat the cause of CF was developed. It was unique in that every prior medical advancement had only treated the symptoms of CF. Getting Kalydeco on the PBS was a mammoth effort, but Kalydeco only treated a specific mutation of the CF gene. Orkambi is the latest medication. It treats the most common mutation of CF, identified as Delta-F508. It’s my mutation. I have a double Delta-F508 mutation.

As a person with Cystic Fibrosis who is post-transplant, Orkambi can’t help me – but there ARE people it can help, if only it were listed on the PBS. I can’t imagine how heartbreaking it must be to know that there is a treatment that doesn’t merely improve the quality of life but extends it, and not being able to access it.

To help those who can be helped by Orkambi, please consider adding your name and comments to the Pharmaceutical Benefits Advisory Committee
You’ll find Orkambi listed under LUMACAFTOR


Cystic Fibrosis Western Australia has played an important part in my life. As the end of the financial year looms closer, if you haven’t chosen a charity to donate to, I hope you’ll consider CFWA – they’re doing important work. Their mission is “Lives Unaffected by CF” Without their help, my life would have been significantly harder.


And finally, if you’ve never had a chat about organ donation, talk to your family today. Let them know your wishes. Knowing their loved one’s wishes plays a huge part in families consenting to organ donation. It’s a hard conversation to have, but having that chat can save lives. It saved mine.

Bonus Fact: I have been extremely fortunate to arrive at May 31st 2018, aged 45, with the opportunity for more great years ahead. Many children born with CF are not so fortunate as the average life expectancy remains at 38 years.

Thank you to all the wonderful people who have helped me on my journey and without whom I would not be here to share this story. Thanks also to all of you who have taken the time to read my daily posts and share my trip down memory lane.

Finally, and most importantly, to my donor family, as always my eternal thanks and love for saying yes to organ donation in your time of loss.


Missed the start of this?

Part 1 starts here