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
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.
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.
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.’
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.
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.
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!!
Missed last years 31 Days of Cystic Fibrosis? Part 1 2018 starts here
May 26th – May 31st
Survival is a team effort
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.
Friday, May 27th 2011 – Transplant
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.
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.
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.
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.
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
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
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
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.