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
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.
31 Days of Cystic Fibrosis. May 27Friday, 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.
Monkey
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
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
http://www.health.gov.au/…/Cont…/PBAC_online_submission_form
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.
XXX
Sandi
Missed the start of this?
Part 1 starts here
Sandi Parsons, reader, writer and storyteller. 