31 Days of Cystic Fibrosis 2019: Part 6 May 24th – 28th

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 24th – May 28th 2019

31 Days of Cystic Fibrosis. May 24
Sixty Five Roses Day

Some dates and celebrations have a special meaning – in May 2011, for me, Sixty Five Roses Day became something more than a day to raise awareness and funds for CF.

Friday, May 27th 2011, started as just another day for me. I knew it was Sixty-Five Roses Day, but I was housebound, dependant on oxygen, and not in any shape to help paint the town red for CF. I was barely able to breathe, despite the oxygen whirling up my nose.

That day changed my life. At 9.30am, I received a call telling me to come to the hospital ASAP, as donated lungs were waiting for me. By 12.20pm, I was prepped and waiting for the last inspection of the donated lungs to take place.

As I lay on the operating table, I thought of many, many things. I ticked off the things on my bucket list I had already accomplished. And I started a new bucket list, for the things I would do. One of the things that sat in both columns was my book, ‘The Mystery of the Sixty-Five Roses’. I’d started writing it back in 2007, and had delivered the completed manuscript to CFWA at the end of 2010. I had indeed written my book. I knew that if I died, my book would continue to go to print.

But I still wanted to hold a printed copy in my hands.

All up, it took six years and a new set of lungs but on February 26th 2013 ‘The Mystery of the Sixty-Five Roses’ was officially launched.

Like Sixty-Five Roses Day, ‘The Mystery of the Sixty-Five Roses’ both creates awareness of CF and raises funds for Cystic Fibrosis WA

31 Days of Cystic Fibrosis. May 25 – 26
Claire Stubber: A Wife’s Story

Claire Stubber met Ian during her first session of pre-transplant gym at the Alfred Hospital. “Like most CFs I know Ian didn’t let on that there was anything wrong with him besides a pretty nasty cough that he couldn’t hide and obviously damaged lungs. I remember his pancreatic enzymes because one of my sisters used to pull the capsule apart anddisperse the spheres as we were sitting and talking.”

“After meeting Ian, one of my sisters decided to do her Year 12 science project on Cystic Fibrosis, and so I learnt a bit more about the genetic mutations then and the physical effects that weren’t that obvious to me at the time.”

Four months after the two met, Ian received his call for transplant. “I was in shock, worried but extremely happy for him. I walked around in a fog for the entire day. When I got to the hospital the next day, one of the staff who knew us both well grabbed my hand and pulled me down two flights of stairs so I could see him in ICU. Ian was sitting up, he looked a bit swollen but very pink, and he had a huge grin on his face. Holding his warm hand was amazing. He only spent 24 hours in ICU; he boasts that was a record.”

“I visited him every day on the ward and saw how quickly he progressed, and despite a faulty epidural and other problems, he was cheerful. I think seeing the process of transplantation first hand was enormously reassuring; I was there the day he had his first and only bout of rejection, he was practically sitting on top of the heater because he was shivering from fever. There were procedures and setbacks that I witnessed that helped me to quickly put things in perspective when they later happened to me.”

Claire received her call for transplant seven months later. At the time, Ian had returned to Perth. “I came around four or five days after my transplant in the ICU, so I woke up to the news that Ian was on his way over. He spent two weeks with me, and it was lovely, I learnt then that he would love me through anything.”

On returning to Perth, Claire experienced health problems. Twelve months went by, and the cause remained mysterious. “My father (a consultant physician) suggested I go to Melbourne for help. We stayed for three months initially, but they identified the problem within days. It seemed a simple decision (apart from leaving loved ones) after that to make the move.”

“I was worried about moving to Melbourne and away from family support when I was hospitalised, but I needn’t have been, I was sick or close to dying for most of last year, and Ian just seemed to know exactly what to do and what I needed when I needed it. Ian introduced me to a world in which there could be wordless communication about how one was feeling and what one had experienced, not just between us but to others, mainly people with CF, who had similar experiences. I seemed to be always accepted as not having CF but still part of the CF community. Luckily there are few times when Ian and I are ill at the same time. I think it makes things easier; we don’t have to explain a lot of things to each other.”

“The physical effects of CF can be horrendous, and transplant does not solve them all, in Ian’s case, it has made most of them more bearable. Ian claims CF as a large part of his identity in a positive way and often points out that his various character traits do have a connection to the fact that he has CF. He showed me so many times how to deal with illness with humour. He very rarely complains. He is generous in many ways and has overcome so much that I am in awe of him.”

The generosity of two courageous families who decided to donate their loved one organs changed both their lives. Combined, the extra time Claire and Ian have been gifted equals 46 years.

Ian is a renowned Cosplay photographer, you can follow him on Instagram

31 Days of Cystic Fibrosis. May 27
Aidan Fraser: A Husband’s Story

Sharon Lawrence I Clinical Nurse Consultant

Statistically, CF patients as a group, have a longer median survival post-transplant compared to other lung transplant recipients. Sharon Lawrence explains the many factors that contribute to this.

“Many people with respiratory problems are referred later in life, and come to end-stage respiratory disease with comorbidity factors. CF patients can be referred any time between the ages of 10 – 50. Although CF patients are living longer and better, and their age to transplant is becoming later, they are still the youngest population of patients that come to transplant.”

“CF patients have life-long managed their illness. When they get to transplant, they understand the reality of changing treatment options from one illness to the treatment options of another. They don’t come to transplant with the expectation of miraculous results.”

“Most CF patients take to complex medications regimes quite well, and although there are somewhere between 10 – 15 medications they need to take, it’s not as aggressive as the medication plan they were on pre-transplant with the nebs and all that chest physio. Treatment is not as onerous- and they get to live their life between morning and evening medications.”

“CF Patients take opportunities they didn’t have before, now that they don’t have the burden of the lung disease component of CF – yes they still have CF, but not the treatment burden that CF lungs require. I enjoy transplant for CF patients because of the benefits and because of what it means to them and also for their families.”

31 Days of Cystic Fibrosis. May 28
Steph Anderson: A Friend’s Story

Steph Anderson and Neely began their friendship in high school. “Neely was always open about her CF. She was coughing one day, quite severely. I asked why? She said quickly. I’ve got CF, and I’ll be dead by the time I’m 27. So I went home, and I googled it.”

As their friendship developed, the pair started hanging out outside of school. “We were at Neely’s house one day after school and Lappa, the physio, showed up and set up the table.” Steph still recalls the shock she felt. “The next thing he’s beating the crap out of her back.”
Steph had health problems also, and on occasion, the pair would even be in PMH at the same time. However, it was when the girls were in their twenties that Steph first noticed the decline in Neely’s health. “Neely started saying she couldn’t go out because was feeling unwell or had landed in hospital. It wasn’t until the subject of organ donation came up that it really hit home.”Five years after Neely’s lung transplant, Steph underwent a kidney transplant. “Neely was a great help explaining the different medications and what side effects came with them.”While Steph’s new kidney was functioning well, Neely was undergoing a tough battle. After surviving bowel cancer and it’s various complications, the medications, Neely required to prevent her lungs from rejecting caused damage to her kidneys.The last year of Neely’s life was an emotional journey. “Me having the healthy kidney, and her needing one, made me feel survivor’s guilt, I wish it was like a liver so I could have shared it with her.”

“I’ve had friends die before, but nothing impacted me like watching Neely. I could do practical things, be there and listen to her. After TAFE I’d go straight to the hospital and leave at 2 am. She fought hard, but she was also my age. I knew she wasn’t going to have a long life, but she should have. She should have had the chance to have children and grow old.”

Steph continues to fundraise for CF, recently participating in the HBF Run for a Reason and selling roses on Sixty Five Roses Day. “I fundraise as a way of working through my grief.”

“The world is a different place without her. Sadder. She’s left a massive, massive hole.”

You can read Neely’s journey in her own words here

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 5 May 19th – 23rd

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 19th – May 23rd 2019

31 Days of Cystic Fibrosis. May 19 – 21
Barbara Jordan: A Mother’s Story

Barbara Jordan worried her way through her second pregnancy. Her first child, Melinda, had been diagnosed with CF when she was two months old. Melinda’s diagnosis came hot on the heels of the news that a relative in the UK had lost her son to CF.

Diagnosed with CF at birth, Julie’s survival was initially predicted to be less than 48 hours. Despite the dire prediction, Julie did survive, and soon, Barbara’s daily routine saw her deal with the medical requirements of two children with CF.

Barbara knew life would be busy with double the number of treatments and hospital visits. “I thought they would help each other understand what they needed to do, and I do feel that having the two girls close together was helpful. They would talk to each other during treatments. The girls had a great connection because of their shared experiences.”

Julie always struggled more than Melinda and subsequently spent more time in hospital. “Melinda was maybe about eight when she told me that I loved Julie more. I needed to explain that Julie was sicker, and consequently needed more attention.”

As a young adult, Julie’s health continued to decline, and eventually, she told Barbara that the team at Sir Charlies Gairdner Hospital, headed by Dr Gerrad Ryan, had recommended Julie as a candidate for a transplant.

With Julie on the path to transplant, life became more daunting for Barbara. The move to Melbourne, while Julie was waitlisted for transplant, meant Barbara needed to leave Melinda behind. “Melinda was living her own life, and I knew that this was the best thing for Julie.”

Initially, on their arrival in Melbourne, Barbara and Julie were across the road from the hospital. Later they shifted into a CF house. Barbara describes the night Julie received the call to come to the hospital for transplant as a moment that was both frightening and filled with happiness.

Extensive roadworks caused one of the main roads to be closed off. Once Barbara explained the situation and the urgency of getting to the hospital, the workmen let her though, and she continued on to the hospital driving on the wrong side of the road.

Barbara felt as if a weight had lifted from her shoulders when she received the call informing her that Julie was out of surgery. “I was over the moon,” Barbara said.

Julie’s recovery went well; Barbara describes her recovery as “Unbelievable. She improved so much. She was breathing and laughing and not having difficulty with any of it.”

Until a rigid bronch, a procedure that visualizes the trachea and proximal bronchi, was performed. “I received a terrifying phone call in the middle of the night. The doctors put Julie under too far. They had to use the paddles to bring her back to life.”

Despite the setback, Julie was soon ready to return to Perth. Once they were home, Barbara discovered Julie didn’t ‘fit’ into the system anywhere. “Julie was no longer being cared for by the CF team, and it was hard to get the new team to listen.”

Then Julie’s new lungs showed signs of rejection.

“We were in clinic, and the transplant team told Julie there was nothing they could do for her. Julie was asked if she wanted a second transplant. She said, “No. No. I don’t want to put myself, my family, and my friends through what I’ve just been through again. With no guarantee that it is going to be successful. So no. Take me off all my medications and let me go peacefully.”

“They were her words, her choices; she was an adult, it was her body; it was her decision.”

“Melinda moved to NSW – it was hard because it was like I lost both girls at the same time, but I knew that was what Melinda needed to do. She had a dream to have a family, and it wasn’t my place to stop her.”

Barbara Jordan describes the news that Melinda was pregnant as initially devastating, but also exciting. Her main worry was how the pregnancy could impact Melinda. “Back when the girls were diagnosed with CF, I never would have dreamed that I could become a grandmother. Melinda went through so much to have Luca.”
For Barbara, Melinda’s transplant journey was different to the hands-on experience of Julie’s transplant. With Melinda living interstate and married, Barbara now relied on the information that was passed on. “I hadn’t realised quite how bad Melinda’s decline had been. I saw Melinda right before she went in for the first transplant. Seeing her sit up with colour in her face afterwards was just unbelievable.”After a rollercoaster ride, with multiple hiccups, Melinda required a second transplant two years ago. “Melinda’s a stubborn girl, and I know she’ll fight her way through whatever CF throws her way for as long as she can. She wants to see Luca marry and have babies all of her own. I know Melinda will be alright.”“Luca has so much of Julie in her it’s incredible to look at. And that is beautiful.”

 

31 Days of Cystic Fibrosis. May 22
Karen De Lore – Business Development Manager, Cystic Fibrosis WA

Karen De Lore came to CFWA with some knowledge of CF, and how the disease was managed. “I felt an empathy for the CF community before I arrived, I thought working for CFWA would provide a cause I could be passionate about. And it is easy to be passionate about CF. Although CF is chronic and awful, the CF community is great.”

As Business Development Manager, Karen is responsible for both fundraising and marketing awareness of CF. “Fundraising, by its nature, also brings a lot of awareness. Both the 65 Roses campaign and Orkambi campaign were targeted towards raising awareness of the battles that families affected by CF face every day. These campaigns bring increased awareness towards understanding the challenges people with CF face in their life.” Karen said.

For people with CF, there is a huge benefit to offering services as an adjunct to hospital services. These focused services not only help relieve the burden on the hospital system; they directly improve the lives of people with CF and their families. CFWA provides additional services on top of those paid for via the Health Department contract – that’s where Karen’s role is vital. Without fundraising, CFWA can’t deliver focussed services in an effective manner.

“Working in the not-for-profit sector in this climate is challenging – asking for money from any part of corporate WA or from the community is sometimes hard – it makes it a battle to secure new funding. Sometimes people want to give money for specific purposes. However, both caring for people with CF and providing funds for research goes hand in hand. It’s no good channelling all the money to research while people are battling and need service delivery.”

31 Days of Cystic Fibrosis. May 23
Aidan Fraser: A Husband’s Story

Meeting Jackie was Aidan Fraser’s first introduction to CF. “Jackie was coping with CF quite well, and it wasn’t evident at all that she was sick. So I was probably a bit naive toward the whole thing . I never imagined it would be this hard, difficult or sad – mostly due to my ignorance as to what CF really was.”

The journey for Aidan has been a learning curve between new routines and methods that can help improve Jackie’s quality of life. “It’s something we deal with and adapt to. This is the new ‘normal’ for us, and you do what you have to do.”
The reality of Jackie’s medical team recommending her for transplant knocked Aidan for six. “It was like being hit with a bat! To hear the words is something you can’t prepare for. Especially when you felt it was still some time away.”

A double lung transplant is not a cure for CF; it is a procedure that trades one set of health problems for another. In an effort to improve Jackie’s quality of life, the couple decided to postpone being waitlisted for transplant while they put in a concentrated effort to focus on Jackie’s health.

Right now, they’re in the midst of a trip around Australia with their dog. It’s a journey they’re taking day by day, constantly reassessing Jackie’s health.

“I can’t explain enough how dealing with CF has changed and enriched my life. Jackie has made me see the world in a different way than I did before knowing her. The biggest thing being compassion and kindness. Without a doubt, she is the kindest, most compassionate person I have ever met. Jackie’s zest for life is contagious, and she has passed all this on to me.”

You can follow Aidan and Jackie’s journey on Instagram

 

 

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 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