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

June 7, 2019July 6, 2019 / Sandi Parsons / Leave a comment

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 3 May 10th – 13th

June 3, 2019 / Sandi Parsons / Leave a comment

May 10th – May 13th 2019

31 Days of Cystic Fibrosis. May 10 & 11
Daniel McKeon: A Husband’s Story

Daniel McKeon thought he had an understanding of the practicalities of living with someone with CF from his friendship with both Melinda and her sister Julie. However, Daniel realises now that everything he thought he’d known about CF was nothing compared to the reality. Daniel said, “I went in blind. But you get through it.”

At first, the focus was more on the daily regimen of physio, nebs and tablets, as Melinda was only admitted to hospital on average twice a year. Daniel describes this time as manageable without a huge impact on their lives. “It was minimal compared to now.”

Daniel is frank about their decision to start a family; he didn’t want to. To Daniel, the benefits outweighed the risk, but only marginally. “I said no. I knew how much of an impact it could have on Melinda. But I got outvoted because Melinda’s vote counted more than mine. We went through a lot to have Luca. It was a big risk, but Melinda is a stubborn woman.”

“When Luca was four it was obvious that Melinda was headed on a path that lead directly to transplant,” Daniel said. Melinda spent the majority of the year before her first transplant in hospital, but as Luca had grown up visiting her Mum in hospital, this time faded into the background for her. “When I told Luca her Mum had new lungs, her first question was to ask if new lungs meant she could have a sleepover with Aunt Jaki.”

Melinda spent the year following her transplant in and out of hospital with numerous hiccups along the way. As Luca got older the impact started to get bigger. “Luca was too young to comprehend what was going on then, but with the second transplant, nearly two years ago, she was older. Luca knew what was happening, what the consequences would be if the second transplant didn’t happen.”

“We never kept anything from Luca, always answered any questions. She knew what was normal and what wasn’t. We’ve always said, if Luca’s got questions, we’re going to answer them.”

Daniel and Melinda McKeon

For the eight months leading up to Melinda’s second transplant, the McKeon’s couldn’t do much as a family. Melinda was dependant on oxygen and spent a great deal of that time in hospital. Although the mother-daughter bond remained strong, Daniel and Luca increasingly spent more time together, “We’d go on little adventures.”

During this period in their lives, Daniel saw a different side to the work charities perform. Now, he volunteers his time to help others, as a way to give back, like as his family has been helped. “People have always been willing to help out. You see a different side of people when you’re going through hell. You get to know who the good people in your life are.” Daniel said.

Financially, life post-transplant is harder, due to the volume of medications that Melinda requires. Dealing with Centrelink is frustrating, time-consuming and mostly pointless. “It’s not about the money,” he said, “We need a Health Care Card.”

Daniel wants automatic access to a Health Care Card without having to justify the need to Centrelink on an on-going basis. Here, Daniel is not alone, despite calls from Cystic Fibrosis Australia for all people with CF to automatically qualify for a Health Care Card, there have been no changes in legislation by the government.

Daniel’s advice to someone contemplating a relationship with someone with CF us “Don’t fear the commitment to someone with CF – because yes it will impact your life, but you’ll get more positive than negative out of it. But get ready because it’s quite a journey.”

McKeon Family

31 Days of Cystic Fibrosis. May 12
Jamie Wood – Senior Physiotherapist, Cystic Fibrosis

While doing his junior rotations as a Physiotherapist, Jamie felt drawn to the respiratory rotation, and in particular, he found himself interested in working with people with CF.

The ability to treat people who all have the same condition appealed to Jamie. From a physiotherapy perspective, the age group and fitness of people with CF differ from other respiratory patients. For example, in the general population, lung disease often manifests later in life and can have a quick impact on mobility and the ability to function at home.

In contrast, in people with CF, the role focuses on integrating the physiotherapy they need – whether it is airway clearance or exercise into their lifestyle. CF doesn’t only impact the lungs, it affects most body systems, and this is a challenging aspect to Jamie’s role as a physiotherapist – even after 10 years, Jamie says he’s still learning about CF.

Another challenge when treating people with CF is that they understand their disease very well, which can at times be a double-edged sword. Outdated and incorrect information in abundance is freely available on the internet. It’s therefore critical to include people with CF in any decision making about their treatment. If you don’t manage to do that well, quite often the treatment plan won’t benefit them as much as you would hope

Occasionally, Jamie sees people with CF that “Try really hard, try their guts out, and still inevitably they get unwell.” And there are always those who manage to pull something out of their pocket. Jamie says, “I was giving someone a hard time about doing more airway clearance, and he got a new best FEV1, jumped up and shouted ‘suck it!!’”

The trade to these challenges are the rewards of working with people with CF. From a macro perspective, providing education about the need for airway clearance and exercise is a critical factor that can make a positive impact on the lives of people with CF.

On a micro level, Jamie can provide someone with day to day tips to keep progressing their airway clearance and exercise along the way. The long-term relationship allows him to layer these tips and strategies more comprehensively than a single session would allow.

However, the ultimate reward of Jamie’s role is seeing people with CF go on to achieve their goals. “To be told you have made a positive impact on someone’s life is pretty special.”

Bonus Fact: If you ever have a spare half an hour, Jamie will happily spend it explaining why exercise is so important for people with CF.

Jamie Wood

31 Days of Cystic Fibrosis. May 13
The Last Walk

Some of the profiles posted this year have highlighted the incredible medical staff that I have been fortunate to meet. Staff who over the many years have turned a blind eye to shenanigans or actively participated. Staff who have brought home-cooked meals, or sat with me in the middle of the night simply because they can.

The Last Walk is not only an #ourvoices story but a thank you to some of the glorious staff that have made hospital admissions easier.

The character of Saanvi is a nod to everyone involved in the care of CF patients from the days of B12 right through to G54.

Saanvi is Sue and her passion for ensuring that Ward B12 always ran like clockwork, that her CF patients lives are made a little easier by cutting through red tape, and who has an uncanny intuitive ability to provide me with what I needed before I knew I needed it.
Saanvi is Pushpa with her eternal night shifts, Tiger Balm, midnight toast and hot chocolate.
Saanvi is Yvonne with her water fights and jokes.
Saanvi is Michelle & Alison and their long chats, which provided distractions at precisely the right moment.
Saanvi is Jamie who carried oxygen bottles with ease, and pushed me to do my best and always managed con me to walk another lap of the ward.
Saanvi is John and his gentle hands as he changed first my PICC, then my infusport dressings.
Saanvi is ’Uncle’ Gerry with his empathy and humour.

Saanvi is a nod to acknowledge those people who are more than medical staff as they became an important part of the lives of people with CF.

And as with every fictional story based on fact, the truth is way more fascinating than fiction, and I had to leave out far more than I included – I’m not sure anyone who didn’t live through the experience would have believed it!

You can read The Last Walk on CF Creative Space

‘B’ Block Sir Charles Gairdner Hospital – Photo by Sonia Sears.

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 2 May 6th – 9th

June 2, 2019June 2, 2019 / Sandi Parsons / Leave a comment

May 6th – May 9th 2019

31 Days of Cystic Fibrosis. May 6
Kohn Rootsey: A Husband’s Story

John and Kate Rootsey have a normal family life with their six-year-old daughter, Molly. Like any family, they are busy day-to-day taking Molly to her various activities and often go away on holidays – but having a family wasn’t originally part of their plans.

When John met Kate after her double lung transplant, he was unaware of CF and its implications. He asked all the common questions. Kate told him that despite her transplant CF complications remained – in particular in the digestive system , and that due to her transplant the possibility of children was unlikely. Furthermore a life with her would present challenges, but John knew they could face them together.

However, with the blessing of the Lung Transplant team at Prince Charles Hospital, John and Kate embarked on a three-year journey to have Molly. It was new territory for everyone involved; as Kate was the first woman in Queensland to have a baby after a double-lung transplant.
As a high-risk patient, Kate was first required to change her anti-rejection medication. Immunosuppression levels need to be fine-tuned to continue to reduce rejection in the lungs and also be a safe level for foetal development. Their three-year journey contained numerous appointments, blood tests, and two miscarriages. John describes this period as a “Very scary and exciting time, as we weren’t sure how the possible outcomes could affect her.”

Having to deal with so many difficult situations has made Kate an extremely tough person, and John says it makes him not only appreciate both his own and his daughter’s health but also to make the best of any situation.

BONUS FACT: John’s top tip – Do NOT eat like a CFer.

Rootsey Family

31 Days of Cystic Fibrosis. May 7
Taryn Barret: A Mother’s Story

For the first 24 hours after Connor’s diagnosis with CF, Taryn Barret felt a mix of shock and disbelief. All Taryn knew about CF was “Horror stories, I didn’t think Connor was going to have a good quality of life – I’d look at him and picture the worst.”
Meeting with the CF team at Princess Margaret Hospital was a turning point, and she came to have a better understanding of the disease and its implications. It was after this meeting that Taryn could look at her son and think, “Yes, Connor is going to have a great life.”

To date, Connor hasn’t needed hospitalisation for IV antibiotics. Early on, Taryn established a routine to ensure that treatments were given priority and physio is completed twice a day. This non-negotiable routine has helped with Connor’s compliance regarding physio. “I hope this has attributed to him being really well, and while I know that could change at any point it’s something that works for our family,” Taryn said.

Some people with CF decline earlier than others, and while Taryn is optimistic, the death of a young person from CF always hits hard. In counterbalance, Taryn finds perspective from talking to adults with CF, “They just get on with things and have a positive perspective on life.”

Taryn feels fortunate that Connor has access to the breakthroughs that have been funded by the generations of research done in the past. “I feel like we are really lucky given that now there has been a turning point and there is so much hope for the young kids – particularity with the new Vertex Pharmaceutical medications – we’re not there yet but there is hope.”

Taryn is a committee member for Conquer Cystic Fibrosis; an organisation run entirely by volunteers. Taryn sees the need for research to be the primary focus in the fight against CF. She hopes there could be advancements that will enable Connor to have a long life, and even grow old. Conquer Cystic Fibrosis channels the majority of its fundraising monies into research therefore it was a natural fit for Taryn.

Through her charity work, along with her former work in both journalism as well as working for a Member of Parliament, Taryn is well aware of the power of advocacy. Orkambi is one of the latest medical breakthroughs and among the first of the drugs that treat the underlying causes of CF instead of the symptoms. However, inclusion on the PBS wasn’t automatic. Taryn’s passion for advocacy during the campaign to see Orkambi listed on the PBS saw her awarded the Patron Award for CF Consumer Advocate in 2017 by Cystic Fibrosis Australia.

Taryn, spoke at the Capel Vale Conquer Cystic Fibrosis Grand Ball 2017 – you can hear her speech here.

31 Days of Cystic Fibrosis. May 8 & 9
Chris Rowe: A Father’s Story – In His Own Words

We discovered Lauren had CF on the day she was born. It was an extraordinary 24 hours as we were on a high following her birth until we were told there was a problem. Lauren was transferred to the kid’s hospital for a bowel blockage, surgery and following that we were told by the surgeon to sit down as he had some news, that he was sure the problem causing the blockage was due to CF.

I hadn’t heard about CF, but Shayne (my wife) had and burst into tears. We had a very sick child due to surgery and other issues, then had to contemplate what this meant for the rest of Lauren’s life. Shayne had complications from Lauren’s birth, so we didn’t have much time to think, we said we would take one day at a time and have lived our lives like that ever since.

We pretty much accepted the diagnosis from the start and immediately switched into “what’s the next steps” and focused on how to get over each issue. The impact is strange as we know our family is different from everyone else’s yet I feel most people do not understand our situation. Some people may not understand why we still live in the same house, have not “progressed”. We decided that Shayne would not work so she can be more flexible to help Lauren.

When Lauren was in hospital, I would spend weekends in hospital to give Shayne a break, and your life is pretty much between work and hospital. It has also impacted our family unit with Lauren’s sister having to accept that a lot of the time she is in hospital and of course we have to comfort each other as there are times where we all get concerned about Lauren.

CF is not an acute disease as in when there is a medical issue; it is dealt with and then done. Instead, it’s a constant grind of issues and hospitalisations that Lauren has had to endure over 28 years. There turned out to be complications and surgeries that I hadn’t contemplated apart from the normal two-week hospital “tune-ups” throughout the year and of course the constant concern about her future.

Chris Rowe with baby Lauren

CF is an insidious disease that destroys young people. If I had one wish, I would like to have the power to do something to stop the lung deterioration and actually be able to reverse it. The worst thing as a parent is watching, to not being able to do something to help stop it, you feel powerless, and it is the worse thing seeing your child like this.

As a parent, you can stop a bleeding limb, help give Physio after a broken bone, pay for a trip to Disneyland if someone is sad, but I just wish I had the power to fix CF.

While I would not wish CF on anyone, however, I could not ever see our family being any other way. It has brought us closer than most families. I think the time we spend together due to hospital or illness does this. We sit around and joke, play games and are fairly irreverent when it comes to treatments or issues.

One day the nurse came to our house to pick up “poo” samples which went into a tin like a paint tin. She had dozens in the back of her car from her rounds and one day they exploded just as she was pulled over by the police. Another time when we were staying in the hospital the fire alarm went off. All the parents raced out with their kids, and each of us had our Creon Forte with us – always thinking ahead!

Lauren is a power of strength as well and watching her helping other CF people, and transplant people is inspirational. We may never have seen the strength in ourselves or Lauren or counted our blessings as much if CF wasn’t part of our lives.

***
Lauren Rowe is the founder of a not-for-profit organisation, Gifted Life, which offers support to transplant recipients, helps create support networks and endeavours to raise awareness of organ donation. You can find out more from Gifted Life

Chris and Lauren Rowe

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