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