After being diagnosed at a very young age and in spite of a bleak prognosis, Justin Wall has endured and ultimately survived cystic fibrosis. Culminating in a bilateral double lung transplant in late 2016, his has been a journey of defying people’s expectations. Justin discusses the inherent challenges of living with and surviving cystic fibrosis and shares insights into his treatment journey, presenting the case for personalised care and a sharper focus on the patient’s experience.
Tell us a bit about your story and the impact of cystic fibrosis
Being born in the early 1970s, the prognosis for people with cystic fibrosis (CF) was grim. At the time, the mean life expectancy was just ten years of age in Australia. My origin story is a little different than many others with CF. I was adopted at approximately two years old after being born in New Zealand. I since discovered that my biological mother, due to life and family circumstances decided to give birth to me in Auckland as I was the result of an unplanned pregnancy and, to avoid her family finding out, decided to move to New Zealand. After discovering I had CF through the heel prick test, she was advised that two options were available — live in New Zealand to raise me or return to Australia to put me up for adoption.
My adoptive mother (hereafter known as mother/mum) worked hard, embracing long percussion hours to help clear my lungs and airways, to ensure I thrived as a child. My lungs were not impacted at this stage, and I could play as any other child would. However, the pancreatic enzymes of the day I was prescribed, to combat pancreatic inefficiency, meant taking about 20 medications per meal (along with other vitamins). As a school-age child wanting to fit in, I often avoided taking them, resulting in the malabsorption of food. As enzymes became more efficient and requiring fewer to take, I developed excellent sleight of hand skills to consume them while no one noticed.
Throughout my childhood, no compensations were ever made to me because of my CF. I was treated just as my brothers were, without exception. I attended school, played sports, and participated in activities any child of school age would do. My mother had arranged my medications, specialist appointments, and any other treatments, but that all changed when I was 15. I was told that my parents would not be around forever and that I should start making my own appointments, ordering medication, and attending doctors by myself. At the time, I thought this a great injustice. With hindsight, however, I can see it made me into a resilient adult; many of my peers who have survived into their 40s shared similar experiences.
The most significant challenge I faced with my CF was the mental battle. When I completed high school and my Associates Diploma in Computer Science, I began working in the family business. At 21 years old I suffered a secondary spontaneous pneumothorax (SSP), requiring a chest tube thoracostomy and supplemental oxygen. This was the first time I had been hospitalised. The experience was traumatic and scarred me for further admissions.
After moving to the Northern Rivers region [of New South Wales, Australia] in my mid-20s, I required more frequent hospitalisations for antibiotic (AB) treatment. It was during this time that I experienced the loss of a CF friend for the first time. I have always been fortunate to be sitting at or above the mean age of survival for a person with CF. While I wanted to undertake further studies and expand my career options, I had it in my mind that I would be dead by 30. I focused on living my life to the fullest with little regard for the future. It was only when I reached 30 that I had the epiphany that I could be around for a while and decided to attend university, completing a Bachelor of Social Sciences.
Prior to completing my undergraduate degree, I always thought that having CF would preclude me from working in a ‘proper’ job. Part of this came from the fact I didn’t see many of my peers in full-time work. Despite having a stable FEV1 (% Pred) of 35% (see figure below), I had heard stories from other people with CF being made redundant when they became ill.
Despite this, I sought the security and appropriate legal protections of working in a not-for-profit. As a side note, it was watching the British version of The Office that helped me decide to work in that environment! I found the workplace very supportive. I based hospital admissions around annual leave and planned sick leave until I resigned due to prolonged ill health and the need for a life-extending bilateral double lung transplant.
By and large, having CF has not hindered me from forming and maintaining interpersonal relationships. My friendship groups have always been small and tight, always looking out for each other, and I learned to counter any negative feelings with humour. Self-deprecation goes a long way to breaking down perceived barriers. While my first relationship was innocent, it was the attitude of her parents that planted self-doubt. She told me her parents exclaimed, “Why are you with him? He’ll be dead soon!” This statement stuck with me for some time and significantly impacted my self-esteem. I built a protective wall and purposefully avoided romantic relationships for some time.
You’ve worked with different health professionals throughout the course of your CF treatment. Can you talk a bit about this, and particularly the allied health support you’ve accessed?
I‘ve worked with several allied health professionals as an in- and outpatient; the patient can have all the health services and groups at their disposable to provide care, but the onus falls back on the patient to listen to their advice. As an inpatient, my primary interaction has been with physiotherapists. Physios have been vital to regaining pulmonary function during admission, keeping me up to date with the best airway clearance techniques. When I was younger, and my BMI was <20, a CF dietician would consult me on strategies to gain/maintain weight. As my lung function decreased and my resting heart rate increased, it was imperative to eat appropriate meals and snacks to combat weight loss through increased energy expenditure and maintaining oxygen supply. On occasion, I sought counsel from a social worker; however, in my experience, I did not require them frequently.
What difference did these AHPs make to your quality of life?
I found the allied health team to be a critical factor in my physical and mental care. Of all the allied health fields, I had the most extensive interactions with physiotherapists. The physios’ role was to work with other teams to improve my lung function and build strength. Their unofficial role was as a friendly ear to listen. I formed close patient/health professional relationships with many of the team over the years. Two of the physios, in particular, joined the lung transplant team some time ago, and it was highly reassuring when I went down that path to see familiar faces.
Being able to maintain professional boundaries but still engage meaningfully with patients is a crucial skill for any health worker, in my opinion. People with chronic conditions like CF will return to the hospital often. Maybe one a year for a week initially, but as the disease progresses, the frequency and duration of stays can feel all too consuming and become your life. In my experience, I did not have my first hospital admission until I was 20 years old. By the time I was listed for transplant, I was spending up to six months of the year in the hospital. My family do not live close by, and often it was the regularity of physio appointments that gave me something to look forward to.
This is not to say other allied health teams did not participate to the physiotherapist’s extent. I found that dieticians would empower me with small tips and tricks to consume extra calories in each meal and assist in developing meal plans based on the foods I liked (everything!). While not impacting me, it is essential in the lead-up to transplant to have a good BMI.
As your appetite may be reduced, it allows some give if you find eating difficult. This was the case for me. The week before I received the surprise news of a suitable donor, my lung function was so poor that it was decided to stop spirometry testing. In addition, my severely limited lung capacity didn’t allow me to eat and breathe simultaneously — the choice was to eat or breathe.
What would you like healthcare professionals to know about supporting people in similar circumstances (ie- being the expert in your management)?
The relationship between healthcare professionals (HCPs) and the patient has evolved in my experience. When I had my first hospitalisation in the early 1990s, the HCP approach could be described as directorial — you were told what medications to take and when without any explanation as to the why. Over time, HCPs consulted with patients as to what course of treatment to undertake. When I felt empowered as a patient and was allowed to have a voice in my own treatment, adherence to regimes improved.
Each patient is an expert in their own body, so I firmly believe HCPs must listen when a patient advises that something doesn’t feel right. One of my long-term concerns has been the reliance on quantitative measures to determine health — blood pressure, spirometry, oximetry, etc. In my opinion, qualitative criteria have been overlooked or under-investigated. The mind/body connection is well established, and, in my experience, if ‘the numbers’ are ok, many HCPs will be satisfied. I have advocated for mental health screening as part of the standard diagnostic tools.
As a self-aware, reflective person, I feel there has been a connection to my actual or perceived level of anxiety before spirometry testing and the subsequent results.
In my experience, I have long felt a distinct lack of mental health care for people living with CF exists. I get it—on paper, it is a highly complex disease that extends from children and adolescents to adults. Each life stage requires a different approach to mental health support. I am self-reflective by nature—I began requiring a hospital admission more than once a year and felt a mind/body connection. Remember that this was a long time before the amount of mental health support services proliferated, as witnessed in recent years. There was also no social media.
To start the conversation, I approached the CF team social worker. Initially, my discussions with them were to complain about the medical doctor’s focus on the quantitative, solid numbers from all the tests. I felt we were being identified by a number and not as individuals with life outside the hospital. The response was mixed: while acknowledging my concerns, it was a funding issue to employ a dedicated psychologist. I understood the health economics and the high cost of care for CF patients. Unfortunately, CF isn’t a ‘sexy’ disease; it’s not widely reported in the media. If mental health concerns were raised, it was suggested to seek an external referral to mental health support.
Until about a decade ago, many inpatients with CF would share a four-bed hospital room unless acutely ill. While this was not the ideal situation due to the increasing awareness and understanding of how far and long cough particles can travel in the air, increasing the risk of cross-infection, it was also an opportunity for people with CF to support each other on a peer-to-peer level. While the medical and allied health professionals would attempt to provide information, many CFs felt skeptical as they did not live with the disease. The informal peer support networks were an opportunity to discuss procedures, health issues, and treatment methods openly.
When a dedicated CF ward was built, all CF patients were in single bedrooms with dedicated bathrooms. On the face of it, this was a massive step forward for CF care. However, many CF people I spoke with said they missed the peer support. During this time, I began to ruminate over ways to improve connectedness among CF patients, not just in Queensland but throughout Australia and the world. From the peer discussions, I established that many people with CF were creative in their own unique way as a coping mechanism for the dark side of CF. In fact, at this stage of my life, I have known more people who die from CF complications than I know alive. Decorative tattoos were a very popular expression of creativity. Whether it is to acknowledge someone special that died, have a CF-inspired tattoo to ‘own’ the illness, or in other cases, it was receiving pain on their terms. I personally have tattoos that fall into some of those categories.
To showcase the creativity of the CF network, I received a grant from Vertex Pharmaceuticals (a significant developer of CF-specific drugs) to develop a website I called the ‘CF Creative Space’. It was a user-driven platform that encouraged people with CF, their family, and friends to post images, stories, audio recordings and other forms with a story behind them. The positive feedback I received cemented the concept of embedding a mental health professional within the allied health team.
Shortly after the website went live, with submissions being posted from the UK, USA, and Australia, I was advised that I was one of seven global winners from 35 submissions and requested to present the CF Creative Space at the Vertex annual conference in Orlando, Florida. Unfortunately, I could go as I was too ill. Little did I know I would receive my life-extending bilateral lung transplant only a couple of weeks later. My social worker went in my place. After I received my transplant, I switched care teams, but I have heard there is a psychologist on the team now. I’m not sure of the particulars, but this is a huge step toward my vision.
What have the AHPs you’ve worked with learned from you?
Not all patients are the same. It is essential to build rapport with the patient for trust to develop and for cooperative care to begin.
In terms of your allied health experience, what would you like to see done differently?
Cystic Fibrosis is a chronic disease with lifelong impacts on physical and mental health. The physical components of the disease that are measurable (spirometry, blood pressure, vitamin levels etc.) provide a detailed snapshot of the patient at a point in time. These results are measured and compared longitudinally to show the progression of an individual’s disease. Unfortunately, mental health plays a significant role in the advancement of the disease. I feel a stronger focus on the treatment and management of depression and anxiety disorders associated with CF could result in improved health outcomes related to food intake (impacting BMI), exercise, and medication adherence.
Allied health professionals should avoid applying a generalised treatment regime or having preconceived notions of the patient group. Respect the patient and be open to allowing and encouraging the patient to have a meaningful contribution to their treatment.
Having a condition such as CF has presented me with the opportunity to meet a diverse cohort of people who are living life to the fullest. While CF does present challenges, it has made me a more resilient person. I believe it is dangerous to (self)label a person as doing well or they are inspirational despite having CF. I maintain that I live my life alongside the disease. It is a constant passenger that I have learned and adapted to live with to live my life.