Most people who have flown internationally in the past few years are aware of the…
The year is 2006. It’s late on a Wednesday afternoon in early February and we (my husband, Cameron, two years, and Todd, just shy of 12 weeks) are driving to visit my in-laws, when I get a call from our family doctor.
He tells us we need to come in urgently and he will stay late to see us that evening. We turn around and head for the doctor’s office. We get ushered straight in on arrival and he tells us that Todd’s heel prick test results are inconclusive, but he most likely has cystic fibrosis (CF).
My husband and I look at one another, wondering if we are supposed to know what this means. The doctor has continued talking but I only hear little bits of what he says. Genetic disease, life-threatening, no cure. The next thing I remember is the doctor giving us pathology requests for further testing, having made us an appointment for the next day.
We leave in a daze, eventually deciding this is just process and the results will show everything is fine. Friday afternoon the doctor calls with positive results. There is no doubt our son has cystic fibrosis.
Monday afternoon was a complete blur. We arrived at Canberra Hospital in time for our 1 pm appointment and it was well after 5 pm when we finally left. We got shuffled from room to room, seeing doctors specialising in respiratory and gastroenterology, as well as a physiotherapist, dietitian, nurse and genetic counsellor. All giving us an overwhelming amount of information and treatment outlines for the worst case scenario.
In less than a week, our normal, regular lives had been turned into utter chaos.
I was overwhelmed, stressed and most likely depressed, suffering in silence and not telling anyone. I went through the daily motions to get through life with two young children which now included an overwhelming amount of treatment and medications.
I refused to leave Todd in anyone’s care or go out on playdates in case he became sick from being exposed to germs and bacteria. I even removed Cameron from daycare fearing he would bring home some illness that would make his brother sick. I often found myself crying, grieving for my child who was still alive but had this terrible diagnosis. Some six plus months later I was finally getting a grasp on what was to be our new normal.
In the first 12 months after diagnosis we had monthly appointments at the hospital with our specialist care team and since then every eight to 12 weeks, depending on his health.
After that first appointment, Todd started on a concentrated formula, vitamin supplements, percussion physio, pancreatic enzyme replacement, preventative antibiotics and various other supplements all aimed at increasing his weight and keeping him healthy.
Todd suffered from many chest infections resulting in him needing to take antibiotics regularly. Todd was 21 months when he was first admitted to hospital for two weeks to have IV antibiotics (a tune-up) to hopefully clear the chest infection that had been plaguing him for around nine months. This was difficult for all the family.
Todd was a regular toddler full of energy and always on the move, the constant infections never slowing him down. Trying to keep him confined to his hospital room, attached to the drip/IV pole was impossible!
When I discovered I was pregnant again in November 2009, my GP recommended we do in-utero tests due to the family history.
The results of the tests showed I was pregnant with a boy and he too would have CF. Initially hearing the news over the phone I was shattered. I called my husband and told him the news. After a few moments of silence, almost at the same time we said variations of ‘We’ve got this, we know what this means, we can do it’.
We spoke with the specialists about what it would mean having two children with CF in the house because sufferers of CF are not supposed to have direct contact with other sufferers due to the risk of cross infection. They told us being full blood siblings that the baby would have a similar degree of CF to Todd and if one got a lung infection the other would likely have the same infection within a week or two.
Nicholas was born in June 2010 and nothing prepared us for the weight he would lose in the first five days. Nicholas lost a massive 630g or 16% of his body weight. We rang his specialist and Nicholas immediately started on pancreatic enzyme replacements, concentrated formula and vitamins. The weight Nicholas lost in just five days took over three weeks to put back on.
As the boys get older we are learning that their CF differs from one another. Todd struggles with appetite and weight gain and minor lung infections needing common antibiotics (although any lung infection is serious and can lead to complications). Nicholas loves food, gains weight well but he suffers from more frequent and serious lung infections requiring stronger antibiotics.
In recent years they have started going into hospital for tune-ups together. It’s a good thing that they have each other as CF can be isolating due to the risk of cross infections.
CF sufferers have a persistent cough even when they are ‘well’. Todd and Nicholas are no different and with each cough you wonder is there an infection causing this cough that may land them in hospital. You ask yourself, ‘how much damage is being done to their lungs?’ You sleep at night with your ears open, listening to every sound they make. There is no rest from CF.
Life with CF is a constant roller coaster of emotions and learnings, you just get on top of and ‘comfortable’ with one aspect of treatment and then you are faced with another challenge.
In late 2014, Todd hit a growth spurt, but his weight remained static and his BMI dropped to 17. We tried increasing the amount of calories in his food, with little result. He continued to grow taller but his weight gain couldn’t keep up. We decided the best course to ensure Todd continued to grow as normal as possible through puberty and teen years was that he would need a gastrostomy.
The surgery was scheduled for June 1 2017, but Todd was admitted two weeks prior fortune-up up and nasal-gastric feeds to combat the possible effects of the procedure. The procedure means that Todd can now have overnight feeds (we’ll have these six out seven nights), giving him an additional 1350 calories a day. Todd’s weight is slowly catching up to where it needs to be and he’ll continue these feeds through his teenage years, most likely until he stops growing.
We try as best we can to provide normal experiences and have regular activities. For us, it’s swimming and BMX and Mountain Biking that we fit in around the necessary and essential treatments. It also includes going to concerts or sporting events, weekends away, longer family vacations and, of course, school camps.
This is just some of what we have been through. The impacts aren’t just on the person with CF, it has affected our whole family, friends, teachers and our broader community.
The future is unknown. There may one day be the need for a transplant or other procedures due to complications from CF. But all we can do is live for today and hope that a cure is just around the corner.
I remind myself daily not think too far into the future, to make the most of today because who knows what tomorrow will bring.
Cystic Fibrosis ACT’s annual fundraiser, the Santa Speedo Shuffle, takes place on Sunday 29 July from 10 am at the Southern Cross Yacht Club, Lake Burley Griffin.
For more information on how you can get involved and make a difference to the lives of Canberrans living with CF, visit santashuffle.org.au.