Sophie is a young woman with cystic fibrosis (CF). She recently got married in Uluwatu, Bali. She is also the adult representative for the Child and Adolescent CF Consumer Reference Group of WA and a participant in research.
Have you participated in any research programs or drug trials?
In 2014/2015 Sophie took part in one of the research studies, which was being done at the Institute for Respiratory Health (IRH), to look at the safety and effects of an exciting CF drug which is used to treat the underlying cause of CF. The study she took part in was for people with the heterozygous F508del mutation.
What was the process like?
Sophie went through a screening process at the IRH. This included quite a few different examinations and tests, including CF genotyping. When she started the study drug she had regular appointments and tests conducted to monitor her closely, which included: blood tests, sweat testing, ECG, spirometry, pregnancy tests and urine samples.
The visits to the IRH were quite time consuming, but she was given the visit schedule in advance so could plan her time off work. She completed questionnaires at every visit which looked at her overall health, quality of life, daily activities and CF symptoms. She also had to keep a study diary card every day. Sophie advised that it was really interesting taking part in the study and the staff at the IRS were really friendly and kind.
Unfortunately, the results of the study she took part in did not show benefit for her genotype and the study was stopped early. While this was really disappointing at the time, she is hopeful that other studies will prove more successful for her genotype and new treatments will be available soon for all CF mutations.
What made you want to join the consumer reference group and what value do you see that the group offers to research and clinical practices?
Sophie is from the UK and prior to moving to Australia five years ago she was very involved with the UK CF Trust as an ambassador. She likes to raise awareness and share her experiences of living with CF. Joining the group has allowed her to advocate from a patient’s perspective. The group offers valuable insight into things that researchers and clinicians may not have thought of.
One of the benefits of the group is that they have the opportunity to highlight things from a family/patient perspective and outline important aspects that researchers and clinicians may have overlooked. She likes attending the meetings because having CF can feel very isolating at times and being around a group of people that understand what it’s like and being able to talk and share experiences is really beneficial.
Lastly, congratulations on your wedding. It looks like you had an amazing day. How did you manage to keep healthy in the lead up so you could enjoy your beautiful wedding?
Planning a wedding is a stressful time and she was anxious that she was going to get unwell and not be in her best health to be able to fully enjoy her wedding day. Sophie advised that she has been seeing a psychologist to help with her anxiety and managing her CF. She also found it really helpful leading up to the wedding as she helped her to work on some mindfulness and self-compassion strategies.
Sophie planned ahead with her CF team at Sir Charles Gairdner Hospital to have a proactive course of home IV antibiotics to give her a pre-wedding boost. She finished the course of IVs the day before she flew to Bali. Sophie advised that she has an incredibly supportive work place and her manager and colleagues helped her to reduce her case load so she could focus on her health leading up to the wedding.
While in Bali, the week before the wedding, she made sure that she kept up with all her treatments and exercise. She went running on the beach or swam in the hotel pool every day, and made sure she kept hydrated. On her hen’s day, her bridesmaids ensured that she had a break in the schedule for a swim, physio and nebs before dinner. On her wedding day, she felt really relaxed and had a lovely morning getting ready. She made sure she fitted in all her treatments prior to the wedding ceremony and then could enjoy dancing the night away! Sophie’s wedding day was a dream come true and she feels fortunate that the sun was shining, that she felt fit and well, and could fully enjoy the day.
Article from RED Magazine, Edition 4 2017.