My experiences of living with a CF sibling started long before Frances Ann was born, as I already had an older sister Claire who had CF. Unfortunately she lost her battle with the illness at the tender age of 6 years.  I was only 4 at the time.  Accessing my memories from that age is like looking though frosted glass, yet like tiny shafts of light there are bright vivid images, like photographs in the minds eye.   Strangely these images are not of illness or suffering…they are of two little girls telling tales on each other, playing games and getting into mischief; they are of everyday life, and for that I’m grateful. 

At age four I didn’t really understand what cystic fibrosis was and I did not connect the loss of my sister with this illness.   When Frances Ann was born the following year she was diagnosed with CF immediately and I thought nothing of it. Cystic fibrosis was my normality; Claire had it, I had it, so it made sense that Frances Ann would have it too.   Looking back I realise it must have been a devastating time for my parents but I was protected from their sorrow and fears, and was allowed to simply delight in the fact I had a new baby sister.

Throughout our childhood CF was part of every day life; enzymes were shared out at meals, we took turns at lying over the physio bed (sometimes both trying to lie on it at the same time!) and we sat together in the kitchen puffing on our nebulisers before school.  Neither of us saw our daily routine as different to normal, because it was all we had ever known and therefore for us, was normal.   Those early years we were like any other sibling; - we played together, squabbled, told on each other and then made up.  CF was never an issue, it was just something in the distance; something that in a child’s mind just didn’t seem that important.

It wasn’t until my early teen years that I started to realise what CF could mean for both Frances Ann and me.  Declining health, an over inquisitive mind, and thoughts about my future brought reality sharply into focus.  I became aware of issues such as life expectancy, transplants and the unpredictability of CF as an illness.   I realised though that my fears were not about my own future, but instead were focused on the terrifying thought that this could happen to Frances Ann.  And that all the sisterly love in the world could not protect her.

At this point Frances Ann was too young to realise the implications of CF herself.  She was enjoying herself; taking part in every activity you could think of and just getting on with life.    I kept my worrying thoughts to myself, but it did start to change the way I acted.  I became aware of the trivial nature of any squabbles and I would drive Frances Ann mad by promptly forgetting about them and refusing to bear a grudge.  When I left home to go to university I would come home at weekends and bring little presents; a CD, a poster, a silly soft toy.  Something that said “ although I’m not always here, I’m always thinking about you”.     Neither of us had other friends with CF as we had all the support we needed in each other. We could talk about symptoms, treatments, the unfairness of it all; but as teenage girls it was difficult to vocalise the real fears and much easier to say it silently through simple actions.

Shortly after I left for university Frances Ann’s health began to decline.  Throughout our childhood she had always been the ‘healthier’ one and I had been the one needing regular IV antibiotics, peg feeds and hospital admissions.  Now I watched helplessly as she tumbled into a cycle of infections, declining lung function and an increasing need for overnight oxygen.  I had always secretly believed that because I was older, maybe I wouldn’t have to watch her suffer; that my time would be up before hers. Yet I also felt the compelling need to be there with her through the difficult times as I was one of the few people who could understand what if really feels like to face uncertainty.

And uncertainty hit hard. At Christmas 2001 she was admitted to hospital with a serious chest infection and showing no response to treatment was told that she might not survive this episode.  She was 17 years old.  On being given this news she asked to see me on my own.  That was a life changing moment for me and one I will never forget.  She told me she was not afraid, but that she was very concerned that I would not study for my final exams if something happened to her. She wanted me to work hard to achieve what I had been striving for and assured me that she had not given up her fight.  We then had the most surreal conversation about the fact that if something happened to her I didn’t want her to come back as an apparition as it would scare the living day lights out of me!  After that we laughed and hugged and laughed some more…and both understood without words what we meant to each other.

Frances Ann did survive that episode and was immediately listed for transplant.  From that moment on our relationship had taken on a different sense.  Frances Ann had realised what I had silently known for a long time; that life was indeed too short, it was there to grab with both hands and we were in it together.  We had an amazing couple of years and spent much of this time laughing together at the simplest of things. She had an amazing sense of humour and we understood each other with a look alone.  I tried to go home as much as possible, where we would drive my parents mad with our nonsense and carry-on, and most other days we chatted all afternoon on the phone about nothing in particular.  Despite her being listed for transplant, she would still offer to carry my bag, or fetch me something if I wasn’t feeling well – it was all about give and take.  I tried to be as supportive as possible as she waited on ‘the call’, but I also drew so much support from her in my own life.  She was nearly 6 years younger than me, but in so many ways was much wiser. 

She waited over two and a half years but the call never came and Frances Ann lost her long fight in September 2004.   Only a month earlier she had been the maid of honour at my wedding and the week before she had been on a holiday with my parents.  Life is indeed unpredictable.

I thought my world had collapsed.  My emotions flurried like snowflakes in a storm….I felt devastated, afraid, and angry at being left alone.  My thoughts raced from the unfairness of her life being cut short, the fact she never got to go to university, to leave home, to sample working life, or to marry her long term boyfriend…and then they selfishly settled on my own fears.  Who was going to support me?  Who else would understand what I still have to face?  I always imagined her to be there with me through my own battle…and I suddenly felt very alone.

That was a year ago now, and I see things a little clearer now and less clouded by the over whelming sadness of that time.  I can look back on our time together with no regrets and no sense of missed opportunities, because we made sure there were none. We shared a bond that went beyond that of sisterly love and spilled over into a shared love of life, and a determination to make the most of everything.  I know she would want me to hold those beliefs close, and I try to play life by the rules she taught me; make the most of your time, think positively, laugh often, never give up hope and always follow your dreams …oh yeah, and eat jaffa cakes and French fancies for breakfast, because CF allows you to do that.