"We passed on an illness we'd never ever heard of to our little boy".

I will never forget the date.  It was Thursday 1 October 2009, 16 days after welcoming my precious new baby into the world when I received a phone call that would change my life.  Within hours, my husband and I were sitting with our baby in a hospital room being told my child had a chronic, life shortening illness – Cystic Fibrosis.

Cystic Fibrosis?  I had never even heard of it, yet it was a genetic illness that we had unknowingly passed on to our little boy.  How could this have happened?  I was numb.  The following day we were whisked up to Brisbane’s Royal Children’s Hospital and were welcomed into the world of hospitals, doctors, medications and worry. It was a worry I had never experienced before. A worry that has never left.

I often feel robbed of the blissful experience of having a newborn. While my friends were basking in the glow of their perfect little babies, the first year of my son’s life was incredibly hard, unnatural and utterly overwhelming.

I always thought I would be a relaxed, carefree mother but cystic fibrosis robbed me of that, just like it has robbed my family of a lot of things. The mental and emotional stresses this disease places on us is enormous and at times it seems unbearable.

Christian is now four and we have experienced many ups and downs with his health.  Cystic Fibrosis affects many of the body’s systems, including lungs and digestion. To look at Christian he looks perfectly well.  Actually, as many people have told us, there is just something special about Christian.

He has a certain spark about him.  Its like he knows he may live a shorter life and he is going to squeeze every drop out of every day.  His zest for life is like no other.  However, Cystic Fibrosis is very deceiving.  If you viewed Christian’s body from the inside, you would quickly realise his little body does not function like a healthy persons.

It is a daily challenge to keep his lungs healthy and infection free.  His medical regime is unrelenting, time consuming and often heart breaking.  Just to stay well Christian takes over 150 tablets every week.  He has hours of chest physiotherapy and airway clearance techniques weekly and also inhales expensive medication daily just to ensure his lungs do not clog up with sticky, thick mucus.

There are no days off with this cruel, debilitating disease.  We can not stop treatments on Christmas day or on holidays.  It is a stress that has continued day in, day out from his first day of diagnosis.    Last year was the hardest time in my life.  Christian fought a chronic lung infection for 9 months of the year.

During that time we endured a two week hospital admission and around 8 months of in-home treatment.  I was giving Christian around 10 different medications around 20 times per day.  I was literally setting alarm clocks to stay on track.

In amongst all this I gave birth to my daughter Jasmine, who is thankfully cystic fibrosis free. I look back and wonder how we got through it. I am so lucky to have the love and support of my devoted husband, Greig.

Unfortunately, we’ve recently learnt this infection has returned so the roller coaster ride continues. We’re now facing four weeks of treatment at home and if that doesn’t clear it, we will be back in hospital for two weeks, on IV antibiotics. I’m stressed to the core.

Some of the cruel facts we were first told about cystic fibrosis is that there is no cure and the current average life expectancy is 37. In amongst all this I can have days where I feel sorry for me, however really, my heart breaks for Christian.  This will be his life long battle.  We have to expect so much from him, day in, day out, whether it is being compliant with treatments, being brave during hospital stays or watching him run to the toilet needing to vomit from certain medications or mucus making his stomach churn.  CF is relentless and sometimes it is just too much for a little boy to bear.

Through the hard days, I am always thankful Christian is living in this era of amazing new advancement.  It gives me much needed hope and optimism.  The buzz word surrounding Cystic Fibrosis currently is ‘Kalydeco’.   Kalydeco is a miracle drug. Whilst it is not a cure, it is the first drug of its kind to treat the underlying cause of CF, rather than just treating the  symptoms. Currently it is used to treat adults and children who are 6 years or older.  Kalydeco only works for about 4% of the CF population – those who possess a gene called G551D.

Out of the 3000 people within Australia who have CF, Kalydeco works for about 220 people.  And my boy, my little miracle is one of those people (yes, we are very lucky). Kalydeco adds an average of 18 years of life and offers a quality of life no CF patient has ever known.  However it comes at a huge cost.  Currently it is $300,000 per year, making it utterly out of reach for the normal Australian.

Imagine knowing that there is a drug that would transform your childs life, but families are simply not able to afford it.  It is excruciating.  It will remain out of reach until such time as it is approved on the Pharmaceutical Benefits Scheme (PBS). At the moment the government has deferred making a decision on Kalydeco, therefore putting 200+ lives in limbo.  This decision stresses me to the core.

Although Christian can not be treated with Kalydeco until he is 6, it does not lesson my desire for it to be fast tracked on to the PBS.  I need some light at the end of the tunnel, some weight lifted off my shoulders.  Knowing it is on the PBS and ready for him when he turns 6 means everything to me.

Myself and the G551D CF community have written endless letters to the Health Minister and Prime Minister etc however we have received little response. I feel totally powerless, voiceless and vulnerable.As a mother I do not want to sit and watch my son’s lung function decline before my eyes when I know there is a drug available that would change his life. I want my son to live a longer, happier, easier and more rewarding life and Kalydeco can give him that.  It needs to be on the PBS now.

Kalydeco is routinely available in America, United Kingdom and the European Union (yes, even Greece) and it is having amazing, almost miraculous results.   When I read comments like ‘I have stayed out of hospital for 11 months which is a 20 year record’, it makes me weak at the knees.  Still, the Australian government is happy to lag behind the rest of the developed world.

Kalydeco would allow my son to experience things he has never known, to brake away from the restricted, isolated existence we currently live in, where routine and structure rules our every day.  In our efforts to avoid catching colds and flus (and anything else that could be harmful to his lungs, including dirt and stagnant water) we are careful everywhere we go.  We miss out on birthday parties, community gatherings, family and friends get to togethers all too often.

Kalydeco needs to be approved now.  Every day that goes by without Kalydeco, irreversible lung and other organ damage is occurring.  Simply, my son’s life and 200+ others is in the government’s  hands.  While they defer this decision, they deteriorate.  It is excruciating.  How can the Australian government deny them of two little pills a day.  Deny them the chance of a future.  Its just wrong.

For those interested in following our quest for Kalydeco, please support us by liking our Facebook page – YesToKalydeco.

Please share this post to help raise awareness of the quest for Kalydeco. Do you know anyone with cystic fibrosis?