Who would have thought that two letters out of the alphabet would cause major upheaval?
Two letters in specific…CF.
These two little letters, to those “in the know”, of course, stands for cystic fibrosis.
My youngest son, now a cheeky 21mths old, was diagnosed with this when he was 4 weeks old. It was a complete shock, while a genetic disease, we had no idea that it was in our family, or that we were gene carriers. Looking back on those first four weeks of life, everything makes sense.
My son was born one day early, after a quick and intense 3 hour labour, he was born a healthy 8lb.
From that very first morning he was born, he was constantly unsettled. He fed constantly (every hour, day and night) and suffering from what we thought was colic, as he was bloated, windy, and generally upset. Of course, the dirty nappies came along with it. The smell…no words could describe the smell. Lets just say that we could smell him at the other end of the house. But this was happening at least a dozen times a day…well more than a normal baby would. But we shrugged it off, figuring it was my breast milk, or that my memory of my three older children at that age was fuzzy.
The coughing started when he was about 3 weeks old. It sounded like a normal cold, and of course, at that age, there is next to nothing you can do. The other kids had colds at the time, so we figured it was just that too. Despite the coughing, I didn’t stress too much, as he didn’t have a fever to go with it. I figured it was just a virus and the doctor would just tell me to do exactly what I was already doing…keep warm, plenty of fluids, raise the head of his bassinet, and I would even stand in a steamy bathroom to help break it all up.
After about 4 days, it seemed he was turning the corner for the better. However, the next day he went downhill…fast! After being at work all day with my fiance and I, his coughing fits were last for longer periods, causing him to go red in the face, and he broke out in a rash that we thought was chicken pox (the rash turned out to be nothing, just skin irritation) So after picking up our older 3 children from daycare, I kicked my partner and the kids out of the car, basically threw their bags after them, waved goodbye and said “Don’t worry, I will be home in a few hours”, and the bubby and I took off to the hospital.
On arrival, I told the nurse at the emergency room that I felt he had bronciolitis, as my two older boys had been hospitalized with it when they were roughly the same age. Without really looking at him, they said they thought it was the same thing. So after doing the usual checks, they hinted that he would probably need to be admitted into our local regional hospital. I was expecting this news anyway.
As our hospital doesn’t have a resident doctor on site, the local GP’s (General Practitioner) take turns being on call. So the nurses called our family doctor (who is absolutely FABULOUS, he managed my pregnancies and delivered all my children…if he made it there in time that is!) He informed them that he was coming up…straight away. I probably should have guessed then from the nurses confusion that it was serious. He wasn’t on call that night, but I put it down to that he was a committed doctor (which he is of course!)
He walked in, did a very basic check of our son, which was very quick, and then closed all the curtains to the cubical and sat on the stretcher beside me. It was then that he told me that he had received a phone call from Princess Margaret Hospital for Children, in Perth. He told me that results from my son’s Guthrie heel prick test (a genetic test that all Australian babies have in the first few days after birth) had come back positive for cystic fibrosis. He started listing what it would entail.
At first, I thought he had said that my son had tested positive of showing signs of having CF, and that it would require further testing. I was panicking internally at this point, but trying to appear calm on the outside. But as the doctor continued to rattle off the details of CF, I had to stop and ask “So, he actually HAS it?”….one sentence brought my world crashing down..
“Yes, he has tested positive for having cystic fibrosis.”
My mind and body went numb. Everything the doctor said after that sounded garbled, like he was talking underwater. To this day, I still have no idea what he said after that point.
I then was given a few minutes alone to try to calm myself so I could make a phone call to my patiently waiting fiance back home. When I called, I struggled to hold the tears back. As my voice cracked, I said “Its a little more serious than a cold, he has cystic fibrosis.” Like most people, he got it confused with cerebal palsy, and asked if he was going to be a vegetable, after reassuring him that wasn’t what was going to happen, he calmly told me that everything would be ok….we would get through this. It was what I needed to hear. After that, my emotions were in check.
Our GP called the respiratory doctor from PMH then, and was advised to do a chest xray, which showed that my son had pneumonia. After calling the respiratory doctor back, it was decided that my son should be airlifted to the hospital in Perth that night.
By this stage, my mum had arrived at the hospital, as my fiance had rung everyone in our family to tell them the big news. She stayed with my son while I was given half an hour to go home and grab some belongings to accompany my son on his flight.
By this stage, it was about 9pm, my older children were fast asleep, but it gave me a chance to fall into my fiance’s arms for a (too short) period. My mother in law drove me back to the hospital, so the I could leave the family car at home.
About midnight, we were taken by ambulance to the airport, loaded into a small Royal Flying Doctors airplane and took of on our unknown journey.
It was midnight by the time we made it to the children’s hospital, and another 2 hours before we made it on the ward due to a very nasty car accident taking precedence in the emergency room (and fair enough!) At 4 am, I sat on the floor of the shower of the hospital, miles from home and family, bawling my eyes out.
The next morning, the respiratory doctor came in, introduced himself, looked over my son, prescribed necessary antibiotics and took me aside and told me what the future would hold.
The next two and half weeks were days filled with specialist visits, physiotherapists, doctors, dietician, social workers, nurses….giving me all this reading material, teaching me about meds and techniques for physio at home. Oh, and don’t forget the pacing of halls as PICC lines were replaced as they kept dislodging, or blocking, within 24hours.
One of the hardest parts was leaving the family behind. My children went to bed not knowing where I was, and we didn’t come home for weeks. Due to their development delay, they didn’t understand what happening, that their new little brother was sick and Mummy couldn’t come home yet.
Since then, he hasn’t had any more hospitalizations, but there have been a few close calls. We have to make three-monthly visits to Perth (which is 4-5hrs away) and annual tests.
He is a happy, healthy little boy, who is still maintaining full lung function. He isn’t pancreatic sufficient, so he has to have about 25 tablets a day just to help him digest his food. But he knows that he can’t eat until he has had his medicine. Some days he is a pain and spits them back at us, but we just scoop them back up, and shove ’em back in again.
I don’t like to think about life expectancy. In fact, the doctor told me that they don’t even like to speculate any more, not with all the advancements being made in treatments.
CF is now just a way of life for our family. It doesn’t stop us from doing anything. I don’t wrap him up in cotton wool (little bugger would just unravel himself anyway, just to cause mischief!)
I don’t see it as a death sentence. But it weighs heavily on my mind some days, knowing that, hopefully a LOOOOONG time in the future, my son is going to die from this one day. That is the biggest emotional rollercoaster for me
But for now….we will live each day as it comes.
So there you have, two little letters that have irreversably changed my entire family’s life.