Operation Fatten Up

Maintaining a healthy weight can be a struggle for people with CF for a combination of reasons. Most people with CF are pancreatic insufficient, which means their body does not break down fats and protein. These people need to take digestive enzymes every time they eat. A high calorie diet is needed to counteract this mal-absorption. It's also needed as people with CF are working harder to breath than you or I. Simply living, burns more calories than average. And during periods of sickness, weight is easily lost., when it is needed the most.
There is a direct link between having a high BMI and being healthier overall. It wasn't until people with CF were told to have a high calorie diet, in conjunction with enzymes being used, that the a age life expectancy increased past teenage years.
All of this information puts stress on parents feeding their kids with CF. Suddenly "healthy" takes in a new meaning. In the early days, your baby is monitored, charted, weighed, measured so closely, you feel like you are raising a prize turkey.
During our visits with our dietician, I was interrogated with hundreds of questions. How much of this? How much salt? How much do you add to this? How much milk? How many poos? What colour/consistency? How long do you spend eating?"
If the dot on the graph drops below a certain point, then there are things to try. Polyjoule, pediasure, formula, appetite stimulants, Ensure, feeding tube. It's like a big black cloud hanging over you, waiting to rain at any time.

And then like everything else on my CF journey so far, I stop listening enough to do my own research and start thinking for myself. There is a whole world of good food out there. Why can't I use it? To be fair, our dieticians have always told us that a CF diet is not a free pass to junk food. But in my experience, healthy alternatives have not been widely suggested.

So the whole point of this entry is to share the following link with you. I started to post it on Team Ruby but waffled on do much, it passed "status length". The post is written by Jo at Quirky Cooking and is a wealth of knowledge about gaining weight in a healthy way. Because, to be brutally honest (as I always am), people with CF never used to have to worry about their health in old age. Getting to old age was beating the odds in itself. Now, people with CF are living past their 30's and overall health is so so important. That's hard to achieve if a high fat diet is a result of empty calories from McDs and KFC.
So if you want to get a bit if an idea on what "real" food is out there, read through this link:
Nourishing & Strengthening Foods . . . for those who are Underweight or Recovering from Illnesses

Oh, and did I mention I'm getting a Thermomix? It's my new weapon in my war against CF!

Annual Review 2012

Ruby's second annual review was today. Sort of like a birthday, but a celebration we would rather not be having. All 4 of us took the trip down to Westmead today. I was very organised and packed a bag full of food and entertainment for the kids.

We got there at 9.30 and started with the normal weigh-in and height measurement. Ruby was very compliant and did exactly as she was told. In fact, she was like that most of the day. It takes a lot to upset her.

Eliza lending some weight to Ruby

Her sweat test wasn't until 10.30 so we started off with the normal nurse checkup. Much to our amusement, Ruby and Eliza burst into squeals of excitement when the suction machine was wheeled in, as it has a huge tin of lollies on it. Ruby had her sputum suction and fir the first time ever didn't cry. It will take a few days to get the results back.  Both kids received a specimen jar full of jellybeans!

Physio checkup was all good too. We discussed different blowing exercises and will be starting off with blowing bubbles in the back with a piece of tubing.  We also discussed getting a vest again. Lot's to think about there especially as a long term study has been completed where it was shown that PEP therapy is more efficient than the vest. 

Then we were off to the sweat test. Electrodes were attached to Ruby's skin to stimulate the sweat glands, then some filter paper was placed onto her skin to collect the sweat for analysis. She left this on for half an hour, but unfortunately not enough sweat was produced and we will have to try again another day. All through the process, Ruby just watched intently what was going on and never got upset. Until it was time to pull the tape off, and then it really hurt her and she had a good cry. 

The electrodes that stimulates the sweat cells to test the level of chloride

Then a consultation with the gastro dr who felt her belly for any enlarged organs and hard parts of the bowel but all was good and squishy. We then chatted to the dietitian which is always painful as I can never actually remember all the things I feed Ruby and I get asked questions like "how much cream do you add" or "how much water does she drink" and my answers of 3 splashes, or showing an amount with my fingers aren't good enough. But dieto is happy with the amount she is eating and the amount of Creon that I am giving her, and no changes need to be made, or supplements added.

Finally, we see Ruby's main dr who checks her lungs (all clear) and we talk about new drugs coming and her general health. We decide to start her on Pulmozyme which is a drug that changes the DNA of mucous, making it shorter, therefore thinner and easier to cough up. This drug is used in many countries as a preventative and works really well. It is try expensive ($25,000 a year) and can only be covered by the PBS if you meet a certain criteria. Which Ruby does, based on her age and recent fight with pseudo. As the doctor said, "we are trying to preserve Ruby's lungs until there is a drug available that will manage all of CF" (referring to the new miracle dug being trialed with different gene types, Kalydeco).

So I feel very lucky that we don't have to push our clinic to try these things, and that we have a health care system that makes this affordable to us. I also appreciate that these things can seem inconsistent between clinics. As Ruby is too young to have the lung function tests, she does not have to show lung improvement to stay on this drug. 

New toys from clinic for being a good girl

So then we pick up a huge bag of meds from the CF pharmacy, pickup the bits and pieces that we have gained (including 2 cute dr teddy bears), make a date to come back in 2 months and treat ourselves to lunch at the hospital cafe.  Oh yeah, then walk to Parramatta Park where we had to park.

A busy day, but both kids did well. Eliza looked out for Ruby and Ruby was charming to the staff as always. 

CF never stops. We are always learning something new, can't rest for  second. 

Dare To Hope

Maybe you have noticed that I don't often post about a cure. Maybe not.
Some big discoveries have been made in the last 20 years, allowing our kids to make it to teenagers and beyond. We are always working towards a cure. It's something I hope for but something I am not relying on.

A few years ago, a company called Vertex started making real progress with actually changing the way the damaged cells work. It was the closest thing that anyone had ever gotten to a cure. With each bit of progress made, it was shared like wildfire throughout the CF community. Statuses updated, links shared, etc etc.

I was a bit of a party pooper. I never shared the news or let myself become excited about it. To me, it was still too far away. Still at "Today Tonight" level.

So it turned out that the drug wasn't suitable for the majority of the CF population. But from it came a different version of the drug which helped people with the G551D mutation.


I'm getting ahead of myself. Cystic Fibrosis occurs when a baby inherits 2 CF mutations. These mutations/genes have names. The most common mutation is DeltaF508. Ruby has one DeltaF508 and the other mutation she has is 621+1G>T. These mutations stop the process of salt being transferred throughout the cells. The drug that Vertex is producing is making that salt transfer happen, therefore stopping all the symptoms of CF. 


As Ruby doesn't have the mutation that the drug targets, I didn't really think much of it. And to be honest, I thought it would be years before anyone could see the benefits.
Then I started hearing things like this:


Since starting Kalydeco (the brand name of the drug)

"His exercise tolerance blows my mind. The increase in energy has been undeniable."


"A week ago I couldn't chase my dog around my back yard more then two maybe three times. I am now able to do it 5 or 6 times before I feel like I'm about to pass out"






 Sweat tests have lowered, people have been able to stop using Creon, lung function has increased. 




Pretty amazing stuff...life changing stuff! The more of these things I read (and there are HEAPS) of them, the more I allowed myself to think that yes, maybe there will be a cure in Ruby's future. Once they have  
a drug that targets her mutation.






So I'm on Facebook today as normal, and a post pops up on my newsfeed. And the post is written by an American mum who got Kalydeco for her daughter who has the mutations DF508 & 621+1G>Tand how she has been on it for a month with awesome improvements! So that means that there IS hope!


A long way to go with getting the drug to Australia and approved and paid for...but now I have something to focus on xx




The lady who posted about her daughter was posting because her insurance will now no longer cover Kalydeco as she doesn't have the G551D mutation. This means for her daughter to still have this drug, she needs to pay retail, which is $294,000 per year.  



.

On and on...

I am a mum, I do mum things for my kids. Cook, clean, nurture, teach, play, raise...the list goes on. We all know how much is involved with being a parent, I am not alone in this!

But I am not a scientist, a researcher, a doctor or a health professional. I don't know how to cure Cystic Fibrosis, I don't even really understand the biological, genetic or technical side of it. I do know that the medical community is confident that a cure is close (and not in a "miracle cure" found on A Current Affair type shows way) and I have faith in that.

So, what can I do to help? I can spread awareness. I can let people know what CF is and how it affects us. I can hound you with raffle tickets, research links and status updates. I can share photos, stories and experiences.

So sorry if my blog posts and Facebook page seem a bit CF obsessive, but it's the only way I know how to contribute towards the best possible outcome.


 


Here is an interview with Dr Michael Boyle about the basic defect in CF and how the two drugs, VX-770 and VX-809, aim to help fix the underlying problems, but in different ways. Good news for CFers, even though it's not very exciting viewing!