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. 

Clinic Visit May 2012

All 4 of us made the trip to Westmead today. I swear the hospital shrinks every time we go there. It used to be a huge imposing city within a building, now it doesn't seem so big, and only slightly less daunting.

As soon as we get there, Ruby gets weighed and measured. Her weight had dropped ever so slightly but that is to be expected after the 3 months of fighting pseudo. Now that the pseudo is gone, Operation Fat is number one priority again. You would think that keeping your child a healthy weight is easy, but actually it is quite hard. It requires planning, calculating, coaxing and buying extra high fat food. And you can't relax about it, it is never ending. Then you have to factor on the enzymes. No point working hard to serve healthy, high fat meals if you are not giving enough Creon (pancreatic enzymes) to cover it. 

Her lungs sound good, her fingers show no signs of clubbing. Although they were embarrassingly dirty! Eliza took the opportunity to ask the dr if people with Cystic Fibrosis were allowed rabbits, and was very pleased when the answer was yes! With precautions of course with cleanliness. 

Dr Eliza!

Ruby had another sputum sample collected. She has finally worked out that a jar of jellybeans follow the procedure, so no fighting occurred. 

Our CF nurse suctioning for a sputum sample

We took in our new Aeroneb Go nebuliser, and our CF nurse and physiotherapist had lots of questions about it. They were all happy with how it worked, even though Ruby didn't want to wear it for them. They have been recommending to to patients but haven't had many personal reviews on it, so we were happy to provide one. They were amazed at how quick it nebulised 4 mls of hypertonic saline. 

Speaking of saline, the concentration that Ruby has been having was increased. So she was on 3% (which is the same as sea water) and now she is on 6%. Hopefully this will produce a more productive cough.

A while back, Ruby's Vitamin A & E levels were very low so we doubled the dosage of liquid vitamins that she was on. So today she had to have blood tests to check the levels again. And my little warrior didn't even flinch. She just watched the needle go in and then showed off her pretty bandaid. That's the first time we have had no tears xx

Showing off her pretty bandaid (and yet another specimen jar of jellybeans!)

We have booked in to go back for a sweat test. This is one of the ways to diagnose CF. It measures the amount of sodium in sweat. A high reading indicates CF. Although we don't need to confirm Ruby's diagnosis, it is good to have a reading to use as a baseline, especially if she is chosen for any clinical trials.

We also booked in for our next visit which will be her annual checkup. My baby will be 2 years old soon, sob!

Clinic Visit April 2012

Our clinic have changed the way they operate. We get assigned a room to stay in and everyone comes to visit us. This means that there is no separate clinic for different ages and different bugs as they used to do for infection control.  We all go on the same day, but as we aren't sharing a common area (like a waiting room), the risk of cross infection is much lower.

So when we turned up, I couldn't stop looking at all of the other kids. It sounds strange, but I hadn't seen a CF "big kid" before. I couldn't help but think, that will be Ruby in 10 years time. Of course I know that Ruby will be going to clinic forever, but this was a little glimpse into the future. Instead of feeling sad about it, I was just a bit curious  and fascinated. These kids were confident and self assured. Some qualities I already see in Ruby. The nature of the disease?

First things first, the weight in. And we have a gain! In fact, it was quite a good gain and Ruby now weighs 10.44kg. She is in a good proportion to her height. This keeps the dietitian happy and we don't have to discuss supplements of any kind.

Next we see a respiratory doctor . It's not her normal doctor and I feel like we didn't get as much out of the consultation as we normally do. But he said Ruby's lungs sounded clear and he is going to get some info to me about the phthalate that is still used in Creon.

After the doctor comes the CF nurse. She brings in the suction machine to collect Ruby's sputum. This will be tested for any bugs that may be growing in her lungs, but we a re particularly looking out for how much pseudo is growing. If the growth is heavier than last time, despite being on very strong nebulised antibiotics, we will be admitted to hospital for IV antibiotics.      (please please let's hope the tobi has done its job)

Sputum collection always causes tears but is soon fixed with a specimen jar full of jellybeans!

Checking her sats

Next on the list is our super friendly physiotherapist, Alli. We go through our current physio regime, and practice our "vibrations" with Ruby. We also do a trial of hypertonic saline. This involves testing Ruby's oxygen levels before, during and after the treatments. She tolerates it well. Hypertonic saline is used through the nebuliser before or during physiotherapy to help the lungs move out the mucus. It also re-hydrates the lungs. When using the hypertonic saline, Ruby gave quite a few big chunky coughs. This is the result we want, it means it's doing it's job. But it was also quite confronting. More evidence that her lunged are full of goop. It's not nice to hear your 20 month old cough like a smoker.

So now our treatment regime will be:
hypertonic saline
chest physio therapy and vibrations
inhaled tobi
twice a day. It should take about an hour each time. We borrowed a new nebuliser from clinic as the previous one we borrowed seemed to take so long for each treatment. We are looking at buying our own nebuliser because let's face facts - she is always going to need it. They range from $450 to $1900.

What else? Oh, we picked up some meds.

That's about it. Ruby slept the whole way home. Our next visit is at the end of May. Now I will go online to my CF mumma friends and have a good debrief. xx