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. 

Amber

I bought Ruby an amber teething necklace when she was 4 months old to as I had heard from a few friends how it help their kiddos with teething pains. 
Ruby was on an 18 *month* course of antibiotics, she was having liquid vitamins and we were also learning about other medications she would need. We had just given in to using formula as well as another synthetic substance called PolyJoule to help with her weight gain. I made a promise to her that I would limit the amount of chemicals her body receives as much as I could. So that was my main decision in buying the necklace as opposed to using the teething gels available. 


So in regards to teething, did it work? Well let's say I didn't even notice Ruby had teeth until one day I heard the metal spoon hit them. I had a good look inside her mouth, and there were about 4 pearly whites shining at me! To me, that shows the analgesic qualities in amber help bubbas with teething pains.

Ruby at 7 months wearing her Amber necklace 

And then I started researching it a bit further (as I do). Amber is fossilised resin, which warms against the skin, releasing its therapeutic properties safely and naturally. It’s believed to help and treat the throat, stomach, spleen, kidneys, bladder, liver, and gallbladder, alleviates joint problems, and strengthens the mucus membranes. The words mucus membranes jumped out at me. This is the first area to be hit with parasites and microbes. Not nice for anyone, even worse for someone with Cystic Fibrosis.  

The claims get bigger and better. It draws disease out of the body, aids digestion, and absorbs pain and negative energy. Now I haven't gone and checked all the sources or looked for medical trials. But I did borrow Ruby's necklace once when I had a killer toothache and held it against my cheek. The pain halved immediately. Only drawback was, as soon as I removed the amber, the pain increased again. So I ended up shoving the necklace in my mouth where the sore tooth was. If you have ever had a toothache, you understand that you will do anything to make it better! And that reinforced my belief that amber is not an old wives tale.

I'm not naive enough to believe that Ruby's disease will be cured or fully treated by non-invasive options such as wearing an amber necklace. But I am not going to pass up the opportunity to help my child feel as comfortable as possible without the use of even more drugs. I have since got a necklace for Eliza and one for myself. It is also supposed to help concentration and memory, so maybe I should wear ten!


EarthyMamma has very kindly given us an amber necklace to give away to a reader! Read below on how to enter.

GIVEAWAY
to enter this giveaway please do the following steps

1. Comment on this blog and tell me what benefit of amber appeals to you the most and why
2. Go to www.facebookcom/earthymamma and like their page (they have heaps of great items!)
3. Make sure you are a liker of our Facebook page also (www.facebook.com/teamruby)

Terms and Conditions 

• 1 x amber necklace will be awarded worth $24
• giveaway closes on Thurs 7th June 2012 8.30pm
• Entry is only available to Australian residents only 
• Winner to be chosen by me

Disclosure: I was not paid for this review or giveaway to take place. The opinions expressed on this blog are my own unless otherwise stated.

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!

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.  



.

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

8th Day - NON-COMPLIANCE

     Well once again I am very lucky to not have much to write about. Has your child ever been on a round of medicine that they didn't like taking? Imagine having that struggle every day.



"eat your crusts or your hair won't grow curly"
"have your medication or you won't be able to live"





Sometimes being a good parent is having to be the bad parent. The parent who physically holds down their child while they are getting shaken by a vest. The parent who forces their child to breathe in salty irritating air through a mask. The parent who doesn't save their child from the prodding doctors...

...all because we love our kids.

2nd May - THE FIRST YEAR, THE WORST YEAR

Well we are still in the first year, so I can't really say it is the worst year! It definitely has bad times, but there are so many wonderful moments as well. 


Compared to others experiences, I would say our first year so far has been quite uneventful. We've had a bit of everything but nothing too serious. It's been like an orientation period. A period of learning and unlearning. All the experience we thought we had as parents to a 3 year old? Out the window! Everything had changed. The way we fed Ruby, the physio, the medication...
She has never even spent the night in our room. She was an independent one alright. Didn't like lying in peoples arms, wouldn't go to sleep if you were holding her, total opposite of Eliza!

Eliza and Ruby meet for the first time

I found it hard learning about the limitations she will have. Being told things like, no indoor pools, no sandpits, no bath toys, no eating dirt, no jumping in puddles, use antibacterial products at all times, avoiding childcare. All said in the same breath as "but you can't stop her from being a kid".


The first year is hard because these are babies we are talking about. Babies who should be at home with their families in a safe environment. Not poked and prodded and detached from their family. 

Ruby's first breath of fresh air EVER! 15 days old.

The first year (so far) has also been so wonderful! Ruby is a great sleeper, that is all I ever wanted! We have seen her little cheeky character come out. She is a determined little thing with a beautiful nature. She wins people over by smiling at them, from the day she learned to smile. She loves watching her sister and I love watching them together. Just like a family without CF, the first year is a special year full of discoveries and special moments. Despite the start that we have had, I am loving this year so far!

 

Our first family photo! Ruby was 15 days old and finally cord free and able to leave the ward

Clinic

Clinic is where Ruby sees a team of specialists to help manage her Cystic Fibrosis. She normally sees  her respiratory specialist, CF nurse, nutritionist and physiotherapist. The team also includes a gastroenterologist and social worker. The clinic is at Westmead Children's Hospital. As a newborn, we were there at least every second week, now that she is a bit older, and more importantly, heavier, we only need to go down once a month. If she stays healthy, the visits will be spaced out to every three months.

Here is a photo journal of our clinic visit today:

 As soon as we arrive, Ruby is weighed and measured. She has put on weight and grown well! Infact she is growing longer so quickly that her weight is just keeping in proportion with her length.

  The staff at clinic always take the time to come and say hello. Ruby gets alot of attention as she always smiles at everyone.

This is the most unpleasant part of our visits. a sputum sample is taken to make sure there is no bacteria growing in her throat. The sample is taken by a suction tube being shoved down the back of her throat. Doesn't take very long to do. 

 This is the snack box in the nutritionists room where the kids are encouraged to help themselves! 

 There is always something going on at a childrens hospital! Today we got a visit from some fairies and superheroes. Eliza was very lucky and was given a bag full of treats! Textas, colouring books, stickers, jewellery, a magnetic "learn chinese" kit(?!)...

 Ruby with her respiritory doctor who is her primary medical carer. He is listening to her lungs to make sure they are all clear, which they are.

We saw the gastroenterologist today who checked out her surgery scar and asked lots of questions about poo!

We picked up some of Ruby's medication for the next few months. We always leave with more than what we came with.