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

6th Day - CLINIC

  CLINIC DAYS!

I will always remember the first time we went to clinic. I hated it. It was like walking into a secret society. The staff were welcoming, they had all heard about Ruby and were excited to meet her. I hated that they were so nice. I wanted to tell them "don't be too friendly, we won't be coming back". I didn't want to be part of this club, I didn't want to meet any of the other parents, I didn't want to have a reason to be there. 

But it is a part of our lives now, and it always will be. I don't want clinic days to be a negative thing for Ruby, so I have changed my attitude. 

Clinic is pretty much an all round check-up. The last one was just two days ago, Ruby was 9 months. First she was weighed then measured. Then a chat with the physio who was impressed by how she can now blow. Then another chat with the nutritionist which is always informative. 

Then the sputum sample. This is where spit is sucked out from the back of Ruby's throat to see if she is culturing any bugs. Depending on the result of the sample, her antibiotics may be changed. The plan is to catch anything early before it causes any damage.

Here is what happens:

Clinic days are scheduled by age groups and what bugs your child has previously cultured. This is to avoid the spread of certain germs that can be very detremential to a CF childs health. Believe it or not, clinic kids look normal. If you walked in by accident, you would not even realise that there is anything wrong with these kids. There's that whole "invisible disease" thing again...

We go to the clinic at Westmead Children's which is over an hours drive away. It makes for a long day for a little bubba but she does well.There is a clinic closer to us at the local hospital, but I just don't feel comfortable changing care yet. Westmead have been there from the start, it's just how it is.

Links to our clinic updates:

http://danni-herewegoagain.blogspot.com/search/label/clinic

Clinic Update April 2011

Today we had Craig's parents come with us as we had a genetics appointment after clinic, but more about that later.

Ruby is starting to become more aware of these visits and she seems to know what is about to happen. As normal, she was very happy on arrival, and we are always greeted with cries of "here's Ruby!" The staff there are very friendly and genuine and are always commenting on how well she looks. Of course, Ruby encourages this behaviour by beaming at them all.

She was weighed, and then measured, and that's when she started to get suspicious. She really fought hard about being laid down for a length check and had quite a loud sook about it.

First up was the dietitian, which is the main reason we go monthly. I really like out dietitian Christy. Even if we don't agree on some things, I always feel I can argue my points and we often come to compromises. Ruby got full marks for being a big eater and liking all types of food. Christy was happy about the weight gain, but still wants her on the strongest mix of formula until her weight catches up with her length. She know I'm not happy about this. We talked Creon and fat content, food ideas etc etc.I tentatively mentioned to her that I made a point of giving Ruby Hydralyte to drink everyday, wondering if I would get told off. But she was happy I was doing this and told me I don't have to be so strict on adding salt to her diet now that I am going this. Yay for me!

Then we had a sputum test, which I found out later is pointless when she is on Bactrim (grrr). Obviously, this is never fun, but it was over quickly. I couldn't help but admire how Ruby maintained direct eye contact with the nurse whilst crying very loudly at her...if looks could kill.   (I wanted to take a photo but thought I would look like a bad mum)

Ruby's respiratorist wasn't there today so we saw the fellow instead. She asked all the right questions and listened to her chest. All clear! I also asked her to check Ruby's heart as I had been told previously (when we were admitted to Gosford hospital) that she has a systolic heart murmur. But she said all sounded normal.

By the end of it all, Ruby was crying whenever anyone looked at her for longer than 2 seconds. She even cried when I changed her nappy, I'm sure she was waiting for something unpleasant to happen. This is why I want to try to space these visits out as much as possible. I don't want Ruby to feel unsafe and scared, even if only for a day. 

So we will be going back in 4 weeks. I actually pushed for 2 months and they compromised with 6 weeks, but there were no appointments available, so 4 weeks it is...

So that was Ruby's visit. Now about me! I have a circle of Facebook friends who are CF mums. I surprised myself by becoming close to people I have never met. Is this how Dungeons & Dragons players feel? Anyway, I got to meet one face to face today! Only very briefly as her and her beautiful daughter were in isolation, and it was a bit tricky with my in-laws there also, but it was still exciting!

Clinic Update Feb 2011

We left Eliza with nanny & pop today as she had a cough and I refuse to be one of those parents who take their kid to pre-school when they aren't well. Craig wasn't well either so he hung out at the cafeteria while Ruby and I went to clinic.

I always forget to prepare myself for the initial walk inside the hospital doors. It's like you enter another world. A world that exists purely on the fact that shitty things happen to kids. It sucks the breath out of me when I first walk in, but it only lasts for a second.

Because Ruby has had a bit of a runny nose, we were put in a room on our own, which was actually quite nice!

First stop, the weigh-in...and she weighed...6.595kg!  And she is 54.4cm long. Good work on both accounts.

Then the physio came to visit us. She is totally in love with Ruby and always insists on giving her big cuddles. Because Ruby is sitting up now, we can add some other aspects to her physio. Mainly bouncing her up and down and using an exercise ball. Physio for CF is fun for kids, as anything that pushes the air out quickly is good for their lungs. So bring on the trampolines and pogo sticks!


Next visit was from Ruby's respiratorist who looks after the overall care of Ruby. He gave her lungs the all clear and wrote of a script for more Creon. As I left, I noticed the script was made out to Ruby Creon so next time I'll be careful not to talk to him whilst he is writing!

Then we had a sputum sample taken. Tube down the throat, a bit of gagging, and it's all over. Hopefully nothing sneaky growing down there.

Last visit was from the dietitian, who came in with a huge smile on her face. She was very pleased with Ruby's weight gain and gave me the "gift" of letting me throw out the PolyJoule. (PolyJoule is powdered carbohydrate, when made up it is as sticky as superglue and I just hate it). We are still on a super dooper strength of formula though, until her weight catches up with her length a bit more. She asked me what I was doing with her solids, and she was very impressed with what I have been feeding Ruby.

And I felt really proud of myself. 

Choosing Ruby's food is the first time I have felt that I have total control over an aspect of her care. I can make my own decisions, my own informed choices. I chose to skip the goopy baby cereal and make a chia seed gel instead. I chose to add coconut oil to her foods instead of the normal lump of butter. I let Ruby decide that she was really for solids, and Ruby only. I may have failed at breastfeeding, but now I can choose what foods she is eating, and as I prepare it all myself, I know exactly what is going in her body. And, the best thing is, they were the right decisions, Ruby is doing really well.

For other CF mamas reading this, here are some questions we asked:

What is the "perfect" climate/environment for a CFer to live in?

"Your house". The doctors way of saying don't do anything drastic. He then said that coastal climates are best, due to the salty air and surf. Also, it is more important to have good health care close by, than the actual conditions of the environment.

Now that Ruby is 6 months, do we buy the next stage formula?

Dietitian informed us that there is no reason to change formulas, there really is no beneficial difference between them all. Discussed how formula companies are evil.

Time frame of giving Creon and continuing eating?

Creon 5000 lasts half an hour, so if still eating after half hour, give some more

Next visit in a months time! 

CF in a Nutshell

So, what is it? Here are some facts without getting too complicated and without having to read all the horror stories out there on the net:

• Cystic Fibrosis is the most common life threatening, recessive genetic condition affecting Australian children. Both parents must be a carrier, you can't "catch" CF.
• Around 10% of babies born with CF have "meconium ileus" - an obstruction in the intestines caused by thick, sticky baby poop. This requires surgery, which is what happened with Ruby.
• In people with CF, salt and water aren't transferred properly from the cells, causing the body to produce thick sticky mucus.
• It affects a number of organs in the body (especially the lungs and pancreas) by clogging them with the thick, sticky mucus. 
• The mucus is a breeding ground for bacteria. Repeated infections and blockages can cause irreversible lung damage and premature death.
• Mucus can also cause problems in the pancreas preventing the release of enzymes needed for the digestion of food, especially fats. Malnutrition can be a problem.  

So what do we do for Ruby?

• Daily physio where we try to "knock the snot" out of her, is how I describe it.
• She is on antibiotics until she is 18 months to give her a good head start of being healthy and strong, and hopefully not getting any chest infections in this time
• Enzymes are given before every feed so her body breaks down food (milk). Otherwise it would just pass straight through her, nutrients and all
• Salt is great for people with CF, so she is constantly breathing in the lovely salt air!
• Excercise is also a very important part of keeping healthy as it prevents deterioration of the lungs and improves physical bulk and strength.
• Regular visits to the CF clinic at Westmead

The life expectancy of a person with CF is 35 years. But don't let that scare you, this is double what it was 25 years ago, and is always increasing. There is no cure for CF, but the faulty gene has been identified and doctors and scientists are working to find ways of repairing or replacing it. With today’s improved treatment most people with CF are able to lead reasonably normal and productive lives. A great amount of time is being directed towards finding new and improved ways of treating CF and of finally finding a cure.

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.