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. 

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!

From Ruby's Dad

These words are from Ruby's dad, Craig. We went through this together, and we were there for each other. During our time at Westmead, we took turns at being the strong one, and we held onto each other for comfort. Absolutely no way I would have be able to go through this without him.

I will never forget the night Ruby was born. Some say there is no such thing as love at first sight. Well they obviously don’t have children, because the moment I saw Ruby, I was totally in love with her. This was also the case with Eliza. To have such strong feelings for someone you have only just met and who has only been on earth a few seconds, is a feeling that has to be experienced to be understood.


I remember the birth and my first hold of Ruby. Looking into her innocent eyes, at that moment I made a contract with Ruby, it was a pretty simple one, it was to do all I could to protect and help her. I believe all parents mentally make similar contracts the moment the see or hold their newborn children.

When Ruby started to have her initial problems at hospital I was shocked as she had looked so well when she was born and seemed happy. She breastfed almost straight away and seemed to be the classic healthy baby. My mind was dealing with a hundred thoughts and as many emotions when they transferred her to the high dependency unit. As her condition worsened and the outlook became more serious, I went into auto pilot. You just do what needs to be done. I never thought I would need to start fulfilling my contract so early.

When Ruby was transferred by Newborn Emergency Transport (NETS) to Westmead, I was so overwhelmed with what to do. I had a wife who was devastated that her newborn daughter was being taken away, a 3 year old who just wanted her parents and a newborn daughter who was seriously ill. What do you do??? I had to leave Danni in tears and take Eliza home and then try and get some sleep. All after having very little sleep and with less to come, I set the alarm for 5am and set off with Eliza to Westmead, racked with guilt that I hadn’t been there for Ruby all night. I arranged for my parents to pick up Danni from Gosford Hospital and bring her down as soon as she could be discharged and to drop Eliza off at Danni’s aunties. I later found out Danni’s parents had called into Westmead on the way from Melbourne and made sure Ruby was ok. This was such a relief, as Ruby hadn’t been totally alone.

The following weeks were a mixture of worry, sleep deprivation, relief and sadness all mixed in with some wonderful joyful moments. I had never before experienced such a time of turbulent emotions. Watching Ruby going to have major surgery at 3 days old was the hardest thing I had ever done. This was the first time I had cried since Ruby’s birth. Hearing her diagnosed with Cystic Fibrosis after being told how well her surgery went just a few moments earlier, was like a kick in the guts. Rocking her to sleep for the first time was a fantastic feeling and wheeling her outside the first time was such a relief. Speaking to the CF team and getting all the relevant information and do’s and do not’s, as well as collecting all the medications just made me feel sadness for the future of Ruby. But when we left that hospital and arrived home I felt absolute relief and joy, as well as a determination to make Ruby’s life a beautiful, healthy and wonderful life.

Ruby looks well but she is sick. Her life has many medications and treatments already and without her enzymes she would waste away. Her future will hold many more challenges, it will also hold many wonderful moments and will be the best life we can possibly offer her. The support network of family and friends Ruby has are the best she could wish her. Her Mummy is the most wonderful mum in the world. The care she gives her, the research she has done, the diet she has worked out, as well as the love she provides are all Ruby needs and then some. This is whilst still providing the same caring and loving mothering to Eliza. Danni blows me away each and everyday.

As Ruby’s Daddy, I aim to fulfil my contract to her. I will be there for her at every part of this journey. I don’t know what the future will hold but I understand the realities of this disease and will ensure Ruby is never alone in this fight. I am not a doctor or scientist, so I can’t cure her but I can fight for her at every opportunity. I can help raise money for research and care and do my utmost to raise the awareness of CF. So when you think that maybe we are bombarding you with CF information and stories and if you think maybe we are a little too focussed on this disease, think about the ‘contract’ we all make with our children. Whether we make this contract consciously or unconsciously we all do it and I would hope, all fulfil it to the best of our abilities. Think how you would react if Ruby was your daughter.

Thanks to all our family and friends who have provided such wonderful support to us and Team Ruby. We love you all………

5th Day - DIET

FAT, CALORIES AND CREON

When we were getting CF explained to us, it was always mentioned how the CF diet required extra fat and calories and salt. I didn't worry about it too much as Ruby was still on intravenous TPN, let alone solids. I envisaged changes like switching from low fat yoghurt to full fat.

That's not quite what they meant.

avocado mixed with Greek yoghurt and grated cheese.

I could feed Ruby McDonalds for breakfast lunch and dinner and get a thumbs up from the nutritionist. I could dip her fruit in chocolate and call it a healthy meal. I could feed her a stick of butter for a snack... ewwwww.......

But I don't want to do that.

So I use other things instead. Here is a typical days meal for her at 9 months old

Breaky: 2 weetbix with chia seeds and milk, or bowl of Greek yoghurt with chia gel.  Vegemite on toast.

Snack: cheese (loves cheese!), avocado, tomato and sultanas

Lunch: meat fingers (made with beef mince, grated vegies, coconut oil)

Snack: baked beans and grated cheese

Dinner: fritata type thing I make in the bowl with egg and vegies and cheese and microwave. Washed down with a drink of Hydralyte

Desert: chocolate (plenty left from Easter still!)

Plus 3 - 4 bottles of fortified formula per day

The snackbox in the CF dietitian's room

When I was introducing solids, I would just cook up some vegies and add coconut oil. Then I started making things specific for her like red lentil casserole.

Before, she was on solids, I did my research. I finally felt like a had a heads up on something. Time to prepare, time to learn. Here was a way I could take control of my baby's health. No prescriptions needed. I could research and make my own decision as to what I felt was best for her. Some interesting thing I have found.

Broccoli helps clear damaged lungs as well as being a great immunity booster

Coconut oil is amazing! Google it! The parts that I like are: increased immunity, proper digestion, had Vitamin K & E, and is has the highest amount of fat. And it smells good!

Chia seeds are also high in fat and are known as a "superfood". They can be mixed with water to make a gel, and have no taste. 

raw tomato, tuna and cheese covered broccoli & carrots

 


I don't do the whole calorie counting thing, I just feed her as much as possible. I adjust the amount of enzymes when I notice her poo is oily, or if I am told to. At the moment, Ruby has 2 capsules/scoops of Creon 5000 for every meal unless the meal is very low fat or I think most of it will end up on the floor. 

Sometimes I feel like I'm trying to fatten up a prize goose to take to market! But I enjoy it, it is therapeutic, and Ruby certainly isn't complaining!

zucchini & mushrooms sauteed in lotsa butter

cheesy tuna & vegie rissoni with coconut oil

.

PI

It scares me that Ruby isn't self-sufficient. Or that I can't provide for her without help.


It scares me that she would not survive without her medication.


What if we had a natural disaster and all of her meds were lost?


What if Ruby and I are kidnapped? She would cry and cry and starve. 


What if there was some kind of massive evacuation and they only took people with no medical problems?


What if we got lost in the bush? I can't even breastfeed her and deal with the consequences of not having her Creon. 

Very unreal circumstances I know, so why do I worry about it so much?


Pancreatic Insufficient is the technical term.

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.