CF Wonder Drug

There have been some amazing developments in treatment of cystic fibrosis over the last few years, changing the lives of those living with the condition.

A couple of years ago, the world exploded with the release of Kalydeco. It was a drug aimed at certain CF gene types (there are many types out there, so this drug wasn’t suitable for everyone) 

It is a little pill that treats the underlying cause of cystic fibrosis rather than just the symptoms. The results of the drug were ground-breaking and life changing. People taking the drug were showing lung function improvement of up to 65% and had a new lease on life.

The downfall of such an amazing drug was the price. The cost of Kalydeco was around $300,000 a year! It simply wasn’t affordable.

After much lobbying and petitions, the Australian government approved Kalydeco on to the Pharmaceutical Benefits Scheme (PBS) which caused a sigh of relief around the country as the drug became much more affordable.

Fast forward to 2016, and another drug has hit the market. This one is called Orkambi, which is aimed at another gene type. Our family is excited about this one as it is aimed at Cameron’s gene type! This drug is expected to help almost half the Australian CF population. It has similar effects as Kalydeco

Again, the downfall is the price. Orkambi is currently about $260,000 a year!!

There was a recent petition to have it placed on the PBS list to make it affordable for everyone. However in the “wisdom” of the Australian government, they REJECTED the bid, claiming it was not “cost-effective”.

I don’t understand how they could think this? It is improving quality of life. It is improving health, which means less of other medications, less treatments, less time in hospital. Surely that all adds up in the end?

How much? Too much!!

So the petitions are starting again. Click here to sign and help show your support.

This drug is important and needs to be made more affordable! Lives depend on it!

Bella 🙂

Our CF journey so far…

On the 16th May, it will be seven years since our family was completed. On the 12th June, it will be seven years since our family member’s lives were irreversibly changed by two little letters.

Cam- our little joker!

The last seven years has had its up’s and down’s. Cameron is a vivacious little boy who lives his life to the fullest. He doesn’t walk, he runs through life (literally and figuratively!) If there is a joke to be found…Cam will find it! His diagnosis has not dragged him down in the slightest.

But that doesn’t mean there haven’t been hard times.

In the last seven years, Cam has been admitted in to hospital four times. There was one when he was diagnosed, sick with double pneumonia and underweight due to malabsorption.

The second time was when he was two years old. A bronchoscopy at annual review that year showed he was growing the dreaded super-bug Pseudomonas in his lungs, which is an instant minimum two-week admission into hospital for intravenous antibiotics.

Hospital is never fun 😦

The third time was unrelated to his cystic fibrosis. He had severe tonsillitis and his throat closed over. It was a scary 24 hours! We were more worried about that than anything he had gone through with his CF by that point.

The fourth time, he was five years old, and another routine bronchoscopy showed Pseudomonas again, which resulted in a gruelling two weeks in hospital and another week of treatment at home. We were all exhausted by the end, and it was a rather traumatic experience for Cam which resulted in a slight fear of needles.

Over the years, he has also had a staph infection twice. The first time, we discovered he was allergic to one of the antibiotics they used to treat it.

He hates nebulizer treatments

He currently has about 30-35 tablets a day to keep him healthy. This includes Creon tablets (which help him digest his food), salt tablets (as salt loss is massive with CF-ers), a multivitamin, Vitamin D tablets (to ward off osteoporosis, which is high risk in CF-ers) and antibiotics to prevent infections. This doesn’t include when he has a moist cough, where we treat him with two weeks of nebulizer antibiotics at home. If there is no improvement after that time, it requires a hospital admission for IV drugs.

Cam using his PEP mask. He will try anything to get out of doing it

He has progressed from percussion physiotherapy to resistance breathing techniques using a PEP mask and “huffing” through a wide pipe to help move any mucous around in his lungs. He is now in charge of his own physiotherapy (of course, under close watch by us, as he is well-known for trying to worm his way out of it!)

His high fat, high salt diet is now easily maintained. It was a massive learning curve when he started solids, but now we just add the “goodies” to meals once separated from the rest of the family’s meal, or add a few extra high-calorie snacks to his lunch box, and extra helpings whenever he requests. Cam is a little on the shorter side, but in terms or weight and build…he is actually a little Buddha for a CF kid , who are generally notoriously lanky/skinny.

This kid LOVES his food!

He is pretty good about dealing with his condition. About two years ago, he had a little breakdown. He suddenly didn’t want to do his physio. He didn’t want to have his medication. He didn’t want needles. He didn’t want to see doctors. He didn’t want to have cystic fibrosis anymore. There was a bit of focus on him at school as he was in pre-primary, and the other kids were taking notice that he was a bit different. In the past, his outlook was “I am different, and that makes me AWESOME!”…suddenly, it was “I’m different, and I don’t know if I like it anymore…”

We decided to just pull back a bit and allow him to work through it on his own. We didn’t push him in to anything he didn’t want to do (with the exception of physio and medication…sorry buddy…no escaping that!) We let him talk things out. I spoke to his teacher, and they tried to make things as discreet as possible at school (ie. having medication away from the rest of class)

I have to admit, I was a bit surprised at how young he was when his “breakdown” came. I am sure that many more are expected over the coming years, but I’m sure we can work through it.

We don’t stop Cam from doing anything. We want him to experience everything he can. He recently started sword fighting classes, which he loves considering he loves pirates and ninjas! He has a great circle of friends who understand his condition, but don’t see him as his condition. He plays pranks on his family, much to our chagrin sometimes. He also gives the best cuddles (but not in front of anyone….of course!)

Nothing is going to stop this kid!

Life certainly isn’t easy when you live with cystic fibrosis, but it certainly isn’t as bad as what I envisioned it would be when he was first diagnosed. I’m not making light of the condition….CF sucks…it truly, truly sucks. But we have been lucky with our experience so far….a lot of other families haven’t.

May is CF Awareness Month. 65 Roses Day is on the 27th May this year. All I ask is that you take a moment to familiarize yourself with the condition. I’m not going to push for donations (though it would be awesome if you could) but the whole focus is awareness.

I just hope that sharing our experiences, it sheds a little light.

 

Bella 🙂

 

That day has arrived!

I have been dreaming about it for four and a half years!

Last week, my youngest son started pre-primary schooling!! All four kids are now in full-time school!!! In the same location!! At the same time (between 8:30am and 3:10pm!)
The idea of five days a week without having to hear “But Mu-u-u-m!!”, or “So-and-so took my toys” or “I’m hungry!” has felt like a distant dream…until last Monday.

First day of school, 2014

While a massive part of me wanted to kick my heel together and skip out the school gates, there was a small bit of worry lurking in the depths of my stomach as well.

Cameron was at daycare for around two years. The staff all had training from the CF Association for his cystic fibrosis,. They worked very closely with me for his meals in regards to his special diet. They worked breathing exercises  into their daily programs so Cam could do his physio, without being obvious to other kids. I also wrote out medical forms every day to specify how many tablets he had to have, and when.

This was the cause of my worry about starting school.

(NOT Cam’s lunchbox!)
This is a representation of a CF lunchbox. Separated into meals. The round, blue containers contain the medication.

The CF Association came to town a few days before school started and did a presentation to the school staff. I was present to make the information more personalised to Cameron’s needs. The staff seem to be switched on and are welcome to approach me with any questions (which they already have- just to clarify they are doing it correctly) Cameron takes his medication in his lunch box, and administers himself. I leave a note in different sections of his lunch box to let him (and the staff) know how many tablets to take for morning tea, lunch…etc..

Let me clarify right here….I am a control freak! This loss of control over knowing how many tablets he is taking…especially IF he is actually taking them just about has me starting the bad habit of nail-biting.

I trust Cam will take them. I trust that he will not start eating his meals without having his tablets. But there is still this niggling worry that something will go wrong somewhere.
At daycare, it was all written out for the staff. They stood over him and watched him have his tablets (he still administered himself, I believe). All staff in the building had CF training, whether they worked in his room or elsewhere….everyone KNEW Cam’s needs and their importance.
My biggest concern is that the teachers, aides (etc.)….but especially relief teachers…will miss the significance. The importance.

One part of my brain is trying to soothe me, saying “It will be ok. Other CF kids go to school!”….but another part of my brain is replying “But this is MY child!!! If they screw up…even just a little…it could mean pain!”

Cameron is absolutely LOVING school. In terms of medication at school….so far, so good!

He missed out on the last three days from the first week of school due to a medical check-up in Perth (another story in itself) but he is keen to return next week.

He is so eager to be a “big boy”. I’m eager to have “quiet child-free time”!! This is what is slowly calming my worries!

Everyone says “You wont know what to do with yourself now that they are all at school.”

Trust me…I know EXACTLY what I am going to be doing! I have a massive stack of books to read. I have a mound of paperwork I have been pushing aside due to lack of energy and concentration. I have friends who I have been neglecting. I have a giant pile of coffee pods to drink. I have many tv shows recorded.

I will be fine!

How was your first day of school?
Bella 🙂

CF annual review-July, 2013

A couple of weeks ago, my husband and I took Cameron on the long trip back to Princess Margaret Hospital for Children in Perth for his annual CF review. We also had our daughter, Eva, in tow so that she could be reassessed for her respiratory issues.

Our first appointment of the day was for Cameron’s lung function test.
He had just started doing this new type of lung function test at his last clinic visit only 3 months prior as he was now at an age to start using the testing machine, “Mr. Wobbly” (called so because of the way it makes your cheeks wobble during the testing phase)

Cam’s stage one lung function test- “Mr. Wobbly”

He aced it on his first go, and had no trouble completing it again this time around. He also did this new test that they have started doing on younger kids where they breath in pure oxygen for a short period, then test how long it takes for the carbon dioxide levels to go back to normal. This apparently helps to indicate if all areas of the lungs are being used. He did very well and completed this task without an issue. Very proud mumma right here!

Cam during stage two of lung function test. I’m sure there is a proper name for this particular procedure, but I missed what it was…

We had a quick look at the results on our way back to the clinic area, but could not make heads or tails or what the figures meant. But since nothing was highlighted in red, we assume everything is still going really well.

Then he had to be measured, as is routine at the start of every clinic. The little fatty boombah put on another 300-400g in the last three months, putting him just under 20kg. This puts him on about the 80th percentile for weight.
I can’t remember how tall he is (110cm rings a bell…) and I’m not sure how much he has grown recently, but they are happy that he is steadily travelling along the 25th percentile for height (which is great, as I was told that height can quite often be stunted in CF)

The first CF team member we saw was the physiotherapist, who makes sure that he is still undergoing his physio techniques properly. This meant watching him use his PEP mask, doing a few percussion motions and making sure we are still using the correct hand motions. She also checked his shoulders. Apparently his left shoulder is a bit “tight” which can cause a hunch. This stops the chest cavity opening up properly and the lungs don’t take a full breath. So we have to get him stretching and hanging (like on monkey bars or tree branches, etc)

We then saw the dietician and gastroentologist together. They were very happy with his weight gain and his dietary intake.

Cameron has an intolerance to fresh dairy (milks, yoghurts, cheese…etc…but once cooked, it’s not a problem…so things like cakes and sauces aren’t a problem!) It’s more of a dairy protein intolerance. We figured it out when he was about a year old and we were trying to make the change from formula to cow’s milk. I tried everything under the sun….goat milk, oat milk, rice milk, lactose free milk….everything….and soy is the only thing he could tolerate. Apparently the protein levels in these other types are very similar to cow’s milk….which is how we came to the dairy protein intolerance diagnosis.
We were told that if kids couldnt tolerate these foods by the time they were about 5-6 years old, they probably would never be able to,

So the team were very happy to hear that is starting to tolerate fresh dairy a bit better. He has normal dairy yoghurt, custards, a bit of cheese (though not too much in a day, or it sets him off all bloated, gassy…and well…messy!!) We havent attempted cow’s milk yet, but he is still happy enough on soy milk. When he has normal dairy foods, we find it helpful to give him an extra Creon tablet (enzyme medication which helps to break down the fat in his foods during digestion) So if he were to have some yoghurt, and fat content requires him to have 2 Creon tablets, we give him 3 instead….which works well.
They told us to keep trying to introduce dairy to his diet and see how he goes. Soy doesn’t have the same calorie and fat content of dairy, which makes it more difficult to boost fats in the CF diet, but we obviously seem to be managing!

They also told me that they now require all CF patients to take Vitamin D supplements as the multivitamin (VITABDECK) was not substantial enough for absorption levels. Since Cam was already on Vitamin D levels, and on a higher dosage than what they normally make standard for everyone, he is to remain on this dosage as it seems to be working for him.

They were going to take some blood samples during his bronchoscopy later in the week to test for other things, like salt levels (which there was a slight increase in his daily intake of sodium chloride solution) and iron levels (which have been borderline needing medication for a long while now, but they are hesitant to prescribe medication for it as it can cause constipation, which can already be a major issue for CF-er’s with their digestive issues)
I havent heard anything since returning home, no phone calls about needing extra medications, so I can only assume that they were nothing to worry about until we see them again in three months.

We then saw the respiratory doctor. He was very happy with Cam’s progress, especially since he has managed to escape all the winter bugs so far (wish I could say the same about the rest of the family though!!)

I’m sure there are plenty out there who can relate…

When we arrived at the hospital (since we had just gotten off the highway and went straight to the hospital for appointments) I realised I made a MASSIVE mistake…..I had left ALL of Cam’s medications at home, including his Creon!! I can’t stress how much an issue this was. Since he had only eaten things in the car which didnt require any medication (fruit, etc) we didn’t realise until then that we had no medications so that Cam could have lunch!
We sucked it up, and gave Cam a sandwich anyway, thinking we would just have to deal with the mess later (without Creon, food passes straight through, undigested. It makes an almighty, smelly mess in its wake 😦 ) 
We got a doctor to quickly write a prescription for all of Cam’s medications and handed it in to pharmacy at the hospital, hoping it would be filled quickly.

But no, we spent more time waiting for the medications than we did seeing specialists. Normally, to avoid the wait, we hand in the prescription and pick it up the next day, but due to the urgency, we had to wait around. That was more exhausting than seeing the team!

There is a lot more to write about, such as Cam’s bronchoscopy, and Eva’s check up…both are long stories that can be set aside for another post. So keep an eye out!!

Bella 🙂

The Dark Hanging Clouds- a personal experience

 

I do not hide the fact that I have anxiety. Given all that my family go through, is it any wonder?

I have what is known as “Situational Anxiety” , which means that I’m not anxious all the time…but drop me in a situation where stress is high, I tend to temporarily take it to the extreme…and if pushed enough, will have a full blown panic attack where I can’t breathe, my heart rate rises rapidly, I want to throw up and I get dizzy and feel like passing out. Thankfully I haven’t had many of those.

Believe it or not, but this is an improvement for me!

Really, it all started around the time that my eldest son was born eight years ago. It would have been classed then as Post Natal Depression (PND). I was moody, sometime teary, overwhelmed and didn’t have a lot of interest in much. But (and this is NOT recommended) like a lot of women…I hid it.

Then I had my daughter, things were a little worse. By the time my middle son came along (bear in mind, this was three children in 2.5years) things had hit a critical point. I had no energy, didn’t want to move from the couch. I had zero interest in anything….zilch! IF I spoke, it was because I had to, and it was barely above a whisper (and for anyone who knows me, knows how much I love to talk!). I felt numb…like a hollow shell.
I wasn’t suicidal. I didn’t have visions of harming anyone…least of all my children…but I just didn’t care about anything anymore.
This was when my husband intervened and said that I needed to get help because I was freaking him out.

Off I trotted to my general practitioner (GP) and burst into tears in his office when I told him that I thought that I had depression. He patted my knee and told me that he was proud of me for coming forward. He did the formal examination to officially diagnose, and I was prescribed antidepressants.

I wouldn’t say that medication turned my life around, but they helped me to cope with life easier. I was functional again.

When I fell pregnant with my youngest son, he definitely wasn’t planned. I had eased off my medication, but was worried about a bad relapse again. Pregnancy with Cameron drained me, making me very moody and tired. Part of it was physical, due to the pregnancy, the  other part of it was mental state. I managed to push through. After he was born, I felt fantastic physically, which improved my mental state. I still didn’t need medication.

Then, four weeks later…we recieved Cam’s earth-shattering diagnosis. While we were in hospital in Perth during Cam’s diagnosis admission, the nursing staff told me that my GP called the hospital every few days, not only to check up on Cam’s progress, but to check up on me as well considering my history of depression.
The CF team knew of my history during this period as well, and considering that nature of what was going on, kept a close eye on me as well. They praised me for keeping it together, for helping out during the dramatic parts instead of becoming a hysterical mess.

Throughout all this, I was still numb from the shock of diagnosis, but Cam’s current health condition took my complete focus. I did have a brain snap moment in the hospital, where I had a complete blubbering meltdown over the fact I had completely lost my milk supply (probably due to stress) and could no longer feed my baby breast milk. We had tried medication to boost it, but it didn’t work. I felt like I was under pressure to keep producing, and felt like a failure that I couldn’t do it. A wonderful nurse closed off our room, talked me through it and boosted my mood and self-confidence again.

When we returned home a few weeks later, Cameron had to have his routine, post-birth checkup at around 12weeks old. By then, I could tell it was all getting on top of me again. My GP was very understanding and I was back on medication.

By the time Cameron was about 12 months old, and I had an annual mental health review with my doctor, we both agreed that it wasnt so much depression any more, it was more anxiety. There is a fine line between depression and anxiety, often symptoms overlap…but we were confident that this was the case in this situation.
Little things would set me off. Passing a police car while driving was a major trigger! I didn’t even have to be speeding, but I usually had to pull over because my heart was racing, my breath was short and I felt like being sick. A few minutes later, I could carry on.

Anxiety attacks really suck!

Occasionally, a panic attack would occur for no apparent reason. I remember one where I had spent the morning getting ready for one of the kids birthday party at our house. Everything was fine…I was happy…everything was sorted and ready to go. When guests started arriving, I suddenly had to rush off to my bedroom for one of the worst panic attacks I have ever had.

Thankfully it left as fast as it came, and I walked back out to the party, with guests none the wiser of what had occurred.

A trip back to the GP and a change in medications fixed that back up.

About a year later, I was able to ease off the medications again, until only a few months ago.

When my anxiety is high, I get very aggressive. Nothing can placate me. My husband urged me to go back to my doctor again. Off I went, where discussing it, the diagnosis changed to situational anxiety.

Basically, I’m not anxious ALL THE TIME. Put me in a high stress situation, you start seeing snippets of anxiety poking through. If you keep prodding me during those moments, it quickly escalates until….KA-BLAMMO…explosions and fireworks, or a panic attack occurs.
Considering the amount of pressure I was under at the time with work, and drama in the family life, these explosions were happening a little too frequently. So I am currently back on my medication again.

It’s not making me feel any different. I am still my happy, chatty, normal self….but when I am dropped in those high stress moments, I am able to cope better.
But it’s not all about medication. You have to tweak your life and environment to help as well.

Support networks are a massive help to me. My friends who pull me out of the house to catch up (and wont take no for an answer!), my online communities allow me to vent, there are so many communities out there in cyber space aimed at depression/anxiety support, for me it is through my parenting communities….they understand most of all since they have families as well. Maybe it is the anonymity of cyber space that helps to spill the beans?

Deep breaths…that is the key

Mediation helps to clear the mind…switch off from all the thoughts of what’s going on, what has to be done…and just general negativity.

Writing is my biggest outlet. It helps me process and deal with my emotions. I cannot recommend enough to people to write their thoughts down. It’s very soothing, and years later, you can look back on it and see how far you have come in the process.
This story may come as a surprise to some of those who personally know me, because while I don’t hide the fact that I am dealing with depression/anxiety, I don’t advertise it either. Some family members didn’t even know I had depression until years later.

I just wanted to share my story in the hope that if someone out there is going through the same thing, they understand that they are not alone, and I urge them to get help! Anyone of you can contact me via email (bellasblog@hotmail.com) if you ever feel overwhelmed and just need a listening ear.

Your life will better for it with some help.

Bella 🙂

*Cough, cough…splutter, splutter*

I have split feelings when it comes to winter.

Special family moments in winter 😉

I love the weather because it is a great time to snuggle under blankets on the couch with hubby and/or kids and lazily watch movies. I have a hot body (I still have fans blowing on me in bed during winter!) and thankfully live in a town with a cold environment (even in Australian summers, it doesn’t get very hot in our town)

My biggest excuse for loving winter is that is easier to warm yourself up, than it is to cool yourself down. I don’t cope well in heat…at all!

But on the flip side, I hate winter for all the extra dramas it brings. This is when the anxieties about CF health rise a little more. You keep you fingers crossed that you don’t catch winter bugs, or that they aren’t severe/last long. Puddles are looked at with dispair…imagining all the bugs swimming around in the water…which tend to be more dangerous to those living with cystic fibrosis than the average person.

It is also when my daughter’s floppy airway act up more than usual.

Due to weak lung muscles, Eva develops nasty, croup-like coughs, which can last anywhere from a few days, to a few months! It is a deep, dry, barking cough. It gets worse in the cold air or at night, and worse again when she lays down at night.

Last year, we were very lucky to escape with only a few coughs that only lasted for a couple of weeks at a time.

Coughs suck!

This year, winter isn’t even officially on us yet, and I have major concerns about her current coughing spree (which has currently lasted about four weeks.)

We had driven to Perth the morning of our last clinic visit, after dropping our older kids at school. It had barely been ten minutes since we dropped her off (and we hadn’t even left town yet) when I got a concerned phone call from the school asking for details about the bad cough. I told them about her floppy airways and the history of coughs, and advised them to keep her inside where the air was warm as much as possible, and to call my mother in law (who was looking after them while we were away) if they felt she needed to go home.

This was when I decided that I needed to address the issue with the specialists when we reached Perth.

Later in the day, after Cameron had completed his CF checkups, I changed the topic to Eva.

The doctor advised to do one week’s worth of antibiotics, and then a week of Ventolin ( a reliever inhaler) just to gauge the benefits of each individually. He was quietly confident that neither would work, but it was worth the test, despite the fact it isn’t a wheezy, asthma-like cough.

After we returned home, we started the antibiotics. No dramas- we have been through this part before. Nothing really changed. I rang the specialists last week to double-check how much Ventolin to give her. They advised twelve puffs, four time a day. I felt this was a bit of overkill. As an asthmatic myself, twelve puff is how much you have when you are in the midst of a full-blown asthma attack, and that leaves me (a tall, overweight adult) feeling shaky afterwards due to overdose.

Hubby and I talked about it, and agreed six puff, four-time a day should suffice (before you say it, I know we aren’t doctors, and should be following specialists orders) But it felt extreme to be going through half a canister of Ventolin a day!

We did about two days of inhaler treatment, and her cough started getting worse. She ended up getting sent home from school on Thursday after about an hour as she was in hysterics over her cough, and kept trying to lay down to sleep on her desk. This change came about after only one day of treatment.

Nebulisers help soother airways and can help loosen mucous

Thursday night was a nightmare. I haven’t heard her cough like that in a very long time. There were times in the night that it sounded like she was going to vomit. But there wasnt much more we could do. We had a heater in her room, raised her bed head so she wasn’t lying flat and even at the point of giving saline (salty water) nebulizer to help ease the lungs.

Ventolin was making things worse, and only leaving her shaky afterwards.

So I rang the specialists today to give the update. The consensus was to take her off the Ventolin and start oral steroids. Apparently, there is a small percentage of children with floppy airways where Ventolin has the opposite effect. Since it is a muscle relaxant, it just loosens the already weak muscles even further.

She hasn’t started the steroids yet, but I will keep you updated on their effects.

But the symphony of coughs and splutter in our house is the biggest reason why we dread winter.

How do you combat the winter months?

Bella 🙂

Never ending- an update

I have been a little quiet lately. My poor husband has injured his shoulder quite badly, which means I have been helping out by covering his work in our cleaning business. It is going to take a while for him to recover, so if you don’t hear much from me over the next few weeks, that is the reason why.

I wish to thank everyone who gave advice about the constipation issue we have been having with my middle son (both on the blog post and on the Facebook fan page.)

Our GP has the series of these books displayed in his office since his name is Toby!

We have started seeing our GP (general practitioner) doctor for the issue. This guy is awesome. He managed my pregnancies and tried to deliver my babies (if he made it there in time!) He also broke the news of my youngest son’s cystic fibrosis diagnosis, which affected him greatly, as it was apparently the first time he had to break “BIG bad news” like that, and he knew our family quite well. He apparently admitted to my mum the night of diagnosis while I went home to pack bags to be flown to the next hospital that he was somewhat glad that it was me that he had to give this heartbreaking news to. It’s absolutely not because he didn’t like us and wished us hardship…it was because he knew we could cope with the diagnosis, and that I was able to hold myself together while he broke the news (I guess in comparison to other patients, who probably would have fallen to pieces.)
He has since managed Cameron’s CF as best he can under the guidance of the CF team in Perth (since we are country patients), the team love him for his support and the fact that he follows their directions…apparently something not all doctors do in their experience.

I trust my doctor completely. I know that he will not stop until we have answers, and he is willing to go above and beyond for his patients. He also knows that if I come to him with an issue, it is a legitimate worry, I’m not just arriving in his office for a sniffle or a meagre problem.

Mutual respect is essential with your doctors!

When I arrived two weeks ago with Ethan in tow, and told him of the serious issues we were having with the chronic constipation, which has caused us to make three visits to the emergency room since the start of the year, numerous amounts of medication (laxatives, suppositories, enemas….tried it all), and the issues the fecal overflow/accidents in the underpants was causing both at school and at home…he immediately took action. We are currently giving high doses of different laxatives to clear out Ethan’s intestines. He is on Movicol twice daily, Actilax twice daily, and Parachoc twice daily. Basically, we wanted him to get to the point of diarrhea to flush out his system before we take the next steps. In no way was he allowed to get even remotely constipated in the next two weeks, or basically, we would have had to start all over again

It took about a week for all the laxatives to take effect. After two weeks of all this medication, we went back to our GP for another assessment (this happened only yesterday.) The doctor was very happy at how much better his stomach felt…nice and soft, rather than lumpy and hard in places. He was also very happy to hear that Ethan isn’t having as many accidents in his undies. It used to happen about six times a day. It now happens only a couple of days. Still a drama, but definitely an improvement!

We are to continue the laxatives for another two weeks, but this time, add Benefibre to his drinks daily, which will absorb some of the extra fluid in his tract, and make his stools a bit more solid. We return in two weeks for another assessment to figure out where to go from there.

We also saw a dietician, as part of a feeding team, to assess his food aversion. This woman told me that Ethan’s diet isn’t too bad. I really don’t agree with that (neither does my GP), but I have taken steps to change that. We have increased his water intake, and I have started making muffins/cakes to put in lunch boxes or to eat as snacks at home, which is loaded with fruit and veggies (recipe at the bottom). We are going to try different types of milk when he has finished his medications, just to test a theory that there might be a bit of an intolerance causing the constipation. Ethan’s main “homework” is that he has promised to try at least one new food before we see the dietician again. He has agreed to try chicken…not chicken nuggets, but real chicken (we haven’t tried it yet.) He has also had a bit of a nibble on a carrot, after our babysitter (who he has a bit of a crush on) enticed him with a promise of a hug and kiss if he would at least try it. Since he is very much a Casanova, the ploy worked!

Basically this is going to be a long, ongoing battle, but I will keep you all updated.

Bella 🙂

***Here is my cake recipe. It’s a bit of a “thrown-together” recipe, as I just add whatever I have in the cupboard until it looks about right, but feel free to make your own tweaks:

Bella’s Fruit and Veggie Cake

1 carrot, grated
1/2 small zucchini, grated

Large handful baby spinach, finely chopped

2 apples, grated

1 pear, grated

2 eggs

40 ml oil

1.5 cup Self raising flour

60 ml milk

1) Combine all grated fruit and vegetables in a large bowl. Add eggs, combine thoroughly.
2.) Add milk and oil to bowl, mix thoroughly. Slowly sift flour through mixture in parts.

3) Add more flour or milk to reach desired consistency
4.) Pour mix into a large, grease cake tin, or separate into muffin tins.

5.) Bake at about 160 degrees celsius until golden and cooked through.

Asthma Peeps (product review)

I have recently been given the opportunity to test a product that is aimed at children who have asthma. The reason I put my hand up for this project was because a lot of people who have cystic fibrosis also do inhaler treatments.

Asthma Peeps is an Australian business who creates nifty little stickers suitable for spacers to make treatment more fun for children. Imagine, a child who is excited and happy to have their inhalers because their spacers are customised to their budding personalities. No fighting. No excuses. Job done.

Pirate theme stickers

In our little sample pack, we were sent some pirate stickers, which was absolutely perfect as my CF son, Cameron, is obsessed with pirates at the moment. Every second day, he is dressed up as one, and I can never keep up with if he wants to be called by his given name, or “Mr. Smee” (which for those who dont know, is Captain Hook’s minion.)

Cameron…or Mr. Smee?

Personalized spacer for inhaler treatments

Cameron was excited to help choose and place his stickers. Under each picture is a little space for you to be able to label their names.(A nice touch in my opinion.) We hit one snag with our testing phase though. Cameron doesn’t undertake any inhaler treatments for his CF! I still gave him the spacer to breathe through, which I didn’t mind as it encourages him to do deep breathing, which is also vital for CF physiotherapy.

So while I was standing there with another two sheets of stickers, I starting looking around for what else I could personalize. Then a brain wave came. Why stop at spacers? Cameron also uses other asthma tools for his physiotherapy. He has that little thing with the three coloured balls, that lift when you breathe in, and the idea is to get as many balls to the top as possible. (I’m sure there is an actual name for the device, but I have no idea what it is! We just call it the “Coloured ball thingy”)

Personalized PEP mask

Cameron also has a PEP mask. It has resistance when he breathes out. The better he gets at it, the more we increased the resistance. Stickers now decorate that too.

He giggles as he uses the mask, and the stickers we put on the “coloured ball thingy” are a good way for him to indicate how many faces he can achieve.

The stickers are fairly durable when you wash them – hand washing that is,not sure how they would hold up in a dishwasher (can these devices even go in a dishwasher???) This is great because there is no point on wasting your money on something that falls off in the slightest bit of water.
They don’t stick too well on silicone, as we did try them on another mask, but they fell off pretty quick!

“Coloured ball thingy” Does anyone know what it is actually called?

Each pack of stickers retails for $9.75. There are other asthma related product available on the Asthma Peeps online store

Visit http://www.asthmapeeps.com/ today and check out all the different designs!

Bella 🙂

Recipes for CF

Thanks to a friend, I came across this great Facebook page of CF fat boosting recipes.  The CF diet is a total learning curve, as it is high fat, high salt, and high energy. It takes a lot of getting used to, and you can sometimes struggle for ideas of how to boost meals.

I am going to add a few of the photos on my blog, but you have a child with CF, head on over and check out some of the comments as well.
I am also going to write a blog post soon about some recipes and tips for preparing meals for CF children, when you also have non-CF children who can’t have the same diet.
Try not to be *too* naughty and cook some of these recipes for yourself. They really aren’t the greatest in nutritional value for the “average” person!

Bella 🙂

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Latest health update

Hi all!

We have been home from our latest check-up visit in Perth for a week now, but I have only just now had a chance to give an update.

Sweat test device

We took our middle son, who is four and a half years old, with us for his sweat test to see if he has cystic fibrosis as well. He was one of our kids that we put a question mark over when we found out our youngest had CF. However, due to the long process we went through with our daughter’s diagnosis (or lack thereof) he was put on the back burner. However, I am very pleased to announce that his sweat test came back within normal levels. He does not have CF 🙂

We do not know yet if he is a carrier. He has to make the decision to be tested for that when he is older. Trust me when I say that we will be strongly advising him (along with our eldest son, whose sweat test also came back negative) to undergo that test!

Back to my CF son….

The respiratory doctors were basically “Thanks for travelling 450km. Everything is fine, we will see you in three months!” I guess you can say that is annoying, due to the travel, but its the best outcome you can hope for!

The dietician would like us to write a diary of his food intake for about a week before we return for the next visit so they can see how we can tweak his diet to boost his iron intake (as he is slightly iron deficient) I’m not sure how else we can do this without medication as he is an absolute carnivore! We try leafy green vegetables, but good luck getting those sort of foods into a two-year old! But they want to retest his iron levels in three months.

My son's daily dose of medications

The gastroenterologist was concerned that my son was at his maximum amount of enzyme tablets (pills to help digest food) a day, yet was still pooping too much. So he has been prescribed another medication, Losec, to help the enzymes break down. They will see how he is going at the next clinic.

We found an instant result with the new tablets. They almost work too well. But we can reassess later on. But for now, at least my house doesn’t stink as bad! But the poor little blighter is now on six different medications a day (that’s just when he isn’t sick!)

Overall, it was a successful trip for my youngest son. But, the biggest thing that had me walking out of the hospital smiling, was the fact that the doctors have taken me very seriously in regards to my daughter’s eating habits. They have agreed that we aren’t very well supported back home, so they going to class her as “CF” so that we can see their child psychologists on their team. Since she is coming with us again on our next visit, they are setting up an endocrinology appointment to rule out anything physical, since no psychologist will ever take us seriously without having ruled that out first. I just about kissed the doctor when I realised he was taking my concerns seriously, and was just as concerned as we are. A weight got lifted off my shoulders when I mentioned to him that it had been suggested to me in the past that she may have Prader Willi Syndrome, and he said it was one of his first thoughts of possibilities, but we have to do the endocrinology first.

It was the first time ever that I walked out of the hospital happy and relieved!

There will be more news on my son at the next visit in three months time when he has a heap of blood work done to test all his levels. So I will update you all again then!

Bella 🙂