Tag Archive | health

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.

kalydecocollA 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.

orkamabiFast 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?

price

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 funny

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.

Cam sick

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.

Cam nebuliser

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 Pep

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.

Cam eating

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!)

Silly Cam

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 🙂

 

The realisation of mortality

Hi everyone, I’m sorry it has been a while….but it’s been a bit rough around here lately.

Cam during nebuliser treatment

Cam during nebulizer treatment

Cameron’s health hasn’t been the greatest for a while now. A few weeks after our last annual review, he got very ill. I hadn’t seen him that sick since he was diagnosed! We found out his bronchoscopy had results showing he was growing Staphylococcus (staph), which can cause major havoc on CF lungs.
We started nebulizer antibiotics, which showed good results for a while.

He is now coming into his fourth week of nebulizers, but in the last few days, he has gone downhill again. He is coughing a lot, breathless, has no energy, grumpy as hell and off his food. This has caused us some major concern!
We are just waiting for two more new antibiotics to arrive from the specialists in Perth. One is an oral antibiotic, designed specifically to treat staph infections. The other is a very strong nebulizer antibiotic (again, designed specifically for staph), which when it arrives, we have to arrange with our local hospital to administer the first dose so that he can be medically monitored for any side-effects. Providing it all goes well in the ER with first dose, we will be continuing them at home. So for the next month, he will be on four different antibiotics!!
I am glad that we have narrowly dodged a hospital admission at this stage, though I have this niggling worry that it will inevitable. Please wish us luck and we welcome all healthy vibes that are sent our way!

I will keep you updated with his progress when I can.

We live with CF every day. We know the risks involved, what might or even what WILL happen during the journey of this condition. But knowing it, and being AWARE of it are two very different things.
I constantly talk about CF. I raise awareness as much as I can. I try to stay up to date with medical updates. But when your little one is very sick because of the condition, you remember, it’s not just about being knowledgable. The “la-la land” bubble bursts and all these things you “know” about are an “in-your-face” reality. This is my son’s life. This is his permanent battle. THIS SUCKS!

While we are dealing with this, the dirty “C” word has infiltrated itself into the family. No…not that dirty “C” word…..the other one….cancer. I don’t want to breach the privacy of the family member who has been battling this for a while by revealing too much on my blog (and I hope they don’t mind too much that I have written about it right now) Our head spaces have been zooming all over. We have also had to come to terms with the fall out of this nasty, evil condition as well.

My own health hasn’t been the best lately either. I am currently trying to quit smoking. I have to do this, I need to do this….I WILL do this. But like previous attempts at quitting, my lungs go spastic on me. I always get chest infections when I attempt to quit. But a recent incident brought home my own mortality.
For a week, I was tight chested, wheezing slightly….the symptoms I usually get in the days after my last cigarettes. But three nights in a row, I couldn’t lie on my right hand side (left hand side or stomach was fine, as was upright…I could breathe). On my right side, I would instantly stop being able to breathe and would go into a coughing fit that would take half an hour to recover from. I tried to hold out until Monday, when I could see my doctor, but on Sunday morning, I woke up on my right hand side. It took forever to be able to catch a breath. I was legitimately worried about dying right then and there….so I took off to the emergency room.

They gotta go. I KNOW I have to stop!

They gotta go. I KNOW I have to stop!

I was treated very thoroughly…more so than I ever have before. I had three nebulizers to relax my airways, and two steroid tablets. They did a chest x-ray, which came back clear and gave a sputum sample. They even inserted a canula into my hand, gave me a fluid drip and took blood to test everything from white blood cell counts, to salt and insulin levels. It didn’t feel like an asthma attack, but it didn’t feel like a usual chest infection either. While they treated and got rid of most of the symptoms, most of the tests came back clear, so we don’t know exactly what caused the respiratory distress yet. I have to call for the last few results tomorrow…so hopefully I get some answers.

While I am feeling much better, the experience scared me greatly. With Cameron’s current health scare, plus the cancer in the family, all I could think of was “I am not allowed to die! I can’t do that to the family…not now! I am too young! My family is too young for me to go!” I came to realise, like most mothers, while I am very good at looking after my family, I also need to look after myself! I am getting back on track with my diet, the cigarettes are in the past, and I need to look after myself better mentally and spiritually as well.

I have been under a lot of emotional stress lately. I am run down. My husband and I have started scaling back our workload a little to take the strain off. This is already an immense relief. I need to make more time for my family.

I need to make more time for ME!

Bella 🙂

In desperate need of advice!

To all my readers, please help!

My middle son, who is five and half years old, has a massive problem at the moment. I will start with a long story to lead up to my question (and I apologise in advance for the information overload!)

Just EAT dang nammit!

Just EAT, dang nammit!

For the last few years, he has been an exceptionally picky eater. This kid doesn’t eat meat, fresh chicken (but will eat chicken nuggets), vegetables, pasta, rice and most fruit! For as long as I can remember, he has been living on bread, cheese, yoghurt, bananas, pears, chicken nuggets, hash browns and polony (only recently branching out to cereal and eggs)
Basically, the more processed it is, the better!
Of course, I realise this is a terrible diet, but the kid has to eat something! I tried to be tough on him, and that if he didn’t eat what was put in front of him, then he would not get dessert, and went to bed hungry. But he is just as stubborn as I am, and would happily go off to bed with his stomach empty. After a couple of days of not eating his evening meals, I would cave and give him cheese on toast, so he would at least have something in his belly!

It’s weird. If the other kids are having pizza, he isn’t interested, but would have cheese, ham and sauce on toast (go figure!?) He did recently try pizza for the first time at my Mum’s house, so hopefully I can convince him to eat it at home now!
At Christmas, we realised how much he loves eggs too, as he devoured about 7 boiled eggs! So now, he will happily eat quiche (but in our house, we have to call them “Eggy cakes” for him to eat them) I have tried to start hiding veggies in them, with zucchini making it down the hatch without any question. However, if I try putting carrot in them, he wont touch it! He wont even eat carrot cake because of 1.) the name, and 2.) because it has carrot in it!

A few months ago, my concerns for a balanced diet reached an all time high, as he was showing signs of malnourishment. He looked like an Ethiopian orphan…skinny arms and legs, spine visible, with a distended belly. So I started giving him a glass of fruit and veggie juice at least once a day. I made him pick out a special bottle and it became known as “Ethan’s special juice” (In no way was I going to tell him what was actually in it…but at least he was excited to drink it!)
About three months ago, I was talking with an occupational therapist, who was a guest speaker at my special needs support group, who recommended that we put in a referral to see a feeding team to get him to eat properly. This is a team of dieticians, speech therapists and occupational therapists.

A psychologist is being considered, as he will start making excuses as to why he cant eat if  he finds out earlier in the day that dinner is something he doesn’t like (ie. Im too tired to eat…etc) If he has the plate in front of him, he will actually start shaking as the food approaches his mouth. If (say, for example, a tiny piece of chicken breast) manages to make it into his mouth, he will gag on it, but hide it under his tongue and will say he swallowed it.

I jumped on board and got the paperwork started. We are still waiting for our first appointment.

We were so happy and proud when we didnt have to use these anymore.Do we need them again? :S

We were so happy and proud when we didnt have to use these anymore.
Do we need them again? :S

For at least a year, he would make a mess in his pants, but not know he had done it. If you asked if he had a “stinky bum” (code in our house for having done poos…sorry for the “too much information”) he would reply with an innocent “No!”
He would have a smear in his pants, but it would stink out the house with a distinctive and unmistakable smell. The problem was that he had no idea that he had done it. Doing wees on the toilet wasn’t an issue, however I couldn’t trust him to wear undies, so he was constantly in nappies or pull-ups.
I tried to give laxatives every now and then, thinking constipation was probably the issue. I thought it worked, but it would just happen all over again a few days later.

The crunch came around New Year’s, where one night he couldn’t eat anymore, not even his beloved toast, and was complaining of a sore belly. I poked around his belly, with possible appendicitis in mind, to find that his belly felt rock hard. I sent him to bed and decided to see what he was like overnight.

By morning, I could actually see his large intestine bulging through his stomach. I knew that there was a serious problem. I took him up to the emergency room at the hospital, where they performed an xray on his stomach. It showed chronic constipation, with a lump the size of a fist in his pelvis, and enough poop backed up the intestine to hypothetically fill a football! He was in some serious trouble! The small amount of mess in his pants daily was because of fecal overflow, where poop was leaking around the blockages. The reason he didn’t know it was happening was because the anal muscles were that stretched, they had lost the ability to tell that there was poop there.

The hospital staff gave him oral laxative, and an enema (which of course he didn’t enjoy) We were sent on our way with a warning that we had about 20min to get home before things started happening.
Unfortunately, I had to take off to work straight after, leaving him in the care of my husband…and Ethan screaming on the toilet over his ordeal. When I came home, my husband said that it seemed to do the trick. He was able to eat again, but we were still getting the smears 😦

About 5 weeks ago (around 2-3 weeks before he was due to start pre-primary) I woke up to a massive mess. I have never seen that much poop before…but it was obviously what he needed, because after that, he was consistently using the toilet, no accidents. We were jumping for joy that he was able to start wearing undies again.
By the skin of his teeth, he was in undies when he started school. A massive load came off my shoulders…until about a week and half ago…

He has started messing his pants again. Despite the advances in what he is eating, he is obviously “bunged up” again 😦

This is becoming an issue at school. We don’t want him becoming notorious for smelling and messing his pants. It can’t be comfortable for him, and it is a bit of a health hazard for the other children. It is happening daily.

His teacher has pulled me aside a few times to discuss the issue. She is an awesome teacher who knows all the dramas in our family (and feels so sorry for me because of this extra stress) She is going to speak with the school nurse for advice in the meantime.

I obviously need to put him back on laxatives for a while. I will also be booking an appointment with our GP. I am desperate for advice from parents who may have gone through a similar situation, as I am honestly lost as to what to do!

I am pleading...I am desperate for advice!

I am pleading…I am desperate for advice!

If anyone out there had any advice, whatsoever….please, please share! (Bare in mind, I have taken the steps to get professional medical advice.)

Bella 🙂

CF fundraisers

There are a few of people doing things to raise money for cystic fibrosis at the moment, so I thought I would share their efforts in the hopes that they will get closer to their goal.

team_cropGavin Pearce is a 49-year-old father of three who lives with cystic fibrosis and has twice been the recipient of a double lung transplant. On Saturday 23rd February Gavin and three teammates – Rob Pearce, Leanne Bodley & Phoebe Robinson – will form the Lung Distance Swimmers and take on the epic challenge that is the Rottnest Channel Swim. They aim to raise $25,000 for Cystic Fibrosis WA as they battle the wind and the waves.
You can donate go to the Lung Distance Swimmers page to keep updated and donate. You can also listen to an interview via podcast here

 
Spinner_logo_finalSix every day office blokes have decided to trade in their comfortable spinning office chairs for spinning bike wheels as they set off on the 2013 Gibb Challenge from the 19th – 23rd of May. This expedition will take them 700km in a team relay across some of Australia’s toughest outback terrain as they work hard to raise their target of $50,000 for Cystic Fibrosis WA. Give them a helping hand to reach their goal by visiting their online fundraising page

 

 

 

Baker's DelightThis next one isn’t exactly fundraising for cystic fibrosis (but does have CF kid as a spokesman!) W.A. Baker’s Delight are holding a Bundraiser for PMH (Princess Margaret Hospital for Children) on the 16th March, 2013.
Other states are fundraising for these major hospitals across the country:
Royal Children’s Hospital (VIC)
Royal Children’s Hospital Foundation (QLD)
Sydney Children’s Hospital (NSW)
John Hunter Children’s Hospital (Newcastle, NSW)
Canberra Hospital (ACT)
Women & Children’s Hospital Foundation (SA)
Royal Hobart Hospital (TAS)
$1 from every 6 pack or hot cross buns sold at Baker’s Delight stores state-wide will be donated to the PMH Foundation

If I come across any other fundraisers, I will let you know. Feel free to share any links to fundraising pages in the comments below.

Bella 🙂

GIANT decisions

Howdy readers,

Tonight’s subject is a bit of a touchy one. I have been wanting to write about this for a little while, and after a couple of glasses of wine, inhibition have flown out the window, allowing me to write about a bit of a taboo subject.

I am constantly asked one of two questions. “Are you going to have more kids?” or “You aren’t having anymore kids…are you??

My answer is always the same: While I feel like my body has the ability to have one more child (and I would love to have another child, especially another little girl) My husband and I have enough factors against us to not have any more.

The movie the inticed my husband and I to have a large family

The movie the enticed my husband and I to have a large family

My husband and I had always decided to have a big family. Hollywood effected us a bit in this department due to the movie Cheaper by the Dozen. While we knew that no way in hell were we going to have twelve kids, we agreed (for a while) that seven was going to our magic number. This was taking into account “whoops” babies, and the high possibility of twins.

Our first child was planned, although he came along a lot faster than we intended. We were expecting to by trying to conceive for a while….it only took about two months of some-what trying (not exactly trying, but not exactly stopping it either) A few false negative home pregnancy tests left us shocked at our first ultrasound when I was seven week further along in pregnancy than we thought. Though, we were excited. We had recently bought our first home together and were ready to start a family.

But then I fell pregnant again six months later (whilst I was on the pill)….then amazingly, I fell pregnant within seven weeks of giving birth to my daughter! Just a year after giving birth to my middle son, I fell pregnant again (and again, while on the pill!)

Four single births in four years is a damn good effort!! But this is not the reason why my husband and I have decided not to continue on to our goal of seven children.

As you all know, my eldest two have significant development delay. It is something that seems to run in my family, with other family members that have had development issues as well. But most significantly, our youngest son has cystic fibrosis. If you have listened to me ramble on enough, you would know it is a genetic condition that has to come from both parents. This is the main (and its a very big one!) reason why we aren’t having any more children.
We have had such a good run with my youngest son.After he was diagnosed,  I was expecting to be constantly in and out of hospital. We have been incredibly lucky to have only had two CF related hospitalizations (one other was due to a bad case of tonsillitis)

What if we aren’t so lucky with the next child? What if that child is very sickly and has a lower quality of life? Since two CF people aren’t meant to be in close proximity of each other due to a high risk of cross-contamination….what if the new baby (if it had CF) makes my youngest son even more sick. Since we have already had run-in’s with CF-super bugs, Pseudomonas, what if my youngest son makes the new baby very sick?
Development delay is so predominant in our little herd, what if the next baby has issues as well? That would be another strain on the family. To be constantly one step forward, two steps back. It’s hard enough already!

These are all very important factors my husband and I have discussed about a lot since my youngest was diagnosed. Please do not judge us, but we have decided that should I fall pregnant again (since contraception quite obviously doesn’t agree with me) we would undergo an amniocentesis( or whatever test they do for this sort of thing) and if test results come back positive for CF, we would terminate. I know it would be an exceptionally hard decision for my to make, and will create a lot of emotional turmoil….but it would be the best thing for our family in the long run.

However, we have been blessed with four children already. It was easy for us to be able to say “NO MORE” (even if my biological clock is still ticking for one more…I know it isn’t the right thing to do for us) It was our fourth child that was diagnosed with a chronic medical condition.

But what if it was your first child, or even your second…and you wanted more? Or if you met a new partner, who was also a carrier, and you wanted to have more children with that person? These are the people I feel for as they have so many emotional, and moral, questions to ask themselves.

To quote someone who I spoke with about this issue a while ago….the following questions are the sort of thing that come to mind (and I am just using CF as an example here. Any medical condition can be placed here):

Do you or don't you?

Do you or don’t you?

*What are the issues of having more children with CF? (eg. financially, mentally/emotionally, physically) especially when there is a 1 in 4 chance. (Or if you have CF yourself, and your partner is a carrier, you have a 75% chance of having a CF child)

*Do you have kids knowingly that you risk creating a child that will suffer all its life?

*Do you conceive, then terminate if it is discovered it will have CF? Then does that diminish the value of life for those who already have CF?

* If you go through IVF and discard the embryos that have CF, does that make it any different to an abortion?

* If you already have CF and your partner is a carrier, will you be able to care for the child when you are sick? Do you take the chance knowing it will be a huge toll on your body (as a female with CF)? Do you have children knowing the risk that there is a chance you may die young?

There is no right or wrong answer to any of these questions. The love for family can overcome all of these issues, but then again, it might be too much than you can deal with. Don’t ever feel pressured into such a big decision. It all comes down to what is best for YOUR family!!

I hope I have given you some food for thought.

Bella 🙂

****EXTRA NOTES****

This post is not about trying to stop people from having children, but more to bring attention to the thought process that plagues some people about extending their family when they know a medical condition is possible.

I know this is a bit of a controversial subject, as it touches on pregnancy terminations, so while comments are encouraged, I will delete any nasty ones!

Still here!

I haven’t written a post in over two months, but I am alive….barely…but I am still kicking.

😦

Our family has had a horrible winter, with the flu hitting the house hard (not a bad cold, a real flu!) I have had it twice so far this season, and I am currently on day 13 (I think, I have lost count!) of my second flu, and this one has absolutely knocked me for six! For the first week I was in a lot of pain, coughing to the point of vomiting (eeew!!) and could barely move. My husband has been absolutely wonderful looking after me and the kids, who also came down sick.
I haven’t forgotten about my blog. I just havent had the energy to even look at it, let alone use the noggin to write something! It’s great to see that despite my silence, it has still been generating a lot of hits!

I have a lot of topics to get to from recent months. My youngest son had his annual review a few weeks ago. My daughter has one very long story to catch up on with her latest diagnosis (or lack thereof). My eldest son has his own story to tell too. I am also hoping to have follow-up’s of both the Mara and MacFarlane diagnosis stories.
So keep your eyes peeled. This stories will probably trickle out slowly, but they will be done!

 
In the meantime, please send all healthy vibes our way!

Bella 🙂