Tag Archive | medical procedure

An eventful CF clinic visit…

Only a few short hours ago, we returned from our latest CF clinic visit at Princess Margaret Hospital in Perth.

The drive up (about 450km) was horrendous due to a lot of road works. The weather was even worse!

When we finally arrived to the hospital car park, my husband was trying to get Cameron out of his car seat (while Ethan and I were loitering around nearby, waiting) when the heavens opened. Hubby had the smart idea, and jumped into the backseat to wait it out.
Ethan and I, already slightly damp, were in a position where we were committed to making a run for it.

That was when things really pelted down!!!

Ethan hates water, especially cold water, so he was screaming and crying during the whole mad dash. Mark and Cameron caught up soon after, but missed the worst of it….but we were all drenched. Luckily, I has the smart idea to grab one of the kids clothing bags (as we hadn’t booked into our hotel room yet) so I was able to change the boys into dry clothes, but we were still bombarded with looks of sympathy from the other families in this clinic area as we were provided with towels by the nursing staff. I literally had to pour water out of my shoes, but my main concern was the boys catching a chill and developing a cough in this cold weather.

Cameron has put on a staggering 3kg in the last three months, which is awesome! While we have never really struggled with weight gain, there was a concern for a while when he was hardly putting any weight on…but that was during a time when the house was constantly bombarded with the gastro bug.
He is very stocky and muscular…and not at all happy with hubby and I at being told he is too heavy to be carried around any more!

Meet "Mr. Wobbly"- called so because he makes your cheeks "wobble" during the test.

Meet “Mr. Wobbly”- called so because he makes your cheeks “wobble” during the test.

Since he turns four years old next week, he is now old enough to try the “Mr. Wobbly” machine to test lung function. So off we toddled to the respiratory department to attempt this new test (previously, he undertook the infant lung function test, where he was sedated, placed in a special vest, then had air pumped into his lungs, then the vest would squeeze it out and record the reading)

I am very proud to announce that his first attempt went very well and we were able to get a good reading! While heading back to the clinic area with test results in hand, hubby and I had a quick read…but could not make heads or tails of the results. We assume that since nothing was highlighted in red to show that levels were outside of normal results, that everything is all good!

With a lot of waiting around, we finally saw the respiratory doctor, who was very happy with Cam’s progress.

We brought up the subject of my daughter, Eva, who is a CF gene carrier, and has floppy airways and frequently gets croup. There is still a slim possibility she may also have CF, (though the specialists are fairly confident she doesn’t actually have it) but due to her current respiratory issues, they are still monitoring her.
Winter is horrible for her, with endless bouts of croup and nasty sounding coughs, which can last for weeks. For the last three weeks or so, her coughing has progressively gotten worse, especially in cold air, worse again at night, then worse again when she lays down in bed.
The doctor recommended undertaking a week of Augmentin Duo (antibiotic) for a week, then trying Ventolin (reliever inhaler) for a week. We are to try them for a week at different times (instead of the same time) to see the results of each. He was pretty sure that neither would work, and she may have to trial Flixotide (steroid inhaler)
Since we have only just come home today, we will be starting these treatments tomorrow, so I will keep you updated with her.

According to Ethan, lollypops do NOT make everything OK!

According to Ethan, lollypops do NOT make everything OK! (not an original photo shown)

Cameron and Ethan both received their flu vaccinations while we were waiting in clinic. Cam is used to being poked, prodded and jabbed, so when the nurse pushed the needle in his arm, he gave her a small, indignant “Ow!” (Hubby was with him, and I was sitting about 5m away with Ethan, and I didn’t even hear a peep!)

When Cameron walked back, proudly showing off his lollypop reward to his brother, Ethan was quick to say he wanted one too. I warned him that he was going to have to have some medicine first in order to be allowed to have a lollypop too. He gave me a brave nod and a confident “Okay!”, took his father’s hand, and went to meet the nurse.

Within minutes, all heads in the clinic turned towards our direction in alarm when we all heard hysterical screaming of pain!!! When the adults saw me laughing, they realised that my son was receiving his shots, and gave us looks of sympathy, while giggling along. One very red-faced, teary five-year-old boy walked up to me, holding his lollypop like a second place trophy while looking at it as if to say “Was it worth it, for THIS?”

Little does he know, since it was the first time he has had the flu vaccine, he is going to have to go through the ordeal again in four weeks time for a second dose!

In a way, it was a pointless trip, as Cameron’s health is pretty good, but we needed to restock medications (and it would have cost a bucket load to have them sent to us), he was able to take the next step is CF monitoring by being able to undertake this new way of lung function tests, and the boys are prepared for winter with the flu vaccine (just need to get the rest of us done now).

As much as clinic visits are a drain- physically, emotionally and financially they are an essential part of CF treatment.

Bella 🙂

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The “Mara” family’s story- part 1

Some time ago, I put the call out into cyber space, for any parents of children with special needs to share their stories.

It wasn’t only limited to Cystic Fibrosis or Global Development Delay. Anyone who wanted to share their experiences as a way to vent, or hopefully help someone in a similar situation, were (and still are) invited to tell their stories.

The wonderful Carly Mara accepted the challenge 🙂

Her daughter, Ava, not only has CF, but was also born with a cleft lip, and was also dealing with hip dysplasia. While all this was going on, Carly’s father had a stroke.

Her story has touched me. I can relate to so much of it (well, the CF side of it anyway!) I found myself nodding along as she describes her emotions. She has explained them so much better than I could!

Thank you so much, Carly, for sharing your family’s experience (and providing pics)

Follow her story. We will be publishing it in installments.

Bella 🙂

 Here is part one of Carly’s story….

 

Meet baby Ava. Isn't she adorable???

The 14th September 2010 was just like any other day.

We had a 3 week old baby girl who was born with a Cleft Lip and was also in a froggy brace for Hip dysplasia. Her cleft was discovered at my 20-week anatomy scan(another day I will never forget!)

 I was devastated. I just couldn’t believe this had happened to our baby girl’s face! It was hard enough getting her here to begin with, being that she is our IVF miracle I was sure that was the biggest hurdle we were going to have to overcome! So once I got those two little lines, and the confirmed blood test, I thoughtWOW. We did it!”

Little did I know there were three things waiting for us around the corner, the biggest blow coming to us last.

I remember the midwife coming to check on Ava. and also to do the Guthrie Test. It’s funny. you know, cos when I think back, the midwife handed me a pamphlet explaining the Guthrie Test, how it was done and what it screened. I had a really quick look at it and placed it on the table. My mother-in-law said “Have you read through it?” I said “Yeah” quickly, then the midwife said I should sit down and have a read.

Once again, I just flicked through it thinking to myself “Nah it’s all good. We’ve got the cleft lip and the brace (which was fitted at three days old)…there couldn’t possibly be anything else.”  So the whole thing just left my head. Test was done, Ava was looking well and doing everything a newborn should be doing so all was great. I never gave that Guthrie test another thought.

We knew Ava’s Cleft would be repaired at 3-months-old, so we were concentrating mostly on that, trying to prepare ourselves mentally and emotionally. So the morning of the 14th September, I was sitting on my bed, Ava was asleep and my hubby was out in the kitchen. The phone rang, he answered it. I could hear him talking for a while but couldn’t hear what was being said. Didnt really think much of it. Then he hung up walked in and said to me ” That was a lady from Princess Margaret Hospital. She said Ava has tested positive for Cystic Fibrosis”

I just looked up at him and said “What?”

Ava in her brace for hip dysplasia

I was dumbfounded! Mostly cos I wasnt too familair with CF. I knew it existed along with a million other diseases. I knew it wasnt a nice one, cos I remember a conversation with my mum years ago talking about her nursing years. She said that from things she saw she never wanted to have a baby with four particular diseases, one of which was CF.

I asked hubby to tell me the whole conversation, so he repeated what he just told me. I demanded more information, but I think he was stuck too, not knowing exactly what had just happened. But luckily he wrote down a phone number, so I called and got Liz on the phone. She explained that the Guthrie test had come back positive for Cystic Fibrosis. She gave me a rough run down of what it was. I just kept asking her if they were sure, like, really, really sure. Is it possible they’ve made a mistake? Are there any other tests Ava has to do to further confirm it?

Liz told me about the Sweat Test. I think I was on the phone to her for about twenty minutes, going around in circles pretty much, trying to comprehend what was being said, and at the same time flat-out rejecting it. I wanted to scream and cry. I also felt totally numb and angry and confused.

I think about a week or two later we had to go to PMH for CF Education, woah! What a head spin that was! So many people, soo much information. All so scary, confusing and overwhelming. I think it was even harder for me at the time, because the same day as Ava’s diagnosis, my dad had his second stroke, so we were between Sir Charles Gardiner Hospital & PMH for quite some time.

Thank God we were given so much literature, cos there is no way in hell I could have retained much information. Everyone time someone walked into our little room to talk to us about their part in CF treatment, I felt defensive and a little stand-offish, like it was their fault we were there. I felt some kind of resentment towards Liz for calling us that day too. Every time we would get a phone call or appointment letter I would feel angry because I just wanted us to be left alone. I had waited sooo long to finally have my little baby, and I was having to share her with all these people.

Being shown all the physio positions and all the medication Ava would be on was very overwhelming. Where physio was concerned, for a few months, it wasnt at the top our priority list because Ava had to be in the froggy brace for 23 hours a day (it could only come off at bath time). Our physio understood, so that was one less thing taking up space in my head.

 Our first lung function test was a whole lot of drama for nothing. Very stressful the first time around. The sedative didnt do its job. Ava was screaming and crying, which was upsetting me. I hated seeing her have to go through all this stuff. At one point, hubby was holding Ava in one arm and vacuuming the main desk in Respiratory Department, trying to get Ava to sleep (she was a white noise baby for quite a few months. I spent many hours with the blow dryer going or the vacuum putting her to sleep, even at 3am!) But unfortunately this day at PMH it just didn’t work. So after an hour or so, we decided it was time to go home, thank God! Once home, Ava slept soundly -of course!

Our first Bronchoscopy was an experience too!

Fasting a baby is horrible. Being in the waiting room with other parents and their children, waiting to be called in, while your walking the floors with a screaming baby is no fun at all! Plus being a bag of nerves knowing what is going to happen, your mind going over what they might find in those little lungs, it was really starting to do my head in! Then finally we were called in. The walk down the corridor felt so long, cuddling Ava soo tight and giving her the biggest kisses as I hand her to my husband, Chris, so he could be with her when she goes to sleep.

I just felt sooo helpless and guilty, and wishing I could take it all away from her, or that I could go through it all for her. We waited in the Friendship Room. I finally sucked my tears up and it was all over. Into recovery and there she was, my tiny baby, sound asleep. So the tears started again. The results from the bronchoscopy came back fine.Yipeeee!

 One bronc down, Cleft lip repair to go!

To be CONTINUED……Keep an eye out!!!

 

 

***Extra Notes***

 The nurse mentioned in this installment, Liz, will be known by West Australian CF families. To those who don’t know her, she is a clinic nurse on the Princess Margaret Hospital CF team. She is the “guru” CF nurse.

The” Friendship Room” mentioned, will also be known to families who have been at PMH. It is a waiting area for parents to sit in while their children are in surgery. It is run by hospital volunteers who serve tea/coffee, provide plenty of magazines and conversation. They receive the call when your child is out of theatre, and escort you down the passages to where your child is in Recovery.

Pseudomonas are bad…

We recently returned home from Perth where my son had his CF annual review…which didnt go terribly well.

The doctors were slightly concerned about his poor weight gain over the last few months. However, once they heard that during this period, he had about three weeks of a gastro bug, on and off, they weren’t terribly worried, we just have to work a little harder to get his weight back on track.

He has a few new exercises to do during physiotherapy, as he is now at an age where he can start deep breathing. They don’t expect too much from him yet, but more jumping around, tickles and blowing bubble are happily prescribed.

The bronchoscopy on the final day of annual reveiw was a massive drama. First off, I hadn’t received the usual phone call for fasting times from the hospital, and I thought I had worked  the times out correctly when I gave him a bottle of milk. I worked the times out wrong, which meant theatre had to delayed by nearly two hours.

When he was in the second stage of recovery, the young one started screaming. He was hysterical!! The nurses felt that it was like he was trapped in a nightmare as he got worse when anyone came within view or spoke, he would just scream harder. After an hour and a half of this, the nurses felt they should call the anethetist back in the hope to sedate him. When they finally tracked him down and he arrived, it had been two hours of screaming!! The doctor wouldnt sedate him however as his lungs werent in a good state and he didn’t want to take the risk of breathing difficulties from sedation….which meant we just had to ride his extreme (and apparently rare!) disorientation. It took nearly two days for him to completely get back to normal.

When the respiratory doctor spoke to me about the procedure, he had said that the lungs didn’t look so great, but they would have to look at the test results . He said there was a possibility that we would have to return to Perth the following week for treatments. I kinda figured then that we had reached the point where Pseudomonas (CF super bugs mentioned in previous blog posts) had entered his life. A gut feeling told me that we would be returning to Perth.

So when I called the hospital back after a few days for test results, the CF nurse told me that he had been flagged for Pseudomonas, but they wanted to watch the samples for another 24 hours to be sure. Yep…the next day, a phone call to confirm that he had them and he needed to return to the hospital to start intravenous antibiotics as soon as possible.

It has been bedlam trying to get organised as my husband is on the other side of the country at the moment for training. Getting a hold of him to tell him the news was a mission in itself! My son and I leave for Perth tomorrow to be admitted into Princess Margaret Hospital for Children, I will try to keep you updated

Now, I know you are probably sitting there wondering “What are Pseudomonas??” Well, to be honest, I can’t really answer that properly right now. I could spout off all this stuff, but I have no idea if its accurate. All I know, as my title suggests…they are bad!!  If untreated, they can destroy lung tissue, causing breathing problems, decreased lung function, and in worst case scenarios….death. There is no way of preventing it, and it is very common in CF patients. It can be found in water, food…all the common places.

I did something I know you aren’t meant to do, and I googled it. I had to stop myself because I knew that what I might be reading may be out of date, or different information due to geological location. I found this article, which was published earlier this year, which may help answer some questions.

My son doesn’t look sick at the moment. He is running around like a headless chook and eating everything in sight…then asking for more! He has a cough at the moment, which gets a lot worse in the cold air. This is the only indication that there is something going on in his little body. They said it may explain the poor weight gain.

But as I said…I will try to keep you updated over the next two weeks that he is in hospital for, but I’m not sure what the likelihood of that is.

Wish us luck and good results!

Bella 🙂