Michael, Cameron and Abbie

Michael, Cameron and Abbie

Links to the beginning of the journey .. and info on his siblings heart conditions

Sometimes it is easy to “forget” that Cam has a serious heart condition and obviously someone forgot to tell him as he has more energy than many of us put together & such a zest for life … I remember seeing somewhere a slogan -“ Half a heart – not half a life” and this is certainly true for Cameron.

We hope you enjoy reading his story and that it will give you some hope with the obstacles you may face …

What is Hypoplastic Left Heart Syndrome (HLHS)?

Hypoplastic Left Heart Syndrome (HLHS) is a Congenital Heart Defect where the left side of the heart has failed to develop properly. There are varying degrees of malformation in the Hypoplastic Left Heart. The left ventricle is usually very small or non-existent. The aortic valve may be narrowed or closed and the aorta itself may also be very small. The left atrium is sometimes small and the mitral valve between the left atrium and left ventricle can also be narrowed or closed. These defects put extra work on the right side of the heart, so chambers and blood vessels on this side may be enlarged or stretched larger than normal.


To see the start of Cam's (and our) journey with his heart please
follow this link ...

Cameron's Hearts of Hope web-pages
Some of the photos and story you may find graphic, just to let you know.

Cameron’s twin brother Michael

Michael was also born with a congenital heart defect (CHD) called Pulmonary Stenosis. Up until the age of 2 Michael was monitored 6 monthly by echos and Paediatric visits and then at the age of 3 he was discharged as the Stenosis had corrected to an acceptable level so that it was classified as mild.

What is Pulmonary Stenosis (PS)?

This is where the Pulmonary Valve is thickened and narrowed leading to the development of abnormally high pressure in the right ventricle. The right ventricular wall becomes thickened ("Hypertrophied"). Stenosis (narrowing) of the pulmonary valve restricts flow into the pulmonary arteries. This leads to the presence of a heart "murmur". Often the narrowing is mild and does not put significant strain on the heart

Cameron’s sister Abbie

In August 2006 Abbie was given an echo to check out her heart "as a precaution" due to the severity of Cameron's heart condition & Michael's condition. Unfortunately we were given the news that Abbie has a congenital heart defect (CHD) called Aortic Stenosis. At that stage it was classified as a mild case at this stage caused by thickened leaves of the valve but because of the nature of aortic valves and she had to have frequent echos to ensure that it doesn't get worse, and just stays the same.

Unfortunately with the last echo in 2009 the Stenosis had worsened and has become mild-moderate and she will be reviewed again before she goes to school to decide whether she will need the corrective surgery on the valve. It seems that like Cam that her valve is bicuspid (the valves should have 3 leaves but both Cam and Abbie have only 2).

Aortic Stenosis

The Aortic Valve is thickened and narrowed leading to the development of abnormally high pressure in the left ventricle. The left ventricular wall becomes thickened ("Hypertrophied").

Stenosis (narrowing) of the aortic valve restricts flow into the aorta. This leads to the presence of a heart "murmur". Often the narrowing is mild and does not put significant strain on the heart.

However the narrowing frequently worsens with growth. If the obstruction is severe, symptoms may develop, or the heart may show evidence of "strain". The valve may require treatment to open it up. This may be surgical or with the use of a "balloon catheter" procedure.

SELECTIVE MUTISM

Abbie was diagnosed with Selective Mutism in March 2011 .. following 2 years of kindergarten where she never uttered a word .. towards her going to school she was referred to Special Education and support is now given to her at school.

What is selective mutism?
Selective mutism is a severe anxiety disorder that 7 in 1000 children suffer from (the same prevalence as autism spectrum disorder). People with selective mutism have social or other anxiety so extreme that they are physically unable to speak or otherwise communicate in certain situations, usually school and other social situations, despite being perfectly able to speak and displaying normal social behavior in others, usually home and sometimes a trusted friend's house. The "talk" and "non-talk" zones are different for every sufferer -- for instance, some may speak to friends at school but not answer questions -- and some find it easy to communicate nonverbally or even whisper in no-talk situations while others are all but paralyzed. Some children grow out of their anxiety, while others begin to speak but develop social phobia and still others enter adulthood unable to speak to most people.

"Selective mutism" is far better than the former name, "elective mutism," but it unintentionally perpetuates two misconceptions: that the child can "select" which situations to speak in and that the disorder centers around mutism. In fact, people with this disorder have no control over when they can or cannot communicate (and very much wish they did), and mutism is only the most visible symptom. Most, though not all, selectively mute people have trouble nodding, pointing, smiling, writing, looking somebody in the eye, or using various other methods of communication when they are nervous. Many therapists and parents fail to realize that these anxieties must be overcome before the sufferer can even consider whispering, let alone speaking. Furthermore, "selective mutism" makes no mention of the anxiety that causes the problem, leading to confusion about treatment methods.

This disorder is not terribly rare, yet most child psychologists and speech therapists have never heard of it, do not understand what it is, or have no idea how to treat it. Some children with selective mutism are incorrectly diagnosed with autism or mental retardation. The majority of them are punished for their failure to speak, since their parents and teachers assume that they are able to do it in all situations and that they are simply being stubborn. This makes the child far more anxious about situations in which they will not be able to speak and lowers their self-esteem as it is constantly being pointed out that they are unable to do something that "should" be easy for them. Many children with selective mutism grow up to adults with multiple severe anxiety disorders, depression, eating disorders, and/or substance abuse. However, selective mutism can be cured with cognitive-behavioral and/or speech therapy, medication in some cases, and understanding and support from family, friends, and school officials. Children who had the benefit of early intervention can grow up up to be confident adults.

QUILT OF LOVE

Visit Cameron's Quilt of Love ... here

CHD QUILT
Here is the link to view Cameron's square on the CHD Quilt .. (Quilt 32 Row 6 Square 4). Michael's square (Quilt # 49 Row 6 Column D) is not online .. but is made.

Blog Updates
The latest updates are at the top .. and the oldest are at the bottom.

Tuesday, August 1, 2006

Cameron's Updates to Aug 2006

Post Fontan Followup

Things are ticking along here ... Cam has recovered very well after all the goings on and we had his cardiologist visit in August ..

I said to his cardiologist Dr John Skinner at the cardiac clinic here that I thought he was hyper - and he laughed and said that parents usually say that 6 - 8 months after the Fontan not 4 !!! I said I wish someone had given me 30% extra oxygen to cope with the extra energy he has ... I thought he was "busy" before and never noticed that he ever tired - but I must say phew he is "busier" with no downtime at all .... (and eat - my goodness - he was a good eater before - but did graze some days - but holy smoke he eats constantly now ;-) ) His sats are more stable around 90 - 95% when asleep & up to 97% when awake ... and his INR is now getting a bit more stable if he doesn't get unwell. He is doing really really well with the blood prick tests & now when I ask him to come & do them he just takes it in his stride ...

He has been having many "anxiety" tantrums when he feels frightened and we have just started kindy so the boys shyness & new people & kids are freaking him out but we are working on this through encouragement & reassurance & on Monday he even sat at the afternoon tea table with the other kids & when playing soccer kept playing when the other kids came to play with them .. My therapist says it is just his natural way of dealing with all the trauma he dealt with during his time up in Starship.

We are to go back up to Starship Children's Hospital in Auckland again in March for a MRI to decide what to do about Cam's narrowed PA, collaterals & now his aortic valve (as it is getting a bit too "leaky") as they now seem to want to MRI's before catheters so they can plan any "work" that needs doing so we will have a discussion post MRI to decide what the next steps are and go from there ... His right ventricle is beginning to show a very minor amount of strain - but at this stage they are not concerned.

We are also maybe looking at having a 24hr halter monitor (if we can get one here) as I checked Cam's sats a couple of times in the last couple of months when he was asleep at night (as he was breathing so very very slowly) and his heart rate was very low (40's) ... and it was mentioned a few times when we were at Starship, but they wonder if he goes in to such a deep sleep that his heart rate slows down too .. anyway to be on the safe side Dr John just wants it checked out ...

When we in the midst of the "Fontan" and all the complications I thought to myself that I wish he'd never had the silly surgery as everything seemed to be going so wrong & I just wanted him back the way he was & so on & so on - but now 5 !!!! months later almost it is just amazing to see how much energy this little boy has got - as I said before I thought he had so much energy before .. but now its like he is hyper because he has adjusted to having so much more oxygen in his blood ... looking back at the photos of him pre-Fontan I never noticed the change in his colour but it is noticeable now - he was getting a bit pale & his tongue was quite blue & when he was cold or hot he was quite purple on his cheeks & lips - but these were so subtle I didn't even notice them, AND the blood shot eyes have gone !!!

We now walk to the mail box & down to the calf sheds each morning - a good 30 mins there & back & not once does he stop or struggle to gain his breathe -- in fact likes to run half of the time - one day I asked him if he was OK as he was slowing down & he said to me " I don't feel very well" I asked him what the matter was and he said to me "My tummy hurts" - when I asked further questions it was because he was HUNGRY !!! - even after a huge breakfast/morning tea & snacks on the walk he was STILL hungry & this is what happens most of the day - he'll come up & say "Can I have something to eat I am sooooo hungry" - you know 3 & 1/4 years ago for 10 months we struggled to get him to drink or put anything in his mouth & now I CAN'T stop him - what a great feeling !!!

Cameron’s Fontan – April/May 2006

We arrived at Ward 23B at Starship Hospital in Auckland on Monday (10th April) very nervous – we had stayed at Chris’s Aunty’s house overnight & driven up in the morning in time for admission at 8.30am. During the day Cam had the usual pre-surgical tests – Blood tests, ECG, Echocardiogram and x-rays. We also completed all the necessary forms with the anaesthetist & nurses, then that afternoon we saw Dr Finuacane in the hall way & she apologised but said she had an urgent case she would need to do and because Cam’s surgery was second on the list that he would be ‘bumped” until Wednesday afternoon after the conference they held each week.

On the Tuesday because of the delay, we had completed all but one of the necessary forms so we took the boys to Kelly Tarlton’s Aquarium – which the boys now call the “fishy zoo”, to help allay our anxieties but also to give them something to keep their minds off the hospital. That night we went back to the hospital and had a meeting with Marion and Dr Finuacane to sign forms and the surgery explained to us and to have any questions that we had answered.

On Wednesday morning (12th April) we came back on to the Ward at 8.30am, and got Cam ready. Both boys had a pre-surgical antiseptic bath & both of them played with the dolls as the play-specialist went through what would happen. Even though Cam was not able to eat or drink he did very well as he spent most of the time in the playroom.

At a little before 12pm, Cam was given a pre-med which after a while made him drowsy so he gave his brother and sister lots of hugs and kisses & we took lots of photos with them together. He got vary attached to the doll that we had got ready for surgery eith the play specialist and the doll actually went to surgery with him. Just after 12 we took him down to the recovery room & soon after this after a lot of hugs, kisses & tears (from me) we handed him over to the nurses and he was wheeled away. He was quite blissfully unaware of what was going to happen.

just before 5 we were phoned to say he was off by-pass and would be going to recovery in just over an hour and that we would be able to see him in ICU about an hour after this. The surgery went well & his surgeon told us it was a straightforward Extra-Cardiac Fontan (with fenestration) - as these are the only type that is completed in NZ. He was off bypass in 3 hours (2 hours shorter than we expected) & we got to see him a couple of hours after that. Michael came with us to see him and was quiet for a while and then asked a few questions - but it didn't seem to overly concern him as we had sat down with them both and talked about Cam having his heart fixed and then with Michael about how Cam would be feeling after the surgery & how he would have tubes etc ...We were quite surprised how accepting they both were about it, although Cam was a bit concerned about it going to hurt when they opened his chest - but Michael reassuringly told him matter of factly "It doesn't hurt - because the doctors will give you some medicine"


A hug goodbye to his brother

Post Op (still intubated)

Michael Sees his brother for the first time Post-OP

Cam was out of ICU in 24 hours & then out of HDU in 2 days & then on the ward doing really well ... with his chest drains out after only 4 days. He had his pacing wires out on Thursday after surgery & coped really well.

Then things all turned topsy turvey on the Monday when he wasn't breathing well & the x-ray showed re-accumulation of fluid on the right side & so they put a mini chest drain in on the 18th (Tues). When they put a new one in they drained 300 mls !!!! and it had a gross milky fluid - Chylothorax !!!!. It wasn't confirmed until Wednesday but he was put on the chylo - non fat diet straight away and he still is on it for another 21 days - as he is on it for 28 days after the last lot of drains came out ...

He is doing quite well and hasn't asked for biscuits, cakes or cheese which he usually likes - but we have taken the temptation away by not having it visible & when we went shopping for groceries he told Michael - when he wanted to get some biscuits "I can't have those because I will get sick and have to go back to the hospital". We are all on the non-fat diet basically to make it easier - although Michael still his own formula & Cam has his "special" milk as he calls it (Super, super trim milk) & Chris & I have butter on our bread etc as I have been told I can't go completely fat free because I am feeding Abbie but it is an interesting exercise thinking of meals that are interesting with limited ingredients - thank goodness for pasta & spaghetti & baked beans!!! Also he loves the white part of boiled eggs which is great for the protein - so phew!!

He developed a pneumothorax on the 20th (Thursday) as the chest drain had slipped slightly & with each breath he was letting air into his chest cavity so all of the pressure caused his lung to collapse. I was shopping for the boys 3rd birthday so luckily didn't see all the drama, but Chris said it was pretty scary and he has never seen so many nurses & doctors converge so quickly when the panic set in & the x-rays showed what was going on. The drain was resited down in PICU and he recovered quite well. He took a while to get completely off oxygen & for at least a couple of weeks the sats would drop a bit so he would stay on 1/4 - 1/2 a litre. Then he was slowly trialled off it and the sats only dropped slightly so it was goodbye to the nasal prongs!

Going “around the block”

Cam got known for his "going around the block" as he borrowed a 3-wheeler bike from the playroom & we would hook his drain up & he would go around the block (out the fire door then around to the entry door) about 20 times a day (no kidding) He walked on and off but was quite sore by the drain site so was a bit grumpy about that. But once the drain was out he was back to his old self & running around the block.


Walking with his drains & oxygen
 The smile that was missing for quite some time
 The drain that was re-sited due to Pneumothorax

On the 23rd (Sunday) the drain seemed to be blocked so they took it out - the day before his birthday - so he had no drain for his birthday YAY! But the x-rays showed accumulation of the fluid so he was nil by mouth on the Monday from 3.30 - 5.30pm while they decided should they or shouldn't they put a new one in. Luckily by about 5.00pm they had told us it didn't look like he was going to have the drain put back in so we didn't have to wait too long and he had had some "birthday fun" most of the morning & afternoon. They did another x-ray the next morning (Tuesday) and found the fluid had worsened so his surgeon put a new drain in slightly lower & also took out 320 ml. The next couple of days the fluid just kept on coming out & we were warned on the Friday that if it continued to flow so much fluid (ie 150 - 250ml a day) that they would have 2 options - 1 to do a cath & coil his collaterals & then if that didn't work for him to go on TPN - great fun for a 3 year old to go nil by mouth.

Cameron, Emma and Michael watching the Wiggles DVD

The Care Package from home sent to us at RMH the first day Cam was allowed on day leave

Cam enjoying the sun at RMH.

Well someone was thinking of us as on Friday 180 ml dropped out in 1/4hr & then another 40ml (220ml for the day) the next day it was 120ml, then Sun was 40ml. On Monday they kept thinking that the drain might be blocked, so the said they would wait for a day & then do an x-ray - well it showed only minimal fluid, so they waited another day & then did another x-ray & lo and behold on Thursday the drain came out.

On Friday after a lot of waiting for the doctors (they were in a conference discussing other cases) - he was able to go back to RMH with me on weekend leave and we only went back in the morning for doctors rounds, obs & meds for the day & then we would go back at night for obs & the other meds, and the rest of the time was ours ... so Cam, Abbie and I went to the park and fed the ducks each day & would play at RMH- nice NORMAL things !

Feeding the geese at Western Springs (first time out on day leave)

Feeding the geese at the Domain

Playing just like any ordinary kid at RMH

Chris was going home for the weekend & had asked what the plan for Cam was and they had said - sorry he won't be able to go home with you on Friday - & he told them he didn't expect to then in the next breath they said to him but Monday was looking hopeful! We couldn't believe it ...

The safari animals at Auckland Zoo

So Monday came around and we did bloods & an x-ray & went to the Zoo for an outing and then got a phone call in the afternoon to say that they didn't need us to come back in that we only had to pick up the script for his meds & that the discharge letter would be ready for us to pick up! YAY -what a feeling!

The last night staying at RMH

We read the letter, then the next morning did our first "solo" INR test on Cam & then I sat down with the cardiology consultant with a few questions that weren't covered in the discharge letter - ie follow up required by our local paed & further work to be done (a cath to coil collaterals & to dilate the LPA) & as a result the discharge letter was changed - which was good in that they had taken on board what I had asked & told them.

He is doing well at home and currently has to restrict his activities - as in no climbing trees & wrestling for another couple of weeks (easier said than done with Michael and him at that stage where it is there favourite thing to do!!!) but otherwise life is back to normal albeit doing us doing an INR test every 2nd morning for the Warfarin levels and remembering to give his meds every 6 hours - phew!! His INR is pretty stable between 2.6 - 2.9 currently so that is one thing off our minds - but will be different I suppose when he is back eating "normal" food again.

His sats sit between 93 - 94 & they want them to stay above 90%. I have noticed a complete change with his eating as in eating more as it seems his sats were lower than they would have liked (68 - 73) and this was obviously making Cam feel terrible - and we thought it was just him being a 3 yr old putting his foot down!

Cams view of the heli-port from the window in his room.

It was quite interesting as we had 6 HLHSer in the ward during our time there - mainly because we have been told that there were not that many in NZ when Cam was born about 30 - 40 - but now the numbers have increased dramatically - a strange phenomenon that the consultants can't explain. Three of the Fontan kids got Chylothorax - 1 before us & 1 after us - so it was nice not to be the only one there with it - although they both went home before us - but were older kids too. It seems Cam's was brought forwards as he has AV malformations that we hadn't been advised about - which didn't make us very happy - which were causing chaos (& low sats) but we were told that these for some reason correct themselves after the Fontan.

So that in a novel was our great Fontan experience - Cam is still quite frightened and is still having the dressing changed on his last chest drain site as it got quite YUCK & had an embedded stitch - just to make life interesting - but phew, we are home that is the main thing. We are staying home for the next 3 - 4 weeks just to avoid the winter bugs around as I have a phobia about him getting sick so quickly after surgery & they have said with the lung injury from the collapse they would like him to avoid getting a chest infection for a while - although Michael seems to have caught a nasty cold with the change of climate from there to here.

Michael and Cameron’s 3rd Birthday - April 2006

The boys spent their 3rd birthday in Auckland and in Cameron’s case at Starship Hospital. Michael woke up all excited at RMH and he was allowed to open a couple of presents & I texted a big HAPPY BIRTHDAY to Cameron. After breakfast Michael, Abbie & I, went to see Cam.

Nana Rose, Aunty Sonje and Jesse had brought Michael back up to Auckland the day before and had brought presents from home and a secret BIRTHDAY CAKE made with love from Aunty Lish. The nurses on the Ward had painted a HAPPY BIRTHDAY sign and made cards, and had hungs balloons. After the Doctors rounds we were basically left to ourselves for the boys to enjoy the day until lunch-time. We had air popped popcorn, lollies & more lollies. Nana also brought balloons and bubbles to blow!

The boys got DVD’s from us (to watch & pass the time), tractor sets, and the BIG present a Thomas the tank Engine Set. They also got lots of wonderful presents from all of our families and were definitely made to feel special. They also received gifts from all of the staff at Ward 23B, along with cards, and so had so many presents they really didn’t know what to do with them all.

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