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.

Sunday, October 21, 2007

Just when you think you can relax .....


Got Personality - Got lots !!!!!!!!!


My favourite photos of Cam.

OK anyone who wants an extra lesson on how a heart works .. read on .. lol .. OK so maybe not that bad ..

Cam went to the visiting Paediatric Cardiologist on Friday, and his echo-cardiagram, and x-rays look good.. very impressive in fact he said .... and was very pleased overall with Cam's progress ..then he said I'll just look at his latest ECG ..

Well unfortunately there were signs of long QT episodes (What ?!?!) .... and so Cam has to avoid medications that can lengthen the QT interval .. and he is to discuss it with the team back at Starship ... he doesn't feel that he has Long QT syndrome ( a potentially fatal condition where they can just faint or lose consciousness, without waking up again!), but said he would discuss it further ... it seems between having a Holter Monitor a while back (to see why his heart rate was so low & to make sure the rhythm was normal ...) and the ECG, something has changed. Also asked if he had had any episodes of just suddenly dropping to the floor (he did but he said it sounded viral related) .. or suddenly going to sleep etc ...

He went back over our extended and immediate family cardiac histories YET again ... looking for answers ... especially asking about any family suddenly dying from heart related problems (none that I knew of, only old age) .. also talked more about the valve issues and the heritable study of HLHS .. especially with all the kids having valve issues, and asked about whether Chris and I had had echos etc ... which we haven't ....

(This was because many of the issues that Cam has seem to now be "heritable" - lots of new overseas research papers out it seems - but we can't find anyone in the past in our families who have had anything like this) ....

So anyway .. he left it that Cam's monitoring may be increased .. and that they are sending a letter to his GP, Paediatrician, and us (which we can also give to the pharmacy we use) .. outlining the precautions etc ..

He is still not concerned about the low heart rate .. but mixed with the long QT episode, highlights that something (electrical) might have been affected in his heart, and that it was not entirely out of the box for children who have had the Fontan for this to happen... then mentioned in passing that to correct it the kids usually having a Pacemaker or internal defribullater, but not to overly worry about it at this stage - YEAH RIGHT!

He said that if he had seen the Long QT episode in a "normal" functioning heart that he would have been very concerned but that with Cam having had the Fontan and his "heart's history" that this was not entirely outside what Cam's heart could be doing.

A great thing though was he talked positively about doing exercise /treadmill stress tests at the age of 6 & 10, and how following the Fontan there was really not too much more intervention, unless things like valves or arteries started causing issues, and these would be monitored.

He seemed quite upbeat about Cam's overall cardiac condition .. (especially talking about the fantastic surprise of not needing further treatment during the cath!) ...

So we were told not to worry- but of course you do!. In fact the last couple of days was obsessing a bit - lol.. (Must say tho' the house got a good spruce over as when I freak out I clean - weird I know) . It was another lets wait and see appointment - with the added spice of don't worry. Which we should be used to by now - but of course you don't - lol.

So I was like - what on earth is a QT interval etc ....so this is from the site I was given by the cardiologist ...

Heart Arrhythmias & Long QT Syndrome

There are many types of heart rhythm abnormalities, also known as arrhythmias or dysrhythmias. If the rhythm is abnormally fast, it is called a tachycardia; abnormally slow rhythms are called bradycardias. Some rhythm abnormalities have a normal average heart rate (also called pulse rate), but are abnormal in that the rhythm is irregular or because some or all beats start in parts of the heart other than the sinus node, the heart's normal pacemaker.

What is the QT interval?
It is a measurement, made from the electrocardiogram (ECG or EKG). It reflects the duration of the electrical activity that controls contraction of the cells of the heart muscle.

and another explanation : Every heartbeat is triggered by an electrical signal that tells the heart’s muscle cells to contract. After contracting, these cells just recover – or relax – before the next heartbeat is initiated. The amount of time needed by these cells to recover can be measured on an ECG and this is called the QT interval. During the last part of this interval, the heart is vulnerable and electrically unstable.

Long QT Syndrome
Long QT syndrome refers to a condition in which there is an abnormally long QT interval on the electrocardiogram. It can be inherited ("congenital long QT") or induced by drugs or abnormal levels of the salts normally found in the blood, such as potassium and magnesium ("acquired long QT"). The inherited form occurs due to abnormalities in certain proteins in the heart cells, and these protein abnormalities are in turn caused by abnormalities in the genes that produce those proteins. Some people, despite having a normal QT interval under normal conditions, may develop a prolonged QT and associated rhythm abnormalities when taking certain medications; this tendency may also be inherited, and is the subject of the research effort.

The scary bit I'm trying to ignore : People with LQTS have an abnormally long QT interval. If the next electrical signal arrives before the muscle cells have completed their recovery period a dangerously fast heart rate can occur leading to a fall in blood pressure and loss of consciousness.

and also a link to a paper that is from Starship re Long QT Syndrome ..Long QT study in NZ

Clear as mud to me .. and soooo not trying to read into it until I've heard back from them...

So trying to think on the positive side that they are being pro-active .. rather than just "oh well .. just wait and see" ...

These kids like to keep you on your toes - lol ..
"No slacking Mum and Dad - just when you think you can relax .."

Now getting these school enrolment forms filled out .. is another story altogether .. did I say SCHOOL !!!!! .. who would have thought I'd be saying that 4 & 1/2 years ago!

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