Sarah McKain growing up with Truncus Arteriosus

Sarah agreed to do an interview with us for April 2011 E-newsletter. What Sarah wrote is inspiring, full of confidence and is a great perspective from a teenager.

How would you introduce yourself? What CHD do you have? How old are you?
I’d introduce myself as Sarah Mckain because although I have a CHD I am still me. I am 17 years old and I was born with a rare heart condition that affects only one in 10,000 babies called Truncus arteriosus, there are three types, sometimes four of this condition and I was born with type two of it, I also have a large Ventricle Septum Defect, also known as a VSD which is a more common heart condition, and pretty much saved my life when I was born because without it there would have been too much pressure in the ventricles (bottom part) in my heart and in turn would have had a bigger impact on my lungs.

I’m from Cumbria in the UK and live with my mum, dad, and younger brother and sister, who all have healthy hearts.

How and why did you survive to become a GUCH/Adult CHD’er?
How did I become an adult CHD’er? It’s all thanks to the amazing team at the Freeman Hospital in Newcastle because without them I wouldn’t have survived and would have indefinitely died within in the first year of my life.

My mum had a normal pregnancy with me and everything was fine with the labour, I was the first child of my mum and dad so, they thought me not taking my milk was normal, but at my three day check the paediatrician noticed I had a murmur he told my parents he wanted me to come back in two weeks because the murmur should just go on its own but he wanted to make sure. Two weeks later the murmur was still there and the paediatrician asked my parents if I could be seen by a children’s cardiologist from the Freeman who was doing re-search into heart murmurs, which they both agreed to.

When I was 5 weeks old my parents took me to see this children’s cardiologist from the Freeman. My mum took me into the room and laid me on the bed, the doctor listened into my heart and said to my mum that I didn’t “just” have a murmur, in fact I had a congenital heart defect known as Truncus arteriosus, and large VSD (all that from just listening to me heart!!) I then went on to have an echo, which confirmed his diagnosis, he then told my mum that I would in fact need open heart surgery to get my heart to a good working condition. The week later I was taken over to the Freemans to have lots of tests done to see how severe my heart condition was etc.

During all of this going on I kept going blue, and stopped breathing, to solve this I needed “shook” back to life to try and get me to take a breath, this without a doubt scared my parents especially the first time it happened! Then because it was becoming more and more frequent I was admitted to the Freeman hospital at 7 weeks old until I was big enough to have my first open heart surgery, because at the time I was too small which would increase the risks even more.

At 13 weeks old I had my first lot of open heart surgery which was basically a re-plumb and patch up operation which lasted 6 hours. This operation required me to go on the heart lung bypass machine for a while during the time I was on the bypass machine they patched up the large VSD which separated the bottom part of my heart and meant the two different “types” of blood wouldn’t mix anymore; they also separated my single artery by inserting a conduit/homograft pipe which would give me “normal” aortic and pulmonary blood vessels, so the pulmonary vessels could deliver blood to my lungs, and the aortic vessels to the rest of my body rather than the pressure being high and my lungs getting too much blood the rest of my organs not enough, finally they also repaired/ corrected my truncal valve. I was in hospital until I was 15 weeks old, then had regular check up’s once a year at least.

I had a catheter at the age of three years to check everything was doing well which is was and I continued doing well up until I reached the age of 7-8 when I started getting very breathless, becoming more tired, sleeping lots, and getting chest pains, I then had my annual cardiac review and was informed I would need open heart surgery number two because my conduit pipe was too narrow and blood wasn’t flowing through it properly. Several months later I went back into the Freeman for my op, I went in on a Sunday night, had the op on Monday which lasted 7 hours, was in PICU (children’s intensive care unit) overnight and by Tuesday tea time I was back on the ward :D and by Wednesday morning when physio was looking for me I was in the playroom! I was then discharged on the Saturday morning and went home, so I had open heart surgery and was in and out within a week, not many people can say that!

I continued having my regular check-ups’ and I was doing very well, until I reached the age of 12 when again I started not to feel well, was becoming breathless etc, I had my appointment at Newcastle when my cardiologist said they wanted an MRI scan done of my heart which took place several weeks later, I then had another appointment with him and they said they would try and put stents in my heart via a catheterisation because my aortic valve was narrowing hence all the symptoms.

I went in for the stents and was taken to the catheter labs, once I came round I discovered that they hadn’t put them in because the narrowing was too severe and it wouldn’t do any good and the only other option was open heart surgery number three, this was in Jan 2007 just after my 13th birthday, I got a date for a few weeks down only for it to be cancelled due to there being no PICU beds available for me, I then went on to get another six dates for my surgery but they too where all cancelled either due to no PICU beds, or no beds on the ward, then I got my 8th date for May 2007, I was admitted again on a Sunday night and was first on the list for Monday morning, I was told I would have theatre all day because they operation was a big one!

I woke up in PICU 2 days later and was out of it shall we say until I was moved down to the ward on the Thursday tea time. I had found out the two amazing surgeons had tried 5 different times to repair my aortic valve but in their words “had withered away so much we couldn’t repair it” so I had an aortic mechanical valve in my heart and best thing is I now tick! :D literally

After the op I kept having temperatures and couldn’t get a stable INR (blood thinning level to make sure my blood wasn’t too thick for the valve) so I was in hospital for a further three weeks and was then discharged.

Then heart wise I was doing fine, shame the same couldn’t be said for the haematology (blood) side of it, I continued having problems getting a correct level on the warfarin (which is the blood thinning medication I was on) I had several nosebleeds which resulted in blood coming out of my eyes o.O and me needing my medication changed to phenidione which is another blood thinning medication, but that didn’t work either and again I couldn’t get a level, so I had another go on the warfarin, but again didn’t work. My haematologist then started me on inohep tinzaparin a type of heparin which so far is working well for me :)

And that is “how and why did you survive to become a GUCH/Adult CHD’er?”

UPDATE December 2011 (since writing this I have been pretty ill….here’s an explanation of what’s happened)
I continued to have problems with my anti-coagulation, even with the heparin, then on the 6th March 2011, I was at school and developed severe pain in my right leg when I moved it, my toes where numb/tingling, the sensation just wasn’t right, I came home and took my shoe off which revealed a very very very white foot! My community nurse came to see me (was coming to put my insuflon in) she couldn’t find a pulse, my leg and foot where ice cold. She rang my CLN who instructed me to get over to A&E right away! I did as I was told and I was in agony every time I moved. We went to casualty and rather than sitting I was taken straight through to been seen (even bypassed triage ;) ) and next minute I know, the vascular surgeon was called, he came down to check all the pulses in my groin, leg and foot and compare them with the left side, fortunately they did find something but it was VERY weak, I was attached to a heparin infusion, and sent off for an emergency CT scan, the results = blockage of my arteries behind me knee, I was told I needed “more tests” but the on-call doctor was in another hospital 2.5 hours away! He decided he’d meet me half way, so I was packed off in the back of an ambulance with my Mam to a hospital an hour away ( I got the full 5* treatment blue lights and sirens!). I arrived at the hospital only to be met by an anaesthetist, surgeon and a full surgical team!!!!! The surgeon came and explained that I’d be having emergency surgery that night (this was 1am) I needed an emergency embolectomy, couldn’t it wait till morning? NO! Another 10 minutes and I’d have lost my leg!!!!! In fact the surgeon said he’d thought he’d be performing an amputation that night as my foot had gone nearly 18 hours without a proper blood supply to it! So off I was wheeled into theatre, not knowing how I’d end up at the other end…….

I don’t really remember much the following day or coming round from the op, but I was told I had been very lucky!! The surgeon came to see me and my Mam, and by this time my dad had arrived (he was with my younger brother and sister) he said to me as I asked what’s the chances are of it happening again? and I was told “this is a one off occasion and the chance of it happening again where less than 1% and I shouldn’t be worried” then the physio came and had me up (with staple in my wound OUCHH!!!) walking around, I know it was best for me but heck it hurt!! After a week of recovering I was allowed home, and had an emergency appointment with my blood and heart docs.

22nd March 2011: I made the trip to Newcastle where it was decided I’d have a T.O.E done just to double check my heart was okay, but on 23rd March I was taken back into my local with another suspected blood clot in my right leg, but this time there was a pulse, after an overnight stay I was discharged back home :) but the specialists in Newcastle weren’t happy so wanted me over so that their vascular team could get involved, and while I was there I’d have my TOE. The vascular docs there were also happy with my leg but not my incision wound that was pretty infected!!! I had the TOE and was told my heart was clear, after 5 days in there I was again discharged home!

But it only lasted 6 ish weeks!!!!

25th April 2011: “De Ja Vu” comes time mind, it was take 2 but this time my left leg not right!!!!!! Yup I had yet another blockage in my artery behind my knee, and again another few minutes and I’d have lost my leg :( so this time I was taken back to theatre but in my local hospital for emergency embolectomy number 2!! I was admitted to a ward for a whole 10 minutes!! Got to see my Dad, brother and sister before I went down and off I went to theatre again, with my Mam there (I may be 17 but I am not going theatre on my own lol ) This was 6pm, I was put to sleep and then remember coming round in the recovery room, I asked the time 11pm, I’d been in surgery 5 hours!! I had to spend a further 2 hours in recovery then was taken to the HDU (High Dependancy Unit) part of the surgical ward and allowed to see my family again (who like me where in shocked it had happened again! So much for “chance of it happening again where less than 1% and I shouldn’t be worried”) They weren’t allowed to stay with me as it was an adult ward and beside we only live opposite the hospital (good job!) so could be there within minutes if I needed them. That night I was on 30 minute obs, hooked up to monitors, a heparin drip (which is ultra sensitive) I also had blood taken every 6 hours because of the heparin drip, and they needed to watch my clotting levels and in lots of pain, so never slept very much at all. My surgeon came to see me and told me I had once again been a very lucky young lady and was clotting far too much, he also said that my blood tests indicated clots were still active in my body, but that could have been because of the op I had just undergone, or because I had clots in my chest somewhere….The following day I was moved round to the 8 bedder and slept most of the day. That night I was very ill. I had started coughing up blood, had pains in my chest, my legs where sore, I was very breathless and not good at all. I had a nurse with me pretty much all night and when she wasn’t there a doctor was. I had arterial blood gases taken as well as more blood tests, and I was told my bloods where bad, and my arterial ones where too low. I was put on 8 litres of oxygen and even then my SAT’s weren’t brilliant, the pain team where on the ward looking after me because it was at the point morphine wasn’t helping.

The next day the cardiac team at Newcastle where called and it was decided I needed to get over there right away!! I was given 15 minutes notice to try and get sorted. In fact they wanted the air ambulance to take me over but it was tied up doing something else (pleased to be honest as I’m not keen on heights lol). My Mam arrived just as I was getting on the ambulance trolley. The escort nurse arrived (yeah I needed an escort!?!) and my two favourite ambulance men!! (They are brilliant have taken me three times to Newcastle and twice to Carlisle! Always have a smile and make me laugh-Thanks Donald and Keith!! :D ) Anyway, one and half to two hours later (we travelled fast!!! it normally takes 3 hours to get there!!) I arrived in Newcastle and was admitted to ward 25, awaiting a plan…3pm my surgeon came to see me, telling me “the only way we are going to solve this is if we do open heart surgery and change your aortic valve from a mechanical one to a tissue one, that way you won’t need the anti-coagulation medication” “when” I asked “Friday!” This was Wednesday, was he really telling me I needed OHS (open heart surgery) in 2 days time?!?! Yes he was! I was to be kept on the heparin drip until after the op as it was decided that my aortic valve was throwing off clots. My consultant also said if the clots that had gone onto my brain I wouldn’t have survived as there was too many of them and they were pretty big!! Due to the heparin drip I had 3 big nose bleeds!! So they took some stopping! Thursday night I saw the surgeon and my consultant; again they informed me at that time there were no ICU (Intensive Care Unit) beds but they were hopeful someone would be moved off Friday morning. I also saw the vascular team over there as I had developed “compartment syndrome” so I needed to be watched so that it didn’t get too serious otherwise I’d have needed a Fasciotomy (no thank you!)

Friday morning (in fact it was the day of the royal wedding!! 29th April 2011 A date some will remember forever, I will too but not for the same reasons!) came I was informed the op would go ahead, I’d be in theatre all day, on bypass machine, defiantly having my aortic valve changed but there was a possibility of some of my other too, I really wanted to see Kate’s dress, just as she was about to come out, who knocks on the door but a porter to take me to theatre!!! So I’d have to wait for a few days to see it. I was wheeled down to theatre, (not even a week ago I was having an embolectomy, now I was having OHS) I even managed to have a laugh at my Mam and Dad in scrubs!!! I was wheeled into theatre and they tried and failed to get my artery exposed for the blood pressure machine which hurt! So rather than be awake while they tried again I asked if they could wait till I was asleep! Which they did, they put the Freezing I mean cooling packs on my back to start and cool me down for the bypass machine and started with the aesthetic.

Next thing I know it’s 1am the following morning and I was in ICU. I had chest drains in that where very painful, a tube stuck down my throat, a line coming out of my neck and attached to every monitor and drip going!!! I was sick twice even whilst on the vent, but drifted in and out of consciousness while the ICU nurses where sorting me out. That night I can’t remember a thing (apart from the sick!) and begging for a sponge to suck on to get the water out!!! The following dinner time I had my drains pulled out cause they killed! (Although the surgeon said it was rare as he would have normally kept the drains in 3 days) and moved down to the ward, or was it HDU? Can’t remember.

My consultant came to see me once I was back on this planet and told me I had my aortic valve changed but no others because I was too ill and my heart was to weak, but I’d need my pulmonary valve done in about 5 years.

The next few days are a blur in more ways than one! Literally I couldn’t see properly, my right arm was twitching like mad, my left was numb, my legs where numb, my lips where numb and my balance was rubbish. Because my symptoms varied my consultant and surgeons didn’t think it was anything to worry about, but after 3 days they sent me for a CT scan, which showed “abnormalities” but they didn’t know what, so I was sent for a more detailed scan, a MRI scan (hate them things!) but I had it done and my doctor came and told me I’d had a stroke.

Heart wise I was doing as okay as I possibly could. I had regular physio to try and help with the stroke but it was decided it would be best if I was transferred over to ward 41 at the RVI in Newcastle, a specialist stroke unit where I’d get rehabilitation. I was transferred over to the RVI and got settled onto the new ward. My new consultant came to see me and explained that I hadn’t had one big stroke instead I had several smaller ones caused by a scattering/shower of clots all over my brain; that’s why it had been so difficult to pick up, as I didn’t follow the normal trend, do I ever? So I needed to be on anti-coagulants! So I wasn’t getting rid of them after all, but at least I don’t have the mechanical valve in!

After 10 days of rehabilitation in the RVI, the docs where happy for me to go home with regular physio and rehabilitation at my local hospital. But I was having a few problems with my scars; both my OHS one and my leg where they went in with the bypass machine, so before I was discharged I went to clinic for a heart check up in Freeman, also to get my stitches out of my leg where they went in with the bypass machine during the op (which I was told they had to clean up as it was seriously infected before they could use the artery!) One look at the wounds and I was informed I wasn’t going anywhere but back to the RVI with the possibility of going back to theatre to have them re-opened and cleaned out! My consultant was happy with my heart so got in touch with the plastic surgeons in the RVI to look at me, so back to ward 41 I go. I had bloods taken, the normal range for them was between 5 and 20, mine where 35,000!!!!! So I defiantly was there for a bit longer! I was started on strong oral antibiotics and reviewed by the plastic surgeons who didn’t think I needed to go back to theatre but wanted them swabbed, cleaned and packed each day. The next day I had more bloods taken to see what my level was, hoping to see a decrease in level, but me being me, it went up! So they started me on IV anti-biotics, next challenge, try and get a canular me-good luck to them! About 1 hour, 12 tries, 4 docs and a consultant later I had one in and was attached to the drip.

I remained in hospital for a further 4 days had more rehabilitation, and IV antibiotics and my infection level had started to come down so I was allowed home! For real!!

HA!!! It’s me! I got home for 1 night, and 4am the next morning I was admitted to CCU with 3 blood clots on my lungs! So back on the heparin drip, and oxygen.While I was in they noticed my haemoglobin level was low 8.1, if it dropped below 8 I’d need a blood transfusion, so I thought why not ;) . I dropped down to 7.6 and ended up needing 3 units of blood. I was also checked for internal bleeding because that was pretty low even though I’d had 3 ops! And it was found I did have a little bit but was easily treated, Thank goodness! After 5 days I was allowed home again, this time I stayed a bit longer ;) .

I have since been re-admitted three times with suspected blood clots again in my right leg, and needed IV heparin again; but fortunately I haven’t needed any more ops, but I did need an angioplasty in my right leg as they wanted to check the flow of blood to my leg and foot, the results of this showed a sluggish blood flow to my leg and very sluggish blood flow to my foot, the vascular docs thought this is cause by my heart. I was sent to Freeman’s with the results and he said he didn’t think it was my heart because if it was my heart it would affect all four limbs not just one! After another load of tests I’ve been diagnosed with “intermittent vasospasms” but I can’t have the treatment for it because of my heart. But the symptoms of them and clots are the same so I’ve to be checked out “just in case it’s another clot”. I’ve also been in with clots on my lungs again and breathlessness, I’ve been told they are the same clots as last time that have calcified on my lungs, so I’ve got chronic pulmonary embolisms too!

Now?? I’m actually waiting to go in for heart op number 6 which will be percutaneous (through my leg!) Although it won’t be open heart surgery as I’m too high risk and chances are I wouldn’t survive it, it is still very risky and there’s a high chance something could go “severely wrong”. I am having my pulmonary valve replaced, and my conduit pipe stretched and stented, (so never got to wait 5 years after all! Instead it’s only been about 7 months) but when stretching the pipe it could burst and I could end up needing emergency open heart!! I’ve had an emergency MRI, lung function tests, arterial blood gases and other stuff done, and I’m pretty much just waiting for a date any time now!

I still can’t see properly, my balance and concentration is rubbish still, my legs are still numb, my fingers are still numb. I use crutches to help me walk short distances but need a wheel chair if it’s long distances. My A levels are on hold now. But will this stop me? NO!!!!

I’m determined not to let any of this beat me, I’m still here and surviving to be a GUCH/Adult CHD’er!

I want to add my thanks to every single doctor, consultant, nurse, and surgeon that has helped me through this from everyone in the Freemans, Carlisle and West Cumberland! A massive thank you also to my Mam, dad, brother and Sister, also to my friends, one who came to see me nearly every other day while I was in Newcastle! Thanks Bea!!!! And to everyone who has sent me messages, cards, presents they are all very much appreciated.

Was it tough growing up with CHD?
Easy answer to this would be no, because overall no it hasn’t been tough having a CHD, there has been lots of times in my life where yes it has been tough; the heart surgery, missing school, not being able to keep up with friends in the yard at school, being ill. But I have just finished my GCSE’s I got 13/13 GCSE’s all at A*-C grade. I am now doing my A-levels in double health and social care, biology, psychology and general studies and I am hoping to go on to university and then finally become a children’s intensive care nurse and give back the care I got.

Has CHD held you back? How were you treated by your peers in relation to your CHD?
Having a CHD hasn’t held me back, I do most things my friends do, yes there is some things I can’t do/ been given medical advice not to do but that is mainly because of the haematology (blood) side of things, I can’t have piercings (apart from my ears) and I can’t have tattoo’s. I am only allowed to drink small amounts of alcohol but general day-to-day things, no my CHD doesn’t hold me back. In school I wasn’t treated any different to my peers (well that I can think of) apart from in PE if I couldn’t do something I was allowed to sit out, but generally I wasn’t treated any different.

How does CHD influence your daily life now?
In daily life you wouldn’t actually know that I had a CHD apart from my scar and I have a small subcutaneous canular in my arm where I inject my medication everyday but apart from that and me being a little bit slower than everyone else you wouldn’t know I had a CHD. .

How did you cope with moving from childrens’ to adult hospital as in moving from children’s to being treated as an adult?
When I first found out I had to move from children’s’ to adults the thought petrified me because I would be getting a different cardiologist and a new team would be looking after me and after 16 years of having trust in my children’s’ team I would have to meet a brand new team.

I only recently moved to adults (24th March 2011) and honestly it was fine, my new cardiologist is lovely and my new cardiac liaison nurse is amazing, they are both approachable and after my first appointment and meeting them I feel much happier about being an adult with a CHD.

How did your parents cope?
For the answer to this one I asked my mum (Anne) and my dad (David):

“When we first found out Sarah had a CHD and needed her first heart operation we both went into auto-pilot and it didn’t actually hit us until it was over and she was back home. For her first operation we both had our parents there for us but for her second and third operations we didn’t as they had all unfortunately passed away, so we only really had each other, and then our brothers and sister for support. But as far as how did we cope? We never really had a choice and just go on with it, and all considering I think we both coped very well,”

What is your thought on the Safe and Sustainable?
I do agree with making bigger and better centres but I don’t think they should close the Freeman centre because apart from it being amazing, there is only them and GOSH that do children’s heart transplants and Freeman don’t look at statistics and decide if they will treat a child they will treat and try to give every child a chance. Also it doesn’t just treat children from the north east it treats children from the whole country and is the only centre “up north”. But I can see why they think making bigger centres will be better.

Wrote by Sarah McKain
9th April 2010
Updated: 19th December 2011

Sarah McKain has kindly given CHD-UK permission to use her photographs