Fifth Heart Surgery (2019)

This blog post is a condensed version of a separate blog that I wrote just for this procedure. Here's the link so that you can read the whole blog: https://newvalve.blogspot.com. There are only nine posts. Alternatively, you can read the summary below.

This was Harrison's first experience of surgery on an adult ward. He'd already had a couple of stays for cardiac catheters (in the same side room) so the environment was no longer unfamiliar to us but the the pre and post op process was. i.e. would we be able to accompany him to the anaesthetic room and go to him in the recovery / ICU area (as we did for paediatrics)? For the cardiac catheters he was brought back to his room and we weren't able to be with him downstairs. The staff had forgotten to collect us from Harrison's room to go down with them to retrieve him. We like to be with him as soon as he wakes, to ease his anxiety and confusion and to anticipate his probable reactions to the anaesthetic.

In theory a valve replacement via cardiac catheter is now a straightforward procedure. However, as we all know, Harrison has never read the text books and likes to do things his own way! I asked him to give his cardiologists (and us) an easy ride this time! I thought you might be interested to read an article on pulmonary valve replacement written by the Royal Brompton team in 2016, featuring Harrison's cardiologist (Dr Michael Rigby):

https://www.rbhh-specialistcare.co.uk/news/innovative-therapy-restore-pulmonary-valve-function

And here's a video on the procedure (Melody Transcatheter Pulmonary Valve Therapy Procedure for Patients):

Harrison signed his own consent form. It was an extremely comprehensive booklet to read through. He was second on the list. Dr Rigby emphasised that this isn't an easy procedure and could take up to 5 hours. He also said that he may not be able to do it at all for various reasons and that there was a 1 in 20 chance that it might culminate in some surgery. He squeezed in a straightforward case before Harrison so that he could allocate the rest of the day to him. 

So here's Harrison waiting for Dr Rigby to do his "warm up case" as he’s called it!

 

Harrison was taken straight into Cath lab 2 for his anaesthetic. He's usually put to sleep in a tiny room outside the Cath lab but this time was treated to the full works on the procedure table itself.

As always, the staff were brilliant and Harrison was our amazing, brave boy. They already had photos of his PA stent and valve stuck up on the cupboard doors. Either that or they hadn't got round to taking them down from October!

 

 

Dr Rigby came to see Harrison and to let us know that the procedure went like a dream! He was delighted that there were no complications and Harrison remained stable throughout. He surprised everyone by finishing an hour and a half early! Bernard and I got the call whilst we were driving around Chelsea looking for a new car parking space. Harrison spent about an hour and a half in recovery before returning to his room.

Whilst Harrison was in the cath lab Bernard and I wandered down the Kings Road in a zombie like fashion. Even though I was looking, I couldn't tell you what was being displayed in any of the shop windows. We had to remain slightly aware of our surroundings as we had to dodge numerous orange blobs from Centrepoint jumping out and dancing around in front of us. We were NOT in the mood to have a chat with them today!

We barely spoke to each other until we sat down with our lunch in the Peter Jones restaurant. Even then the conversation was interrupted with us checking our phones for any missed calls or messages from the hospital. 

After lunch, we ambled around the back streets of Sloane Square heading in the general direction of the hospital. I suddenly realised that we should have moved the car twenty minutes previously! Cue rapid walking back to the car searching for any vacant spaces en route and hoping that we beat the traffic warden! Thankfully we did but finding that space seemed to be more difficult than replacing a pulmonary valve today! Bernard dropped me off at the hospital so that I could be with Harrison whilst he continued the game of hunt the space.

Harrison had obviously been reading the medical textbooks closely because he was recovering very well ;-) He just had the usual equipment - ecg, blood pressure cuff and saturation monitor along with this contraption on the wound site (he wanted me to photograph it to show you):

 

As the time went on he became more chatty and fussy about all the lines and plasters. Despite everyone telling him to rest, Harrison said he couldn't because "the sleepyness has all gone" the anaesthetist had done such a good job reversing the anaesthetic! We've now got a problem keeping him lying flat for 4 hours. Nice problem to have though!

 

Compared to October this is a brilliant recovery, so fingers crossed it continues. I'm quite optimistic because his colour and numbers look very good. The plan for tomorrow is for a chest x-ray, echocardiogram and the blood tests for the immunologist.

We had two attempts at removing the pressure device. The wound site was fine as long as he didn't move. He was finally allowed to sit up a bit but unfortunately this caused him to spring a leak! 

Another hour laying flat coincided with the arrival of his Pizza Express Piccolo meal. 

The new valve is already having a dramatic effect. Prior to the procedure Harrison was quite lethargic all the time and eating was a laborious process. Now he is chatting excitedly and managed 6 dough balls and a child sized pizza without any problem. It was the first meal he'd had since the previous evening but was still impressive!

We settled down for an early night but I'd forgotten to give Harrison his watch back. Despite his melatonin medication, I found him wide awake and sitting up at 1:43am thinking that it was already morning. I, on the other hand did not feel so wide awake at this time! It was my second night on the camp bed and although I'd made attempts to soften the mattress with a thin quilt, it was still one night too many. It felt like I was lying on a slab of concrete!

We're extremely fortunate to have only one of two side rooms on the ward. The disadvantage though, is that we're quite cut off from the rest of the ward.

However, we know everyone is still out there when the banging of doors starts at 6:30am! We're sandwiched between a shared toilet and the entrance to the pre-assessment unit / offices.

 

I'd barely sat down in the chair outside the x-ray room and Harrison was out again. It wasn't the case in the echo department though. Bernard timed his entrance perfectly this morning. We were leaving Harrison's room to go to the echo department as he turned up with a coffee for me. We both followed Harrison and the porter over to the other part of the hospital to wait his turn. As it was so warm on Paul Wood ward and in x-ray, it didn't cross my mind that it might not be the same in other areas. Fortunately, Bernard hadn't had time to take his jumper off so he put it on Harrison. We'd been waiting in the chilly corridor for over 20 minutes and nobody had been called from the packed waiting room. Bernard to the rescue and 10 minutes later Harrison was settling himself on the bed next to the echo scanner. The doctor conducted a very thorough review of Harrison's heart taking lots of images, measurements and pressure readings.

On our way back to the ward we saw another paediatric consultant who joined the Brompton team as a Registrar at the time of Harrison's last open heart surgery in 2009. She stopped to chat with us to find out why we were there. She had observed part of Harrison's procedure yesterday, not realising that it was him on the table! She commented on how proud Dr Rigby was in completing the procedure so quickly.

The x-ray and the echo both looked as good as they could so Harrison could go home! The only concern the staff had was his continued low blood pressure. After a couple of repeat readings and specific questions to Harrison (not that he was going to own up to anything other than feeling fine at this stage!) they collectively decided that this was his normal and agreed to discharge him.

He was put back on aspirin again (a double dose for the next 6 months) in addition to his other medications and we need to be aware of signs of infection in the valve. He was back at the Brompton for a repeat echo and outpatient appointment 6 weeks and 3 months later.

Usual morning medication (still the same today 26/02/22)

I noticed that since recovering from the procedure, Harrison started asking lots more intelligent questions and contributing meaningfully to conversations on current news events. It made me realise just how tired he must have been feeling for so long, yet he did his best to carry on as normal. 

And he's free!

Compare this procedure to the one in 2009. You can clearly see the immense benefits to the patient, their family and the NHS when it's possible to perform procedures percutaneously. A rough estimate for the bed only (procedures, medications, equipment, doctors and food not included) was around £38,000 for the 2009 operation and £800 for this week. Thanks to the ongoing research and development, the outcome for Harrison is the result of all that hard work. There are a lot of problems in the NHS but without the expertise and commitment of Dr Rigby, Mr Shore and their teams Harrison wouldn't be able to look forward to such an exciting 21st birthday year of activities.

Fourth Heart Surgery (2009)

Here's the link to the whole blog that Bernard and I wrote back in 2009:

 

https://harrisonsheartop2009.blogspot.com 

There are 37 posts in the original blog so this is a very brief summary! I've covered the first posts in more detail and just summarised the themes from the rest. 

Wednesday, July 8, 2009

The Day Before

All of Harrison pre-op checks (blood test, ECG, ECHO, X-ray, general obs, surgeon consent, anaesthetist visit) happened in quick succession. All the medical staff who have come to talk to us today have also checked whether Harrison has had any questions. He signed his consent form and said he was feeling confident about tomorrow! We feel more reassured having spoken to one of the surgical team although I can't say that I'm feeling confident! So, what are they planning to do? The plan is to replace the current pulmonary conduit (donated artery fitted over top of Harrison's heart which by-passes his own pulmonary artery) with an aortic artery. The reason being, that should they need to replace the valve in the new conduit, it is now possible to do this by cardiac catheter (through the femeral artery in the groin) which means that Harrison's chest shouldn't need to be opened again. They are also going to cut out part of the remaining pulmonary artery above the conduit where it joins into the left and right lung arteries. This has always been a problem for Harrison as it is a narrowed area which has stents (metal cages) in it, to keep it open. They are going to replace the dissected part with a patch which will increase the size of the artery. At the same time, they will take out the stents as they will no longer be needed. The operation should take around 8 hours in total with Harrison being first on the list at 8am. In true Harrison style, he doesn't want to give the surgeon an easy time, so his current conduit is lying extremely close to where they need to open him up. As a result, they may need to put him on femeral bypass before they even attempt to open his chest. Bernard and I were quite relieved to hear how they were going to tackle this slight technical hitch. We feel a bit better about the procedure now. As I write, Harrison is just off for a chlorahexidine wash. This is a new procedure where he has to shower using a special orange lotion to specifically clean his torso, arm pits, arms, groin and neck. He then has to repeat this cleaning regime in the morning at 6am. (Note Harrison and Bob's matching outfits!)

Thursday, July 9, 2009

Bit Rocky but Good News!

Harrison was anaesthetised at 8:20am this morning so now we are wandering around like spare parts. We had a brief visit from Harrison's surgeon this morning just wanting to ensure that we'd been informed about the probability of Harrison going on to femoral bypass and therefore returning with another wound. The surgeon seemed very relaxed and did say that they would be reviewing Harrison's MRI scan again before making any incisions. 

When we arrived at PICU at 6pm, there was an empty bay where Harrison should have been. He'd been delayed in theatre. It was 8pm before we finally got to see him. He had certainly been making his presence known to all the PICU staff. When he arrived in PICU he dramatically dropped his blood pressure and then started to flip in and out of heart rhythms. He had developed a problem with the top chambers of his heart. The electrical pathway wasn't triggering them to contract so he was losing 30% of his blood pressure as a result. There was a lot of tweaking of medication, two bags of platelets and three bags of plasma infused very quickly; clearly evident by the empty blood bags abandoned around Harrison’s bedspace. He settled for a bit but then started to flip his rhythms quite regularly. They were also having to cool him internally so there were ice packs all around him. As he was quite unstable, they called the surgeon back in from home, his assistant, the paediatric electrophysiologist, consultant intensivist, cardiologist and several other doctors. Harrison was certainly keeping everybody busy! They were considering putting in atrial pacing wires but fortunately he finally settled on a particular drug. We left his side around 12:15am this morning as he seemed more settled. Since then, he has been much better and when his blood pressure dropped after that, he was able to recover it quite quickly himself. Since returning from theatre, they had paralysed him to keep him very deeply asleep. As he had improved overnight they stopped the paralysis at 6am so that when I arrived at 8am he was beginning to wake. Now it was my turn to be busy! Every time he woke a bit, Harrison went straight for his 3 chest drains and the ventilator. It was a bit like trying to catch an octopuses arms at times. Considering he was only semi-conscious he was doing a good job of out witting me! As he became more awake, the ventilator was distressing him more and more. He was doing so well at trying to breathe for himself that they extubated him at 9:30am. He now just has minimal support from oxygen through nasal cannulae. What a difference a few hours can make! To say we are relieved is an understatement. I am expecting him to still be quite tired today but hopefully now he will make steady improvements. They think that he's over the worst of the rhythm problems but they still need to keep a close eye. Because of last night’s dramas, we had the opportunity to speak to Harrison's surgeon at length. He wasn't able to patch the pulmonary stenosis because when he looked at the narrowing inside the chest, it went right down into the lung, so just widening part of the artery wouldn't have sorted the problem. Harrison is left with the stenosis but the surgeon is going to ask the cardiologist whether he might be able to dilate the artery a bit more by cardiac catheter. As for the donated artery; he replaced the calcified one and everything looked great on the ECHO. Apparently before the operation, Harrison's ventricles were struggling to contract properly because the conduit / homograft was so stenosed. The new one immediately enabled the ventricles to contract properly but it showed that the valve inside the new artery was already leaking. The surgeon wasn't happy with this as it would have meant a valve replacement operation in about six months’ time, so he removed that conduit (basically repeated the whole operation) and put in another. This one is working perfectly with no regurgitation (back flow of blood). That was one of the reasons why the surgery took longer. The other was Harrison having his first rhythm problem. So all in all, Harrison is doing brilliantly now.

Friday, July 10, 2009

A Good Afternoon

When I got back to Harrison he was being put onto a 'NIPPY' BiPAP machine. The oxygen through the nasal cannulae wasn't giving him enough support with his breathing so this machine is part way between a ventilator and the nasal cannulae. It is set to push air into his lungs and to give him extra help when he breaths for himself. It is administered with a face mask held in position with straps. As you can imagine, Harrison was not impressed by this and got a bit stroppy! Once he got used to it though, he tolerated it quite well. He was allowed a little break from the NIPPY around lunchtime as they needed to scan his diaphragm. The base of Harrison's lungs were silent on examination so the intensivist wanted to check whether his diaphragm was moving downward to allow his lungs to inflate. The scan confirmed that it was moving - just, so he needs to stay on the mask for the time being. As we removed the mask for the scan, Harrison was quite agitated and clearly upset about something. We were all concerned that he was in pain but he managed to say why he was upset - 'Grand Prix'!! He was worried that he'd missed Sunday's German Grand Prix!!  The only other event to note this afternoon was another rhythm episode. He went into nodal rhythm for quite a while but managed to maintain his blood pressure this time. The reason for this episode was Harrison's chilled out position with his arm above his head which occluded the neck line where the drug (Isoprenaline) was infusing to stop the episodes. He also had some vomiting around this time and told us that his heart hurt just before the episode started. He will be remaining on NIPPY overnight so fingers crossed he should stay quite settled.

Saturday, July 11, 2009

The Night Shift and a Busy Morning

Apologies for the late post but our boy has been keeping us rather occupied! We left him last night on the NIPPY machine with his usual melatonin medication sending him to sleep. Our plan was to have a good rest and return to him early in the morning. Harrison had other ideas! He awoke at 12:30am asking for us. We both went back to the ward and stayed with him until 3am. He had hold of both my hands and every time I so much as twitched a finger, his eyes would open and he would hold my hands even tighter. He was also asking me what time it was every few minutes. It must be very distressing and disorientating to be in a room full of strangers, dosed up on medication and feel so rough. (Despite antiemetics, he was still vomiting everything that went into his stomach, which is currently only sips of water and medicines). The doctors finally decided that the only way any of us would get any rest would be by sedating him. Fortunately, this did the trick for a few hours and he didn't wake until 8am when he asked for us again. He had been on Nippy all night (which apart from assisting his breathing was a great way of keeping his nasal gastric tube away from stray little fingers!) but despite this, he was still working really hard to breathe. A chest x-ray was done before we arrived this morning and it showed that there was a lot of consolidation at the bottom of both lungs. Basically the alveoli were closed up and air was not able to enter his lungs. The decision was made for lots of physiotherapy and if that didn't work then the doctors were considering re-intubation as to look at, Harrison was absolutely exhausted.

Bernard and I were quite happy with this decision as we were concerned at the amount of effort he had to make to breathe. The physiotherapist was just about to start working on him, when Harrison had a dramatic tachycardia (192 bpm) complained of chest pain and went out of rhythm again. It took a while to settle but he is OK now.

After the ward round, the surgeon asked for two of Harrison's three chest drains to be removed. The first drain caused Harrison a lot of distress when it was pulled out and he asked to be put to sleep for the next one. He had a bolus of morphine to help instead. During the second procedure, the nurse inadvertently cut the stitch which pulls the skin together once the drain is out. This meant that the surgeon had to come and put in another stitch. He was certain that the drains had been labelled up incorrectly (he was the person who had inserted them initially) and that the one which they were removing should have been the one that stayed! As this one was no longer draining, he decided to take them all out. Since their removal, Harrison has been better with his breathing, but still needs a careful eye as there are signs that he's still working hard. The past 18 hours has been so hard for Harrison but he is being so brave. He is making everyone laugh by the way he gets stroppy and orders the intensivist and daddy out of the room! 

A summary of the rest:

Harrison added apnoea to his bag of tricks! Every so often he would stop making any respiratory effort for what seemed like several minutes, but in reality is probably only a couple. Still, that's more than enough when you're watching it happen!!

Harrison has probably always suffered from sleep apnea. Whilst he was dependent on oxygen 24 hours a day that alleviated the situation. Following his third surgery though (where he was finally weaned from the continuous oxygen) the apnoeas became increasingly more apparent.

At that point, we were advised that it was safe for Harrison's oxygen levels to drop to 90% before we should be alerted. Previous to this his oxygen levels hovered around the high 70s to mid 80s and that was normal for him. We'd been allocated an oxygen saturation monitor from when he was a very tiny baby and we secure this to his foot at night so that we can be alerted to any drops in his oxygen saturations. We continue to monitor his levels to this day. It's one indication that he's starting to become unwell so we're able to take preventative action at the earliest opportunity. We were becoming increasingly alerted by the oxygen saturation monitor to a fall in Harrison's oxygen levels overnight. Sometimes it would be up to five times a night. We would immediately jump out of bed and go into his room. We'd initially observe the equipment to see whether it would resolve on its own. Invariably, we'd have to give Harrison a shake to make him start breathing again. It was a welcome relief when he started to display the apnoeas post-surgery and the doctors realised that he needed a bipap machine overnight to support his breathing. Looking back now for the 12 years that we've had the machine, Harrison definitely wakes up more refreshed then he ever has done and I can honestly say that the machine has made a huge difference to him. I also think that by not being as tired his immune system is more robust, so he's actually caught less chest infections since too.

It took several overnight sleep studies during this fourth post surgery period to get the appropriate settings for the BiPAP machine partly due to complications following the surgery - a collapsed lung, partially paralysed diaphragm and a dose of Swine Flu! You can read the daily dramas of these in the full blog -

https://harrisonsheartop2009.blogspot.com 

As for previous surgeries, the other main issue post surgery was Harrison's inability to eat. This time he lost around 4kg in weight and couldn't be tempted by any tasty treats. He was prescribed Calogen (which does exactly what its name suggests - gives you far too many calories in a tiny quantity), multivitamins and vouchers for the restaurant so that he didn't need to eat his evening meal so early. He was also allowed extra items from the menus on the ward.  Harrison put his jeans on for the first time in two weeks and they really illustrated how much weight he'd lost; you could virtually fit two of him in them! 

Finally everything was under control and Harrison was safe to be discharged with an appointment to return in 4 - 6 weeks for a respiratory review.

The gap between Harrison's third and fourth surgeries highlighted the advances in PICU care. I was amazed how some children were now coming back from surgery extubated and barely spending a few hours in PICU before being transferred back to the ward. As usual, Harrison lagged behind, making himself at home in his PICU bed!

Glossary:

BiPAP - Bilevel positive airway pressure (BiPAP) ventilation is a noninvasive technique used to provide ventilatory support to a spontaneously, but insufficiently, breathing patient using a facemask or nasal mask. 

Sleep study - The most common kind of sleep study is called a "screening study". It makes overnight recordings of:

• oxygen levels (by attaching a small clip to the patient's finger)
• breathing (by taping a small sensor between the patient's nose and upper lip)
• chest and abdomen movements (velcro straps are used to hold small sensors in place)

Harrison's Third Heart Surgery (2000)

*Please note that there are photographs of Harrison in intensive care in this post. I don’t take photographs of him when he’s unconscious or unstable. However, I’m aware that some of the photos with Harrison having therapeutic oxygen /  non-invasive ventilation support, and with various lines going into his body may upset some people.*

 

Harrison spent his time on the ward confined to his highchair because his tube feeds had to be virtually trickled into him . His body was struggling to keep him going, and feeding and digestion was just another stress on his system. At least we had the Sydney Olympics to keep us all occupied! We rarely made it outside the hospital to enjoy the summer sun because Harrison was not well enough.

He painted this abstract picture a couple of days before his third surgery. It's proudly displayed at the top of our stairs at home:

Harrison had to wait until October 9th for this surgery. His surgeon had gone away for a foreign conference and the intensive care unit was blocked with long-staying cases. Harrison wasn't in the best shape and the complex surgery meant that there was a 20% chance that he wouldn't survive it. I had to sign the consent form......

Harrison had a major bleed towards the end of the surgery; the surgical team experienced great difficulty in tracing, and eventually stemming the bleed. The surgeons had closed off more surplus arteries they found, and had fitted a homografted pulmonary artery from the lower end of his right ventricle, over the heart, and reconnected to the junction of the left and right pulmonary arteries. The large VSD Harrison had, was patched. His right pulmonary artery was widened. The homograft would have depressed Harrison's right-sided aortic arch onto his trachea and oesophagus, so the surgeons stitched the homograft onto the chest wall. They had not performed this step before in similar operations. One large surplus artery remained which the surgeons physically could not reach safely. 

Harrison recovered fairly well initially, but after several days on a ventilator, his progress stalled, and the intensive care team couldn't deduce why. Five days post-surgery, Harrison was diagnosed with parainfluenza and was isolated. This could often be fatal. He spent a month in intensive care with three weeks being sedated and ventilated to give his body time to recover from the virus.

We spent as much time as we could next to his bedside just talking and reading to him, holding his hand and reassuring him. However, we obviously needed breaks to eat and rest so that we could support him, although I mostly kept going on adrenaline to minimise my time away from Harrison. 

During our absence and to give Harrison a well-deserved break from the monotony of our voices, we played his favourite video to him via a Walkman (hands up if you remember them!) and headphones. Harrison had developed an obsession for a particular Tweenies video – “Tweenies Song time”. He would watch it on repeat day after day and it brought him much joy. He even managed to monopolise the single television in the bay he shared with 5 other patients whilst waiting for this surgery! 

Knowing Harrison’s past history of recovery from surgeries and procedures, I’d anticipated that he may not be able to watch his video for a while and had recorded it onto a cassette tape prior to his untimely re-admission. It was something that I had decided to do during his intensive care stay following his first surgery. At that time, I’d repeatedly read some of his favourite books to him but wanted to work out a way of providing a bit more variety whilst he was unable to interact. I hoped that he may appreciate hearing familiar, favourite songs. 

An added benefit of playing tapes through headphones to Harrison was that they helped to block out the sounds of the intensive care unit because although he was sedated, he was still aware. During this hospital admission a close friend sent him a Thomas the Tank Engine story tape too. Thomas was another favourite of his which we then alternated with the Tweenies.

Harrison was extubated on Bernard’s 30th birthday. What better birthday present could you ask for?! Although Harrison was still quite drowsy from all the sedation, being off the ventilator and just on non-invasive ventilation was a huge boost for both Bernard and I. So much so, that we both felt able to celebrate the occasion by going out for a meal with Bernard’s eldest sister and her family who’d very kindly came up to share Bernard’s special day with him. Until that day, we had only eaten in the hospital restaurant and coffee shop, not wanting to be more than a couple of minutes away from the children's ward  or Paediatric Intensive Care Unit (PICU).

Spending time without supplementary oxygen was initially very tiring for Harrison.
He already had poor muscle tone due to 22qDS but had lost even more
and needed extra support to sit up.

As Harrison recovered in intensive care, he was given some simple puzzles to do to see whether there had been any lasting impact on his brain.

Forget the baby puzzles; he wanted proper toys
(aka dad's Blackberry!)

Being such a long time in PICU the staff became very familiar with him and decided that he should start doing some work and one nurse in particular liked Harrison to help her log notes about his care onto their computer system!

He was treated like royalty, in that a television was wheeled in for him and as you can see from the photograph below, he was glad to be able to see and catch up on his favourite television programme of the time which was ‘Who Wants to be a Millionaire?’ A very strange choice for a 2 ½ year old but he even had the quiz book in his toy box!

It doesn't matter how poorly you are, 
you still have to have your hair washed!!

When he was finally well enough, Harrison spent another three weeks recovering on the ward. His gut motility had stopped working so he ended up on Total parenteral nutrition (TPN). He'd also been fitted with a gastrostomy (a more permanent replacement for the nasogastric feeding tube) a few months previously which was vital in helping his recovery back to 'eating' normally for him. You'll read in other blog posts how Harrison's eating always takes a hit post surgery; he fancies food but just can't eat it. 

It took his stomach a couple of weeks before he could digest food properly, and because he had spent several weeks in bed, he could no longer sit up or walk unaided. 

He was so impressed with his Happy Meal - not!
We couldn't tempt him to eat more than a bite of one nugget. 

The physiotherapy team brought the special armchair (pictured above) to the ward for Harrison to use. It was completely supportive and could more or less mould to Harrison's body shape thus keeping him in a better seating position. If we sat him in his high chair, he would just end up falling to one side because he couldn't support himself any longer. As you can see from the photograph he spent time lying down in his bed playing with his toys because he was no longer strong enough to sit up in bed.

 

Harrison was admitted to The Royal Brompton in the height of summer and discharged back to our local hospital for a few more nights in December. 

I’d not experienced an admission where we spanned two seasons before. However, we were home in time for Christmas!

 

After four months of intensive occupational and physiotherapy at home, Harrison was able to walk unaided again. 

Things were going well, but then he started catching recurrent infections again, so is now taking regular prophylactic antibiotics.

 

Glossary:

Extubated: when a doctor or nurse removes the ventilator tube that was helping a patient to breathe.

Gastrostomy: a tube which is inserted through the abdominal wall into the stomach. It is usually used to give nutrition to an individual unable to consume sufficient calories by mouth.

Non-invasive ventilation (NIV): uses a machine to help with your breathing. It's a bedside machine, connected to tubes and a mask, which is worn over your nose and mouth. As well as helping with your breathing, it supports your lungs, giving your body the chance to heal .

Prophylactic antibiotics: are antibiotics taken to prevent infection. Prophylactic antibiotics decrease the risk of an individual developing infections that can sometimes be life-threatening. They can also stop a chronic or recurrent infection from coming back. 

Total parenteral nutrition (TPN): is a method of feeding that bypasses the gastrointestinal tract. A special formula given through a vein provides most of the nutrients the body needs. The method is used when someone can't or shouldn't receive feedings or fluids by mouth.

Harrison's Second Heart Surgery (2000)

Harrison had a frustrating habit of becoming unwell a few days before or even the night before a scheduled surgery. It’s a surreal feeling when you‘re waiting for your child to deteriorate sufficiently to qualify for the next stage of surgery to improve their quality of life. Until you receive the date, you try to carry on with your usual routine, doing your best to balance developmental and socialisation opportunities with protecting them from infections and viruses which invariably lead to further hospital stays. 

Just before my second surgery. My uncle took full advantage
of my reduced mobility by introducing me to video games aged two! 

The first date scheduled for Harrison’s second heart surgery was in June 2000. This was the first time that we became aware that rather than summer being a healthier time of year for Harrison, it seemed this was when he was most prone to develop pneumonias. It was a pattern that presented for several years throughout his childhood. Another round of antibiotics and time to recover, then another date was diarised. The second time, we made it to The Royal Brompton and during the pre-op checks the night before surgery, Harrison spiked a high temperature. Further symptoms swiftly developed so he was sent home again, this time with a plea from the surgeon to Harrison’s local consultant paediatrician, to get him well enough for surgery!

It was not only incredibly frustrating for the surgeon, but it was difficult emotionally for us too. As soon as the surgery date has been confirmed (we usually have a couple of weeks’ notice), I can’t help but start thinking about what might be. It’s really difficult to explain; I feel relief and optimism that Harrison’s being given the chance of better health but at the same time, I think about possible complications that might arise during or post-surgery. With so many surgeries behind us now, I have too many experiences to draw on! From the time the date for surgery is confirmed until he’s safely breathing on his own again, I’m functioning on autopilot; feeling empty / numb. So, you can imagine how stressful it is when surgeries are cancelled for any reason and we’re back to being in limbo not able to resume our life because we need to keep a very vulnerable child free from infection in anticipation of an imminent surgery date. 

Anyway, after a few false starts, Harrison’s second surgery (a right-sided unifocalisation of the multiple collateral arteries) went ahead in August 2000. 

How do Bernard and I fill our time whilst we're waiting for Harrison to return? Our routine is always the same. He’s more often than not first (or the only patient) on the surgery / procedure list for the day. 

We never eat before Harrison is taken to the anaesthetic room. It doesn't seem fair when he's not been able to eat anything since midnight the night before. We established a routine from the first diagnostic cardiac catheter when he was about 8 months old. 

After leaving Harrison anaesthetised, we eat breakfast in silence in the hospital restaurant. Next we amble (no need to rush; we've usually got at least eight hours to fill) across Albert Bridge to Battersea Park and sit on our usual bench backing onto the Thames. We try to busy ourselves by watching the world go by (land, water and air) whilst trying not to look at our watches more than 100 times an hour! We barely speak a word to each other but when we do, it’s never to make any post-surgery plans. At lunchtime, we head back across the river to get a main meal. Not because we're hungry, but in case we don't get a chance to eat later because we want to sit next to Harrison's bed. 

Battersea Park or the Peter Jones department store at Sloane Square (if it's raining) are the furthest that I’m willing to be away from the hospital when Harrison is having surgery. They’re both approximately 15 minutes’ walk away in different directions but close enough to be able to run back to the hospital in a few minutes. After lunch, we wander back in the general direction of the hospital pausing for another couple of hours in St Luke’s Gardens. It's another tranquil spot which happens to be conveniently located directly opposite the hospital! 

St. Luke's Gardens.
The building in the background is the Sydney Street wing
of The Royal Brompton Hospital.

When we can bear it no longer, we saunter into the hospital failing miserably at pretending that we haven't a care in the world! We always return to the hospital far too early but we’re forever optimistic that Harrison’s surgeries and procedures may be without complications. The only places for us to hang around and wait in the hospital are the parent room or the coffee shop. The bed space that Harrison had previously occupied on the ward would have been reallocated to another patient. Even if the pager we’d been loaned and our mobile phones haven’t indicated that it’s time, we impatiently head to the intensive care unit to ensure that we're there at the estimated time for Harrison’s return and to see if we can catch a glimpse of him on his way back from the operating theatre. 

We follow the same routine every time……

Thankfully, for this second surgery, Harrison didn’t follow his usual path and recovered very quickly. He was extubated the following morning and discharged from the general ward eight days after the surgery. This was the first of only two out of five textbook heart surgery recoveries for Harrison, so he took everybody by surprise!

He was sent home still requiring oxygen, but it was hoped that his need would diminish rapidly soon after. 

Completely spoilt by my grandparents
but very handy when you're too tired to walk!

This was not to be the case. He became more and more irritable, and soon needed more oxygen than ever before. To ease his breathing overnight, we raised the head end of his bed. He still sleeps on an inclined mattress today. 

He was extremely breathless, and couldn’t walk two metres without his oxygen levels in his blood dropping to low 40%. This was while he was being given THREE litres of oxygen per minute. This amount gave Harrison severe headaches, and the extreme breathlessness caused vomiting and weight loss. He was sent back to the Brompton only two weeks after going home. The Brompton staff decided that Harrison should remain with them until he could undergo a total correction for his heart condition. 

Heart Awareness Month / Harrison's First Heart Surgery (1999)

Once again, I’m introducing a temporary break in Harrison’s story. 

This image popped up on my Facebook timeline
but I can't find it again to attribute the creator.

February is heart awareness month, so it seems appropriate for me to share the trials and tribulations of Harrison’s five major heart surgeries to date. 

As you can see from the graphic above, approximately one in 100 babies are born with a congenital heart defect (also known as congenital heart disease) (CHD). Not all heart conditions are due to an underlying genetic condition. Congenital heart disease is a general term for a range of birth defects that affect the normal way the heart works. The term "congenital" means the condition is present from birth.

For individuals with 22q11.2 deletion syndrome approximately 70% of them will also have a heart condition. As with other aspects of the condition, these will vary in severity.  Unfortunately Harrison ended up on the more severe end of the scale but we view ourselves as fortunate because he's less affected by other symptoms of 22q11.2 deletion syndrome.

Twenty three years ago I didn’t document each day of the heart surgeries, however the major events (good and bad!) of each surgery will be etched on my mind forever. 

Harrison will require ongoing surgeries throughout his life until such time that a more permanent solution can be developed for his replacement parts!

 

In addition to these surgeries, Harrison’s had countless cardiac catheterisations under general anaesthetic to widen and / or stent his narrowed arteries and diagnostic procedures to accurately check pressures in various parts of his heart, in preparation for the surgeries.

Before I recount his first surgery, I want to show you what the surgeons were dealing with. Here's a picture of normal heart courtesy of The Brompton Fountain:

To recap, the diagnosis of Harrison's heart condition at four months of age was Tetralogy of Fallot's with pulmonary atresia, ventricular septal defect, Major Aorto-Pulmonary Collateral Arteries (MAPCAs), left-sided aortic arch. Pictures below courtesy of The Children's Heart Clinic at Children's Minnesota

A rather blurry normal heart from the same source 

to show the comparison with Harrison's heart

Harrison's pulmonary artery was completely blocked which meant that blood couldn't get to his lungs for oxygenation. He had a hole between the two pumping chambers of his heart which meant that the blood to and from the lungs was mixing, hence the purple colour in the first diagram. To help out, several extra arteries had grown to try and get blood to his lungs. Very helpful in keeping him alive in the short term but not effective long term. They make the corrective surgery more complex. Harrison was due three surgeries to complete the 'repair'. I've put repair in quotes because as you'll realise, this isn't a one time fix. Tweaks are required lifelong to keep things working as well as possible. Harrison will never have a 'normal' heart but it can function adequately with surgical interventions and medicinal support.

The day before admission to The Royal Brompton for surgery

Harrison underwent his first heart surgery (a left-sided unifocalisation of the multiple collateral arteries and the addition of a Blalock shunt) at the Royal Brompton on 8th April 1999. He was 15 months old. A unifocalisation joins the collateral arteries into the main pulmonary artery and the Blalock shunt provides an additional, temporary route to get blood to the lungs.

Below is a link to a fantastic animated video not only explaining the process of unifocalisation (and letting you have a go at a virtual repair) but it also explains pulmonary atresia and covers the rest of the stages in Harrison's subsequent surgeries. It is presented as a single surgery but in Harrison's case the surgeon decided that each stage should be performed separately in order to provide the safest outcome for Harrison:

https://www.stanfordchildrens.org/en/service/heart-center/unifocalization

It may take a few seconds for the page to set up, but it really is worth watching all three sections of the video if you'd like a better understanding of Harrison's heart condition and the surgeries.

The Blalock shunt part of Harrison's first surgery isn't discussed in the video so I've created a separate page using a PDF resource from Children's Minnesota to explain it. You can find the page on the top left section of my blog or follow this link: blalock-taussig-shunt-procedure

To access the area necessary for the procedure, the surgeon made the incision around Harrison's left shoulder blade.

Harrison had complications after the 8 hour surgery. They started as soon as he was returned to the Paediatric Intensive Care Unit (PICU). He was difficult to ventilate because his lungs were stiff and he was trying to fight the ventilator, despite being sedated. In the end, he had to be given a drug to paralyse him to enable the team looking after him to gain control. They had already started to realise that Harrison doesn't like to follow the medical textbooks. He likes to do things his way which in this case meant that our 15 month old baby required an adult dose (or in the intensivist's words, enough medication "to slug an elephant"!) Whilst ventilated, the respiratory team were called in to perform a couple of bronchoscopies to try and identify why he was having difficulties. 

Even while sedated, Harrison had worked out a way to communicate with us how he was feeling. We were at his bedside for as many hours as possible each day; I was usually the first parent in the unit in the morning and the last to leave at night. I spent the majority of the time just sitting holding his hand and talking to him. To let us know that he was upset or uncomfortable, Harrison used to squeeze our fingernails between his finger and thumb. It's incredibly painful and I have no idea how he knew to do this or had the strength to inflict such pain! It was a small price for us to pay, given how much he was probably suffering! He continues to do this to this day whenever he's unwell and especially during hospital stays. 

Six days post surgery, he finally appeared recovered enough to be weaned from the ventilator. This is a fine balancing act in withdrawing the sedation and allowing the patient to wake up sufficiently to take over their own breathing again before the tube is removed. Not easy when the patient is aware of the uncomfortable foreign body down their throat and does their best to remove it themselves. Quick reactions from all onlookers were required to prevent Harrison from extubating himself too soon!

The ventilator tube was removed and the nurse looking after Harrison decided that he deserved a long overdue cuddle with me. To give Harrison some privacy and to hide the other patients from Harrison's view (so as not to scare him with all the equipment now he was more awake), the nurse pulled the curtain around Harrison's bed space. She busied herself outside supporting the other nurses with their patients. In the intensive care units (paediatric and adult) the nursing ratio is 1:1 with every patient having at least one nurse devoted to their care. In some instances, patients require more than one nurse to look after them. There's always at least one floating nurse who can support with running bloods for testing, fetching equipment,  drawing up medication etc.

I was uncomfortable being hidden from view and left in complete charge of a drowsy Harrison who was clearly not in any hurry to wake up properly. I was watching him like a hawk and could see that his efforts to breathe were becoming less frequent and more shallow. I called the nurse back but she assured me that he was doing as well as expected. I was far from convinced and felt that there was something wrong. 

Fate intervened just at the right time (although I'd have been much happier to have seen her much earlier!) when a senior physiotherapist turned up to give the patients in our bay, chest physio. It's vital that the patients are helped to clear any secretions that lay on their chests, not only to improve their breathing and prevent infections but the secretions can also block the ventilator tube. 

By the time of Harrison's first surgery, he was already known to many of the Brompton staff due to several stays on their children's ward. The physio was one of those who knew him. I can't tell you the relief I felt when she scooped him out of my arms, returned him to the bed and called for help. By now, Harrison was barely breathing. 

Unfortunate timing, but Bernard's parents had just turned up to visit and I noticed them looking through the window. Bernard was with them and distracted them whilst Harrison was re-ventilated. 

Following another three days of ventilator support, the second extubation was successful and Harrison was carefully watched to ensure that he didn't deteriorate again. That evening he was moved into the High Dependency Unit where there was one nurse to every two patients to ensure further recovery before being discharged back to the busy ward. The regime of chest physiotherapy continued and was supported by one of the nurses. She was over enthusiastic in her approach to clearing Harrison's lungs by whacking him on his back over his wound site. It was covered by a dressing but every time he was hit, Harrison screamed in pain and tears streamed down his face. After witnessing this traumatic event a couple of times we had to ask her to stop. Our baby was incredibly brave and resilient but once again something wasn't right. Chest physiotherapy can look quite brutal sometimes but it shouldn't make the patient scream in pain. When the dressing was removed, it revealed an infected wound site. Unfortunately, this incision didn't heal as it should, and Harrison is left with a thick lumpy scar. 

This was a couple of months after he arrived home again.
You can see how red and thick the scar under his left shoulder blade is

The corresponding scar on the opposite side under his right shoulder blade (later from surgery no. 2) is just a thin line and barely visible. Thankfully, Harrison has never been bothered by the look of his scars (not that he can see the two on his back!)

The smiles are back and he's well on the road to post surgery recovery

He left hospital three weeks later.

The day after he arrived home

Glossary:

Bronchoscopy: A Bronchoscopy is a procedure in which the doctor passes a thin tube  through the nose, down the windpipe through the voice box and into the bronchial tubes of the lungs. This procedure is used to find the cause of a lung problem. It can detect tumors, signs of infection, excess mucus in the airways, bleeding, or blockages in the lungs. It also can allow the doctor to take samples of mucus or tissue for other laboratory tests, as well as to insert airway stents, or small tubes, to keep the airway open to treat some lung problems.

Cardiac catheterisation: A cardiac catheterisation is an invasive diagnostic procedure that provides important information about the structure and function of the heart. It usually involves taking X-rays of the heart's arteries. The resulting images can be used to help plan future treatments and carry out certain procedures (some of which used to be performed via open heart surgery).

During the procedure a long, thin, flexible tube called a catheter is inserted into a blood vessel into the groin or arm. Using X-ray images as a guide, the tip of the catheter is passed up to the heart and coronary arteries.

A special type of dye is injected through the catheter and clearly highlights any blood vessels that are narrowed or blocked. (Source – NHS)

Unifocalisation: The objective of a unifocalisation procedure is to create a single source of blood flow to each lung by combining the MAPCAs.