Harrison has 22q Deletion Syndrome or 22q DS (also known as DiGeorge Syndrome and Velocardiofacial Syndrome (VCFS)). The purpose of this blog is to outline our lives so far (he was born in 1998) and to provide information for other families who also live with a 22q DS diagnosis.
Several years ago, November was chosen as the month to promote awareness of 22q11.2 Deletion Syndrome, with the 22nd being the date to actively highlight the condition. I can't let today go by without a post in my blog. We'll never have a date more closely representing 22qDS. On this memorable date, it seemed fitting to me to celebrate the condition.
Whilst running our charity (The 22Crew), we were regularly contacted by parents whose child had just been diagnosed with the condition. Even as recently as ten years ago, much of the information highlighted the challenges these families were likely to face. As a charity, we were always determined to showcase the possibilities and opportunities rather than dwell on the difficulties. A deliberate act on our part, to not only help ourselves cope but also to offer hope to families often still reeling from the shock of their world being changed forever.
Parents are naturally proud of their offsprings' achievements and many love to share their children's successes on social media. Examples often include; examinations passed, sporting events won, teams joined, awards received; all benchmarked against recognisable standards. The multitude of symptoms associated with 22q Deletion Syndrome imposes various limitations on individuals so they may not be able to achieve in the same way as their typically developing peers. Although their successes may need reframing, it doesn't mean that they are less worthy of recognition.However pleased you are for other people's children, it can be difficult knowing that you won't be announcing similar successes for your non-typically developing child. Most of us have incredible families and friends who love our children unconditionally but sometimes you just want to be like everyone else.
Around 2013 / 2014, The 22Crew created the hashtag #22qCan-Do to encourage the whole 22q community to recognise, celebrate and support the achievements of those affected by 22qDS. To give families a sense of being like everyone else but in a forum that understands the context.
Generally individuals with 22qDS achieve milestones later than those unaffected by the condition, however there are always exceptions, and we're proud to know some incredibly talented people with 22qDS. Talents right across the board from the arts and music to academic excellence and successes in independent living and solid careers.
We believe that it's important to recognise achievement however small the steps might be. What could be considered 'small' to some, will be an incredible achievement for others and several small steps will eventually add up to one big one.
We also introduced 'Friday Achievements' for our members and had a dedicated page on our website to capture a broad range of successes. In addition to public recognition for the individual, our aim was to give actual examples to families who had just received their diagnosis. It was extremely popular.
High self-esteem is vital to give any individual confidence and belief in themselves which in turn maintains good mental health. With an increased incidence of poor mental health for those with 22qDS our charity was keen to play a part in lifting the spirits of the whole family.
Our key messages were:
diagnosis doesn't predict potential
success can take many forms
Even though our charity no longer exists; we're delighted that so many in the 22qDS community around the world still regularly use the phrase #22qCan-Do when reporting updates.
Some of Harrison's achievements over the years. Some obvious, others less so
"This exploratory study aimed to gain an understanding of carer reported experiences derived specifically from persons caring for someone with a rare disease. The survey took place online on the SmartSurvey platform from November 2019 to January 2020. The facilitated workshop took place in Bangor Carnegie Library, Northern Ireland. To be eligible to participate in the online survey respondents had to be adults caring for someone with a rare disease. Fifty-seven respondents took part, 15.8% male, 84.2% female. Thirty- two attendees were part of the facilitated workshop. While carers reported several positive aspects of their caring role, the majority of comments highlighted challenges such as sub-optimal interactions with healthcare professionals, insufficient (or absent) emotional, psychological and social support, lack of financial support and lack of awareness of existing support services. It is important that strategies are put in place to ensure that carers are given the time they need to care for themselves, and that awareness is raised of what support options are available for carers of people with a rare disease(s) from health and social care providers, charities or support groups.
What is known about this topic and what this paper adds?
Caring for someone with a rare disease, both formally and informally, can be an extremely demanding role requiring intense and unique care tailored to each individual's specific needs.
The impact of caring for someone with a rare disease can be seen in many areas of an individual's life including psychologically, economically, physically and logistically.
This study provides insight into current challenges, and some requested solutions, based on reported experiences from carers of people with a rare disease in the UK and Ireland.
The use of an online survey promoted flexibility and accessibility for person's unable to attend the in-person workshop.
The facilitated workshop enabled carers to network, discussing challenges and potential solutions with their peers."
To conclude my posts for Carers Week I thought I'd share this paper with you. it's one of the most comprehensive reports on carers experiences that I've come across to date. Although first published in May 2021, I only became aware of it a couple of weeks ago. When I read it, I realised that it echoes many of the points that I've highlighted in the 'My Story as an Unpaid Carer' posts. I found it easy to read and there are some clear illustrations to emphasise the key points. The link at the top of this post will take you to the original article which you can read online or alternatively, download a twelve page pdf version. In the event that the link no longer works, here's a link directly to the pdf:
One of my aims as a Peer Leader is to be a voice for unpaid carers whenever possible and I'll likely make reference to this paper in future if asked to speak again about my role as an unpaid carer. My grateful thanks to the authors and contributors for highlighting the needs of an underrepresented group.
Do you remember the second part of the definition in my post titled ‘My Story as An Unpaid Carer - A Carer Profile’? It stated, ‘needs support to manage a life of their own.’ This part is often not considered by anybody; least of all the person caring and that can have a huge impact on our health and well-being. So how have I been helped or helped myself?
It goes without saying that our family and closest friends have all been the biggest contributors to our well-being as a family, and their support has been invaluable. I’m very mindful that they’ve also been impacted by Harrison’s condition to various extents, so I’ll be sharing their perspectives in future posts. We certainly wouldn’t be coping as well as we do without them.
Other individuals who have supported me by thinking ‘outside the box’ include:
Responsive Medical Staff – I’m referring here specifically to the to the two incidents in my post ‘His Story - Post Diagnosis 5 - 8 Months’ where I briefly wrote about my collapse from exhaustion on the children’s ward and again when the community nursing team found me barely able to stand at home due to my relentless caring schedule.
In both these examples the nursing staff immediately recognised that I needed support to be able to continue my caring role. Without delay (and no bureaucratic form filling!), they reorganised their schedules to temporarily look after Harrison so that I could take a short rest. Thanks to their compassion, this was all I needed in these circumstances to re-energise me.
‘Can Do’ Administrators – In the past couple of months I’ve had a great experience with two non-clinical hospital staff members. One has chased different departments to save me calling each one and the other gave me her work mobile number so that after I’d organised an appointment for a different department, I was able to call her directly to co-ordinate an appointment in her department for the same day. Only having to travel into London on one day for multiple appointments not only gives us time back (a single appointment can disrupt a whole day once travelling and clinic waiting times are factored in) but also reduces our fatigue generated from the forward planning and travelling to and from the hospital. Small acts of kindness that make a big difference to a carer’s workload.
As I mentioned in my carer introductory post, the carer support in an individual’s own local area will vary, and although in theory there are carer support groups, the only carer support I’ve ever had, is from other parents in online forums. Having said that, I’m the sort of person who will seek out my own coping mechanisms, so most of the strategies below are my personal approaches to keeping myself moving forward:
Voluntary Work – Enables me to use my skills, knowledge and experience in a positive way to improve the lives of others. It gives me a sense of fulfilment without the pressure of a regular commitment. It also helps to improve my confidence and self-esteem, develop new skills and / or improve existing ones. Examples my voluntary work include; Chair of a national charity, patient and public engagement opportunities through the Royal Brompton hospital and national Peer Leader Network.
Dance Classes – I’ve always danced, and pre-pandemic I would attend several classes per week. It was my escape; I couldn’t think about anything else when I was concentrating on learning the routines or improving my technique. It was also where I’d developed some good friendships so it was my main source of socialisation too. It was the only place where I was known as myself and not for being Harrison’s mum.
Lockdown gave me the opportunity to try dance classes from different providers. Although online classes didn't work out for me with the dance school I used to attend in-person, everyone had to become more flexible. It no longer mattered where anybody lived and I suddenly had access to virtual classes being offered worldwide. Not knowing the other participants and with everyone being online, worked much better for me. Many of the online classes have now ceased but I’m still able to subscribe to virtual classes which actually fit really well around my other commitments.
Crafting – I love creative activities and usually have many different types of projects on the go simultaneously, which means a constantly messy work area! Unfortunately, I don’t usually start working on my projects until about 9pm, by which time I’m already falling asleep! In June 2020 I fulfilled a long term goal of learning amigurumi. I'm slowly building up a large community of crocheted animals. Many of my unfortunate friends and family have been the recipients of my bespoke creations! This was my first attempt:
Video calls with friends – When the whole country locked down, a friend for over 35 years who lives abroad, started a regular Sunday night video call with me and two others. We’ve kept it up every week since and have all benefitted hugely by reconnecting in this way. Incredibly, we usually speak for nearly three hours but never run out of anything to say! It's become the highlight of my weekend. I also have video calls with other friends that I’d usually have met up with socially.
Writing blogs – I wrote my first blog in 2009 to inform friends and family how Harrison was getting on following one of his major heart surgeries. It meant that I could give informative updates without taking me away from his bedside for too long making phone calls. From then on, I continued writing and sharing short blogs for holidays and other hospital admissions.
When lockdown started in March 2020, I wrote a private lockdown blog (effectively a diary) every day for the first 600 days to manage my mental health around our forced isolation. I still add to it sporadically. Writing things down really helps to clear my head.
The purpose of this blog about 22q Deletion Syndrome is more than that though. My aim is for it to be a tool to educate, support and inspire too.
Having established my coping mechanisms it's not all rosy. Some days I have a complete mental block and can’t function. However, I’ve learned to give myself some time, whether it’s a few minutes or a whole day, and by choosing one of my personal coping strategies, I’m able to get myself back on track and on with the job of caring for Harrison.
For me personally, it’s my choice to care for my son full time. Despite this, it has had a big impact on our lives.
At the centre of the circle on this slide is Mr Nobody from the Mr Men. The reason I picked him to illustrate this topic will hopefully become clear soon. In addition to being Harrison’s carer, I’m still trying to maintain a sense of who I am too.
In no particular order here are the main impacts of caring on me:
Loss of income – Not contributing financially to our family is hard for me. We did try out adult social care for Harrison so that I could go back to work but the stress that it caused all of us trying to implement it appropriately to meet his needs, outweighed the financial benefits. Instead, we adapted our expectations from life and consequently appreciate different things now.
For being Harrison’s main carer I’m paid an allowance of £69.70 per week by the Department for Work and Pensions. This equates to roughly £3,600 per year. I did a little research into the salaries for each of the jobs in my caring profile (see previous post). The average salary for those 9 jobs would be approximately £33,000 per year. So, you can see that there’s quite a substantial difference between what I’m paid for caring and what the roles would pay if I was seeking employment in just one of those positions.
This is before you consider what my salary would likely have been if I’d have been able to continue working. The average Training Manager salary is £46,000. I like to think that I might have achieved a management role after 24 years! Apart from some temporary work which never lasted very long, due to the unpredictability of 22q Deletion Syndrome, I’ve not worked properly since Harrison was born. As a very rough comparison of my actual and potential income looks like this: £86,400 in carer’s allowance versus £1,104,000 for full time employment or £552,000 for a part-time role for roughly 17.5 hours per week over 24 years.
Loss of social life / isolation – Having to be responsive to Harrison’s medical condition has caused my relationships with some friends to deteriorate over the years. Being unreliable and cancelling meet ups at short notice meant that some friends fell by the wayside. It wasn't their fault; I can only imagine how frustrating our situation was for them. At the time it was upsetting to lose their friendship when I desperately needed their emotional support. However, over the years I've developed new friendships who are all understanding of our family situation.
The Covid-19 pandemic has re-highlighted my sense of invisibility. I felt on an equal par with the rest of society when the whole country was in lockdown. It was brilliant! Suddenly everyone had a glimpse into our lives. A highlight for me was the dance classes that I attended for my own mental health benefits, moved online. However, as soon as they were able, the dance school reopened for in person classes. Although I was able to join in virtually, it highlighted my isolation which upset me. I could see all of my friends together in class and I couldn't physically be there with them. I won’t even mention my appalling spatial awareness and inability to work out which way I was supposed to be facing or which arm or leg I should have been using! All exacerbated by having a screen between us. Unfortunately, instead of reducing my stress levels, it started to increase them, so I no longer attend. I’m coping much better by just knowing that all my friends are there but not actually seeing them in class without me.
Overwhelming sense of responsibility – I feel as though I'm muddling my way through trying to coordinate Harrison's care in adult services both medical and social care. It seems that if I don't do it nobody else will. There have been examples of this where he's been under several consultants for various parts of his condition but for whatever reasons he's not been followed up as stated in clinic letters. In paediatrics Harrison's paediatrician would have picked this up and now, because Harrison can't do it himself, it falls to me. I spend a fair amount of time calling medical secretaries trying to organise tests, therapies and appointments. I also seem to be the middleman between our local and London hospitals because they don’t always communicate very well with each other.
Emotional drain - Having to constantly think outside the box to solve problems or find a solution that works for several parties; all the chasing and coordination required; and being alert to any changes in Harrison's condition are extremely draining.
Loss of identity – Along with the loss of income, there’s also the loss of career which for me was part of my identity. I was proud of the job I had, which had improved my self-confidence and self-esteem. No longer having that to talk about with friends and family was really difficult. Perhaps I have the wrong mindset about ‘just’ being a carer but medical and other professionals involved in Harrison’s care unintentionally reinforce that when they refer to me as ‘mum’ which makes me feel inferior in the relationship with them.
When I’m trying to establish a partnership in the best interests of my son’s care, there’s never time to find out who I really am and what skills, knowledge and experience I have.
Burnout – In the first few years of Harrison’s life, his medical condition was far more unstable and required constant supervision and interventions (therapies, feeding, medications, 24 hour oxygen support). This led me to burnout and collapse from exhaustion a couple of times. Whenever he was admitted to our children’s ward, I’d be asked who our social worker was. We’ve never been assigned one and we didn’t qualify for respite. Apparently, we appeared to be ‘coping’ and didn’t qualify for extra support schemes because we weren’t socially disadvantaged.
Close Relationship – Although this post sounds quite negative, caring for Harrison has given us an incredible bond because we spend so much time together. I’m extremely grateful for that and can't imagine what it would be like to not be with him all day, every day. He really is a joy to be around.
Harrison is our only child and as you may recall, he was diagnosed at the age of four monthswith a complex heart condition and 22q Deletion Syndrome. Although I was already caring for him as a mother, from the point of Harrison’s diagnosis that stepped up into becoming his main carer too.
Let's explore the roles that I personally do as a carer. Obviously, some of these do overlap with being a mother, however the extent to which I do these is more intense than if I was ‘just’ his mum.
For each carer, they will concentrate their efforts in different ways. The individuals they care for will have different needs and disabilities not just from each other but possibly over time too. A carer needs to be flexible and responsive.
These are my roles:
Once again, apologies for the tiny writing on the slide. The roles are all listed below with a brief explanation. I've taken them in a clockwise direction starting at the top with all the burgundy boxes first and then the purple ones at the bottom of the post.
Secretary - Keeping and organising appointments and records; dealing with doctors, nurses, the local authority (education and adult social care teams), filling forms, making holiday arrangements (an extremely complex process!) and ensuring all paperwork, insurances and travel adaptations are in place.
Finance manager – Dealing with benefits agencies, local authority finance team, sourcing special equipment and aids. A perfect example of this was today where Harrison had been randomly chosen for a telephone review of his Personal Independence Payment (PIP). This is a non-means tested benefit for individuals who have a long-term physical or mental health condition or disability. For those who have never seen a PIP assessment form, it's a forty page A4 document and in Harrison's case, I have to attach several additional pages of my own typed responses because there isn't enough room in the original form to fully answer the questions. The payment is awarded for various lengths of time so a claimant (or their representative) has to recomplete all of the paperwork from time to time, and supply any evidence requested, to continue receiving the benefit. As Harrison's appointee, I took the call today on his behalf.It lasted an hour and a half!
Nurse - Giving medicine (which over the years has included been taught to draw up and give long courses of intravenous antibiotics at home; tube feeding; managing delivery of oxygen therapy 24 hours a day for two and a half years); ordering and administering multiple medications several times a day; delivering therapy programmes at home - speech therapy, physiotherapy, occupational therapy; setting up, monitoring and maintaining medical equipment; generally being on constant alert for any deterioration in Harrison's wellbeing (he's not able to identify this for himself).
Chauffeur – Driving Harrison to activities and appointments.
Teacher and facilitator - Providing and supporting appropriate stimulating activities and learning opportunities, socialisation, mindfulness, creative activities, exercise and sometimes even doing things just for fun!
Personal assistant – Supporting Harrison with washing, grooming, dressing; accompanying him to all his appointments and social activities; helping him to plan and organise his day; constantly reassuring him and reminding him, whilst trying not to come across as overbearing or a nag!
Friend – Confidant and sounding board. I've always been honest with Harrison regarding his condition and life in general. This has given him the confidence to tell me about things that are worrying him. He doesn't always do this in a timely manner so I try to pick up on any emerging signs and start a conversation with him.
Housekeeper – Harrison doesn't have any practical skills. I've tried teaching him over the years but he doesn't retain the need to do daily tasks such as laundry, washing his dishes, clearing up after himself etc. He will do part of the task with direct reminders e.g. put his clothes in the washing basket and plate in the dishwasher but genuinely doesn't realise that his plastic drinking cups that he leaves next to the sink, need to be washed up.
I have to ensure that I cook appropriate meals for Harrison (he has difficulty eating some foods) which often means that he has a different meal to us, and I sometimes need to encourage him to eat.
Researcher - For advice; medical papers; social and educational activities; clubs; accessibility of places of interest; places to visit.
Now you could say, that as Harrison's mother I would be doing a lot of these things anyway, however my friends who have typically developing young adults the same age as Harrison no longer need to do a lot of these roles or have never needed to do them.
Over time, they've been able to take a step back and allow their young adult to take more responsibility for most areas of their life.
In our case, we can try and give Harrison some sense of independence with choices of activities and things like that but a change in circumstances throws everything off course. A recent example of this would be the COVID-19 pandemic.
Harrison was classified as clinically extremely vulnerable (CEV) which led to us shielding him for the past 800 days, so inevitably the independence Harrison did have has taken a step back. For example, pre pandemic he had a good routine. With activities going online during the first lockdown it was somewhat maintained and he was able to more or less organise himself with reminders and checks from me. As the country opened up again, and Harrison’s activities went back to face to face, the online classes ended and he hasn’t been able to settle into a new routine yet because he's still stuck at home.
As his main carer, I’m on call 24 hours a day although I can actually sleep at night because Harrison’s overnight medical equipment will alarm if he suddenly becomes unwell.
In addition to all the caring, I’m also:
A casual worker on an ‘as needs’ basis for a couple of hours per week doing an administrative role usually working from home. There’s no specific daily commitment required so I can fit the work around Harrison’s needs and other activities. A volunteer as the Chair of trustees for a UK charity. I'm now also a Peer Leader working on patient engagement projects nationally, locally, and at the Royal Brompton hospital. All but one of these projects are conducted via completely virtual meetings.
A wife. Although we’re in complete agreement that Harrison’s needs come first, I still need to consider our needs too. Whilst the three of us have been 100% ‘working’ from home over the past two and a half years, I’ve tried to be mindful of everyone’s needs and prepare all of the meals (no take aways or meals out). It's a real challenge to try and offer interesting and varied options day in and day out, especially when you don't enjoy cooking!
Harrison’s mother. It’s becoming increasingly difficult to balance his medical needs versus mental health needs. Individuals with 22q deletion syndrome have a higher incidence of mental health conditions than the general population. I’ll be covering this separately at a later date so I'm not going to go into too much detail here except to state that the mental health aspects of 22q deletion syndrome can range from anxiety all the way up to schizophrenia. When you know that there's a higher risk of a mental health condition developing, you have to more carefully consider the impact of decisions regarding generalised medical needs.
It’s safe to say that I’m fully occupied from the time I get up to when I go to bed!
Carers fulfil several different roles which I'll explore in my next post. The picture on the lefthand side of the image below highlights some of the generic aspects of caring:
Apologies for the tiny writing. Hopefully you can get the gist via the images if you're not able to enlarge the slide.
The first part of the definition for a carer is pretty self-explanatory - 'Cares for family or friends who have a disability, illness or who need support in later life.' I think it's safe to say that we'd all probably come up with something similar if asked to briefly describe what a carer does.
As I've just mentioned, I’ll go into more depth into what I actually do as a carer in my next post but the point that I really want to highlight here, is something that I've not actually seen in the definition of a carer before and that's the second part of the definition where it mentions ‘Needs support to manage a life of their own.’
I think you could look at this in two different ways. Clearly, the person being cared for needs the support to manage a life of their own however I’ve learned over the past 24 years that the carer needs support too. In my experience, that’s often forgotten and to be fair, the carer usually puts their needs right at the bottom of the pile and doesn't give them any regard because they consider them less important than those of the individual and other family members that they're caring for.
Depending where you live in the UK there are various support groups for carers. Sometimes the local communities set up their own groups which are either in person or online and some local authorities are mindful of carers needs and try to support them the best they can. However, in my experience, carers need to source this support themselves and it's not always easy to find.
In my personal experience, I joined various chat forums (pre social media!) for Harrison's genetic and cardiac conditions and used them for support rather than a specific group for carers. I think the important thing is, that carers are able to engage with individuals who understand what it's like through their own lived experience. If carers have social media accounts, it's far easier nowadays to get virtual support from all over the world. This can be invaluable when a carer is looking for a quick response. However, sometimes you just need a bit of in person support too and the simple act of making a carer a cuppa shouldn't be underestimated!
I know, I know, I keep promising to finish Harrison’s story to give you an overview of our lives for the past 24 years and yet again I'm remaking that promise to you! As these next five posts are in relation to National Carers Week I hope the by the time you've read about my caring role you'll understand why I haven't got round to finishing off the summary of his story. It is on my To Do List and I promise I will get there but I can't say when it will be. Please bear with me because it will help you to understand more about life with a complex genetic condition.
So, this is the first time that I’ve ever examined my role as a carer. When I tell our story, it’s usually about Harrison or specific aspects of his condition, and the impacts on him. Now that I’ve done it, I’m glad that I decided to share my perspective of caring.
Over the next few posts, I’m going to share the presentation that I gave in November 2021 during my participation in the Peer Leadership Development programme. We were allocated ten minutes each, so I only had time for an overview. This surprised me because I really didn't think that I'd be able to fill the time talking about being a carer. However, it turns out that I could have gone on for much longer as you'll realise if you wade through all these carer posts! I’ve taken one slide per post so that I can expand them out and add extra comments whilst still keeping things fairly brief (most of the time)! I've changed some of the original text so that it fits better in this blog. Some of the actual wording that accompanied my slides in the presentation, has already appeared in previous posts. Where this is the case, I'll reference the post title so that you can look back for the full detail, if you wish.
One last point to note before I get started - this is my account as one parent carer for one individual with a genetic condition. All carers will have their own perspective on their role which may be different to mine.
Here’s my introductory slide which highlights the topics for my following four posts:
The man juggling the hoops illustrates my life. Being a carer is just one aspect of it and I can’t explain how I spin so many hoops at once; it’s become a way of life for me. I can honestly say that I don't usually give it a second thought. I was talking to a friend the other day and it suddenly dawned on me the adjustments that we, as a family, automatically make to our daily lives to accommodate Harrison's needs due to his condition.
My message for other families in a similar position to us, is to do what works for you and your family. People may comment and offer their opinions or suggestions but unless they're in the same situation as you (and even if they are, the adjustments may not necessarily need to be the same) don't feel that you should justify or think that you're doing the wrong thing because your actions don't fit into the 'normal' expectations.
In the past I always tried to fit our family in with everyone else. Now I accept that we do sometimes need to do things differently which will have an impact on arrangements we make. Thankfully we have very understanding family and friends.
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):
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.
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.
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.
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 -
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)