A CHD Journey for one parent #CHDVOICE #HeartMonth

We are very grateful that as a Charity we get to be there to support the whole family, when they may need it most. Here is the journey of one parent and her family.

for abbreviations see the GLOSSARY* at the end

SO, Heart week is upon us & as a couple of charities we are members of have asked for stories, I thought I’d put pen to paper & share ours. I’m “Mum”, by the way & this is my version, from my perspective.

My eldest son was born at 05.05 on the 5th of the 5th after a remarkably easy pregnancy & birth. All the 5’s! I initially thought it was a lucky sign. But, at a mere 9 hours old he aspirated following a feed & stopped breathing. That, was nearly 25 years ago & yet I remember it as clearly as if it were yesterday: watching my baby turn blue & rigid in my arms… that was the first time this first time Mum felt panic, the indescribable panic that only one of “us” truly understands.

 

I’m going to have to skip bits & fast forward others as a 25 year old story IS going to be long… But, our little community hospital did manage to resuscitate him & we were transferred to our local, big hospital, whereupon a 1st examination they could find nothing wrong & even suggested that both G & I would be discharged the following day. However, call it mothers instinct?- but, something one of the midwives had said to me was niggling away & so we asked for a 2nd opinion. George was diagnosed with a Hypoplastic Left Heart Syndrome the following day. I remember seeing my baby in NICU alongside lots of premmies, with other mothers trying to comfort me. Saying “he’ll be ok. Look at the size of him. He’s the biggest, healthiest looking baby in here”. He wasn’t.

We were given 3 options. To remain at RUH, Bath & receive palliative care so that he could pass peacefully (probably, literally within a few short days), take him home with a support package in place so that he could pass peacefully at home OR take the last available ITU bed in a centre of excellence: Birmingham Childrens Hospital, one of only 2 hospitals in the whole of the UK that could offer life saving open heart surgeries. As we know, it’s a 3-stage procedure.

We travelled through the night following the ambulance carrying our son, in torrential rain. But it was tears that made the drive so incredibly difficult.

At a mere 2 days old, already baptised our son underwent his Norwood. This wasn’t entirely successful, although it most definitely did save our sons life & bought us time for Stage 1 to be revised. It was here, in Birmingham, June 1997 that our CLN introduced us to Little (Left) Heart Matters. Still within its infancy, the charity was very small, but had a familial feel. Most family’s knew each other.

At some point during this 4 month admission I was told that my son was within the first 100 babies in the UK to undergo this particular surgery (he sits somewhere in the 50 or 60’s) & it really does frustrate me that I was once told the exact number, but have since forgotten something so significant.

 

We were actually asked to return to the ward during that first LHM open day we attended – they took place within the hospital grounds, back then. G was desaturating. And a night or so later as he continued to sporadically de-sat I remember the staff on “PBG” advising me that they’d requested a bed on ITU. The radio was playing softly in the background & I spent the night sat on a plastic chair with my son. Each time I held him, his sats picked up enough – so, I held him listening to the powerful lyrics of “Every Breathe You Take” & “In the Name of Love”…

 

George, ultimately sailed through his revision & in a matter of weeks was discharged back to Bath, to establish “feeding”. He was already a pro at pulling out his NG tube & after pulling it out on his first day back at the RUH, it was never replaced.

 

G was a poorly baby, multiple admissions for URTI, but it was at 11 months of age we were back at BCH for a routine cardiac catheter to determine exactly when he would need his Stage 2 / Glenn, or CP shunt as we called it back then. Only to be told that it had now become an emergency & he had already been added to “The List” & was listed 2nd for the following day.

Nil by mouth & only around an hour from going down – it was dramatically called off. George was neutropenic & they didn’t know why. The next few months we’re traumatic. Too poorly for surgery or to go home & so we celebrated Georges very first birthday at Ladywood & moved as part of the furniture from the Old Hospital to the new site at Steel house Lane. Tests were invasive and included a bone marrow aspiration but the night of my birthday, June 1998 George deteriorated again, suffering rygors & desaturation episodes. A plan had to be put into place for the surgery his little body so desperately needed. GCSF treatment (intramuscular injections to boost his white cell count) commenced.

 

At this time, it was primarily G & I sharing a room with baby A & her mother on the ward. And I really do feel the need to mention this family. Bonds are made during these hospital admissions & a friendship blossomed. So, when surgery day arrived: G was 1st on the list & A was 2nd. I actually left ITU that night. G safe was his Dad watching over him, I spent a rare night in our room within the Parents Block.

Upon entering ITU the following morning my eyes immediately were drawn to an empty bed space. Baby A had not survived the night. This part is not my story, but helping her mother dress her in the “Going Home” clothes that we’d jointly chose for our respective children, to this day remains one of the most difficult & poignant things I’ve ever done…

 

Back on the ward, my mothers instinct kicked in again. Something was majorly wrong with my boy. I could feel it. I knew he was in unimaginable pain & couldn’t even cry. I remember it being a Sunday. And initially being told it was quite possibly trapped wind, which can be excruciating – but, I believed it to be more. Can you imagine my relief when our consultant happened to be “just passing” & upon hearing G had stepped down from ITU to HDU, came & checked in on us – & took my concerns incredibly seriously. G was diagnosed with NEC (necrotising enterocolitis), something not commonly seen in babies his age, but, left untreated could result in septicaemia & even death. We started 2 weeks of “suck & drip”, to give his gut a rest & hope that he would recover. But, his wound became infected too. He was taken to the treatment room daily for it to be cleaned & packed whilst we anxiously awaited an ITU bed to become available. He needed to go back to surgery for it to be debrided & a plan was made to set up a kind of irrigation system to try & flush out the infection. Those few days waiting upon a bed were agonising & when the call came our Surgeon wasn’t on site. One of Birminghams amazing cardiac consultants actually performed the surgery with the surgeon on telephone link. Video links were unheard of back then.

 

Yet again, BCH saved Gs life. G fought. He got knocked down, but got back up again. He won round 2.

 

With Stage 2 behind us, Stage 3 – The Fontan procedure was no certainty. His Pulmonary Vascular Resistance (PVR) was too high. Significantly so. His was reading 23 & it needed to be around 12. We were called into what we had nicknamed “The Bad News Room”, yet again to be advised that transplantation was not an option. It hadn’t been carried out on anyone with his particular chromosome defect before (DiGeorge Syndrome/ 22q11). But, not giving up, George embarked on a new drug treatment, a lot of hope & faith & positivity. We got our miracle.

For once, our little trooper sailed through his Fontan in just under 3 weeks with just a couple of little “blips!” What a hero!

 

And this is where, I think “I” got a little complacent…

Heart wise, G did really well. Beyond expectations, if I’m honest. He enjoyed swimming, horse riding, could cycle & run & walk. Walk! This lad of mine could easily join us on hikes up & down all over Salisbury Plain. Five miles and plus was regularly achieved.

But, His 22q11 diagnosis soon overtook the list of challenges my young man has grown up with & faced. I know behavioural issues are not uncommon in teenage years, but Gs were accentuated & fuelled by fear, anger & anxiety.

And very sadly by 2019 he found himself or ourselves in the middle of a mental health crisis. As a mother, this was on a totally different scale of scarey – but I called upon help from the relevant medical teams. I can’t & wont comment on this, protecting my sons confidentiality. Those that know, know. And complaints / investigations are under way.

Fast track to May 2020, when our world began to catastrophically tumble down. Middle of lockdown 1 & G was hospitalised for a spontaneous bleed (from a varicose vein). He was taken to our local hospital, stabilised & discharged. Problem not solved, it happened again & was taken to a different, local hospital, treated & discharged. The underlying health issues hadn’t been addressed & following a phone call to our GUCH team, G was seen at a HOT clinic & admitted immediately. Absolutely overloaded with fluid. And as an adult, services are very different to those as a child.

We were introduced to Xander from Youth@Heart during this admission & feel extremely fortunate that our paths crossed & that my whole family receive support from this little charity, based in the SW.

It was during August 2020 our Team revealed the true extent of Gs non compliance with taking his meds or attending appointments – dating back to 2018. My amazing & beautiful son, is now in irreversible heart & liver failure. We’ve been assessed (it took 4 attempts, due to Covid, to spend a week of investigations at the Freemans, Newcastle), the day before Gs 24th birthday we were tragically given the news that it’s simply not viable.

We’ve experienced all kinds of difficulties in the last few years & I think the scariest was September 2020: blue lighted back to Bristol, I sat in a different “bad news room” & was prepped that my son was highly unlikely to survive the night, with creatinine levels just a few digits short of 2500. Yes, you read that right. Bristol, have never given up & neither has my son. They made a plan to put him on emergency dialysis & it worked.

 

This last bit, is the hardest bit. For my incredible & stoic trailblazer It’s all about quality & not quantity of life now. It’s a cliché, I know: Live for today, tomorrow isn’t promised. But, that’s exactly what we strive to do….

 

I saw a “thingy” on social media, just last week: “Would you trade your own life, to save your child’s?”. In a heartbeat, if only I could.

You see, remember that night back on PBG, June 1997? Radio softly playing in tune to machines bleeping & alarming in the darkest hours of night… Echoes still hauntingly fill me head & take me right back to that night & day 1 where the Cutting Crew cut open my own heart when I hear “I just died in your arms tonight”. You already did. Once. And I don’t think I’ll ever be ready to go there again my son. So, with a “doof doof doof” that resembles the Soap-operatic drama of a life we’ve led. I’ll leave it with, the rest is left unspoken…

I write remembering & honouring both Baby A & Noodle. Forever young, forever in our hearts ❤️‍

#CHDVoice #CHDAwareness

For more information on what support we can offer or to get in touch – please contact Xander
ACHD Youth Worker on 07832 668907 or email xander@youthatheart.co.uk
facebook.com/xander.heart.18

For general enquiries please contact us at contactus@youthatheart.co.uk

Norwood

Part 1 of 3 for Hypoplastic Left Heart Syndrome (HLHS) patients and some other CHD conditions too!

 

Build a new, larger aorta. The bottom part of the pulmonary artery (which normally goes from the right ventricle to the lungs) is joined with the aorta. This new, bigger aorta now goes from the right ventricle to the body. (The Aorta is the systemic blood vessel)

 

Create a shunt (path) to get blood to the lungs. Because the pulmonary artery now goes from the right ventricle to the body, a shunt is needed to take blood to the lungs. The surgeon will use one of these shunts:

a Blaylock-Taussig-Thomas shunt, which moves blood from (and through) the aorta to the lungs.

a Sano shunt, which moves blood from the right ventricle to the pulmonary artery. From the pulmonary artery, the blood goes into the lungs.

 

Close the patent ductus arteriosus (PDA). Until now, the baby needed the PDA to stay open so blood could flow from the right ventricle to the body. Now that the right ventricle can pump more blood through the new, bigger aorta, the PDA isn’t needed and can be closed.

 

Make the atrial septal defect (ASD) bigger. This lets more blood with oxygen get back to the right ventricle so it can be pumped out to the body.

 

(Words from here – https://kidshealth.org/en/parents/norwood.html)

 

Desaturating

Blood Oxygen levels decreasing

 

 

NG tube

Nasogastric Tube…. This is a tube up the nose and down into the stomach. Patients are then fed “food” through it. Its more like babyish mushy porridge like food.

 

Stage 2 / Glenn, or CP shunt

Part 2 of 3.

During the Glenn procedure, the surgeon disconnects the superior vena cava (SVC) from the heart and connects it to the pulmonary artery. Now the blood from the upper part of the body flows directly into the pulmonary artery. The pulmonary artery takes the blood to the lungs.

 

If the baby had the Norwood procedure, the surgeon will remove the shunt that was placed then.

 

Words – https://kidshealth.org/en/parents/glenn.html

(The SVC brings blood from the head and neck back into the heart)

 

HDU

High Dependency Unit sometimes confused with Cardiac Intensive Care Unit (CICU)

 

Fontan procedure

Part 3 of 3.

Disconnects the inferior vena cava (IVC) from the heart and connects it to the pulmonary artery using a conduit (tube).

Makes a small hole between the conduit and the right atrium. This hole (or fenestration) lets some blood still flow back to the heart. It prevents too much blood from flowing to the lungs right away so they have time to adjust. Doctors can close the fenestration later by doing a cardiac catheterization procedure.

 

Words – https://kidshealth.org/en/parents/fontan.html

 

(The IVC brings blood from the lower body to the heart)

 

GUCH team

Grown Up Congenital Heart… an old term used to describe our patient group. Now its ACHD

 

 

These are from Jo

 

Creatinine levels

Measure of kidney function, G was in kidney failure at those levels due to severe dehydration & possibly underlying infection

 

Neutropenic

zero & very low neutrophil count (white cells that fight infection) – hence too dangerous to perform surgery & probably added to his wound infection

 

Debrided

removal of all infected tissue. (It was deep & if it reached his heart, we were warned he would die)

 

PBG

It was the official name of the baby cardiac ward at Ladywood. We nicknamed it the Parent & Baby Group

 

URTI

Upper Respiratory Tract Infection (bronchitis, bronchiolitis)