Once again, I’m introducing a temporary break in Harrison’s story.
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This image popped up on my Facebook timeline
but I can't find it again to attribute the creator.
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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
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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.
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| 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:
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.
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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!)
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| The smiles are back and he's well on the road to post surgery recovery |
He left hospital three weeks later.
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| 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.
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