The Procedures

Willow had three major surgeries during her life and one invasive procedure to insert a stent in a narrowed aorta. Of her 9 months of life, 7 were spent in hospital so needless to say most of my memories of Willow have hospital decor in the background, the sound of beeping monitors and the god awful smell of alcohol hand wash. HLHS is one of, if not the most severe form of cardiac deformity that babies are born with today. The explanation for its occurrence is that the heart struggles to form correctly in those first few weeks after conception, for reasons unknown. The affliction is random, it strikes the children of young parents and older ones, of all levels of healthiness, of all nationalities and socio-economic backgrounds. Until a couple of decades ago there was no procedure to prolong the life of HLHS babies, they would simply die within days or weeks of birth. American and English doctors have long since been performing the three open heart surgeries that can prolong the life of an affected child (usually until they need a heart transplant in their teens) and Australian doctors have been doing the surgeries for the last decade or so.
The three operations are called the Norwood, the Glenn and the Fontan. The Norwood is the first, usually performed at 5 days of age. The baby is put on bypass and it's body cooled, the chest is open and a shunt inserted into the heart to redirect the flow of blood to pump to the lungs. I will fail miserably trying to describe the inner workings of the heart, I did once understand it but many of the technical aspects of Willow's illness have long since faded from memory.
Willow was given IV fluids for those first 5 days, but no milk or nourishment of any kind. I spent many hours in front of a breast pump listening to the unnatural mechanical wheezing of the instrument attached to me where my daughter should be, but Willow couldn't have my milk until after the operation.
Willow survived the first operation, and we eventually became somehow accustomed the shocking sight of her hooked up to machines and sprouting drainage tubes and cannulas in every hand and foot. There was a feeling that one hurdle had been surmounted. We were one step closer to life.
Stage two, the Glenn, is usually performed at around three months of age. Willow had hers early because her cardiac function had deteriorated. The Glenn involves inserting a different shunt that the child can grow with, until the final operation- the Fontan- is performed at around 2-3 years of age. The Fontan normalises blood oxygen levels and relieves the 'blue' look of HLHS kids.
Willow had a brief complication with her Glenn operation. She came back with reduced cardiac output and a lot of pressure built up on the lungs. Her surgeon simply enlarged the shunt and the problem was fixed. Sounds all good and well and blissfully easy in hindsight but it was absolutely terrifying, one of those moments where a nurse says 'there's been some complications', or something to that effect, and you stagger into a room to see your child surrounded by what looks like every doctor in the hospital, smiling weakly at you in some attempt at reassurance. Anyway, they fixed it. By 'the' I mean Big D, Willow's incredible surgeon (his name isn't really Big D obviously, just as my partners name isn't really Bill, but I don't want to use real names in here, except for Willow's, because I intend this blog to be very honest, warts and all).
Willow's 3rd big procedure was an outwardly fairly simple process to insert a stent into a narrowing aorta to help the blood flow more freely. It was performed like an angiogram, a long wire inserted up through the groin and snaked into the heart. It didn't work and it made things a hell of a lot worse. Already suffering from arrythmia, Willow had three cardiac arrests during the procedure and took longer to recover than after either of her major surgeries. Her heart failed, her lungs struggled, her kidneys and liver switched off. She developed seizures and showed signs of brain damage. She was unable to use her left arm and needed physiotherapy for months to get it working again.
It is a testament to Willow's strength that she did recover, albeit very slowly. Bill and I were with her every night and day, nearly lost in our grief at times but hanging on by our promise to Willow to be with her through everything.
By the time she had a pace maker inserted two months later, Willow was very much on the mend, though still suffering AV block (the chambers of the heart were beating slowly and out of sync). She recovered from this operation too, now with an unnatural bulge where the pace maker sat under her skin. The instrument did improve her arrythmia for a time, and then ironically her heart was suddenly beating too fast. They didn't get a chance to fix this problem. Willow caught a bug in hospital, got a sniffle, and went back into heart failure within days. She was intubated and sedated but continued to decline, her lungs filled with fluid and her heart nearly doubled its normal size with swelling. She arrested twice and recovered, but the third attack was prolonged and fatal. Only the day before she had woken briefly to look around. I had looked directly into her blinking eyes and spoken to her with a smile, not knowing it would be the last time I'd see her awake. She died in my arms at 6:10 am on the 8th of January 2010.
We met many HLHS bubs in hospital, some of them survived and others didn't. The process of temporarily 'fixing' these babies is always the same, but they each respond differently to treatment. That is a very clinical way of putting it but now you know.