Saving Zali

by Lisa Venables

  There was a risk that a piece of calcium could break off like a shard of ice, and if that happened, wherever it lodged would cause an immediate painless death for Zali. He had examined the way the calcium looked in the scans, though, and thought it very unlikely that this would happen.

  Surprisingly, nobody interrupted him or talked over him, despite it being obvious that an earnest discussion had just taken place and many opinions had been offered. I scanned the room for resistance, but they were silent.

  We were completely gobsmacked. My heart was racing and my palms were sweaty. I asked him how quickly this could be done as we had no time left. We were on borrowed time. I thought I was going to choke on the question as I squeezed it out in a squeak. I wanted to impress upon him the urgency, but I didn’t want to cry in front of all these doctors – though they expected it of me, I did still have some dignity left. He said the pamidronate was in the hospital, and he could get it to us that day. It would take four hours to go through Zali and it would be a regular infusion until he saw a result. He could start as soon as we left the meeting. I was impressed that he thought we would have enough time to make this a ‘regular’ infusion. I loved his confidence and I was immediately sold on his solution.

  As soon as I agreed, Dr Y was the first to talk. He told me there was no guarantee that when the calcium melted off Zali would survive. They had no idea what the organs looked like under the calcium, and from his understanding they had been very badly attacked by the Langerhan’s cells before they had been calcified. Once the calcium wore off, they could very well fail again and it would be unlikely that they could revive Zali.

  Professor Kellie was quiet, and said that although there was no sign of the disease, Zali had indeed been very unwell from the side effects and there was just no knowing what condition she would be in after this treatment. The side effects had been very serious and the long-term implications could not yet be judged. Removing the calcium to calm the heart and return nutrients and blood to her body did not guarantee that Zali would get better. There was no way to tell what damage had been done. Also LCH has a tendency to come back, and Za’s LCH had spread extensively throughout her body. Almost all the doctors at the table warned us that there was still a risk, and that Zali was still very sick and fragile. It was the first time this had been tried and there was no guarantee it would work.

  It didn’t matter to me. There was only one step forward we could take, and that was this solution, so that was the direction we would go. Dr Munns was calm and clear and absolutely certain. His demeanour seemed to suggest that if someone had replied to him earlier this could have been fixed much sooner. He was a genius confused at the limitations of the mere mortals around him.

  He repeated clearly that this drug would fix the problem of the calcium in Zali’s heart and arteries, and the hyperactive parathyroid. The PICU doctors were in support of Dr Munns and didn’t say whatever everyone else had been arguing about but not sharing with us. They supported him, and he was supporting us. Dr Kellie’s support was cautious but he was willing to try.

  I ignored everybody else and spoke with Dr Munns. I told him that I wanted to go ahead with the treatment, and asked if we could start immediately, like right now, and started to stand up to leave. Dr Munns got up with me. The pamidronate was in the fridge in the PICU. He was going to get it and supervise the nurse on its infusion.

  Of course we didn’t know what problems would still exist, but surely if her heart could beat long, strong beats she would be using less energy which might allow her to put on some weight. Maybe this would make up some ground and give us some more time with our baby. Even if a chunk of calcium broke off, the effect would be instant. There would be no grappling fights for breath or against pain. It would be an instant, peaceful death.

  We went back into the room, I felt like I was in a trance. Zali lay on the bed, sleeping and pale, her skin lying lightly over twig-like bones. The machine breathed for her, my own heart beat for her.

  Dr Munns came in with the pamidronate and began to organise its infusion into Zali. It was a bit fiddly because there wasn’t a four-hour gap where something new could be put in, but the nurses worked it out as they always did. If this bought us time, then there would be time for the other missed drugs to be put back in.

  At 5 pm it started. It took four hours to go into Za. I waited until 11 pm that night but nothing had happened. Nothing had changed by the time I left but at least it looked like a shard of calcium wasn’t going to kill her immediately, and she hadn’t died that day – another big plus. I promised myself that I would come back in at 6 am no matter how tired I was, in case it hadn’t worked and tomorrow would be the last day we had with her. I wanted as much time as I could fit in.

  Throughout the day her heart rate had been 185 beats per minute. As I tiptoed out of the room, it was 160 beats per minute because she was sleeping. I felt a bit confused, a bit disheartened but still hopeful. Dr Munns had been so certain. Zali’s disease and side effects were certainly unpredictable, but surely that went both ways. Was is possible to hope that we could swing another unlikely miracle our way? That we could be the first to beat this calcified ventricle? I was disappointed it hadn’t worked immediately, because immediately was the only time frame I had left. The thought of waiting seemed such a big ask. How far could we extend our borrowed time? When would God ask for payment? Would she even make it to the next day? I desperately hoped that Dr Munns was going to be a man of his word. I went home, had dinner and went to sleep.

  Chapter 51

  Saturday 26 September 2009, 147 days in hospital

  100 days in PICU

  Two days past the predicted last day

  The day of Dr Munns’ revelation had taken more out of me than I thought. I slept through my alarm the next morning and woke in a panic at 7.15 am. I threw on some clothes and raced into the hospital, shouting to Lach as I left that he could come in if he wanted to or just hang out at home, to just ask Kala to help with either.

  My heart was in my throat as I entered the hospital. I raced up the stairs to PICU and ran to Zali’s room. As I ran I looked at the nurses going about their business in other rooms and even caught the PICU director’s eye, but I couldn’t tell one way or another what had happened overnight. Nobody was giving me any indication of what had happened. In the past that had meant bad news, was I racing to find out this second request for a miracle had not been granted? Was I making a fool of myself to place my hope in Dr Munns? I didn’t know if I was strong enough to enter the room if it was bad news, especially if Zali had passed away. I wanted to both run away and run towards the answer.

  I ran heavily to the glass door and almost bashed into it as I didn’t give myself enough time to open it before I pushed in. As I dragged the heavy door open, dreading the seconds slipping by that brought me closer to the answer, I looked up and saw Mercy at the computer. She gave me an enormous smile. I caught my breath as I looked at Za’s monitors and saw that her heart rate had dropped. After months of it climbing upwards it had dropped. It was between 150 and 155 beats per minute. Ten beats per minute less after the deliberate intervention to make it so. It was displayed loud and proud in bright colours on the monitor next to Zali. A wave of relief rolled over me as I expelled the breath I’d been holding since the infusion went in yesterday, and I let go of a flood of tears and laughter and shouted out, ‘Oh my god it worked!’

  Mercy told me to wash my hands then put her arms around me in a tight hug while she closed the door firmly behind me. The heavy calcium that had weighed on Zali’s heart was moving, and I felt so light I could have floated away. I wanted to cheer and whoop while bawling my eyes out. I laughed and laughed as Zali slept and slept. Andrew came to the door and I could see on his face that he was worried my tears were for bad news. I yanked the door open and shouted out, ‘It worked!’ laughing as he grabbed me and hugged me.

  Over the next couple of hours her heart rate dropped even lower. We were big news in PICU, t
hen throughout the hospital. Doctor after doctor came to see these results for themselves, some we remembered, some we had never met but who had been working to help Zali anyway. Each one was overjoyed at Zali’s progress.

  She was still extremely skinny, extremely weak, frail and tired, but she was here, and her heart rate was slower. That morning Dr Munns came with his offsider as she received another infusion of pamidronate. I was a bit surprised at his aggressive pursuit of the calcium – he didn’t look like the type to pursue anything aggressively but he was pushing the pamidronate into her as often as he could, which was twice a day, and I was very grateful for his tenacity.

  He was pleased that I was so pleased, but again a little surprised, because he had known all along it would work. His offsider couldn’t stop grinning as I laughed and cried and thanked him. I wanted to hug him but I think he would have crumbled with embarrassment if I had. Andrew shook his hand as he laughed and cried too and thanked him over and over. What I, and everyone else was calling a miracle, Dr Munns was calling obvious.

  Za got another infusion of pamidronate that afternoon, and as large areas of calcium were melting away quickly, even over that day, the results were almost immediate. Her lungs were making a fast comeback and her ventilation support was dropped a little that day. Za seemed the tiniest bit more alert and a lot further away from death.

  By the end of that day she had received another dose of the drug – they were really pushing it into her. The following day the result was even better, and her counts for phosphorous, magnesium and calcium were all within normal ranges whereas only days before she had been receiving magnesium in her IV drip. The calcium change meant the parathyroid gland wasn’t freaking out any more and dumping calcium on the heart.

  As each organ and artery was slowly uncovered, dug up from its long slumber under calcium, it began to perform its job perfectly. There appeared to be no permanent damage done to anything – it was all fine. The risk that had most worried the majority of the doctors had not eventuated at all. The magnitude of coming through something like this unscathed was phenomenal. I was aware that doctors were worried about the possibility that things might look like they were going well when they weren’t. Despite everything, despite the craziness of the past ninety-nine days in PICU and 145 days in hospital, Zali’s body was falling back into synchronisation.

  I was blown away by the perfection of the body to do as it should. These organs and arteries behaved as if they had been frozen mid-motion and now, as the calcium thawed, they continued as if nothing had ever been wrong. I was very proud of each and every organ and artery in her body and so overwhelmingly grateful firstly to Professor Kellie for taking such a risk and pushing the second protocol of chemo through Za, and Dr Munns for healing her heart. They valued Zali’s life as much as we did and had taken care of her when I hadn’t been able to fix the problem on my own. They had carried us through this difficult terrain and now we were moving safely to the other side.

  Chapter 52

  Sunday 27 September 2009, 148 days in hospital

  101 days in PICU

  By Sunday Za’s blood test results began to be so different from previous tests they could have been mistaken for those of a different person. Zali started to smile again, and you couldn’t wipe the enormous smile off my face. The ventilation was starting to be turned right down as her lungs were able to shake off their heavy chains and start to flex again. It may sound silly to those who do it without thinking, but Zali breathing a little bit on her own was an incredible achievement and I bragged about it to nurses who came to visit.

  As calcium went into her bones, her bone marrow grew back very quickly and the amount of blood in her body increased, which made her feel more alert, gave nutrition to her recovering organs and improved her immunity.

  Later that day we had a nurse we hadn’t had before. He was a young Asian who didn’t have oncology experience. We asked him excitedly what her neutrophil count was as we had every day. A child sick with LCH does not have many neutrophils. For the whole time Za had been in PICU, her neutrophil count had been zero.

  He said, ‘There’s not much to report, I’m sorry.’

  ‘Not much?’ we asked. ‘So there was something?’

  ‘Yeah,’ he replied without looking up from his computer. ‘Neutrophils are 0.2.’

  The cheering Andrew and I let out was deafening. We scared Zali and the nurse as we jumped up with excitement.

  The histiocytes, the white blood cells that in Za’s LCH had been produced in huge quantities and consumed her other blood cells, weren’t consuming her blood any more and her IgG (and therefore her immunity) was improving. Her blood counts were quickly surging forward and we dared to imagine them being in the normal range, which had been inconceivable just ten days before. It seemed like a glut to have enough blood to take for a blood test. The supply had returned; the drought was broken.

  I was awestruck and grateful for Zali’s ability to recover and on the greatest high I had ever felt. The relief that washed through my body was like the opiate Za was on. I was calm and certain for the first time in a long time.

  Each day was like a new surprise party full of cheering and congratulations and joy fuelled by regular infusions of pamidronate. The first day everyone was celebrating because the heart rate was dropping and Zali got four hours’ sleep at night as well as sleep during the day. It made a massive difference to her ability to cope and she was starting to smile. Each day her heart rate dropped further and further, and even though it was only by a few beats, as the very thick calcium took some time to melt off, it made the world of difference. As the muscle got stronger it would be able to shake off the calcium as well as have it moved off by the pamidronate.

  Za’s chest X-rays showed her lungs were no longer one giant fuzzy white cloud of calcium and fluid. Calcium only covered half of her lungs and it was possible to see their outline clearly. Her lungs were starting to do their own work and needed less ventilation support, and she started to be able to breathe on her own because they weren’t holding fluid any more either. The heart was pumping the blood vessels in the lungs full of blood so they weren’t wallowing in water.

  The same afternoon the nurse had told us Za’s amazing neutrophil count, her ventilation was turned down to minimum levels as the doctors raised the idea of removing it altogether. We had tried twice before with disastrous results and I was really nervous. I was reassured that she could have a CPAP machine (an air pump with a mask) to provide some support if the lungs were still weak but that this was definitely going ahead.

  The next day, Monday 28 September, the PICU doctors decided it was time for Zali to breathe on her own. I’m glad they made the decision, because it wasn’t one I could have made. I was too scared she couldn’t do it. I didn’t know if we could rely on Zali’s body to do the right thing when it was tested. Until now, she had been showing off. I wondered if she really had what it took under pressure.

  She wasn’t given any bolus doses of fentanyl or ketamine. She had developed resistance to their effects so there was no point. She was left conscious and alert as they extubated.

  Nurses she trusted the most pinned her down as tape was removed from her face. It must have hurt such delicate skin. I stood next to her and soothed her as best I could. I could hear her crying in my mind as I waited. Under the direction of Dr Elle, the nurse began to pull the tube out as we stood at the end of the bed. It slithered up slowly out of her lungs, up her throat and out her nose.

  I heard her cry for the first time in 100 days as her lungs inflated again on their own like a newborn at birth. It was croaky and quiet and weak but it was her. She was upset and protesting and it was the best noise I had ever heard. Andrew and I raced to her side to reassure her. We could touch her face again and stroke her cheek to soothe her. I kissed her soft cheek and forehead and she cried and watched us. She could see us clearly again, feel our breath on her face, turn her head unobstructed towards us. She was scared and uncert
ain but we kept telling her how proud we were of her, that it was all right and she was okay as we cuddled her less awkwardly than before because there was nothing to squash that would take her breath away. I could place my heart near her heart without hurting her. I put my cheek against hers and told her, ‘I love you so much, and I am very proud of you.’

  Her face looked exactly as I had been imagining it this whole time. Of course her cheeks were sunken, and the circles under her eyes were dark, but her skin colour was good – the normal flushed pink of toddler skin. She had red strips across her face where the tape had been attached and some chaffed parts where the skin had pulled, but her eyelashes were growing back already, her lips were red and plump and soft, and she could move her head whenever she liked. The freedom was incredible. She kept touching our faces, and I stroked her cheeks in return.

  Over the next hour we watched her oxygen levels carefully on the monitor. We toyed with the CPAP machine during the afternoon as she slept, giving her small blasts of oxygen directed towards her nose, but she didn’t like it very much, and didn’t really need it. The ribs around her lungs were weak and thin, and the muscles holding them were wasted, but her lungs inflated fully and deflated on their own, with no need of assistance. They were as strong as those of any Olympic athlete.

  I knew our days in PICU were numbered after that but I was scared to leave. How could I go ahead into the world without all this support? These people had fought all my battles with and for me. They had buffered me when I felt like I would break. They had supported all of us and taken our load for us when we couldn’t carry any more. How could I go into the world now that I was soft and fragile and vulnerable? How would I know how to take care of Zali without them?