Saving Zali

by Lisa Venables

  Chapter 43

  Tuesday 15 September 2009, 136 days in hospital

  89 days in PICU

  6–8 weeks left

  The next day Zali had a bone marrow test called an aspirate to remove a sample of marrow from her bones to see what the disease was doing and if it had been affected by the chemo. Unlike a biopsy, an aspirate only takes marrow, not bone, by inserting a special needle into the bone and extracting the marrow. It was done in Za’s room, and the surgeons took advantage of her body being full of drugs so it didn’t take much to knock her out.

  I remember going home that night and falling into bed, feeling like I had been beaten up and thrown around a boxing ring. There was nothing left to give, but the disease just kept taking.

  That week Lachlan had two more panic attacks. He was losing his grip more often, he could sense something was up, but couldn’t face whatever it was. On Wednesday morning he came into PICU as Za was having the tape around her ventilation tube re-strapped so I went for a walk with him. He stopped in the corridor and started crying, his head held in his hands.

  I hugged his tall skinny frame. ‘What’s wrong, baby, what’s wrong?’ I asked.

  ‘It’s all fucked. Everything is shit and it’s fucked and it’s all just shit. I want to go home. I miss my friends. I miss my bike. I want you to come home. This is shit.’

  I agreed with him completely. ‘You are one-hundred per cent right, Lachie. This is absolutely fucked. I hate it. I don’t want to be here. I want to be at home with you. It’s shit, it’s fucked and it’s shit. As soon as we can go home we will. I won’t wait here one second longer.’

  We stood holding each other for a little while and he calmed down. It turns out that acknowledging his feelings was the best salve I could offer him. It was just as well, because it was all I could offer.

  Everyone around me gave their absolute best. Lachlan was holding on longer than he thought he could, Kala was trying to keep communication lines open between us and her, and us and other family members. Andrew came to the hospital during the day and took great care of our life outside the hospital. Lots of different doctors were researching the best ways to treat Zali, talking at conferences internationally, reaching out to experts, giving lectures locally and looking for an answer.

  Nurses watched her closely, ordered various blood products and briefed doctors constantly. RMH told us we could stay in their unit as long as we needed. My current command had helped me take unpaid leave, my previous command had done fundraising for us and even the bank had cut us a break on our mortgage. Everyone was giving us everything they could.

  I gave my time, my company, my sleep, my soul to Zali’s recovery, but at the end of the day it felt like all this effort was falling into a giant gaping hole. I think this was caused by my guilt that this disease was my fault.

  When Zali was first diagnosed I asked one of the doctors what caused LCH. She told me that the cause was unknown. It could be viral, environmental, genetic or something else.

  I had pushed Andrew to have Zali. I wanted to have another child very much. I wasn’t in terrific shape when I had her. I feared that poor diet had possibly led to this problem. We also lived on the coast in humid conditions. Could a mould spore have caused this? We had been pretty stressed at home. Had that caused it?

  Ultimately I had brought Zali into the world. Her health had been my responsibility. She was created from half my DNA and carried in my body. She may have had this disease when she came into the world. She was born with some weird eczema in her armpit. Had I done something wrong that had brought this down on my little girl? No amount of anyone’s help, company or medication could take away my dark fear and guilt.

  Looking back now, I can see that cancer is simply a horrible, horrible thing to happen to a completely undeserving person. Zali didn’t deserve this, but I didn’t either. I had done nothing wrong. I had only loved her. It just happened. At the time, though, telling me it wasn’t my fault wouldn’t have made any difference. She was mine. I was responsible for her. If she was suffering I would suffer too.

  Chapter 44

  Thursday 17 September 2009, 138 days in hospital

  81 days in PICU

  6-8 weeks left

  We had a pretty spectacular falling-out with the treasured Professor Kellie that hectic week. To this day, Professor Kellie is a man we respect highly and rely on to have the last say on Zali’s illness, symptoms, treatment and future health direction. He is an international expert in this obscure field. He was the one who told us how sick Zali was and he was the one who came up with an alternative when we had run out of choices.

  After telling us Zali was not recovering from LCH, Professor Kellie had organised the bone marrow aspirate to confirm what he suspected. If the disease had gone it would be a big relief for us. Zali was dealing with many other health problems, but if the disease wasn’t attacking her organs as they coped with other problems, she had a much better chance of getting through.

  Only three days after the aspirate Professor Kellie came to our PICU room at the end of the day before he finished work. Andrew and I had waited anxiously all afternoon for him. I was so hopeful I was almost unable to stop myself from clinging to him as he walked into the room. There was no other treatment left if this one hadn’t worked, and this one had been a long shot.

  ‘Well, I have some great news,’ he said. I held my breath. He smiled as broad a smile as any I had seen on him. He went on to explain that it had been a difficult decision to push on with chemotherapy while Zali was in a coma and there were certainly problems we were still dealing with but he was very pleased to tell us that the Langerhan’s cells they found in her bone marrow sample were at a level he felt could be managed. They didn’t appear to be haemophagocytic any more either, which meant they weren’t consuming other blood products or cells in the organs. He smiled a relieved grin at us.

  My stomach dropped like a plane falling out of the sky.

  ‘So the cells are still there?’ I croaked out.

  ‘Oh yes, but at much lower levels,’ he replied.

  The nurse’s eyes started to well up as I looked to her for guidance, my breathing fast and shallow. Andrew looked furious. I thought I was going to throw up. I turned my back on everyone in the room and faced the window. I started sobbing into my hands.

  Professor Kellie was confused. He thought this was a great result. Nobody in the room could explain to him why we were so upset so he left.

  I did not understand what Professor Kellie was saying about Zali’s status. I thought that if the cells were there it meant she was still sick. I thought that chemotherapy should have destroyed these cells, and when the bone marrow had grown back it wouldn’t produce these cells any more.

  But here was Professor Kellie smiling and happy that they were there. Was he some sort of lunatic? Was she still going to die, but we had just put it off a little? Would she always live with serious quality-of-life problems because these damn cells kept coming back? Would she still die but not the painful gruesome death previously predicted? Why was he so happy these cells had come back ‘within the normal range’?

  I told the doctors I didn’t want to speak to Professor Kellie should he want to come back. I was angry and upset and couldn’t face him without being ungracious. I knew he had taken a big risk, and I was grateful for that, but if I spoke with him now, it would not appear that way at all. Little Za Za still had LCH.

  The next morning the lovely, gentle Dr Luce Dalla Pozza, the director of the Oncology Unit, came and spoke with Andrew and me. He explained to us that Langerhan’s cells are normal white cells. They are supposed to be in the blood. They can’t be eradicated, and you wouldn’t want them to be because they perform important immune functions. Professor Kellie had hoped when he had ordered the chemotherapy that he would wipe out the old defective, overproducing ones, and the new ones produced by the new bone marrow would perform their normal function, thus finishing the disease. It appeared that this in
deed had occurred.

  Before the bone marrow aspirate they thought Zali had multisystem LCH with haemophagocytosis, calcification in the major arteries and major organ failure. There is zero survival rate for this. Death should have come quickly. But it hadn’t.

  Professor Kellie had performed a phenomenal task. He had achieved the impossible. He had attacked LCH with aggressive chemotherapy and it had worked on the disease. Zali’s cells were no longer overproducing, no longer attacking the rest of her body and were behaving normally within her body. The chance of beating such developed and extensive LCH was so minimal it didn’t have a full number. It almost couldn’t be measured. It wasn’t even a one in a million chance. It was a one in a Zali chance, exceptionally rare.

  LCH could come back throughout Zali’s life, but when a patient was diagnosed before two years old it was very hard to treat and survival was extremely low, so they had no real data on what happened in the long term. With her complications, there had been no chance. That’s why Professor Kellie was excited.

  I let out a long, deep sigh and smiled as the news washed over me, cleansing me, and calming my ache. Andrew breathed out, swore and started laughing.

  I started giggling. Andrew leant forward and shook Dr Della Pozza’s hand, pumping it vigorously. It seemed obvious now we were on the other side that of course it would have worked. Any chance, even the minimal one we had been given, meant that there was a chance Za could beat cancer, and she had. Dr Kellie had. We had busted through the thick wall that seemed an impossible obstacle to get through. My heart raced as the implications of beating cancer sunk into every cell in my body. The biggest fight Zali had was over. The hardest bit was done. I began to laugh and cry at the fact that this tiny chance to beat cancer had been given to Zali, to us. We were winners.

  I realised my mistake and agreed to see Professor Kellie whenever it was necessary. He came back the next day to visit us in PICU to explain what this new diagnosis meant. He said that the disease would no longer be the reason if Zali passed away. The complications she was still facing though—the major organ failure, the lack of blood cell recovery, the high heart rate—were very serious though. It was still unlikely, based on these problems, that she would live to Christmas. Also, as this was a new area of medical achievement, and LCH is very partial to returning, he didn’t know how long the effects of the chemo would last. There was the very real chance that at any time it could come back.

  The clock ticked on in my head, but we kept holding on. We just had to fix the high heart rate and wait for them to work out something for the blood counts. Those obstacles seemed very minor in comparison to beating rampant LCH.

  Zali had beat cancer. Right then she was cancer free, and it wasn’t going to kill her. That was all that mattered.

  Chapter 45

  Friday 18 September 2009, 139 days in hospital

  92 days in PICU

  6-8 weeks left

  Despite my renewed optimism, Zali’s high heart rate was still a big problem. It had been high for a long time now and none of the intervention that had taken place had been able to slow it down. The high heart rate was very detrimental to her body. Most significantly, she was losing a lot of weight because all the nutrients and calories that went into her via the TPN were being used up by her heart. I had been told that the best way to find out definitely what was wrong with Zali’s heart was for her to have a CAT scan, but that it could only be done in the Radiology Department. The machine was not transportable.

  Moving Zali out of her room was highly problematic and dangerous for her. She was only skin and bones, with tubes and catheters and machines all around her. It was difficult to move her life support machines, create a gap in her medications and keep her calm as she was moved. On top of this, Dr Anil would be going with us. I didn’t rate him much. He was her treating doctor the first night we had come in, and I think I simply associated our struggle with this doctor.

  That afternoon before the scan he gave Zali a bolus dose – an extra amount – of ketamine on top of her usual dose of fentanyl to make her sleepy for the trip so she wouldn’t be too upset and to make sure her heart rate didn’t go higher than the 165 beats per minute it was sitting at.

  It didn’t have the effect it should have had on her because she was already on so much pain relief from the fentanyl drip. In general, ketamine was not her friend, but it did have memory-loss qualities that were valuable. Instead of gently lulling her off to sleep it made her restless and groggy. Most disturbingly, it made her eyes roll outwards in opposite directions.

  As the small team started to roll the bed away from the wall, Zali’s blood pressure dropped, her breathing rate dropped and her heart rate went up to 200 beats per minute. Alarms started screaming and nurses started acting. Ventilation was increased, the bed was stopped and we waited until she returned to her normal rate. This happened three times before we got to the door of her room. As we all finally got to the exit to leave the ward I started crying. Dr Anil was shocked and worried by my tears. Dr Gilles, the doctor who had recommended that Zali go on dialysis on her first day in PICU, was there and he put his hand on my arm. Even though I towered over him I could feel the strength coming from his little body.

  I didn’t have any words as I looked at him with my sad crying eyes. He returned my gaze with his own sad, world-weary eyes and said, ‘It’s just too much, isn’t it.’ I nodded my head.

  He repeated, ‘It’s just too much.’

  Everyone waited for a few seconds and I pulled myself together. Dr Gilles put his hand on my back and said, ‘Come on, then,’ and we all went to radiography without any more delays.

  The CAT scan machine is enormous and very expensive. It is a big cream-coloured ring about two metres high and 75 centimetres thick. The patient lies on a long bed that moves through the hole in the middle as the machine produces X-rays and takes pictures of cross-sections through the body. The whole procedure takes two minutes.

  Normally the room is cleared and just the patient stays but Zali was distressed and coming out of the ketamine dose. I stayed with her, holding her hand and moving in and out of the ring with her, taking care not to get in the way of the photos they needed to take.

  I did wonder absent-mindedly if the X-rays could cause tumours. How ironic would that be? As the images were displayed in another room I could see through the window that the experts were surprised and pointing out details to the PICU doctors. I didn’t really care at this stage what they were talking about. I just wanted to get Za back into her room and have this over.

  When it was finished we returned quickly to the room and Zali was hooked up again. She calmed down immediately and went to sleep while I ducked down the hall to go to the bathroom.

  The results of the CAT scan were back when I returned from the toilet. They were displayed in vibrant multicolour on the computer screens in the nurses’ station. They were single photos but when scrolled through quickly it was possible to see what happened when the blood passed through the various organs.

  There was a lot of excitement about the results. All the doctors on duty and some of the more senior nurses were looking at them. Normally I’d be peeved that they had information they weren’t sharing with me, but today I just didn’t need the punches.

  I tried to slink past the station into Zali’s room next door but Dr Anil stopped me. He was very happy, as was Dr Elle. The doctors in this ward are normally very cautious because life is always in such a delicate balance.

  They told me that from the CAT scans they could see why Zali’s heart was failing, why they couldn’t get her off intubation and her lungs couldn’t inflate on their own. They invited me over to look at the pictures. As they scrolled through, all I could see were lots of blobs surrounded by psychedelic rainbow blobs and stark white and grey patches. The doctors said that the white patches all over her heart and lungs were calcium. On her heart it formed thick hard shields. Across her lungs there were long white ribbons and large hard patches
of calcium.

  Professor Kellie had suspected that the disease had been in her heart and lungs. It had never been recorded in these places before, but this was because toddlers usually didn’t live with such morbidity for so long. Zali had lived longer than predicted, with the result being that the disease had travelled further than ever recorded. When the disease had caused inflammation, the parathyroid gland had gone berserk and made calcium, which was dumped on the inflamed spots, in this case her organs.

  So now they had a reason why her heart rate was so high. The heart muscle was calcified, as hard as a rock in a lot of places. The left ventricle, which is supposed to pump blood throughout the body, had calcified like a big gobstopper lolly, and the lining of her heart had also calcified. The parts of her heart that hadn’t calcified were beating as fast as they possibly could to keep a machine that should be working on four chambers working on three. The muscle couldn’t contract to bring the blood in or to push it out. When the blood went through the lungs there wasn’t enough power to help them inflate, and they couldn’t expand because of the heavy ribbons of calcium.

  Her stomach had calcified, and there was subcutaneous calcification in her thigh muscles, in a bed sore on her back, and on the back of her skull. The only thing that didn’t have calcium, in fact, were her bones.

  I was relieved because the doctors were relieved. They told me they would get the cardiologist, Dr Roberts, to come and talk to us about what could be done. I assumed there was some sort of drug that would fix this. We had already had some very expensive heart drugs, and I imagined we could go down that path again now.

  Dr Roberts was a world specialist in his field and he came to Za’s room that afternoon because Zali needed him. It was really amazing and I was grateful for his immediate attention. I outlined what I had found out, but of course he already knew. He agreed that it was great we had found out what the problem was and that yes, it was very interesting.