A bunch of hardy souls are dusting off their walking shoes for a sponsored walk in aid of St John's Ward and Ronald McDonald House. These brave folk will be setting out from Bennett's Bar in Warrenpoint at 2pm on Sunday 24th Feb and will be trudging up to Newry and back.
There aren't words to describe the thanks we owe to St John's and Ronald McDonald House, so please help by giving LOTS OF MONEY. Just click on the Donate button on the right to pay securely with PayPal.
Just a quick note to say that we're all back home now! We were able to escape last Friday and have slowly begun to get some kind of normality back in to our lives. I can't thank St John's Ward and Ronald McDonald House enough for the support they gave us during the 105 days of our stay, having to look after ourselves will come as a shock. Also, the Tucker family sends a huge thanks for all the words of support, presents and letters we've received. In particular, thanks for all the Christmas presents Ciara received (where are we going to put them!).
Ciara's doing well and is in very good form, getting home was a great tonic for her. She's been eating well and that and the NG feed have got her back on her feet and looking fantastic. She still has a big belly from the VOD and we don't really know how long that's going to take to get sorted out. The MIBG results showed that there were some neuroblastoma cells dotted around, and in particular there were some near the kidney. We don't know if these are active or progressing and we probably won't ever know but for the time being at least, we're not seeing anything from the other results indicating neuroblastoma activity. Next week I think, Ciara will have another CT scan concentrating on the Kidney area to see if they can map a route for radiotherapy.
Today, Ciara wants to write her letter to Santa and put up the Christmas decorations. Only eleven months too early!
Well it's now been a few weeks since I last posted, probably the longest break since I started this but I needed a rest. No excuses now so it's time to update y'all on what's been going on since the start of the year. Since Christmas, Ciara's health started going downhill and she's only just started climbing out of the slump. She had an increase in vomiting which ended up with her not being able to keep a thing down, including oral medication. Basically her gut stopped working, she had a nasty session of pooing blood and then stopped going altogether. There's a few things that could have been behind this, gastritis and morphine being two causes, neuroblastoma another.
The days and weeks following Christmas were a bit scary, the threat of neuroblastoma was hanging around and it went to the top of list of possible reasons causing liver and gut problems. Ciara's temperature was spiking and urinary catecholamines test showed that while most of her levels were normal, there was a slightly higher than normal level on one of the counts. This was beginning to show a picture of returning neuroblastoma. On quizzing the consultant regarding the spikes, he did think that neuroblastoma was a probable cause.
After about week 1 or 2 of 2008, I was getting confused as to what we were treating; there seemed so much going on. What were we fixing today? Was it VOD? Gastritis? Malnutrition? Oh, and what was that other thing? That was it - the cancer. Priorities kept changing from day to day. With the liver condition (VOD) not resolving, a plan had to be made to include more aggressive treatment. A multi-discipline team came up with some kind of plan for going forward and it was looking likely that Ciara was going to need liver surgery to get on top of the disease. However, before we put her through this, we had the cruel task of finding out whether it was going to be worth it, or whether we were going to be sent home with a morphine driver.
So last week we started the full work-up of finding out Ciara's cancer position. First off there was another urine test, which would take a couple of days to come through. Then an ultrasound, followed by a CT scan, then bone marrow aspirates and a biopsy. Tests end this week with an MIBG (injection of radioactive isotope to show cancer cells under a scan). But before this, we needed to build her weight and strength up.
Her recent sickness resulted in Ciara's weight plummeting, with her body digesting itself just to survive. Her nasal gastric tube had to be removed a couple of weeks ago as all the vomiting had blocked it up. As she was pooling food only to throw it up again a few hours later it wasn't doing any good anyway. A strange thing happened as soon as it was taken out: she started talking about food. A new one was put back in the following day but as an experiment we removed that one too to see how she would get on feeding herself. She did very well, and managed cornflakes, cookies, the odd pancake, even some sausage and chips. She was also started something called TPN which is intravenous feeding.
Ciara had her bone marrow aspirates and biopsy under general anaesthetic last Wednesday. Just after she went under GA, she vomited. That night she was under very close watch and was pumped with antibiotics. She was extremely groggy and seemed very poorly, much more than is normal for a small GA like this. The risk of a lung infection from the vomiting was very high. Her heart rate went very high, she was peeking at over 40 degrees and her oxygen saturation was very low. We had an oxygen supply close to her pillow to help this out. I think the night would have been more scary if the doctors had told us how concerned they were. They did this the following day once she was out of the woods.
Thursday afternoons are when the oncologist have their Tumour Board to discuss patients progress and plan treatments. We knew that we would have to wait until after the meeting before we would find out anything from the results and we were expecting the worse. As ever, we leave it to others to say "Oh she'll be all right!". I think parents are excused from thinking this - it's called self-preservation. Nevertheless, the time came when Ciara's consultant came in...and gave us a list of good news.
First off, the ultrasound showed that the vein going through her liver, the one that flows the wrong way with VOD, had started flowing the correct way - a sign that the VOD is on the way out. What the ultrasound also showed, or didn't show, was any sign of neuroblastoma. Next came the CT scan. This showed that her gut was full of air, which would be causing huge pain and needed to be cleared before we got anywhere with the digestion. What the CT scan also showed, or didn't show, was any sign of neuroblastoma. Next where the urinary catecholomine results. They had gone down and were as near as dammit normal, that's normal as in you and me normal, not cancer patient normal. Finally, the biopsy showed one percent of what looked like old, benign cancer cells. Nothing active, nothing on the rise. So all good news there then So the only thing we are waiting on now is the MIBG, and the scan for that is tomorrow.
Ciara's girth has slowly been coming down, and while not constant, any economist would notice a downward trend. Over the last couple of days she's been perking up, and this evening I could swear she was putting weight on round the cheeks. Enema's and other interesting stuff seem to have kicked her gut back in to gear. The vomiting has stopped and it's been days since she was last sick for no reason. Her interest in food did wane so we had to put the NG tube back in last night. This caused her to be sick, but it certainly wasn't three days of milk worth (she's allowed to drink lots of milk), so something is working.
It seems the only question mark now is why she is spiking. That hasn't gone away and she's hitting 38.2+ nightly. Nothing has shown up yet and we are scratching our heads...
(Sorry Finn, footnoted again). Finn is doing great, he has a supreme interest in food. I'll cook him a steak tomorrow to see if that will satisfy him. He is the spitting image of Ciara when she was his age.
We've got a lot to catch up on since the last post. I've given myself a break from posting for the Christmas period and it means I've got a bit of a backlog to catch up on. In the last post I talked about how Ciara had the catheter removed. Well it took most of the weekend following it's removal for the leaking to stop but by Christmas Eve it had sealed up nicely. There is now a small protective patch that we can probably remove already.
She's been getting increasingly uncomfortable over the last few days and has enough abdominal pain to stop her achieving any kind of good mood. On Christmas Eve, it was a struggle getting her to be even a little excited and she couldn't get to sleep because of discomfort, Santa only had a 30 minute window to deliver presents. During the night there was blood in her poo - whether she was able to get out of her room on Christmas Day was now in question. As it wasn't a huge amount of blood, she was allowed out but if any more blood appeared, we would have to bring her back to the ward. Christmas Morning her haemoglobin count was low and she needed a transfusion but we managed to postpone this to the evening as a transfusion would have taken about 4 to 5 hours. We were over at Ronald McDonald House by 11am, where Ciara made the most of her extra 60ml of fluid she'd generously been allocated for the day, but she was pretty uncomfortable and didn't really get in to the spirit of things, and nor did anyone else for that matter.
After more talks with Ciara's Oncology, and now Gastrointestinal consultants, we found out that the blood in the poo (melena) could be caused by a number things. The GI doctor thought that Ciara had varices, which are like varicose veins in the oesophagus, but he didn't think they would be causing the bleeding as there wasn't enough blood. The varices would have been as result in the increased pressure caused by the VOD (veno-occlusive disease) but the bleeding could have been something else such as an ulcer or gastritis. The only way to be sure would be to perform an endoscopy. If during the endoscopy they found varices, banding of the varices would be carried out to prevent further bleeding.
The endoscopy was carried out on the day after St Stephen's day, under general anaesthetic and after a brief wait for platelets. No varices were found but there were signs of gastritis with blood oozing through the stomach walls. This oozing is either caused by the high back flow pressure from the VOD or just the clinical stress of long-term medication. The internal blood loss will need to be very carefully monitored as it is extremely dangerous. This brings me on to further discussions about Ciara's treatment. We had a good chat with Dr Capra on Friday and he is still in the belief that a conservative approach to treatment is the best way for now, i.e. sit tight. However, after a few more weeks, say mid to end of January, if no progress has been made, or if more complications arise from the VOD, we may be talking about liver surgery. This would be in the form of a shunt to allow blood to bypass a liver vein to reduce backlog. This is not a treatment of VOD - VOD will still be there afterwards - but it does allow us to reduce risks elsewhere. This kind of surgery is not to be taken lightly and is is a dangerous, one-way process. And then, of course, we have to fit in some cancer therapy as well.
As I said earlier, Ciara is getting more a more uncomfortable, and restless nights are the norm. It seems to be all gastric pain, the kind of thing a good poo used to sort out. It's getting harder by the day to make her happy, she's not interested in anything while in pain and the pain is breaking through the codeine. She's fed up and wants to go home. Before we had a mostly happy Ciara with the odd day of down and fed up. It's the other way around now.
After three unpleasant nights of having the catheter in Ciara's belly, it was removed today. Regardless of what it was designed to do, the thing did start leaking last night. The yellow, odourless liquid, soaked a few pairs of leggings and pyjamas just from walking around to Ronald McDonald House. Overnight a gause was applied to try and soak up any leaks. Today Ciara was given a seditive, which as usual didn't make her go to sleep, it just made her a bit drunk. At least this time she wasn't aggressive. The doctor snipped the stiches holding the catheter in place and pulled out the 12 inch tube. As a doctor-nurse-parent team, we then spent the rest of the day trying to stop fluid pouring out of the wound. If too much fluid is lost, even if it's useless fluid, the body will try and compensate by drawing good stuff from the blood system. A drop in blood pressure would cause serious kidney complications. So what would seem to be the obvious thing to do, being draining the fluid while the tap is in, is in fact a terrible idea.
Removing the catheter while so much pressure existed was a bit of a new thing so the doctor was trying different dressings to try stem the flow. We didn't want to wrap her up in a strong adhesive dressing as removing it would be very painful for Ciara. This attention to what could be seen as a minor detail impressed me greatly. For a few hours, we took turns holding a gause tightly against the wound. This stemmed the flow completely so we could see that pressure was more important than a water-tight seal. She now has a pad tightly secured with a bandage. Any fluid loss today was compensated by a drip and her levels and blood pressure are fine.
Throughout all this, Ciara was very co-operative, and a credit to herself. Even when lying on a bed soaked with fluid, with people fulling at dressings and pressing at wounds, she was helpful, kind of calm, and sometimes even courtious. She's asleep now but was in excellent for this evening; glad to be rid of that horrible catheter.
Ciara had her pigtail catheter fitted this afternoon. Nasty looking thing that looks like part of a home brewing kit (or a heart plug to any sci-fi fans). She's asleep at the moment, thankfully, as it has caused her quite a bit of pain.
The liquid drawn off was clear so it's definitely not chyle. Earlier, the ultrasound finally showed signs of actual VOD, namely backflow on one of the liver's veins. So we are back to treating as VOD again. She did manage to lose some weight and girth over the weekend so hopefully that will keep up. I'm yet to find out if they will use the tap to draw off liquid or if they'll let her body do the work. Either way the catheter will be removed after a couple of days.
There was a change of plan over the weekend with regards to using the heavy duty diuretic Metolozone. Ciara was given this for Thursday and Friday but on advisement from nephrology she had no more. Regardless, she has lost a small amount of weight and her girth is down a little bit, so her body is managing to do some of the work itself. Tomorrow, under anaesthetic and following an ultrasound, Ciara will have a pigtail catheter inserted, designed to stop leakage when inserted and to allow controlled drainage of fluid. This important as Ciara's abdominal region is under a lot of hydrostatic pressure; we don't want her whizzing around the room as soon as anything gets plugged in. From the catheter, a small amount of fluid will be drawn off and analysed. There is a possibility that Ciara could have chyle leakage as a result of her tumour surgery. Chyle is a milky, fatty substance produced in digestion. If Ciara's bloated abdomen turns out to be chyle, then we would need to reduce Ciara's fat intake to help heal the leak. If after further analysis we are still unable to identify the cause of the fluid build up (ascites), then we will move to a transjugular liver biopsy on another day.
It's too early to be worrying about this now, but reducing Ciara's fat intake is going to be tough. Even though she is heavy, she is so thin. Her arms are like sticks and her face is very drawn. I know she's been increasingly image conscious too, and most of the time photos are out of the question. Today though, Ciara came over to Ronald McDonald House and agreed to a group photo. Volunteers had put on a Christmas Party at the house; turkey, wine and Santa included. It was a lovely afternoon. Unfortunately Ciara couldn't join in with all the fun as she isn't allowed into crowded rooms full of germy kids. Not that she would have wanted too though. She's become quite insular and we have a huge amount of work ahead of us to bring her out of her shell. She does one-to-one very well though, but is pretty nervous of crowds. Instead, we all stayed in the R-McD's TV room, with her mum, grandparents, Aunty Annie, Tony, and her new little brother.
In the weeks and months leading up to Finn's birth, Ciara had pointed out repeatedly that she does not want a little brother and didn't like babies; they cried and made a noise. She even offered him up to a nurse, asking if she would like to take him home. Luckily for us Finn is as good as gold, and crying is soon resolved by applying a simple clean-feed-burp-cuddle algorithm. Ciara met Finn for the first time yesterday afternoon. She went from not wanting to be in the same room as him, to picking up his dropped hippo for him in the space of a couple of hours. Today Ciara even tip-toed up to Finn and stroked his hand, making my heart go squelch. While still nervous, she's getting there, and went so far as acknowledging he was part of the family. The bribery, and spending all possible moments with Ciara on our own, seems to have paid off.
One of the team grabbed me last evening and told me they had been talking to nephrology - kidney people - who have experience dealing with a condition called Nephrotic Syndrome. Ciara doesn't have this but they have experience treating the excess fluid it produces. They have advised seeing how a heavy duty diuretic called Metolazone acts over the weekend before going down the surgery route. Fingers crossed for gallons of wee over the next few days.
The stubborn VOD is refusing to go anywhere and now it appears to be getting worse. Ciara had an ultrasound the other day and I could see on the screens huge black areas, indicating fluid. As far a the liver goes, the scan showed that it appeared the same as it did a few weeks ago. It seems to be doing the right thing; they check the flow direction of fluid through a vein, and that seems correct but the increase of fluid has caused a swelling of the pancreas. Over the last few days there has been a steady increase in Ciara's weight and girth. This morning when I lifted her I nearly put my back out; the increase in weight since the day before was so much that I hadn't compensated when lifting. She is getting increasingly uncomfortable and had a very troubled night. Amazingly she is in pretty good form, though she's not happy that the nurses have dressed up as characters from Snow White, and wants the dwarves to take their beards off.
The liver specialist visited yesterday to discuss where to go next. It's obvious that the defibrotide is not doing its thing, though she is still receiving it. He - along with the oncology and surgical teams - will be discussing options for diagnosis and treatment. Diagnosis will be in the form of a bioposy involving sending a device down a main vein from her neck, down in to the liver. Here a tissue sample will be extracted and pulled back up the vein. This is not a procedure often carried out in paediatrics and Crumlin do not have the expertise, so outside help will be required.
Treatment consists of puting in a tap to allow the fluid to drain off, plus some very strong diauretics to keep the fluid off. Both diagnosis and treatment involve risks. Certainly oncology will frown on the idea of an abdominal tap due to the risk of infection. Disucssions will be happening this week but I've been told not to expect news until next week. In the mean time, fluid intake has been drastically reduced and Ciara's oral allowance has been halved.
In other news, Finn has been put on antibiotics. He had a little episode on day two where he stiffened up and went grey. He was choking on mucas that hadn't cleared from birth. He was quickly rushed off to be cleared and revived and everything was okay. The whole episode lasted seconds and later he was given a stomach washout. However, while the paediatrician was checking him over, she noticed that his resting heart rate was a bit slow. An ECG indicated mild bradycardia - slow heart rate - so they took bloods for tests. One level indicated an infection so he'll have five days of antibiotics. This extends Yvonne and Finn's hospital time by a couple of days so they should be out at the weekend. Back to Ronald McDonald House. Unfortunately, due to a winter vomiting bug, the only visitor Yvonne and Finn is allowed is yours truly. Even his grandparents aren't allowed to visit.
