Mixed News

Good news: two suitable donors have been identified for Kay's bone marrow transplant. The specialists are in the fortunate situation that they can choose between donors and therefore they will run further tests to identify which if the two would be the better candidate. Unfortunately this meant that they had to take quite a lot of blood from Kay today to support this testing. Poor Kay had 100ml taken via her portal, an unhappy surprise. Once a choice has been made arrangements will have to be made with the selected donor for the appropriate physical examinations and paperwork to be completed, then a date for their operation will have to be planned. Kay's transplant will then be planned to synchronize with the availability of the donor cells. It is expected that this will take another 4-5 weeks. So the transplant process is likely to start at the beginning of April.

Bad news: the transplant itself, particularly the conditioning phase leading up to the transplant, is more nightmarish than I had expected. The conditioning phase begins around 10 days before the transplant itself. The first step is to kill off all of Kay's T-cell lymphocytes - the white cells produced by the Thymus - since the presence of these cells would cause rejection of the transplanted stem cells. We understand that this is a tricky process that will require Kay to be admitted to the High Dependancy Unit while it is conducted. The en-masse death of T-cells can cause an allergic reaction of varying severity that requires constant management. This process will take a couple of days. Note that it takes the body 4-6 months to repopulate itself with T-cells and that this is the reason that Kay must be isolated for such a long period after the transplant. T-cells are responsible for the body's immune response to viruses and therefore Kay will remain particularly susceptable to viral infections for a long time.

The second step is chemotherapy that targets Kay's existing bone marrow. This will take around five days. The final step is total body radiation which takes a further two days. The transplant itself follows immediately after the radiation therapy is complete. Side effects of this include the certainty of post transplant infection and damage to the body's mucus membranes leading to sore/infected mouth, throat, etc.

Following the transplant, the recovery process is essentially a question of waiting for Kay's body to start producing bone marrow products again whilst managing the various infections that are likely to occur. Under normal circumstances this process can take 5-7 weeks post transplant. Therefore we expect that Kay will be in hospital for around 6-8 weeks. Then she will be isolated at home for a period of 4-6 months. Of course, this all depends on how well Kay responds to the whole process.

There are all sorts of rules and consequences, ranging from dietary restrictions to the removal/cleaning of the curtains and carpets at home to the separation of cats and Kays. But, yeh, all part of the price that must be paid for a healthy Kay. Hopefully we will get some decent documentation on the subject from the hospital so that we don't have to commit everything to memory.

There are also some interesting technical effects. For instance that Kay will end up with potentially a new blood group, that of the donor. During the recovery period her body will slowly change from one blood group to another. She will require transfusions during this period, which will initially be done using universal blood, but in principle her body won't reject blood from the new blood group even while 'old' cells are in her system. But enough of the techie talk.

On Wednesday we have to return to the hospital for a bunch of tests and a couple of meetings. Otherwise we expect that the next few weeks will remain fairly 'normal'. Kay's blood levels today were excellent, so hopefully the next weeks will be uneventful.

Countdown to the countdown

Kay's check this week went well. Her blood levels are still good and she looks great. Her hair has started to grow back a little but how long that will last I don't know. She is rather too full of energy, the bad weather limiting her ability to burn it off. And when Kay has too much energy, every one suffers. She gets short tempered, bossy, bored, fed up, manipulative, etc. But equally one can enjoy her energy, she's always up to something, always has something to say, is always busywith something, is always willing to help, is always looking for jobs to do, is ready with a loud laugh or a big smile, wants to sit on you knee, to snuggle in your arms, to fall asleep next to you...

The news from the specialist is that the search for a donor is progressing positively. They have a number of promising candidates that have returned samples for further typing. More will be known in the coming weeks. However they are certain enough of finding a donor - or of using Marion or I - that they are starting to plan the transplant. Next week on Monday Marion & I will sit with the BMT specialist to hear about the planning and the details. On Wednesday Kay needs to be in Nijmegen for a bunch of routine tests preliminary to the BMT. My guess is that the countdown proper will start within the next 3-4 weeks.

So the end of this period of rest is in sight. We're all off to the "Hof van Saxe" for four days to get a break. Kay is fit enough to be allowed to go swimming, so I suppose that's where the girls will be found during the coming days. That or bowling. In the coming weeks we'll need to get ourselves organized for an extended stay in the hospital. So I supposed that we'll have plenty to do.

More when we've heard about the planning on Monday...

Eye of the Hurricane

Following the MRD result last week we have entered a period of relative calm before the oncoming storm of the bone marrow transplant. Kay has started maintenance chemo, designed to repress / continue reducing the amount of cancer in her system. We have been told that the BMT will happen just as soon as a donor has been found, but almost certainly in the next 4-6 weeks. So we're assuming that we have around four weeks of relative peace and quiet.

I have taken the opportunity to go down to our holiday house in France to work/cycle/rest/check the house out. Next week we will take a couple of days off all together. Hopefully we will all be just a little stronger and more rested before having to face the BMT.

There is no more news about a possible donor and we're not asking. Asking won't speed the process up but will cause us more worry if it's not going well.

I just put some video on facebook showing Kay using her WebChair software to take part in school lessons whilst she was in hospital a few weeks ago. Check out: Kay's WebChair Lesson (1)

Ray of Sunshine

Kay's MRD result is well under the target level. This means that she will be put on maintenance therapy until her bone marrow transplant. We have an appointment with the specialist on Wednesday morning to hear the details and to start the maintenance cycle.

What a relief...

MRD Result on Monday

Not much to add to the title. Kay's blood levels were reasonable on Thursday and she didn't need a transfusion. She's been to school and yesterday had a (very delayed) birthday treat with her friends - they went bowling and then spent the evening making pizza.

Bone Marrow Taken

Kay had a bone marrow sample taken yesterday for the MRD. This procedure is performed under general anaesthetic (had to look that up in the spell checker...) so she was pale and tired when she got home last night. She'd also been sick in the car on the way home, including her NG tube. So she had to face the delight of having it refitted when she got home. Today she's a little tired and has decided not to go to school. Her teacher will come to our house this afternoon to give Kay some dedicated attention.

Kay's Hb (red cell) count was a little on the low side yesterday, but the rest of her levels were OK. However we have to go to the Catherina on Thursday for a blood test. I suspect that if her Hb is still low then we'll be there all day while she gets a unit of red blood.

For now we're waiting on the results of the MRD. Fingers crossed!

What next?

Kay got through this week's chemo with only a medium amount of trouble. The dreaded asparaginase caused an allergic reaction again, right at the very last moment. The doctors took great care to ensure that Kay was effectively protected by anti-allergic drugs before the asparaginase was given. Also, 25ml was infused over a period of 4 hours, 4x slower than normal. If any reaction, such as a rash, was noticed the infusion would be paused. In theory this protocol should have prevented an allergic reaction and largely it did. However, at the end of the infusion Kay broke out in a fever of +39C. She was sick, etc, in other words seemingly an allergic reaction. During the course of Wednesday evening her blood pressure dropped to 87/29 and her heart rate rose to 140 - 150. All pretty worrying. Marion says that the doctors were not really sure what was happening. The symptoms of the reaction were not typical and the lateness of the reaction was also strange - effectively it started after the infusion was complete. Kay symptoms stayed this way through Wednesday evening but slowly during the night her pulse & temperature dropped and her blood pressure rose. On Thursday morning her blood pressure was 90/47 and her heart rate was around 120. Still her face was quite puffy. So, the doctors were cautious and, much to both Kay & Marion's frustration, kept her under observation until late in the afternoon when they were allowed to go home.

Now it turns out that there's an interesting adder in the grass with this protocol: the line from the infusion pump into Kay's portal has a volume of about 1.5 - 2ml. Before the start of the procedure this line is filled with saline. At the start of the procedure 2ml asparaginese is rapidly pumped into the line, ie 2ml in 30secs or so, to fill the line. Then the infusion is started at a rate of 6ml per hour. After something less than four hours, Marion said that the pump alarm went off indicating that the infusion was complete. However Marion worked out that given the time that the pump had run only 23.5ml had been run in. She discussed this with the nurse who pointed out that there was still about 1.5ml in the line. The nurse then proceeded to flush the contents of the line, meaning that Kay got the last 1.5ml in about 30 secs, the time it took to flush the line. And this is when her reaction started. Seems pretty obvious, doesn't it? So then the question is why the line was flushed in this way?

Anyway, Kay is back home and in good form. In spite of being tired and having no appetite she insisted on going to school yesterday morning. The child amazes me, truly. How she can go from low BP, high temp & pulse to school in 36 hours is incredible. If determination was a treatment, Kay would have cured herself by now.

So the question arises: what next? Kay has reached the end of the second phase of the ALR 10 protocol and, before our meeting with the specialist last Wednesday, we had no idea what would happen next. But the meeting all was revealed...

On Monday Kay will have another bone marrow sample taken for another MRD. This MRD will be done very quickly, within a few days and will determine the immediate course of action. If the MRD is below 10^-3 (ten to the minus three) then she will be put on maintenance chemo until her bone marrow transplant. The lower the MRD the more time there is to find a suitable donor. However, we have been given to understand that there is to be no unnecessary delay, even with a low MRD the BMT will follow as soon as possible.

If her MRD is higher than 10^-3 then she will get an extra cycle of chemo starting a week on Monday, this time a type of chemo that is normally used with AML leukemias. This chemo is of the short-sharp-shock type that will hit Kay's system very hard. It will probably knock her out for up to three weeks and it will IMMEDIATELY be followed by the BMT. In this case the choice of donor will be a compromise, driven by the time available. If no suitable donor is found then either Marion or I will function as donor, ie a cell type match of 5/10. We're told a haploid transplant (ie where the donor has only 1/2 the same chromosomes) is a realistic and manageable option. However, obviously it's not a preferrred option and therefore I assume that there's more risk involved and more intervention required after the BMT. So, in principle, if her MRD is too high then we're now about 4-5 weeks away from the BMT, irrespective of the 3rd party donor search.

So, I can summarize by saying that if Kay's MRD remains too high, from a week on Monday we will be entering a very intense and tough period in her treatment. If it's low enough, then hopefully we will get a few weeks of relative calm and rest before a BMT, with a better chance of having a more highly compatible donor.

I really hope that it's the second alternative: both Marion & I could do with a break. I'd like to package Marion up and send her down to France for a long weekend so that she can spend a few days sleeping in the sun, recharging her batteries.