Good afternoon to you all.
It’s been a while since my last update and I apologise for not updating earlier. We were running at 100 miles an hour the last 2 weeks.
We have been working extremely hard to get Chiane’s weight up and helping her body to repair itself. It took very close and intensive coordinating between us as parents, our homeopath, our nutritionist and the support of our local hospital and Great Ormond street hospital.
Chiane was on a feeding pump for a week during night time when we first got home, but she is now eating well enough for her not to be on a feeding pump overnight. Whatever she is short of in her daily nutrition intake, we give to her early evening through her PEG before she goes to bed.
One of our biggest challenges are her platelet levels to date. Her immune system got to a super strong level, with her Neutrophils and white blood cells being very high.
We have been going back and fortsh to our local hospital almost daily for platelet transfusions. This in itself has a knock on effect because Chiane is allergic to platelets and she has to receive counter medicine to reduce side effects. She “puffs up” every time she receives platelets and we lost count how many platelet transfusions she has had. The “border line” level for brain cancer children is a platelet level of 30. Since Chiane had her last chemo it has not been above this level… For 9 weeks now. To give every one a perspective… A platelet level of a 100 is low, below 30 it gets dangerous and Chiane’s platelets drop as low as 8. It got to the point where her platelet level was so low, blood blisters started showing on her eye balls…. It was painful for her. She had so many transfusions they run out of platelets in London and had to go nationwide.
Things got more complicated when we realised her immune system levels started to drop (white blood cells and neutrophils). At first we thought she might have picked up an infection, but after a few days she still had no fevers. We continued to boost her immune system and her neutrophil and white blood counts continued to drop. It was at this point we realised Chiane’s body has become immune against platelet transfusions and that her own immune system is attacking the platelets she receive.
Why do her platelet levels struggle to recover you might wonder? Because her body is using the platelets to repair its damage that was caused by the last chemo. As soon as her body produces platelets, the platelets just get sucked up by her body as its thirsty for platelets to repair all the damage. Why is she getting transfusions? Because her body doesn’t produce platelets fast enough… When her platelet level was 8 for example, it’s not that her body doesn’t produce platelets… It is producing platelets but her body can’t keep up making enough platelets quickly enough. Once her platelet level count starts going up by itself, then we know that her body has repaired itself.
The blood blisters are now gone and her eyes are white again.
The fact that her body is now immune against platelet transfusions complicate things a bit more, as her body is now attacking platelet transfusions she receives, which means her immune system is starting to take a knock as well. We are therefore working day and night to boost her immune system levels as much as we can.
Furthermore, things got really intense on Friday last week when Chiane’s blood cultures were taken while she was in hospital getting a platelet transfusion and it showed that her potassium levels were below 2,4. Potassium is a very very important component in the body and if it is too low, it can lead to Hypokalaemia. A quick “trip to the hospital” to receive platelets turned in to a very very long intense night at hospital. If the potassium level is too low in the body it can have devastating effects. Small changes in the extracellular potassium level can have profound effects on the function of the cardiovascular and neuromuscular systems. Chiane complained about “pain in her chest”. She didn’t know it was her heart that was causing the pain and neither did we tell her that it was her heart. Chiane was given potassium through IV over a 4 hour period. She also received red blood cells transfusion the same night because her red blood cell levels started to drop and it is important that it doesn’t because red blood cells carries oxygen through her body. If this drops, and she doesn’t get enough oxygen to the brain, combined with low levels of potassium it can cause her to get heart attacks, damage to the brain or much worse. I’m not going to go any further, all I will say is that it was a very very very long night and I kept staring at the heart monitor machine that was connected to her heart. We are now giving Chiane potassium supplement at home to manage her potassium levels to avoid any future complications.
Chiane is home and doing much much better now. Since we got out of Great Ormond street hospital 2 weeks ago it was a challenge to get her to eat, and daily trips to our local hospital for transfusions had me and Andia running around like crazy people trying to make sure she “gets what she needs, when she needs it”… (Nutrition and medicine).
When is her next round of chemo?
It was originally agreed that Chiane will come home for a week and then we will be back for a 2nd round of Thiotepa. (this would have been week 7 after her first round of Thiotepa). Why do I mention week 7? What’s so important about week 7?
The Milan treatment plan Chiane is on, is a very new treatment plan. It’s only a few years old. The results of the first groups of children on this treatment plan started to come in, and the latest publication was only 3 weeks ago on pubmed. On pubmed (a medical source that doctors use and publish there work) the latest publication stated the ideal window for the 2nd round of Thiotepa is more or less 6-8 weeks after the first round of Thiotepa. The medical have realised, based on the latest results, if they start too early (week 1-5) with the 2nd round of Thiotepa it had devastating effects on those children. It’s too soon, and too toxic for the children to handle it.
If they wait too long (9 weeks and more) with the 2nd round of chemo they don’t get the “satisfactory results” meaning the whole exercise of giving Thiotepa in the first place didn’t work on the cancer; that the gap or break was too long between the 2 Thiotepa’s to push the cancer back.
So ideally, the “golden window” based on the latest studies is week 6-8. We agreed Chiane would start week 7 when we left GOSH, but at week 7 Chiane’s body was still to week. We agreed that we will wait another week… And start week 8. There was a lot of discussion towards the end of last week whether we should start this week or not (week 8). It was a mutual feeling between us and the consultants at GOSH that we could not start the 2nd round. Why? Because her body is sucking up platelets like its gold dust, which means mean her body is still repairing itself. Starting now with the 2nd round of chemo will be too toxic for her to cope. We have therefore decided and mutually (us as parents and the consultants at Great Ormond street) agreed that we will wait another week…. So, it’s week 8 and Chiane is home and we are planning to start week 9 (next week and Monday) with the 2nd round of Thiotepa.
First thing you might ask now is..”are we not missing the window of 6-8 weeks now”?
The answer is yes… On paper… BUT, every person is unique and you can not continue pushing people in to a “protocol”. We need to look at each case individually because that’s what we all are…
The balance and window period between the 2 Thiotepa’s are extremely important based on the latest results but we ignored the exact weeks. All it said to us as patents were… If you start to early with the 2nd round of Thiotepa and the body is not healed, it is lethal. If the body has recovered and you wait too long the exercise doesn’t work. Each child is different, and in Chiane’s case, her body is taking longer to recover. Once her body “hangs on” to her platelet levels above 30 without any transfusions, then that will be a sign that her body has repaired itself. A platelet level of 30 is still a long way off a 100, but we won’t wait till then. As long as we can get it above 30 (out of danger) then it will be a green light to start. Her platelets were 28 last week and Monday; 18 last week and Wednesday; 8 on Friday last week… She had loads of transfusions and despite all these transfusions it continued to drop. Clearly, based on the above her body is not ready yet! Today, her platelet levels were 28 again, but after more transfusions today. As soon as it is above 30, we will stop transfusions, wait and see if her count drops again or if she maintain/increase her platelet count by itself. If it increased by itself we will start next week and Monday with the 2nd round of Thiotepa, if it doesn’t and it drops again then we will have further discussions with the consultants towards the end of the week.
How is her pain? Much better. When we first got home Chiane was in amends pain (in her bones) because her body was repairing the bone marrow. Now, she has the odd pain may be once every 4 days.
She is starting to eat well, her body weight went up by 500g that last week and mentally/morally/spiritually she is doing fantastic.
Thank you for your continues support, prayers and patients with us. Again, apologies for not updating as often as I should have, but hopefully everyone will understand that we are off our feet busy supporting Chiane at the moment.