Fourth Line Treatment Options

Posted: 19th March 2021
Interim update: 30th March 2021
Final Update: 7th April 2021 (jump to)

Following an MRI scan in early March 2021, Eleanor’s main tumour showed significant enhancement and some further growth. This is something we knew may happen following the third line chemotherapy treatment she undertook – a bounce back effect if you like. Obviously we had hoped Eleanor’s tumours would stabilise with the limited progress that had been made during the third line treatment regimen, but it wasn’t to be.

Time is now of the essence as Eleanor is no longer receiving any form of treatment, therefore giving her main tumour more time to grow and possibly even giving the smaller tumour time to recover too. This now presents us with some very difficult decisions, which need to be made soon. This is a fluid situation as we learn more and more about the options we are faced with, so this page will be updated in due course until we make our final decision. Whatever we decide will have to be what it will be, we cannot afford to look back and think “BUT what IF we’d have done it this way, or that way”, especially if things don’t work out how we hope for them to.

Below is a MRI image of Eleanor’s main tumour, just a slice for comparison. The right side image is when she started this awful journey at age 3 years & 9 months, and the left side image at age 9 years & 11 months. When looking at these images, the things that have changed are Eleanor’s head size and the quality of the MRI imaging, but as even the untrained eye can see there is a marked difference – you are looking at the big white cloud in the middle, nothing else on these images is of concern. This is why we must act quickly.

Below are our options…

• Watch & Wait.

This is by far the least preferred option, particularly by the medical experts; for reasons of disability / residual vision preservation, possibly even life preservation. To be frank we would NOT take this risk and are very much guided by the professionals that lead Eleanor’s medical team at The Royal Marsden, it is probably an option we aren’t even allowed to consider. 

• Proton Beam Radiotherapy – Craniospinal Irradiation (CSI)

This form of treatment can be started relatively quickly.

Possibly the most definitive solution but in our opinion carries the most risks to Eleanor’s quality of life. Short & long term side effects like stunted growth, brain function (processing speed & memory), chances of further cancers (particularly to the brain), increased risk of strokes, are all strong possibilities, sometimes a given, and these are just a few. There is a whole list of potential risks.

This type of challenge can also aggravate the tumour in such a way that Eleanor would eventually need quite invasive neurosurgery to reduce the tumour size.

• TPCV Chemotherapy

This form of treatment can be started almost immediately.

A challenging 12 months of chemotherapy which requires very strict medication schedules and periods of dietary restrictions. The majority of side effects are short term. Although, as with any chemotherapy it also carries the increased risks of secondary cancers. Eleanor has already faced these risks with the other chemotherapy regimens she has undertaken, but with each regimen, the risks increase.

If this form of challenging the tumour didn’t work, after 3, 6, 9 or 12 months (at each one of these intervals there would be an MRI scan to monitor any changes), we could end up back at the proton beam radiotherapy option or possibly even an emergency trip to a hospital for quite invasive neurosurgery.

• Tumour Biopsy & TPCV Chemotherapy (a ‘hybrid’ option if you like)

Along with the above TPCV chemotherapy we would first opt for Eleanor to have neurosurgery to take a biopsy / sample of the tumour so it could be typed. At no point in the last 6 years has this been attempted but it has always been a potential option. Knowing the exact type of the tumour (the fusion or mutation) would perhaps give us future treatment options using drugs called Inhibitors, which have often shown significant efficacy with these types of low grade glioma tumours.

Neurosurgery has risks, adding any additional time before getting the next chemotherapy underway has risks… however the benefit of going this route would be that if the 12 months of TPCV didn’t quite work, we ‘might’ then have further targeted treatment options. These further treatment options could possibly only be given for a further 2 years (due to toxicity and effectiveness). So if this hybrid option didn’t work, it would at the most buy us another 3 years before we would ultimately have to visit the Proton Beam Radiotherapy option.

There is now a slight caveat that has been added to this option, with some recent information that has come to light. See below…

Following a meeting at St George’s University Hospitals NHS Foundation Trust on 24th March 2021 with Miss Samantha Hettige, a lead consultant neurosurgeon. Her professional opinion was that with the endoscopic equipment she has available to her, and with what would be deemed to be least risk surgery; would be to take a small biopsy of Eleanor’s tumour from a high point at the top of it, keeping away from Eleanor’s optic nerves as best as possible.

With all neurosurgery there would be risks and although this would be a fairly straightforward operation, reaching Eleanor’s tumour is difficult (in the centre of her head) and delicate brain structures need to be bypassed. Sometimes taking only a very small specimen is just not enough to obtain extensive pathology from, so it could be necessary to go back in again to get more.

Once a biopsy is completed and Eleanor recovered, she would need to immediately start the fourth line chemotherapy challenge, which would attempt to shrink her tumour, as it is obviously continuing to get bigger. However any chemotherapy challenge would not immediately begin to shrink the tumour, this takes time. During the course of the chemotherapy, applications would be made to the drug companies on compassionate grounds, for the special inhibitors, that’s IF we get enough tissue from a biopsy and IF Eleanor’s tumour is a type that can be treated with inhibitors! Lots of IFs!

After a little research and a direct approach by us, we found that there was possibly another option, from some other professionals that are also involved in Eleanor’s wider medical consultation team. So, after a more detailed MRI and extensive meeting at Kings College Hospital on 30th March 2021 with lead consultant neurosurgeons Mr Bassel Zebian & Ms Cristina Bleil, their professional opinion was to take a slightly different approach with the ever so slightly larger endoscopic equipment that they have available to them. This would be not only to take a biopsy of Eleanor’s tumour but also to debulk the tumour, again starting from a high point at the top of the tumour, keeping away from Eleanor’s optic nerves as best as possible, but hopefully removing enough to improve things in that location, including the all important CSF pathways.

From recent scans, there are quite possibly initial signs of an impending fluid blockage in Eleanor’s brain (hydrocephalus), maybe not soon, but eventually. This approach would be a lengthy and delicate operation, well over 5 hours… with similar but slightly higher risks as the above biopsy option. Reaching Eleanor’s tumour is difficult (in the centre of her head) and delicate brain structures need to be bypassed. This would also hopefully result in a good chunk of tumour tissue being removed to be able to obtain extensive pathology from.

This surgery option ‘might’ also be enough to stabilise and reduce Eleanor’s tumour enough for us to be able to watch and wait without any further treatment for the time being, but we are prepared for this not to be the case and to head straight in to the final chemotherapy challenge that is available to us after Eleanor’s recovery.

IF we did get a period of watch & wait, and IF the tumour grew back or became dangerously active again, we ‘could’ by that time be ready to give any special inhibitors that would specifically attack it – providing of course that once the exact pathology comes back, that there are special drugs that will attack Eleanor’s particular tumour type AND the drug companies that provide such drugs agree to  provide them compassionately, as they are not available on the NHS! Again lots of IFs!

Helping Eleanor to understand these scary options is a heartbreaking process, she cries, she’s scared and she doesn’t understand all the detailed implications, but she knows we are at a turning point and we are running out of choices.

To say we are scared, is an understatement. Eleanor isn’t going anywhere anytime soon, but as adults we are terrified and this has thrown us a complete curveball of options that we are literally drowning in. No one can do anything to help us but listen. This is a decision we must make and must make soon.

Please keep us in your thoughts, especially Eleanor. x

Further update: 7th April 2021

We have decided that the best way forward to try and keep our talented & cheerful little girl just the way she is, for as long as possible, is to opt for the ‘hybrid’ option with neurosurgery option 2. If you’ve studied the information above, we are certain you’ll understand how agonising and emotive a decision this has been for us as Eleanor’s parents.

We feel that getting a little more of Eleanor’s main tumour gone by debulking (along with an additional procedure that opens more of a CSF pathway in her brain), might just give Eleanor a fighting chance at avoiding a potential emergency admission to hospital in the future; particularly if her tumour continued growing at the rate it has been, as it could eventually cause a dangerous blockage in her brain.

Getting more tissue from the main tumour by debulking also loans itself to a more conclusive pathology report, potentially leading to more targeted treatments in the future. We know that more chemotherapy is certainly on the horizon, possibly as soon as she recovers from this surgery.

Finally, pushing the possibility of any type of radiotherapy further and further in to the distance and maybe over the edge in to never being required is our ultimate goal – although the possibility of this being required is always going to hang over us, until a point where Eleanor’s tumour is no longer active or medical science catches up.

Therefore, Eleanor will be admitted in to Kings College Hospital on the 13th April 2021 (now changed to 14th April), her Neurosurgery will take place on the 15th April 2021. The early admission is for pre operative tests; further detailed MRI brain imaging and many other neurological / neuropsychological tests we probably aren’t even aware of. The operation itself can take well over 5 hours (often running in to double digits) and we will not truly know how successful it has been until 1. Eleanor wakes up properly & 2. a post operative MRI scan of her brain has been carried out.

There’s likely to be many other cognitive follow up tests to ensure Eleanor’s short term memory has not been affected (as this is one of the critical structures they have to pass through), as well as the potential for many other checkups.

At this stage, due to the covid pandemic, only Kelly & Eleanor will be going in to the hospital.

The risks are REAL and we are truly terrified.

There really isn’t anything anyone can do, so on the 15th April 2021, whatever your beliefs; please please keep Eleanor firmly in your thoughts and prayers, sending as much healing energy and positivity as possible her way. It will be a long day for us and an even longer few days for her.

Thank you.

Read more about Eleanor’s Neurosurgery here…

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