Let's Help Betsy To See Again
"Those of you who know Keith and I well, know that our family is our world.
In May this year, our world was turned upside down when we received the devastating news that our darling, beautiful, comical, granddaughter Betsy, who is only two and half years old, had a large brain tumour - a diffused optic nerve glioma.
The location of the tumour means that Betsy's eye sight has been very badly affected and she has been registered as 'severely visually impaired (blind).' Glasses cannot help her eyesight.
An operation to remove the tumour is not an option for Betsy because of its location, so she is currently undergoing an 18 month intensive course of treatment at Great Ormond Street Hospital. The hope is to shrink the tumour and maintain her eyesight but the type of tumour she has, although benign, can be hard to shrink and there is a chance her eyesight could deteriorate further.
But there is hope for Betsy. There is research currently happening to improve vision for children with optic nerve gliomas including a drug called Bevacizumab, (Avastin) which has shown some good success in helping to improve vision in children with this condition. Unfortunately, the NHS is unable to fund the drug for Betsy at present. As a family, we are of course keen to pursue the treatment in order to give Betsy the best possible chance of improved sight. The 3 month cycle of Bevacizumab will cost £9,600.
We are also aware that chemotherapy may not work, and are therefore keen to raise funds for additional proton beam treatment, which Betsy could have when she is 3 in October.
There is exciting research happening to regenerate the optic nerve and Betsy's ophthalmologist has said that it is realistic that there will be something out there for Betsy in the coming years.
it would be fantastic that when any of these are on offer to Betsy that the funds are readily available so that we give her the best possible chance in life to have improved or full vision.
We aim to organise a series of fundraising events over the next 18 months; we are keen that Betsy's long journey ahead is filled with as much fun and positivity as possible. Those of you who have met Betsy, will know she will relish any opportunity to have fun and meet people!
I will keep you updated with our fundraising plans.
If anyone would like to donate, please click on the link below. Our heartfelt thanks in advance x
As promised in my December email. Please find below, details of Betsy’s next fundraising social event! “Bop For Betsy!” A 50’s themed night which will include an American themed menu, live music and a very special auction and raffle! We have also been promised a jive demonstration from a 50’s jive and Lindy hop group!
Those of you who kindly supported Betsy’s Ball, will have been sent an email with the details of this event. Please check your junk and spam boxes of you haven’t received!
You can click on a RSVP button on this email to say you would like to attend. The link to buy the tickets online, will then be sent to you. If you haven’t received the email and would like to come, please email me directly: email@example.com
We usually update you at this time of year with Betsy’s progress and look back at fundraising highlights across the year.
2018 was certainly a year of two halves for Betsy! After completing 18 months of gruelling chemotherapy, Betsy rang the special bell at Great Ormond Street and Watford last December. Betsy had her port insert in her chest removed in early 2018 and was given permission to travel abroad for a holiday in April.
We all had a wonderful time in the canaries and Betsy was able to put her swimming lessons into good practice!
Unfortunately, when we Returned home in May, Betsy’s MRI scan showed that the tumour had grown quite significantly and was bigger than it was when she was first diagnosed.
Betsy had to undergo a 4 hour biopsy, which revealed her tumour has a particular mutation which means that Betsy’s tumour type tends to behave more aggressively, but at the same time is more likely to respond to targeted therapy.
We were therefore very encouraged to hear that Great Ormond Street, were about to open up Part D of a research trial for children with optic nerve gliomas who have failed all other standard line treatment and have this v600 mutation. After lots of discussions and important health checks, Betsy was accepted on to the trial in July this year.
The new treatment involves a combination of 2 drugs, which are thankfully, a lot less aggressive than her chemotherapy treatment.
There is quite a regime around timing of food and drinks, and there could be side effects related to her skin. This means Betsy must be creamed from top to toe, morning and evenings. Betsy also has to have very regular blood, kidney and ECG tests, as there can be possible side effects in these areas too.
Betsy had to have her port re inserted in her chest, before she embarked on the trial. The port allows the Drs and nurses to take bloods without having to access veins in Betsy on a frequent basis.
The research team arrange MRI scans for Betsy every 8 weeks. The first scan in September, showed the new drugs had shrunk the tumour significantly. The second scan in November, showed the tumour had shrunk to almost a pin point! To say we are all over the moon, would be an understatement!
A lot of people have naturally asked what this means in terms of Betsy’s eyesight. As the damage to her optic nerves has been significant, it would be unlikely her vision would recover. However, the new drug trial has demonstrated what wonderful things are being achieved. It gives us hope that one day Betsy’s vision could be improved or restored by medical science, particularly in the area of stem cell research.
Rochelle has been in touch with pioneers in this country and abroad. They are hopeful they may be able to consider her for treatment in the future. The caveat has always been that this treatment could only happen, at such time that the tumour has been treated.
So fundraising for Betsy has never been more timely or important. We would like to thank the following people who have all arranged the following fundraising this year:
February - Triathlon February Toby and Tanya Clark
June - Tube Challenge Janice, Gary, Liam & Wayne
July - Band Night The Malden Brothers Band
September - Quiz Kim & Lynn
December - Christmas card donations from a number of friends and family.
February 9th will be the next fundraiser, a 50”s themed night at the John Lyon School. Look out for further details on how to book tickets!
Betsy continues to be an absolute inspiration to anyone she meets. She is brave, funny, kind and very musical! We have attached a video of her drum lesson progress and singing in her nativity play! She is starting piano lessons in the new year too!
She is doing very well at School and has made brilliant progress with writing and reading Braille.
We would like to take the opportunity to thank you all, from the bottom of our hearts, for your generosity, kindness and support. It really does mean the world to us all.
We hope you had a lovely Christmas and you have a very happy and healthy new year!”
A huge thank you to everyone’s continued support and interest in how Betsy’s treatment is progressing. Sorry it’s taken so long for us to update you; we have been wanting to make sure all details are correct before we gave you any news.
When we last updated you, Betsy was having a break after 18 months of weekly chemotherapy. She got to ring both ‘End of Treatment’ bells at both Great Ormond Street and Watford General! She also had her port (which administered the chemo and nurses took bloods from ) removed in April too.
Unfortunately, a month later, Betsy’s MRI scan in May, showed the tumour had grown significantly and it also had a cystic element to it.
We were always realistic that Betsy would most probably need further treatment, but so sad that Betsy has not had much of a break.
We knew that the plan would be that if the tumour grew again they would do a biopsy. So, a few days later, Betsy had a 5 hour operation whereby they successfully managed to take a small sample of her tumour. The wait for these results took ages! We were hoping that the tumour would show a mutation, which would mean Betsy could potentially start on targeted therapy rather than another 18 months of further chemotherapy. We finally received the call that Betsy had the Braf V600 mutation. This mutation does tend to behave “badly”, however it often responds better to targeted therapy. So, once Betsy had recovered from the biopsy she then had her port put back - more general anaesthetic! But this was a relief in all honesty, because up to this point, all of Betsy’s bloods were being taken through a Cannula, and despite Betsy being brave and calm the nurses and drs could not get blood from her! Betsy was so bruised and fed up, that the port was actually a welcomed return in the end!
We were then hopeful that the treatment would get started but we had another long wait! But we now understand what the hold up was. This trial treatment is now in to its fourth stage of development: Part A was one drug, Part B, the other drug, Part C both drugs together and Part D is both drugs with a change in dosage. So, as you can imagine every time the drug company changes something it takes a while for it to get accepted. So, Betsy will be one of the first children to be accepted on Part D. A natural thing to do is to think about “why didn’t we try and privately fund ourselves with the money we had raised and trial these drugs earlier?” However, if we had trialled it on Part A or B and then had tumour growth we wouldn’t have been able to go back on this trial. It sounds as if they’ve had real success with the combination of both drugs together, so as we always try to do, we look at this next part of our journey with positivity and hope.
The fact it is a trial has meant Betsy’s has had to put up with so
many tests before they could start, including another MRI earlier this month, which showed further tumour progression. The tumour is now putting further pressure on structures of the brain so we were relieved when treatment could finally start last Thursday. It was a very long and hot day at Great Ormond Street, but Betsy was a really trooper and took her medicines well, which means we can now give them to her at home. She will be monitored a lot initially, but hopefully over time we won’t have to do as many trips there.
The medicines are taking a bit of getting used to for us all- they don’t taste like strawberry Calpol! Plus you have to rejig the routine a bit as you can’t eat 2 hours before or an hour afterwards. However, 6 days in and we are getting used to it. Because it’s targeted treatment we are hoping the side effects will be much more tolerable. Rather than the breaking down of cells it works on breaking down the protein make up of the tumour. However, it is likely to have some effect on her skin. So Betsy’s skin will need to be very looked after and monitored closely. She has been told her hair might turn curly which she is very excited about!
Of course this is a new drug so long term effects are not yet known, but compared to other treatments this does seem the best option .
Although this tumour is low grade, they do behave badly- especially Betsy’s mutation, and with it being in the brain, we need to try and get a hold of the tumour and stop it from growing and causing further damage.
Betsy vision is still the same and she is still registered blind. Thankfully, she still has some useful vision and we hope that the tumour growth won’t cause any future damage and this treatment will preserve her remaining vision. Because Betsy’s optic nerves are damaged the team does not hold out much hope that her vision could improve if the tumour were to shrink. However, what is most important right now is to get the tumour under control. It has damaged her endocrine system too; she has low thyroid and is growth hormone deficient. She has started on thyroid replacement and will
hopefully be able to start growth hormone replacement once treatment finishes which is estimated to be between 18 months -2 years time.
So as you can see there has been a lot of information to take in - especially for Betsy who is now that bit older and asking tougher and more intelligent questions! Yet she still proves to be a fun loving, charismatic and resilient girl who isn’t going to let her illness define her.
She is doing well at school and received a very good end of term report. She is where she should be in all areas of development and exceeding expectations in some - amazing if you consider her vision and energy levels from the brain tumour and low thyroid.
She still has a huge passion for music and seems to enjoy Dolly Parton’s songs the most at the moment! We have to describe to her what Dolly looks like to her in detail, she then dresses up as her, listens really intently to one of her songs then gives us a full rendition! She recently made a recording of Coat of Many Colours at one of her Nordoff Robbins music sessions, we will hopefully be able to share the video with you all when it’s been edited. In the meantime, please enjoy the clip of Frank Lampard joining Betsy during one of her music sessions!
Betsy is also going to start drum lessons and performing arts classes in Semtember, as well as continuing with her swimming lessons - all being well with her skin. So life is not on hold, it just needs to be adjusted a little bit.
Thank you all so much for all you do to help us on this journey! From organising your own fundraisers, your donations, your time and love. We still hope that a lot of the funds raised will go towards stem cell research to give Betsy and others a real chance of seeing again. But In truth, we learn as we go on this journey that the amazing funds that together you have helped us raise maybe needed at any time.
Thanks again for all your kindness,
With love from Betsy and all her family
"With recent progress in stem cell research, neural stem cell therapy is emerging as a new approach that may be promising for restoration of sight after optic nerve injury. Attempts have focused on activating dormant stem cells, already present in the eye, to participate in the repair process in response to injury. In addition, it may be possible to harvest and transplant donor stem cells into the eyes of patients whose optic nerves have been injured, to replace the neurons that have been lost or to provide a permissive environment for nerve regrowth. Although these studies are still in their infancy, the field is progressing rapidly and may soon turn the stem cell approach into a viable therapy."