Congratulations to all of the 4 Down's runners - Liz, Fran, Mehboob and Michael all finishing well under 2 hours 15 minutes - well done and thank you to you all. Great result on a v hot day - a true challenge! Let's raise a glass of pimms to them all and if you have a spare penny to put in the pot as a sign of thanks to them and congratulations, I would be so grateful - as would they. True stars motivated by my story - if anyone feels they would like a similar challenge please get in touch! But for tonight let's raise a glass to these 4 stars from Down's Solicitors and Notaries! #teamjen

Good luck to our runners Liz and Fran from Down's today as they take on the big half around London - may they know of our support - race numbers are: 12785 and 4507 - they can be tracked and sent messages of support on the big half page this morning - if anyone is at home and able to send some messages I know they would appreciate it hugely. There are 2 further runners from Downs who I send my thanks to for taking part but I have no details of their names/numbers to give out I'm afraid. The next big milestone of £9000 has been hit, let's see how close we can get the bar to £10,000 in honour of the efforts of these 4 runners stepping out of their comfort zone for me - to them I am just a colleague's niece, we have never met, they really do deserve our support and thanks! x

Thank you for all donations, raffle and auction entries to date - I continue to be overwhelmed by everyone's generosity in times of adversity and when there are so many other appeals which also need help.

A quick update: Active fundraising has restarted with Downs Solicitors taking the baton forward with their running group who will next Sunday - 3rd September be running the Big Half starting at Tower Bridge, London and running the distance of a half marathon - please lend them your support through pledging further fundraising and support towards them - they would be so grateful also for anyone who is able to send virtual cheers to them through the Big Half's live radio system - more details to follow as I receive them. Donations for all runners are to be placed on here for simplicity purposes, thank you. And let's raise a huge Cheers to Down's! If anyone else feels hungry for a challenge please get in touch direct.

Currently I am not doing at all well medically, but we have been able to get me the home oxygen which is having a large impact on me in seizures - which are now approx 4-6 per day/night; pain remains a huge challenge for the doctors but they are doing all that they can to try to help - this was always going to be a challenge for any doctor due to the speed at which my body metabolizes medication but it is not for anybody's part in not trying. I have had some sessions of palliative counselling. We have been able to on some weeks - part time - move me back to my flat which makes me incredibly happy and reminds me this race is not over just yet; we have also found something that comes from America - "Fluff" - liquid marshmallow which I can tolerate to swallow (all other foods I can only chew and spit out, I cannot swallow) meaning that I have some strength to fight the increasing seizures with even though it is far from healthy, it also has the benefit of keeping my weight somewhat stable.... sadly it isn't a miracle and it doesn't make me feel better in myself, I am covered in bruises from having 'vacant seizures' - seizures in which I lose all sense of all around me - incredibly scary and stop me being able to call for help because I am 'vacant' - we hope a seizure alert mat is on it's way to help with alerting nurses that I need help. A temporary bespoke method for getting me in and out of my flat is also currently being made..... so whilst I may be far from well there are lots of good things happening. I am surrounded by family and friends as well as a good, London agency recruiting for more care staff for me to carry the load which can only be good, but costly; and the local hospice will be coming out next week to undertake their latest clinical review - we are ever hopeful for more support.

In the meantime please keep sending positive thoughts my way, and have your glasses ready to cheer on Team Downs Runners next Sunday - 4th September - Cheers!

(Please note - I am only 38 years old and receiving palliative care with a terminal prognosis. Most of the photos contained within this article were taken 2 years+ ago -they are not an accurate reflection of what I look like or can do today......)

I am Jen, I am 38 receiving palliative care for a terminal diagnosis - not expected to see the year out - I have Ehlers Danlos Syndrome (EDS) with several of it's co-morbiditities- all being linked and all having an effect on my life in some form, especially my Gastroparesis, my future looks fairly grave. Due to my gastroparesis I have been unable to eat solids in months - I am simply surviving on fluids which whilst one can do this for a relatively long time it is not a forever possibility, hence my prognosis. I am now seriously malnourished.

What is far worse is that due to lack of knowledge, expertise and resources the NHS cannot help to even make me comfortable, to relieve pain and other symptoms holistically which is required because of my allergies to medications due to my mast cell disorder.

Without the private medical world it is clear that my remaining time will be spent in anything but comfort. I am looking at being left in severe agony with pain from dislocations, increase of symptoms from my co-morbidities, experiencing infrequent states of hypoxia (lack of oxygen), and other overwhelming symptoms.

Privately there is some hope not for a cure but of being able to bring comfort through licensed holistic therapies unavaliable on the NHS to me during the time that I have left. Being private however this care all costs a great deal of money which I and my family can no longer fund alone hence I find myself in the very vulnerable place of having to ask friends, family and relative strangers to help me to raise as close to the £15K that is needed (a cost breakdown is available in my story of where this money will go). but I am left with no option - the sooner that these funds can be achieved, the sooner my anxieties over the future can be alleviated and I can hopefully then have the chance to go back to having 'fun' and spending quality time with those closest to me again.

It means the world to me that you have taken the time to visit my page and it would mean even more if you were able to help either by supporting me financially or simply by sharing my story on social media helping to reach far more people than I could ever reach alone thus increasing my chances of raising this all important money.

Thank you from the bottom of my heart for whatever you can do to help, whether it be a donation or simple sharing my page on your social media - it means more than I ever can put into words but will stop the horrific suffering at the hands of my condition as it progresses and the lack of NHS support/resources to control it.

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Please read on if you would like to understand in greater detail my journey and what the money will be spent on.......

My fight for survival was shared nationally 4 years ago when my spine and neck became so unstable through 2 completely unrelated accidents - causing muscles, tendons, ligaments etc to be ripped, for whiplash to occur and for my neck to become even more unstable than I had ever realised that it was. It became a case of raising as much money as quickly as possible for intensive physiotherapy treatments in the UK, artificial nutrition and PICC line insertion for pain relief and total paternal nutrition due to the nerves in my throat being unable to tolerate food textures passing against them.... plus mobility equipment, rigid neck collars etc to avoid me having to face financially crippling American surgery which would have rendered me disabled from hip to skull through the insertion of metal rods. To this day I remain reliant on my neck collar and continue being in a wheelchair but now full-time not just intermittently when my legs don't work or I'm tired but I am presently alive with an intact spinal cord and no urgent need for high-risk surgery. However through the whole ordeal I developed medical trauma as my conditions were repeatedly disbelieved in the UK - we have not kept up with other countries in the world and instead many EDS patients are disbelieved rather than helped - I was one of them.

Since that point I have fought back time and time again to live and to defy medical science - I have fought amongst others to restore what was felt would be permanent renal failure yet turned out to only be 48 hours, frequent seizures lasting up to 2 hours when most seizures cause brain damage after 30 minutes, a shortened life prognosis due to the Chiari Malformation Cranial and Atlanto-Axial Instability having potential to kill me as a result of a wrong move due to my spinal cord becoming ripped in 2; numerous anaphylaxis from my MCAS etc. but I am now at a juncture where it would seem that no medical intervention or consultant is able to reverse my condition of severe Gastroparesis - a condition related to EDS which sees one’s gut becoming paralysed and stops the body being able to digest food painlessly etc - I am in receipt of palliative care from a private GP as most of my care must come privately now because "I am too complex for the NHS"

I am appealing to you to help me to raise enough funds in the next couple of months to be able to live secure in the knowledge that I will be able to afford to live comfortably for as long as time allows and that I will not suffer needlessly. As a result of severe malnutrition and muscle loss I no longer have sufficient fine motor control or strength to grip onto small objects or even to put a mug away into a cupboard above my head; due to the muscle loss in my chest wall I am experiencing distressing and frequent bouts of hypoxia (lowered oxygen levels in the body) which cause me to become drowsy - slipping in and out of fitful sleep several times per day whilst also becoming very disorientated and unable to hold a conversation of any sense - life is incredibly scary right now and I have no idea what the future will look like as I decline further and lose further muscle tone / gain further symptoms. I have bones protruding over my body, I am dislocating at a far greater rate than ever before due to having less muscle tone to hold my bones in place - EDS causes dislocations; Gastroparesis is getting worse and I feel more nausea everyday. My seizures are becoming longer and more violent and my pain so severe that it requires the use of several interventions (reflexology, massage, specialist creams etc) some days to even begin to control it, something which the NHS do not have the resources or knowledge to fund so I have to get this privately at a cost close to £1.5K per month - I have been doing this for several months now but can no longer afford it on my own. I am 99% bedbound, relying on a commode and bed-baths for personal care, I have a care package for carers to look after me 24/7, and I am mostly wheelchair bound inside my flat and completely outside.

Yet the NHS still refuses to help - they simply don’t know how to - not enough research or resources have been put into investigating EDS because it is too rare a condition. As a consequence there are few NHS consultants left specialising in EDS due in the main to prescribing issues - in the UK you can only prescribe a drug on the NHS that has been approved through research for a number of years for a set purpose - just because EDS sufferers experience sickness and nausea that does not mean that an anti sickness drug researched for cancer patients can be prescribed even if it would be the best and most effective drug in the circumstances - as there is little research, few drugs are licensed for EDS... privately this barrier does not exist. And to make it worse very little new research is coming forward to help patients overcome their symptoms leaving us feeling that any that help on the NHS is not fast approaching - it will most probably be years before it does, for many like myself it will be too little, too late. Privately though a drug can be prescribed off license so long as it is of benefit and does no harm.

Privately therefore there are inevitably some small things which whilst they will never be a cure for the extreme level of my EDS, will enable me to at least be more comfortable but in the main off license treatments/medications - on my own I can only dream of being able to continue to afford these, my parents are pensioners with my Father equally unwell and potentially needing future health care - so they too are very limited in their own resources and so this is why and where I have to ask for your help again.

For those that do not know me - I struggle according to my GP with one of the most severe, disabling and complex cases of Ehlers Danlos Syndrome known about in the South-East of England. EDS damages the vagus nerve causing gastroparesis, affects all connective tissues, organs, muscles and blood vessels in the body due to a lack of collagen - it causes severe fatigue, severe muscle and joint pain, cognitive symptoms, sleep reversal as well as too much or too little sleep; nausea, migraines, heart palpitations, dangerously low blood pressure, seizures, amongst other skewed medical readings - it can in some individuals, as is the case with me become a multitude of related conditions; alongside it. I have gastroparesis - paralysis of the gut and due to a severe form of Mast Cell Activation disorder (MCAS) I cannot be tube fed. Due to being allergic to not only the material that the tubes are made from but also to the artificial nutrition that you would put inside said tubes - MCAS is a very real, dangerous and life limiting condition in its own right as it makes one allergic to environmental factors, medical procedures and interventions, foods, drinks, lotions, potions etc. it sees even the most simplest of things like tap water and food as being a threat to it - it can become deadly and in my case it is certainly turning that way - it is preventing me from having the nutrition that I need to survive; the treatment that I need to be able to slow down the paralysing of my gut and the treatment that I need to be able to stop me being allergic to almost everything.

Despite the resilience that my body has shown to date it does not take much more than common sense to realise that this time my time is most likely nearly up - science, medicine and biology all point to the fact that without food one cannot survive forever- it can survive for a long time with fluids alone but ultimately it needs nutrients. But potentially some new, little unheard of treatments, holistic therapies etc have the ability to make me comfortable during this time even if they cannot save me. None of them are available on the NHS though.

- Private, specialist, regular blood tests - today for example I had 17 tests done costing £300+

- Private, specialist, regular GP care (My NHS GP has not seen me in 2+ years, home visits with my private GP are costly)

- Regular twice-weekly specialist massage and reflexology appointments to relieve some of the most severe pain imaginable.

- Appropriate seizure treatments that could help to reduce the length of time that I am in a seizure for (currently up to 2 hours) and their frequency (currently several times per week) thus reducing chances of dislocations, further pain, anxiety and at worst brain death etc - this will require the oversight of a private neurologist with sufficient experience of my neck and spinal injuries to investigate and give me the most appropriate, potentially ground-breaking treatment available - it would be a dream for my seizures to abate, even just a little.

- Oversight by a private respiratory consultant to attempt to lessen my breathing problems and periods of hypoxia. The NHS have limited funds and resources to help long term breathing symptoms.

- Updates to my neck orthoses so that they are comfortable and fit me again now that I have lost so much weight.

- Regular palliative counselling for myself and my family to help us all deal with the situation.

- In time a suitable wheelchair upgrade which enables my wheelchair to be a model that is safe for travelling in - so that I can safely be transported very short distances - such as visiting my parent’s home.

- A car adaptation which enables me to stay in my wheelchair whilst being transported thus reducing unnecessary and painful transfers.

- Suitable ramp adaptations at my front door again eliminating unnecessary transfers.

- A suitable shower so that we do not have to make do with a makeshift shower solution which is not comfortable for me and sees my carers almost having a shower too!

I receive very light touch hospice care and they are currently installing a ceiling hoist so that when I can no longer weight bear at all there will already be equipment in place for me to able to be transferred to my commode or wheelchair. The hospice freely admits that after this installation they will be out of their depth and that they like the local NHS do not have the knowledge to properly deal with my conditions either - that is until I am approx. 2 weeks from the end of life which is nothing short of terrifying.

.......for considering to help me to make the impossible possible for as long a period as it can be for.

Thank you.

Jen and Family

What is EDS?

What is MCAS?

What is Gastroparesis?

What is CCI/AAI etc?