Ruban's Wish To Walk
Thank you for taking the time to visit my fundraising campaign page.
My name is Ruban 'The Legend' Selley. I am six years old.
When I was in my Mummy's tummy, everything was going OK. Mummy, Daddy, Nanny and my big brother Mason would often talk to me and rub Mummy's tummy and tell me all about how life will be when I arrive. None of the scans picked up on any of my 'Quirks'.
I made my entrance 3 weeks early on the 8th of June and changed everyone's lives forever. My family would tell me I was perfect, and the only thing that was picked up was that my eyes weren't quite right. Nobody knew if I could see anything because I had clouding over my corneas. Mummy, Daddy and Nanny knew from then that my life story would be different to the one they told me about inside Mummy's tummy... But they knew that even though it may be a different story, it would be just as amazing, when on that very first day I was born I gave them a very big smile.
At 8 months of age, I was admitted into hospital for 6 nights, with a severe case of Bronchiolitis and a chest infection. My Mummy and Daddy believe that this was due to my undiagnosed Dysphagia, which was causing me to choke on my fluids. I had a feeding tube, due to dehydration and was on oxygen. I was very close to being put into induced sleep, for aided breathing.
But like everything else, I surprised the doctors by fighting it and smiling as soon as I had the strength.
After many hospital visits, Mummy and Daddy made the difficult decision to have the lens in my left eye removed at 9 months of age, to remove cataracts and dead blood vessels attached to my lens. The hope was to clear the way for light into my eye and improve my vision. Unfortunately this operation was unsuccessful, as the lens could not be reattached, due to the size and shape of my eye and the damage to the lens by the clouding.
This procedure left me with Glaucoma (high pressure in my eye) and means I must have daily eye drops, otherwise I could lose my eye.
I am partially sighted. My Opthalmologist thinks that I may be able to make out light and dark in my left eye, but not shapes and objects. I am in effect blind in one eye.
I also have something called Nystagmus in both eyes, which means my eyes shake uncontrollably. This gets worse when I am tired, or distressed.
From a very young age I learned how to find the 'Null Point', where I turn my head, to stabilise my good eye and minimise the shaking. But this also means that I get pain in my neck after a while, due to my unusual head position. This is known as Ocular CHP (Compensatory Head Position).
I also suffer from Photophobia, which means my eyes are very sensitive to sunlight and bright indoor lights. Specialists liken the combination of this with my other eye conditions, to driving a car over very bumpy ground and trying to look through the dirty windscreen with blinding sunlight shining through!
I showed my Mummy, Daddy and Nanny just how special and unique I was when I wasn't reaching any milestones. I tried to sit up, but no matter how much Mummy and Daddy helped me I just couldn't do it. My body wasn't doing what my mind was telling it to do. Mummy and Daddy thought that I might have spasticity in my legs and 'scissoring' (inwards pointing feet), but the professionals kept telling them that I was just still very tight and to just massage my feet. But nothing helped and finally I got to meet my Physiotherapist who said that I have something called High Muscle Tone.
Since then I've brightened the lives of many other professionals, including my Neurologist. I must have a brain MRI scan under general anaesthetic, every six months, to monitor my brain.
This is because the first scan revealed two separate, rare brain conditions:
The first one is something called ACC (Agenesis of the Corpus Collosum). It means that the part of my brain that connects the two hemispheres together is slightly missing. Mummy and Daddy believe that this is the cause of many of my symptoms, especially my balance.
The second one is Chiari-1 Malformation (which it has now been diagnosed as Chiari-2 Malformation). This is where the bottom of my brain is sinking into my spinal canal. This means that I get intense and extremely painful pressure headaches when I laugh, cry, cough or play for a while. Mummy and Daddy believe that this is why as a baby, I would cry until I passed out - because of the pain.
I have learned how to try and stay calm, take deep breaths and relax when the headaches come. But I still cry - and sometimes I might get several headaches in a day, especially if I am unwell, with a cough or cold. Also, Mummy, Daddy and my big brother Mason have to stop me if I am getting too excited and doing what all little boys get up to.
My Mummy and Daddy increasingly believe that I actually have Chiari-2 Malformation, which will need diagnosing. This condition has many more symptoms than Chiari-1, which I am showing. Fortunately it's not apparent that I have Chiari-3, which is life-limiting.
In 2018, I had Fornum Magnum Brain Decompression surgery, where I was put to sleep and a team of Neurosurgeons spent three and a half hours taking my headaches away.
They opened my skull, removing a part of it. They chipped some bone away from the inside. Then they removed the top vertebrae from my neck, then scored the Dura (protective layer of my brain). This is all to make room for my brain and reduce pressure.
I surprised everyone with my fast recovery and for a couple of weeks, I didn't have any pain. However the headaches did come back and they were worse and more frequent than before. This continued for a few months. I had another brain MRI scan in Februry 2019, which we are waiting on the results from.
My headaches only come back when I have a cough, or if I am crying a lot due to being upset or frustrated. My Mummy, Daddy and one-to-one teaching assistant at school all know how is best to stop this happening.
I must have regular Botox injections in my saliva glands, to control my excess saliva production. For this, I am given yucky liquid which makes me very sleepy and forget what happens after. I have these every 6 months.
In late 2018, I had Soft Tissue Release surgery, where my surgeon at Evelina Hospital, Doctor Gough cut the muscles in my hamstrings, adductors (back and inside of my thighs) and calves. This reduced some tension in my legs and has enabled me to sit more comfortably and use toys that I couldn't use before. Also my muscles have been strengthened, so that I can get better results with my physiotherapy. I have even been able to sit on my Daddy's shoulders and sit at school with my legs crossed - both things that my friends can do that I couldn't before.
This procedure should also reduce the risk of my hips dislocating.
Because my combination of symptoms is so rare, my Mummy and Daddy were asked to volunteer me to have blood samples taken for genetics testing. My DNA was tested in something called the 100,000 Genomes Project. This is a groundbreaking global science project involving cutting edge technology and is the first of it's kind. I may be able to help other children in the future!
In 2019, my Mummy and Daddy were given the results from this project. I have a condition called Syndromic Micropthalmia Type-12.
This condition is so rare that I am only the fourth person ever to be registered with it.
Here is a list of the 'Quirks' that make me 'The Legend':
- Peters Anomaly A.S.D.A. (which is my diagnosed eye condition).
- ACC - Agenesis of the Corpus Collosum (the white matter of my brain, in the centre of the left and right hemispheres did not form properly).
- Chiari-1 Malformation (the back of my brain goes beyond the base of my skull, where it should not - this can cause extreme headaches and irreversible stiffness of the arms, requiring Brain Decompression Surgery (which I have recently had done).
- Four-Limb Motor Disorder, equivalent to Cerebral Palsy GMFCS Level 4 (this is the way my arms and legs move about - very similar to someone with Cerebral Palsy).
- Photophobia (my eyes are extremely sensitive to sunlight and bright light).
- Stigmatism - my eyes are an unusual shape, which means that lenses must be adapted (and one of the reasons why my own lens could not be reattached during my eye surgery).
- Nystagmus (my eyes shake uncontrollably - from a very young age I learned how to position my head, to find the 'Null Point', where I could stabilise my good eye to get better vision).
- Ocular CHP (compensatory head posture - I have to hold my head in a certain position to be able to see properly with my good eye).
- Glaucoma (I have pressure problems in my left eye which I need daily drops for, otherwise I could lose the eye).
- Elliptical pupil (the pupil in my right eye (the good one) is shaped like a cats eye. You have to get close to see it though!). The pupil in my left eye is fully dilated and stays that way all the time.
- Dysphagia IDDSI Level 3 (I choke on liquids, so need to have special thickener powder added to my water bottle. I cannot eat mixed consistency food, like my favourite fruit - strawberries. I only drink water and it must be thickened to custard-like consistency. In the summer of 2018, I almost had a stomach peg fitted (so that liquid would bypass my windpipe and go straight into my stomach). But luckily I was prescribed a new type of liquid thickener, which so far has not made me cough too much and I've gone the longest time without a chest infection.
- Sialorrhea (hyperactive saliva glands, due to poor muscle control). This puts me at an increased risk of choking.
- Global Development Delay (this is where I learn everything a little bit slower than other children).
- Motor Dysfunction (this is where my muscles do not function the way they should).
- Axial Hypotonia (this is my lack of stability and balance in my trunk area).
- Dystonia (the mixed muscle tone in my hips, legs and feet - and also in my shoulders).
- Chronic Consipation (due to my increased muscle tone, I have special powder put into my drink before each bedtime. If I miss any, I have very traumatic and painful number twos).
My Mummy and Daddy set up for me an official charity campaign with Tree Of Hope children's charity (treeofhope.org.uk/rubanswishtowalk) and a fundraising campaign with JustGiving (justgiving.com/rubanswishtowalksdr), in order to raise funds for my cause, which I will detail below.
The original goal of the campaign was to raise enough funds to enable me to have SDR (Selective Dorsal Rhizotomy) and vital Physiotherapy and aftercare in the months after.
SDR is an invasive surgical procedure which will remove the spasticity in my legs (but not my arms, which I have to a lesser degree).
The procedure has a 100% success rate, but requires months of intensive physiotherapy afterwards, due to the muscles then being completely the opposite of their original tensed state (high muscle tone). My whole family were so happy when they discovered this procedure - and saw how it has helped some of my friends, who I met at Little Stars disabled children's playgroup (which has now had to close down due to the removal of government funding).
This surgery and the physiotherapy and aftercare following it should allow me to walk, reducing the pain, discomfort and tiredness associated with constant high muscle tone.
It should also prevent further complications and therefore the need for numerous very painful operations on my hips and legs in the future.
With the extensive and intensive Physiotherapy, and aftercare I will learn to walk from scratch. My legs will never be the same as other children's, but I will eventually be able to walk, play and maybe even run and jump with other children, hopefully without the aid of a walking frame.
So the goal of the fundraising campaign has now changed - due to the circumstances, I need to have extensive Physiotherapy, which can be funded by my Tree Of Hope charity campaign, from the money that all of my kind supporters have raised and donated so far. My Mummy and Daddy have looked into this and have found a couple of Physiotherapists which specialise in disabled children. These are KTB Physio in Bexley and the Freddie Farmer Foundation in Bromley.
In 2018 the SDR procedure (but not the Physiotherapy and aftercare) became available on the NHS, to children whose parents/guardians could prove that they had the funds available for the vital Physiotherapy and aftercare. So there is no need to raise funds for SDR surgery.
If I am able to have the SDR surgery on the NHS, my fundraising campaign will also be for the extensive Physiotherapy and aftercare needed following the procedure.
So my fundraising campaign is for the following:
- Extensive and intensive pre-op Physiotherapy, to get me to a point where surgeons can determine if I am a suitable candidate for SDR surgery. This takes place at KTB Physio, Bexley @ £60.00 per hour and Freddie Farmer Foundation, Bromley @ £30.00 per hour.
- Extensive and intensive post-op Physiotherapy, aftercare and any necessary medical equipment to aid my rehabilitation (if I am suitable for, and have the SDR surgery).
- Ongoing Physiotherapy and any related treatment, any necessary medical equipment and therapy to help improve my quality of life (if I am not suitable for the SDR surgery).
However, I do have a long and difficult road ahead of me. I must have lots of physically demanding Physiotherapy.
Only then, will we know if I can have the SDR surgery to help me to walk.
If I cannot have the SDR surgery, my chance of walking, playing and maybe even running and jumping like my friends will be snatched away from me. That means my life will become more difficult and most likely more painful as time goes on.
In that worst case scenario, my fundraising campaign will be to try and make me as comfortable as possible, so I will still need all the help and support that I can get.
Please visit my Facebook page 'Ruban's Wish To Walk' regularly and follow me on Twitter @RubanTheLegend, to stay up to date with my progress. Please do not feel expected to donate. Spreading the word for me and increasing the reach of this cause is just as important.
Thank you from my Mummy (Becki), Daddy (Darren), big brother aged 11 (Mason), baby brother Koah, Nanny (Karen) and of course the 'Legend' myself, Ruban xxx
Now over to Mummy and Daddy, for the final word:
Thank you for taking the time to read about our beautiful boy, Ruban.
We are lucky enough to live in a country where interventions can be made, albeit limited on the NHS. We are even luckier to live in a time where we can build up a large following of kind supporters, donors and fundraisers, who really can make Ruban's wish to walk come true.
Darren, Becki, Mason, Koah, Karen and Ruban 'The Legend' x
Should we exceed the target amount, or if we do not raise enough funds, or if they cannot be used for any other reason, the funds raised will go to the general funds of Tree Of Hope to assist other sick children.