UPDATE!!!

On the 15th of March Phil underwent two operations at the Tecknon hospital in Spain. His Drs, Dr Gillette and Dr Oliver, fused from C0-T1 on his spine, th

ats his whole neck to you and me! They also removed a small part of bone from his skull to allow the portion of his brain, that had dropped down into his neck, room so that it was no longer getting squished. That surgery took just over 10 hours. They then had a short break and went back in to release his tethered cord and do a small fusion at the base of his spine. Tethered cord is where the bottom of the spinal cord, which should be free in the spinal fluid, is attached to the spin by connective tissue. That part of the surgery took 2.5 hours! Phil had some serious complications with bleeding during the surgery which, thankfully I was unaware of at the time. After a rocky first week, where we discovered Phil does not do well on opiates, the Drs decided to take him off all but his paracetamol and Ibuprofen. At this point Phil fully woke up, in pain but glad to be with it he decided not to take any strong pain meds and remain alert and start his recovery.

Amazingly, within a day of waking properly, he was up on his feet and able to sit for a short time in a chair. From there things have just continued to improve. The surgery to his neck, we knew, was life threatening but after the surgery the drs were very clear how serious his condition had been. He is still in a lot of pain from the surgery due to the amount of muscle they had to cut through and to a number of nerves close to those muscles deciding to fire. So, currently he doesn't have full range of movement in his arms and he is finding holding his head up for any length of time is tiring (its now a lot heavier!!) but his neck is stable and now does not pose a threat to his life, which was the aim of the surgery.

The releasing of the tethered cord has had dramatic results! For the first time in a decade Phil has no pain or numbness in his legs, feet hands or arms. After five years of very limited mobility, he will need to work hard over the coming year to regain his strength and stamina but we are hopeful that he will be able to drive and lead a relatively normal life within 18 months. Another result was that all urinary symptoms have also disappeared, some he has had all his life, his friends are well aware of what we fondly call the Clark Bladder,lol! Well it exists no more!!

For anyone finding this page who may be thinking of having the surgery it is important to know that Phil can not move his head, see his feet, look up or see what is next to him. This makes everyday tasks difficult, try eating sitting upright and not seeing your plate!! The bone grafts take upto 18 months to grow and fuse successfully so the recovery period is long. He can not open his mouth wide , try holding your head still in a neutral position and open your mouth with out moving your head. Your jaw does not go far so eating hamburgers and going to the dentist is no longer enjoyable!! Also, he can no longer be ventilated,if needed, in the standard fashion which make future surgery and emergencies difficult. Depending on where you live long term the pain in the bones of the neck may be worse, the UK is cold and wet most of the time. These are important things to consider even when your life depends on the surgery and it is not an option to not have it.

For now we are all as a family learning to appreciate the improvements and come to terms with the limitations of Phil being unable to move his neck or head. As you can appreciate this is difficult for the children and Phil to get used to but with time they will.

My Hero

Fifteen years ago I married the love of my life, we'd met at university, and our adventure together began. We were nothing alike, I had grown up in the middle of Swansea, Wales, and Phil had grown up in the middle of a common in rural Cumbria, near a small town called Ulverston. At first, activities like climbing Coniston Old Man on a Sunday afternoon were so far out of my comfort zone but slowly with Phils support I learned the skills I needed to enjoy the active, outdoor life that was normal for him. By the time our first of our three gorgeous kids was born, I was confident enough to think nothing of slinging him in a carrier and taking him up some hill or mountain with Phil at my side.

Our Eldest, Owain is 13 now and following in his dads footsteps. He is at the top of Coniston Old Man here.

University was never the place for Phil and accounting never stuck but he forged his own way becoming a windsurfing instructor, lifeguard, personal fitness trainer and then finally plumber. That was until about ten years ago. At first, he experienced what he thought were shin splints that became so bad he had to stop running. Then the pain began affecting his foot so much so that he feared he'd be unable to carry on plumbing and so he went back to the Drs. for help and took a Christmas job at an Amazon warehouse to ensure we had a backup plan. Fast forward two years and he was working full time in Amazon, as the pain was now affecting his thighs and calves and he was unable to work as a plumber with no idea what was wrong with him. However, our then 6 year old daughter was our priority. At six she was unable to walk for longer than five mins and that was painful, her elbows, shoulders and hips caused her a lot of pain due to them subluxing (popping in and out of place, she had no bladder control and was constantly in pain and extremely tired. At this point we were living in Wales and battling a local system that kept refusing to allow either Phil or Rhiannon access to medical expertise over the border in England. Thankfully Amazon had provided Phil with health care for the family and we used this to get Rhiannon to London where she was diagnosed with Elhers Danlos Hypermobility type (learn more here https://www.youtube.com/watch?v=6kgcDyqcF08). It is an incurable, genetic disorder of the collagen (the stuff that holds your body together) and finally we had an answer for what was happening to Phil, why my eldest suffered with knee pain and headaches, why my daughter was in so much pain and experienced so much fatigue and why later, my youngest would wake up at nights screaming in pain.

Now, we have a good understanding of what is happening to my husband. EDS can not be cured but in many cases can be managed with physio therapy, to strengthen the muscles to help them cope better with the stress of doing their own work and the work of the ligaments (they hold your body together), pain management and pacing of activities. Phil threw himself into everything his Drs asked him to do, he was determined to show the kids that they could have an amazing life and to teach them good ways of coping. Unfortunately, his condition worsened to the point where he had to stop working. At this point he still had no NHS specialists or Drs and his GP just kept giving him more painkillers to take, that never worked and had horrible side effects. Thankfully, we were able to use his health insurance to see a number of other specialists in England and to cut a long story short he happens to be one of the unlucky EDS suffers who also suffer from Cranial and Cervical instability and Chiari Malformation.

Basically the soft tissue in his neck is unable to support the weight of his head, or to restrict movement as it should. This means that during everyday movements the bones in Phils neck are moving in such a way as to rub his spinal cord. This has created a whole host of symptoms from excruciating pain in lower limbs, sever headaches, numbness and pain in arms and hands (he has limited use of his right hand), inability to control his own body temperature, mood swings, sleep apnea and restless leg syndrome and at present can on good days manage short distances using walking poles and longer distances using a mobility scooter (although he is beginning to struggle with the scooter due to the vibrations and the pain he experiences after using it). Some of these symptoms are made worse by the Chiari malformation, which is where the ligament at the base of his skull is too soft to hold his brain in his head so a small portion has dropped down into his neck. This section gets a bit squished when his neck is moving around.

So to where we are now. Phil has seen many Drs who have confirmed his diagnosis. Recently, he managed to get an appointment with a specialist in London who is trying to help people in this country with these conditions. The Dr again confirmed what was going on and that he needed surgery to stabilise his neck, if his neck stays still then there will be no more damage and his symptoms should settle; he will get a new but stable normal. He was very clear that the only option was having the spinal fusion surgery and that if he did not have it then the symptoms would progress to paralysis, organ failure and then finally, if he survives that long, internal decapitation. His own body will severe the spinal cord. This really was not in our five year plan!! The issue now is that there are no Drs in the UK performing the surgery on patients with EDS. There are Drs trying to present a case to the NHS to begin offering the surgery in the future, which is great, but not in time for Phil. So, we are left like others in this country with three options, do nothing and watch my husband die, go to Spain for the surgery or go to American for the surgery (where they have been doing it successfully for many years).

I can not face the thought of bringing up my three beautiful children knowing what they have to face medically now and in the future without my partner by my side, thinking that if only we had been richer and had more money he could have had the surgery abroad. At the moment, Amazon are in the process of medically retiring Phil and we will lose all access to the healthcare plan. I am his, and our daughters, full-time carer and so we have no income right now. We have moved into a converted bungalow that Phils parents own in Cumbria, to have better access to NHS healthcare and to give us some security and stability for the kids. For us raising the £55000 for the surgery, flights and accommodation seems impossible, it is an unimaginable amount and that is why we are asking for your help. Before we can even book a surgery we need to have a 10% deposit and realistically, before we pay that we need to know we can pay for the full surgery and flights and accommodation for the months stay it requires in Spain. My family would be forever grateful if you could help us by donating or by organising a fund-raising event that we can support you with. We may not be able to do this alone but with your help I know we can.

A huge thank you for taking the time to read this from me, Phil and this adventurous bunch of bendy people!!