This is a story we believe many people are living through, right now.
It is sadly a desperate plea for financial help. But it’s more than that. It’s a statement on the position that the Department for Work and Pensions (DWP) and NHS leave hundreds, possibly thousands, of people across the UK in. This is just one example.
My partner has to go to Spain for life changing surgery. But it costs. The initial amount needed to begin the process is £8,000.
You can donate via PayPal at http://www.paypal.me/NicolaCJeffery
Read below to find out why we’re having to do this.
I wouldn’t wish chronic ill health on anyone. But if people could spend a day in my partner Nicola Jeffery’s body, then maybe the world would be a very different place.
She lives with 16 different illnesses and conditions. You can read more about that here. But sadly the UK’s public health service doesn’t treat many of these. Some of the ones it either doesn’t deal with, or doesn’t treat correctly, are:
- Myalgic encephalomyelitis (ME).
- Hypermobile Ehlers-Danlos syndrome (hEDS), which has led to craniocervical instability (CCI) and atlantoaxial instability (AAI).
- Mycotoxicosis [pdf doc].
I gave up work full time to care for her. Since then, the DWP awarded her just the standard daily living component of Personal Independence Payment (PIP). You can read more about that here. We sent them a Mandatory Reconsideration. It’s been 11 weeks and counting for its decision. My claim for Carer’s Allowance has now been seven weeks, complicated by the fact I am self-employed. Because of the waiting on these we cannot apply for any housing support. We are now in a very dire situation with no seeming way out.
A ‘thank you’
We’ve been fundraising to continue Nic’s ME treatment under the care of the amazing Dr Sarah Myhill.
We raised enough to buy the majority of her ME treatment for six months, so thank you. This cost just over £1,800 in the end.
This is just about it, excluding the £500 worth of antivirals and £400 worth of mycotoxins treatment (because both are prescription only):
As an example, this is the cost of six months worth of thyroid and adrenal hormone replacements:
I note some people have said on Twitter ‘why can’t the NHS provide these?’. Firstly, because the NHS repeatedly claimed she had no thyroid issues. This, after proper evaluation, was not the case. Secondly, if it did diagnose it, Nic would be given chemical substitutes, which we do not believe in. The hormone replacements she is on are totally natural (bovine and porcine) – and her blood work has shown they have had the same effect as their chemical counterparts.
We are confident this regime will resolve Nic’s ME. We’re loathed to go into detail until the course is over, but suffice to say the signs so far have been good.
What we can say is that she was living with polycystic ovary syndrome, first diagnosed in 2009. But since starting Dr Myhill’s regime, this has been completely cured. Effectively, the regime for ME has also cured this other illness.
The NHS, meanwhile, says polycystic ovary syndrome “cannot be cured”. We think we know why it has with Nic, and it is absolutely unbelievable when compared to what the NHS offers for those living with it.
Sadly, running parallel to this has been a severe deterioration in her EDS-related CCI and AAI.
‘Rare’, or ‘rarely diagnosed’?
We first discovered Nic was living with these in March. I’d suspected as such, as she was symptomatic of them. So we had a £1,250 upright MRI (not available on the NHS and the only way to properly diagnose the conditions). Then, a Spanish surgeon interpreted the results and confirmed both.
As MEpedia describes, CCI is:
a pathological condition of increased mobility at the craniocervical junction, the area where the skull meets the spine. In CCI the ligamentous connections of the craniocervical junction can be stretched, weakened or ruptured. This can lead to compression of the brain stem, upper spinal cord, or cerebellum and result in myelopathy, neck pain and a range of other symptoms.
CCI usually develops as a result of physical trauma such as a car accident, an inflammatory disease such as rheumatoid arthritis or a congenital disorder such as Down’s syndrome. More recently, physicians have reported an increased prevalence of CCI in patients with hereditary disorders of connective tissue such as… (EDS).
AAI is kind of the same. The main difference between the two is which vertebrae are involved.
In short with both, the ligaments holding her top four vertebrae (the cervical junction) in place are floppy due to the EDS. So, the vertebrae are not held in place properly. They move in ways in which they shouldn’t, interfering with many of the nerves that come out of that part of the spine.
We know she has a 42° overshoot of her C1 over her C2 (her top two vertebrae). It should only be 35°. She has brain stem compression due to odontoid displacement (a piece of bone that allows the C1 to pivot on the C2). We know that her facet joints sublax (partially dislocate) on turning her head to both sides.
The effects of her CCI and AAI are systemic and overarching. Vomiting, seizures, difficulty swallowing, loss of bladder control, cardiac and pulmonary dysfunction and weakness in her muscles and joints to name but a few.
Overarching, systemic effects
Nic has numerous conditions and symptoms which I believe the instabilities are responsible for:
- Gastroparesis and dumping syndrome. These are conditions which seem to alternate in her. The former is where the stomach doesn’t empty properly, the latter where it empties too quickly. Both cause various, severe symptoms like uncontrollable vomiting. They are both probably related to brain stem compression; specifically the vagus nerve. She was free of gastroparesis from August until October 19, when it returned with a vengeance. This was the longest she had been without it. We believe it came back partly due to a strain on her cervical junction which at first resulted in severe pain extending into her thoracic area and down her left arm. But it also flares up whenever she has a ‘crash’ due to the ME, which she also appeared to have. I think the process here is this: any form of exertion results in post-exertional malaise (PEM) due to the ME. This, in part, impairs her already compromised mitochondria function (due to the ME), thus disrupting energy delivery. Therefore, any underlying EDS/CCI/AAI-related issues (like impairment of stomach muscle/nerve function in gastroparesis) are made even worse. Hence when Nic has PEM, her gastroparesis flares up. This time, the additional issues with her cervical junction appear to have been the trigger of it.
- Dysphagia. This is where swallowing is difficult due to nerve and muscle dysfunction. Again, this is probably related to vagus or accessory nerve interference in the brain stem or cervical junction.
- Focal autonomic/impaired awareness and tonic clonic seizures. A neurocardiologist agreed with my theory that the below is likely to be the process which leads to her seizures; in my opinion, once again at the root of this is brain stem compression as the heart’s sinoatrial node’s rate of production is ultimately controlled by the vagus nerve:
- I can accurately predict (with an 89% confidence rate) when she will have a seizure. This is due to an average 11% drop (versus her usual mean) in both her systolic and diastolic blood pressures the preceding night.
- Postural orthostatic tachycardia syndrome (POTS). This is where the heart fails to respond properly when posture is changed. It causes dizziness and imbalance. This could be caused by interference with the vagus nerve, which controls the parasympathetic aspects of the heart; essentially heart rate at rest. In short, the baroreceptor reflex is not being communicated to the medulla oblongata in the brain stem correctly. This causes her heart rate to remain increased after going from a supine/sitting to standing position.
- Peripheral neuropathy. This is loss of sensitivity in her extremities, like hands and feet.
- Cerebrospinal fluid (CSF) leak. This is where the fluid that cushions the brain and/or spine leaks out due to a tear in one of the duras (membranes). It happened in August during the postictal stage of a tonic clonic seizure. Nic projected herself out of bed, hitting her head and neck on the dressing table. Kings A&E said it was rhinosinusitis. We had to see a private neurologist to confirm the leak. The symptoms passed by the middle of October. But the leak showed that the vulnerability of her cervical junction has increased, as this was the first time she has had one.
- Reflex micturition/neurogenic bladder. This is an increased need to urinate and a degree of loss of bladder control, which has manifested more since the CSF leak. Nic has to urinate around once an hour, sometimes more – even though her fluid intake is less than mine. Whether this relates to the brain stem compression is more complex. If it does, it may be due to signals from her bladder’s afferent nerves being miscommunicated. Or, it may be faults in the hypogastric/pelvic/pudendal nerves. But if it’s the latter, that may be an indication of a fault lower down her spine; specifically tethered cord syndrome which we need to get her checked for.
- Cervical radiculopathy. This is nerve interference in the cervical junction, which causes pain to radiate from this area to other parts of the body (depending on what nerves are compromised).
- Chronotropic incompetence. This is where Nic’s heart rate does not increase appropriately during exercise. I believe this is caused by interference with the cardiac plexus via the sympathetic trunk and/or left vagus nerve; this controls heart rate over 100bpm. But this could also be related to hormone dysfunction due to the ME, as well as the vagus-controlled parasympathetic response to initial heart rate increase.
But Nic’s muscle and joint strength has also deteriorated. It has got to the stage where she sometimes has to use a wheelchair to go out. This is because the weakness in her legs and lower back pain means she cannot walk more than a few metres without having to stop or being in pain. Sadly, one of only a handful of neurosurgeons in the UK who understand the instabilities didn’t think her weakness was bad enough to warrant surgery on her cervical junction.
What he failed to realise is that Nic practised karate to a proficient level as a child/early teen. This has meant that her strength started out as being more than your average patient. So, what the neurosurgeon considers to be strong now, is actually a worsening by Nic’s standards. In this case, one size doesn’t fit all.
Off to Spain
So, we have to begin the process of potential surgery in Spain. Why Spain? Because the NHS doesn’t recognise the CCI or AAI, therefore there are no treatment options available.
This surgery will cost anywhere between £60,000-£200,000, depending on the extent of what Nic has to have done. This will be a separate fundraiser. In short, the surgery involves fusing/screwing her vertebrae together to hold them in place. This is not without it’s own serious complications and risks.
To do this, we have to have more non-NHS tests done, like a 3D CT of her cervical spine, MR angio/venogram, cineradiography flexion/extension and CT of her lower spine to check for tethered cord syndrome. This will be around £1,200 plus consultation/interpretation fees. Then we will need to travel to Barcelona for a face-to-face consultation with the surgeon. The cost of the appointment is around €300 plus travel costs. Nic can’t fly with her instabilities due to the risks posed by pressure changes, so boat or train it will have to be. This makes the trip even more expensive.
We also need to have her genetic testing for Ehlers-Danlos done. This is because there is now a question mark over her subtype. It may be classical. Ultimately, we want Nicola to be tested so her son can then get the diagnoses he needs. This is because EDS’ are inherited conditions. A positive genetic test for her means he won’t have to endure the years of being told his symptoms (some of which he already has) are ‘all in his head’, as his mother did. We don’t want him to suffer in the same way Nicola has. This will be £2,000 each.
UPDATE: for those asking why the NHS cannot help with this. Nicola’s EDS diagnosis has all been done privately, due to two-year NHS waiting lists. The EDS specialist at NHS UCH is now no longer taking patients. This is who we saw privately. We’ve tried to get the NHS to do the genetic testing. But the request from the GP just got bounced back, as did my own request direct to the centre that does the testing.
The additional spinal scans are not available. This is because the NHS does not even recognise Nic’s CCI and AAI. Moreover, if you want cases in point of how the NHS views people who have had spinal fusion surgery abroad, check out my podcasts with Samantha Smith and Victoria Cheney:
CanaryPod: Topple Uncaged meets… #SaveSamantha
All in all, we think this is going to cost around £8,000 to begin with.
It is a damning indictment of the NHS that any of this has to be private, let alone abroad, in the first place. But Nic is not alone. Google “craniocervical instability” and look under “news” and you’ll see countless other people in her situation. All are fundraising as the NHS doesn’t help.
It’s also a damning indictment of the DWP that the instabilities, plus all Nic’s other illnesses, only ‘deserve’ £58.70 a week.
We hope that once the ME, CCI, AAI and mycotoxicosis (more on that in another article) are resolved as best they can be, Nic can hit the ‘reboot’ button on her life. She has never known being a mother, or an adult, without chronic illness. A whole world of possibilities awaits her, if we can just get her well.
So, once again – if you can help please, please do.
I want to give her some quality of life back. But I need your help to do it. Please donate what you can via:
Hi Nic and Steve. I’ve just donated, all be it small,after my daughter (19) sent me the link. We’re on the same path for her! Not as chronic as yet but 3 years on fighting to get support/help before it gets worse, we think related to her idiopathic scoliosis (spinal correction in 2014). She’s got the ME/CFS diagnosis then a goodbye. First private appointment booked December. We hope your journey is safe and reaches a positive recovery!All good wishes and here’s to the NHS & medical fraternity taking heed and running with the overwhelming evidence.