Post-surgery update on Jenny

We are in a state of shock and devastation.

Jenny immediately knew after the operation (an occipitocervical fusion and also a posterior fossa decompression) that something was very wrong but wasn’t able to communicate it for a while, especially while in the Critical Care Unit after the operation and having lost so much blood during the operation.

Her head and neck immediately felt more unstable, not less, but more critically than that, it felt that the place on her head/neck that she had been going on about for ages, was blocked and closed up (the place where, before the operation, if it was lifted and supported, she suddenly felt something open up at the base of the skull/top of the neck and blood/lymphatic fluid would start entering her head and neck and whole body in much larger amounts (normal amounts for ordinary people); the results were tangible – you could see her skin change colour and turn pink, and red streaks would appear on her skin that looked like blood vessels, where blood vessels that were used to having a reduced amount of blood were suddenly opening up from an increased amount of blood. She would also regain feeling in her tongue, nose, head, mouth and lips when the blood reached those points, regaining her ability to taste and smell; and her vision would also become less blurry when it reached her eyes). Jenny was always trying to find a way to find this place on her head/neck (it sometimes took her hours to find and it all depended on finding the correct angle to access it), that when lifted and supported slightly, had this effect. She assumed that she would wake up from surgery fixed in this position – with that specific spot supported by the fusion and blood flow returning to its correct amounts.

Not only was this not the case, the place on her head/neck where she used to lift up and support to get the increased amount of blood flow back had disappeared. Or at least, the surface area of the specific spot had been reduced to such a tiny dot that even when she does find it now, it doesn’t have the same effect and she doesn’t get that sudden ‘bam!’ of blood coming back. On top of this, whenever trying to sit up now, her skull feels jammed down and crushing against something, trapping the specific place in her head/neck that used to open up. It’s painful and feels like it’s doing damage.

After waking up after surgery, she was able to communicate that she had lost a substantial amount of her vision. She thinks this is because the previously mentioned place on her head/skull that, when lifted and supported, more blood used to get through to make her vision less blurry, is now more blocked and so the proper amount of blood isn’t getting through to her eyes. She’s no longer able to see more than blurry images so she’s not really able to use her phone to go on social media or watch television or films, which were her lifelines. She’s currently experimenting with font sizes to see if she can just about make out words.

Not only this, but now, whenever her head and neck aren’t supported in a specific position against a pillow/raised hospital bed, (i.e. whenever she tries to lift or move her head and support the weight of her skull and neck by herself), her head and neck begin uncontrollably, involuntarily jerking and twisting and yanking violently and rapidly in all directions, which not only is immensely painful and dangerous after surgery, but she can’t support her head or neck and they flop and jerk all over the place and she feels that her skull jams down on the wrong place on her neck, which feels like it’s cutting something off and also doing immense damage, grinding against something it’s not supposed to. Every time she moves her head or neck at all, she can hear a sort of grinding sound in her head. She’s described it as sounding like teeny tiny fragments of bone or “bone dust” crunching away, or maybe scraping metal.

Because of the violent uncontrollable movements when she tries to support her head and it crushing downwards whenever she tries to lift her head or sit up, she is no longer able to use the commode, which she used to do easily and unaided before the operation. This is a big blow to her and has affected her a lot. She now has to use bedpans, using them while lying down flat, and is dependent on other people to go to toilet. This has also made caring for her a lot more difficult, as she frequently needs to go to toilet throughout the day and night.

The trouble is, the supine MRI and CT venogram aren’t showing anything wrong with the operation, according to the neurosurgeon and radiographer. So Jenny has had to fight hard, while extremely ill and incapacitated after the operation, to try to get them to believe/understand that something is wrong. Unsuccessfully. The neurosurgeon is happy with his work, according to what he can see on the scans, and is convinced he has performed the operation perfectly.

They have sent Jenny home from hospital, saying that there’s nothing more that they can do for her. So we are suddenly left with a much more disabled daughter. At least she is now away from the hospital noise and trauma.

The degree of numbness that she experienced before the operation has increased all over her body and there are places on her head that she can’t feel at all now, which is distressing for her. We don’t know if maybe a nerve was nicked in the operation.

Jenny is frightened and keeps communicating “please help me”, “please fix this”. She is also mentally diminished (not so much that you’d notice if you’d never met her before but compared to what she was like before, for her it feels like a gaping chasm). She’s struggling with comprehension, finding the correct words for things and basic maths. This is very upsetting for her. It’s a huge loss on top of all the other losses.

The only plan of action that we had was to try to get an upright MRI instead of a supine one, because it’s when she lifts her head and sits up that all her problems increase even more and things are even more blocked and jammed at the back of her head/neck and the rapid involuntary movements start. But we heard back from the upright MRI scan place today and they say that it’s too unsafe at this moment in time after the operation for them to agree to do the scan, given the flexion and extension positions that they need patients to sustain during parts of the scan. So we’re stuck, unable to prove that there’s anything wrong.

We have all been left reeling and just trying to cope with the basics of surviving and her increased care needs. Jenny feels like she has lost everything and is distraught. She feels like she has let everyone down, especially after the incredible kindness and generosity that was shown to her during fundraising. She feels embarrassed.

We don’t know what to do now. There are only a few more neurosurgeons in the world (none of them in the UK) who do these operations on EDS patients. But it’s hard to know who to trust now. And turning to any of them would cost vast amounts of money. We would need to raise even more than we did before, by a very significant amount.

The only bright moment since the operation for Jenny was when she was attempting to “watch” (listening and trying to make out some of the blurry shapes) the episodes of Doctor Who that she missed while in hospital. In the most recent one, when she heard the beginnings of Captain Jack’s voice all of a sudden, she was smiling with joy and excitement at the surprise. He was back! So thank you Doctor Who and thank you John Barrowman.