One thing I’d say is a constant everyday every moment thought in our life with Amy is posture/transfers and equipment. Another constant is the fear of the unknown - what new and terrifying diagnosis lies waiting around the corner?
Very very early on in Amy’s life we were introduced to positioning. Making sure she’s upright enough that reflux is minimised. Not leaving her on one side too long. Rolling up towels and wedges to give her support and symmetry. It becomes second nature eventually as you try and ensure a healthy spine and hips.
Amy is now 9 years old. She has quadriplegic cerebral palsy. Her type is athetoid/dyskinetic. Whilst she is non-mobile, she has a severe movement disorder and moves constantly. The movements are sometimes referred to as dystonia/chorea/ballism/dyskinesia. She’s really strong and her muscle tone fluctuates - she can be super stiff one moment, and like a rag doll the next. She’s on quite a few medications to help with the pain and to help her gain some more control of her movements.
A few times a year we are invited to a cerebral palsy clinic with the orthopedic doctors.
They give her a thorough check over and send her for an x-ray. Thus far we have been so lucky that whilst her hips are tighter on one side - she is not currently displaying any signs of hip displacement or scoliosis. So far I have left pleased each time that things haven’t worsened. We have several friends who sadly have needed or need hip surgery, or who have scoliosis. It’s a devastating journey, fraught with some very difficult and unfair decision making. No one wants their child to need surgery or be in pain. Amy's constant movements though troublesome, do minimise her risk of things such as pressure sores, or being stuck in one position for too long in the night for example.
Everyday I say to myself hmm she has been in her chair a few hours… maybe we need to bob home so she can have a pad change and a stretch.. I don’t want to put too much pressure on her hips and have her sat uncomfortably for too long. Equally I may think oh gosh she’s been lay down a lot today. This isn't good for her chest or reflux, maybe she should go in her walker to help build up some strength etc.
It can be really difficult ensuring she has all of her postural needs met and I know that her school also work hard getting her in her standing frame plenty to help her bone density. I feel a daily guilt that we don't reintroduce her sleep system - she already has real difficulty sleeping and the last few attempts were a disaster!
So imagine my surprise last week when I learned that Amy actually has several small fractures in her spine!?
In December last year Amy was very poorly in PICU with a collapsed lung. As a follow up from this she had a CT scan under general anaesthetic to help us establish the severity of her lung damage. Amy’s chest infections have become worse and worse over the years and medically she is much more complex with it than before.
The idea of the scan was to see in detail what is happening in her lungs to help us build a better treatment plan for her.
The scan showed mild bronchiectasis. We expected this.
What we did not expect were the fractures.
We were told this is due to osteopenia (or low bone density). This can be quite common in non-mobile folks as they are not weight bearing as much, thus don’t have the same density or strength of bones.
I had heard osteopenia mentioned in relation to Amy before but didn’t pay it much attention as I knew she was having all of the right physio and until recently hadn’t sustained any fractures.
We have now been told that she will not be safe to have percussion chest physio and thus her treatment will be more reliant on nebulisers and suction. But even worse, she now needs to have IV bone infusions to help strengthen her bones.
I am so relieved she had this scan otherwise she would have been living with these painful fractures without us knowing. As Amy is nonverbal it can be really difficult to ascertain why she is distressed. Often you are treating it blindly hoping paracetamol or a position change will help. But it really made me sad to know that she is contending with this on top of everything else.
I am now in a position where I am not sure what to do for the best. Do we amp up the physio to help her bones. Or does she need more bedrest? We will be liaising with various specialists (endocrinology, respiratory, dieticians and chest physio) to make sure we get Amy exactly what she needs. We have a lower threshold now for giving pain relief, and we are to take much more care when doing transfers.
She apparently won't be in agony with the fractures but it will be causing her pain.
Sometimes it can be disheartening that in spite of your constant attempts to do the best thing, that things still go wrong. Each new diagnosis she receives I hope to myself that this is the last one. It seems sometimes that you all just can’t catch a break. For years we struggled with horrendous gastro issues. No sooner were they resolved we were dealing with life threatening uncontrollable epilepsy. Once the epilepsy was better managed then chest infections became a huge and constant issue.
It seems now that in our pursuit to help her lungs, we have found a new obstacle to tackle. In the process of trying to minimise interventions and medications we are very often thrust back into reality with a bang and made to consider even more of those things. I don't want to rant - I want to be mindful, and grateful, and happy - but equally no parent wants their child to be in pain or to suffer and we will move heaven and earth to make sure they get what they need for the best quality of life and happiness.
I am torn sometimes between accepting things as they are, and wanting to scream up at the sky "why her? why us?".
It all does seem very unfair at times. Why should our little girl ever have to suffer. I'm not sure Amy would call it suffering as she is a tough cookie and faces each day with a tenacity I have never seen in a person before... but part of me thinks that's because she has no other choice. She knows no different.
I know she and we will get through it. And we will continue our constant ‘umming and arring’ over positions, equipment, comfort and posture, and we will continue to hope for the best. We hope to extend the life of her lungs, to avoid as much as possible those difficult conversations relating to hip and spine operations.