Year in review (and some medical updates)

The little lady’s birthday is nearly upon us.  Freya spent last night at hospital having a sleep study to check up how she is doing respiratory-wise. It’s a bit of time since our admission at the respiratory wards and everyone was impressed how well Freya is doing – with the big two looming in front of us it made me think the way we have come and where we are. We all got our fair share of hugs – Freya enjoying all the attention and love she was getting from the team that works so hard last year to get her well enough to come home.

It’s been a good year medically

As opposed to Freya’s first year when she had some serious wobbles we have only had three acute intensive care admissions, all lasting less than a week. Freya’s had three operations (all on her jaw) and although her jaw has improved we’ve learnt to expect delays and prepare for setbacks – improving a complex airway and a little jaw is a difficult and a timeconsuming task.

It has also brought it home to us that the trachy (and the vent) is here to stay for the time being.  I had long chats with both the consultant from the sleep unit and Freya’s respiratory consultant. Both agree that although Freya is doing well respiratory-wise she needs her trusted ventilator at night to help her rest properly and have energy during the day (and oh boy, she has tons of it). Freya has rather unconventional design in her chest and lungs and the respirator is going to help her develop and grow in these areas.

I cannot but feel a little deflated as our target of getting rid of trachy and vent seems to be slipping further out of our grasp. It means we will continue living this highly medicalised life for years to come. It means continuing living with the restrictions it imposes us will still be here – it will affect our careers, finances and our ability to travel (something that I have really been missing recently). I feel battle weary having spent best part of two years fighting to get things in place for Freya and my heart sinks at the thought of hardship coming our direction by way most recent wave of cuts affecting both hospital funding in our area and significant cuts to continuing care at home (https://www.theguardian.com/society/2017/jun/20/leak-shows-devastating-impact-of-planned-nhs-cuts-in-london?CMP=fb_gu).

But there are some real benefits to the ventilator too – it helps us keep Freya healthy and growing. It helps us manage bugs and illnesses at home better and shorten hospital stays when they cannot be avoided. In short it allows us to give Freya the best chance to fight CCMS and all the trouble it causes her.

We will be introducing another team of specialists in Freya’s care. Many of the CCMS kids we have got to know in the last two years have issues with their spines. Freya has so far been lucky enough not to have had any issues, but recently a slight curve in her spine has become visible. Although it is unlikely that she needs any corrective action now we all agree she needs to be introduced to orthopaedics. None of this is urgent now or even affecting her much, but it’s better to get on their radar and keep and eye on the situation. Scoliosis combined with thoracic insufficiency can be an extremely dangerous combination and may require extensive surgery at a further date. Again, this is a stark reminder that so much of what future holds for us is so unpredictable – there is no such thing as being “out of woods” with a kiddo like Freya. So we may as well make ourselves at home.

It’s time to push beyond comfort zone again! The past year has been a period of adjustment for us getting used to living at home after long periods of hospitalisation and we have been able to find a routine that generally works. Knowing that care-wise things are unlikely to get easier for foreseeable future (and that there is a chance that this period of relative stableness may not continue indefinitely) it’s time to grab life by the goolies and broaden our horizons. So we are continuing trying to get Freya into nursery (currently we have received a lot of talk but no real action from Haringey). I spoke to Freya’s medical team about possibility of air travel and they are happy to arrange a fitness to fly test. We are already planning some overnight trips in the UK.

It’s not a life we chose, but it  is the life we have. We can either sit back and try to desperately risk manage every aspect or we can try to build ourselves a life despite the challenges we face. So we enter into the third year of this remarkable little lady a bit braver than before. Happy birthday little-muru. Mommy and daddy love you loads.