Sunday, 17 July 2016

The reality of having a medically fragile child

In the last six months, Jess's health has significantly deteriorated, and as a result her medical management has become very complicated. Multiple, unexpected emergency hospital admissions take their toll and affect every family member and aspect of family life. We have found that we have had to completely adjust how we live our life, sometimes in the most unexpected ways....

At home:

  • I no longer buy a week's worth of food through online shopping, as Jess will inevitably get admitted on the day of, or on the day after, its delivery and it will end up being thrown away
  • I always try to keep up with the laundry so we always have clean clothes ready to go into hospital with 
  • I always try to leave the house tidy. We never know who might next be in it - either collecting supplies for the hospital or looking after Anna.
  • We always try to have a back-up child care plan for Anna - everyday, just in case
  • When booking and planning days out or holidays, we do it knowing there is a good chance it won't actually happen
  • When booking and planning days out or holidays, we not only have to consider where the nearest A&E is, but also where the nearest paediatric intensive care unit is (only specialist hospitals have these units)
  • When booking and planning days out or holidays, we have to consider how complicated it would be, and how much money we would lose, if we had to cancel at the last minute
  • Despite the above, when Jess is at home and well, we try to plan lots of things as we don't know when we will get another chance
  • When booking and planning days out or holidays, we don't tell the girls until the last minute so they don't get disappointed if it doesn't happen
  • If we have a list of chores to do (and when don't we?) and some free time, you can't say "I can't be bothered, I'll do it tomorrow" as you may end up in hospital tomorrow and be there for weeks -  therefore you never really sit down and relax
  • I carry round pretty much all Jess's medical letters with me (which explains my huge handbag), just in case we end up at a different hospital or with a doctor we don't know
  • When I ring my husband at work, he never knows if I'm ringing to ask him to buy milk on his way home, or to tell him that Jess is in an ambulance on the way to hospital and isn't breathing
  • I'm NEVER without my phone. If I go swimming, I give school the number of the swimming pool reception....  Just in case 
  • Jess's wheelchair no longer always fits through doorways, due to the vast amount of medical equipment we have hanging on it

Our hospital experiences can also be somewhat unusual:

  • When the paramedics radio ahead to say they are bringing Jess into resus, the A&E staff  reply with "see you soon Jess!"
  • We are on first name terms with the staff in A&E, all the paediatricians and the ward staff
  • When getting our bed space ready, the staff on the ward set up all Jess's emergency equipment, including a "Frozen" DVD
  • Everyone gets excited when you go 8 weeks without an admission
  • When you arrive on the ward, other patients already there think you are a celebrity because all the staff rush over to say hello and give you a hug
  • When you get admitted for a chest infection, all the hospital staff are really excited - because it's not a urinary infection this time
  • The same doctors that you try and dress smartly for when seeing them in outpatients (in a vague effort to try and convince them you are sane and responsible, so that they listen to and act on your opinions and requests) end up seeing you on the ward - just out of the shower and wearing your pyjamas.
  • The above-mentioned doctors also wake you in the middle of the night, and observe you trying to be sufficiently coherent to answer whatever obscure questions they are asking, whilst half asleep, bed-head hair, no contact lenses in and unable to find your glasses
  • You get used to sleeping in a bay with six (often crying/screaming) children and six snoring adults (who are often ignoring said crying/screaming children), lights on, alarms going, phones ringing, bleeps bleeping, doorbells ringing etc etc etc. 
  • You actually sleep better in the above environment than at home, as listening out for Jess, and ensuring she is alive, is someone else's resposibility
  • Anna will request which hospital, and which ward, she would like Jess to be on, depending on what toys she wants to play with that day
  • I know where things are kept on the ward better than some of the staff

Despite all the above, we try very hard to give the girls a childhood as "normal" as we can (whatever that means!). This is helped by the fact that when she is well, Jess loves days out, holidays and new experiences. We want to build as many family memories as we can, while we can. Who knows what the future holds? 

Tuesday, 29 March 2016

The new normal? I hope not

I haven't updated this blog recently, mainly because I'm not too sure what to say, and to be honest we have been too busy just trying to survive. Jess has had a pretty rough time of it health wise over the last few months, and I'm not sure things how things are going to stabilise out. 

After being diagnosed in June 2011, Jess didn't have any real health problems associated with Rett. That's not strictly true- she never slept, and did have severe reflux. But everyday life carried on, and we mainly focused on developmental skills and making sure that Jess enjoyed as many experiences as we could let her. 

Then in the December of 2013, Jess went into Great Ormond Street Hospital for a 48 hour EEG, to see if any of her numerous night time behaviours were seizure related. In true Jessica style, she had complications completely independent of the study she went in for, and we came home (a week later, with instructions not to travel over Christmas) with home oxygen, an oxygen saturation monitor, a suction machine, an NG tube, a feeding pump, a diagnosis of seizures, and dates for two lots of surgery! It was a lot to take in. 

Life never returned to how it was before. In the subsequent year, we had numerous hospital admissions, ambulances, tests and appointments. Her list of medical complications grew, and her care became more complex. 

However, between April 2015 and August 2015, she seemed quite stable.  She was tolerating her spinal brace, her reflux and chest infections had reduced when her feed was changed to a jejostomy feed, her recurrent UTIs reduced with prophylactic antibitoics, her seizures were well controlled unless she had an infection, and her sleep had improved with the addition of a drug called Clonidine, to the melatonin she was already taking. 

For those five months, we had a glimpse of how life could be, with predictable (although still not enough!) sleep, and not worrying that Jess was going to be rushed into A&E at any moment. I will say that I never totally relaxed, and enjoyed this time. It was always a feeling of when, not if, we were going to return the previous state of life. But, at the time, we were still thinking "this can't be it", "this can't be as good as it can get". But now, looking back, it actually was a pretty good period. Isn't hindsight a great thing?!

Since September 2015, things haven't been so good. Jess's UTIs returned with multiple drug resistant infections, and her reflux became a problem again, all leading to a major deterioration in her sleep pattern. And everything is related, so anything you do for one problem has a direct knock on effect on another problem- nothing happens in isolation. As well as these obvious things, Jess just hasn't been "quite right" since September, which is far to vague and unspecific for anyone medical to even know where to start. 

However, things have taken a distinct turn for the worse since December. In the last week of school before the Christmas holidays, Jess was unwell, and having some small absence seizures, which were not bad in themselves but concerning. She was then admitted to hospital on Christmas Day (so rubbish!) with an exacerbation of her seizures. She has since had a further four hospital admissions, including an admission to Paediatric Intensive Care at Great  Ormond Street for Status Epilepticus, nearly all due to uncontrolled seizures resulting from infections. 

Jess now has even more medical complications to add to the list, and her medication list is crazy! Her new care needs are even more complicated with new equipment being needed, everyone who looks after her needing more training, she now needs 1:1 on school transport (which is turning into a nightmare to be organised). I have also found myself making comments, which to us are part of our new "normal", only realising by the look of horror on people's faces, that this is not normal at all! Statements like "anyway, I just need to go and check Jess is not unconscious" and "it's ok, she doesn't stop breathing every day".

I really don't feel we are in a good place with Jess at the moment. Her UTI's are frequent, with limited drug options for treatment, and the doctors are finding it increasingly difficult to get IV access where she has had so many IV lines in. And as well as the infections making her ill, they also have a devastating impact on her neurological status each time. 

Five hospital admissions in 3 months is a lot. And its's not just the acute side of things that is difficult to deal with- all the investigations she has had recently turns up new things each time. A routine X-ray showed osteopenia (low bone density which increases the risk of fractures) which we always knew is a risk factor in Rett Syndrome, especially as Jess is non-weight bearing, but we didn't know this process had already started for her, when she is so young still. And Jess had a brain scan when she was in Intensive Care- it fortunately didn't show anything acute to be worried about, but commented that she has "frontotemporal atrophy in keeping with Rett Syndrome". It just brings home just how physically devastating Rett Syndrome can be, and how much it is destroying her little body. 

As well as trying to cope with everything going on with Jess, one of the hardest things has been trying to support Anna through this time, and make sure she receives the love and attention she both needs and deserves. It's a difficult juggling act, but Anna is coping as well, if not better, than I would ever have hoped for. She is quite an amazing little girl and sister. 

Jess is now out of hospital, although still on IV antibiotics for her most recent infection, but she is actually on great form. She is the best we have seen her in a very long time, and it's both lovely and amazing to see her so well, and so happy given all that she's been through recently, and how precarious her long term health actually is right now. I've said it before, but she is amazing with what she copes with, and I am very proud to be her mummy.