Thursday, 24 April 2014

One down, one to go.

Jessica has now had the first of the two operations she is due within a few weeks of each other.

The first was a couple of weeks ago, and was to remove her tonsils and adenoids. This is an information sheet from Great Ormond Street Hospital where she had it done.

Jess was an absolute star. We went in the morning to the pre-admission ward, where we met all the ENT surgeons and anaesthetists, and got Jess ready for theatre:

We then went down to theatre, and I held Jess on my lap while she was put to sleep. The operation took about an hour, and then we were called to go to the recovery room to see her. When we got there, she was just waking up, and was obviously feeling a bit sorry for herself, and then she was taken to the post-op ENT ward, where she mainly slept for the rest of the day and night:

Clearly that is what is needed for a good night's sleep - a general anaesthetic, followed by melatonin - as the ward was ridiculously noisy, and I didn't get a wink of sleep!

The next morning, with the help of regular (and massive) doses of paracetamol and ibruprofen, Jess was on great form, we even began to wonder if they had actually done anything in theatre!

As the surgery hadn't been as complicated as the doctors had thought it might be, we were allowed home after just one night, which was excellent given how loud the ward was! Jess did so well, and didn't complain too much, although she wasn't particularly into eating anything, and we fully depended on the NG tube for all her feeding.

However, five days after the operation, we hit a complication. It appeared that it was too painful for Jess to swallow her saliva. She is not normally a "dribbly" child at all during the day, and I don't think I had realised how much she swallows! She was drowning in secretions - coughing, gagging and everything was constantly soaked, including the dressing holding the NG tube in.

Inevitably the NG tube therefore fell out, despite our best efforts, and a midnight trip to A&E took place. Here they replaced the NG tube for us, but wanted to keep her in for observation in case the re-insertion of the tube had disturbed the operation site, and caused any bleeding. They were also worried about all her secretions, and the consequence of these on her oxygen levels. We were admitted to the children's ward for two nights, for observation, and commenced a medication (a Hyoscine patch) to dry up her secretions, and therefore make it easier for her to cope with them.

She is now much better, and back to her more usual happy self. She is eating again (well, as much as she did before) and has returned to school.

I found the whole thing incredibly draining and exhausting  - three nights in hospital with no sleep, and even when we were home, I was setting my alarm clock for three hour intervals so I could give her pain killers - it is so hard when she can't tell us when she is pain, and what is the matter when she is upset. But she did brilliantly, I am so proud of her - she puts up with so much, and yet so rarely complains.

It is a bit daunting to think we have to go through it all again in just a few weeks for the gastrostomy, but we definitely need to lose the NG tube, life will be significantly easier then.

Thursday, 17 April 2014

Matching accessories

I posted previously here and here about scoliosis in Rett Syndrome. For approximately a year, Jessica has been wearing her Lycra body suit to try to stop her scoliosis deteriorating. Jess was reviewed and re-x-rayed in December, and while her scoliosis is not significantly worse, it was decided to move Jess into a rigid body brace. This is because we were having some problems with the Lycra body suit causing damage to her skin. We were also given the very depressing statistic that 60% of girls who present as Jess does now will need major corrective spinal surgery by the time they are 10 years old. Given that the body suit was causing problems, and that she was always going to end up in the rigid brace at some point, it seemed the sensible thing to do.

So in January, I took Jess to be cast for her brace and I was asked to choose from a selection of designs. I narrowed down the choices, and gave Jess 3 or 4 to choose from. She VERY clearly chose one. Now, it wasn't the one I would necessarily have chosen for her (so I guess it is just as well I asked her, and didn't just decide for her!) and I kept double checking with her that I had understood her correctly. But yes, and it was some of the clearest communication I have seen from Jessica in a long time.  We now have the brace, and have to gradually increase the time that Jessica wears it every day. However, this has turned out to be a very slow process, as Jessica has been so ill since December.

However, this last month Jessica also needed new leg splints (ankle-foot orthotics) and again I took her to be cast for these. And again, I gave Jess a choice of designs- and she chose exactly the same design as she did for her rigid brace, while giggling hilariously! She clearly knew exactly what she was doing, and she now has matching accessories. Every girl should have matching accessories, even if they are slightly unconventional ones!!

Monday, 14 April 2014

Doing something right

Jessica was our first child. We had planned to start thinking about having another just about the time when the doctors started saying "we think there is something wrong, but we don't know what". At which point we felt we had better wait for all the genetic tests to be carried out. The rest as they say is history and I've written about receiving Jessica's diagnosis of Rett Syndrome here..

So with all the knowledge that receiving a diagnosis of Rett syndrome provides you with, we decided to carry on and have another child. Now the fact that Rett's is not generally hereditary was only part of the decision. Would we cope? Would it detract too much of our attention from Jessica? What effect will having a profoundly disabled sibling have on the child? Of course, many families have their Rett daughter as a younger sibling to older children, and all these questions go out the window- you cope and do the best you can. But that wasn't the case for us - we made a conscious decision to have another child.

And there are many other worries; I never want Anna to feel neglected because of all the attention Jessica needs, or all the limitations having a disabled sibling places on our family life. I never want her to feel embarrassed by Jessica, or to be bullied at school because of her, and I never want her to feel that Jessica's needs are a burden on her. And while I sincerely hope that Anna will be happy to ensure that Jess is ok in later life when we, her parents, are not around to do it, that has to be Anna's decision.

It's not easy having a nearly five year old disabled daughter, and a lively nearly two year old- I can't leave the house with the two of them on my own, with Jess in her wheelchair and Anna in a single buggy. I can't even get the two of them to bed on my own: I turn the oxygen on, and while I am putting the mask on Jess, Anna will turn it off/disconnect it/change the settings etc. The other day I watched Anna  break the mobile hoist in Jessica's room while I was re-doing the dressing on Jessica's NG tube and I could do nothing about it. If I'd gone to Anna, Jess's NG tube would have fallen out.

And (no surprises here) dealing with a toddler is not easy. But this is all new to us- we have never had to cope with a child throwing a tantrum in the street because we aren't going "THAT WAY...", a child who jumps out of her cot at night, and a child who refuses to let mummy out of her sight (or even her touch) in case she disappears off into hospital again with Jessica.

It is hard to know if we are getting the balance right, between disciplining and accepting behaviours; and what is normal toddler behaviour, and what we shouldn't be too hard on her for because it must be difficult for her having an older sibling who needs so much attention and regularly takes mummy away for long spells at a time.

But Anna adores Jess. She is distraught in the mornings when the school bus leaves, and will stand at the living room window at 4pm waiting for it to return. And when she sees it, there are loud exclamations of excitement and she runs to the door, shouting "quick, quick". When Jess has arrived home she then helps me take off Jess's shoes and splints and put them away.

When Jess is upset, Anna strokes Jess' arm and says "what matter Jess-ca?" and she has now started sharing her toys with Jess, and will give one to Jess saying "Jess's toy" and then "Anna's toy". Today we went to the local Farmers Market, and there were some fruit juices being offered as samples, with a cup of straws on the table. Now Anna is quite fascinated by straws and decided to help herself. She carefully choose the colour  and then took two, and walked over to
Jessica and gave her one saying "Jess's straw", then "Anna's straw". It was so sweet.

Again, the other day, we were shopping to buy the girls some summer clothes, and Anna very excitedly saw a Peppa Pig bag, full of Peppa Pig hairbands. Before I could stop her, she had it off the shelf. I told her she shouldn't touch and to put it back, but before I knew it, she had another one off the shelve which she promptly gave to Jessica. Jess became very excited at this, and actually managed to pick the bag up. Consequently, I couldn't bring myself to be cross, and HAD to buy both bags!

And Jess loves Anna too, although half the time I think Jessica wonders what on earth Anna is doing. But tonight, Jessica was very upset going to bed, until Anna came in to say goodnight to her, and she tried really hard to give Anna a hug.

But, despite how challenging the toddler years are (and I fear they may get worse before they get better!) as long as Anna loves Jess, then I feel we are doing something right.

Saturday, 5 April 2014

But she goes to school.....

As I wrote here, we have had huge problems obtaining the help, support and equipment we need from Social Services and the Health Service (but not the acute hospital-based services, or Jessica's school - they have both been fantastic).

Social Services and Health Services have clearly been clutching at straws to come up with reasons why they shouldn't provide us with what we need or are entitled to, even in respect of services that we have previously been assessed as needing, and were receiving up until December when Jess became ill. 

Some of the more classic lines they have come out with are:

But she is only 4

Other children don't get that

She is too medically dependent for Social Services to help

She is not medically dependent enough for the Health Authority to help

Well yes, technically our assessment is incorrect, but if we assess her correctly we then have to provide the service

We don't do that. Oh, are we really legally obliged to? I didn't know that

No we can't possibly do that. Why? Because we have never done it before

Yes, well I agree she needs two people to lift her safely, but once you get the ceiling hoists, she will only need one person to hoist her. Yes, I do know you don't yet have the ceiling hoists but that is not my department so it's not my problem

Well I agree she currently needs this medical input but we can't help you with it now, because she may not always need it

Where is her father?

And my favourite: She goes to school, what do you spend your time doing when she's there? 

All of these prompted exclamations of anger, disbelief or tears of pure frustration and exhaustion at one point or another. But the last one was the one that broke the camel's back and prompted the fuming email below. I'm sure this will look very familiar to parents with a child with special needs.

This is an example of 3 weeks from my diary: it is representative of our normal level of appointments, although obviously they change, for example there are no hospital appointments in these few weeks, yet in other weeks we may have 2 or 3 hospital appointments a week.

09:30- tour of place Jess could go to for respite
11:00- TAC meeting at Jessica's school

09:00- home visit from 2 OT's from Social services re major home adaptations
 9:30- home visit from rep re equipment for major home adaptation
09:00-17:00- have to stay at home to receive a delivery of equipment 

14:30- visit from architect re major home adaptations
 7:30- assessment from Outreach Hospice

16:30- home visit from school OT and rep to set up Jessica's new home chair

14:30- Special needs opticians
15:30- second assessment by Outreach Hospice

10:30- IEP at school

09:00- meeting with Social Worker
14:10- orthotics to be fitted for new AFO's

10:00- appointment with dietician and speech therapist re feeding

09:30- appointment at wheelchair service

0945- school open morning

This is my "to do" list for this week. Again, this is representative of the volume of administration I do on behalf of Jessica.

> *order repeat prescription from GP, ensuring that they have received all the recent reports from Great Ormond Street Hospital, our local hospital and the Community Paediatrician, and have updated their records accordingly, with new medications and drug dosages
> *arrange an appointment to meet up with a rep re equipment for major home adaptations
> *order Melatonin from Community Paediatrician and get prescription to be posted to us, as GP not allowed to prescribe this drug
> *send the OT a list of equipment that Jess had grown out of, and that can be collected
> *confirm time for appointment with Wheelchair service as they have sent me 2 different times and liaise with school physio so she can attend.
> *contact community nurses and request additional oxygen saturation probes
> *cancel a duplicate hospital appointment sent in error
> *start filling in DLA renewal form (a very time consuming process)
> *arrange and confirm nursery space for Anna for week after Jessica is due to have surgery, so can confirm surgery date with GOSH

> *contact Rett UK re info required
> *complete paperwork required for care agency we are using to supply help in evenings to get girls to bed
> *email school physio re Jess starting to wear her new spinal rigid brace
> *Re-arrange Jessica's appointment with special needs dentist as previously had to cancel as Jess had been admitted to hospital
> *arrange for technicians to go out to Jessica's school and fix wheelchair
> *fit equipment that was sent home from school by school OT onto Jessica's new home chair
> *chase ph study report from GOSH (this has so far taken 8 phone calls and I am still no closer- this report should have been ready for our appointment with consultant surgeon at GOSH 2 weeks ago, but hadn't been written up).
> *email dietician and school and ask for statements for the DLA form

> *photocopy all recent hospital reports for DLA form
> *email dietician with update regarding Jessica's progress with feeding (her routine is changing weekly at present while we build up)
> *order next months worth of nappies, ensuring prescription for next size up has been received (it hadn't last month)
> *make sure next months order for feeding supplies (pump giving sets, syringes, feed, ph strips is amended to meet our requirements
> *Contact school OT re new house chair as battery and brakes are not working
> *Contact school nurse to request additional nappies in our monthly allowance as increased feeding and hydration is meaning we are having to change Jessica's nappy more often.

This is all representative of how I spend my time- these are normal weeks. I am attempting to manage all this while also doing everyday things like laundry, grocery shopping, cooking, organising for the 
gas man to come out etc, while also caring for Jessica's very complex needs, looking after my other 22 month old child, and trying to ensure that both children have as "normal" a childhood as possible.

Hmmmm. What do I do when Jess is at school???! Whatever it is, I wouldn't call it respite.