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.  

Tuesday, 24 November 2015

Amazing... but bittersweet

The last couple of months have heralded a new start for Jess. One that should, hopefully, make such a massive difference to the quality of her life.

In October, I went on two courses. One was run by Rett Education UK, and the other (a two day course) was run by Rett University. Both courses were presented by the amazing Susan Norwell, who runs Rett University, and were focused on teaching our Rett girls to communicate, read and write.

There was so much information presented on these courses, and at times I felt very overwhelmed  - both at the steep learning curve ahead for me and Jess, and also the sense of responsibility of wanting to make sure we do the best for Jess, and give her every opportunity possible.

I so much want Jess to be able to communicate fully with us - to be able to say whatever it is she wants to say, whenever she wants to say it; and not be forced just to choose from two options. I want to get to know her properly. Being able to communicate fully like this would improve her quality of life - for her entire life - so much. Being able to tell us when she is in pain, who looks after her well, being able to communicate with ANYONE, not just us or those who know her well. The difference this would make to her life is immeasurable.

The courses really brought home to me was that Rett Syndrome is NOT a cognitive disorder, but a neurological, physical disorder. Rett girls are traditionally thought to have a severe learning disability, but with the advances in technology and the use of Eye Gaze computers, this assumption is being challenged. Many Rett girls, given the appropriate tools to communicate, are showing that actually they fully understand everything going on around them - they are just not able to express themselves easily. Susan had so many amazing videos of Rett girls and women communicating in a wide variety of ways - all in context and meaningfully, demonstrating that they had full understanding of the topics being discussed.

One of the factors that significantly affects Rett girl's ability to communicate is their apraxia. Apraxia is the difficulty or delay that they experience in completing a physical act on command. In simple terms it is the delay after the message leaves the brain, and then results in a physical action, even though the cognitive understanding and intention were there. This delay in a Rett girl responding to a question or command may mean their response is missed or misunderstood. It also means that people may assume that the Rett girl didn't understand the question or instruction, but this is not necessarily the case.

The other major take-away message from both courses was, no matter what form of communication your Rett girl is using - PODD books, Tobii, high or low tech - the key is in modelling it. Talk to them using their form of communication - in essence, learn to speak "symbol"! By constantly using their form of communication, they will see how to use it, as you are demonstrating it to them. They will be able to learn what lots of different symbols mean, and you will be able to really immerse yourself into the techniques required, and will see what works, what doesn't work, what symbols are missing and be able to amend as needed. However, this is much easier said than done, and this did made me feel very overwhelmed - how on earth are we going to achieve this?

There was so much information over the three days - far too much to include on the blog here - even if I had understood it all, which I didn't! So I will just put the links to Susan's two websites:

Focused Learning Solutions

and

Rett University

Rett University provides on-line courses for teaching Rett Girls literacy, reading and writing. If you are thinking of doing this, then definitely do look at these websites, if you haven't already. The guided Reading 1 course provides suggestions on how to adapt reading books and activities to teach the girls to read, which is something we are planning to do with Jess.

So, using the information from these days, we have started to use the flip chart books, sold through Rett University:



 Here is a link to Susan Norwell demonstrating how to use these books:

flip book demo

The first time I showed these to Jess, I showed her how to use them by writing "I love Jess". I wrote the "I" and the "love" using the core word book, and then spelt out "Jess" with the alphabet book. This is what she wrote back:



I was so touched - and amazed! She wrote "love" using the core word book, and spelled "mummy" with the alphabet book. It was such a lovely thing for her to say, it took my breath away and I was just amazed that she was able to communicate it.


Jess has used this system now several times:

This is what Jess wrote on the day her new hospital bed was delivered - she was very excited!!!

Anna is Jess's little sister.


The other amazing news is that Jess has received her first Eye Gaze device. An Eye Gaze Device is a computer that Jess can use by using her eyes to move the cursor, and click in the same way we would use a mouse. The device took over a year from being ordered to arriving (which is very frustrating). We opted for a device that could be mounted on to her wheelchair, so it will be able to always be available to her for communication:

This is the Eye Gaze Device mounted to her wheelchair


I have a few videos of her using the Eye Gaze Device at home. In the first video, she is playing a game where she has to throw snowballs at characters as they appear. In the second video, she has played a game where she has to make a spooky cake, and put creepy toppings on it. The video shows her re-telling what toppings she had put on the cake. This is a major part of being able to communicate - re-telling a story of something that has happened, so I was really impressed that she was able to do this.
 
 
 
 





This next video was actually taken at school by Jess's Speech Therapist. She is asking Jess what she thinks about the book they have just read, that is sat on her tray. However, I don't think the school knew that this is actually one of Jess's favourite books at home. In the video, Jess repeatedly says "cool" and "great", and then makes a sentence "I think it is cool". I am so proud of her.
 
 
 
 However, this sounds like we are steaming ahead, and everything is good. However, for every example given above, there are numerous times where Jess hasn't managed to communicate  - she's been ill, she's been too tired, she just hasn't been interested. Or I've been too tired, or Anna too grumpy to give Jess the time she needs with me to do it. Integrating all this into our already very full, overtired lives is definitely a challenge and it is going to take us a lot of time to get settled into a new routine with it all. BUT, this is Jess's future - teaching her these skills is the ONE way (bar The Cure for Rett Syndrome) that we can significantly enhance and improve Jess's life, so we have to do it. And we will. I just wish it wasn't so very difficult for us all.
 
 Through all this, one thing that has shone through, is despite all the medical issues Jess faces, the pain, the discomfort - my little girl is in there. She understands everything, which is amazing to know as it shows she has the skills to further develop her communications skills. But it is also very bittersweet - she is locked in, and everything is so very difficult and frustrating for her.
 
I wish her life could be easier. 


 

Saturday, 17 October 2015

I've posted before Here how much Jess enjoys watching Strictly Come Dancing. Well, autumn has arrived, the nights are drawing in and Strictly is back. Jess, Anna and I all had early baths or showers, put our pyjamas on and snuggled up on the sofa to watch.







As always, Jess loved it!!!




Saturday, 10 October 2015

A Good Friend


I blogged here about our friend Sam, who raised an amazing £1055.09 for Rett UK.

We met her originally when she came to work with Jess in her role as a carer. However, she very quickly became like a member of our family and, as well as carrying on working with Jess, also came to family events like birthday parties.

Sam loves Jess, and the feeling is mutual. Sam completely understands Jess, and genuinely enjoys her company and chooses to spend time with her. Equally, I fully trust Sam to look after Jess, and value her opinions and input.

So when Sam said she was leaving the UK to work abroad (in Hawaii, no less!!) and then to go travelling, we had mixed emotions. We are very happy for her, but sorry to be saying goodbye (for a few years at least) to someone who has played such a big part in our lives.

Sam spent three weeks with us over the summer holidays, which I blogged about here helping me to look after the two girls, before she left for her new adventures. It was very sad and emotional saying goodbye. Jess doesn't have many people in her life who genuinely understand her and choose to spend time with her, and who also clearly enjoys being with her. It was such a shame for Jess to be losing someone like Sam from her life. And I will miss her too!!

We do however, wish Sam all the best for her new life and travelling adventures. I'm not sure a visit to Hawaii is possible for Jess unfortunately, but we plan to keep in touch!






Good luck Sam, have lots of fun!!!

Saturday, 26 September 2015

Children grow!


Children grow. As they get older, they tend to get bigger. I don't think anyone should be surprised by these facts.

For the average family, when children have a growth spurt I assume the parents just visit the shops of their choice and expect a larger than normal credit card bill at the end of the month.

For us it is not that simple. Jess has had a massive growth spurt. So much so, that her dietician has reduced the calorie intake that she receives through her jejostomy feed. So much so, that not only has she grown out of all her clothes, but also all of her equipment, like her wheelchair, her hoist slings, her sleep system and, very nearly, her bed. She has already needed a new rigid spinal brace and foot splints.

But it seems the public sector is just not set up to accommodate growing children.

Imagine if your child had grown out of his shoes, so you took them to the only shoe shop available to you. Imagine then, if the shoe shop said:

"Yes, we have shoes in all designs and sizes, there is bound to be something for you. However, you can't have any new shoes until we've measured your child's feet and there is a 12 month wait for measuring. We'll be in touch when you can bring your child in to be measured."

Hmmmm. That's kind of the position we are finding ourselves in with equipment provided by Social Services.

The NHS works slightly differently, but with similarly frustrating results. They seem to carry out the assessments reasonably quickly, but by the time the request for the equipment has been approved, the finances processed, the equipment ordered, delivered and then a rep has been out to set it up,  9-12 months may easily have passed. By which time the child has probably grown again and the new piece of equipment is already too small. This has happened to us on at least two occasions, with very expensive pieces of equipment.

If you follow this through to its logical conclusion, it means you always need to be requesting equipment two pieces in advance.

However, to actually do that would, first of all, be ridiculous and secondly would place even more pressure on an already overburdened system.

There is no obvious answer to this very frustrating process!! 

Monday, 14 September 2015

Seven Weeks

Jess's school had seven weeks of school holidays this summer.
 
Seven weeks with a child who can't do anything independently to entertain themselves is a long time.
Seven weeks with a child who might be up for 20 hours out of every 24, is a long time. That's a lot of entertainment to provide. That's a lot of Peppa Pig and Disney's "Frozen".
 
But we survived!
 
The first week, we went to stay with the girls' grandparents in Devon, where we had some fun days out:
 

 
 
The next three weeks, we had a carer, Sam, working with us, whom I've mentioned before on this blog here as she has raised a lot of money for Rett UK.
 
On the second week of the holidays, Sam and Jess went together to attend a week-long workshop run by the Chickenshed Theatre Company. Chickenshed is an inclusive theatre group, who in the holidays run workshops for children. There were 85 children on the workshop, of which several had special needs or disabilities. It was an amazing week, with lots of arts and crafts (not usually Jess's favourite activity, to be honest) and lots of music and dancing (which she loved), all building up to the group putting on a performance for family and friends on the Friday afternoon.
 
The show was amazing - the talent and effort of all the children was breath-taking - but I think the real success was the rest of the week. Jess loved being included in such a big group of children, and I think the other children benefited from spending time with her as well. Sam said that even from the beginning of the week, the other children were very interested in Jess, and asking a lot of questions. By the end of the week, they were actively looking out for her, making sure she could see, that there was space for her wheelchair in the circle, and were able to understand some of Jess's communication to help her to be included. I think this would have been a valuable experience for the other kids to develop alternative communication skills, and the awareness that everyone is different but can contribute, as well as empathy.
 
Jess and Sam after their Chickenshed performance


 
 
The next two weeks we spent at home, some days chilling out - long baths for Jess (which she loves but doesn't always have time for) and time in the garden, as well as some fun days out. We went to a fair:
 



 
 
We went to the butterfly exhibition that was on at The Natural History Museum:
 

The butterfly thought Jess's hairclip was a flower!

 
 
We also went to see The Gruffalo live on stage:
 


 
 
We then had a two week holiday in France, which deserves a post all of its own. I will try to write that soon!
 




 
The final week of the holidays we spent at home, catching up with things and getting ready for the return to our normal routines.
 
This describes the fun things we did. It does not cover having to go to Great Ormond Street Hospital four days in a row for appointments, the ridiculous length of time spent waiting around in various pharmacies for medication and the vast amount of time spent organising everything that had to be in place to take a child with all of Jess's needs to France for 2 weeks. It does not cover all the lifting of Jess that we had to do for her to participate in activities, and the difficulties we had changing her nappy when we were out and about.
 
I feel I spent the whole seven weeks telling Anna to "just wait a minute" while I attended to Jess, and she clearly missed the time with me one-on-one that she has when Jess is at school. Unfortunately, this resulted in more attention-seeking behaviour, which just resulted in me saying "just wait a minute" even more. A vicious circle.
 
Seven weeks is a long time. I found it quite hard work - trying simultaneously to meet the very different needs of my two daughters, and make sure they both enjoyed the holidays, as well as carrying on with the normal demands of keeping on top of Jess's medical appointments and management.
 
But we survived! And I think the girls enjoyed the majority of their holidays (although when told we had to go the the chemist for umpteenth time, Anna did exclaim "not again!") However, Jess is back at school and settling really well into her new class. Normality has also returned for Anna - nursery, swimming lessons, ballet classes as well as time with me.
 
And I've had my first morning child-free in 2 months, when Anna was at nursery and Jess was back at school!! I went back to bed for nap.