Filling The Week

Transition might be dominating our thoughts but life goes on and Sian is now 5 weeks in to her CLAIT and Sign Language courses. As I had expected, Sian  is finding CLAIT quite easy. Her IT skills are pretty good already so she’s able to jump ahead. I’m surprised how much she is enjoying herself and starting to feel part of the group. Sian and Graham have just finished the basic signing course and will progress to the level 1 course in January. Sian is picking it up quickly, Graham is a bit slower! At least they have each other to practice on.

Sian’s plan to do some voluntary work hasn’t yet got off the ground, despite her best efforts. She contacted the local volunteer bureau back in the summer and had hoped to persue a couple of opportunities – one helping out in the classroom in a primary school and the other doing some admin work with a local youth organisation. The admin vacancy has been filled and the school is not wheelchair accessible. Sian is continuing to look at the do-it.org website but I suspect we will end up having to use our network of contacts to help her find something.

Fortunately we have now found a second person to work with Sian under the Direct Payments scheme, bringing her hours up to 8 per week.  These are social hours, not care hours, allowing Sian to go out and about without one of us in tow. It’s working brilliantly and helping to fill the gap in Sian’s week.  If you add in the inevitable hospital appointments there really isn’t much free time left at the moment.

Moving Forward ?

I’m pleased to say that  the recent joint renal/metabolic clinic went well. From a clinical point of view Sian is doing really well with no hospital admissions since the end of June. Her blood results look stable and the only slight blip has been Sian’s weight loss. Sian discussed her goal weight with the metabolic dietitian at her appt earlier this month and they reached an agreement on a healthy weight. Despite maintaining Sian’s calorie intake her weight has dropped fractionally again so the calogen has been increased and we will continue to weigh Sian on a weekly basis. The slight weight loss may well be down to fluid balance but better to be safe than sorry. The other slight problem is that Sian is still anaemic despite increasing the dose of Micera (EPO ) back in July. The dose will be increased again and hopefully Sian’s haemoglobin level will improve. Other than that, everything is going well. Sian hopes to have her fistula created at K&CH anytime from the end of October onwards.

Transiton was inevitably the main focus of the appointment. The current plan is for Sian’s Nephrologist to approach one of the local Endocrinolgists to ask whether he would be prepared to accept Sian as a patient. In the meantime Sian’s home care nurse will continue to liaise with adult nursing staff and explore the admission route - either through A&E or the Medical Assessment Unit. Continuity of care is a priority for us but obviously can’t be guaranteed. The Trust has apparently agreed to follow the  ACT Transition Care Pathway (see link) and Sian will be one of the first young people in this area to follow the new pathway from beginning to end. Unfortunately for Sian, time is running out. The process should take years to complete, not a matter of weeks.

Right now Sian doesn’t have any choice but to go with the flow. We are not in a position to put our house up for sale tomorrow and to rush in to making such a huge decision without knowing all the options would be foolhardy. We do know that if we lived closer to Canterbury Sian would automatically be admitted to the renal ward via A&E, beds permitting. Moving remains our Plan B and something we will seriously contemplate if necessary. Sian has a follow up renal clinic on 5th November so we hope to have more news then.

Transition Again – Grrrrrrr!

Sorry for the lack of recent updates. Sian’s good patch has continued and we’ve been out and about, making the most of what’s left of the summer.

Sian had her six monthly metabolic review at St. Thomas’ earlier this month, the first appointment with her new metabolic consultant. This is the 3rd metabolic consultant that she has been under since transferring from Great Ormond St in 2005 and we’re hoping he’s here to stay. He will be joining Sian at her next local renal clinic on 24th Sept – a good opportunity for both teams looking after Sian to meet and discuss any issues.

The biggest ‘issue’ for us right now is transition to local adult health services and it’s something that I’m seriously starting to lose sleep over. We’re on borrowed time now and I suspect that Sian’s birthday in January will be the cut off point for paediatric admissions. We’ve just been told that the proposed adolescent unit at our local hospital isn’t going to get off the ground. No great surprise there! Intentions were good but the problem was always going to be funding and viability. There simply aren’t enough young adults admitted to medical wards to justify designating beds to adolescents.

At present  no local adult consultant has been identified as suitable or willing to take over Sian’s care. This will be on the agenda for the joint clinic next week. It seems likely that all  future admissions will be via A&E or possibly the medical assessment unit (MAU). However, the route  to admission is in some ways the least of our concerns. Adult medical wards at our local hospital are a far greater worry. The worst case scenario is that Sian will be admitted to whichever adult medical ward has a free bed. She is likely to be nursed alongside adults who are on average 50 years older than her, hopefully in a single sex bay but not necessarily. There will be little, if any, continuity of care and with no renal or metabolic expertise in the hospital (other than the visiting nephrologist on clinic days) all advice will be given over the telephone.   I’m inevitably worried about the very real possibility that Sian could suffer further neurological damage if  doctors/nurses with only a textbook knowledge of MMA fail to spot the signs of deterioration which we are now so familiar with. Sian is equally concerned. I also worry about the psychological impact of frequent admissions to different wards and of Sian’s needs not being met. If Sian could get herself up and move freely around the ward I would be slightly less concerned but she will be dependent on a member of staff finding time to help her to do all the things that we would normally do.  The loss of independence and control will impact on Sian’s wellbeing. A transfer to the renal unit on every admission would, in our opinion, be the best option. Sian would benefit from continuity of care, renal expertise and care shared between two units (renal and metabolic) not three. However, it seems unlikely that the renal unit will accept a transfer for what they woud probably consider to be routine admissions. The only other alternative would be to move out of the area so that we are in the catchment area for K&CH in the hope that all admissions could then be to the renal unit, beds permitting. It’s something we’ve mulled over in the past and would definitely be prepared to consider  now. Nevertheless, it’s a big commitment and not something that can happen overnight. I’m frustrated that almost 3 years after discussions for local transition started we’ve made absolutely no progress. Whatever happens now won’t allow Sian time to make the gradual transition we had all hoped for.

Hopefully our options will become clearer after Thursday’s meeting. Decisions are difficult but we want ours to be an informed one. We owe it to Sian to ensure that she continues to get the best possible treatment.  Some people may feel that we’re over-reacting but what would you do if we were talking about your son/daughter?

I’ll let you know how we get on.

Popham 2009

Last Thursday saw us up bright and early to meet the coach at St. Thomas’ for the annual Evelina’s Pride trip to the Popham Air Show.  Despite a worrying forecast the weather remained good and, as you can see, Sian had a wonderful day.

Sian with Becky, waiting for the helicopter ride.

Becky with Sian

Up Up and Away!

Above Popham

Back on that hovercraft!

Plan B

You might remember me mentioning that Sian’s goal, on finishing her Business Studies course, was  to look for part time and flexible employment with the help of the Pathways to Work scheme.  Back in March, when Sian first met her employment advisor, this seemed like a realistic possibility. Amost six months later it’s looking increasingly unlikely to happen in the forseeable future. The recession is at least partly to blame, or so we’re told.  Sian has also been chasing up the promised CLAIT course but to no avail. She remains on what would seem to be a rather long waiting list.

So, two months after leaving school, the novelty of not having anything to get up for is starting to wear off. Sian is bored and frustrated. I am just frustrated! Every day is like ‘Groundhog Day’ only broken up by the numerous hospital appointments. Sian has decided, and we agree, that it’s time to implement Plan B.

Last week Sian enrolled on a weekly CLAIT course at the Adult Education Centre which starts in September. There’s no waiting list and although Sian will have to pay, it’s at a reduced rate and worth every penny. The course will give her a basic IT qualification and will also allow her to progress to the ECDL course later on. Sian has also signed up for two evening courses, just for fun. One to learn basic sign language which she will do with Graham and the other is an introduction to digital photography which I’m going to do too.

Earlier today Sian had an appointment to discuss volunteering opportunities at the local volunteer bureau and is pursuing two possibilities. The first involves providing some classroom support in a local primary school (mostly listening to readers) and the second is an admin role with a local youth organisation. Whether either will come to fruition remains to be seen but Sian’s week appears to be filling up nicely and it will all look good on her CV.

Holidays

We’ve given up booking holidays now, having spent so many in hospitals dotted around the UK, but have booked a long weekend in South Wales and also have some lovely days out planned. The first of which was a day in London combined with a trip to see “Oliver” last week. The show was fabulous  and the weather was perfect for a stroll along the South Bank. The London Eye is almost adjacent to St. Thomas’ Hospital so it’s an area we have become very familiar with.

The Golden Jubilee Walkway bridge

Entertainers on the South Bank

View from Westminster Bridge

Sian had hoped to be able to attend the first Teens Unite residential camp at Mill Hill school in north London later this month (travelling daily instead of staying over) but we  have just heard that the camp has had to be cancelled due to the risk of young people with pre-existing medical conditions contracting Swine Flu. It’s undoubtedly the right decision but such a shame after all the hard work that has gone in to organising the camp.

DON’T PANIC!

That’s what we keep telling ourselves, and Sian, every time we hear, see or read anything on the subject of Swine Flu. Media coverage seems to be at saturation point and dominates every news bulletin and headline.

I rang the metabolic team for advice this morning – something I’ve been trying to avoid doing as know they will have been inundated with calls from other worried parents. As I had expected, the only advice in the event of Sian spiking a temperature and developing any of the other symptoms is to take her to our local hospital (she will need to be admitted) and ensure that she is started on a course of Tamiflu as soon as possible.  I had originally thought that Tamiflu was contraindicated for anyone with Sian’s degree of renal impairment but, having done some research, it looks as if Sian would be able to have it but that the dose would need careful management. Everything else will depend on how Sian responds if she succumbs to the virus.

In the meantime the only other advice is to avoid crowded places and contact with anyone who has, or is is in close contact with somebody who has Swine Flu. It looks as if things may ease off slightly over the next few weeks with the next wave of infection hitting in September when the new school and college terms start.

What can we do? Life has to go on as normal but with us all taking sensible precautions.

Calorie Crisis

Sian’s weight has been dropping, almost imperceptibly, since her new feed was introduced at the beginning of April. Trying to determine Sian’s actual weight is a challenge as her weight fluctuates depending on how well hydrated she is. However, her target weight is aprox 50 kilos – she weighed in last week at 45 kilos, not good news. It looks as if  Sian’s calorie intake was miscalculated when she switched from her original Pepdite feed to the new overnight XMTVI Maxamum feed and daytime Pepdite feed. With a calorie deficit of around 300 a day it’s no surprise that her weight has dropped. Thankfully Sian has remained well and doesn’t seem to have suffered any ill effects from the loss of calories, apart from the fact that her clothes are now falling off her. The solution is to add 90 mls of Calogen, a fat supplement, to Sian’s enteral feed which should give her an extra 400 calories a day and add up to a weight gain of just under 1 lb a week. It’s going to take a while to get the weight back on – with luck Sian will regain a reasonable amount before the next hospital admission. I’ve had a week to calm down and stop panicking. This is a situation that should not have happened.

Sian met the local consultant taking over care of her Osteoporosis last week too. He has suggested that she re-starts the Actonel (Risedronate) but only take it once every other week. We’re hoping this may reduce the nausea that Sian was previously experiencing and Sian is prepared to give it another go. The alternative treatment would consist of an intravenous infusion but is something that would need to be discussed with the renal team as may not be suitable for someone with Sian’s reduced kidney function. He will review Sian in 3 months time.

Staying Active

I thought I’d share some more of the photos taken when Sian went to the Arethusa Centre recently. These are just amazing – I still can’t believe that it’s Sian on the climbing wall. It must have taken all her energy and determination to get up to the top but she managed it, with a  bit of help, as you can see.

Sian on the climbing wall - Arethusa Centre

Almost there.

Still feeling active Sian decided to try out handcycling when we visited Beyond Boundaries Live the weekend before last. It’s a lot more difficult than it looks.

Handcycling - the bike was a bit on the big side for Sian

There was also an opportunity to try out Boccia (pronounced Botcha). It’s similar to Boules/Bowls and Petanque and perfect for wheelchair users. Sian seems to have a natural apptitude for the game so we’re hoping to identify local opportunities for her to play.

Boccia

On target to hit the jack

Back In Hospital – July 2009

Well, we almost made it to 3 months without a hospital admission … but not quite!

Sian started to droop the weekend before last, nothing major, just looking tired and washed out. In view of the warmer weather it seemed sensible to get some blood tests done and the doctors on the ward agreed that 24 hrs of intravenous fluids would help perk Sian up.

Here she is, with a cannula almost under her arm, actually looking remarkably well considering she is in hospital.

We had hoped to be home again within 48 hours but ran in to several niggly, but not serious problems on the second day so decided to leave things another 24 hours. We’re all hoping this admission was a blip and not the start of another period of instability.

Sian had a renal outpatients appointment last week and that brought some good news for a change. Sian’s consultant has decided not to push for dialysis to start immediately and is hoping that a combination of starting the new feed and stopping one of the drugs (Actonel – which had been making her nauseous) might be enough to maintain her stability for a longer period. The plan now is to go ahead with creating a fistula early in the autumn and leave it plenty of  time to mature.  We all know that this is the right decision but are back to being on that emotional rollercoaster that I’m so fond of referring to. We have been making the most of Sian’s good health but have found it difficult to focus on helping her to look for employment/voluntary work or training knowing that she would probably be committed to dialysis sessions thee times a week for up to four hours at a time. However, the goal posts have moved once again and  Sian has an appointment with the Employment Advisor from RBLI next week so can discuss her current options then.