Archives for the month of: February, 2015

I bloody love Twitter. I joined it in 2009 and confess, at first, I didn’t know how it worked, who to follow, what the appeal was. I was really into Facebook at the time, and also member of a now defunct forum called FirstFoot, so my social media needs were being well met, thank you very much. I have to thank sincerely @robertflorence and @iainconnell for really getting me into Twitter and can’t say how many great people I know online and met in real life because of it.

Last night on my Twitter timeline I came across a tweet by Wee Moo ( @clairetheweemoo ) about ‘person’ ( @vic_person ) telling a funny story about having a gynae exam. Call your ladyparts what you will; foofs, vajayjays, chuffs; it was just a joy to read. Sometimes, I think, we can be too serious about medical procedures and her story reminded me about my own funny gynae story.

I’m now leaping forward in Joff’s story to 2000, Miss Effie Snr was 7, Joff was 6 and Mr Effie and I finally decided we were now in the position to try for a third baby. I was especially conscious of my own age, and the increasing risk of Down syndrome, along with our own risk factor of 25% chance of having a second child with Lowe. Luckily for us, work by the amazing Dr Robert Nussbaum and colleagues in the USA meant that there was now a highly reliable biochemical diagnostic test for Lowe, one that could be carried out on a placental sample. Dr Nussbaum has been closely associated with the Lowe Syndrome Association for over 30 years. I love him.

So we worked with our lovely geneticist to plan our third pregnancy, the testing, the potential decision making thereafter. I fell pregnant in October 2000 and the plan to test the placenta was scheduled for 10 weeks, by which time I had been hospitalised for a week and put on a drip for hyperemesis, which happened with both our girls, but not Joff, oddly enough.

The day of the test arrived. The sample would be taken using ultrasound to guide the doctor, and some of those cells would be tested primarily just for the sex of the baby. If a girl, then, yippee! If a boy, the sample would be cultured and sent to the USA for biochemical testing, telling us if the baby had Lowe or not. It’s a moot point now, but we had thought and talked about it very deeply and decided we would not keep another boy with Lowe. Perhaps that shocks some people, and thankfully we never were put in that position. It’s one thing saying it and another to carry that decision out, but by then we had seen some of the vast range of challenges that boys and men with Lowe face, and no one could say with any confidence how severely a new baby would be affected.

Technology has since moved on apace, now families like us can avail themselves of PGD – pre-natal genetic diagnosis, where the cells of an in-vitro embryo can be tested before the embryo is implanted, thus virtually guaranteeing a boy free of Lowe.

We were sitting in the waiting room, me drinking water to help the ultrasound and the doctor called us in. We went from the busy corridor with bright fluorescent lighting to a large darkened room only dimly lit by the screen of the ultrasound and walked toward the bed. I didn’t take in the surroundings at first, so when the doctor asked if it was ok for medical students to observe and I said yes, she waved vaguely towards the back of the room by way of introduction, where 6 students stood silently in two rows of three, all in white coats, like some weird kinds of meerkats on guard. They waved back and muttered ‘hi’. I immediately got the silent giggles.

The doctor asked me to undress behind a portable curtained screen. the curtain was a clean, non-descript pastel material, but the paint had definitely seen better days. Mr Effie whistled silently through his teeth. Being an engineer, he had some appreciation of how much money was invested in this very specialised ultrasound equipment. I remember thinking how glad I was that the priority was the good machine and not nice paintwork. I got up on the bed and the doctor proceeded to prepare for the test. A nurse was also there to help. The doctor took a sample of the placenta and scurried to have a look at it in the Petri dish. Sorry, she says, I need to do it again. It wasn’t the worst thing I’ve ever had done, but it wasn’t nice either. I tensed somewhat, becoming suddenly absurdly aware of the 6 students silently watching me. Can I have some more light here please? asked the doctor, and the nurse’s serious face duly appeared over the doctor’s shoulder, shining a £15 Halfords box torch up my foof.

Well, no-one told me I had a nice cervix, or wonderful ovaries, but a second good sample having been collected, I got dressed behind the screen while the doctor completed her part of the test, I was silently shaking with suppressed laughter, relief, nerves and the pure absurdity of the situation. The irony of getting dressed and undressed behind a screen and showing everything I have in between was strong. Even stronger was the contrast between the expensive ultrasound machine and that £15 Halfords box torch, Box. Torch. Oh man, I crack myself up.

That test was done on a Wednesday afternoon at 2pm. The next morning at 9:15 the doctor phoned to say the baby was, indeed wee Miss Effie. Yippee!

Our next scheduled delivery was in December 2010, but if you cast your mind back, we had epic snowfall in Scotland and many roads and pavements were difficult, if not impossible, to negotiate. Our delivery was delayed and delayed again, and we tried in vain to track down our supply.

We finally found out that the van drivers had been stuck in Glenrothes due to the bad snow but they couldn’t tell us exactly where for security reasons. Mr Effie asked that the pads remain in the depot and he would drive over and collect them in his works vehicle, at that time, a 4×4 Discovery. There was a lot of huffing and puffing about delayed Christmas presents being on the vans, a fact we couldn’t have cared less about. Nobody we phoned could care less about us. Meantime we had 2 pads in the house. I was seriously wondering how to fashion a nappy out of a carrier bag and tea towels. I was that close to tears, and phoned our local MP’s office asking how we could stop being treated like rubbish by our incontinence service, and their contracted deliverers. They promised to escalate our written complaint to the health board.

In it, I described the horrible situation we’d been put through. I concluded by asking for a months supply for us to keep laid by at home so we’d never be caught out by delayed deliveries or manufacturer’s delays again.

The delivery guys eventually got to our house, oblivious to the earlier frantic calls to their boss. I flew out of the house, nearly flying on my arse in the snow to help them get the boxes into the house. I kissed both guys roundly, I was that pleased to see them!

After a month or so passed, we got a reply to our complaint, an apology, and a months supply to hold in stock at home.

22nd October 2010

So Thursday at five to four I got a message on my phone from Incontinence service to phone back. She doesn’t sound happy. I wonder if it’s because she was expecting to speak to Mr Effie. However, he’s away in Greenland getting ready to run a marathon on the polar icecap. After mentally girding myself for a half hour to reply –

“Have your pads arrived yet?” “Nope”

“Oh I was told by Hartmann they’d be with you today, and they told your husband that too”

“No, they guessed Thursday to get him off their backs, they actually said Friday first till his voice rose an octave and backpeddalled it to Thursday”

“Well I’ll go and find out what’s happening”

Rung back, yes the pads did arrive with them, but they didn’t make it onto the vans coming to Scotland on time so definitely tomorrow.

OK. On their letter we’re scheduled to get 9 packets ( 4.5 daytime pads a day ) but we need 12 ( 6 daytime pads a day )

[Wait. There’s a lot of “days” in that sentence. I apologise.] [and what’s with the 0.5 pads a day???]

You’re down here for 12 packets.

Well, that’s really great if they come. Then we’ll be back bang on schedule with the nighttime delivery. ( and I can stop sweating )

The pads just arrived 10 minutes ago.

9 packets.

I despair.

It’s now 6pm and the whimsical thought just occurred to me to rip all the pads out the packets, lie them on the floor… stick on a swimming costume and lie on them a la “American Beauty”

However I’ll refrain for now. 😀

As part of bringing my old blog forward, I was reading this old post from 2010, when Joff was still at school. Classic gatekeeping vs parent situation. I describe below some of the simple toilet training we were trying at the time with Joff.  Sadly, it didn’t work out and he’s still doubly incontinent 24/7.

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This post was written on Tuesday 19th October, 2010, and first posted on a discussion forum.
I used a technique like this before when I was very upset and angry at how services were going for us – to let people in the general public know how lack of support affects us, and then forwarded a hard copy as a complaint.
I still haven’t decided if this will become a complaint as I don’t think our local Health Board will recognise it as such.
However, the facts remain that I am very angry and disappointed with the situation.
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I’m gonna set out my stall here then encourage as many people as I can to read it because I think this story, this situation, is disgusting. I’m savvy enough to realise that with all that’s gone on in Team Effie’s house this year that I’m likely to be reacting somewhat more to this situation than I would normally.
However, the facts remain that we have the incontinence supply blues.

For those who don’t know, our profoundly disabled son Joff is 16 and doubly incontinent. To cope with this he uses disposible nappies supplied by the NHS as is his right from about age 3. To be more age appropriate for him, we call the nappies pads.

I actually thought that incontinence was essentially par for the course for Lowe Syndrome, but in fact the majority of boys with LS achieve some degree of control of bowel and bladder control, and many are fully toilet trained, delayed in some cases, sure, but Joff is actually in the minority of boys with LS who are doubly incontinent at his age.

To me it’s a mulitfactorial thing, his delayed neurology means that the sensation of bladder fullness, urgency or being ready to go are likely to be missing or misinterpreted. I’ve certainly never seen him squirming or clutching in the way a toddler often will do before they need to visit the toilet.

His kidney disorder, Fanconi syndrome, means that almost all the liquid that passes through his kidney for filtration is sent afterwards right to the bladder, in effect, it’s a water concentration defect as he cannot produce a dark urine as you or I would when thirsty/dehydrated. It also means that urine is continually trickling into his bladder and perhaps he never achieves a full bladder as he would just wee it out just as quickly as it is produced. Overarching this, he cannot be fasted from fluids, unless in a hospital setting.

His phosphate medicine, taken three times daily, causes an effect similar to diahorrea – such that I’ve only seen a normal looking poo from him two or three times in his life, all when he was dangerously overdosed on calcitrol medication. I asked in the past about taking a medication to firm up the stool as, to be frank, it is a shitty job to clean him up. His doctor reports that it can be done, but if you go too much the other way, the ensuing constipation is potentially medically dangerous to undo, especially if you use osmotic medications which would interfere big time with his already fragile fluid balance.

Lastly, his learning disabilities mean that he is socially and physically oblivious to being in pads. By that, I mean it doesn’t embarrass him to be in pads, and it isn’t uncomfortable for him to be in an overly wet or soiled pad. If he wets/soils his clothes, his bedclothes, his wheelchair, his car seat, the carpet, a mattress – it’s all one to him. It’s certainly not all one to us. He depends on us utterly to maintain hygiene in his personal care as well as his dignity. I appreciate that I’m talking about his personal care publicly, but this situation needs to be read about and understood by more than Team Effie and families like us.

More than a few months ago I contacted the local incontinence service to discuss the product Joff uses as we were getting a lot of leakage. The reasons for leakage are multifactorial too, and I’ll say something about that in due course. However on this occasion, the incontinence advisor came to the house, saw me putting the pad on Joff and said that the leakage was happening because I was folding over the top of the pad before taping it shut and thus creating a problem. So I stopped doing that and things seemed to settle again. The lady declared that it was probably better for Joff to use a smaller size pad and she would sort us out some to try. Well, the samples never appeared and because it seemed so counterintuitive to what we’d seen as the solution; moving up a size, I never chased the small samples up.

Over the period of time since then, we’ve had a truly miserable time with Joff’s incontinence, mostly his weeing. On an average week, we will have about 5 or 6 washloads purely dedicated to clothes and bedding that he’s wet. Over and above the washing generated by a family of 5.

Some of the causes are.
He is simply getting bigger, drinking more and producing more wee.
Although the pads say they fit up to size 80cm waist, the gusset (as such) isn’t coping with the volume of urine produced.
He is sometimes having erections and so he is weeing out the top of the pad as he doesn’t have the nous to push his penis to point back downwards.
Some staff at school change his pad standing up and not achieving the best fit they could. This is currently under negotiation.
I feel that sometimes Joff wees, it fills the front of the pad and doesn’t wick backwards, so the next wee he does has nowhere to go but out the sides.

Some recent plusses.
He is coping with sitting on the toilet these days when we take him. When we take his pad off we now take him to sit on the toilet. On average he will have a bit of a wee perhaps 4 or 5 times out of ten. Nothing spectacular, but we hope that he will eventually make a connection in his head as to why he sits there and what happens after.
Previously, he really panicked sitting on the toilet, he had no idea why he was there and would hold onto your neck for grim life till you took him off. There is currently no way for him to ask for/anticipate needing the toilet and he still isn’t uncomfortable in a wet pad so we’re in a bit of a developmental stalemate as well as being limited by the supply of pads – perhaps if we had pull-ups that would make the proceedings a little easier. You see, pull-ups in the NHS is the gold standard of product. I have no idea how you’d go about getting them. That’s a pipe dream I don’t even allow myself.

Incontinence services up and down the UK are notoriously oppositional.
More than many parents and patients, I suspect, I sympathise and understand that there is a budget to stick to. Joff’s allocation is more than most because of his medical background and had to be backed up by a letter from the school paediatrician. Joff is allocated 6 daytime pads and 1 overnight pad, which has a better absorbency but bulkier to wear. I don’t know what the “normal allocation” is for patients in Fife, we service users don’t get to find out such things, the service is cloaked in secrecy.

No service level agreement, no checks with users about how the service is going for them, no clear path as to who to complain to if the service is going wrong.

For instance, when we first came to Fife the pads supplied were made by Tena. They were soon changed to a product by Paul Hartmann, a German company, whose various pads are called Molicare, or Moliform.
We had a meeting at the school hosted by the then incontinence advisor to allay fears about the new product.
We were shown just how amazingly absorbent they are, and all the advantages. Then she came out with this pearler… ( conversation paraphrased as far as I can remember it )
“when you take the pad off for your child to try using the toilet, and it’s not too wet, you can put it back on them afterwards”
One mum said, “my son can sit on the toilet for up to 25 minutes before he decides if he’s going to use the toilet, and you’re telling me to put a cold wet pad back on him after.”
“Oh yes, they’re very good to the skin it won’t cause infection etc.”
One lone dad who was there said, “how would you like it if I said to reuse a used sanitary towel after a bath/shower?”
“Oh that’s not the same thing at all, we want you to use these pads to their capacity and not throw them out just because there’s a bit of urine on them”

In another area of the UK, a man was told by his advisor to dry his wife’s wet pads out on the radiator before reusing. In Cornwall I heard that they were substituting disposable pads with a one off supply of a dozen terry towelling nappies regardless of the age of the client. Round about that time we were given a number of packets of pads in Joff’s size that another parent’s child had grown out of. This meant that we had a surplus, and the rumour going about at the time was that Fife were going to be cutting back everyone’s supplies so we continued to take Joff’s quota even though we had extras. Thus is the system that’s engendered. People are wildly conservative of personal supplies because we can’t be sure of future continued supplies. Recently though, we discontinued Joff’s supply to use up the backlog ( no pun intended ) and then had to begin again recently as we used the spare ones up.

Bloody idiots we were. Idiots.

The last delivery of pads to our house at the end of August ( they come in batches of 8 weeks supply at a time ) had a delivery note that said that Joff was sent a shorter quota of 4 pads a day and one overnight for that cycle. On Wednesday last week school asked for their customary packet of pads and I sent a packet in on Thursday morning…then utterly panicked when I realised that it was our last packet of daytime pads. We still had two packets of nighttime ones which is what threw me, I thought our next delivery was last Thursday.

So I phoned the incontinence service and told them that we had enough pads to last us till Saturday afternoon with our next due delivery at the end of October. Firstly I was told to reclaim pads from school as we have a fortnight’s school holidays just now. Secondly I was told his allocation was 5 and not 6. Thirdly I was told not to send packets into school, rather I had to send in a daily allocation ( somehow guessing how many times school would change him that day and send in that amount of pads – the reason being that if you sent in a packet the school would “use them” ) Fourthly I was told that the soonest a delivery could be made to us was today, the 19th Oct. ( see, Chile can rescue a miner in 3 hours, but a delivery of pads takes 5 days to appear across Fife )

The advisor and I had a meeting scheduled for Friday last week at 3.45 so that she could see how wet Joff usually is getting off the school bus. Friday morning I was in school, humiliatingly asking for pads back. When I got home later that morning, I was greeted with an answer message from her to say she had an emergency meeting at 3.30 that day and she would phone this week to reschedule.

I was so relieved. I never had dreaded a meeting so much. Me, aye, me who regularly takes on people from all services on Joff’s behalf. Part of me was ready to burst into tears, and the other ready to let rip at being put in such a vulnerable postion, being lectured about what pads I send into school…( HOW DARE THEY!) and generally feeling that the tone was – well if you’re going to have a child with disabilities, how can’t you have one that’s toilet trained.

So the weekend passed, and last night I had a restless night thinking of the supply coming today…would it be a full delivery, or a short one to catch up to the nighttime pads at the end of the month…and as the woman never phoned since Friday, when would she want to visit again.

We got a letter this morning directly from Hartmann, dated the 15th Oct. We don’t have supplies of your product, and we apologise for the inconvenience.

Now I don’t know about you, inconvenience to me is not getting a sandwich filling you wanted, running out of milk when the shops are still open, missing a bus and having to get a later one. Not having any incontinence supplies for someone who is permanently incontinent is a catastrophe.

Mr Effie phoned the incontinence service and asked them “what next then?” Lots of umming and ahhing and a promise to phone back. He called Hartmann who knew about the delay to deliveries last week – so I’m guessing that the emergency meeting last Friday afternoon was possibly something to do with that. Hartmann said that they thought products would be with them today and in Fife on Friday. Friday!!? says Mr Effie. Well, Thursday possibly.

Incontinence service calls and says that they’re reiterating that we’ll get supplies on Thursday as told to us by Hartmann. No, Mr Effie says, possibly Thursday…and that was said to keep us sweet. So what do we do till then? We have one packet of overnighters here you can have. You have to collect. ( so buggered if you can’t drive, don’t have access to car during the day ) He drove an hour to get the pads.

Incontinence lady was told in no uncertain terms that if the delivery didn’t come on Thursday that further supplies would be sent to our house – no more collection by us. She was told that we would continue to send a packet of pads into school on request, a system that has worked and worked well for 12 years. She was told that she would be dealing with him from now on as I was too upset and distressed by her attitude.

Now I have a friend who has been in various high heid yin roles for various national children’s charities, here and in NI. She often talks very knowledgeably about the politics behind services like education, health and social services. One of her recurring themes is of the professional as a “gate-keeper” to services. You have to fulfill conditions to get the support you need.

Never ever have I felt the gate keeper effect more than this week with this incontinence service and it is utterly hateful.

Have you ever been out and about and really really needed a toilet and remember the utter relief when you get somewhere because there is an underlying fear about wetting or soiling yourself. Imagine being told when you got to that blessed toilet that you’d already had your quota of weeing and pooing and you had to essentially negotiate the use of that toilet?

This has been a long post and isn’t quite the full story, but it’s enough for you all to get the flavour of the oppositional, idiotic, distressing beaurocratic hoops we have to jump through to achieve a support service for our son.

It isn’t the only support we need, but it’s pretty high up on the importance list. And day in, day out, we’ve still got all the demands our young man places on us and I think that this is a prime example of uncompassionate support.

21st century Scotland, you can only go to the toilet if we let you.

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I’m sending this to the blog at 2pm on Thursday 21st Oct and I still have no forecast from our incontinence service or Hartmann as to when Joff’s daytime pads will appear with them or with us.

So during this October school break, we are confined to the house until they appear.

That’s how I like to spend my precious time…waiting for help.