Rare Disease Day 2014

Today, 28th February 2014, is Rare Disease Day.

There are over 6,000 rare diseases and these effect 1 in 20 of the population.  That's 3,500,000 people here in the UK.

Very often there is no treatment for these conditions, and sadly little or no research into developing a cure or an effective treatment.

Most of the conditions are chronic and debilitating, and people with them live lives which are hugely impacted by the effects of these conditions.  (See my blog 21st September 2013 for an account of a "normal" day in my life.)

I have Dermatomyositis and Inclusion Body Myositis, both of which are rare diseases.  I was diagnosed with these in 1983 and 2000 respectively.  In addition to these, and as a consequence of them and the medication I am required to take, I also have:

Raynauds Phenomenon

Limited Cutaneous Systemic Sclerosis Overlap

Steroid Induced Diabetes

Pulmonary Fibrosis (NSIP Stable)

Hypertension

(Acute on) Chronic Lipodermatosclerosis

Lymphoedemarosis

Since 2008, I have been unable to stand or walk, and am now in a wheelchair full-time.  Prior to this I had great difficulty walking and had to use a walking stick, but could only walk very short distances, due to the weakness in my legs.

I use a BiPap machine, which helps me breath properly at night, so that I can maintain the proper levels of oxygen in my blood, and circulate this round the body.

Doing everyday tasks is always a struggle, due to the weakness in my arms, legs, shoulders and torso.  This means that doing even basic tasks, like making a cup of tea, can tire me out, and I have a constant struggle with fatigue.

I have carers who come to me three times a day to help me with tasks that most people wouldn't think twice about doing.  Without there help my life would be impossible.

I am supported at home by my mother - who is 82 - who does so very much for me.  Words cannot express how grateful I am for her help.

And yet despite all this, people look at me and say how well I look.  I have even had a doctor ask me why I'm in a wheelchair as I don't look ill.

Diseases, whether they are rare or not, aren't always obvious to a person looking in from the outside.  Likewise, a disability isn't always obvious, and just because I'm in a wheelchair people shouldn't jump to conclusions.

It isn't always obvious who has a rare disease.  Very often people try to hide it, to avoid having to explain a condition nobody else has heard of or understands.  Sometimes they will hide it, as people don't want to hear about them being ill, and for fear of being shunned by friends, and socially excluded.

Next time you're in a crowd, or with a group, try and see if you can spot who has the rare disease. 

A sightseeing trip to hospital.

Last Wednesday was time for my regular 6 weekly infusion of Methylprednisolone at the Royal Free Hospital in Hampstead, London.

My carer, Marta, came just before 6.00 am to get me up, toileted, showered and dressed, ready to be picked up.  Marta is a new carer and has only been coming for a couple of weeks.  She is Polish, but has been in the UK for sometime. She is very hard working, and has picked up what to do very quickly. 

After taking my tablets and having a quick breakfast, the ambulance arrives, and once I have been strapped in we are on our way about 6.50 am.

With all the flooding locally in South Croydon, Purley and Kenley, I am expecting the traffic to be bad, but we actually sail through without any hold ups.  From Croydon we travel by the usual route through Thornton Heath, Norbury, Streatham and Brixton, again with very little hold up.

We cross the river Thames over Vauxhall Bridge, passed the MI6 Building.  When we get over the river, we turn right into Millbank and drive along the river to the Houses of Parliament.  We go round Parliament Square, and the statues of past Prime Ministers, and then onto Parliament Street.  This takes us passed Downing Street, the Cenotaph and the back of Horse Guards, and then up to Trafalgar Square. 



We go round the roundabout and up the right hand side of the square, and follow the road passed Leicester Square and up to the Tottenham Court Road.  This route would usually be full of slow moving traffic but we manage to get through without any hold ups, so its a nice sightseeing diversion.

From there we head up to and through Camden, as the market is opening, and then up the hill to the hospital.  We arrive about 8.10 am, which is some sort of record.

Usually I'd have to dash to the toilet, after having sat in the ambulance so long, but we've been so quick I can go up to the ward, and book in, and then find a toilet.

Because I am so early the nurses are only just coming on duty, so they help me take my coat off, then make me a cup of tea - yes, I a being spoilt - and the go for their morning staff meeting.

Whilst I'm waiting for them to finish, Jacqui from the cannulation team, comes and puts a cannula in my left arm.  I've known Jacqui for years, and despite me having terrible veins, she is the only person I can confidently say will get the needle in first time.  Jacqui always calls me Anthony.  I'm not sure why, or how it started but its stuck and doesn't feel right if she now uses my real name.

When it gets to 9.00 am, I ring my respiratory physio to let her know I have arrived and to see when she will come and see me.

Just after 10.00, my drugs are ready and I am started off.  I have a 250mg bag of Methylprednisolone, which takes 1 hour to run through.  The line is then flushed with saline, but this is only about 10 minutes, so its not too long before I have finished.

It was then a case of waiting, reading my book, before the respiratory physio turns up.  She arrives just after midday.

I have known Christine for about 10 years.  Issues with my breathing had previously been dealt with at the Royal Brompton Hospital in Chelsea.  Then a new consultant had been recruited at the Royal Free Hospital, and after some discussion, it was thought best if I transferred to Professor Wedziche, and have most of my treatment managed under the same hospital.  As part of the Professor joining the Royal Free, she had brought Christine with her, as one of her team.

Sadly, Christine advised that she is retiring in the next couple of months.  This is a great shame, as not only is she very nice person, but she is really good at her job.  She really goes out of her way to ensure that if I need equipment for my BiPap machine, that she gets this quickly and looks out for the best available options.  She is also very quick at spotting problems and dealing with these.  I have met her replacement, Steve, and he is also nice, but its always a shame to lose someone from your medical team, who you like and trust.

Also, Professor Wedzicha is leaving to return to the Royal Brompton.  She has offered to take me with her as a patient, but I haven't actually needed to see her for the past 6 years, as Christine is so good, so we agree that I'll stay with the respiratory team at the Royal Free, but if there are any problems, the option to transfer is still there.

We chat about a few things for next time, and the Christine is on her way.

I make my appointment for my next visit, and then head off to the transport lounge to wait to be taken home.  After a little over an hour waiting, a driver comes and collects me.

We have to drop two other patients off on the way, local to the hospital, but after that we are off home.  There is much more traffic and this means the journey home takes over 2 hours, but I'm indoors just after 4.00 pm.

It has been a long day but worthwhile, and its always nice to get to see the sights of London.

Mobile phone masts!! - Beware........

In 2000, I moved into a new house.  It was a mid-terraced house, in a quiet cul-de-sac, with a very nice park at the end of the road.

The house was relatively cheap, as it needed some working doing, but this was mainly cosmetic, so I thought I'd be able to do most of the required work.  It had a nice garden, with a very high wall at the end.

A few months before moving in I had been diagnosed with Inclusion Body Myositis (IBM), but I was still physically quite fit, and was able to walk easily, with little problem.  To give you an idea, on the day I moved in, I stripped the wall paper in the front room, because it was so awful.  I can't imagine that now.

Anyway, having settled in I started on the work indoors, getting professional help as and when I needed it.  I needed a bit more help in smartening up the garden, but I still managed most of this, including laying a crazy paving path up the garden to a decking area I built.

As time went on I started to slow down, and things became more difficult.  I struggled with the work around the house, and was always having to have rests, where I would invariably fall asleep.  I put this down to the progression of the IBM.

Mum, used to come and stay with me quite regularly, and always complained about feeling lethargic when she was staying.  When she went home, within a couple of days she started to feel much better.

As I got worse, mum came and stayed more.  But the lethargy she felt seemed to come on more and more quickly.  It seemed that as soon as she stepped through the door she started to feel tired.

As I said, at the bottom of the garden was a very high wall, and behind this was an industrial unit.  This was occupied by a cable television company, and the site was covered with satellite dishes and mobile phone masts.

Mum became convinced that these dishes and particularly the mobile phone masts were the reason for how she felt when she came up to me.  I was a bit sceptical, but it did seem to be the only explanation.

In 2005, I sold the house and moved to the bungalow where I am now.  Physically by this time my walking was terrible and everything was a struggle. 

However, within days of moving I started to feel a bit better.  I was less tired, had a bit more energy to do things and even a bit more strength.  It was also as if I'd been given some drug to pep me up.

Now obviously, these things are relative.  The boost was only small, but it made a big difference to me.  And mum has never complained about the feeling of lethargy she had at my old place.

And now we might have a terrible mobile phone signal, but there isn't a mobile phone mast within 1 km of the house.

I mentioned this to my consultant at the time, and he was interested to listen to what I had to say.  I'm not sure he ever did anything with this information.

I'd always been a bit sceptical about stories on mobile phone masts and their effect on peoples health, but after this experience, I wouldn't say I'm a believer, but I certainly would never move close to one again.

 

How do you cope?

A about a week or so ago, someone posted on Facebook about how they try to maintain a positive outlook and how this helps them get through the day and cope with their condition.  This gave rise to other comments both from people agreeing that a positive attitude helps, and some from people with a more negative outlook.

Now I'm not going to tell you that I've got the answer to what attitude will help you best, or that you should follow my example.  Each of us, needs to find our own ways of coping that work for us.

When you're diagnosed with a chronic condition, and when the prognosis starts to sink in, along with the realisation of the impact this will have on your life. it is inevitably going to have an effect on how you feel and how you approach life.

To me there are two ways of going.  One is that it can overwhelm you and take over your life, and this can lead to depression and despair.  I often describe this as being a miserable old man, who sits in a chair and spends his life moaning about how awful his life is.

The other, is to accept what you have got, and to get on with your life.  To concentrate on the good things in your life and get on with living it.

And I realise that that's a fairly simplistic way of looking at it, but most people to some degree fall into one of these two areas. 

I was diagnosed with Dermatomyositis at age 17, and to me I had too much life left to live, to let it take over my life, or to define me.  I remember a friend saying to me, that he could not believe how happy I was, and how I could laugh about the things that were happening to me.  My reply was, "Well how else am I supposed to be?"

It wasn't really a conscious decision to be like that but I was too young to let this condition control who I was and what I could do.  It just seemed better to get on with my life and live it as best and as fully as I could.  I'd always rather have a laugh and a joke about things, and didn't see why that should change now.

You can say that this attitude is brave or strong, but to me it just seemed the right way to approach things.  Either that, or I was too stupid to really understand what was going on.

I'm quite a practical man.  My job was as a Business Analyst/Project Manager, and some of that role meant that I was faced with problems, and needed to find the best solutions.  I also needed to make risk and cost benefits assessments.  I have transferred those skills to my personal life and to how I approach my condition. 

When I find I can't do something, or something is becoming more difficult, I will think about the best solution, and then look to see what is available to help me achieve what I want.  Sometimes I don't get the answer first time, but generally I get there in the end.

Recently I've been having a problem with a sore and uncomfortable bottom.  This has impacted on my standing in my Levo C3 stand up powered wheelchair.  My initial thought was that I needed to get a better cushion for the chair, as the one currently there is quite thin.  However, after looking at various options, it was apparent that the existing cushion was the right one for the chair, as you could not have one any thicker, as this might inhibit how you stood in the chair.

So I then needed another solution.  I looked at various pads that you could place on the cushion, but the difficulties with these were that they would move during transfers and then not be positioned correctly once you are fully in the chair. 

Eventually, I started looking at silicone pads, similar to those that women use to enhance the size of their bottoms.  (Why?).  These pads were soft and were designed to go on the bottom, exactly where I was getting the discomfort.  They could easily be put in place after I had transferred and not impact on my standing.  So I bought a pack, and they work.  It might not be the solution that works for everyone, or that anyone else might think of, but it works for me.

Many people have faith, of one kind of another, that they use to give them strength to help them cope, and I wouldn't knock that for a moment.  You need to use anything you can, and draw inspiration and strength where you can.  I don't have any faith, but I understand its place, and I would never criticise anyone for holding on to their beliefs.

However, there are two sayings which people often say to justify why you have a condition.

The first is "Everything happens for a reason." 

I simply don't believe this can be true.  What possible reason can there be for all the pain, discomfort and suffering that occurs in the world. 

I have been friends with a couple since we worked together in the late 90's.  About 9 years ago, she was diagnosed with breast cancer, and despite repeated treatments and operations, eventually it spread, and she passed away after a battle of almost 2 years.  She left behind a husband, who had lost his soul-mate, and a young son, who had lost his mum.  What is the reason for that?

Things happen - and that's it.  There isn't a reason I can see, or some grand design that we aren't party to.  Whether its a good thing or a bad thing, they're just random things that happen.

The other is, "You get what you can cope with."

Well that covers everyone that copes well with things.  But what about all those people who don't cope.  The people who find themselves ill and then hide themselves away.  The people who sit and moan, and complain about how hard life is and how unlucky they have been.

To me, things happen, and how you cope is down to the individual.  For me, I have a chronic condition - or two - but I won't let it control me or define who I am.  I'd rather be positive about my life and the way I can live it.  Concentrate on what I can do, rather moan about what I can no longer do.  And I'd much rather have a laugh and a joke.

But that might not work for everyone.

Each of us needs to find what works for us.  I'm not going to criticise someone for being negative, or for getting down about their conditions.  Nor am I going to put someone down for holding a faith, if that helps them find solace and strength.  Its about finding a way through, to enabling each of us to get through the day, and hopefully make the most of our lives.

And this is why the support groups, many of us belong to, are so important.  Because they bring all of us together, with our different personalities, ideas, faiths and attitudes, and enable us to be there for each other.  Not to judge or criticise, but to say, "I'm there for you," regardless of who or where you are. 

Carers - Again

I like to do the occasional blog about carers.  Hopefully it will help some of you understand what is involved in having a carer and how they work.  It might help you see some of the problems and difficulties that can arise with carers.  But mainly I do it to as a way of trying to show that having a carer isn't always a bad thing, that despite what the media report, in the main most carers are very good, and as a way of expressing my thanks to the carers that have come to me.

Currently here in the UK, the work of a carer seems to be massively undervalued, and as a result under funded.  There was a report yesterday that highlighted how little is paid by local authorities to fund carers.

Most carers are supplied by agencies, employed by the local authorities to provide their home care services.  To do this the local authority pays the agency a fee, which is supposed to cover the carers wages, tax and insurance contributions, supply of gloves, and other protective items, uniforms and ID badges, travel costs between jobs, and then the agencies costs which amount to about 30% of the fee.

Currently the average rate paid by councils to agencies is £12.26 per hour.  However, in one case it was found that a council was paying as little as £9.09 per an hour.

The UK Homecare Association is pushing to have the rate per hour increased to £15.19.  At this level they say the standard of care provided would increase and carers could be guaranteed to receive the national minimum wage of £6.31 per hour, for over 21's, something they don't necessarily get.

My local council currently pays £12.56 per hour - so above the national average.  The carers receive £6.50 per hour from the agency, but they have to pay for their own uniforms and do not receive any travel expenses.

Carers are employed on the dreaded "Zero-Hours" contract, which means they do not have any guarantee of work, week to week.  They do not receive basic benefits such as holiday pay or sick pay.

The job is physically demanding and very often means working very long days, usually 7 days a week.  To give you an example, I'm 6ft 2 inches tall, and the best part of 96kg (about 210lbs), and first thing every morning I need to be helped from lying down in bed, to sit up on the side of the bed.  I can do very little to help, except move with the carer as they get me upright.  Its a bit like trying to move a very big sack of potatoes - do it right and its OK, do it wrong and its difficult and could lead to either my carer or me, being hurt.

My carers come to me about 6.00am in the morning, but they will very often say that they didn't finish work until gone 10.00pm the previous evening.  Margaret, my regular carer, has been known to come into me, to put me to bed, after midnight, because an emergency has arisen - such as a client being on the floor when she gets there - but she is still expected to be back at work early the next day.

When I first came out of hospital and rehab, I didn't know what to expect from carers.  I had seen all the horror stories in the media about carers, about mistreatment and abuse, and these made me nervous of what to expect.  Naively, I though I might need a carer for a few weeks, and the once I'd adjusted to being in my own home, I could do without them.  Well, five and a half years later, I'm still waiting to make that adjustment, and realise that having carers is now an essential part of my life, and one of the things that allows me to live independently.

I have had some bad carers.  Carers who aren't really interested in what they are doing.  Carers who come in and you know straight away that they won't last very long.  Carers who have no idea of time keeping and how important having a regular routine, with carers coming at a regular time, is.  Carers who keep needing to be reminded of what they need to do.

But generally the problems that arise are as a result of the agencies administration staff.  They are generally people who have never done the job, and therefore don't know what's involved.  They give carers unrealistic work schedules, without factoring in travelling time, so that a carer can leave me at 6.45am in the morning but be expected to be at their next client 3 or 4 miles away at 6.45am.  But if the office gets a complaint about the carer being late, they will never accept that it is the office staffs fault and always blame the carers.  They are disorganised, leave arranging cover for carers going on holiday until the last minute, and communicate very badly with clients.

However the vast majority of the carers who have come to look after me have been excellent.  They understand what needs to be done, and allow me the time to do what I can, without taking over or rushing me.  They are friendly, and we can chat about things, and hopefully have a laugh.  They help me with little things, that don't necessarily come under their job description, and are kind to Mum, which counts for a lot.

On the 19th January this year, it was 3 years since Margaret first came to look after me.  She has been coming ever since.  Sometimes all three calls in a day, sometimes only one or to, but she has been a regular carer for all that time.  She is more like a friend now.  We know about each others families, and she regularly talks with pride about her little girl, Dora, and how she bosses them all about at home.

I have other carers who have come to me on and off for the same period, but no one as consistently as Margaret.

I have said before that a bad carer can be a negative influence on your life.  The can cause you stress and anxiety.  If they do their job badly, this leaves you uncomfortable and sore, and possible even dirty.  If  they come through the door and are unhappy or uncommunicative, this leaves you feeling nervous and vulnerable.

A good carer is a very positive influence on your life.  Seeing them can just give you a lift.  If they look after you well, you feel smart, clean and worth something - just all round better about yourself.  And if they are the only person you see all day, then its better if its a happy face that you can talk to properly.

So here's to the good carers.  To Jane, to Mary, to Natalia, to Jason, to Marilyn, to Hildreth and to all the others who have come, and gone, but have been a positive influence on my day.  And especially to Margaret, to whom I simply don't have the words to express my gratitude.

We seriously undervalue carers, as we do many in the caring professions, such as nurses.  Until we start to appreciate them, not just in terms of the money we pay them, but also for the importance of the job they do, and the effect they can have, then we will always have a problem with care provided to disabled and elderly people.