Stanmore, 1 year on.

On Friday 5th April I went to RNOH Stanmore for my 1 year follow up appointment.  I still cannot believe that it has been over 1 year since I was in, the time went by very quickly. The last time I was there the ward was still closed after the flooding under the floorboards had happeend.  Now that has all been fixed and they have swapped the mens and womens wings around so it was quite disorienting.

The first part of the follow up involved a group session to refresh things in our memories, catch up with how everyone was doing and any helpful tips that people have picked up along the way.  It was very interesting and I was quite pleased that on the whole it was a positive experience with very few negative comments.  It was nice to see some familiar faces, I got chatting to some of the people who had been on a previous course too.  They hadn't heard of e-motion/power assist for manual chairs so I told her about that, so I was useful for something!

After the group session I had a one to one session with one of the psychologists.  I had seen her for a few sessions in the last week before I had left.  We talked about how I was doing and what I wanted to do in the future.  I expressed some concerns about ongoing needs going forward once I am discharged from there.  I was very concerned about the lack of knowledge locally, most drs don't know what to do with me.  We also talked about a possible second stay, 2 weeks instead of 3 weeks.  Part of the plan for the second stay would be intensive physio and psychology sessions, I won't need to go in for all the other sessions that I had already been to last stay.

They have made some changes to the program since I was last there.  One of the big things was that before you only routinely went to the psychology sessions if you were on the pain management program but not the rehabilitation one. Now both groups of people do the psychology sessions, as they figured out that both groups of people had needs for them.  I think that was one thing that was overlooked while I was there, as for my group you had to ask for the help and I wasn't confident enough to do so until the last week, days before I was leaving.

The psychologist is going to put forward the recommendation that I go for another stay, then it will be up to Dr Cohen to say yes or no.  Apparently I shouldn't have a problem getting in for another stay because I can show that I'm trying to do the right thing and am applying the things that I have been taught so far.  I'm seeing Dr Cohen in May so hopefully it wont be that long of a wait until I know whether I will be going again or not.

'Moving Around'

This is the response that I got from my MP regarding the 'moving around', mobility component of PIP.  What do you folks make of his response?

Dear Mr H,

Thank you for contacting me about the Mobility component of PIP. I apologise this reply has taken some time to come, however I needed to seek clarification from the Department before getting back to you. I recognise that this is an area of some concern to many disabled people and I hope I can provide some reassurance.

First, I appreciate that there has been some confusion on the issue of qualifying for the enhanced rate of mobility by being able to walk 20 metres “reliably, repeatedly, safely and in a timely manner”. The change from 50 metres to 20 metres as the qualification for the enhanced rate of mobility is designed to provide greater clarification over how the assessment process will work. The 20 metres threshold is designed to act as an automatic qualification for the enhanced Mobility component because clearly if someone is unable to walk such a short distance, they are severely disabled. However, this change is not designed to preclude anyone who can walk 20 metres, being unable to receive the enhanced rate. The guidance has also been moved away from a focus on aids and appliances, to that of distance.

The key test for those who can walk further than 20 metres will be whether they are able to do so “reliably, repeatedly, safely and in a timely manner”. This therefore means that even those who can walk up 50 metres can still qualify for the enhanced rate Mobility component if for example, regularly walking over 20 metres would have an adverse effect on their health or leave them fatigued. I understand that, based on modelling that the Department for Work & Pensions has carried out, the number of people who score 12 points on Activity 12 as a result of this change from the draft assessment criteria is broadly the same.

I understand there is some difficulty including the term “reliably, repeatedly, safely and in a timely manner” formally in the regulations because defining this term legally in regulations is quite problematic, even though it is clear what this means for the PIP assessment. This term will be used as a guideline and it will also be in the contract with providers. However, the Minister for Disabled People, Esther McVey has also agreed to examine again whether this term could be incorporated into the regulations.

Finally, I would like to assure you that under PIP, the proportion of people who will receive both components at the highest rate will go up with PIP compared to DLA (20 per cent compared to 16 per cent), as will the proportion of people who receive at least one component at the highest rate (56 per cent compared to 55 per cent).  Ministers will also continue to spend about £50 billion on disabled people and their services. Moreover, the UK’s spending on disability-related benefits continues to be a fifth higher than the EU average which is a real testament to our country.

Thank you again for taking the time to contact me.

Best wishes,

Jack

Autonomic Clinic (NHNN) follow up appointment

This morning we left Bristol to go to London for my appointment at NHNN autonomic clinic.  The journey started off well but soon started going wrong.  The train from Bristol Parkway had no functional disabled toilets and this is not the first time it has happened on that particular route.  This meant I had to try to get myself into the tiny regular toilet.  I couldn't hold it in for the whole 2hr journey.  We should have got to Paddington at 13:00 and was about 30mins late.  The taxi ranks are a bit out of the way!  Luckily we had hospital transport booked so we didn't have the stress of navigating Londons public transport.

The autonomic clinic were running about 40mins late.  The waiting room for the clinic was so hot I thought I was going to faint. We had to wait outside where it was cooler.  The doctor I saw was very nice and I could actually understand what he was saying, compared to the last doctor who spoke way too quickly (One I nicknamed Dr Talks Very Fast).  I spent a lot of the appointment explaining my symptoms and answering a lot of questions.  I've already forgotten a lot of what was said, good thing they send a report out.

The outcome of the appointment was pretty much what I was expecting.  They diagnosed me with pots and syncope.  They gave me a prescription for fludrocortisone (florinef) to hopefully help with the symptoms.  I also have a followup appointment in July 2013.  I guess now I just wait for the report.

All in all a stressful but successful day.

HMSA Residential 2012

Friday we left Bristol to head up to Hothorpe Hall in Leicestershire for the HMSA residential.  The journey to the residential was pretty slow taking a lot longer than it should have done.  Being stuck in a car for hours in 30 degree Celsius heat is not good for someone with POTS.  There had been an accident on the M5 and what should have been a 2.5hr journey became a 5hr journey.

The residential started off well with a talk by Isobel Knight talking about her books; A Guide to Living with Hypermobility Syndrome: Bending without Breaking and the one that is due to come out next year.  She also talked about Bowen Technique and her work raising awareness of HMS/EDS-HM.  It was a brilliant introduction to the conference and the themes of moving forward and staying positive.

Saturday started with a talk from Dr Andrew Lucas (Andy) the Consultant Lead Psychologist from the Rehabilitation Programme at RNOH Stanmore.  He talked about his role in the psychological aspects of the rehabilitation programme.

After Andy was the talk by Dr Helen Cohen, Consultant in Pain Management at RNOH Stanmore also.  She did a talk about the pain mechanisms in hypermobility, the chronic pain cycle and how they attempt to deal with it at Stanmore.  It was a very interesting talk about the role of pain as an evolutionary defence.  Parts of the talk were the same from the last residential but there were some new points in there so it was worthwhile sitting through it.

The next talk was from Julian Walker of the Genetic Alliance about the importance of user participation and the role of the Genetic Alliance.  I had heard of the Genetic Alliance before but I was unsure of what they did.  I was surprised that they represent over 150 charities and they are involved behind the scenes to do with policies regarding genetic conditions.  Having a look on their website they have a lot of information available.

Rosemary Keer's talk was about Joint Protection, exercise and HMS.  She talked about the structure of joints and the importance of correct posture and the negative effects of incorrect posture.  She spoke about joint control, proprioception, exercises and braces, splints and supports.  This was the viewpoint of a physiotherapist and she used plenty of practical examples getting the audience to try different exercises and stretches.

I missed the majority of Dr Jane Simmonds's talk as I was flagging a bit and had to go lay down.  The conference room at Hothorpe Hall was very hot! Several people were flopped on the floor during a lot of the talks.  I kept having to lay down just to stay conscious.

Dr Jessica Eccles (Clinical Fellow in Psychiatry, Neuroscience, Brighton) did a very interesting talk about brain structure and the prevalence of psychiatric symptoms in hypermobile individuals.  This was probably the talk that I found the most interesting as some of the other talks were about stuff that I had heard a lot of before.  She mentioned that there seemed to be a link between Autism, ADHD, Dyspraxia and hypermobility.  This is something that a lot of us had observed anecdotally in the groups and on the forums that there were quite a few people that had both HMS and an ASD.  It would be interesting to keep an eye out for future research regarding this.

Before the party there was a wheelchair dancing display by the wobbly wheelers with an appearance by Dr Hakim as a Robot.  It was a lot of fun and I thoroughly enjoyed it.  In the evening was the 20th anniversary celebration party! It was a lot of fun! There were a lot of people in wheelchairs dancing and we even had a wheelchair conga line that was 14 wheelchairs long.  I laughed so much and it was refreshing to just have some fun.  Even the medical professionals joined in with the fun and one of them got accidentally walloped in the face and came out with a fat lip, poor lady had to go do a talk today at the EDS-UK conference! I'm sure plenty of pictures will appear on facebook or the internet in the coming weeks.

Sunday started with a talk by Dr Adam Farmer (Consultant Neurogastroenterologist) about the need for funding of gastro issues in hypermobility syndromes.  It was interesting to have the gastro issues explained in an accessible way as a lot of the information out there tends to be aimed at other medical professionals.  It helped me understand my own gastro issues a bit more.

The wonderful Hannah Ensor, Author and creator of the stickman products and Patron of the HMSA for children and teens.  She talked about adapting to life with HMS and her involvement with the HMSA.  I thoroughly enjoyed it, and made me think about my own thoughts about HMS.

Eloise Summerfield did a talk about medication and answered a lot of peoples questions with regards to medication.  It was very interesting about how the drug companies measure the effectiveness of pain medications, that this is measured by the amount of people who experience 50% pain relief not 100%. It was  surprising which medications came top of the chart with regards to effectiveness.  She reiterated what a number of the other professionals had said about not going on strong opioids such as morphine as they don't do a lot for chronic pain and come with a lot of side effects.

The event ended with a talk from Dr Alan Hakim about patient experience survey results.  There were a few pleasant surprises in there but much of it wasn't a surprise with regards to the delay between first presenting with symptoms to actually getting diagnosed was for a lot of people 10+ years.  He talked about how services are commissioned and emphasised the need for regional multidisciplinary teams rather than having one super centre based in London, that there needs to be a lot more change at the local level.

The slides from the presentations will be available from the HMSA soon and they were also filmed and the DVD will be available to buy too.  There are also slides and a DVD from the 2010 residential available.

All in all I had a fantastic time while I was there! Even though I felt like death warmed up I still had a wonderful time.  I would like to thank everyone who put in the hard work to make the residential fantastic and I look forward to the next one.

Autonomic/POTS Testing at NHNN

From the 20th-22nd June 2012 I went to the National Hospital for Neurology and Neurosurgery in London for testing to find out why I have been experiencing the sympoms that I have. The tests are designed to trigger the symptoms so that they can measure what was going on, so I was bound to feel dreadful after them which I did.   The testing was specifically for POTS (Postural Orthostatic Tachycardia Syndrome) and this is my experience of the testing.  

Day 1

Wednesday mum and I got the megabus to London and then the bus to Holborn. For some reason TFL told us to get off at Tottenham Court Road to get to NHNN. Stupid TFL, luckily a man on the bus told us a better way so the journey went pretty well, and we got to the appointment on time. The staff at the day care center were really nice, I quite liked Paulo one of the nurses who made sure that everything went smoothly.

The first afternoon of testing I had to have the exercise test, this was cycling whilst lying down. The scientist increased the difficulty after every 3mins. I managed to do the whole 9mins but the last few minutes my knees were quite sore. They took a lot of readings getting me to lay, stand and sit for 5mins at a time.

After the main tests I was hooked up to a 24hr BP and had to write how I felt each time a reading was taken, this was every 20mins sometimes less if there was an error. The band for the BP got very tight and left bruising on my arm. I was glad that it was only once an hour during the night otherwise I wouldn't have got much sleep. I woke a few times but not long enough to disturb my sleep a great deal.

Day 2

The next morning I went back to the day care center and there was some slight confusion about what I was supposed to be doing as they hadn't been informed that I had tests at 2pm that afternoon. The tests I had that afternoon were rather horrible. First they tested me lying down, after that they took readings while I did breathing exercises (long deep breaths then fast shallow ones). I had a cuff on my right arm, one on my finger on left hand and things on my chest. My finger went a rather deep shade of blue. They put my hand between some icepacks, my hand and half my fingers went blue, the fingertips went bright white, it ached in my bones.

Next came the dreaded tilt table test. The blood pooled in my feet pretty quickly and extended quite far up my leg. I felt rather sick quickly but I managed to last the whole ten minutes before I nearly fainted. My feet had turned a deep shade of blue and by that point so had my hands. Towards the end I came very close to fainting, luckily they stopped it when they saw my BP go down. It took a good ten minutes to recover with my feet above my head. They were supposed to do testing with my blood but they couldn't get the cannula in my vein, I did warn them that I have difficulty getting blood out.

Day 3

The third day started with a meal challenge which is complain with extra glucose that I had to drink. It was sickly sweet and made me quite nauseous. I had to lay there for about 45 minutes while my stomach digested it. They took measurements during this. I had a lot of wires on me and the blood pressure cuff, the good thing about having a bendy neck is that I could see the screens behind me. Obviously I'm not trained to read them but I found watching them quite calming. After the meal test we then had to do the testing that couldn't be done the day before because they couldn't get blood out. I had to hold on to hot water bottles and this helped a little with getting the cannula in my arm. They then tilted me up and left me there for 10 minutes. They took a sample of blood afterward, I can't remember what they were testing for.

After that I was done for the day and could go home early. Luckily the hospital had arranged transport so we didn't have to get the bus back to Victoria. Only problem with finishing early was that we had a long time to wait around because our bus wasn't until 7:30. I finally got home around midnight.

All in all the stay went well and I hope that they figure out what is going on with me. Now I just wait for the results and go to my follow up appointment in October. I will put up another post once I find out what is going on.  

Ehlers-Danlos Awareness Month

This month is Ehlers-Danlos (EDS) Awareness Month.  I was diagnosed with the hypermobility type of EDS in 2009. EDS is a connective tissue disorder it can affect many systems including the gastro-intestinal and autonomic systems.  I am currently awaiting testing for postural orthostatic tachycardia syndrome (POTS) which is a form of autonomic dysfunction.  EDS can cause widespread chronic pain and fatigue, but to most people you wouldn't realise it because it is a hidden disability and unless we happen to be using crutches, a wheelchair or other aids it is not noticeable to the average person this can lead to people being wrongly accused of making it up to gain access to strong painkillers.

A lot of doctors do not know about EDS and have not learned much about it since leaving medical school this means that a lot of people go undiagnosed for a very long time.  I myself started to get dislocations at an early age but wasn't diagnosed until I was 25.  This seems to be a common theme among EDS patients who according to the poll on the HMSA website 53% didn't get diagnosed until 10+ years after the start of symptoms.  Many have gone through several diagnoses before seeing someone who was knowledgeable in the condition.  There are very few clinics in the UK that specialise in hypermobility syndromes and the ones that are available are incredibly over subscribed.

My hope for the future is that more doctors learn about EDS and that less people have to go through the long journey to getting diagnosed.  The two charities HMSA and EDS-UK are doing great work to increase awareness of these conditions.  I am incredibly grateful to the HMSA and my friend Lisa as without their advice and guidance I would not have got the diagnosis and possibly would have had many more years trying to figure out what was wrong with me.  This years HMSA conference theme this year is "Moving Forward and Staying Positive"  so hopefully there will be a lot of positive information coming out of the conference this year.

State of me nearly 3 months post Stanmore

I thought I would take the time to update everyone on my progress since leaving Stanmore almost 3 months ago.  I have my 3 month follow up appointment and there are a few things that I need to rectify before that appointment.  I have to say that the last few months have been rather tough health wise so I haven't done as well as I would have liked to.

The first few weeks following Stanmore all I wanted to do was sleep.  Being ill with a glandular fever type illness didn't help matters.  I have found it very difficult to integrate what I had learned at Stanmore into my life here at home, it is not out of lack of motivation, somehow I find myself over focusing on other things and forgetting to do important things such as take medicines, eat and go to bed at reasonable times let alone all the extra things I need to do such as physio and exercise.  I need something/someone to prompt me gently to do these things otherwise they will not get done.

This year I have regained the weight I had lost over the last few months of last year which I am disappointed about but not surprised.  To have my lower surgery and the last part of my chest surgery I need to get my act together and get down to 12st and I'm currently just under 15st so I have a long way to go with that, the shame is that I actually got rather close to that goal weight but let it all slip away.  But this post isn't going to be a pity party, it is to clear my thoughts and figure out how to proceed.

My goals on my target sheet are as follows:

- Create a daily routine - This is something that I am really lacking in now that I don't go to the university anymore. I have no structure to my day and no reason to get up in the morning.  I wish I were able to work but I just am not able to right now, but that doesn't give me an excuse to stay up late and lose half the day because I didn't get up until nearly midday.  My expected level is to set up a computer programme and set up a timetable so this one will be achievable before I go back so that is now on my to-do list.

- Eat breakfast - Something that I do remember to do on the days that I'm up in time for breakfast so if I get my sleeping times in order then this should fall in line pretty simply.  It is usually lunch that I forget, I should eat smaller meals and often to help with my stomach problems and pots.I just don't remember to eat that often so it will have to be something that I have to timetable in to start with until it becomes habit.

- Uni study - This is one goal that I really have down which is good.  I am in the process of signing up to open university, my credit transfer has gone through and I just have to send in my disability form and register for the module that I want to take this year.  The module I'm taking is a 60 credit one so is half what I am used to taking so I'm hoping that I'll be able to cope with that amount.  I need to talk to someone about funding as well as I'm not entirely sure how that is going to work.  For this goal I am in the most favourable outcome so that is rather nice!

- Exercise - I haven't been that hot on doing a lot of exercise lately I must admit, a lot of days I forget to do my physio let alone exercise as well.  The expected outcome for this goal is to carry out exercises once a week so not too much expected so I can at least mange sorting that out in the next few weeks.  I now have a  trimbike that my mum gave me and have started using it today. Luckily my joints seem to be okay with doing it but my heart rate doesn't shooting all the way up to 150bpm

- Swimming - I don't think I have been swimming since I have been back home from Stanmore which is a real shame as I have some physio exercises that I could do in the pool and walking around in the pool will help my legs somewhat.  I am planning on starting to go to the disabled swimming at the local pool on a Thursday morning as they heat up the pool so it is warmer than it normally is.  My joints really ache in the normal temperature of the pool.  My expected level is to go swimming once a fortnight - once a month which is more than achievable.

On the whole the goals that I was given weren't all that difficult so I'm not entirely sure why I haven't been able to get my head in the right position to be able to achieve these.

Plan of action


- Be up and out of bed before 9am
- Do stretches after breakfast
- Set up reminders for all meds
- Take all meds
- Eat 5 meals a day of around 360 cals each
- Drink at least 3 bottles of drink a day (20oz water bottle)
- Do physio every day
- Bed by 11:30pm
- Swimming at least once a week

Once I have those few basics into my routine then I will add other things into the mix.  I don't want to try and change too many things at once otherwise I'll never do any of them.

My follow up is 8th June so I better get myself going with these things!

Appointment with Dr Cohen in November so that gives me a chance to get a lot of things back on track including the weightloss.