Wednesday, 28 April 2010

Social Services and Care Support

I have just found out from my social worker today that a care plan has been approved for me to provide me with a carer to support me with personal and social tasks.

Social services have approved the following support for me:

1 hour 3 times a week for a carer to come and give me a bath and get me into bed.
2 hours twice a week for a carer to take me to do my shopping or go to appointments.
4 hours once a month for social stuff eg going for a night out, going for a girly shopping spree with my mum or friends etc.
1 hour once a week for a carer to clean my flat and do my ironing.

There is also a contingency fund so that if my condition deteriates at times and i am stuck in bed they will send a carer to get me out of bed, dressed (if possible) and give me a meal and then come back in the evening to get me back into bed.

I am so happy i can't believe how much support they have said i will get. At first it was only going to be 2 hours twice a week to go shopping and give me a bath. I really hope that getting all of this support will make a big difference. I especially feel hopeful that it will get me back out there and getting a social life back. Iv lost alot of friends over the past year due to my condition deteriating and that is the one thing that i have found hardest of all. I have one friend who visits me regulaly and takes me out in my wheelchair but as he has a milder form of the same condition, he is finding it harder to push me. I also hate my friends having to push my wheelchair or having to rely on friends to go to the bar to get drinks, or help me find a disabled loo, carry my shopping etc. To be honest, I hardly see anyone now so I don't even have the chance to ask them to do these things for me.
It will be great if I can get out more and start spending time with friends again knowing that I won't have to ask them to do anything for me other than just enjoy our time together.

My social worker has spoken to the care agency and they have carer in mind who they said should really suit me. I don't know anything about her yet other than she is young which is what I wanted. That way when we go shopping or out on a night out it will just look like I am with a friend rather than a carer or an older relative.

I feel like this is a massive breakthrough in my region as I don't think there is anyone else with hms/eds who gets care support , or at least to this level. I'm hoping that with them approving this amount of support for me it will make it easier for others with hms/eds to get better support in the future.

People with hms/eds find it extremey difficult to get this type of support due to the lack of understanding of the severity of the condition. It makes it even harder to get support becuase the condition can fluctuate so much within such a small amount of time. This often means that when people with the condition do ask for help, it is refused becuase it is assumed that they can function much better than they can. It is also hard for service providers to provide care on the basis that the support is helpful becuase it PREVENTS deteriation and injury rather than it being as a result of deteriation or injury.

Of the few hundred people I know with hms/eds from the forum and facebook, only a very small handful recieve any support with care. Those who do either have another condition that has justified they need for support or they are at the extreme end of the spectrum being completely dependant on others for even very basic tasks. They often also have children with the condition so the support they recieve is often more support for them to be able to care for their family.

I know of a few people in my town alone, and more in the surrounding area who would benefit from some kind of support from social services, either in terms of care support, or simply with loan of equipment such as bath seats, electronice raising beds etc. I feel positive that with the local social services accepting and approving this level of support for me, that it will mean that others in the area will find it much easier to get the support they need too.

Big step forward for recognition of hms/eds in the care system today!!

Tuesday, 27 April 2010

eds/hms videos

here are a couple of videos that look at eds/hms. Not up to posting a long blog today due to a naff day pain wise but very pleased to say i passed my driving theory exam today Woohoo!!

here are the links to the videos. enjoy!

Sunday, 25 April 2010

My bendy story

Like everyone with hms/eds, I was born with the condition. There were obvious signs something was wrong very early on but due to the common lack of knowledge of the condition i did not get diagnosed until I was 27years old, by this time the condition had become quite bad.

Although There didn't appear to be any problems when I was born, I did have hms/eds symptoms as an infant. I did not walk until I was 4 years old, which is common with hms/eds, but this was put down to me developing slowly.
When I did start to walk I would become tired easily and it took my parents a few years of hard work to get me to walk normally.
I complained of pain in my legs from an early age but drs put it down to growing pains (again very common for people with hms/eds). At age 12 I started to get alot of pains in my chest. This was the start of my ribs dislocating (yes ribs can and do dislocate in people with hms/eds although alot of drs will swear blind they do not, even when they see it themselves). Again, after various hospital visits and lots of tests, it was put down to growing pains.

I injured myself very easily and was covered in bruises and sprained joints as child but it wasn't until I was 20yrs old that I myself started to think something wasn't right. I started having problems with back pain which I visited my gp for on a few occasions but this was dismissed as being 'psychological' rather than a physical issue. I also started having more and more problems with my digestive system which was put down to IBS as a result of 'stress'.

By the time I went to uni at the age of 22 I was struggling more and more with poor energy levels and widespread pain. I studied performing arts and a weekly dance class would leave me stuck in bed exhausted and usually with at least one injury. My 'IBS' became much worse and I was eventually seen by a specialist who tested me for Crohns disease (which runs in the family). However no crohns showed up so again it was diagnosed as IBS. In my 2nd year at uni I spent a month bedridden due to the back pain and stomach pain become so severe that I could not move. Eventually after lots of rest things improved and I was back to my normal self.

At age 25 things started to deteriorate and my symptoms became more and more frequent. I was finding walking tiring and was becoming more and more fatigued. Even after a good nights sleep I would need to go to bed for a few hours in the afternoon. My back pain become more severe again and the pain episodes became more frequent. Again I asked my gp for help but once again it was put down to depression, which was becoming the constant cause of my problems according to drs.

When I was 27 I was becoming less able to stand and walk. I started to use a walking stick to help me stay on my feet longer and even started hiring electric wheelchairs from Shopmobility (a wheelchair hire company) in order to go shopping. I noticed at this point that my toes were curling under each other. I thought that maybe this was the reason I was finding walking harder so I decided to visit a private chiropodist. I was fed up with my gp at this point who was equally fed up with me.
The chiropodist I saw was the first person to mention the word 'hypermobility'. I had never heard of this before so didn't think much of it when she told me the bones in my feet and ankles were very hypermobile. She gave me some orthotic supports to wear in my shoes which would lift the flat feet and support my feet. I went home and decided to look into hypermobility rather than go to my gp as she had advised. I was gobsmacked when I came across hypermobiltiy syndrome as I was like someone had written all my problems down and given it a name.

I couldn't believe how much the condition fitted my history or problems. I was amazed how I had had so many obvious signs of the condition but no one had picked up on it.
I didn't dare go to my gp and ask for a referral to a rheumatologist as advised by the information I had found because by this point the word 'hypochondriac' was beginning to appear on my medical notes. Alot of people with hms/eds are labelled as hypochondriacs or attention seekers by gps and drs who refuse to take the person's concerns seriously. When tests continue to come back normal and more common conditions are ruled out, gps often put the symptoms of rare conditions like hms/eds down to psychological issues.

I carried on as I was using my walking stick and spending more and more time in bed and less and less time doing things. After about 6months of finding out about hypermobility syndrome, my back pain kicked off big time. The pain level was so high that I was spending more and more time unable to move and stuck in bed crying with the pain. I was trying everything I could including heat wraps, hot baths, massage, over the counter pain relief and even psycological techniques but nothing worked. My hips and knees were now becoming very wobbly and were starting to dislocate. One night my knee dislocated badly while in bed and it took me 30 minutes before it eventually went back into position. The next day I went to Boots the chemist and asked the pharmacy for the strongest pain relief they had. She asked what problems I was having and she said that I needed to ask my gp to refer me to a rheumatologist to examine me for hypermobility syndrome. After another week I finally bit the bullet and made an appointment with my gp.

I took the letter the chiropodist had written for me and told him all about the problems I had had and showed him information on hypermobiltiy syndrome. He was not impressed at all. He told me that I had no right to assume I knew my body better than he did and that I had to stop trying to seek attention and to stop making things up. I couldn't believe how horrible he was. I had gone to him with loads of information and the possible key to solving the mystery of years of endless symptoms. I burst out crying as I thought, 'this is it, this is the end, I'm never going to find the answer'. Crying obviously reinforced his opinion that my problems were down to depression but he told me he would refer me to a specialist just to prove to me there was nothing wrong with me.

The day I saw the rheumatoglist was the happiest day I had had in a long time but also, just like with my gp, became a day of having any hope of help crushed.
The specialist examined me testing the movement in every joint. At one point my hip dislocated as he moved it which made him panic. Eventually after 20mins of prodding and poking he confirmed that yes, I did have Hypermobility Syndrome. I couldn't believe it! I was so happy I had finally been told it wasn't in my head.
But then it turned sour. The specialist told me that the condition doesn't really cause any real problems, that it just means you are very bendy and that it can actually be a good thing if you are a dancer or sports person. He said that the level of pain I said I had couldn't be caused by the condition and so he was doubtful that my pain was a bad as I said it was. He also said that I shouldn't need a walking stick and that i just need to exercise more and spend less time in bed. The exhaustion, he said, was also not related. I was devastated. I had found a forum on the Internet for people with hms/eds and had read that many people had experienced the exact same thing. They finally get a diagnosis but are told that its not that big a deal and to just get over it and get on with your life.
I was offered no treatment. None of the recommended things I had read that would help such as physio, pain medication, splints etc.

I went home very confused. I was so happy I had finally got a diagnosis but had no idea what I was going to do about it. I decided to change gps that day as I had succeeded in proving to my gp that he was wrong and did not look forward to having to see him again.

My condition continued to deteriorate over the following year and eventually I was refereed to physiotherapy after a very bad dislocation of my shoulder. At last I was going to get help. However I was seen by a trainee therapist who, after just 3 sessions, told me that physio won't make any difference and I was discharged.
My new gp was more sympathetic than my previous one and by this time had put me on codiene to help with the pain. He also refereed me to be assessed for a wheelchair and also refereed me to a specialist nurse to help manage the digestive system problems I was having by that point.

After a further year of my gp trying to help me, I discovered there was a dr in Leeds called Professor Bird who specialised in Hypermobility Syndrome. He had a specialist clinic of physiotherapists, occupational therapists and other staff who he had trained to treat and understand hms/eds at a high level.

I asked my gp to refer me to see this amazing dr who had developed a great reputation on the online hms/eds forum. My gp agreed.

6months later I had my first appointment with Prof. Bird. He examined me and took a very detailed medical history of myself and all of my family. He confirmed I had Hypermobility syndrome and that most of my joints were affected. He also told my mum who was with me that she too had the condition due to her medical history and from what he could see of her joints. He also said that it sounded like my father also had the condition and this is why, by this point, the condition had become very serious for myself.

After taking xrays and blood tests he refereed me to see his physiotherapists and occupational therapists.

I went home extremely happy that at last the pain and exhaustion I had were genuine and that I had been right all along to fight for someone to take me seriously. However I was also very upset that it had taken so long and as a result my joints were now in serious condition.

Prof. Bird advised my gp to put me on a high dose of tramadol and amytriptaline for pain relief. He also asked that I would be refered to see various other specialists to look into the related problems I had.
A few months later Prof. Bird contacted my gp to say that the xrays I had done showed that I had already developed osteoarthritis in my lower spine and that the Sacralilliac joints (where the spine joins the pelvis) were degenerating. Although this can be a result of hms/eds, because of the family history of crohns disease he wants to look into this again as this type of degeneration of these joints is often a result of crohns diesease.

My physiotherapy and occupational therapy assesments confirmed that my joints had become very unstable and weak as a result of recieving no treatment for so long. I was refered back to my local physio department under a senior therapist with the advice that I should recieve treatment indefinatly on a weekly basis. My new therapist is amazing and understands the condition so well.
I was given various splints for my wrists and hands by the occupational therpaist and advised to ask my social services to provide me with various aids such as a bath seat that lowers me into the bath, as well as equipment to lift me up to sitting position in bed.

I am due to see professor bird again in about 6 weeks time. I am hoping that he will have more information on my test results and has decided whether to be tested again for crohns disease.
Prof. Bird is due to retire in the summer so I am hoping to be reffered to another rheumatologist in Newcastle who also specialises in hms/eds.

I now have a social worker who is in the process of setting up a care plan for me in order for me to recieve support from a carer a few times a week in order to help me bathe, shop, and make meals.
I am also waiting for a new wheelchair that has been made for me by the nhs. The new chair is much lighter than my current one so I should be able to push myself. Its also designed for much more frequent use including for inside my home as my mobility has become less and less.

I am also having driving lessons in an adapted car so that I can remain independant.

My story shows just how quickly and rapidly hms/eds can deteriate when no treatment is offered. It shows how much damage can be done by ignoring the problems and why it is essential for gps and drs to gain more understanding of connective tissue disorders.
I am luckier than some as I am now receiving very good support and treatment. Even though it was a long hard fight, some people are still fighting.
I have no idea what the future holds but I do know that I am unlikely to be able to walk properly again and that my condition will continue to deteriate rather than improve. I have learnt alot in the past year about how treat my pain effectively with medications, TENS, and heat treatment. I have also developed more understanding of the importance of pacing myself strictly so that I use the little energy I have effectively. I have learnt not to feel guilty or upset when I am unable to get out of bed and to feel ok about not being able to do alot of things that I used to be able to do.
I feel much happier in myself and while my quality of life has reduced a great deal, I no longer suffer from depression and feel very positive about life.

I hope that in the future more drs gain understanding of the condition and it my dream that one day, people with hms/eds recieve a diagnosis and appropriate treatment very early on so that no one has to become as disabled as I am.
It is also my dream that one day a cure will be discovered so that people with hms/eds can at least be prevented from deteriating further.

What is Hypermobility Syndrome/Ehlers Danlos Syndrome

Before I start blogging about living with Hypermobility Syndrome/Ehlers Danlos Syndrome (hms/eds) it makes sense to explain what the condition is about.
Alot of people have never heard of hms/eds and those who have, often don't understand what exactly it is, after all its a very complex condition that even most drs don't even understand.

(added 2010)---
Hms/Eds is pretty much the same condition. Previously Hypermobility Syndrome and Ehlers Danlos were thought to be different conditions (with Ehlers danlos being more severe). However Drs have now agreed they are in fact the same condition, or there is so little difference between them, that people with either 'diagnosis' need treating exactly the same. So they are now seen as the same condition. Ehlers Danlos:Hypermobility Type. This is why often a person who is diagnosed with hms often hears drs refer to their condition as eds.

So in this blog I will use the term hms/eds. (in later entries I will refer to it as simply EDS).
They are connective tissue disorders which basically mean there is something wrong with the person's connective tissue, Collagen. You are born with hms/eds, it is not something you suddenly get, although some people (like myself) have very few problems until they reach their 20s). It is an inherited condition which means at least one parent will usually also have the condition however it often becomes more severe each time it is passed onto the next generation.

Collagen is a protein based material that has the basic job of allowing body parts to move. Collagen is present in the joints, mainly the ligaments, tendons and muscles, organ tissue, and skin. Its job in the joints is to allow the joint to stretch and move but also stops it from moving too much. If our joints didnt have a 'stop' point then our joints would not be able to support us and basic movemnets would be impossible. Our joints would fall apart. This is basically what happens in hms/eds. In people without hms/eds, the collagen is made up of fibres that stretch like an elastic band. If you stretch an elastic band eventually it snaps back to its 'unstretched' state which stops the elastic band from snapping. In hms/eds the fibres are like chewing gum rather than an elastic band. If you stretch a piece of chewing gum you know that eventually it tears and you end up with sticky gum all over your fingers! This is what the collagen is like in people with hms/eds. The collagen stretches and stretches but does not snap back. This means the joints stretch and move but move and stretch past the point they are supposed to. They are very bendy and wobbly and don't snap back into place and eventually when a joint stretches past the point its supposed to, it dislocates. Ouch!!
In hms/eds the main symptom is joints that dislocate on a very regular basis, often many times in just one day.
When you have joints that dislocate easily and joints which are bendy and wobbly, they don't do a good job of keeping you in one piece. Your body becomes very weak and unstable. Just trying to stand up can be impossible as you may have knees and hips that don't lock and wobble all over until you end up in a pile on the floor. This makes walking pretty tough too.
If your spine is unstable it makes it difficult to sit up straight and also contributes to the problems with standing and walking.
In the upper body, bendy joints in the shoulders, elbows, wrists and knuckles make it difficult to lift things, push yourself up from a lying position, and use things like cutlery, a toothbrush, a pen and even the crutches and walking sticks that help you to stand and walk become difficult to use.

Because of the bendiness of the joints, a person with hms/eds will use their joints over a 1000 times more in one day than a regular person. This means their joints grind and rub against the ends of their bones many more times than someone else of the same age. As a result the joints and bones get worn down very quickly which results in a very early development of osteoarthritis. People with hms/eds usually develop osteoarthritis as a result of degeneration of the joints as well as inflamation of the joints often before the age of 30. They also can develop osteoperosis, where the bones become weak, very early on.

Becuase collagen is present in organ tissue as well as the joints, people with the condition can also have problems with their organs, in particular, organs that are designed to move. The bowel and intestines work by muscles contracting to push food and waste through them. Because the muscles in hms/eds are much laxer(more relaxed and stretchier), they don't contract as well so in the bowel and intestines, the muscles don't do a very good job of moving food and waste along. This means that people with the condition often have alot of problems with the intestines and bowel, in particular ibs, slow transit, incontinence and other similar problems.
The same goes for the bladder too.
In women with hms/eds, the womb has to contract much harder during her period in order to shed the unused lining (this is basically what a period is). This results in very painful and very heavy periods. It also makes it difficult to maintian a pregnancy and many women with hms/eds experience more miscarriges and problems conceiving.
The heart is another organ that can be affected due to the amount of movement it requires to work. In certain types of Ehlers danlos, the heart can have serious issues such as heart murmers, slow and irregluar heart rates. People with the vascular type of Ehlers Danlos are at great risk of anurysisms and heart failure due to the rupturing of the blood vessels. The same with the brain which can result in stokes.
The skin is another major body part that is affected by hms/eds. In particular it is often very stretchy and soft which makes it more fragile. This makes it prone to tearing very easily and very poor at healing. People with hms/eds often have alot of scars that look like they are from serious injuries when in fact they can be from something as simple as a scratch. The skin also bruises very easily, often from simple things like kneeling on the floor or getting a hug from someone.

Because the joints in hms/eds are very unstable, the muscles around the joints have to do all the work to keep them held together. This means that most of your energy gets used up by the muscles. This means that people with the condition suffer really badly from exhaustion and very poor energy levels. Something as simple as walking up the stairs can exhaust the person so much that they have to lie down for 30mins to recover. This means that alot of people with the condition are also diagnosed with CFS/ME. Having such poor energy levels makes it even harder to do basic tasks such as dressing, washing etc.

For some reason, people with hms/eds are much more prone to alot of other conditions. Some conditions occur for obvious reasons to do with the weakend collagen and energy issues, other conditions occur but don't seem to have an obvious reason why.
The related conditions that people with hms/eds tend to also have include:
Fibromyalgia, flat feet, raynauds syndrome, insommnia, curvature of the spine, kyphosis (a hump at the top of the spine), hernias, neuropathy, carpel tunnel syndrome, premature aging of the skin, strokes, heart murmurs, rheumatoid arthritis, automic dsyfunction (a dysfuntion of the body's ability maintain normal function of systems such as blood pressure) and much more.

Hms/Eds varies massivley in severity. Some people may have very few problems and lead normal lives, able to do the same as people without the condition. At the other end of the scale, some people are seriously affected by the condition resulting in severe disability. They may depend on a wheelchair, they may need help to dress, bathe, change position in bed etc. In some cases, a person with hms/eds may become bedridden for periods of time due to the severity of the condition. People who are effected badly may not be able to work and rely on the help of others to get through the day. And of course in the case of Vascular EDS, the condition can often be fatal.

Because hms/eds is caused by a defect in the collagen fibres, there is currently no cure. Treatment concentrates on keeping the joints as strong as possible to delay the onset of degeneration. Physiotherapy concentrates on building the muscle strength around the joints. Occupational therapy looks at aids that can help make tasks easier.

Pain is a major symptom so becomes the main focus for treatment. Pain in hms/eds is difficult to control becuase it can fluctuate easily. But in alot of cases, medications such as tramadol, codiene and even morphine are used to help manage the pain. It often does not take the pain away but only brings it down to a more mangeble level.
Sometimes surgery is required to stablise joints but this is done less and less now as drs are aware now that operating on a joint just makes the other joints more unstable. Steroid injections are used more and more now to stablies joints by reducing inflamation. Steroids are injected directly into the joint which is extremely painful and doesnt work for everyone.
Other treatment focuses on stablising the joints with splints and braces so a person with the condition may wear various splints on their wrists, elbows, knees and ankles. They may also wear splinted supports to stabalise the spine, hips and shoulders. And of course the related conditions need to be treated. So someone who suffers from the hms affecting their bladder may need to use catherters and incontinence products.

Becuase the joints of a person with hms/eds are so unstable and dislocate so easily, alot of the time they go back into the right position very easily so people with the condition often learn fix to dislocated joints themselves. Sometimes however they do need to go to hospital as a result of a bad dislocation. People who suffer from the effcts of severe hms/eds often spend alot of time in hospital reciving intensive physiotherapy and pain treatment. They often have many drs from different areas such as rheumatology, endochronology, gasteonterology, etc and can have a hosptial appointment every week sometimes more than once a week!

Hms/Eds is becoming more recognised as a seroius condition by drs. Many people with the condition spend years trying to get drs to take them seriously and to get a diagnosis. Even then treatment can be very difficult to get. This is still the case in alot of places and as a result many people with hms/eds have to travel alot to recieve treatment and care from specialists who have more knowledge of the condition. There are currently only around 4 hms/eds clinics in the UK!

One of the biggest problems people with hms/eds face is the lack of understanding from friends, family and collegues. A person with hms/eds may appear to be fine, especially if they can walk. Often a person with hms/eds may be able to do something one day but not the next such as pouring a kettle or walking up the stairs. So it becomes difficult for others to understand just how difficult the condition can be. Even those who use wheelchairs or crutches to walk can experince problems with people not understanding. This can make relationships and friendships difficult and often a person with hms/eds becomes isolated as friends slowly disapear. Often a person with hms/eds has alot of bad days where they are unable to get out of bed or to go out. This means that friends often only see them on a good day so they then think that this is how the person is all of the time. They often don't comprehend how bad the bad days are.

Hms/Eds is a very complex condition that can affect the person's life in many ways. The condition often affects social and work life and can even greatly affect quality of life. Becuase of the massive range of issues the condition causes, people with hms/eds often struggle with many things that others take for granted. The lack of understanding of the condition and due to how it can fluctuate on a daily basis leads to the person finding it difficult to maintain friendships and to recieve emotional support from others.

This blog is intended to allow people to understand the daily issues living with hms/eds causes. It also is intended to highlight the strength and determination people with the condition have to get on with their lives and the ongoing fight they have to recieve appropriate treatment, and more importantly, understanding.