Hypermobility Syndrome

This week Imogen has been diagnosed with hypermobility syndrome.

Joint hypermobility means some or all of a person's joints have an unusually large range of movement.

People with hypermobility are particularly supple and able to move their limbs into positions others find impossible.

Many people with hypermobile joints do not have any problems or need treatment. However, joint hypermobility can sometimes cause unpleasant symptoms, such as:

• joint pain
• back pain
• dislocated joints – when the joint comes out if its correct position 
• soft tissue injuries, such as tenosynovitis(inflammation of the protective sheath around a tendon)

If hypermobility causes these types of symptoms it is often called joint hypermobility syndrome. Read more about thesymptoms of joint hypermobility.

Living with joint hypermobility syndrome

Joint hypermobility syndrome can be very difficult to live with because it can cause fatigue (extreme tiredness) and long-term pain. It may also take time to receive the correct diagnosis due to the wide range of symptoms that joint hypermobility syndrome can cause.

However, once diagnosed, joint hypermobility syndrome can be treated using a combination of exercise andphysiotherapy (where physical methods are used to promote healing). An exercise programme to improve fitness and muscle strength may also be effective at reducing pain. Read more about treating joint hypermobility.

The nature of joint hypermobility syndrome means that you are at increased risk of injuries, such as dislocations and soft tissue injuries. Managing joint hypermobility syndrome may therefore involve treating short-term injuries as they arise, while following a long-term treatment plan to manage daily symptoms. 

Causes of joint hypermobility

Joint hypermobility is often hereditary (runs in families). One of the main causes of joint hypermobility is thought to be genetically-determined changes to a type of protein called collagen.

Collagen is found throughout the body – for example, in skin and ligaments (the tough bands that link two bones together at a joint).

If collagen is weaker than it should be, tissues in the body will be fragile. This can make ligaments and joints particularly loose and stretchy. As a result, the joints can extend further than usual.

Most cases of joint hypermobility are thought to be linked toEhlers-Danlos syndrome hypermobility type, which is a group of inherited conditions that affect collagen proteins in the body.

Occasionally, joint hypermobility may be part of a rare and more serious condition such as:

• osteogenesis imperfecta – a condition that affects the bones
• Marfan syndrome – a condition that affects the blood vessels, eyes and skeleton
• Ehlers-Danlos syndrome vascular type – a condition that can cause the arteries, bowel or womb (in pregnant women) to rupture (split)

Read more about the causes of joint hypermobility.

How common is joint hypermobility?

It is not clear how many people in the UK have joint hypermobility. There are estimates that up to 3 in 10 people may be affected to some degree. It affects women more than men, possibly because female hormones increase flexibility.

Joint hypermobility is common in children. Children with joint hypermobility can bend into unusual positions (often referred to as ‘double-jointed’).

In many children, the joints become stiffer by the time they reach adulthood, although in some people, joint hypermobility and its associated symptoms continue into adult life.

Marfan syndrome affects around 1 in 5,000 people.

Update

Well the Enbrel and MTX mix seems to be working for Imogen at the moment. There is very little active arthritis in her joints though her right ankle is still being difficult.

She is getting on better with the injections now as well. We do Enbrel on a Saturday morning and MTX on a Sunday morning. We did try to do them both on the same day, but it was too stressful for everyone that way!

Imogen in herself is much happier, although she still tires very easily and complains of pain fairly regularly. We always have naproxen on standby just in case.

She amazes me with the way she copes with this disease, she doesn't let anything stop her, and she very rarely has days where she doesn't move from the sofa any more!

So all in all things are going well. We just need to get this ankle to behave and then we should have things under control!

1 Year On

It's a year today since Imogen was diagnosed.

This past year has flown by in a blur of hospital appointments, blood tests and numerous injections at home. I can't quite believe we're a year down the line!

I still remember how I felt a year ago, having no idea of what lay ahead of us, wondering where we went from diagnosis.

So Imogen still isn't in remission, although she is much better than last year!

We have done methotrexate since April, we've done oral and iv steroids, eye tests, physio appointments, xrays and orthotics.

This year we have started Enbrel and we are hoping that this will be our wonder drug.

Hopefully this time next year we will be able to say that Imogen is totally in remission!

On the Radio at Breakfast :)

http://www.964eagle.co.uk/news/local-news/1180258/video-a-three-year-olds-arthritis-journey/

Our Radio Bit

Ooh a quiet few minutes!

Well, Imogen is going to be starting Enbrel in the near future. Hopefully in the next week or so! This drug can take 4-6 weeks to work effectively, but she will be taking it alongside MTX for the moment.

Physio would like to cast her foot again. She was in casts for a week previously, but she then went into flare again so they weren't as effective as hoped. It will only be one leg this time, but for two weeks rather than one!

We did some filming with local radio station 96.4 The Eagle on Monday. That will be going out on their breakfast show tomorrow morning which is quite exciting!!!

We have eye tests at the end of the month. Imogen is thankfully getting better as time goes on with these. Hopefully again we'll get a clear result and then not have another for three months.

After that appointment, I actually have nothing in the diary for the whole of Feb!!! Which is amazing, although it will change and we'll have at least one.

On the 6th Feb, it will be a year to the day that Imogen was diagnosed! Where has that gone? It seems mad. I remember that day as though it was yesterday! We've been through so much this last year, and become a stronger family unit for it. I never thought I'd be doing injections weekly and it seeming the most normal thing! Hospitals seem like our second home, and we know most of the nurses at our local hospital by name!


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Raising Awareness

So, last year:

• I was a Social Media Champion for Arthritis Research UK
• Imogen and I appeared on Daybreak during National Arthritis Week
• I did a radio interview for BBC Sussex and Surrey for National Arthritis Week too

This year I want to do even more!

So far I have done some filming for local radio station 96.4 Eagle. As soon as it's available I will post a link.

I have also been approached by NRAS to become part of their Parent Panel when they revamp in the summer.

Exciting things are afoot this year already!!!

The Reason for the Forum

• 
  
• My daughter Imogen was diagnosed with Polyarticular Juvenile Idiopathic Arthritis in Feb this year. She wasn't even three years old.

  I remember feeling so alone after diagnosis. I didn't know anyone else who had a child with this disease and we were literally just given a diagnosis and shoved out of the door by the local hospital. One of the first things I did when we were settled at home was Google, to see if there was anything out there for parents with children who have arthritis and other than places who gave lots of information, there wasn't much where I could discuss things with other people going through the same things.

  I have talked to a few arthritis parents since and everyone has said the same. There are no real places to go to talk to others in the same situation.

  So the idea of Our Arthritis Journey was born. I believe there is a real need for somewhere to share our experiences, get advice and support if we need it, and also somewhere for the younger sufferers to have somewhere to chat and talk to others like themselves.

  I have been on many forums throughout my life dealing with many different things. I decided that having a forum would be a good place to start. That way people could join, see that they weren't alone and that there were many other people going through the same thing.

  The forum opened yesterday (16.12.13) and we have 5 members so far. Eventually I would love this to expand and be a great community for people to come and discuss anything, from the weather where they are, to the next step in treatments for their child. Also it doesn't just have to be for parents, it can be for grandparents, aunts, uncles, siblings or anyone else affected by the bombshell that is JIA. Because the one thing we all know is that JIA does not just affect the child, it has a knock on effect for the whole family.

  http://ourarthritisjourney.proboards.com/ http://imogensarthritisjour.wix.com/ourarthritisjourney

I'm Sorry

I've neglected the blog :(

Imogen had over 20 joint injections on Monday 9th at Great Ormond Street Hospital. That was her third lot.

We are waiting for an enbrel referral so hopefully that will happen soon and we will get an idea of when that will be started. They have also upped her MTX to 12mg. Sadly Enbrel is another injection, so not much fun for poor moos!!

However she is still smiling, had a speaking part in her xmas play (extremely proud parents moment) and all in all she is doing ok.

I'm still spending time doing  a lot of calling around to hospitals to chase things up which is annoying!
Edited to add: We have a website and a forum now too
http://imogensarthritisjour.wix.com/ourarthritisjourney
http://ourarthritisjourney.proboards.com/
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1 Hour 30 Minutes

Doesn't sound like very long does it?

90 minutes, 5400 seconds.

It's the longest time ever when your child is under general anaesthetic. It's almost like the clock goes backwards.

Imogen had 7 joint injections done today, on top of having an infection (which we didn't realise until her bloods came back afterwards)

She is covered in plasters and feeling very sorry for herself.

I hate arthritis. That is all

Our Story

On Sunday 3rd Feb, Imogen had gone to my mums for the day. Some time in the afternoon, my mum rang, and asked if Imogen had hurt her ankle at all as she had been limping and it was quite swollen. We hadn't noticed if she'd fallen over or anything to cause it. 

Once mum had dropped her off, we had a look and the ankle was huge! It was also warm to touch. So off we went to our local A&E Dept, where they told me it was probably just a sprain and to come back in 48 hours if the swelling had not gone down.

Cue 48 hours later, by which time Imogen had a swollen wrist as well. We went back up to A&E to see if they could do XRays to see what was going on. Xrays showed no breaks, so we sent back to the A&E dept to wait for the Doctor. We were then told that they thought it was possibly Arthritis and that she'd be transferred to the Children's Ward so see Doctors up there.

A Doctor came in to see us and looked at the wrist and the ankle, she also noticed that since we'd been at the hospital, Imogen's knee was starting to swell. They wanted to keep her in overnight for observation and high doses of Ibuprofen to try and reduce the swelling.

The next day we saw the Consultant who took bloods for numerous tests to try and diagnose Imogen. Once these bloods came back we had a definite diagnosis of JIA (Juvenile Idiopathic Arthritis) They thought to begin with that it was Oligoarticular but as more joints became affected it was switched to Polyarticular.

So that's the beginning of our story!

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How Things Have Changed!

Well since I last posted, things have changed a lot. Imogen is happier and much more comfortable! She still has bad days, but there are less of them at the moment.

She still has active arthritis in her wrist, knee and ankle - however it has improved greatly from where we were.

We go back up to GOSH on the 28th to see the Consultants there, and I think they will probably up Imogen's MTX slightly to see if that can get rid of the active arthritis.

She had a joint injection under general anaesthetic last week! Her wrist, the worst joint, was swollen and sore, so they decided to give a steroid injection under GA to help. It was horrific for us, however she was in and out of theatre within about 20 minutes. We spent less than four hours in the hospital!

So all in all things are certainly better! All I can say, is it's about time! x

Wow!!

Well, after the blood tests, canula insertions, stupid amounts of steroids being pumped into my baby girl's body, there has been a noticable difference.

When they do Imogen's blood tests, they test the ESR rate:

"The erythrocyte sedimentation rate (ESR), also called a sedimentation rate or Westergren ESR, is the rate at which red blood cells sediment in a period of one hour. It is a common hematology test, and is a non-specific measure of inflammation. To perform the test, anticoagulated blood is placed in an upright tube, known as a Westergren tube, and the rate at which the red blood cells fall is measured and reported in mm/h.

Since the introduction of automated analyzers into the clinical laboratory, the ESR test has been automatically performed.

The ESR is governed by the balance between pro-sedimentation factors, mainly fibrinogen, and those factors resisting sedimentation, namely the negative charge of the erythrocytes (zeta potential). When an inflammatory process is present, the high proportion of fibrinogen in the blood causes red blood cells to stick to each other. The red cells form stacks called 'rouleaux,' which settle faster. Rouleaux formation can also occur in association with some lymphoproliferative disorders in which one or more immunoglobulins are secreted in high amounts. Rouleaux formation can, however, be a normal physiological finding in horses, cats, and pigs.

The ESR is increased by any cause or focus of inflammation. The ESR is increased in pregnancy, inflammation, anemia or rheumatoid arthritis, and decreased in polycythemia, sickle cell anemia, hereditary spherocytosis, and congestive heart failure. The basal ESR is slightly higher in females."

Now last time this was tested on the 2nd April, Imogen's ESR was 97! The normal range for her age is between 3-13. She was tested again on the 30th April and her ESR is now...11.

Well within the normal range! So obviously we are on the right track and things are getting better. Her White Blood Cells are still a little high but with time hopefully they will come down a bit more.

Happy day :)

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Buzzy

Hey all :)

Ordered Imogen's Buzzy this morning :) We've gone for the LadyBuzz with the Bee-Stractors to hopefully make things a little easier.

Hydrotherapy did not go as well as anticipated. I think Imogen is afraid of water. I'm not sure why, but there is definitely something there. The Hydro room was boiling!!! I could have quite happily stripped and got in the pool too!! Riley ended up with just his jeans on in there as it was just overpowering!
We're hoping as she is going to be having weekly 30 min sessions that we can overcome this fear and we are going to take her swimming every weekend as a family to hopefully conquer it.

MTX day tomorrow. Community Nurse is coming out at 2pm to do the injection and fill in all the relevant paperwork. I'm so sick of paperwork! I seem to spend my life filling in forms for something or the other!!

Trying to contact the Consultant's Secretary today as well, as when Imogen gets her next blood tests I would like them to check her immunity to measles. She had the original jab at 13 months, but was due a booster in a few weeks. But due to MTX she cannot have any live vaccines. I just want to check that she's at least a little bit covered!

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Hydrotherapy Day

Imogen has Hydro this afternoon. She's looking forward to it which is nice. It'll be nice that she'll have a treatment that she enjoys rather than all these invasive treatments that hurt her.

We finally got our DLA decision this week too... We got awarded!! I am amazed, as everyone I have spoken to, told us that it was doubtful to be awarded first time. She will now be getting High Rate Mobility and Middle Rate Care. The money will be used for hospital trips and things that Imogen needs. We are also going to buy the Buzzy out of it when it arrives in my bank.

Joints don't seem to be swelling so far, but I think we are just waiting for them to come back up. Hopefully they won't but with 5-7 weeks before the MTX begins to work efficiently, I have a horrible feeling that another pulse of IV steroids may well be on the cards.

But PMA and hopefully Imogen is now going to feel better as we get her condition under control.

More later after Hydro

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So far, So Good

Imogen had her Methotrexate injection (hereby known as MTX as fed up of having to type it lol) on Friday morning. So far so good, no adverse side effects which is good. It's a low dose of chemotherapy to try and knock out her immune system so it stops attacking itself. Weekly injections for at least TWO years, which is not so good, but as always we will manage.

We are looking at getting Imogen a Buzzy http://www.buzzy4shots.co.uk/index.html
They are supposed to help with pain from injections, blood tests etc. To my readers, have you used one? Did it work for you?

I will write up a review once we have got one and tried it out.

But Imogen has been in fine form these last few days. She spent time today running around with her older brother and her cousin :) It is lovely to see, and she's so happy and cheerful at the moment.

The steroids have started to fill her face out though, she is beginning to look a little chubby bless her :)

Have got to ring the Dr's tomorrow as Imogen is extremely constipated due to the amount of medication she's taking :( She's just woken up due to the fact that she cannot poo!

But in the grand scheme of things... to be honest it could be much worse!!!

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Ahhh Bliss!

Imogen has gone to nursery!!! 3 hours of blissful me and Riley time! With Easter Holidays Imogen has been off nursery for 3 weeks. It is lovely to have the time just me and the boy :)

Imogen had all three doses of IV steroids this week. It finished yesterday. She goes for her Methotrexate injection tomorrow at 10 am, then it's just waiting for it to begin working. I need to get onto the Community Nurses and find out if it has all been arranged for them to come out and give Imogen her injection at home. Although it's going to be tricky working it as she's at nursery 8.45-12.30 then comes home for an hour and then hopefully will be off with her brother to another nursery at 2pm so I can go to work.

Yes I am now a working mum!! I had a job interview on Monday and they offered me the job. Hours are slightly akward, but am trying to get the kids into a nursery just down the road from me for 2 afternoons a week so that I can start work at 3.30 and daddy can pick them up when he finishes.

Seems to be a pretty positive week this week. Hopefully it continues :)

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What a Week...

It's been a very long week this week.

We saw the Paediatric Registrar on Tuesday, who agreed that Imogen had improved enough to begin the Methotrexate. However nobody on the ward was qualified to do it this week as it needs to be administered by a Chemotherapy trained nurse. It is now set for next Friday at 10 am. It is still going to take another 6-8 weeks to begin working.

To continue her treatment Imogen was put on oral steroids to help deal with the swelling and painful joints. However, sadly this has not been the case. As of this evening, Imogen is back in full flare of all 8 joints.

We go back up for another round of IV steroids on Monday, Tuesday and Wednesday, she has physio on Thursday and then Methotrexate Friday. Oh joy another all weeker :(

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Yay!!

Imogen finished her last dose of IV steroids yesterday! She also had her eye test which shows no signs of Uveitis!!

She is now on 4 days of oral steroids which finish on Tuesday. We go back up to the hospital Tuesday morning to see the Paediatric Registrar, who will give us the next lot of steroids and hopefully a date for her first Methotrexate injection. She is going to be on the steroids for two months as the Methotrexate will take about that time to work.

Injections once a week are going to be tough, but I'm debating learning to do them myself as I think it will be less stressful for Imogen if it can just be done at home by me. As much as I hate the idea, I'd rather make the whole thing as stress free for Imogen as possible.

Anyway, we have noticed a definite improvement in Imogen herself after being on the IV. The swelling has definitely gone down, it's not completely gone, but it's so much better than it was. She is moving so much better as well. Although we are certainly getting some mood swings, she is a lot happier and we're not having so much crying and just general upset.

It's almost like I have my little girl back :)

I am so proud of the way she is just getting on with it all. She's dealing so much better than I would!!


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Eye Test...

Well it's the dreaded eye test tomorrow. Not looking forward to it at all :(

Second day of steroids today and it's knocked Imogen for six. My poor baby girl looks so drained. Big dark circles under her eyes, pale, grumpy and just not her usual self. I hate seeing her like this, but I know it's the best thing for her. The saying "you've gotta be cruel to be kind" is keeping me sane.

We have noticed some improvement already though which makes me feel a little better. Swelling has gone down a bit and also the joints are not so hot to touch. She was moving a lot easier today too.

Last dose tomorrow after her eye test, then she has four days of oral prednisone. We see the Dr next Tuesday and if they are happy with the improvements, we should get an idea of when we start Methotrexate.

It's been an emotionally draining week for us all. We have been in one hospital or another every day this week. I can't wait to get tomorrow out of the way and then have the weekend off. We definitely need it!

Day One

Of IV steroids. Not too bad at all really. Bit of crying when the canula was put in, then a bit more once she got hooked up to the IV, but that was more because she couldn't go anywhere. You try telling a 3 year old that they can't go far, it's impossible. However the fabulous Play Specialist was there saving the day. He bought Imogen a TV in so that she could watch Toy Story whilst having her infusion! Cue happy child sat on Daddy's knee for the whole 30+ minutes of her infusion. After infusion was given we were allowed to go home. They have kept the canula in to save having to put it in daily.

We go back at 11am tomorrow for round two!!