A Day Out with a Zebra

Last week we were invited out to test some bouncy castles and afterwards spend the day at Hatton Adventure World.

To most people we were just a regular family enjoying a day out, which is I guess, how most zebra families are viewed. I wanted to write something to show just what that day really was like for us.

We started out almost on time which is good because it meant we had no 'accidents' or 'incidents' to delay our morning. By this I mean that Lucy did not soil herself or have a meltdown, sometimes we experience at least one of these in the morning.

I have to consider what may happen during the day out, so much like preparing for a baby, I prepare a bag for Lucy. I have spare clothes in case of accidents, baby wipes, extra drinks and painkillers. 

When we arrived Lucy was a little fatigued from the journey but she soon perked up and was rushing along with the other children across the field to the bouncy castles. She was playing and having fun straight away, being the eldest she had no inhibitions and no shyness. At one point she was asked to help a younger child climb up the bouncy castle to the slide. She didn't register this question. I asked her sister to help instead. This is her autism kicking in. It's not that she doesn't understand, it's just that she was playing and not registering that her help was needed. To get her to help I would have had to remove her from play, make sure I had her attention and then explained what help was needed. This is time consuming, it may frustrate her being stopped from playing and as she is not that physically fit helping another child might not work anyway. It was just easier to ask her sister.

At first Lucy was able to climb up to the slide herself, most of the other children where managing fine and it wasn't that high. As she became tired her body no longer had the strength to get her up and she began struggling. Then she became frustrated with herself. I had to take her off the bouncy castle to rest and recuperate. We did this by sitting on some chairs that had been provided. She leant against me and at one point was laying on my lap. She felt quite calm which was good because I knew she was trying to curb her anger and frustration at not being like the other children. She tried again but as it was hot she didn't really get enough energy back. Her frustration increased and she nearly went into meltdown but a cuddle from me managed to calm her and again I had to remove her from the bouncy castle. I'm glad she didn't go into full meltdown. She was the eldest and biggest child there and it may have been a little scary for the other children to see her like that. Luckily we were in a place where I (or her Dad) were able to take her off, away from everyone else. That's something we have to think about and why we tend to avoid enclosed places like soft play areas.

When the bouncy castle play was over, which came at just the right time for Lucy but the other children probably would have played for longer, we went for some food. Lucy perked up again and was able to walk over to the marquee where the buffet was laid out. She complained that she was hot and thirsty but the thought of sustenance kept her going.

Once in the marquee she was happily tucking into the food, thankfully there was plenty of things that she liked. She is quite a fussy eater but is quite happy with buffet type food and finger foods. After re-fuelling she joined the other children in colouring in activities.

Then we went to enjoy the rest of Hatton. We started with the animals, Lucy loves animals so she's always happy here. Then we took them to play on the mini tractors. She is a little big for this type of play but she is also a little immature so she really wanted to join in. Luckily, some of the tractors where quite big and she managed to find one that she actually could fit on.  The three of them played together for a while while Dad and I took a rest on a bench. We then went to play in the sand with the diggers. They all loved this so much and could have stayed here all day.

We went panning for gold and played in the park, taking plenty of rests in between activities. I was surprised at Lucy's stamina, she must have been enjoying herself to have lasted this long. She was showing signs of waning though even though the other two were still going strong. They wanted to go and play on the fairground. I had two reservations about this, one was that I thought Lucy had had enough for one day and two, fairground rides can be a strain on her neck. There are only a few rides at Hatton and they are aimed at younger children, but they are still bumpy and the tea cups spin around. I know I can't wrap Lucy in cotton wool, but I do try to limit her activity for her own safety. She was too tired to go to the rides anyway, she didn't want to. So I took the other two while Lucy had a stroll around the bird cages with her Dad. Everyone was happy.

It was late afternoon now and we were all feeling tired so we decided to make our way home. First we had to make our way through the lovely shopping area. Usually we would take the time to browse but we decided on a quick pop into just a couple of the shops.

There was some grumbling on the way home and we had to stop for cold drinks but overall I believe we had a successful day out.

Painkillers were administered at bedtime and it didn't take long for her to fall asleep. Some nights it can take up to two hours, so she must have been worn out. I had to go in and massage her knees around midnight but other than that it was a peaceful night and she didn't wake until just after 7am.

Rheumatologist and Physio Therapist

We were referred to several departments after Lucy's diagnosis of Elhers Danlos Syndrome to see what she needs and what help she can be given.

The first appointment through was for the physio therapist which was for  Friday. Then on Tuesday we received a phone call asking us if we could make an appointment with the rheumatologist on Thursday.

I'm glad the appointments worked out the way they did because visiting the rheumatologist first was a the best step.

The Doctor we saw was really nice. She spent about 15 minutes talking through Lucy's history asking me lots of questions. I'd written everything down but I didn't need to consult my paper because it's all etched in my memory. There is always a chance of the brain fog descending though, so best to have some guidelines. Then she asked if I had questions and mostly I just wanted to know if she agreed with the paediatricians diagnosis and if there was anything that could be done for Lucy.

She completed a  thorough examination of Lucy before answering my questions. Lucy scores 6/9 on the beighton scale for hypermobility. However, she does have other joints that are really hypermobile that the scale doesn't take into consideration. (As a point of interest, I score 7/9) We both score two major on the brighton criteria.

She pointed out that Lucy had a mis-aligned ankle on her left side and asked me if she'd had an accident. We are not aware of her hurting her ankle. She thinks it can be corrected with shoe inserts and has recommended that Lucy sees a podiatrist.

At the end of the diagnosis she went through her findings with the information given by the Elhers Danlos Syndrome Organisation and agreed that Lucy does have EDS hypermobility type.

She recommended Lucy seeing a physio therapist but I told her that Lucy already had an appointment for the next day. She is also referring Lucy to a gastro specialist for her bowel problems.

Overall the visit felt positive. It felt like a step in the right direction and that Lucy was going to receive some help.

The next day we saw the physio therapist and the experience was a little different.

Lucy was not feeling her best and complained every time she was touched. Maybe all the stretching and poking the day before had left her feeling sore? The physio was doing practically the same things but I have never heard Lucy say ouch so often. She never normally complains, even when she was at her worst with her neck she would only admit to pain if we asked her.

I had told the physio that Lucy had been given a diagnosis of EDS but she didn't seem impressed. She said a lot of Lucy's pain could be put down to growing pains and had she grown much in the last few months....she's 9 years old, she's always growing! She's been growing since she was a baby so I guess all the pain she has had over the past 9 years are growing pains then?

She saw that I wasn't very happy with her comment, event though I didn't say anything, so she tried to make it sound as though she was only blaming some of the pain on growing..the pain in her ankles and knees. The rest was EDS, if that's what she had been diagnosed with. I had the feeling she did not believe that EDS existed.

She then went on to say that Lucy would need help to recover from her ankle injury...I hadn't said anything about her ankle...there has been no injury, surely I would know? I didn't argue with her, if Lucy needs help for her ankle she can have it. It's just a mystery as to why she has a bad ankle.

Finally she said that Lucy would be referred to a rheumatology physio therapist. Then she left the room to fetch some exercises for Lucy. When she came back she said she'd made the referral to a community physio therapist who would come to see Lucy at home.

I must say I found the whole experience with the physio therapist like a step backwards. I'm so glad we didn't see her first I think I may have been more upset.

In the meantime we are taking one day at a time.

School and Hospital

A few weeks ago I went into the school to speak to the Special Needs Teacher about Lucy. I told her all about her condition, what it was and what it meant for Lucy. The teacher had not heard of Elhers Danlos Syndrome so she was happy that I took a long a print out about the condition.
I gave her a list of what I wanted for Lucy in school which wasn't too difficult. The main thing being I've stopped her from doing p.e. It's not that she's not capable of physical activity, it's just that I don't trust the school to make sure she's not doing something that may harm her. Also, the other kids in the school are pretty rowdy and I don't want her getting hurt by them.

I also told the teacher that she may get tired easily and finds it difficult to walk long distances. Also, that she may find sitting on the floor for long periods painful.

I'm allowing her to go outside at playtime so long as she's taken seriously if she gets hurt or falls. What is a simple bump to an ordinary child might be quite serious for her.

We will see how it all goes.

This week we have two hospital appointments. Finally we have an appointment with the rheumatologist so I'm busy writing down all the information that he will need and the questions I want to ask. You need to be prepared.

The second appointment is with the physio therapy department who will assess whether Lucy needs any physio and if it will be beneficial for her.

I'll let you know how we get on.

I've also asked for her to be referred back to the psychologist about her sleep issues as she is taking longer and longer to fall asleep at night. She's always woken a  lot during the night, but generally she's fallen asleep quite quickly which balances it out a little. Now, she's really not getting enough sleep at all.

The Diagnosis

Well, almost ten years after she was born, and seven years since we first went to the hospital with her painful knees, Lucy finally has an official diagnosis. Elhers Danlos Syndrome.

It's a little ironic that it should arrive in May, EDS awareness month.

She will still be seeing the rhuematologist at the hospital and possibly have physio therapy, but she's had that before and it didn't help.

As it is Elhers Danlos Syndrome Awareness Month I will tell you a little about Lucy's condition. In fact there are seven types of EDS, Classical, Vascular, Hypermobility, Kyphoscoliotic, Tenacin-X deficient, Arthrochalasia and Dermatosparaxis.
Lucy's type is is Hypermobility.

• She has hypermobile joints
• she has suffered dislocations/subluxations
• she has reflux
• she has gastro symptoms
• she bruises easily
• she has joint pain
• she takes longer to heal

There are also implications for the future with autonomic problems such as POTS.

As always getting a diagnosis is a bitter sweet experience. It's good that you have an answer, a reason for your child's problems. It's also good that the medical professionals are recognising the problems and will do what they can to help. It's never good finding out that your child is ill, particularly with something incurable.

I also have some good news. I posted back in February about Lucy's alopecia. Well, it seems to be stopping, her hair loss is very little now and the growth is doing well. This is a photo of brand new hair grown from her being completely shaven last June.

Good and Bad News

We had Lucy's hospital appointment on Monday. She was a bit disorientated going to different part of the hospital, when we got there she assumed we'd be heading straight to neurology. We waited in Outpatients for just over an hour and if I'm honest, Lucy handled the wait better than her dad who got bit grumpy!

She was weighed and measured first and for a nine year old she's pretty big at 4ft 8" and 5 1/2 stone. (1.43m and 35kg) She's already up to my shoulder!

The Doctor was nice, he was willing to listen and I'd taken two pages of things I'd written down for him to listen to. As you may remember (if you've ever read this blog before) our reason for going was to find out possible reasons for Lucy's fecal incontinence and maybe a solution. However, Dad seemed to have  different agenda and wanted to know if our suspicions of Lucy having Elhers Danlos Syndrome were right.

For the incontinence we had no answers. The Doctor said it was very unusual unless she was suffering from constipation. However, it was possible that loose ligaments meant that she could have little control over her bowels so it could be a possible effect of EDS. He couldn't help us but hopefully a specialist in EDS could.

So basically he was agreeing that Lucy did look likely to have EDS. He took some family history and checked over her joints, and her tummy.

She is now being referred to a geneticist for a proper diagnosis, a rheumatologist to help with pain management and a physio therapist. The clinic that she has been referred back to for physio is one she has been to before and it's the one where her psychologist works, so I'm thinking of booking her in for another chat with her as she is still on the system there.

So, it's all about moving forward. We have no miracle solution to the incontinence but maybe someone further down the line will be able to help. A proper diagnosis will help Lucy in the future, especially if she gets worse or other symptoms appear.

As for the pain, well that seems to be getting worse. When we went to the hospital we decided to visit Forbidden Planet afterwards, it's about 5 minutes walk from the hospital. When we got there we found the shop had moved and had a further 5 minute walk. Lucy barely made it and was complaining all the way. This was a shop she wanted to visit so she wasn't been dragged somewhere she didn't want to go, like when we had to go food shopping the other week with her and she couldn't make it around the supermarket. She did manage 30 minutes of play in the park one sunny afternoon last week though.

Night times are the worst and she is waking up most nights again now. It's usually her knees that hurt most and I have to give her painkillers and massage them. Sometimes her neck hurts but thankfully not too often, and occasionally it's her ankles and lately she's been complaining of her wrists.

We are lucky if we get five hours a night sleep now.

Looking for Answers

Today Lucy did a poo on the toilet!
Ewww I hear you say, Too Much Information!
Well, maybe, but this is such a rare occasion that I thought maybe it was time I talked about it.
Lucy is 9 years old and incontinent with faeces as she has always been.

When she was around two and half I decided that maybe it was time to toilet train her. It didn't go well. She hated it and refused to sit on the toilet or the potty. So I left it for a while.
She started nursery at three and they wanted their children to be toilet trained. Lucy was suspended when they caught her in pull-up pants. So we tried really hard to train her, it was awful but we managed to get her to wee on the toilet. Poops were a different matter but we thought they would come later.

At six years old Lucy was being diagnosed for Autism and Hypermobility Syndrome. We talked to the psychologist about her toileting problems and she told us to go back to the beginning and try training her again. We hadn't stopped.

Over the years we'd tried.....

• potties of all different shapes and sizes, even ones that made music when you pee'd.
• wall charts with stickers,
• rewards for getting it right (She didn't get any)
• restrictions on favourite activities for not getting it right (We gave up on this because it was too cruel)
• timed regular visits 
• we read toilet training related story books
• activities during visits (books, handheld games)
• we even painted the smallest room pink and added girly pictures and curtains and a lovely Disney Princess toilet seat.

Basically, if there was trick, we tried it. 

Nothing worked.

When we spoke to her GP about it we were given meds for constipation. If she was pooing in her pants then it must be overflow from constipation. Nope, it was just poo.

Today's poo wasn't really a success, she hadn't gone for one, she'd gone for a wee and it had just come out, This has happened a few times (probably too few to mention!)

She says she can't feel anything, she has no idea that she needs to go and can't feel it coming out. The first she knows about it is once it's out. Sometimes she's quite quick to tell us and we get away with only a little in the pants if we get to the toilet quickly enough. Most the time it's a disaster. It's distressing all around. Her only saving grace is that she generally goes either morning or night so it hasn't happened at school very often. We've tried just making her sit and wait for it but without her having any idea of when it's coming we haven't had much success. She doesn't like being told to go to the toilet all the time when she doesn't feel any need to go.

So now we are waiting to see what the hospital has to say about it. Just a couple more weeks until her appointment. Will they take us seriously? Is there anything they can do? I don't know, but I really hope so because the current situation is really no fun. 

Alopecia, Telogen Effluvium.

Since Lucy had her operation last year she has had a lot of hair loss, alopecia, specifically Telogen Effluvium. This is where there is a widespread thinning of the hair all over the scalp, but not necessarily causing bald patches. I can be caused by lots of things but in Lucy's case it has probably been caused by the stress of her operation. It cannot be treated and will eventually stop.

When Lucy had the halo fitted before her operation I put all her hair up in a pony tail. This left her forehead free for the surgeon to screw in the halo at the front. The screws at the back were put through her hair. Then when she had her operation the ponytail was still there but her head was shaved at the back and on the right side. When we were finally able to release her ponytail most of her hair just fell away because it had been shaved from the root. She wasn't really left with much hair, but she still had her fringe and enough to tie up in a ponytail. With the halo on it didn't really matter.

It wasn't really until after the halo was removed that I noticed she was still losing hair. At first I just thought it was loose strands coming free from around the halo, but then I realised it was what was actually left of her hair that was falling out. Every day her clothes would be covered in hair, every morning her pillow covered in hair. I did worry if there would be anything left at all.

The shaved areas started to grow again, first the side which grew really quickly, then the back which has been slow but is growing steadily. Her original hair which wasn't shaved is still falling out, but the new hair is growing well. Maybe it will all even out in the end.

This weekend I was able to sweep all her hair into a ponytail, the freshly growing hair is finally long enough.  Her hair is not growing where she has scars but I'm hoping that eventually it will be thick enough for it to be unnoticeable.

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This photo is a weird angle but it is actually the top right hand side of her head where she had the bone graft taken, her ear is just below the bar on the right of the picture. It gives some idea of how high up she was shaved.