Perthes Disease

Written: Thursday, lunch time, 26th April 2001, 14:24

This is the story of why I can’t sit in the floor with my legs crossed. Why I was in a cast from my waist to my ankles for 2 months when I was 11, and why I have to have a hip replacement in the future.

This is the first time in the 10 years since my hip problem started that I am actually making notes or documentation about it in any form. So bear with me if I am uncertain of dates and details :)

I will also add scans of my X-rays, and photos of me in my cast at a later date. The pictures and Xrays will give you a much better idea of what it was like, but for now I ‘ just going to tell you about it.

First of all you are probably thinking “What is Perthes Disease?”

Well let me give you a brief introduction to what this disease is, and what it does.

PLEASE NOTE: A lot of this information is just from what I remember being told about it (which was when I was around 10 or 11) I have done some extra research to make these details more accurate. But if anyone in the know reads something that they think is inaccurate please let me know.

Introduction

What is it?

Perthes disease (also known as Legg-Calve-Perthes disease (LCPD) ) is basically the loss of blood circulation leading to the head of the femur (the ball that sits in your hip socket). You have blood flowing through the centre of your bones, and the blood flow through to your hip is supposed to be as thick as your average index finger, but in Perthes disease the blood flow becomes as thin as a hair! This loss of circulation leads to the avascular necrosis (deterioration) of the femur head. So because it isn’t getting any oxygen or the other good stuff in blood, the bone cells start to die. Then when the doctors get to fixing it (which isn’t a whole lotta fun) they need to get the blood flowing to the femur head again. During this healing time the head is all soft and stuff, and vunerable to fracture under pressure, causing it to collapse (in simpler terms the ball part gets all distorted and mis-shaped). Over time once they have the blood flow back, the femur head grows again and remodels in the collapsed position. This causes stiffness and pain in future.

The usual start of Perthes:

- Hip or groin pain, which may be referred to the thigh
- Mild or intermittent pain in anterior thigh or knee
(My pain started in the knee and spread to my hip ever a period of a few months)
- Limp
(My limp has faded almost totally over time, I only limp now if I over do it and my hip gets tired, but then I am probably the only one to notice)
- Usually no history of trauma
(meaning you haven’t done anything to your hip to spark this all off like break your leg or anything)

What Physicalls happens next:

- Decreased range of motion (ROM), particularly with internal rotation and abduction
- Painful gait
- Atrophy of thigh muscles secondary to disuse
(Because your affected hip gets tired easily you tend not to use it as much as your good leg without even realising you are doing that)
- Muscle spasm
- Leg length inequality due to collapse
(yup, my left leg is shorter than my right one by a centimetre or two.)
- Thigh atrophy
- Thigh circumference on the involved side will be smaller secondary to disuse.
(Yeah, my left thigh (on the side of my bad hip) is thinner than my right hip, but you can hardly even notice it, infact I am probably the only person who can tell)
- Short stature
(So atleast I have an excuse for being a short arse, hehehe :) )
- Children with LCPD often have delayed bone age.

What causes it?

Good question, I don’t know and neither do the doctors. After you fracture your femur (like you trip and break your leg or something) sometimes the loss of blood flow if restricted and deterioration of the femur head occurs similar to that in the case of Perthes. But with perthes there is no history of trauma (meaning you normally have never done anyting to bugger up your hip in the first place).

These are the stats that drive me mad though:

In the US it occurs in 1 in 1200 kids, Mainly in boys between 4-8 but also occurs up to the age of 13, mainly white Caucasian children, and the age you get it has a great deal to do with the prognosis, after 8 years represents a poor prognosis. :(

And I was a girl of 11 years old when I got it!

My Story

So how did this all start?

Well I remember the exact moment that it all started! I was in standard 3 (10 years old) and it was a normal school day and we were all leading into assembly, we all had to sit on the floor, and as a I went to cross my legs, I couldn’t! Basically my left knee wouldn’t go down, It wasn’t sore or anything, but it just didn’t want to go down to the left. It was just to strange!! I had to ask the teacher if I could sit on a chair against the wall of the hall, and I couldn’t explain why but I just couldn’t cross my one leg! Try explain that one when you are 10! Especially when you don’t understand it yourself! And I have never crossed my legs since. :(

So what happened next?

Well after that day my knee (yes my knee) would ache sporadically (imtermittently, like every now and again). It wasn’t bad pain just really uncomfortable. My mum thought it was just growing pains, normal in a kid of my age. But after a while (I can’t really remember how long, maybe a few weeks or a month or so) we decided maybe I should go to the doctor because the pain was getting worse and more frequent. The doctor sent me for Xrays, and said I had “fluid in the hip”. A build up of fluid (I guess normal plasma stuff or something) around the hip joint, which was causing pressure. So he said I should spend 2 weeks in bed. Now this might sound fun, 2 weeks off school just lying in bed reading books and watching movies, but trust me, after a day or 2 it gets REALLY boring. Especially when you feel like you don’t need to be there, I mean it wasn’t like I couldn’t walk or anything.

So I did my 2 weeks in bed, I was told to take it easy, but carry on as normal. Which I did, and after another month or so (around september/october) the pain was back again , but worse! And in my hip aswell as my knee. Back to the doctor, who then sent me to a specialist, an Orthopedic surgeon, more Xrays, and this time he tells us I have now fractured my hip! I fractured it just through normal everday stress of walking on it!! It was round about then that I was diagnosed with Perthes Disease and given crutches :( According to the reseach I have now done, I have learnt that the fracture is totally a textbook syptom associated with Perthes. Fun hey?

THE OPERATION

Yup, I needed an operation to “fix” things. I put fix in inverted-commas because nothing they could do would permanently fix my hip so that I could go on as a normal kid. That actually sounds really harsh but it isn’t really all that bad, I guess you just get used to it.

When the doctor told us that I needed an operation my mum got all upset and started crying, and so I started crying just because she was, I didn’t really understand at that stage what I was about to go through. I had the choice of waiting till after Christmas to have the operation, but then I would have missed more school, so we decided to go right ahead with it and schedule the operation for November.

So anyway… the operation. So what they needed to do was return to blood flow back to the femur head to stop its progressing deterioration. And to do this they had to cut the pelvic bone on the affected side, re-arrange it to relieve pressure so the blood can get back to its job of flowing, and pin it back together again. And while the bone growed back together again I was to be in a cast to restrict movement. (Same as if you break any bone). I will explain better what bone they cut and stuff like that.

Picture your hips (your pelvis) from the front. It looks kinda like 2 ears next to each other right? Well what they did was cut the “ear” on the affected side horizontally, clean in half, through the middle, from left to right! Yeah, I know, it sounds really disgusting and horrible and painfull and yes it is. :( Then then wiggle it about a bit, put it in a better position for the blood flow, then pin it back together again, to heal. This is where the fibreglass cast from my waist to my ankles for 2 MONTHS comes in. Well to be more accurate the cast only went down to the top of my knee on my right (good) leg.

THE CAST

I remember waking up from the operation feeling VERY groggy and sick from the anasthetic, and in ALOT or pain, and trying to push the cast off (causing more pain), and crying, begging my mum to get them to take it off me, and making promises that I would lie still and not move. That was really a horrible experience, feeling so confused, trapped, and restricted.

Needless to say, they didn’t take it off me, and christmas day was spent lying on a fold out desk chair-thingy in the lounge. That year I got alot of books and videos :). My dad adapted one of his garage trolleys that you use to lie on when under the car, he nailed pieces of wood to it so that my deskchair-bed-thingy (what do you call those things?) could go on it, then my brother could carry me outside, and I could lie outside on the patio, and push myself around using my crutches. Another thing we had was a bronze bell on our bar in the lounge, so we tied string to that and led it through to my room incase I ever needed anything. Shame, my poor family ran around getting things for me for 2 solid months :) It wasn’t like I had any choice though.

Those 2 months were really hard, and full of lots of tears and pain. One of bad times was within the first week of having the cast on, the doctor who put the cast on made it too tight around my waist, it was really uncomfortable and was just squashing me. So that needed to be cut back. Actually that wasn’t too bad, there were worse times but I won’t tell you about those because they will make you cringe. A major thing was being lonely, my friends from school would visit me, but I would cry when they left :~( , I feel really bad about that now, because I am sure I made them feel really awful. But I was seriously distressed.

What I am going to say next is going to sound really really stupid, but I will try to explain. When it came to the last week of having my cast on, about mid January, my family would ask me if I was exited that I was getting it taken of soon, and you know what, I wasn’t. Only a very small part of me was looking forward to it, but having the cast on had become such a comfort zone. I had gotten used to life lying flat of my back and not being able to sit up or anything. And the thought of having to leave that was kinda scary. I guess you will only understand what I mean if you have been in a similar situation yourself. After I had been in the cast for about a month I learnt to flip myself over onto my stomach using my leg that was free from the knee down. And my arms had got pretty strong from lifting myself and shifting around using the monkey bar my dad rigged above my bed. So I wasn’t totally restricted anymore, and I had become really comfortable in the cast, it was how I had become used to living.

GETTING THE CAST OFF

Getting the cast off and the pins from my hip removed was a simple operation and I would be released from hospital the same day.I woke up feeling all light and really weak, and standing up for the first time again was a very strange sentation, and also rather a painfull one. My Muscles in my left leg had become VERY weak, and as a walked back to the car in the parking lot (with crutches) I had the most excrutiating spasms in my knee, my right leg was a bit better because i had still been able to bend my knee, so it wasn’t totally weak like my bad side. As far as I remember the spasms only lasted for a few days, until my legs got used to using the muscles again, it could have been a bit longer, but I really can’t remember.

THEN WHAT?

Well after that I had to go back to school, I had already missed the first few weeks of the new year. I had to walk on crutches for about 6 months after that, which in school is actually a major hastle. You can’t carry anything in your hands and walk at the same time! Even the small things like lining up to get your book marked at the teachers desk is a major event, carrying your book in your mouth, or asking ppl to pass it down to the front of class while you get there. So my friends carried for me alot back in those days :)

It was actually really weird when I think back. Everyone is always being nice to you, and trying to be really helpfull. Like they don’t want you to do more than you really have to, like you are made of glass or something and might break any second. I think it got really annoying after a while, but you can’t let on, because they are only trying to be nice.

Getting rid of the crutches was a great relief, by the time I was to get rid of them I could put full weight on my left leg fine. So it wasn’t really a major transition from having them to not having them.

And after not having the crutches anymore there isn’t really that much to tell apart from things like I wasn’t allowed to do sports and stuff at school, including the fun things like playing rounders. Before the operation I used to be a really good sprinter and would beat the other kids by a mile, but that was something else that was long gone. I would get really upset when the other kids got to go out on school outings that would involve physical activities and I couldn’t go :( But that eased up a bit as I got older and I had had enough and started doing things that I guess I shouldn’t have been doing. So in standard 8 (15) i still went to the berg and absailed down a cliff :) So I guess now that leads us to the present day.

Nowdays my hip still bugs me, but I am so used to it now that when it does hurt I only sub-conciously aknowledge it. Matt says I mention when it is sore, like if we walk around town and it gets tired, but I don’t even realise that I am telling him it is sore. It is just a part of everyday life for me. I can also expect arthritis in the future aswell acording the the research I have done, Maybe I already have arthritis? It aches and stuff. What does arthritis feel like? I haven’t seen a doctor about my hip in years, I mean really years! Which I plan to change some time soon. When I get round to it i guess. They told me back then that I would need a hip replacement when I am about 40, but with the advances in technology since then I am hoping that there is something they might be able to do sooner than then, and maybe something not so drastic as a hip replacement. But we will have to see about that.

DOCUMENTS I REFERRED TO:
Legg-Calve-Perthes Disease from Emergency Medicine / Trauma And Orthopedics
LEGG-PERTHES DISEASE – THE SILENT HIP INVADER
Orthoseek Orthopedic Topics (Legg-Calve) Perthes’ Disease
Perthes Home Page – OFFICIAL WEB SITE OF PERTHES ASSOCIATION (also has a good selection of photographs)
Radiology In Ped Emerg Med, Vol 4, Case 16
Wheeless’ Textbook of Orthopaedics

GLOSSARY
Avascular Necrosis – deterioration
Gait – a particular way or manner or moving on foot.
Atrophy – A wasting or decrease in size of a bodily organ, tissue, or part owing to disease, injury or lack of use.
Muscle Spasm – Painful involuntary muscle contraction
LCPD – Legg Calve Perthes Disease
Orthopedic – The branch of medicine that deals with the prevention or correction of injuries or disorders of the skeletal system and associated muscles, joints, and ligaments.

GALLERY (click images for a larger resolution)

Around the time of diasnosis – Age 10 – 91-07-29

Just before surgery – Age 11 – 91-11-13

Immediately after surgery (while in the cast) – Age 11 – 91-11-20

After surgery – still healing (out of cast, pins removed) – Age 11 – 92-02-??

Post Surgery – 6 yrs on – Age 17 – 97-11-26

Post surgery – 11 yrs on – Age 21 – 02-08-28

Post surgery – 12 yrs on – Age 22 – 03-05-21

While I was in the cast Jan ’92
My parents holding me up was the first and only time in the 2 months I was in the cast that both my feet touched the ground. I remember it gave me a head rush :)