Dutch guideline for the treatment of scoliosis in neuromuscular disorders

Authors: M. G. Mullender, N. A. Blom, M. De Kleuver, J. M. Fock, W. M.G.C. Hitters, A. M.C. Horemans, C. J. Kalkman, J. E.H. Pruijs, R. R. Timmer email, P. J. Titarsolej, N. C. Van Haasteren, M J. Van Tol-de Jager, A. J. Van Vught and B. J. Van Royen


Background
Children with neuromuscular disorders with a progressive muscle weakness such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy frequently develop a progressive scoliosis. A severe scoliosis compromises respiratory function, makes sitting more difficult, and is perceived as unaesthetic. Spinal surgery is considered the primary treatment option for correcting severe scoliosis in neuromuscular disorders. Surgery in this population requires a multidisciplinary approach, careful planning, dedicated surgical procedures, and specialized after care.

Methods
The guideline is based on scientific evidence and expert opinions. A multidisciplinary working group representing experts from all relevant specialties performed the research. A literature search was conducted to collect scientific evidence in answer to specific questions posed by the working group. Literature was classified according to the level of evidence.

Results
For most aspects of the treatment scientific evidence is scarce and only low level cohort studies were found. Nevertheless, a high degree of consensus was reached about management of patients with scoliosis in neuromuscular disorders. This was translated into a set of recommendations, which are now officially accepted as a general guideline in the Netherlands.

Conclusion
In order to optimize the treatment for scoliosis in neuromuscular disorders a Dutch guideline has been composed. This evidence-based, multidisciplinary guideline addresses conservative treatment, preoperative, perioperative, and postoperative care of scoliosis in neuromuscular disorders.

Scoliosis: Fighting the Pain

The last 9 days have been a little hectic, bad news on our adoption from China which has really taken it's toll on Chris and I. We completed our home study last June and passed panel last October (2007), a few weeks ago (August) we were requested to send our money orders to the DCFS ready for our paperwork to be sent to China, this made us very excited indeed and we were loking forward to the day we would receive our LID (log in date).

All these great plans and positive feelings we soon shot into space when our adoption agency (PACT) called us and asked us for CRB checks from Spain, we had never hidden Spain from them and in fact was a positive part of our passing panel, anyway, they had forgotten to ask us to get these done, putting us weeks behind sending our dossier.

2 weeks agao the CCAA (China Centre of Adoption Affairs) relased a statement to the French ministers indicating that many Chinese families are now eligible to domestically adopt these children, meaning the wait for a Chinese baby could be upto 6 years, with me being 32 and Chris being 40 makes the decision we now have to make very hard, do we continue and become parents at a very late stage in our lives, or change countries in an attempt to become parents earlier, being better for the child and for us, the younger we are the better. These decisions have yet to be made and our lives are in turmoil right now.

My husband was put on a very strict diet by his Dr for 6 weeks to see why the liver is showing signs of an alcoholic! He is not a drinker and since the diet started he has not had a drink now for 10 days, unfortunately, he had a gout attack on Friday due to the new diet he has been put on, consisting of fish, beans (being the main part of a meditterranean diet), fruit and vegetables. The fruit and vegetables have always been a large part of our daily diets so it was not an issue to have to eat more of that, however, his body cannot take all the foods that are high in purines and consequently the attack - he has still not recovered.

I joined him on the new eating regime as losing a bit of weight wouldn't do me any harm. I have noticed dramatic changes in my body since we have increased our garlic intake, instead of letting myself go hungry I eat 3 square meals a day and that includes breakfast. I went to bed on Friday night and slept for 6 straught hours, got up and walked around the house for a bit and then managed to sleep until 11am, I was over the moon!

I hope this will continue and if that is the case I will continue this eating regime forever, I am just so shocked that good healthy food can dramatically change the way your body feels.

The pain is barely noticeable, I did have to go into the spare bedroom this morning as I woke with pain but it is bearable and for me that is the key, I think my back will always give me grief so being able to manage it is the key to my success.

I start physio this week so I hope this also makes a difference. I will report here how that goes and how it makes me feel

Looking for a Dr

I had a lady call me this morning who is in Hull, UK.

She has an 8 year old daughter who has lumbar scoliosis, however, her GP will not refer her to a specialist as the GP does not agree that her daughter can be in pain, nor will she prescribe pain killers.

The woman who called me was clearly distraught and in her words said “I am ready to kill someone”.

Her daughter is having hydrotherapy but this is not working and renders the little girl in pain for the rest of the day – this is like a broken record for UK GPs, they don’t listen and think they know best – they are NOT always right and it disgusts me that a GP can let a child of that age suffer.

If there is a member here from up North, please get in touch if you can recommend a Scoliosis specialist in your area – the lady is prepared to travel so if she cannot go closer to home she will go down south and see someone there.

Thanks for any help that can be offered :)

Must sort this back ache out

I've got a new sort of back ache as well as the rib bump one just now, it's right below my shoulder blades in the middle, every time I wake up in the morning. Plus my neck hurts. And quite often I wake up with a numb arm. I wonder they're all scoli things? I have a habit of blaming everything on scoliosis though...think I will go to the GP and see if I can get something done. But what treatments are there for adults, that you don't have to pay for, other than pain killers? Will they think I'm malingering or trying to jump on some sort of bandwagon because of Laura's surgery?

I once went to a chiropractor but it cost £££ and all I got was an X ray taken at a dodgy angle that made me look like I had a 90 degree curve and didn't even show me in sexy shoes like the one on the right. The treatment for it all consisted of trying to wrench my head off my body (which wasn't having any - loud popping and crunching noises told me that), while I sat with a silly grin so that the kids (I'd had to take them along) wouldn't be scared. He should have paid me!

This was all prompted by my calling Laura "Scoli Girl" and her reply "You've got it worse than me now!". Does you good to have a laff.

BTW and in case anyone read that post, I didn't take the teaching work. And any regrets I might have had vanished the very first time I woke up at the weekend, realised I didn't have to get up for that job and went back to sleep again, despite the back ache!

A visit to the Drs

Well I had my follow up appointment with Dr Ayllon yesterday (I would have blogged last night but we didn't have internet!).

I was scheduled for an x-ray prior to meeting with Dr Ayllon and I have to say I was impressed, especially for the Spanish! Bless the Spanish we always diss them for being late and slow and yesterday in the hospital they blew me away - we arrived at 17:45 for my x-ray and by 17:58 I was out having 2 x-rays done, waiting in reception for my results! In the UK you have to make an appointment which takes weeks, wait around for ages for the x-ray, pay an absolute fortune and it gets very tiresome, then you make another appointment weeks later to see the Dr with your x-rays.

I turned up at the USP Hospital and had my x-rays done in 13 minutes, walked to their other office to see Dr Ayllon and was in for my appointment with him on time - bloody marvellous, but hey I guess that is also the beauty of private healthcare, however, my last x-rays in the UK cost me 199 GBPs for one x-ray compared with €68 for 2 x-rays in Spain - they just don't compare, especially when you also compare speed of results and the lesser waiting times, where are the UK going wrong? Too many people skimming off the top of the NHS system I reckon, lining their pockets with tax payers money and consequently it all goes tits up!

Anyway, back to the job in hand, I could go on about the health system politics of the UK for far too long! - my appointment with Dr Ayllon.

He looked at my x-rays that I had of me bending forward and bending back, good news on this front. My lumbar spine is working together rather than vertebrae being lopsided or off course - excellent.

Dr Ayllon is sending me to Spine School for 4 weeks as he wants to see that I am exercising the right muscles, I did tell him that I force myself to be very active and I have never been one to sit around and complain whether I am in huge amounts of pain or not, but I see his point, he wants to be the judge of whether or not I am doing enough to help myself.

I wish I was as confident as he is about spine school giving me great benefits but right now I am not sure, don't get me wrong I will give it 110% but I have been doing many variations of exercise over the years, everything from Yoga to Walking to Pilates, Cycling (stationary bike), Swimming and other variations of exercises from different books (one being Pain Free by Peter Ecogscue). The spine school is a combination of pilates, muscle manipulation and physiotherapy, I will be in a class of people ranging from 4-12 adults with various spine problems, I start on 2nd October for 4 weeks with 2 sessions each week - Monday and Thursday from 19:30 to 20:30.

Once I have finished spine school I will have another review with Dr Ayllon I expect and discuss how I am feeling, has the pain eased and so on, I will of course continue to document how I am feeling and report on my sleeping patterns.

Should spine school not help me all that much then I have another option which is to have a small operation to insert a device called the "Wallis Interspinous" - I have yet to do research on this device or meet anyone else who has had this inserted but so far what I have learnt from Dr Ayllon seems promising.

The Wallis device is inserted bewteen 2 vertebrae to act as a shock absorber, give me back some support and hopefully reduce the pain from the discs that are wearing out.

Studies have been carried out about this device and patients have reported a 95% success rate with the Wallis, this gives me great hope for the future as I have not been offerred this kind of way out by any Dr before Ayllon.

As I said earlier I need to do further research on the Wallis device but right now I am positive about moving forward and gaining more of my life back, I am fed up with fighting pain all the time but like Dr Ayllon said I do have a life right now and he would rather keep it that way than fuse anymore of the spine.

I think the underlining factor is that he would rather see me manage my pain than have further fusion which may cause other problems in the future and while I can still get about let's try and keep that way. Besides all that, surgery us final, there is no going back, with the Wallis device I can have it removed if it proves to do nothing for me.

Don't misunderstand me here, I never went to see Dr Ayllon in a hope of being offered surgery, that is not what I am about, who wants to volunteer for surgery anyway? Let's face it nobody really would like to go through any kind of surgery, let alone major spine fusion!

I went to him to see if I can find answers and see if there is now another option for me, yes I can get about during the day, yes I can go on holiday, yes I can do gardening and swimming and walking but if I am really honest I have a lot of bravado about me, I cover up a lot of what I am feeling for fear of becoming a complete numpty, I don't want to complain all day long of pain, my friends wouldn't want to spend time with me if I was like that, so I am a silent sufferer where a lot of my pain is concerned.

In the mornings (3AM) when I wake up because I am hurting I feel awful, I struggle to walk or stand straight and the forcing myself to stand or walk is complete agony, short sharp pains up the spine like electric shocks and what feels like muscles spams around the hips - bloody murder! This is when I feel at my lowest, my mind goes off on a tangent and I wish I could end it all, life seems grim at that time in the morning when everyone else is in bed and I am laid on the bathroom floor hugging the side of the bath!

However, during the day when I am more mobile, these thoughts go away and I get through my days twinges, aches and short sharp pains by thinking about those who are in a much worse position than myself - there is always someone out there worse and I guess when you look at it like that - life isn't that bad after all.............. But again at 3AM when I am screaming down the house in pain because I can't move, I don't think about those people, all I am thinking about is how I am going to get myself out of this situation and continue smiling and acting like I can handle it - the long and short of it, at 3AM I CAN'T HANDLE IT!!!!!!!!!!!

One other thing before I go, I don't have flatback syndrome which is fantastic, I believe the biggest factor in play there is because my fusion ends at L1, all the harrington rod patients I have met over the years have had a longer fusion to L2, L3, L4 and even L5, they are the true flatbackers - Dr Andreen said "of course you have flatback syndrome, you have a harrington rod" (the harrington rod can cause a loss of lumbar lordosis) in 2004 but that it was nothing to worry about because my fusion didn't go that far into my lumbar spine - phew! I always thought that the reason I was sooooo stiff and bent over almost double when I got up from a chair was due to the small flatback syndrome shining through - Dr Ayllon said that was not the case and it is my muscles going into spasm instead - good stuff :)

Well I will love and leave you now and look forward to any comments you may have about harrington rods, flatback syndrome and most importantly - the wallis interspinous device

Laters :)

Scoliosis and Stiffness

I am back from my holidays, we went to Doñana National Park camping with my husband's brother and his wife in our lovely Quechua tent.

Before we left we bought 2 camp beds, we usually sleep on a double blow up mattress but our family needed something to sleep on and I am not all that happy with sleeping on the floor, I struggle to get up in the morning.



You should have seen what we managed to ram into an Audi A6 for 4 people to go camping together - hilarious, I swear that the boot gets larger when I show it "stuff" that I need to get into it! HEHEHE. The photo does not do it justice to be honest, not everything was in the car at this point, we still had a table out because we were cooking breakfast on it, so the last things to go in were the cool boxes, food, camping grill, backpack with all knives/forks etc in it, not forgetting 4 rather fatter adults to when we arrived! LOL

I had an appointment today to see a new spinal surgeon in Marbella called Dr Ayllon. My 2 year check up was due and I also want an explanation for the new lumbar pain I have been experiencing over the past few months (which I suspected was a disc).

He looked through all the history I took with me that dated back since 1998, the time I had my S1 injection.

He also went through all the x-rays and mri scans that I took with me and confirmed that it is my L2/L3 and L3/L4 disc that is causing me the pain, he also noticed a fracture in L4 vertebrae that has been there for some time and out of all the Drs I have seen he was the first to notice! I mentioned this fracture to a Dr here and he dismissed as being fine so I walked away and ignored it ----- grrrrrrrrrrrrrrrrrrrrr!

I am going for another x-ray on Monday as all I have had taken over the years are x-rays of me standing up, he wants to see x-rays of me bending over backwards and then forwards to make sure there is no abnormality happening there, apart from the discs.

He said my body is trying to naturally fuse anyway so he would suggest a further fusion using screws and a plate for L3 and L4 discs-----------eeeeeeeekkkkkkkkkk

I was upset earlier and had a little cry, although I knew what was coming it is painful to hear someone say it to you, that is a professional, all it does is confirm your worst fears.

I am not sure I have big enough balls to agree to further fusion, not sure where I will get the bottle from. I can of course stay as I am, but I don’t think I can cope to be honest, and he did say that a natural fusion could cause its own problems such as trapping a nerve which would then need to be addressed anyway - *sighs*

So I now wait until Monday with baited breath to see the outcome of the x-ray and what he would recommend from there. We also talked about artificial discs and he said that he did research when he was in Cambridge with artificial discs and he is not convinced that they actually work or help, perhaps they do help a healthy person but not someone with Scoliosis.

More surgery scares me, when I had my first surgery I was only 12 and non the wiser, it seems so much more frightening now I am all grown up! (well maybe ;)

Scoliosis in Males vs Females

Background
Studies on adolescent idiopathic scoliosis have well documented the differences between natural history of male and female patients. There are also differences in responses to nonoperative treatment, but the results of operative treatment in male patients compared with females have not been widely reported. Only few studies had compared the outcomes of operative treatment between male and female patients with different results.

Methods
We retrospectively reviewed the outcome of 150 (112 girls and 38 boys) consecutive patients with diagnosis of adolescent idiopathic scoliosis who were managed surgically between May 1996 and September 2005. Next, male radiographic parameters were compared with female ones pre- and postoperatively. Then, a subgroup of 38 matched girls was compared regarding the age, curve type, curve magnitude, and the instrumentation we used.

Results
In comparing male patients with unmatched girls, the boys had greater mean age (17.3 +/- 2.3 vs. 16.3 +/- 2.9; p=0.049), greater primary curve (71.4 +/-21.3degrees vs. 62.7 +/- 17.5degrees; p=0.013), less flexibility (30.1 +/- 13.5% vs. 40.3 +/- 17.8%; p=0.01), and less correction percentage (51.3 +/- 12.9% vs. 58.8 +/- 16.5%; p=0/013). The loss of correction was comparable between the two groups. In the matched comparison, the flexibility in boys was less than girls (30.1 +/- 13.5% vs. 38.1 +/- 17.5%; p=0.027). Also, the boys had a smaller correction percentage compared to the girls, but this finding was not statistically significant.

Conclusion
There was similar distribution curve pattern between male and female patients with AIS. Males had more rigid primary curves compared to females but a similar degree of postoperative scoliosis correction. Male AIS patients were older at the time of surgery. These preoperative gender differences, however; did not compromise the radiological outcomes of surgical treatment and the results were comparable between the genders.

Authors: Ebrahim Ameri, Hamid Behtash, Bahram Mobini, Farzad Omidi-Kashani and Behnam Momeni

My daughter had a go at me!

Just goes to show that someone reads my posts! My eldest (23) has reminded me that she is also a member of the scoli community. She's got it as much as I have and describes the same symptoms (pain in icky chairs, goes in more on one side than the other). Well, in my case I am too fat to have much of a waist so it's harder to tell, but I know it's there!

So that's two out of three kids, and I am going to have to watch the youngest like a hawk. The fact that he's a boy will, I hope, mean less chance of his developing scoli. He's autistic and it would be impossible to explain any treatment that became necessary. Still, he's lost weight and, as far as I can see, he "goes in" to the same extent on each side.

I hope Simone is feeling better and that the trip was great.

Best wishes to all