There are a number of side effects of having Ehlers Danlos Syndrome: grumpiness, laziness, cynicism towards the entire medical establishment, and also unfortunately weak bones. EDS causes joint pain… which makes it hard to exercise… and inactivity over a long period can cause your bones to waste and get weaker.
I’ve had joint problems for many years now and haven’t been able to exercise much. So I’m at high risk of getting weak bones, or to use the medical term ‘low bone density’. To monitor the strength of my skeleton I’ve had several bone scans, the first in 2006, then in 2010 and two weeks ago in 2015. Bone scans aren’t invasive or uncomfortable at all. You lie on a firm bed while a robot arm moves over you. They take a 30 second scan of both your left hip and lower spine. These areas apparently give an accurate representation of the bone density across your whole body. The scans have to be processed which takes a week or two.
Bone density refers to the amount of mineral matter per square centimetre of bones. The results are given in two forms:
T score – Your bone density compared to a healthy 30 year old female (I’m not kidding. Unfortunately the comparison is not made in person. I did offer).
Z score – Your bone density compared to a person of your gender and of a similar age.
The T score is more commonly used as it better predicts the risk of a future fracture. The results are expressed as a ‘standard deviation’ away from a normal result eg. -2 SD (standard deviations).
A result of 0 is normal.
A result of -1 or more gives you a diagnosis of Osteopenia (bone density which is lower than normal).
A result of -2.5 or more gives you a diagnosis of Osteoporosis (Osteoporosis literally means ‘porous bones’).
A result of +9000 or more means you’re probably from the planet Krypton (I am such a nerd!)
Below are my T score results for the three bone scans over 9 years. My Z score results are similar but slightly higher.
200620102015
Left Hip -1.7 -1.8 -2.8
Lower Spine -2 -2.6 -3.1
As you can see my bone density has been getting steadily worse. In 2006 I was diagnosed with Osteopenia. As of two weeks ago my diagnosis is now Osteoporosis as both my hip and lower spine have bone density more than -2.5 SD away from the norm.
For long periods in the last five years my nutritional intake has been really poor and I’ve also been very inactive. So I was expecting my latest bone scan results to be worse, as was my GP. I wasn’t upset about the new diagnosis and I’m not in any extra pain. After my 2010 bone scan I spoke to a rheumatology consultant and I asked for the problem to be treated, but he declined. Now my diagnosis has changed to Osteoporosis it’s very likely I will be given treatment. So I’m actually rather relieved that an issue I’ve known about for many years will finally be dealt with. It’s odd, but the problem needed to get worse for it to be taken more seriously.
Osteoporosis is not life threatening, but if I had an accident I’m more likely to break a bone. Thankfully Osteoporosis is treatable with drugs called bisphosphonates. Bone undergoes constant turnover and is kept in balance (homeostasis) by osteoblasts creating bone and osteoclasts destroying bone. Bisphosphonates inhibit the digestion of bone by encouraging osteoclasts to undergo apoptosis, (cell death), slowing bone loss.
I had a blood test last week which showed my calcium and vitamin D levels are in the healthy range. So thanks to parenteral nutrition my dietary issues have been resolved and won’t contribute to this problem anymore. However, due to inactivity from joint pain my bone density could get even worse so I’m being referred to Dr Alan Hakim, a rheumatologist and specialist in hypermobility. I’ve mentioned Dr Hakim’s name on this blog before. I’m in safe hands.
I found a couple of studies which showed the link between EDS and low bone density may not just be lifestyle related and there may be an inherited structural element. Several connective tissues diseases such as Osteogenesis Imperfecta affect the strength of bone and the whole body shape of people with EDS can be different (marfanoid habitus). The link is very plausible however low sample groups were used in a small number of studies, so this currently seems to be considered as unproven. Further research needs to be done. (ref) (ref).
It was my Dad’s birthday on the day of the scan, but he kindly came with me to the hospital. Happy Birthday again Dad, it was great spending time with you on your birthday. Sorry I dragged you to a boring bone scan. I promise next year I’ll take you to a much more exciting medical procedure. 🙂
Here is a bar chart showing my emotional response to my latest bone scan result:
Here is a pie chart showing the percentage of my body expensive medical scans have found to be puny:
Thanks for reading, all comments welcome. Perhaps you might enjoy listing the other ways in which I compare unfavourably with a 30 year old female.
This blog is interesting on three specific counts;-
– your clever usage of long medical terms – almost as if you understood them;
– your newly created, informative pie charts; I can recall when you thought that Pie charts referred to Desperate Dan’s diet in your weekly Beano;
– your kind birthday wishes – more than I actually got on the day in question.
I suppose I should add, on a less serious note; it’s ever a pleasure to be your accompanist. Next time, I’ll bring the guitar.
D
A Reluctant Contortionist
March 16, 2015 @
4:55 pm
Thank you for your comment. – No birthday wishes on the day? Do expensive gifts not count? I’ll remember that next year.
I learnt one long medical term early in life as it was used so often in our house, Minoxidil (Regaine Hair Loss Treatment). Despite my genes I have no use for it.
You’re welcome to accompany me any time. But no guitar, sorry. Not even on your birthday.
A Reluctant Contortionist
April 2, 2015 @
10:10 pm
Thank you Scott. It’s reassuring to know that you’re there for me, …unless things get tough.
As for my fathers guitar playing, I’m supportive of a hobby he loves and I carry no psychological scars from his regular home performances during my childhood (well apart from that twitching catatonic state I enter when I hear any song by The Everly Brothers).
Hi Ceri, Looking at some of your gallery photos you did appear to have bobbed hair like a 30 year old female but no other comparisons I can see 😉 Glad you can still find humour in everything you have to go through. Hopefully this diagnosis will be the start of some very important treatment for you, All the best xxxx
A Reluctant Contortionist
March 16, 2015 @
8:43 pm
Hi Amanda,
My girly bob is my favourite of my many bad haircuts. Thankfully I don’t have photos of all of them. – Osteoporosis isn’t a nice diagnosis to add to my collection. But there are effective treatments and I’ll be on one of them soon. So no biggie!
Thanks for your message x
You must be radioactive after all those scans! I’ll bring my geiger counter next time I visit. At least it was only a robot’s arm waving at you and you didn’t have to lie in one of those claustrophobia-inducing rumbling tunnels…. Good luck with the treatment!
A Reluctant Contortionist
March 22, 2015 @
12:44 pm
No sign of radiation sickness so far, unless it’s causing the grumpiness and laziness? – The treatment won’t start for a while and it’ll be a couple of years at least until my next bone scan. So there won’t be an update on my bone density problem for a while. Thanks for you comment.
There are a number of side effects of having Ehlers Danlos Syndrome: grumpiness, laziness, cynicism towards the entire medical establishment, and also unfortunately weak bones. EDS causes joint pain… which makes it hard to exercise… and inactivity over a long period can cause your bones to waste and get weaker.
March 15, 2015 @ 11:42 am
I’m surprised the ‘not puny’ rating is quite so high. I had it at around 3%.
Great article Ceri, keep them coming.
March 15, 2015 @ 9:07 pm
Thank you. – In case you were wondering, the part of my body that expensive scans found to be ‘not puny’ was my …brain. 🙂
March 15, 2015 @ 12:12 pm
This blog is interesting on three specific counts;-
– your clever usage of long medical terms – almost as if you understood them;
– your newly created, informative pie charts; I can recall when you thought that Pie charts referred to Desperate Dan’s diet in your weekly Beano;
– your kind birthday wishes – more than I actually got on the day in question.
I suppose I should add, on a less serious note; it’s ever a pleasure to be your accompanist. Next time, I’ll bring the guitar.
D
March 16, 2015 @ 4:55 pm
Thank you for your comment. – No birthday wishes on the day? Do expensive gifts not count? I’ll remember that next year.
I learnt one long medical term early in life as it was used so often in our house, Minoxidil (Regaine Hair Loss Treatment). Despite my genes I have no use for it.
You’re welcome to accompany me any time. But no guitar, sorry. Not even on your birthday.
April 1, 2015 @ 5:30 pm
‘Next time, I’ll bring the guitar’
Ceri, there are people you can go to. You do not have to suffer these vile, violent threats alone.
I will be there for you*
*unless he brings his guitar. In which case, you are on your own!
April 2, 2015 @ 10:10 pm
Thank you Scott. It’s reassuring to know that you’re there for me, …unless things get tough.
As for my fathers guitar playing, I’m supportive of a hobby he loves and I carry no psychological scars from his regular home performances during my childhood (well apart from that twitching catatonic state I enter when I hear any song by The Everly Brothers).
April 2, 2015 @ 11:10 pm
Just think how the Everly Brothers feel about it!
March 15, 2015 @ 2:44 pm
Hi Ceri, Looking at some of your gallery photos you did appear to have bobbed hair like a 30 year old female but no other comparisons I can see 😉 Glad you can still find humour in everything you have to go through. Hopefully this diagnosis will be the start of some very important treatment for you, All the best xxxx
March 16, 2015 @ 8:43 pm
Hi Amanda,
My girly bob is my favourite of my many bad haircuts. Thankfully I don’t have photos of all of them. – Osteoporosis isn’t a nice diagnosis to add to my collection. But there are effective treatments and I’ll be on one of them soon. So no biggie!
Thanks for your message x
March 19, 2015 @ 10:34 pm
You must be radioactive after all those scans! I’ll bring my geiger counter next time I visit. At least it was only a robot’s arm waving at you and you didn’t have to lie in one of those claustrophobia-inducing rumbling tunnels…. Good luck with the treatment!
March 22, 2015 @ 12:44 pm
No sign of radiation sickness so far, unless it’s causing the grumpiness and laziness? – The treatment won’t start for a while and it’ll be a couple of years at least until my next bone scan. So there won’t be an update on my bone density problem for a while. Thanks for you comment.