Ehlers-Danlos Support UK

Blue sclera

Blue sclera

I have an UCTD diagnosis (since 2014) and my unknown collagen disease is getting worse. I got two newly discovered symptoms: blue sclera, see picture (is anyone familiar with this?) and slightly parrot beak nails on my thumbs, indexfingers and middle fingers. My GP just suggested to Google these symptoms.

Anyone recognizing this blueish sclera?

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Blue sclerae can be a symptom of EDS, along with being a symptom of a few other conditions too. I don't have them.

I have not heard of 'parrot beak' nails before. Mine are just very white (look like they have just had a French polish) and very soft - they just tear off. Edit: just looked at my nails and they curve over at the ends rather than carrying straight on - it just isn't obvious because they are usually short due to just tearing off. I don't know if that is the same thing as you are describing?

There are other connective tissue disorders, including osteogenesis imperfecta, which lead to blue eyes and bone problems, which I assume has been ruled out because you have never broken a bone?

UTCD is really not a helpful diagnosis - each one of the different collagen disorders requires a different set of tests and treatments - not to cure it but to catch any of the common side effects before they turn into something serious. eg. for hEDS we should have a check for mitral valve prolapse. For vEDS much more medical supervision is necessary.

I would push for a more definite diagnosis. I cannot believe your GP told you to google it...even a diagnosis of hypermobility spectrum disorder, poor as it is, is better than UTCD. Are you hypermobile?

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Dear Jay,

Thank you for your very informative respons. I did have hypermobility syndrome as prior diagnosis but my current consultants say I am not hypermobile.

I am hypermobile in my wrists, my feet and every other small joints in my body. My current consultant checked my large joints only and concluded I am not hypermobile at all.

When I was young (I am 42 y/o now) I was also hypermobile in some large joints (but some exercises like placing my hands flat on the floor and having my legs straight I could never do).

Unfortunately my feet went very bad past few years and I can only walk about 15 minutes in orthopedic shoes that are made especially for my feet. Because of this and because I get easily fatigued/exhausted I currently even use a wheelchair if I expect to need to walk longer than 15 minutes.

I also need wrist bandages to be able to work at the office.

Fortunately I have never broken a bone.

I have an uterine prolaps which is also strange at my age (I never delivered a baby due to extensive endometriosis and adenomyosis) and with my slender posture. My mother had many miscarriages and she is hypermobile too.

I have different skin marks of classic EDS.

I have many symptoms that overlap several collagen diseases and am tested positive for auto immune markers ANA en ANCA (every time it is tested so multiple times).

I am in twilight zone for a long time now.

I will have a major surgery on March 2nd. The endometiosis and my uterus will be surgically removed. I told my gynecologist that I have collagen problems and after my last surgery the surgical wounds did not heal for a long time. However as I don't have a real diagnosis about my collagen malfunctioning they don't take this seriously and I am quite worried...

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The qualifying description for being hypermobile is can you now or, (if older) could you ever do the qualifying bends. What consultants are you seeing? You need to see a rheumatologist who is qualified to diagnose EDS. Also, some EDSers have quite limited hypermobility - in just a few small or large joints rather than overall. My shoulders are not hypermobile, for instance, but I scored 9/9 on the old Beighton test.

Have a rummage around this website and see what sounds familiar - then I would definitely push for a referral to a qualified rheumatologist and flag up your skin healing issues again in writing with your gynaecologist too before your surgery.

ehlers-danlos.com/eds-types/

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Hi Jay,

Regarding the nails. Mine are similar to how you describe yours. They curve downwards at the end. And mine also curve and both sides downwards. Also the angle between nail and nailbed seem much more flatter.

My nails turned like this in a few weeks time.

Greets,

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Forgot something really important - if you do have EDS, many of us are resistant to local anaesthetics such as lidocaine. Neither local anaesthetics nor epidurals work on me - although sometimes they start working, the procedure begins, then they stop working almost immediately. My life with surgery is straight out of history - just grit teeth and ask them (without crying) to please get on with it. Get your gynae to double check you are under general anaesthetic and to not assume that a post op local will work.

edsinfo.wordpress.com/all-a...

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Dear Jay,

That is very relevant info. Thank you so much! I will get general anesthesia and a few years ago with a simple surgery that worked fine with me. However only dormicum, used at colonoscopies, did not work and I woke up in pain in the midst of the procedure scarying the specialist.

As pain relieve after the surgery they offered my a morfin pain pump. My experience with morfin is that is does not relieve pain only for a bit and I get very nauseos from it. So I requested an epidural drip for postop pain and they promised me they will give it to me.

So fingers crossed and I will make sure to mention it to the anesthesist.

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It's kind of a relief to hear about others' problems with local anaesthetics. My bottom teeth are very difficult to numb, but it took me a long time to convince some dentists. I was once thrown out by a dentist for being "hysterical". It typically takes about half a dozen injections to numb me.

I also cannot take dental anaesthetics that contain adrenalin. They completely overwhelm me. Does anyone else have this?

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I cannot take adrenaline - it feels as though I am having a heart attack. The last time I went to a dentist (new one) I heard him talking and realised he wasn't taking what I said seriously. I asked him to speak to my last (retired) dentist. He did, and looked it all up online, and when I came in for an attempt at an extraction he was completely different with me. He had two more powerful anaesthetics lined up and was happy to stop anytime. He also understood the urgency of doing the procedure before the anaesthetic wore off.

If you ask (well in advance) for articaine without adrenaline (4x lidocaine strength) , they should be able to get you some. It will still wear off quickly. My dentist managed an upper tooth extraction, with a different anaesthetic 3 x the strength, and gave me 3x the amount. It was wearing off by the time he was giving me the wads of cotton. He said I was metabolising the anaesthetic 50x faster than an average person.

Anyhow I have no more wisdom teeth left, so hopefully shouldn't need any more teeth out.

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Hi Patricia,

I agree with Jay66 regarding your up coming operation. Please make sure that the "ward" you are sent to after your operation, as we as your anaestesiologist, are also fully aware of your connective tissue issues.

I recently had surgery and was lucky to have an anaestesiologist who knew about EDS. He made sure that I had a very slowly diminishing anaesthetic and also strong pain medication before I came round which helped to keep my pain levels under control directly after the operation.

I had also spoken to the admissions nurse prior to my op and told her about my EDS and how I experience both chronic and acute pain daily with this condition. We had also spoken about my having a pressure relieving mattress (I have a special pressure relief topper at home) and also about my being able to discuss how my EDS might affect me post op with both the surgeon and ward doctors the night before my operation. After this conversation I felt quite confident about going ahead with my operation but despite these preparations, things did not go according plan.

It was once I was back on the ward after my operation that everything went so horribly wrong. For my widespread EDS joint and muscle pain which unfortunately affects "all" my joints and muscles, I take slow release MST Morphine tablets twice a day and Amitriptyline at night. I also rely heavily on Ibuprofen Gel and my GP also prescribes me Liquid Morphine for break through pain however I only ever use it as a last resort when my pain levels are through the roof, 10/10 or above!

Unfortunately the ward staff made the mistake of thinking that my GP daily EDS pain medications, were what I was being given for my post operation pain and therefore I wasn't given anything other than paracetamol and the occasional dose of liquid morphine for my post op pain.

I also wasn't allowed to use my ibuprofen gel because of the bleed risk, so effectively I had less pain relief post operation, than I'm able use at home for my EDS pain. I should have been able to be discharged within about three days after the operation however by the third day I was in the most excruciating pain I have ever experienced and could barely even sit upright!

Because I was being given morphine and amitriptyline, the ward nurses and doctors wouldn't believe that I could be in so much pain, plus they had no idea about how EDS can cause both chronic and acute pain everywhere in the body. They also had no idea that if post operative pain is not controlled in someone with a connective tissue disorder like EDS, that it will then start raising their pain levels everywhere!

I didn't get seen by the Acute Post Operative Pain Team until eight days after my operation and at first the pain team doctor was really horrible to me, saying that I couldn't possibly be in so much pain because I was already being given morphine tablets, amitriptyline and liquid morphine. When I explained that those medications are my GP prescribed daily pain medications for my EDS pain, the Pain doctor suddenly looked very worried. The penny then dropped as she realised that I hadn't been given any additional pain medication for the post operation pain and she preceded to tell me that I was now in what they call a "pain crisis"!

She told me that she would need to raise my morphine much higher than my usual dose, in order to get my acute post op pain under control. I really didn't want to do that because going up on my morphine dose meant that I had to come back down again, which is a very unpleasant experience (hence only ever using liquid morphine as an absolute last resort) however the pain levels all over my body were now so acute, that the pain doctor said I would need this higher level of morphine in order to get my pain under control.

I was also given Lidocaine Patches to put on my abdomen which I wish I had known about years ago! I now use these at home for my EDS pain relief and also for the abdominal pain I am still experiencing since my operation. I don't know if you have ever tried them but they definitely helped with my acute post op abdominal pain?

Re hypermobile joints, I am no longer hypermobile due to my pain and injuries but I was quite hypermobile in my youth. I did ballet, tap and gymnastics etc and just thought that everyone else had pain in their legs when they did these things. I think that my joint pain prevented me from being more hypermobile than I actually was because it hurt me so much when I did the splits in gymnastics class, or hurt too much to lift and hold my leg up very high when doing ballet.

When I was younger only my leg joints were affected but in my twenties my back, neck, arms and hand joints joined in but I wouldn't say that I was very hypermobile in all these joints. I'm 51 now and some of my fingers dislocate but I would describe my joints as being loose, like they could come out of their sockets but I've only ever subluxated as opposed to dislocating my joints like many EDS sufferers do.

Like Jay66 says, EDS affects everyone differently and they are now finding that many connective tissue disorder sufferers have a mix of EDS symptoms, ie some from the hypermobile type, some symptoms of the classical type and so on, so it's not acceptable for your rheumatologist to be unwilling to consider a diagnose of EDS, just because some of your joints don't appear to be hypermobile.

I too have a prolapsed womb although I haven't been able to consider surgery yet because I've had other more serious health issues, that my doctors said they must address first. I would be very interested to know how your operation and recovery goes however, if you're willing to share, as it's something I may have to face in the not too distant future?

Good luck with everything, don't let the doctors fob you off re your connective tissue issues because the sooner you can get a proper diagnosis, the sooner you will be able to get the correct treatment.

Claire 😀

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Hi Claire - I have not heard of lidocaine patches. I wonder if it would help? Do you need a prescription?

I've just come back from Spain where the warm, not hot, but very dry weather plus daily walking left me not needing anywhere near as much pain relief. Back in England one day and my leg feels like it has separated from my body - the joint is so loose and everything is starting to hurt again.

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Hi Jay66 , Oh crikey, you have come from warm weather to Arctic weather, I'm not surprised you're in pain. Yes you need a prescription for lidocaine patches but my GP was happy to prescribe them for me. Having read the information on the patches it says that it can take a while for them to give a full pain relief affect so I guess you're supposed to put one on every night /day which ever you need the pain relief for but I tend to put a patch on as and when I need pain relief, ie at night when my pain is bad to help me sleep, or in the day if I visit my mum and need extra pain relief.

I would definitely ask your doctor if you can have them, I don't think they are contradictory to many other medications but your GP should know. Hope you can have them. Get a hot pad or water bottle on your hip in the meantime as the cold will make your pain even worse.

Claire

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