Im back again…. with another health update. I am conscious these health updates are quite clinical and factual and you may feel like you’re reading a medical encyclopaedia or studying for your finals. The truth is when writing about my various health conditions I find it much easier to write in this tone and almost detach myself from the situation and be quite factual in my writing. These conditions are having an enormous impact on my life and those closest to me with much emotional upset and turmoil however I feel I want to write separately about that soon. I am not being very descriptive about how some of these symptoms actually physically feel like day in, day out and again I intend to do posts talking about this aspect. I have so many ideas simmering away for future blogs from emotional well-being, the massive impact this is having on us, the health care system and much more which I plan to run alongside weekly blog posts. As mentioned yesterday I aim for the updates to be completed by weekend so I can get stuck in.
With Ehlers danlos syndrome being a connective tissue disorder it means our collagen, the most abundant protein within the body is weak, fragile, stretchy and overall not made very well. Collagen is found throughout the body providing support to skin, tendons, ligaments, blood vessels, bones and internal organs. Essentially the glue that holds our bodies together is of poor quality. As you can see by the role connective tissue plays within the body this can give rise to a whole host of multi-systemic symptoms throughout the body. One area largely effected are the joints.
A large portion of my joints have fully dislocated over the years whereby there is a full separation of the bone from the joint (ouch) these include my right shoulder, fingers on both hands, right wrist, both knees and ankles. I also get what are called subluxations which is a sort of full dislocation “tease” otherwise known as a partial dislocation or significant structural displacement of the bone. I get subluxations of the above mentioned joints as well as my neck, left shoulder, hips and jaw. Personally I am more plagued by chronic subluxations of said joints numerous times a day. With the subluxations being daily the pain which accompanies them and that they leave behind is chronic in nature. That isn’t to say I don’t get full dislocations as I do however some joints are affected more than others. My worst areas for full dislocations are my knees, right wrist and fingers on my right hand. My right knee (the devil) is a daily occurrence and on average wrist and fingers weekly depending on what I am doing. My knees have dislocated whilst turning over in bed which believe me is more efficient in waking you up than any alarm clock going !! At present my main struggles relating to my knees are firstly navigating the stairs in my house, going down them isn’t as bad as going up. Im either having to bottom shuffle up, which to be fair the old girl could do with a work out or if able take the stairs one at a time. Secondly getting in and out of the bath, I’m still working on my partner getting a walk in Jacuzzi bath however don’t seem to be making much progress!!
When a joint fully dislocates it is painful not to mention looks damn right weird. I have had so many dislocations, like many people with EDS we learn to relocate the joint ourselves or we would never be out of A&E (ER), due to the nature of our stretchy collagen some joints believe it or not can be quite easy to relocate/pop back in. There have been times that I have been unable to relocate the joint namely if my left knee dislocates inwards instead of outwards which thankfully isn’t a regular occurrence or when I have thought I had relocated a joint only for it to repeatedly dislocate and find out via x ray it wasn’t quite in. When a joint dislocates it isn’t just left at that it can cause damage to the surrounding ligaments, tendons, muscles and nerves thus feeding in to the cycle of further dislocations and chronic pain.
I find the chronic pain of frequent subluxations almost worse than a full dislocation. When a joint fully dislocates the pain is acute, I maybe known to shout out the odd naughty word, however mentally you know that level of pain will inevitably fade until the next full dislocation whereas the chronic joint pain I find much more exhausting and problematic. I have a lot of aids and braces to hand which I use as and when needed. I alternate heat and ice daily and myself and bio freeze are very good friends. I will only use a brace if a full and particularly nasty dislocation has occurred or a joint is going through a phase of persistently dislocating. The braces rest and immobilise the joint which in turn gives a good level of pain relief and time for healing. I am however very mindful if the braces are on for too long the muscles can atrophy quite quickly which ultimately feeds into the problem further. As we rely on our muscles to hold and support our joints as many of us have ligament tears, damage, long stretchy ligaments that are unable to to do the job they were created for it is vital to keep the muscles as strong as possible (easier said than done).
I recently saw my wonderful physio, I absolutely adore this lady. She has just the right amount of care, compassion, understanding and empathy whilst telling you just what needs to be done. I told her about my ever increasing problems with my shoulder, limited range of motion and that around my shoulder blade keeps swelling when trying to do certain basic tasks using the right arm/shoulder and that the pain has me either in tears or screaming when its nerve type pain. When you have so many pains you learn to differentiate between muscle and nerve pain quite well. She suspects a rhomboid strain or even tear as well as the rotator cuff and labral tear I have. My shoulder blades are also misaligned. We arranged for me to go back to my shoulder surgeon for review which is coming up very soon. She brought another physio in who looked at my shoulders and both were concerned about marked muscle wastage around the shoulders, shoulder blades, arms and upper back which a few consultants have noted recently also. I explained when I try and do even the most basic of the exercises the shoulder blade pain flares so severely I am left having to lay with ice on it to bring the swelling down as the blade disappears through the swelling. After a discussion it was felt I would benefit from hydrotherapy as the water will not only support my joints but the heat will also ease my pain whilst we start to mobilise the joints and build up the muscle in surrounding areas. The muscles are not only wasted, whats left are imbalanced and weak so there is very little to support me right now. Sadly this isn’t just exclusive to my right shoulder this is going on throughout my body and my goodness the pain!!
We have to be cautious getting in the pool with my POTS but as long as I’m well hydrated I’m hoping I will be fine. They have had a few POTS fainters in the pool before but we can only try I say and I’m all for it. Best get myself a decent cozzy!! Ill be starting this as soon as I have had my upcoming iron infusion. The physios taped my shoulder and gave me two very basic exercises to do which although uncomfortable are bearable and don’t seem to be swelling the area up. We had another little discussion about pacing and listening to my pain instead of fighting against it (Don’t seem to be catching on with this very well) often letting stubbornness and sheer frustration take over the show.
I hobbled off to see an orthopaedic surgeon about my knees who examined both knees. Based on my MRI scans a number of years ago I know the ligaments are not in great shape and the grooves of my knees are shallow allowing the knees to slip out quite freely. In fact on the MRI it was noted both knees were subluxed in neutral lay down!! The consultant sent me for an X-ray and an up to date MRI of both knees and I see him in clinic in August for the results. He discussed creating synthetic ligaments as its no good using my own tissue and pinning the knee caps in place however I firmly want, when I am more able to get some physio underway before taking surgical routes. I will however see what he says in August. I was also sent to podiatry to have some insoles ordered which will try and align my knees better.
Next came the multi-skeletal clinic and the topic of conversation was my wrists. For many years my hands have been going numb, tingle with pins and needles and change colour particularly if the wrists and elbows are bent. My GP had already sent me for an ultrasound of both wrists to rule out anything structural so this was step 2. The specialist took a detailed history and began to examine my wrists and elbows. She put my wrists and elbows in various positions and pressed certain areas which brought on the symptoms of pins and needles and numbness quite quickly. She explained she was putting pressure on the nerves which is what happens in people with carpel tunnel syndrome and ulnar nerve entrapment when the wrists and elbows are bent. The longer the nerve was pressed my hand changed colour, I explained to her I could never hold a cup of tea for more that 2 minutes before my hand would go numb, change colour and id drop the mug – my poor carpet and what a waste of brew. She briefly explained the surgery which involves releasing the pressure off the nerve and said as it is happening in both my wrists it would make sense to have my dominant wrist done first. She has referred me on for nerve conduction studies and said once in receipt of the report a referral will then be made to consult with an orthopaedic surgeon.
For as long as I can remember I have had problems with my bladder, for a long time it was put down to “irritable bladder” that absolutely annoying phrase used by some doctors, usually when they aren’t quite sure whats going on and don’t wish to pursue with testing. I persevered with it for many years until a few years ago it became much more troublesome. When you have so many health problems you sort of create a priority list in your head of level of importance. Almost like a mini risk assessment of how much is this impacting my life through pain, discomfort etc and this id say was around the middle of my list. Before my fusion surgery I saw a fabulously thorough NHS lead urology consultant who did not feel this was “irritable bladder” by how I was describing the symptoms. I won’t be shy…. I have a constant pressure feeling I explained to him in “that region” and a consent feeling I need to pee, I will pee and have to almost push it out and I never feel like I am finished which results in a large portion of my day sat trying to piddle, I think a reason why there should be some kind of entertainment system in my bathroom to join the walk in jacuzzi bath (I can but dream). He knew all about EDS and how the bladder can be lax, nerves not necessarily functioning correctly and much more. By the symptoms I was describing he suspected a stricture. He ordered urodynamics testing which I must admit I did not find pleasant at all involving a catheter inserted up both ends shall we say whilst you pee, stop, pee etc during this pressures are taken and how much fluid you retain is detailed. The results came back as normal filling capacity with high pressure, slow flow and some retention. Below is some of the tracking results, not that I have a clue what it means.
I was advised I would need a rigid cystoscopy under general anaesthetic and urethral dilation and was advised to start self-catherterising at least x1 a day and self dilate my own urethra (fun, fun,fun). Given we were shortly heading for surgery I did not do any of the above as the main focus was getting to Washington. Since our return I have been back to urology and the pain remains, I am currently awaiting an appointment with the urology nurses to learn the process and I’m awaiting an appointment with an anaesthetist to try and get a date booked in for the rigid cystoscopy to open the urethra and have a peek inside my bladder. The consultant did say this is something I may need to have numerous times over the course of time as the urethra can narrow again and again. Sadly the urology services are so busy I am still awaiting my appointments.
I have a few skin “things” going on and after showing my GP she sent me onto dermatology for review. After explaining the symptoms and showing the dermatologist I was diagnosed with a few different conditions. Firstly Erthema ab igne, this is quite a common condition seen a lot in the elderly. Essentially my skin when exposed to heat sources such as heat pads, hot water bottles, if I sit next to the fire, rest a brew on my lap will burn, mark, scar and go extremely red initially followed by brown pigmentation which can either last months or scar. There is no cure for this or magic potion other than removing the use of heat sources however like the consultant said is not easy to do when you have a chronic pain condition. There are laser options to try to make it look aesthetically better. I am trying to experiment with different heat sources to find one that does not cause such significant marking. I am also finding aloe vera gel quite useful and bio oil. Secondly is a condition called Livedo reticularis which involves a disturbance of blood flow to the skin causing slow blood flow and reduced oxygen to the skin which results in what I have crassly nicknamed “the dead body” or “corn beef legs”. Alongside this I also like many with POTS have blood pooling, if I stand for not much longer than a minute without moving my blood will drop to my feet and legs, they go very cold/sting/burn if I was to stay in this position I would eventually faint unless I moved around again to get the blood pumping back up to my head. Whilst in the hospital the other day a mild version occurred whilst stood in the queue, I moved one leg and foot purposely for comparison. I’ll put the picture below however with a WARNING as if you’re anything like me I HATE feet and I certainly don’t want to be putting anyone off there dinner!! This would be a mild version of blood pooling for me however I do not want to lose readers by showing the real deal. Needless to say fake tan is my ultimate best friend for my legs however I often feel I am just replacing one problem with another as I am very challenged when it comes to the application of it, basically I’ve swapped red/purple blotchy legs for orange legs instead !!
What a way to finish this blog with a picture of my purple foot, how lucky you all are!! Ill be back soon with my final 2 health updates. I hope you’re all having the best possible day you can.