The Critique of my Marfan Physique.

Written in response to the unwelcome yet frequently made remarks on her strikingly slender physique, Lucy’s piece will resonate with many Marfan folk whose slim stature is also the recipient of such unwarranted attention.

 

Hey guys, here's a blog written by me, published by Marfan Trust. The Critique of my Marfan Physique.

 

 https://www.marfantrust.org/articles/201-the-critique-of-my-marfan-physiquehttps://www.marfantrust.org/articles/201-the-critique-of-my-marfan-physique

A Real Pain In The Behind

 Earlier this year, I wrote a blog detailing the issues I've faced as a young woman with Pelvic Organ Prolapse- A condition brought on by my Connective-tissue Disorder, Marfan syndrome. The Marfan Trust very kindly offered to share this blog & I was delighted to hear it went down well with a number of their followers. I have added a link to that blog post below and I hope it helps to shed some light on what life is like as a Marfan patient with Pelvic Organ Prolapse. I hope it goes down as well with my readers. Some words may have been changed or edited by the Marfan Trust and the image was added by them, but the overall blog was written by me. Thank you! <3 https://www.marfantrust.org/articles/4-marfan-syndrome-can-be-a-pain-in-the-behind

 

Dental Distress (Connective-Tissue Issues)

It's almost a decade now since my disastrous dental problems began. I was still a teenager & suffering relentlessly with toothache and painfully sore gums. I consulted my Dentist numerous times regarding this miserable matter but he couldn't pinpoint an actual cause. He said my gums were severely inflamed and my teeth were very sensitive. That was the best answer he could provide me with. The annoying thing was that it couldn't be treated as there was little that could be done for sensitivity & inflammation. so I was just advised to keep my oral hygiene as healthy as possible, and avoid eating anything that triggered it off. I didn't understand why this was happening though as I wasn't someone who lived off sugary snacks, so as a rule, my teeth & gums should not have been so inflamed and sore. I mean, I liked a chocolate bar and a cake as much as anybody else, but I wasn't consuming enough to cause so much dental distress. It became an on-going issue, and over time, it got to the point of me not even being able to enjoy an occasional sweet treat as it just caused an epic "dental flare up" as I called it. Which resulted in days of toothache and swollen gums. Eventually, I was having to avoid a wide range of food & drink because anything that contained sugar (even a small amount) seemed to be the biggest contributing factor. It felt like an endless circle which was never going to get resolved. I was mostly keeping this problem at bay purely by swerving sugar, but I wasn't wholly convinced that there was no underlying cause.

It became a bit of a nightmare though as I couldn't bite down on anything solid anymore or chew hard foods without my gums starting to bleed and throb, so I resorted to blending up some foods and consuming them through a straw to avoid further irritation to my mouth. It was also a way of adding more variety into my diet instead of keeping it bland for the sake of my teeth, which I had already been doing for too long! Blending certain pieces of food up into a smoothie isn't ideal for everyone, but it's keeping my fragile gums and teeth in tact, so it's a wise & worth it alternative for me. I'm sure it's not everyone's favourite way of eating their apples, but it's my 'normal' now and I'm used to it. At least I'm not missing out on my fruit anymore! I do miss the occasional sweet treat though, but I'd tried on numerous occasions to reintroduce my mouth to a sugary snack in the hope that the same old problems might've cleared itself up, but it seemed that was a little too optimistic. I was growing really tired of not knowing what was causing this now though. I know I had permanently inflamed gums & sensitive teeth, but why? I felt like something was being missed or overlooked. There was more to this than what was meeting my Dentists eye, and I knew it! 

 

It all became clear in 2018 after a few dental X-rays revealed I had severe gum disease, and on examination, it was now obvious that I had a fair bit of gum recession as well. Recession and periodontal disease are both known causes of inflammation & sensitivity, but although I now had an explanation of sorts, I still didn't understand it. I wanted to know how this had happened when I'd avoided not just sugar filled snacks, but anything & everything that contained the slightest trace of sugar for the last few years. I now felt more bemused than ever but I listened carefully as the Dentist expressed his new take on the matter. He now believed my Connective Tissue Disorder, Marfan syndrome to be the root cause of all this. Well, he and the Dental Hygienist both suspected this to be the case because the gums are made up of connective tissue, and if that tissue isn't as healthy as it should be (which mine isn't) then it will lead to receding gums and periodontal disease, which inevitably causes toothache and inflamed gums. I was shocked, but not surprised to find out that Marfan syndrome was the culprit. It's affected most of my body over my lifetime and now it seemed my teeth were no exception. It all made sense now I'd been made aware of it. It was the Issues with my Tissues!

 

Disappointingly, at the moment, nothing has been resolved. There isn't really any treatment for receding gums and tooth sensitivity. The Dentist mentioned having Gum Graft Surgery which might help, but I have a lot on my plate with my health already, and the surgery isn't guaranteed to work, so I'm still debating whether I should put myself through it or not. I obviously don't want to live with so many dental issues forever, so something needs doing soon, but to be honest, if replacing my teeth with false ones would solve the problem, I'd have them all taken out. That's how fed up I am with this unrelenting issue. But, at least I now know that there always was an underlying cause like I suspected, and it wasn't all in my head. 

           Lucy.. From Life Of Lu.. X

The Daily Realities..

Over the last few years, I've been very open on Blogger about my life with Marfan syndrome and numerous health conditions, but I haven't been quite as forthcoming with those around me because if I'm to be honest, I struggle to fully express the significant impact that chronic illness/health conditions have on my day to day life. I don't look like someone with chronic illnesses. I don't use a wheelchair, a stick or any disability aids, so I'm afraid of telling some people in case they think I'm over dramatising it. But that is far from the truth. I've lived with chronic pain for a long time now, gradually building up and becoming more widespread over the years. I've learnt to become good at pushing my unpleasant physical sensations aside to get on with my every day life though, so no one would ever guess it. No one would know (unless you are my Mother) that on a typical day I experience several different types of pain and discomfort throughout my body, ranging from moderately annoying to severe. My connective tissue disorder, Marfan syndrome is the main cause of all this. My connective tissue isn't strong enough to support my joints, ligaments or internal organs, so this causes me to live in almost constant chronic pain. I'm usually experiencing various aches and pains when I get up in the morning, which worsen as time goes by and more often than not, I go to bed with the same amount of discomfort that I woke up with. But as a matter of necessity, I've learnt to carry on with life's daily activities regardless of how I'm feeling. I get up. I dress up, and I go about my day as normally as I possibly can. I smile, laugh, and enjoy my family's company, but I am hardly ever completely symptom free.

 

I also have an autonomic nervous system disorder called PoTs, something else I've picked up as a result of Marfan syndrome. PoTs alone can be a lot to cope with, let alone combined with a connective tissue disorder! The autonomic nervous system regulates vital bodily functions, so mastering the art of adapting to life with PoTs hasn't been an easy challenge. The symptoms for me consist of near syncope, nausea and tachycardia, as well as some less obvious ones like fatigue, weakness and GI dysfunction. It frequently impairs my ability to do things in a 'normal' way and I have to set aside some recovery time after carrying out simple tasks like washing my hair, dressing and showering. I often end up sitting or lying down in the middle of doing these things as being on my feet brings on the symptoms. I can't finish a meal in less than an hour because my PoTs related gastric issues prevent me from eating as fast as 'normal' people. I'm known very well by my family for being the slowest eater ever! I don't talk much about life with PoTs though as it's all a bit hard to explain when no one's ever heard of it. Plus, I don't 'look' like someone who has to rest after taking a shower, so would anybody even believe it? I can stand, walk, and talk, so I feel people would assume it can't really be that bad. 

 

I don't just live with Marfan syndrome, PoTs and chronic pain. I also have various other conditions including Epilepsy, a full thickness rectal prolapse, and PCOS. I'm currently awaiting bowel surgery to fix the prolapse but at the moment it's causing it's own set of problems. I hope to have it rectified by next year at least, as it's something I've lived with for almost 2 years now, and again, it was caused by my faulty connective tissue! It's not exactly a pleasant topic to discuss so I've mainly kept it to myself apart from on my blogger account where I have spoken out about it occasionally. The purpose of today's blog was just to bring attention to the fact that a person can 'look' good, or even great, and still be living with long term health conditions or chronic illness/s. Someone can be happy and enjoy life, but still feel unwell due to their medical problems. More often than not, I just choose to remain silent about it because I don't want to moan, and it's hard to explain. But it's important for others to remember that chronic pain is invisible, and so are some long term health issues, like Marfan, PoTs, and epilepsy. 

Share Your Victories (Marfan Syndrome Awareness Month)

Aaand I'm back, guys! I know I've been away from Blogger for quite some time now, but I was determined to make a comeback for Marfan syndrome Awareness Month this February. I usually have a blog planned and ready to post well in advance but I'm writing it on a bit of a whim this time as I've had difficulty getting back into the swing of things, so forgive me if this all sounds a bit rushed. I've given some thought to what I want to write about today though, and I've chosen to do a 'Share Your Victories Challenge' which is an idea that stems from the Marfan Foundation as they are asking those affected by the condition to publicly share their victories against Marfan syndrome. It's definitely a challenge I feel able to participate in because as some of you will know, I've faced a few trials and tribulations of my own due to Marfan syndrome over the years. I haven't been through as much trauma as some Marfan syndrome patients have, but I still feel like I've been up against it for much of my life. I suppose just living with this unpleasant condition every day is a victory in itself, as well as putting up with all the unwanted comorbidities that go with it, but I might save all that for next time. Because out of everything Marfan syndrome has thrown at me, I think the ten-hour Spinal operation I underwent at just 13 years old was the most life-changing thing, so I've picked that as my Share Your Victories topic.

The operation I am referring to was a spinal fusion, which will be familiar to a lot of people with Marfan syndrome. We hoped that I wouldn't need the surgery until I was a few years older, but I had an aggressive curvature of the spine which was squashing my lungs and restricting my breathing. The curve was progressing all the time and my back was visibly hunched over, so I had no choice but to go ahead with the operation, whether I liked it or not. Two titanium rods were inserted at each side of my spine, along with a number of bolts/screws to hold them in place. It was almost the end of the day by the time I came out of the theatre, but everything went well and I got through it, so to me, that was a total WIN against Scoliosis/Marfan syndrome, although I don't remember thinking that at the time. I do remember spending five long weeks in the hospital afterward though, which was tough. I went through some excruciating pain, but with each day that passed, although I didn't think it at the time, I was overcoming this huge hurdle, and looking back on those days now, I see that as ANOTHER WIN against my condition!

After getting out of the hospital, things were difficult for a long time. I was laid up at home for months, unable to do much at all. But eventually, I began to adapt to life with rods in my spine. It wasn't easy, I struggled a bit, physically and mentally, but in hindsight, I guess I should've felt victorious because as rough as it was, I overcame it all at just 13 years of age. And that my friends, is called triumphant!! I just wish I had a picture of my 'hump' so I could show you all how much better my back looks now that it's visibly straighter. I only have photos from after the surgery, so on the first pic, you can vaguely see my 14-inch scar running down my back, which I love. I definitely do feel victorious about my perfectly straight spine and neat scar. I'll happily show it off with pride. Unfortunately, the other two photos aren't as attractive to look at as I've experienced some issues with hardware protrusion. This means the bolts and screws at the top of my spine have somehow come loose and are visibly protruding through my skin. It's really painful some days, and it's one BIG inconvenience to live with on a daily basis, but I don't look at these photos and feel defeated, I feel victorious yet again because by enduring this every day, I'm also overcoming a massive obstacle that's been thrown my way post-surgery, and that alone is a VICTORY in it's own right!! #WhatsYourVictory #MarfanSyndromeAwarenessMonth

The first pic is what my back is like on a normal day. The other two shows you what my spinal hardware is like on a bad day. It shows the reality of life for me with spinal hardware protrusion.

Life As A POTS Patient (Postural orthostatic tachycardia syndrome)

Postural Orthostatic Tachycardia Syndrome is a controlling condition, and everyday life is quite restrictive for its patients. Especially when it comes to things like leaving the house. I can't just take myself off to browse around the shops without thinking, I have to spend some time carefully planning my route before going anywhere because I need to make sure I'm never more than a few minutes away from a seat as my body can’t handle being on my feet for too long. If I don’t take regular sitting down breaks, I quickly go into a POTS flare up which makes me feel physically awful. So as soon as I start experiencing heart palpitations and dizziness, I make my way to the nearest seat as swiftly as possible so that I don’t end up passing out. I very rarely go out alone due to my medical problems, particularly POTS, but that’s just the reality of life with chronic illness.


Postural Orthostatic Tachycardia Syndrome has even taken away my ability to shower as and when I feel like it because it brings on a POTS attack and my body can’t handle the stress it causes. unfortunately my personal hygiene often has to suffer because I have to wait until I feel mentally prepared to take on the challenge. I always try to shower at night time because I end up in bed afterwards due to physical exhauston. POTS doesn't affect everyone in this way though. Some people are fortunate enough to only experience mild symptoms, but for people like me, it can be more debilitating and affect the quality of their everyday lives. It can affect anyone at all, but it more commonly occurs alongside a more serious health condition such as Marfan Syndrome, which is the case with me. I was diagnosed with Marfan Syndrome as a teenager and at some point I want to discuss all the secondary ailments it's brought with it and how they also affect my life, but I'll save that for another time. I just wanted to bring light to POTS today because I feel like it needs more awareness than it gets.

Postural Orthostatic Tachycardia Syndrome is an autonomic abnormality of the nervous system, causing a drop in blood supply to the heart and brain when a person is in an upright position. So that’s why I’m unable to be on my feet for a length of time. The typical symptoms for me include heart palpitations, dizziness, nausea, sweating and fatigue. I often end up going for a nap in the middle of the afteroon as POTS, along with all my other health issues, has drained me of all energy. It's not a curable condition, but medication is keeping my heart rate steady at the moment, rather than shooting up and down erraticly throughout the day. It still increases rapidly when I suffer from a POTS attack though. A few other things are supposed to help ease the symptoms, such as drinking plenty of water and increasing salt intake. So I'm often pottering around the house with bottled water in my hand.

                                 Life as a Postural Orthostatic Tachycardia Syndrome Patient
By Lucy Atkinson..x 

Musculoskeletal Therapist Update..

I don't know if I'll be able to finish this whole blog in one go as I'm in pain with my spinal hardware right now and all my joints have flared up, but I want to make a start now whilst today's appointment is still fresh in my mind. I'm typing this up on my phone from the comfort of my bed which is where I've been all evening after spending too long at the hospital. I went to see a Muscluskeletal Therapist who I was referred to earlier this year. He started by taking notes of all my health conditions and the symptoms, which took almost an hour in itself and then I had to have a physical examination. So that’s why I was there for so long. His job was to work out which route to go down in terms of exercise based on what I can and can’t do. I told him all about my limitations due to my spinal hardware and the protrusion of some screws, and I explained the problems I have with my joints due to my connective tissue disorder, Marfan syndrome.

Image found on Pixels.

Whilst he was assessing my joints and range of movement, I began to suffer from a POTS (Postural Orthostatic Tachycardia Syndrome) attack which is a secondary condition of Marfan syndrome and causes palpitations and dizziness. It was brought on by so much moving around during my physical examination so we had to bring things to a halt whilst I sat on the edge of the bed waiting for it to pass. I knew it was going to happen because I could feel myself getting lightheaded and sweaty, but I stupidly carried on and tried to persevere with the therapists assessment so that I didn’t have to disappoint him by saying no, when in actual fact I should’ve just said “sorry, my body isn’t capable of doing this much.“ Instead I pushed myself and ended up over doing it but luckily the Musculoskeletal Therapist understood and we gave up on the examination for the time being.

I sat in a chair next to my Mum (who was with me) to fully recover and the therapist talked to us about Pacing oneself, which was very fitting given what I had just done. He said he thought it might be wise to teach me how to practice Pacing at my next appointment. I know a little bit about Pacing but I have never properly practiced it. So I'm looking forward to that. He went on to tell me that my joint problems and chronic pain can't be cured because unfortunately Connective Tissue isn’t fixable, but if it’s done in the right way, we could try building up muscle strength which may enable me to cope with everything better. Apparently if the muscles are strong, my body might not feel as much pain. I don’t know if there’s any truth in that but I’ve agreed to give it a try. So once he’s taught me the rules of pacing, we’ll move on to building up muscle strength and see how that goes. I don’t know if it’ll go the way it’s supposed to, but one thing I do know is that I have a long journey ahead with the musculoskeletal therapist.