I am sure, a lot of you would recognise similar events and/or difficulties in your life and this would not be surprising. People with Marfan's, usually struggle with problems that any other person would have, the difference is that all of these problems, combined with a Connective Tissue Disorder such as Marfan's, compounds and stack up to complicate things and turn "Benign" conditions into more serious and sometimes life-threatening situations.
As a todler, my mom struggled with me. I never wanted to walk and she always ended up carrying me everywhere. They toke me to doctors who told her that there is nothing wrong with me and that I was perfectly able to walk. Then, started the misery years. I would wake up in the middle of the night with excruciating leg pains. The obvious conclusion that most doctors made, was that it was merely "Growth Pains" and that it would eventually go away with time. Now, I don't know what growth pains is supposed to feel like, but I can gaurentee you that this was way beyond normal growth pains. At times, it would feel like my legs was being pulled taught like a bow and at times, it would get so bad that it felt as if tho either the legiments was going to tear out of my legs or my leg-bones was going to break. These pains would continue on relentlessly through the night. No pain killer, no salve, nothing on this planet seemed capable of suppressing the pain.
This carried on from age 5/6 right through to about 13/14. At some point, my parents toke me back to the doctors and one of them somehow came to the conclusion that my leg pains was associated with my sugar in take... I won't rule out the fact that I might have some sort of sugar-related illness, but, it might have just been that the growth rate was equal to my sugar intake and therefor less suger meant less growth? I don't know. Suffice it to say, that untill I was about 16, I was never allowed to eat any suger products. That was funny, seeing as I was already as scrawny as heck and waitresses would look at me with a frown when I would order "Diet Coke" or buy the diet variety chocolate.
When I started primary school, apart from serious seperation anxiety, I was always the one who could not keep up with the rest of the students when it came to track and field. I would run my heart out but to no avail. I would either brake down crying from being out of breath and with serious cramps or end up with my legs just stop working half way through. My legs would just turn to jelly and refuse to carry me along at a rapid pace. I can even remember "friends" making a game out of running away from me during break. I had never really done any active sports because of these issues, that is untill a later stage of my life.
Now, through out my life, my family had been fighting with me and accusing me of being lazy and self-centered because whenever we went shopping or on outings, I would start complaining and whining a quarter of the way through. It is kind of weird thinking back on it... You as a child, have no real concept of what is supposed to be "normal"... even with something like pain ... especially when doctors keep on telling your parents that there is nothing wrong with you and that you are just plain spoilt.
My grandma was watching us for about two weeks while my mom and dad was in America on a company trip. After the first night, she was livid! She eventually grabbed a tub of ointment...god knows what it was but I think at that point she was frantic for something just to make this child shut up so she could get some sleep. She rubbed the ointment onto my legs vigourously, stating that it was some sort of "special" cream that would take away the leg pains. Ughm... I might have been a child but at that point, I was smart enough to realise what she was doing, so for the remainder of the two weeks, I just clamped a blanket between my teeth and held it in.
When I was 14, st 6, I was running along when I had this immense pain in my right knee. The pain overshadowed everything and I could not even remember falling down. When my senses returned, I was lying on the floor, clutching my leg. I was nautious, sweating and my whole right leg was numb. Slowly but surely, the feeling started to return, along with a dull pulsing pain. I dragged my self to the wall of the house and creeped up it's side and started hopping to the kitchen.
I explained the event to my mom and the next day we went to a specialist. The specialist found it "interesting" and stated that he didn't want to do anything just yet as I am still growing but once I was 21 and the problem persisted, he would most likely operate to shorten my legiments. I don't think he particularly understood what was going on with my knee or my parents didn't understand what he told them. Also, this is the same specialist, that years later, performed surgery on my sisters toes to straighten them... The operation caused her severe pain and never toke as her toes is back to what it was.
The other day, I happened across a site stating that people with a connective tissue disorder, should avoid surgery on their feet at all costs...nice... My sister can't be classified as having Marfan's as she doesn't have enough of the "indicators", however, before she had the surgery done, she stopped in the middle of shopping, looked at my mom and asked her "Does one's feet always burn when you walk long distances or does it eventually stop?". This was a while after my initial dislocation when my mom finally realised that there was more to my walking difficulties than just plain stubbornnes and she looked around at me, trailing as usual, and her jaw dropped. "Eurika!" I thought, they finally get it... or did they ...
I'm not going to go into a pitty song over my friendship problems during my school years, so suffice it to say that I eventually ended up with the "Down & Out" crowd...the ones no one wanted to sit with. I became quite good friends with the one guy and he was involved in the school "Tenniquoits" team, which is basically tennis with a rubber ring that you through, instead of a racket and ball. When I was in Std 8, I also became interested in it and became a part of the school team. For the first year, things went great and me and my friend had dreams of getting individual and double's national colors.
At the biginning of the first semester of my Std 9 year, we were competing in a huge competition between various clubs. I wasn't doing too badly in the singles and me and my friend were getting close to winning in our class in the doubles. Then, disaster struck. As I went forward towards the net, to catch the ring, my left knee buckled. The pain was excruciating and the only way I can describe it, is as a "white hot" pain followed by my body just cutting out the pain and all you feel is total numbness. The whole court went quite and the teachers were drummed around me trying to get me to tell them what was wrong, but I couldn't speak a word. Eventually, once the initial shock passed, I was able to explain to them what had happened.
I basically missed the whole first semester of school. My knee was constantly swollen and every single movement would reverbirate through the fluid inside of it sending excruciating pain through it. Every so often I would have to go to the doctors to get the fluid drained and a lot of the time, it would turn out to be blood mixed in with the fluid. Initially my doctor thaught it would be a very simple case and all that was needed was rest. As time went on, my doctor started to become more and more quite untill he eventually mumbled something about not wanting to perform surgery as I haven't stopped growing yet.
I eventually had to drop out of school as there was no way that I was going catch up for the lost time. I enrolled in Technical College to complete my Std 9 & Senior years, most of which was done on a pair of crutches and throughout the years, I have gone through a ritual of dislocation, doctor visits, threats of surgery and carrying on untill eventually, the recovery time went down to two weeks instead of months and I became used to the pain of dislocations. I became so used to the dislocations, that when people rushed to help me, I would tell them to give me a couple of seconds and limp away once I had feeling in my leg again, leaving them with confused looks on their faces.
One of the reasons why I never had surgery done when I turned 21, was due to the failure my sister had experienced with her surgery on her toes. The other, being my family who was dead set against it ... more than likely for the same reasons ...
All was fine and dandy, between the dislocations and the anti-inflammatories and the pain-pills, that is, untill Feb 2007. As I stood up, I realised I couldn't straighten my left leg. Every time I got to a certain point, I would get an excruciating pain shooting through my knee. Eventually, I had my parents take me to the hospital. I have never had a dislocation like this before but apparently, due to all the prior dislocations, my legiments now has a sort of "Button Hole" scar on them and my knee cap had slipped off of it's position and had gotten stuck on the scar? I still don't quite know how it worked. No matter how hard I tried, I just could not relax my muscles enough for the doctor to reduce my knee, so off to surgery we went...
The first thing I remembered when waking up, was the heavy feeling of the cast, then the nurse asking me if I had a nice nap ... apparently they struggled to get me to wake up from the anheasthetic and was quite frantic at one point ... Then, the doctor comes along, lifts up my hand and comments about my long fingers. After that, I basically fell back into sleep. When I woke up the second time, the doctor spoke to me but I could hardly understand him through the haze. All I could remember is him saying that I had Osgood Schlatter's as a kid (I remember that cus it sounded like "Slaughter") and that he suspected Marfan's (That I remembered because it sounded like "Marshans") and most likely would want to operate on my knees once the cast came off. While in hospital, they had me on constant morphine drips or I would constantly be ringing the nurses complaining of the pain (I was so out of it, I could hardly remember doing that).
Eventually, before they released me, they sent me for X-Rays to see if the knee is properly reduced. After the X-Rays, the nurse that had taken them, comes up to me and asks "So when are they going to reduce your knee?". I looked at her, frowned and said..."It is allready done". She came back several times asking if I was sure. I would later find out the reason for her confusion...
They released me from the hospital and I spent the following two months in the cast, wheeling my self around in a wheelchair. The cast was just too darned heavy to walk on crutches and would sit painfully on my knee.
When the day came that the cast was going to come off, I was soo excited, I could hardly wait. Sitting around for two months is no fun...wether you have buggered knees or not. The doctor started cutting open the cast, very excited to see his handy work but when the cast came off, he fell silent. He told me not to even try to bend it until I have seen a physio. He looked striaght at me and told me that he would not be performing surgery and that he has no idea as to how to help me. He continued to explain that I have what is called Bilatteral Patella Alta with Chondromalatial Osteoarthritis. What this means, is that both my kneecaps, sits too high up, causing extreme pain when pressure is applied due to the mal-allignment and causing degeneration of the joint. This is why the X-Ray nurse was so confused. On the X-Ray, it looks like my knee is totally out of whack.
It toke me another two weeks to regain use of my leg and knee. At times during this whole process, it was a very real possibility that I could lose the use of my knee completely. The same reason why the specialist didn't want to perform surgery. There is an 80% chance, that I would end up with a rigid knee.
So that is where I am at in terms of my knees. Untill such time as my knees are totally useless, they will not operate...the moment they are useless, I won't be able to walk properly and they will most likely have to replace the cartlidge in my knee's, my knee-cap and who know's what else. Every time I walk, I run the risk of a dislocation. If I step in a ditch, receive a knock against my leg or even just twist my ankle, I WILL dislocate my knee. It is not a matter of I might...I Will and everytime I dislocate my knee, I get closer to total joint failure.
Chondromalatial degeneration can be slowed but it can not be reversed. I don't know what the future of my knees are. One way to prevent further degeneration, is to go to a Biokineticks sentrum. The problem is, that as I mentioned before, I can't ride an exercise bike or run or even walk on a treadmill as it will cause more wear on my knees. The other factor is Osteoarthritis in my other joints occuring more and more, due to the hypermobile/lax nature of my joints. I can't even do weight exercises with my legs, as I am not allowed to exert my self over and above 10kg's as the increased blood pressure combined with the two regurgetating & prolapsing heart-valve's, will cause my aorta to rupture. So now, I am thinking of looking into swimming as an exercise.
But, we all have our problems, trials and tribulations. It doesn't help crying about spilt milk...especially not if the milk was destined to spill no matter what you do. I can still walk and for that I am thankfull...