Hi, my name is Aaryanna.
first of all I’d like to thank the MPS-society for the nice invitation to give a lecture. I feel so honoured to be standing here in front of you and I am grateful for this opportunity. I am going to talk about health, well-being and quality of life after having been diagnosed with MPS I (Hurler Scheie).
When I was diagnosed in 2006, I was four years old. Three months later, I started the enzyme replacement therapy in the hospital. It was Jean Mercer, who gave me my first infusion. 11 years later I am in home therapy and haven’t missed more than three infusions in a whole year. That means that I administered 588 infusions and many needle sticks.
Recently, I have often thought about health, well-being and quality of life more than usual. I am still recovering from the decompression surgery on my spine, which was done in April. Just a few days ago, I was allowed to take off my neck brace and it was only last Thursday that I – after three months – went back to school. Seen in this light the conference is a little “coming out party” for my neck.
While I was recovering at home, I was going through any kind of feelings, thoughts and emotions you can possibly imagine. In these days, my thoughts and emotions were overwhelmingly negative. I was mentally and physically exhausted from the operation and I was literally trapped at home for 12 weeks. Pain, frustration, and the loss of my independency together with the claustrophobic imprisonment in a neck brace for 24 hours a day, were sometimes simply too much to cope. Struggling and fighting, that’s what it was.
Since the day I was diagnosed with MPS, my parents have been motivating me to live a positive life and keep a positive composure. However, to be honest, the last three months were the toughest in my life. Away from school, away from my friends and life – my mother had to do almost everything for me. Sometimes it was difficult to enter my “bank of positive way of life”. My account was definitely locked for a while. But I was happy and relieved that the operation was even possible and as successful as it was it could solve a problem.
The definition of quality of life is the standard of health, comfort and happiness as being experienced by the individual.
Everyone in this room who lives with MPS knows that these levels of health, comfort and happiness keep changing. It often is a question of minutes, hours, one day, one week, one month or year whether they rise or fall – steadily in synch with the stress caused by the one and single aspect of our lives: the permanent visits to doctors and care centres, tests, test results, bone marrow transplantations, operations, weekly enzyme replacement therapies … the list is endless.
Besides, I haven’t even mentioned being overloaded by stress which, caused by different symptoms, pains and personal needs, is a constant companion and forces us to bring life with MPS and reality face to face.
For many of us this burden is additionally reinforced by the fear based on the permanent statements to reduce or cancel the essential financial resources for rare or extremely rare diseases, meaning the important funding of the medication, which is necessary to be able to live at all! And I`m not even talking about quality of life!
MPS itself is relentless in depriving each one of us of the quality of living. And this is not only about the patients’ quality of living. No, MPS is very generous. It extends its range to each single member of the family – parents, siblings, grandparents… nobody in the family is untouched by MPS. It is a constant fight.
The connection between health, well – being and life quality is strange. At first sight they exist independently of each other. Yet, it is a fact that they are interconnected. The Ministry of Health notes that there is a two-way relationship between health and well-being:
● health – both physically and mentally – influences well-being
● well-being influences health
● both are very individual and personalized
●they are driven by what we are and by the cards we hold in our hands
●we can’t control these cards
●with these cards we start the game of life and it’s up to us whether we win or lose. Seeing, accepting and working with the cards that are available for us is essential – if we want to win.
And I want to win. I want to win a lot. I want to be able to grab life with both hands forming it as I please. I want to live as if I had no physical limitation. I want to live as If I had no MPS – and that’s the difficult part. This last bit. I can do everything. But this last bit is exactly what I can`t do. That’s the bit I have to accept.
Thanks to MPS, the list of things I can’t do or are physically difficult for me to do is quite long. I can’t run without being completely exhausted. Roller-skating, ice-skating, mountain climbing, ballet dancing or running the cross country with my school. To be honest, not being able to run doesn’t feel that bad – especially when I see my friends having to run on the sports ground in any weather.
I can change none of these things. It is as it is. These are the cards I received.
But it’s only human to long for things we know we can’t have, things that are out of reach.
It’s like a child with a toy that wants to play with just the one being used by another child.
But unlike the child with the toy, those born with MPS can’t grow out that easily from this desire. In today’s fast moving modern world we are spinning on a digital axis driven by technological applications like twitter, snapchat, Facebook and Instagram that serves as a source of energy.
It really is a small world we live in and we are all so close to each other. We have digital access to everything, but what we do want is a real one. MPS patients have just as much right to this as those without MPS.
It’s difficult to accept our limitations. It’s difficult to stay positive. It’s difficult to stop longing for things we can’t have or are out of reach. But that’s exactly what we have to do, if we, at the same time, want to get the most out of the things we are able to do. We must reach out for any opportunity to make our dreams come true. In other words: fight for our quality of life.
If we keep on fighting against the cards we have been dealt, we risk the craving for the things that cannot be done to grow and influence our subjective experience towards ourselves. And that can endanger our wellbeing and have disastrous consequences for our health.
I am a teenager and I am like any other teenager. I want to do the same things, just the usual teenage stuff. I enjoy life just as well. But I have MPS and I know this fight like all my MPS friends. We must be encouraged by a strong and powerful force, an inner force. Fearless, ambitious, focused, challenging and loud it is and doesn’t sit still on a chair in the corner. We have to march like an army through negativity. Only then we can win the war and be rewarded with a limitless positive attitude, with which we are able to achieve anything.
I have very often talked with my parents about the stage of my life – their lives at which I was diagnosed. I was only 4 years old. My mom and dad filled all the gaps for me.
Now I know that in these early, terrible days they tried very hard not only to accept my diagnosis but also to become a MPS expert over night. They wanted to gain a perfect understanding for the disease and its effects on the body.
My parents knew that knowledge was power. The MPS society supported them wonderfully and provided them with information so that they really became experts for MPS IS, Morbus Hurler Scheie, over night. The team of the MPS society became the heroes of the hour. They sent numerous flyers, brochures and information to my parents, who sucked in any of it thankfully.
Due to social media, online discussion groups, forums and websites – including our own fantastic homepage – today newly diagnosed families have more options to inform themselves and get in contact with other affected ones all over the world. That really is wonderful. MPS families are no longer alone during and after the diagnosis. They can take this journey together. Mutual information, support and exchange of experience – that’s the 21st century.
I received my very first infusion on 3 July 2006. That was 11 years ago. At this time my parents promised me to do everything possible to help me unlock my full potential in order to be able to live an active life – WITH MPS. They taught me to take responsibility for my life.
By then they were very clear on the size of the medical barriers they had to face and swore to raise me in a way that would guarantee full control over my life. Because only then would I be able to control MPS.
Life is what you make of it
I am not a health expert, though I know good physical health doesn’t guarantee well – being and vice versa. I drew my cards, just like each of you did. And I swore to myself without respect for medical barriers, challenges and setbacks – I am sure there are still many more of them to come – that I am neither going to stop rising after every single setback nor looking ahead, moving on.
Life is what you make out of it. It’s not my intention to let MPS take control of my life – for MPS is not my life, it doesn’t define me.
In 2006 my parents made peace with MPS. They embraced MPS, welcomed it in their home and set a place for it at the table. They thought that if we as family were not able to accept MPS, we would not be able to live with it, either. They thought that being under control and power of MPS, is anything else but life. What kind of life would that be?
That’s why I never say I suffer from MPS. Since my day of birth I keep saying I live with MPS. MPS brings great sufferings, which we all know. To say I would suffer from it would be nothing more but a double negative energy and would not be helpful. If we want to accept MPS, we do need an abundance of positive attitude. Not only to accept the disease, but also to accept ourselves.
But please do not get me wrong. You can`t be happy and positive any time and anywhere. It can be terribly difficult. But nothing comes from nothing. The main thing is not to lose sight of the objective goal, if we want to win this game.
Our health may be predetermined, but our well- being is certainly self-determined. Our quality of life depends on it. For a high quality of life we must love our life. We more or less have to love MPS. But above all this, we must love ourselves.
It’s not just me who thinks this way. Fact is that many in our big MPS family share my view. Just like me they take life into their own hands and form it. They share the same passion for life and just like me fight for their quality of life. They want to be part of this speech – that’s why I ask you to join me in congratulating the members of our big MPS family who want to share this message with you now.
Thank you Aaryana L (speech)
Editor`s comment: Aaryana made contact with many other MPS teenagers via facebook and other media to ask for a picture, which showed them with the message: I love my life.