Introduction

Introduction: Life pointed at me

I wasn’t even sure I should go that day. I had decided that there would be nothing that could be done for me. I sat nervously in the Conologist’s waiting room at Poole General Hospital at 9.00am on an Autumn Monday morning. I hadn’t spoken to a specialist about my unique bowel condition and the incontinence I have endured, since I was last seen as an outpatient at Great Ormond Street Hospital, London, when I was ten years old. That was over thirty five years ago.

The lovely, cheery, bouncy nurse called my name and I duly followed her through to an anti-room adjourning the room where the Consultant lay in wait with the door to his empire firmly closed.

‘Please take a seat’. The nurse gestured for me to sit in the corner next to a freshly prepared NHS starched white bed, obviously prepared for a victim to have their bottom examined. I swallowed hard. That won’t be me today! I would make sure of it. Today I wanted to speak to someone who could give me answers, tell me what my options were. Advise me how I could make my life more manageable and reassure me that I was doing all the right things. However, life often gives you want you want in a way you hadn’t envisaged. Always though life points at you, laughs with you and makes you ultimately responsible for choosing our own path forward.

The nurse turned to me and handed me a white form and a brown envelope.

‘Would you mind filling this questionnaire in after you have seen the Consultant please? Then hand it over to reception.’

‘Yes, of course I will’. I smiled willingly.

The nurse left me with the questionnaire and hurried off through the closed door. I heard her say to the Consultant, ‘Mrs Hunt is ready now boss!’ Humour and sarcasm mixed in for good measure. I liked that though, if she can tease this man he must be flexible and approachable I thought. Meanwhile, I sat in the chair clutching the white form and hoping to God that the Consultant wouldn’t keep me long, as my bowel was doing so well, but I couldn’t hold on forever and if I got more nervous I knew I couldn’t sit there I would have to dash off to the nearest toilet. I always assume that they won’t be close enough.

To refocus my thoughts away from my uncomfortable difficulty I started to read the questions on the sheet. The title of the questionnaire was ‘St Mark’s Incontinence Score’. It was obviously a questionnaire to gage how my incontinence was affecting my life. For each question I was asked to indicate how much of the time the issue was a concern for me due to accidental bowel leakage.

1 = Most of the time   2= Some of the time  3= A little of the time   4= None of the time

As I read down the list of the 28 or so questions I started to smile.

1a. I am afraid to go out……

The smile turned into a snigger

1.b. I avoid visiting friends

The snigger turned inwards to a sarcastic laugh

1.c. I avoid staying overnight away from home

At that point anger spilt over. The questions I read were so relevant to my life but no one had ever asked me about them before. I had been born with a hidden syndrome which had affected me every waking and sleeping moment, but I had never had any help. I hadn’t even fully understood I had coped with life up until the point of reading these questions. The questions made me realise how much I had had to deal with, and how I had found my own way of turning these situations and feelings around in order not only to survive but to thrive. My anger came from the feeling that the medical world had actually abandoned me.

In 1968 the Paediatric specialists had only just perfected the operation to save my life. If I had been born 10 years earlier I would not have survived. So it was no wonder they didn’t know how to help or support me or my long-suffering parents, or advise us what to expect. About 20 years ago the medical world did begin to understand this condition, and the other potential physical abnormalities that were hidden, waiting to be found. By then though, I was well out of the Great Ormond Street radar, being a fully-fledged adult. An adult who could survive and adapt to situations rather than making a fuss and asking for help from the NHS.

I have had to live with incontinence all of my life due to being born with Imperfornate Anus (IA for short) and I have had to find my own way of dealing with embarrassment, bullying and anxiety. It was discovered that I had one kidney, two wombs, two vaginas, absent coccyx, extra clavicle rib, and a tethered spinal cord. Through this story I hope to bring awareness of the emotional and physical difficulties people with incontinence have on a day to day basis. I believe that incontinence disabilities are akin to many others hidden disabilities and I would like to think that my personal story will help and inspire people who suffer from any form of disability, hidden or otherwise, so they can stop the internal fight trying to be normal, make peace with their differences, and tap into who they really are both physically and spiritually.

My physical and emotional issues have mostly been caused because I was born with the IA. However other people with IA can be born with more complicated syndromes and associations. I consider myself as one of the fortunate people. As an Emotional Therapist and CBT therapist I have travelled with people through many different emotional traumas and situations. Those who were successful at making the changes necessary to heal themselves needed to accept their situations in their entirety and end the power struggle between what they wanted and what actually is. This power struggle is not unique to emotional upset but to every part of our lives. When we are faced with life struggles and physical disability we tend immediately to become fearful and angry. Our anger comes from feeling like a ‘victim’. A victim of circumstance: of our bodies, mind, society, and life! My own personal power struggle with my bowel has resulted in me struggling with extreme anxiety, embarrassment and low self-esteem most of my life.  I know that a person does not have to have a physical disability in order feel this way. It is a natural state of being and it is not an ideal way to live but we can live with it rather than fight against it. I am not suggesting that we all ‘give in’ to our situations and weaknesses, but rather look at the painful, dark parts of our lives and release the need to change or control that part of us, make room for it as it were and learn to accept the situation is as it is for now. By releasing that need to control or change it we learn to trust that we can actually cope and live with this feeling/situation. In essence we are learning to trust ourselves and the organic process of life itself. In my experience, those people who are ready to make changes to their lives have no more energy left to invest in their internal power struggle. The way we think about our difficulties does not work with us but against us. While we struggle instead of shaking hands with our internal issues and the only way out of this situation is to go through it, trusting in something other than ourselves, thus releasing control, accepting and then looking at our choices.

None of our struggles are unique, but the way that we deal with them is. What works for one person may not work for another. When you are faced with fear, anger and panic you become locked into a sort of trance state which makes you lose all connection with everyone and everything you ever trusted: doctors, friends, family, yourself and with any deity or spiritual connection to.

Back to the questions in hand……..

I worry about not being able to get to the toilet in time…..

Tears welled up in place of the anger. Finally, someone will know about my emotional and physical difficulties, someone will be able to give me some support. There was a knock at the door and in walked the Consultant. A tall kindly man shook my hand with gentleness and respect.

‘Mrs Hunt, it is so good to meet you. Thank you so much for sending me the medical notes from Great Ormond Street and for your letter explaining what is happening for you right now. First, I would like to say what a brave and courageous lady you are. It is so apparent that life has dealt you a bad hand from birth, but you have obviously have been able to overcome these difficulties and turn them into a positive rather than letting them overwhelm you and drag you down.’

His heart-felt words shocked me to the core! I think I must have stared at him like a deer caught in the headlights of an oncoming car. I never thought a medical person would ever think of me in this way let alone talk to me in such a caring and empathetic manner. In the past I have seen Gynaecologists, Neurologists, Cardiologists, GPs, Chiropractors but no one had ever spoken to me as if I actually matter, or that they had an ounce of understanding of my condition and what I have been through before. This gentleman was quite extraordinary!

‘Thank you,’ was all I could say to him, still stunned.

‘I am not sure what I can do for you though. You are so unique we are very much in the dark about your condition.’ His expression took on a more serious tone. ‘I was hoping that you might be eligible for a device to be fitted into your rectum which is like a magnetic ring which would work like an anus with muscles, which you have little or none of. However, if you were to have this ring fitted you would not be able to have another MRI scan, which you say you might need in the future to see if your spinal cord had re-tethered or if there was more tethering. We need to look more closely to your internal muscle structure to see if this is possible. When your bowel makes your life unbearable - or more unbearable that you can’t stand it anymore - we might have to fit you with a colostomy, but I am not threating you with that, it is just another option down the line, when you are much older.’

‘In the dark’, or a ‘dark horse’ is something I have been told time and time again about my genetic condition. It didn’t surprise me to hear it once again.

‘Well I don’t’ have the answers after 45 years, so I didn’t expect magic solutions. I consoled him. ‘I know I can cope with many things. If you had given me this questionnaire when I was 10, 20 or even when I was 30 years old I think you may have been able to help me in more ways than just offering me an operation. However, I believe I have found a way to overcome most if not all of the issues underlying these questions.’

The consultant smiled knowingly. ‘You are really a brave lady, and you’ve been through so much. You have such resilience.’ I took this opportunity to tell him how I really felt. I shook his hand firmly and warmly.

‘Thank you so much for your kind words. You do not know what they mean to me. I feel I am not alone anymore with all this.’

His smile was warm and generous and after confirming that I would be getting an appointment for a further test and that we would meet again he disappeared behind the door once more, now more a haven of light than a secret empire!

I floated out to the waiting room where my lovely husband eagerly greeted me with concern in his eyes.

Instead of answering the questionnaire in the hospital I asked if I could take it home so that I could study it in earnest. I began to realise that I could not just tick one box to answer the questions in full. How can a tick represent my life experiences? I became aware of exactly how much anxiety, stress and victimisation I have endured in my life time. More importantly, I realised that I have managed these situations and mostly overcome them. On close inspection I began to realise that not only do the questions apply just to me and my disability, but they can also apply to many other disabilities and even to anyone with emotional issues. Thanks to my years of working as a counsellor I realised that the questions being asked could relate to any physical disability or mental/emotional health issue, with a little tweaking of the odd word or two.  It became clear to me that by answering the questions in such a detailed way it might be of interest to other people who have, or who care for, people with incontinence issues: people who are struggling to cope with changes to their physical and emotional lives. I also hope that the parents of children who are born with physical difficulties may find the answers interesting and discover an insight as to what it is like to accept and manage their physical difficulties and ultimately support their children’s emotional growth.

Each chapter deals with a different question. When addressing each question, I relate back to how the particular issue has affected me throughout my life: I have also compiled the psychological tools that I have gleaned from workshops, courses, reading books and working with clients, but mainly from my own spiritual development and inner knowing. Not all the tools I have used will connect with you and your particular issue. We all hide behind the smile but from what I have experienced as a counsellor everyone who has ever struggled with emotional issues (and who do you know that hasn’t?) knows what it is like to feel embarrassed, fearful, angry and shameful. The degree of feeling might be different from one individual to another. We are not alone with our struggles but if you believe no one will ever understand you then you do stand alone, a victim whom no one can rescue. Your story counts and it is one which is echoed across the world, because we can all relate to it to some degree. We are not our bodies; we are not what we have allowed life to make us!

I was born in 1968 at the time the doctors could only see that there was no opening to the bowel, and that I had only one kidney. The full spectrum of my abnormalities was not diagnosed until later in life. I have looked for a label for my disabilities since I reached the age of 23. Mum and Dad did not seem to worry about ‘what’ condition I had I think they were more interested in me living a ‘normal’ life as much as possible. It is also possible that as I had been discharged from Great Ormond Street as they thought nothing more could be done for me. When I asked the Geneticist to investigate my condition in the 1990’s I was told that I had Hand Foot Uterus Syndrome which would explain my two wombs, vaginas, and finger like thumbs. However, they couldn’t find the chromosome responsible for this condition within me, so I was told that I was unique. In fact, there was no one with the same abnormalities as me in the whole of Europe.

I was strangely satisfied with this outcome. I began to think of myself as an ‘alien’ and that my parents were right all along, that I was a ‘special’ person. With the coming off Facebook I discovered, at 40, that there were other people in the world who suffered from part of my condition at least! It is through the IA Facebook community that I was introduced to something called the VACTERL association. It is a disorder that affects many body systems. VACTERL stands for vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. As far as I was concerned I certainly ticked at least four of these criteria. I also befriended an amazing lady called Sammie Hunt who has been classified as having VACTERL and who was born with even more severe abnormalities than me. However, after a recent visit to the Geneticist where I thought I would finally be classified as having VACTERL and finding a label, home and community where I belong, they have decided that I do not sit comfortably into this Association either. As I write, I am being tested for Townes Block Syndrome which they believe could describe me better. I have to point out that VACTERL is not hereditary. However, Townes Block Syndrome is and it may have consequences for my daughter and my nieces. Only 1 in 200,000 are born with Townes Block and there only 200 confirmed cases to date. I don’t care now about having a label. The label is for the medical world, and to make prognoses about my physical future more accurate. A label does not say who I really am, for I am not the sum of my body. But I do care about the genetics of my condition and whether it can be passed down to other generations.

In 1968 and since, all medical journals and professionals state that, as a child, depending on the severity of your condition, once you have had all your operations, you should be able to achieve a 'normal life' without  further consultation. Unfortunately, this is not the reality. What I want to explore in this book is the real story of living with this condition and I want to try to influence and change the medical opinions and those of society in general, through my own  real life experiences.