Living With Neurofibromatosis (Nf)

The following are quotes from a series of interviews conducted in the United States by Joan Ablon. They are published in a book entitled 'Living with Neurofibromatosis' and subtitled: 'The impact of Neurofibromatosis 1'.

These interviews give a very good idea of how people who are affected by a disabling and disfiguring condition cope with everyday life, their families, their friends, their school, work and their future. While Nf is a complicated disorder that can affect every part of the body both internally and externally, I am sure many of the sentiments expressed also relate to others who have a disability.

We are grateful to the publishers, Greenwood Publishing, for permission to reproduce this work by Joan Ablon.

Jim

"When I was in the fifth grade in school, my brother started to say 'Get lost. I don't want you around.' He would do his homework in the bedroom and close the door. He wouldn't let me study in there. I had to study in the kitchen with the TV blaring in the living room. I had all these learning problems and then I got distracted by the TV all the time. I'm now and adult and he still doesn't talk to me. He still says 'Oh, get lost.'. That's how nasty he is."

Terry

"My sister is beautiful, intelligent and witty. I mean she's wonderful, she's a great person. We are starting to get close. It was really hard for her growing up because I was the different one and I was always made to feel defective and she had to be extra perfect to cover for that. Since I was the defective one, I was always set up to fail. But she had to be perfect. She had no choice. She was made to be Daddy's perfect daughter because I was Daddy's defective daughter. It has been a lot of years but we are finally starting to get a kind of sisterly relationship."

Lou

"My sister, she's Oh, how can I put it? The way she presents herself is appealing, attractive. She dresses well, she has nice posture and she gets stared at a lot by guys. She said she knows that sometimes she tries to block it out because it makes her feel uncomfortable. How could that make you feel uncomfortable, or make you feel weird? To me, I think that should make you feel good. How would you like to be just the opposite? To have someone making fun of you or looking at you because you have a disfigurement like me.

"A few weeks ago we went to the grocery store and there's this guy who kept staring at her. Once she even met a guy when they were both driving down the freeway. They pulled over and talked and she went out with him the next day. She could meet somebody just walking to work. She doesn't have any problems meeting men.

"That's why I don't like to go out with her. There's so many guys looking at her that it makes me upset sometimes. Also she never knows what she wants to do. I said 'you are one of those people that are never satisfied.' To me a person that can never be satisfied can never be happy in their life. If she had to go through what I went through she could never do it. I think she'd commit suicide."

Cecelia

"I know it's not very nice but I've resented and hated my mother for a lot of years for giving Nf to me. I looked at my mother and I couldn't help but see this ugly woman, this disfigured ugly woman and I wished she didn't have it because everybody else's mother was normal looking. When people said 'This is my mum' I used to feel a little bit of embarrassment to say 'This is my mum' because I didn't want anyone making any comments or asking any questions.

"I have learned that it's not her fault. I can't really blame her, it's not like she deliberately chose to give Nf to me. I've been told that I will not necessarily have it as severely as my mother has. I used to think that would happen and it used to make me upset. I used to be convinced that I was going to be exactly like her. I mean we look alike - the same height, basically the same weight. Everything is the same about us, the same fat fingers. I've always thought 'Oh no, if I have everything else like my mother, I must really have her genes and I'm going to be as severely affected.' But now I know that is not true.

"When I was younger I did not think whether Nf affected my mother's life. I have to admit I was selfish and only thought of how it affected me. I resented her for giving it to me. But I couldn't help it. When I did go to therapy over these past nine years, I've talked about it a lot. All I thought of was me - how it affected me. I wasn't bright enough or sensitive enough to think of how it affected her.

"My mother never talked about her Nf but, when I was in the dating age, she used to say thinks like 'Look, your father loves me. When a man loves you, they love you for what's inside.' So she never really talked about it. Once when I was covering up a scar, she says to me 'Why would you do that?' I mean, I think it's pretty obvious why you would want to cover up a scar with make-up. So she would say things that led me to believe that Nf didn't bother her."

Harry

"I guess I can thank my mother for my attitudes. My mother had a lot of pain with her Nf. She walked on two crutches all over the country before she went into a wheelchair. I was brought up the same way. You see, if you can't do it one way, you can do it another way. My mum helped me to know handicapped people. I've been around handicapped people all my life. There's an old saying I grew up with and I've tried to live by it 'I felt sorry I had no shoes until I saw a man who had no feet.' I think, 'How would I feel if I were in that person's shoes?' There's always somebody worse. Just deal with people the way they are, accept it for what they are, not for what they look like or because they are missing a few things."

Lee

"You know, I've been thinking about this lately. Since my children are having to deal with the problems of Nf, I should try to deal with it better myself even though I think I am on top of it. I know my children are very angry. Well, I guess I am too but maybe I'm a different type of person. When I ask my son about it, he says he's angry at grandma - who was also affected - and I say 'You shouldn't be angry at grandma, if anybody you should be angry with me.' They won't discuss it with me and they never would go to a support group meeting with me.

"At least I am admitting to myself that I'm angry about something, even if I haven't really found out or admitted what I'm angry about. I don't think I'm angry at my mother. I am angry at myself for bringing my children into the world with this condition. I feel bad about that, guilty. And sometimes I probably must feel 'Why me?' That's a really hard feeling. My children feel that.

"Carl's very angry. He's hit me and even threatened to kill me. He said that his brother and he had made a pact, that if they got too bad they were going to kill themselves. Now, as to that, there's nothing I can do about it, if I can't get them to go to a counsellor to try to become accepting and be able to live with it.

"I am trying to push them into knowing more about it just so they have the knowledge. I've told them, 'You have to get genetic counselling. You can't have children unless you really know what you are doing'. At least they have that knowledge that I didn't have. Maybe if I had been a little bit more intelligent, had gone out and sought the knowledge . but it's too late for that now as it's done.'"

Rachel

"It was very, very difficult growing up. And energy levels were always low. Socially I was a pariah in school. I mean I never had dates. I never had a lot of friends. I was a pretty lonely kid. And it didn't matter what good grades you got. Good grades don't mean anything when you eat lunch alone.

"I felt very, very isolated from my peers. I missed lots of school because of the surgeries. And, when I finally returned to school, I didn't know anything. I mean, I knew the answers to the questions but I didn't share in the little secrets. I didn't know what was happening in the school. I wasn't socially adapted. I had a hard time making friends because you make friends and then you go back to hospital. And all I ever did was read.

"There were definitely times when I used to think, why am I alive? You know, I used to sort of contemplate killing myself when I was an adolescent but I never could figure out how you do it. I just thought about it a lot because I couldn't see going on living with the pain. And I was really lonely and I was really ugly. And I thought, who would ever love this ugly body? Adolescence is no fun anyway. Even with a normal, healthy, well developed, balanced child, adolescence turns them into monsters. It is even more so with a genetically challenged childhood. It was really tough going.

"I think now there's a difference just how men and women respond on anything, given our culture, given the socialisation of baby boys and baby girls. Having never been a man, of course, I'm going to have to really just delve into stereotypes. I think that men probably respond with 'It's not that bad, I'm tough, I'm going to hide my hurt and hide my injuries, my social wounds'. So I think they'd probably come off as a little nonchalant, a little more 'laissez faire' about things which may or may not be true. I mean their real feelings, when given permission to have them and given a safe place to express vulnerability, would probably just be the same as a woman. But men are never given that permission.

"My parents were told that Nf was a very rare condition, that the doctors never saw anyone with it. In fact, it is quite common, affecting 1 in 2,500 people across the world. My Nf was diagnosed when I was eight. In bathing me, my dad found this little lump on my spine the size of a plum. And in a week, it was the size of an apple so, in other words, the curvature of the spine was starting. And it rapidly got worse and worse. My mother took me to the hospital and they told her there was nothing wrong with me, it was all her imagination!

"Eventually the doctors were persuaded to take X-rays and still they said there was nothing wrong with me. However, there was a consultant who had helped my brother years before at another hospital and we went to see her. She got the X-rays and then other doctors joined her and there was this conference. They diagnosed neurofibromatosis and then scoliosis (curvature of the spine). Within a week I was in for surgery - and the first doctors had told my parents there was nothing wrong!"

Sarah

"When you are told that you are ugly, stupid and are not worth anything and you're not invited to play games with people and so on, it tends to make you fell not so great. I had the worst case so that left me vulnerable both at school and at home. I hid my vulnerability in the way that I carried myself differently because I was so vulnerable.

"My head would be down. I was terrified of people most of the time. I figured that when they spoke to me they would say something nasty or not say anything at all. That, of course, reinforced more vulnerability and more sadness. I think as a result of that, I believe that victimisation is a learned role. I also believe that once you have been victimised in a learned role, it has a tendency to self-perpetuate and because you get deeper and deeper into that, there is no reference point for yourself as a person who is not a victim. If I am treated normally, I might tend not to believe it. At one point it was hard for me to understand why anyone would want to be nice to me. I probably felt that I didn't deserve to be treated any better.

"I think that a disfigured woman is far more devalued as a human being than a man is. I think it's harder on a woman. But I also tend to think that, at least in our society, there is still enough sexism left that a disfigured man may be affected more in terms of his employability because he's expected to support a family, probably more so than a woman. I also think that if a good-looking man or woman applied for a job and a disfigured person came in and applied for the same job, I'm sorry, they are going to take the good looking person. I think that men have it harder if they are disfigured and for purposes of employability are devalued on the basis of disfigurement.

"But women are devalued on both levels. It is more acceptable for a man to have a scar and so on. Women may be devalued by our whole culture because they are not beautiful and because they are disfigured. And then the double bind to that is that you're out in the community expecting to be hired. So I am full of contradictions because I can't figure out which one is harder. It is a little difficult to try and sort out which gender has more problems."

Carrie

"If I start to read, I won't remember what I've read from the top to the bottom of the page. By the time I get to the bottom, I don't know what they have said at the top, so I don't read. It gets to be frustrating. I buy my little grandson, Ray, books that he likes and I read them because I like them too. But I can't remember what happened by the end of the book - and these are his books. So if I can't remember that stuff, why should I bother to sit down and try to read anything else that won't stay with my anyhow. I just gave it up. Now I don't get so frustrated because I just don't read at all."

Sarah

"People who look different, people with disabilities, often do get treated pretty much like The Elephant Man. You may not literally end up in a freak show but on one level or another there are many equivalents of freak shows, many dead-end jobs. And when you think about it, the person with the dead-end job is the one you hand the rubbish work that you don't want to be bothered with. You don't have time to do it, you don't get paid to do those things, you get paid to do more valuable work. And the person in the dead-end job also stays in the dead-end job because there is nowhere to move and there are no opportunities. It's kind of the equivalent of a freak show in itself."

Larry

"In the beginning I couldn't ever understand why nothing happened with relationships. But after a while I started feeling it was the Nf. Sometimes I would meet someone and they would have a bad reaction to my lesions. I've had people ask me to leave their beds and others got up and left my place when they saw my body. Several years ago when AIDS came along some people thought I had AIDS. It can be very painful to have someone recoil from you like you're a leper. And that's happened. But if I was 'normal' and I met someone with these things, I guess I would also be inhibited to get involved with them. So I don't feel hateful towards these people, they're reacting normally. It's a matter of finding that extra special person where it doesn't matter."

Randy

"Men are used to doing things by themselves. They normally do not open up. If there is a problem men are more likely not to admit it. They are so used to dealing with things one-to-one while women share things. The relationships between women and girls, especially when they are growing up, are much closer than friendships between guys. Women share these inner secrets whereas guys make jokes back and forth about what not. It is a real sharing. Especially now, when women seem to be supporting each other more and men seem to be supporting each other less, this is why the divorce rate is so high."

Sharon

"Men don't share their feelings. It is okay for a woman to say 'I need help. I need support; I need to talk to other people about this and get that support.' It is not okay for men. Macho men don't need groups like that; men don't cry; men mustn't appear to have feelings and all that. I think that is rubbish and I think that is too bad because you could really get some good networking there if you have both men and women."

Pam

"The most irrational worry, and I recognise that it is irrational, is that I'm going to look like The Elephant Man. I think the more rational fear is that I'm going to grow so many more of these neurofibromatosis and they might become malignant, that I'll die as a result. Perhaps that's also an irrational fear, but no knowledgeable medical person has ever been able to tell me whether or not that could happen. After all, the neurofibromatosis really started to grow steadily when I was thirty-four. They continue and I'm forty-two. They began to grow more on the body than the face and I said 'thank God, I don't have any on the face.' By the time I was thirty-eight or thirty-nine, they were really starting to grow on the face. One doctor told me that any kind of reconstructive or plastic surgery is impossible because, he said, 'we'd have to cut away practically all of you that there is."

Nathan

(who died shortly afterwards)

"I was first seen by a dermatologist and I also have a GP. He referred me to various doctors for my problems. I've seen a neurologist, a neurosurgeon and a plastic surgeon. When I have scans, I always see the same radiologist too, the list goes on and on, so many different specialties for one disorder.

"I wouldn't wish Nf on anyone, even my worst enemy. It's with me all the time. And it's just sort of like a time bomb inside you ticking away. It could explode at any moment. Or it could never explode. It could be a big explosion; it could be a small explosion. It's just no knowing what's going to happen! I can't put aside a little ache or pain. Every one is a serious issue until I do something. Usually it is nothing, but I have to go through the process of doctors, the tests, and waiting for the results. Is this going to be it? Is something going to happen? Like I'm going through that now with this ringing in my ears. Is it the ears? Is it something in my brain? What is it? It may be something totally unconnected with Nf but the Nf is just such a powerful force that it's the first thing I think of. I have very little control over it. I can have responsibility for it and try to do something about it when there's a problem by going to the right doctors and insisting on things. But there's really no control. It is being very much alone."

Harriet

"I mean how often can you say that you feel bad because of the way you look and that you are not able to accept it? I feel really angry at myself for not accepting myself when there are things that other people have that are worse than what I have. I can see; I can function; I can work. But this society is based on how we look. I mean everything is. Not what we are but how we look.

"I would think that I am able to bury it pretty well and sometimes, even when I'm looking in the mirror, I can bury it enough to think 'Well, you know, I'm not unattractive.' I tell myself that so that I don't, for one thing, feel sorry for myself and think how ugly I am and repulsive. And there's times I look in the mirror and put on my make up but not see myself. I'm not seeing anybody else; I've not seen a perfect image; I'm not saying that. I'm just saying that I'm looking in the mirror and not looking. I think it's hard for most people to be able to perceive themselves, because you may have whatever image you have, it may not have to do with what you're seeing. I sort of have a hard time with my sister in this way. She has a lot of self-confidence and she's more outgoing. I would say that I'm real introverted. I'm drawing more and more into a shell. Sometimes it's very hard for me to go out at all."

Anonymous

"I had won a subscription to a health club and I went to the club and they showed me all around. I was to come back the next day to sign the contract. The next morning before I left, I got a phone call from the health club saying that they would prefer that I not come back. Whatever I had was unsightly and would frighten other people away and not to come back till I was cured. That was really the worst thing that had ever happened to me."

Rudy

"If people ask me what is wrong with me, I have different reactions. If it's an adult and they're curious and concerned, no problem, I'll spend as much time as need be. If they're really abrasive about it, I'll cut them off. It's on a case by case basis. As for kids, a lot of times it's normally about my eye. 'Is there any eye in there?' and I'll say 'No.'. And they'll swallow it. If they want to know why it was taken out, I'll explain 'Well, see one of these, these bumps here, there was one on the eye and it was no good.'"

Anonymous

on going to a support meeting

"I was very frightened to go in because I didn't know what I'd see or what degree of handicap I'd see. Outwardly, you don't know I have it, so I was kind of curious to see how other people with Nf looked. It wasn't so bad. I mean, I don't have it that badly and I couldn't imagine what someone who had it badly would look like. I was thinking that someone would look like The Elephant Man. It's a little frightening to see. But you couldn't tell people had it either. I think I was afraid to see someone that might have had it worse than me and think 'My God, there but for the Grace of God go I.'"

Theresa

"You feel strange and you don't know what you're going to experience when you get there. But you feel better about yourself, I think, because, especially at university, people come from all over. You might think that we're badly off, and then you see other people, someone worse off than yourself, and someone else even worse off still; you count your blessings when you go there.

"We were not really aware about how serious it can be until we started going to these meetings and then I was appalled. We've been fortunate that we haven't experienced the things that some of the people we've met there have experienced."

Michael

"I was very active when I was a child and I have numerous skeletal problems associated with the neurofibromatosis. The coordination problems contributed to the fact that I have skeletal injuries. I raced motorcycles. In reality, I think I spent more time healing than I actually did racing.

"I think the accidents were because of the lack of coordination. I have broken fingers, and a knee that had surgery on it in high school. I have ankles that are kid of like rubber from, again, just lack of coordination. I have what I jokingly call 'radar'. If there is something to trip over, my feet will find it. I always thought I had 'radar feet' until at one of the support group meetings it came out that one of the big problems that people with Nf have is the coordination problem. During the ride home, my wife said 'No wonder you're always falling down. I thought you were just clumsy, now I see there's a reason you're always falling down.'"

These are just a few extracts from the book but give some idea of the problems encountered by people who are 'different'. But they still have feelings and emotions so should be treated just the same as everyone treats their friends.