Plenary Session P4

Disability, Education and Employment

Chaired by Anne McCarthy - Dublin Eire

and Richard Brown - San Francisco

Richard Brown: Welcome to the Plenary session Disability, Education and Employment. We have a variety of people this afternoon, six in all, and we want somewhere along the line to be able to have some question time.

Anne McCarthy: Well, ladies and gentlemen, our first speaker is Ueli Buhlman and he is going to speak about adolescents with cystic fibrosis, transition issues. He is the chair of the department of paediatrics Triemli Medical centre in Zurich, Switzerland.

Adolescents With Cystic Fibrosis, Transition Issues

Ueli Buhlman.

The last speaker this morning said how tough it was to be the last one before lunch, I think, it is tougher even to be now after lunch, of course, it’s harder to speak on something that 2 speakers in the morning session have already dealt with. So my task this afternoon will be to talk about the same issue chronic disease and I picked the subject of cystic fibrosis because it is cystic fibrosis that brought me into adolescent medicine. The additional points to my talk are transition issues or the question how to bridge the gap between childhood and life as an adult.

I didn’t know actually what people in this room knew about cystic fibrosis I thought I would briefly read you about the disease, it’s a genetic disease, it’s the most frequent one among the patients, it’s a disease that occurs in 1 in 2000 new-borns in Switzerland, it’s a disease that affects severely the lungs, pancreas and the sweat glands and it is the lung disease that in most cases responsible for the limited life expectancy. I think when we talking this morning Dr Suris was introducing the topic by saying how things have changed and I think it is very much true when we look in that particular disease of cystic fibrosis. The first description has been made in a dissertation, in a doctor’s thesis in Switzerland in 1935, it was then further published in the United States about the disease adding the sweat glands, infection as well, and the important thing was, was it curable when it was first defined with a very high mortality in early childhood. It was shown again this morning in the 70s life expectancy was around early teenage youth somewhere between 12 and 16 years, and then in ‘89 there was an important discovery it was gene defect that was discovered, the CF gene was first described, the first mutation, right now we are in a situation where over 800 different mutations on that particular gene are described, so there was a lot going on in these last 10 years. And as it has been shown earlier today life expectancy is now somewhere about 30 years of age.

What does that mean? It means that life expectancy is better, the age of our patients has changed quite a bit. You see that age range in just about 60 patients that we see on a regular basis in my centre there is about half under the age of 15 and the other half, 50% are older than that and a few patients are into their early 40s. So, we have a disease where from training we knew and from the history we know that that occurred in the early childhood and now I just come back the most recent North American conference about cystic fibrosis that took place last week, now we talk about going into 30s, 40s, 50s, nobody knows. And the question is what does that really mean to grow up to adulthood with a chronic disease. Because we cannot expect neither from the patient or a physician that in such a short period of time with so much research going on that this is going to be an easy step to take.

It has implications during early childhood. I had talked to many of my adult patients about what they heard from their parents, when they were informed about the disease, we have to change our approach to the parents of a child with a chronic disease being very careful in predictions of life expectancy. Parents in fact have to be prepared in early childhood that this is not a disease that will just be a problem to them during childhood but that the child will move on into adolescence and to an adult life like everybody else. I think it’s important to teach the children very early, in fact as early as they can understand as much as you can about therapy. Taking the example of cystic fibrosis and medication they have to take perhaps 5 times per day sometimes even more frequent, I try to teach the kids as early as possible to do that on their own, to take their own responsibility, because the earlier the children are involved in therapy and also in decisions they have to be taking, the less they will use medical issues during adolescence to rebel against the grown ups, being the doctors and the parents.

It is important that the kids grow up in a peer group where there are other children with chronic disease but also, like Bob Blum has shown it in one of his studies, also within a group of healthy peers. That means it depends a lot when we talk about school and how well the child has functioned on a social level in his first 10 years because it’s the basis for adolescent functioning and then moving to adult functioning. Which means that we should not always talk just about what we should do during adolescence to make lives easier for our patients but that we should think of cases during childhood and then look forwards carefully into the age of adolescence.

What roles do we have? Adolescents want to become independent, they have to become responsible adults. There has to be satisfactory integration of the body image, there has to be a way that healthy adolescents find their way into sexuality and as I just mentioned before how important it was to help them find their peer group. It is important and I work with many adolescents and now adults who did not plan their careers because all they knew during childhood was that probably in their teenage years they were either going die or would be so heavily disabled that they would not be even thinking of finding a profession or doing a job. So this is a very important point that specific goals are set and goals that can then further be implemented and of course on the level of cognitive development it is important to have them go all the way through these developmental steps.

There are of course limiting factors, it has been mentioned this morning and I mention it again that disability and mobility will play an important role, the more mobility the patient has during adolescence the easier it will be for him or her to really adjust into adult life. It is a question of the energy level of course with lung disease this level is sometimes low so we have to find ways to help the patient like with oxygen or whatsoever to find the energy needed to make the steps. There are questions of physical appearance and of course as it’s been discussed widely and I think Gerben, next speaker is probably going to talk more about it, psychological issues like self-esteem on the affective stage and of course social integration all these factors can be very limiting, and it is important when we see a patient on a regular basis in the centre that we carefully evaluate where the patient stands in terms of his well-being even with a chronic disease. Well-being is so important it has to be checked every time we see a patient.

The better the patient feels in terms of independence the more he’ll adhere and it’s been shown in several medical studies the more adherence or compliance to the medical treatment will be better, there will be less depression, less other mental disorder issues, there will probably be less rebelling behaviour and will have a patient who will also comply with a health care system that’s set up for him and it’s important. I’d like to just say a few words about the peer acceptance. I think that it is probably one of the areas where at least in Switzerland we still have a lot to learn. We do not involve the peer group of our patients enough here at the hospital or even making sure that in their schools that there is a strong peer group there for young patients with a chronic disease. There is a risk if that peer acceptance is not good enough there will be an overprotection tendency of the parents and there is a risk of social immaturity with further problems in the peer area -  a force that can either lead to depression or to failure to actually move on to adolescence.

When you attend a cystic fibrosis conference in Europe and in America and  you listen to the doctors there talking about extended life expectancy what happens is that we go away with the idea how well we are doing our job with new treatments new medication. But there is a lot more to it, there is a lot more to the emotional health of a patient with a chronic disease and when we look at the development of cystic fibrosis and the lives of our patients as nowadays it’s not just to be explained by the advantage of antibiotics or new treatments in inhalations but it has a lot to do with how well we manage our patients through childhood and adolescence into their adult life.

Anne McCarthy. Our next speaker is Gerben Sinnema who is going to speak about chronic ill health in adolescents. Gerben is a clinical psychologist with the Wilhemina Kinderziekenhuis in Utrecht, Netherlands.

Chronic Ill Health In Adolescents.  Gerben Sinnema

Thank you lady chairman, dear colleagues, As you all know adolescents are a special branch of people, a special species, they are young attractive and healthy. Well, at least most of them are and as you heard from colleague Ueli Bulhman from Switzerland many of these young people are not so healthy at all. About 10 to 20% of all young people in population suffer from some sort of chronic conditions seriously affecting their daily life activities. And about 3 to 6% have serious conditions with serious consequences for daily life.

As is true for cystic fibrosis and it is true for many childhood conditions that treatment improved over the last decade and life expectancy went up. Nowadays it is estimated and I quote from an American study of Bob Blum, that about 90% of all children with chronic conditions survive into young adulthood. So we should be very aware and the previous speaker already said that, that it’s not only the quantity of life counted in years but also it’s very important to look at the quality of life. We are talking about chronic illness and since the 70s the studies of Stein and Jessop there is a general trend in the literature that consequences of the chronic disease can be generalised to many diseases. And of course this is true, the social consequences, the risk for social isolation, the risk for problems with mood and acceptance and things like that but we should keep in mind that for the young person him or herself the social and psychological consequences of the disease are linked to that particular disease.

So a young man or a woman with a diabetes considers the consequences of daily life as a consequence of the diabetes mellitus. Chronic illness comprises a lot of different diseases from the medical point of view with difference in ideology, clinical expression and prognosis but there are difference from the psychological side. As I said for a young person himself it’s that particular disease that is causing trouble, from the psychological point of view there are differences between these diseases as to the point of whether a genetic disease opposed to an acquired disease. We think that adaptation and coping with a genetic disease goes a little bit more smoothly, it’s there, the disease is present from birth and it’s going more quietly and smoothly as opposed to the acquired disease, for instance juvenile arthritis when a kid is 12 or 13 and is confronted with a serious and crippling disease and there has to be a period of sorrow because of the lost health and lost normal life expectancy.

We are talking about illness and treatment on the one hand and growth and development on the other hand and of course as Ueli Bulhman said before the more serious the disease manifests itself the higher the severity of illness the more chance you have for some problems, but the opposite is also true. We see a lot of young people with only mild forms of the disease for instance the juvenile arthritis with only a few joints involved where there are a lot of psychological and social problems. Probably because they compare themselves and they try to compete with normal healthy adolescents. I am glad that Ueli Bulhman spoke already about self-esteem so I can be a little bit shorter on that. Positive body image of course is difficult to acquire when the body feels or is hindering you with impediments. As to autonomy we as health care workers have to teach young people that it is not shameful to need a little bit of help of your fellow people. So the development of independence in the emotional sense is quite another thing than have to be dependent in the physical sense. But that’s a long story before young people and adults grow up to that point.

As to education most of young people with chronic illnesses go a regular school, in our country it’s up to 85%, but sometimes they are somewhat delayed in educational process and as doctors or psychologists we have to be aware that some diseases as well as some treatments, schedules go along with cognitive problems. As is true for some types of cancer treatment. And to social adjustment, integration would be a better word, the more seriously the child or the adolescent is affected the more chance that the range of social activities is restricted. Well, it’s true that a lot of conferences about chronic illness in adolescence last years stressed the point of resilience and coping abilities. We should keep in mind about 85% of all adolescents with a chronic condition adjust very well. A few groups might be at risk, in the first place youngsters with invisible handicap are often inclined to keep the condition secret, this has some advantages, for instance you can avoid reaction of pity or misunderstanding, but very often the price is too high because the youngsters are permanently afraid of disclosure of other people noticing their condition. Youngsters with the unstable condition as the arthritis, youngsters with juvenile arthritis, as well as with a progressively worsening disease it’s very difficult often for peers and adults to react and respond adequately. And the last point that true for cerebral palsy for instance when the brain is involved or central nervous system, you have a double handicap.

All chronically ill youngsters and this is a familiar issue should be family oriented. We have to focus on the stress of the disease as it is affecting the work or the relationship with the parents and now and then and we do that in clinical practice we should have a little talk with the siblings as well. And the literature tell you that most brothers and sisters of adolescents with chronic conditions don’t have so many behavioural problems or problems with social adaptation. That maybe true but still they may suffer not so much from lack of attention of the parents but they may feel for instance a little bit guilty that they don’t have the disease, for instance they may feel a little bit anxious about the genetic side of the disease and may feel anxious about their own offspring and so on and sometimes they develop psychosomatic complaints. On the other hand when you speak with these siblings you will be struck by the huge amount of solidarity and social support they give to their affected sib. Well, family even in adolescents remains very important for the social and emotional support of the adolescent, open communication within a family is very important, but the communication between the treatment and the family is very important too. And you have to enhance the coping skills of the family. This is very important because one of the major coping skills is of course the power that allow them to stay in control because the disease goes on for many years, lifetime, there should be a balance demands of treatments dictated by the disease and the demands of the normal daily life activities. And you have to negotiate between these demands.

As to social integration, as the afternoon goes on we go more to the practical side and what can we do to help and support these youngsters and tomorrow in our workshop on chronic illness we’ll focus more specifically on these points, there are a lot of services to support young people for this social integration, focus independently on special services, focus at education, training and employment. So what’s clear is that it is a joint effort between the adolescents, family, health care workers and society. And as health care workers what we should do is educate young people about practical consequences of the disease, we do that as a rule, educate them on the emotional and social consequences, it’s a little bit less the rule, but we still should do that and nowadays we focus a lot on skills training. It’s not a point to assess to social difficulties for young people with chronic condition, what you should do is reverse, you should teach them skills.

Well. This is problem when I speak for my own country I can say that the unemployment rate is very low and the productivity is very high. That’s good to say and that’s marvellous form the economic point of view but the other side of picture that a lot of young people with physical handicap are not able to get into the labour market, so they are put on a disability allowance. What appears to happen and it’s a sad story that we buy our disabled. What we should do from the society point of view is create facilities for social and economic participation otherwise it’s useless to train people and support people to be young adults with a lot of facilities and abilities and then they will be stuck in their development process. We should do that so not only healthy people but also young people with physical disability can face the world proud and self-assured.

Richard Brown: Thank you for that very cheerful summary of a very complex problem.

Question regarding talking to siblings.

A: Well, it’s an ideal, we tried to do that, but in a hospital setting it’s often difficult because there is no registration number for a healthy seat you have to say what you are doing because of productivity in a hospital these days, but we ask parents how are the sibs doing and if we sense a problem on a clinical radar we say bring them along sometime and you don’t need to talk for hours here you need quarter of an hour just to scan the daily life of a brother or sister and give them a little of explanation it’s very worthwhile to do.

Chairman. Thank you again. I am pleased to introduce our next speaker, Doctor Helena Fonseca who is a paediatrician from Lisbon, Portugal, she is a head of the adolescent out-patient clinic in hospital De Santa Maria since 1994, she has trained with Bob Blum at the University of Minnesota in adolescent medicine and she’s got an MPH. Her topic is chronic illness and youth.

Chronic Illness And Youth  Helena Fonseca.

Good afternoon. I am sure that working with adolescents with chronic illness is not easy but undoubtedly it is very stimulating, I have been doing this for many years. I have always felt that the effects of the chronic condition on the adolescent very often outweighs the impact of the illness itself. We have to keep this in mind when we have an adolescent with a chronic condition in front of us even if the chronic condition is not very serious, it’s impact can be huge. It has been quite interesting for me thinking the impact of the chronic illness on the adolescent not only thinking about the age of onset of the condition, the nature of the condition but also trying to think about the first impact about the development of tasks of adolescents.

So thinking about autonomy and identity I will go a little bit through all of these, I will try to be clear and just beginning by the age of onset I do think this is an important point just because if the chronic illness starts at birth or in early childhood what happens is that sometimes this leads to ultra parental expectations so what we see and this is really very usual we see that parents don’t have the same expectations towards these kids as they have towards another one. And this is quite serious when the start is at early adolescence. At this stage what happens is that the adolescents is very concerned about his or her body and chronic illness may lead to extra concerns. Also at this stage because the adolescent has yet to separate from his family what happens is that this can lead to little struggle for independence. And you can see adolescents at this stage feel very dependent from their parents.

When the beginning is at the middle adolescence stage this age may be the most devastating time for a chronic illness to start. And the reason is because during this period the adolescent is intensely involved with parental separation with peer involvement and concerns with his or her own sexual development and so a chronic illness appears here and the adolescent will no doubt face extra difficulties in all this process. If it starts in late adolescence I do think that the main problem is that it will disrupt vocational plans, it will disrupt prospective of leaving independently and things related with this. Of course it is important to look as my colleagues said previously it is very important to look at the nature of the illness. And of course its course, the chronicity, the side effects of the medication all this is very important, the prognosis of course but I would like you to stop a little bit at this second point, the visibility.

I have been noticing that visibility is really very important. And from our experience highly visible disease even if it is less serious may cause more disruption than a very serious one. I have some adolescents with dermatological problems and these problems which are not so serious for instance eczema but they are really disruptive. As clinicians we have to be really aware about this point.

So as I promised and this framework has been very important to me at least it was developed by Laurence Neinstein and I like it very much so thinking about identity and autonomy the adolescent with a chronic illness may have difficulties with his or her developing identity. And this is linked with a body image. Adolescents are highly concerned with their bodies and either because they have delayed puberty or because they are carrying some kind of malformation this can lead to a lower self-esteem, so segregation from peer and increased anxiety over sexuality and like at the end of this continuum sometimes depression and really very negative feeling. So I would say that a chronic illness at this point can severely interrupt the whole process and the whole movement towards independence. Especially if it takes place in the early or middle adolescence as we have already said this can be a problem much more than the disease in itself in my opinion.

As far as autonomy is concerned sometimes they feel tired, sometimes they feel like and this is a reality for most of the cases, they spend a lot of time in medical appointments, so they do miss school and they do miss activities. So this problems may of course lead to fear of peer involvement and can also lead of course to social segregation. If we think about autonomy and this is the like the nice way I like to look at it if we look at autonomy as the adolescents capacity to take responsibility for their own behaviour to make decisions regarding their own lives, what is so so important, to maintain supportive social relationships, if we think about autonomy in these terms I do think that we can agree that the development of autonomy is perhaps the major goal for adolescents with a chronic condition.

As I have already said this adolescent will face additional difficulties especially related with sexuality. Because of the delayed development, having of having too protective parents, because of feeling uncomfortable in expressing concerns about this field, they are very often seen as asexual, and I do think this is huge problem and I do think that paediatricians as me because perhaps we were trained for dealing, for working with little kids we are not sometimes aware of these problems and really need to be.

Talking a little bit about the adolescent girls I would say as many other people said that these adolescent girls living with a chronic condition as Juan Suris and someone else this morning addresses this issue very well, she will have an increased health risk if becoming pregnant because of the main chronic illness but also she is an increased risk of becoming pregnant. And this because perhaps she wants to prove herself that she is able to and there might be many other reasons, we can discuss this later.

So I shall say that the social and sexual aspirations of adolescents with chronic diseases are not different from the rest of the population. And the other point which I think if very important is that this kind of adolescents are really specially sexually vulnerable. And if we think about sexual abuse all the research point to the fact that these kids are much more sexually abused than the rest of the kids of the same age. OK. So I would like now at this point to talk a little bit about the family and efforts we have developed to work together with the family especially in Minnesota where I learnt with Joan Paterson about the FAAR model I will talk a little bit about it if I have the time. I learnt that if fact that having a family with us is really very important and much more important if we are working with adolescents in early and middle adolescence. And I do think that we have a very important role in preventing something crisis that could be unnecessary if we have provided a sympathetic guidance. If at the right point we would have been able to talk about the right things, to discuss the right things to give the right help I do think that  some problems would not happen.

If we look at the family as a important resource we can understand how much a successful adaptation to a chronic illness can best be promoted by focusing on the family system. If the family system will be our unit of intervention and not the adolescent individually I do think we are working better. And of course all of us are aware if the family is happy the adolescent with a chronic illness will do better.

So what I’d like to point at this stage is that if we think about poor outcomes as feed back increasing demands we can also look at good outcomes as feed back to family systems that increases their repertoire of capabilities. What I mean by this is when I talk about good outcomes I am thinking about having been able individually or as a family to make the right decision at the right time or having been able to take responsibility for a particular behaviour, I do think that this gives power to the family, this makes the family feel that perhaps the nest time they will be able to  do the same or even better because they have a positive previous experience.

So as clinicians we really need to pay attention beyond the somatic aspects to emotions and this was already very well discussed here today. So what kind of emotions does the illness raise inside the adolescent and how is the adolescent dealing with his or her chronic illness and also how does his or her personal and family history influence all this. And at this point I do think at least for me the FAAR model, FAAR as the initials for the Family Adjustment and Adaptation Response, this model has helped me a lot for understanding the family dynamics and learn how to manage with the family. You have here the balance with the two plates and you can see that when a crisis appears what happens is that strain becomes really heavier and this upsets the balance. So what happens for balancing the system again we have to put more strength in the other plate, and I do really think that we can help indeed. We have already this morning talked a little bit the possible behaviours of the chronically ill adolescents I would only like to say something we listen this morning and I do think it’s very important is that we are aware that in order to be able to cope with difficulties and frustrations the adolescents usually have this kinds of behaviour first ones or the second ones but the important thing  here is that as we said this morning not being compliant either way of showing that the adolescent is independent.

Sometimes we are tired about the adolescent who is not doing the medication, we are concerned about the increased risk-taking behaviours, I understand all that and I have that personal experience but the problem is that if we look at this as a healthy way, a healthy process of autonomy I do think it’s a good way of thinking and of working on this. Sometimes the adolescent who copes very well with everything sometimes because she was able to reframe the situation and of course developing coping mechanisms, sometimes this is done at the expense of lots of autonomy.

Just to end I would like to say that behind the physical face of the adolescent’s medical condition consideration of the adolescent’s psychological development and tasks is very essential as I have already mentioned and this could be bases on this list of principles by educational process I mean informing the adolescent and the family of the nature of the illness, its course, possible limitations of the treatment. and something that is very important is to adapt our language to the kind of adolescent or family we have in front of us because we physicians we are not trained in that, we speak with very difficult words and sometimes we are not able to reach the audience. The other important point already stressed is the continuity of care. When we think about physicians, issues I do think that we really have to think about the continuity and how to deal with it. Of course I have already talked about involving the adolescent and the family and the evaluation of the impact of the illness on the needs of the family. I would only like to say something about the multi-disciplinary team. Something actually I discussed this morning after the chronic illness presentation. I do think that nowadays we are aware that we have to involve in the team many health care professionals and I could like issue huge list but I feel at least in my country that communication sometimes is not easy among these people and this is really a point I think we need to improve. And I say this just because sometimes I do feel that this makes the difference. OK. Thank you.

Dick Brown. We maybe have time and discussion a little bit later to keep on schedule. I am very pleased to introduce my co-chair Anne McCarthy who is a historian by background, she told me and also has a degree in education. She worked with Ronanstown Community Training and Education Centre in Dublin, Ireland. Her experience is with youth, with learning disabilities and education and employment needs of youth with special needs and with behavioural difficulties. Her presentation is entitled Job retention.