The Need for Information for Parents of Children with Angelman Syndrome
Presentations at the 1st World Conference IASO - Tampere, Finland, 4-8 July, 2000
Presented by: Tamara Stranders The Dutch Angelman Syndrome Association T. Stranders, H.W. Borne van den, L.M.G. Curfs, Dutch Angelman Association, Dutch Federation of Parents Organisations, Utrecht, The Netherlands, Department of Health Education, Maastricht University, Maastricht The Netherlands, Clinical Genetics Center
My name is Tamara Stranders and I am working at the Dutch Federation of Parents Organisations, especially for the Prader-Willi/Angelman Association.
As you noticed, in Holland parents of children with Angelman syndrome and parents of children with Prader-Willi syndrome join together in one organisation. This is a pure coincidence, although a lot of parents with Angelman children will recognize the symptom of the uncontrollable appetite of the Prader-Willi children. Fortunately most of the Angelman children don't gain so much weight as their Prader-Willi brothers and sisters. But besides this and the related genetic causes, both syndromes do not have very much symptoms in common.
Details About the Dutch Prader-Willi Organisation.
The Dutch organisation counts 610 members. 457 members are connected with Prader-Willi syndrome and153 with Angelman syndrome. Of those 153 'Angelman members', there are 118 families with a child with Angelman syndrome. 15 Families live in Belgium. This is because of the fact that Belgium does not yet have an Angelman organisation of their own. The other 27 members are relatives and professional workers. About those 118 families: 64 families have a daughter and 50 have a son with Angelman syndrome. The other families have two children with Angelman syndrome: three families have a boy and a girl, one family does have two girls with Angelman syndrome
The Age Span of the AS Children and Adults.
I don't pretend to be a statistician, but we can learn a few things from these figures.The fact that there is only one member in the range from one to three years old probably means that most proper diagnoses will not be made untill the age of three. So even today at this point there is still a lot to improve. Many parents still have a long way to go before they get a final diagnosis: autism and epilepsy often are first mentioned as separate diagnoses and many parents experience accumulating problems untill they receive the final diagnosis.
The next thing we learn is the fact that most children are between seven and twelve years old now. This also is logical: most parents will need some time to get used to the idea of their child having Angelman syndrome and some of them will not immediately join the association. So most diagnoses will be made somewhere between the age of three and seven. Above twelve you notice a decrease of children and adults. Since we may suppose that there has been no increase in the birth rate of children with Angelman syndrome over the last years (Angelman syndrome in most cases is a spontanous mutation as you know and only in some cases hereditary) this must mean that there are lots of of (young) adults and adults with Angelman syndrome without a diagnosis. Every now and then some attentive professional will recognize the symptoms and a diagnosis is made after many years, but for most of the adults the diagnosis will never be made. I am speaking about the Dutch situation of course: I don't know whether other countries systematically try to diagnose all those people in institutions with an unknown cause of the mental handicap.
Well, back to our parents again. As everyone will understand an early diagnosis is the first thing parents need. That is one of the reasons why we try to involve physicians of the Dutch centres for clinical genetics and pediatricians in the making of our information books and papers. A few years ago we made this little book about Angelman syndrome and although it is written in Dutch of course, you can take one if you are interested.
Probably next year there will be an updated version of the book. This book, by the way, is part of a series of books about specific syndromes, published by the Dutch Federation. The new version of the book will look like this......
These books are not only used by parents, but also by social workers, general practitioners and other professionals. Besides the book every four months we publish a newsletter: A mix of scientific news, stories from parents, siblings and practical tips and advice for treatment and everyday life.
The Internet as a Source of Information
Another source of information for parents has come to be the Internet of course. The Prader-Willi/Angelman Association, however, does not (yet) have a website of its own, both syndromes are described at the site of the Federation and parents can reach us through that way. One of the parents (he is here at the conference himself), Frank van Hof, has an interesting and up-to-date website. In addition to many links to other sites there is a nice family photo album with stories and photographs of every family who wants to join. You can see a few addresses on the slide now. My own experience with the Internet is that, on the one hand, it is a new and quick source of information for parents and, on the other hand, for a lot of people it is very confusing as long as they do not have a proper diagnosis: they see many syndromes with a lot of symptoms of wich often more than one match with the symptoms of their own child.
Video on Angelman Syndrome
Another instrument the Dutch organisation has to offer to parents is a video about Angelman syndrome. The video shows, in my opinion, in a very positive way, three children with Angelman syndrome in different situations: at home, during daycare, in a physiotherapy session etc. In between we mixed interviews with their parents. It is not a 'medical' type of video.
So there is a lot of information available, but far more needs to be done. Little is known about how to teach children with Angelman syndrome to communicate for instance. And due to this lack of communication many children show behavioural problems. For some behavioural problems like sleeping problems and tidiness some programs exist which may be succesful. But at our last family meeting in Holland we made a long list of other behavioural problems, about which parents wanted to get practical and helpful information. How should they deal with aggressive behaviour like biting and scratching, pulling someone's hair, concentration problems, hyperactivity, shouting and jealousy.
Study to Identify Psychosocial Problems of Parents
In 1998 a little study was done in the Netherlands to identify the psychosocial problems of parents of a child with Angelman syndrome or a child with Prader-Willi syndrome. In addition, the study described the strategies these parents apply to cope with these problems as well as their need for information. To assess these topics, parents filled in a questionnaire.The results indicated a great need for information about the possible consequences of the syndrome for the child, about how their child may develop in the future and how education might contribute to this development. These needs were found to a significantly higher degree in parents with a child with Angelman syndrome than in parents with a child with Prader-Willi syndrome. This may be due to the fact that little educational material for children with Angelman syndrome has been developed until now.
Another significant difference between parents of children with Angelman syndrome and parents of children with Prader-Willi syndrome was found in the fact that parents of children with Prader-Willi syndrome are most concerned about the negative consequences of the syndrome for the child; how the child would cope with challenges because of the syndrome; about a possible worsening of disabilities, and they also worry about future loneliness of their child. Parents of children with Angelman syndrome were a bit more concerned about negative consequences for themselves such as the fear of losing their patience in caring for the child or the fear for not being taken seriously when talking to others about the problems they have with their child. The most significant difference between both groups was found in feelings of loss of control. Parents with a child with Angelman syndrome have a significantly stronger feeling of loss of control than do parents of children with Prader-Willi syndrome. This loss of control is indicated by a feeling like their hands and feet are tied, not feeling able to handle their affairs as before, having concerns about their child and they also have heavier financial burdens. A possible explanation for these differences could be that children with Prader-Willi syndrome as compared to children with Angelman syndrome on average, are less mentally handicapped, and consequently have a higher level of awareness of their condition. Because of this higher awareness, parents may anticipate and fear that their child in the future will be confronted with limitations more often and more intensely.
The results from this study indicate a high need for adequate education and information for parents. Also, helping parents understand that specific problems are a direct consequence of the syndrome and teaching parents techniques for coping with these problems may well support the parents' coping strategies. This teaching should be done before problems in the child emerge. And educating fathers as well as mothers may lighten the burden of mothers and reduce possible avoidance behaviour of fathers. Facilities and organisations that may provide periodical relief from heavy care duties, particularly for mothers, may help to prevent the development of depressive feelings.
Contacts with Other Parents
And finally of course, contacts with other parents with children with Angelman syndrome may help to learn that you are not the only one with a child with special challenges. In such contacts parents may also learn particular strategies that have been found to be effective by others in coping with similar problems.
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