Angelman Syndrome: Family and Social Issues

Presentations at the 1st World Conference IASO - Tampere, Finland, 4-8 July, 2000
Presented by: El Finn, Father of Lars Erik

Our son Lars Erik is now 22 years old. Caring for him for 16 years and then looking after his interests when he moved into his own apartment, has given us some experiences and thoughts we would like to share.

Before we start: This is the experience of my family, from one city in Norway, probably not a better or worse city than most. The individuals, the administrators and caretakers are fine and caring people, I do not intend to criticise them in this lecture. But I do mean to criticise the fact that health and welfare services are not organised to meet or to attempt to meet what I hold as the number one priority in the care of our AS children: stability in the caretaker group. And I do criticise that in addition to caring for an extremely taxing child, parents are left the heavy burden of being administrators for the complex and demanding care a child with AS needs.

In this short lecture I have to oversimplify many issues, please keep that in mind. I do hope this lecture will help younger parents to be forewarned and more prepared to plan ahead. And I do hope that professionals and administrators will understand more and take steps to avoid increasing the extreme burden placed on the shoulders of the parents.

There are no quick solutions to the problems, they requires hard, diligent work through the AS child's lifetime. More money is not the solution to all problems. In many instances stability is a major part of the solution.

Here in Tampere we have learned that there are differences between countries as how they take care of their handicapped. In Italy they have the culture of the extended family where many relatives take part. In my country as in many others, it is more common with nuclear families, parents and children living in a separate home, away from the rest of family, often in another part of the country. Having an AS child will then have more of an impact on family life than in an extended family. Adding to this problem is the fact that it is more common with divorce in families with a handicapped child than in others.

To the new Professionals

To newly graduated professionals I want to say that being educated in your field is but an introduction to the problems of the AS family, and in our experience nobody, not even our closest family or friends understands the burdens of being parents to a AS child. The child's happy and charming ways mask many of the problems. Maybe our experiences can help you understand the situation a little better, and please, do not become parts of the problem.

The First Years:

Kindergarten Primary school, special class His own apartment, age 16 High school, special class Diagnosis of Angelman syndrome, age 19. Year 2000 Day care centre He is now 22 years old

Lars Erik seems to be the archetype of an AS person with a deletion on the 15th chromosome. I will not go through the signs and symptoms as I assume you know them. I will show you a few photographs to give you a general idea of him. But pictures do not say it all, as mostly they are shot in the happy moments of life. He was hyperactive to the extreme, and we did not know that he would become more docile as he grew older. Some of our worries would have been lessened if we had.

It is thanks to his mother being ever-watchful, a nurse by training, that he survived childhood at all as he was often ill and had severe feeding problems.

His little brother very soon became the big brother and he was the best physiotherapist he could ever get, they played wild games all the time.Of course we did not have a normal home.

Not nearly enough sleep Feeding problems and vomiting Spills and messes Diapers Sickness Hyperactivity Always looking after A home adjusted.

Don't believe you can begin to understand what this does to a family. You have to live it.

Problem Solving

The father of an AS child should be required to be a handyman and an engineer like me. We had to make all kinds of adjustments to our home, even to the TV set to ensure his safety. We tried to stay ahead and find ways to cope with problems as they arose. We made toys and playgrounds, even a trailer for a bicycle so we could go on bicycle trips.

Norwegian Health Insurance

In Norway there is universal health insurance. People are entitled to have their needs met irrespective of their ability to pay for services. But even in an ideal system like this the problems abound.

The Problems of Relating to the Caretaker-System

We are willingly doing our duty as parents and caretakers for our handicapped child. Of course! But, in addition to the problems at home, there were problems outside the home to cope with. Paramount to our problems was the sheer incredible number of people -- caretakers and other professionals of all types - with whom we had to relate and deal.

There was the added burden of having to become multi-specialists, administrators, co-ordinators, lobbyists, lawyers etc. to make sure our son got the help he needed. There are so many agencies, offices, professionals and caretakers for him and us to cope with. In this sea of faces, titles and names the parents added burden comes to light, we have to be not only parents, but the hub and engine of all this extra activity too!

To give you a general idea: I estimate the number of meetings and appointments we have had to attend through the years because of Lars Erik to be approx. 800-1000! Of course this has had major consequences for normal family activities.

The Star

I visualise our role as being in the centre of a star with rays going out in all directions, towards kindergarten, medical specialists, school, educators, personal assistants, offices of social welfare, county administrators, politicians, the parent interest organisation for the mentally retarded, high school etc. The rays are meetings and appointments, letters and forms, phone calls and contacts of all kinds. Between the points of many of these rays there was no contact. We had to initiate and co-ordinate an incredible number of activities. We should have been spared much of this, the professionals are paid to do their jobs and should not rely on us to co-ordinate them. They should have done so on their own. That would have left us to do our bit, to be a family and to be the parents of a severely handicapped child.

We will always be his parents as opposed to the caretakers who can quit the job when they fancy it or when the going gets rough. We cannot quit.

The Wheel

We should expect the offices and caretakers to act like a wheel - with spokes in to us, but with a firm, solid outer rim with close and frequent contact between the administrators and caretakers in the rim, aimed at preventing and alleviating our problems, not adding to them.

Our ideal dream would be to have a neutral county co-ordinator with a special responsibility to see to this, to plan ahead and to be our contact. He or she would literally earn his paycheck if he managed to keep a few handicapped children in their families and out of residential care for one more year.

The Handicapped Family

When an AS child is born into a community, the community does not only get a handicapped child, it gets a handicapped family. The arrival of the AS child has a profound effect on the family members and their lives. Caring for the needs of an AS child takes more and more time as the child grows older. Much of the time normally spent on the other children, family, friends, and leisure activities, disappears. Even the parents' ability to work and have careers is affected.

Parents have to cope with too much work at home, too many contacts external to the family and with trying to keep their marriage and family together. They do not get enough sleep and they have a set of difficult feelings involving sorrow, guilt, episodes that hurt, frustration, forbidden thoughts and downright exhaustion. After 22 years of this I am getting angry. The community administration, specialists and caretakers should not expect the parents to take on such a burden without doing everything possible to alleviate it! Do not take it for granted that the parents shall participate in all the work, year after year. The caretaker system should rather help them survive by doing as much as possible without them. They should take the initiatives, plan years ahead and contact the family only in order to inform them or ask for their consent or opinions.

Help the Family

As we do know quite a bit of what an AS child can and can not do or learn, it is important to teach the parents how they may help the child best. The child spends most of his/her time in the family. As soon as possible after the diagnosis of Angelman syndrome is made, help the parents and siblings to put their energy into productive efforts. Inspire the parents to take care of themselves and their marriage, their other children and their relationship to relatives and friends. They are under a tremendous pressure.

A New Life.

Lars Erik moved into his own apartment when he was 16 years old. The apartment is fully equipped and there is no common living room for other inhabitants. He has 1:1 personnel in his apartment, but less than that in the day-care unit. There is a sleeping caretaker on call at night who is alerted by an alarm if Lars Erik moves from his bed. Because of problems leaving any public bus without considerable protests from Lars Erik, he has his own car.

We naively thought we would have an easier life as parents when Lars Erik moved, but the problems have just become different, not easier.

Parental Replacement

It is almost impossible to substitute the all-day parental care with personnel working in 8-hour shifts, often people of his own age. The difference is evident in many ways, but can often be attributed to one common denominator: The overall interest for and attendance to details - or lack thereof. There is a certain need to register and put in writing everyday occurrences etc, but it is hard to believe a single person needs as many binders, written routines, registration forms and instructions as the personnel has around Lars Erik. He is the cause of a veritable small business! Keep the bookkeeping simple!

Too Many Personnel

This is what worries us most, and causes him the most trouble: The personnel are not stable enough. In the first year he lived in his own apartment he had to relate to 36 different caretakers! A young, frightened boy away from home, unable to make them understand.

Frustration

As you will know, he has no verbal language, but he communicates all right. The problem is that it is mostly his private way of communicating, using pictograms, sounds, gestures and facial expressions. This is where most of the problems start, the caretakers often don't understand him, as he does not always understand them. Because they don't know him well enough the caretakers act and communicate differently and the limits they set for him are not always the same. No wonder he is frustrated!

When he is frustrated, he acts out, mostly by passive resistance or pulling hair. Through one year he was registered as having pulled hair a total of 500 times, 1.5 times a day. This is of course a problem for the personnel. To us parents the answer is obvious: In one way or another: Make sure the personnel is stable and stays with him for years, not just for a few months. That way they will learn his ways (and he will learn theirs) and aggressive acts will be dramatically reduced. What might be left has to be coped with, but the problem will be reduced to a minimum. We know this is true. By chance, the last 9 months the personnel have been fairly stable, and the number of incidents has decreased substantially.

Our words fall on deaf ears. There are almost no administrative initiatives to search out the kind of people he needs, no creativity or unorthodox ideas to solve this vitally important problem for him. We do not have the solutions, but dramatically new ways should be found. In a few places in Norway they have experimented with 7-day work shifts in the care of mentally retarded people, with very positive results. Maybe that is a solution to be tried out. There also seems to be a need for mature, experienced persons in the caretaker group. The caretakers must have professional counselling and time to meet and discuss their experiences, thoughts and feelings. That will increase their interest, knowledge and professional status which in turn will increase their interest in the AS person and the job of caring for him.

Another problem for him is that many people he knows and befriends quit their jobs and leave, never to come back. The bonds to people he has learned to love and feels affection for are broken. He obviously misses many of them and experiences sorrow and broken trust. Do the so-called professional caretakers ever think of this - and make a responsible decision to stay for a while when being employed to care for a mentally retarded person? Is that too much to ask?

Lars Erik has developed a peculiar way of coping with many personnel to maintain his sanity: He makes a virtual, protective shell around himself and the person on duty, with clear boundaries. He guards this shell and needs to be specifically informed when other people want to enter his sphere. This is the case even when we, his parents arrive: "Are you here for real, or are you leaving again"?

We are Tired

We are getting older, more tired, more frustrated by all the repetition of information, and because the real problem is not addressed. And we worry about the day when we no longer will be here to look after him.

Our Advice

We advise parents and people working in the health, welfare and educational services to be extremely aware of the need to have a preventive attitude towards problems that may lie ahead in these realms:

Communication skills Medical problems Sorrow and loss Psychiatric issues Aggressive behaviour Lastly, in the all-important issue: Stability in the personnel group!

Back to: 1st World Conference Presentations Index

Top of Page