How can we encourage the development of good quality support and care for adults with Prader-Willi syndrome?

 A blog written by François Besnier for Prader-Willi France and shared with permission.

These few lines are personal reflections, inspired and developed following contacts and exchanges with families and professionals in the caregivers’ world. They do not pretend to be general, as the expression of PWS is variable and depends on the personal and family history of the people in question. But they attempt to describe an observable trend which should raise questions about strategies for the care and support of individuals and their families and the role and responsibilities of all institutional and associative parties.

First of all, one observation: today’s children and young adults are not the same as those of 20 years ago. The photo galleries testify to this, like the videos that circulate on the internet. Progress in the knowledge of PWS, early diagnosis and therefore the possibilities of very early care, have changed the trajectory for children with this syndrome. Better weight control, speech therapy, psychological follow-up, establishment of an adapted educational framework, alongside other developments, in many cases allow educational paths and acquisition of skills that can be almost comparable to those of children without PWS.

But we are also seeing a growing number of very complex and dramatic situations in adolescence and around the transition to adulthood: weight gain, the appearance or increase in behavioral disorders, violence towards those around or against oneself and psychiatric disorders. There are also very difficult periods in the ESMS (Social and Medico-Social Establishments) which, very often, after analysis, could have been avoided by a better knowledge of PWS, the person and their history. Parents and professionals can then be in great difficulty, and the person with PWS and their family in great pain.

Let’s examine some hypotheses:

The framework and its evolution

Justified in large part by the necessary control of access to food, a framework is put in place very early in many families, a framework that is sometimes very (or too) rigid, motivated by the fear of obesity. But with schooling, the comparison with other children, the greater freedoms outside the family framework and the lack of understanding amongst wider friends and family, this framework set up in the family can be undermined. It is also difficult to change this framework, a relaxation claimed more or less violently by the person saying “I am an adult!”

The absence of a framework

Another extreme situation: certain weakened families, single parents without support, in a complex social situation, “give up” and find themselves with a tyrannical teenager, who is master of the game, but who is still not happy. It is also sometimes for the sake of not “adding” to the injustice of disability, that this necessary framework is relaxed or abandoned.

The person decides little for themself

From an early age, the person with PWS has been the object of discussion and little room is left for their own free choices and desires. At first, they often integrate the choices of their parents and experience a conflict of loyalty which can be expressed violently.

Real or supposed capacities and desire for autonomy

The person finds it very difficult to see the contradiction between the capacities that they think they have and their desires for an “ordinary” future. They want to have their life and make their own choices.

What choices are currently available to adults? 

Sheltered housing, with or without medical support

When care homes accept people with PWS, it is unfortunately with little, or insufficient, training for the team. They tend to favour autonomy and the management can be reluctant to allow the control of access to food. Fortunately, there are remarkable organisations working with Prader-Willi France and/or the Reference Center, but whose work can be called into question by a change of management and the turnover of teams.

Sheltered workshops (ESAT)

Accommodation is provided most often by the family or by a residential housing provider (different from sheltered housing) and a diverse range of activities are offered. The problem of access to food in ESATs arises very often because the importance is underestimated by the team, who are also reluctant to impose the necessary constraints on other workers. Accommodation in a residential housing often leads to unacceptable situations: in the name of respect for the liberty and rights of individuals, the young adult is left to themself. We then see serious hygiene problems and weight gain linked to the great freedom left to residents (trafficking and exchanges, delivery of food to homes, etc.). In addition, there is not always coordination between the ESAT and residential housing, with inconsistent attitudes and support. Accommodation with the family is not a long-term solution and a source of difficult conflicts for everyone.

Accommodation in their own home

Support is then most often provided by the family. These solutions rarely last in the long term, the person being socially isolated with very great difficulties in managing their diet. The person can work in ESAT, have voluntary daytime activities, very rarely an adapted job and exceptionally a job in an ordinary environment. To my knowledge, there are very few solutions for supported shared housing, which should nevertheless be possible following the Piveteau Wolfrom report and the decrees that followed.

The future adult, whose course may have been close to the average for a while, rebels in front of these prospects, they dream of a life like everyone else and their parents dreamed of. Finding a “place” is not a project. How to support the person and their family to build a chosen life project? 

 This picture is not very positive and rather describes situations of relative failure or those that are unsatisfactory. Fortunately, there are more successful stories that should be identified and analysed too. What made things go well in these examples?

Experiences abroad show that there are frameworks and solutions that take into account the wishes of the person and their abilities and skills and support to extend the areas in which they can exercise their freedom of choice, in a progressive self-determination process.

What to do next? 

  • With current knowledge, imagine possible futures with the necessary support.
  • Look at solutions in other countries without wanting to copy, because the cultural and social contexts can be very different.
  • Do not favour a solution but consider the diversity of situations.
  • Work with similar family associations to be stronger and pool possible solutions (shared housing, for example) .
  • Exchange and work with associations of rare diseases encountering difficulties similar to those we are experiencing (Angelman syndrome, Fragile X, etc.).
  • Make an appointment with your government institution to present a file with the status of the situation and request strong action and support towards the various actors of the caregiver sector, the national education system and disability support.
  • It is abnormal that the management of large associations dealing with disabled people, which display a very inclusive discourse are also reluctant to welcome people with PWS, or reject them as soon as the situation becomes difficult. These difficult situations are often linked to a lack of knowledge of PWS and insufficient resources put in place. For information, in Germany a training program of 150 hours is recommended and implemented for the facilities which accommodate a person with a PWS.
  • Support for families is also an essential element to develop, in ways to reflect on. The association does not feel legitimate today to offer training for families. There is a great void there to be filled.
  • Listening to the person, self-determination. We must also think about the accompaniment of people with PWS in a progressive evolution towards self-determination thought of as the progressive acquisition of possibilities of free choice in concrete fields. The development of ETP (Therapeutic Patient Education) programs by the Reference Center goes in this direction.

These few lines, very schematic, incomplete and sometimes caricatured, above all aim to contribute to a debate necessary to put in place a strategy for the years to come. In this debate, Prader-Willi France, must take the initiative.

François Besnier – October 2021