Changes in the body: Puberty with Prader-Willi Syndrome

Puberty is the time of transition. The body begins to grow, sexual characteristics mature and the reproductive function begins. In people with PWS, puberty development takes a different course, because for normal puberty and fertility to begin, the gonads, i.e. the testicles and ovaries, the hypothalamus and the pituitary glands must be intact. This is not the case in people with PWS. They are normally born with underdeveloped sex organs, and the hormonal control of testicles and ovaries by the hypothalamus is severely impaired. That is why puberty is usually delayed, can sometimes take place at a normal age or even too early, but then 

it often stops and remains incomplete. Pubertal characteristics occur in girls and boys with PWS in an altered order, i.e. in boys the axillary and pubic hairs begin to grow very early on (at the age of approx. 9 – 10 years). Girls with PWS can develop breasts relatively early. Their periods are irregular and can sometimes even stop completely. Boys with PWS often do not experience their voice breaking. Facial hair often only grows partially and their facial features remain partly childlike. Since it is important for a person’s self-image and social identity to go through puberty, and since disabled people also have a right to puberty, boys and girls with PWS 

“I can do this!” – On the way to realistic self-determination and positive self-esteem

Puberty is also a time in which a child strives for greater self-determination and tests itself, and a slow process of detachment from the parental home takes place. Teenagers – and mostly their families – often go through a rollercoaster of emotions. Even if teenagers with PWS experience puberty at a later stage – and in most cases incompletely – due to their special nature, they too have to go through a period of emotional turbulence. They express their urge to expand their inner and outer horizons by demanding a say in many decisions. The parents and carers have the task of finding the balance between the teenager’s ideas and abilities. They need to be protected from an unrealistic and distorted self-image and should be offered support and structure, for example on the subject of money. Putting the value of money in relation to the object to be acquired can be an abstract concept for a person with PWS. That’s why they should be helped to manage their money when it comes to certain things. Conflicts regarding food can often occur.

A family sometimes reaches its limits because it is not always easy to endure having to constantly “manage” the strong urge for food of a family member. Nevertheless, parents should stick to teaching their child that self-control is in their best interest regarding safety, well-being and satisfaction. Making sure that they are of a stable weight and preventing obesity is a lifelong priority. Time is also a foreign concept. A person with PWS lacks the imagination for time frames, for example when tasks have to be completed. In any case, their motor skills are slow and they may experience great stress if they notice a change in a situation due to sudden time pressure. Fixed family rules, routines, daily and weekly plans, but also increasing (co-determination) freedom help teenagers to structure themselves on the way to greater independence and to feel secure. Parents and teachers should always consider small successes in self-management with praise and recognition. Because a balanced and emotionally stable person can only grow through the help of positive self-esteem.

Behavioural abnormalities, which already became apparent in childhood, usually increase during puberty in people with PWS. Families usually experience this as a major stress test and sometimes feel “at their wits’ end”. On the one hand, young people with PWS are very kind and good-natured. They constantly make you smile when they comment on situations in their own appropriate and humorous way. On the other hand, they often display demanding behaviour, which always revolves around themselves, and try to use this their advantage – especially when it comes to food. It is often the case during the bustle of everyday life that both parents blow hot and cold. When living together with people with PWS, it is extremely important that ALL members of the household are of one mind. This is not always easy when you may be suffering from a stimulus overload, multi-tasking and a constant workload that is too high. Taking a deep breath, slowing down and concentrating more on what is important often seems desirable, but this can only be achieved with great difficulty.

Just as we have to learn to deal with our own stress, a person with PWS has to work on his or her neuralgic points and the associated stress. Parents should try to develop a sixth sense, for example of when an outburst of anger is imminent. Sometimes it may still be possible to prevent the outburst. Perhaps the child’s concentration can be directed to an activity that he or she likes. If the tantrum is unavoidable, it should not be suppressed by any means. Anger should be expressed, because suppressed anger can turn inwards and cause depression. During an outburst it is often the case that the only option – difficult as it may be – is waiting calmly for it to end. However, you should always try to prevent the child from breaking things or from hurting themselves or others. Due to their reduced ability to change perspective, people with PWS in a crisis situation hardly notice that their behaviour can be problematic or hurtful to others. After the tantrum, they often feel depressed and sad. During this phase you should comfort and support your child.