Changes in the body: Puberty with Prader-Willi Syndrome
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The time of transition
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
are given the missing hormones through injections, pills and ointments as part of hormone treatments. These are performed by experienced endocrinologists in the PWS centres located in Berlin, Essen, Hildesheim or Munich. Each treatment is individually tailored to the patient. By adding additional sex hormones, boys with PWS can experience their voice breaking and a stronger growth of body hair, and can develop more masculine facial features. In girls with PWS, pubic hair begins to grow and their menstrual cycle becomes more regular.
Further differences between boys and girls
In boys with PWS, the doctor first has to check the sex hormone levels in the blood and the bone density. Since the testosterone level of boys with PWS is only 2/3 as high as that of healthy boys, a low level can trigger bone disease. An additional dose of testosterone would counteract this. Prior to the treatment, the doctor will also evaluate the patient’s mental state. Some parents are worried that their son will become aggressive due to the additional testosterone. But testosterone alone cannot trigger this. Special psychological dispositions also have to be present in order for it to lead to increased aggressiveness. If the biography of the boy indicates this, the physician makes a decision, if necessary, after weighing up all the facts.
Girls with PWS produce their oestrogen in the ovaries, but also from hormone precursors in fatty tissue. This is why they have a basic secretion of oestrogen. However, they do not have a regular cycle and bleed irregularly. There are fertile follicles in the ovaries of girls with PWS, but these do not mature. Although oestrogen substitution gives the girls a regular menstrual cycle, it does not affect their fertility. This remains low. Parents should, however, contact the endocrinologist if they have any questions about contraception. Hormone substitution also has a positive effect on bone density in girls with PWS.
“I can do this!” – On the way to realistic
self-determination and positive self-esteem
Tips for behaviour in an acute crisis
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.
- be clear about his/her own goal/ do not weigh up or signal any alternatives
- do not insist on having the last word at all costs
- take control of the situation/keep calm/ignore the outburst of anger
- use precise, clear language, preferably in words that a child can understand
- do not talk to the child about consequences
- do not interrupt the thought process / pattern, for example by changing topics / take yourself out of the situation, possibly create a spatial distance between each other (source: PWSVD Workshops)
“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.
Tips for behaviour in an acute crisis
- be clear about his/her own goal/ do not weigh up or signal any alternatives
- do not insist on having the last word at all costs
- take control of the situation/keep calm/ignore the outburst of anger
- use precise, clear language, preferably in words that a child can understand
- do not talk to the child about consequences
- do not interrupt the thought process / pattern, for example by changing topics / take yourself out of the situation, possibly create a spatial distance between each other (source: PWSVD Workshops)
“I am what I am” – Help me to understand you
Many people with PWS feel different to others from childhood on. But they also wish to be part of a group and strive for recognition, especially during adolescence. Peers do not always react sympathetically and amicably if someone falls outside the established code of conduct. Exclusion and bullying are often the result. Unfortunately, many of our children with PWS are used to such situations. People with PWS don’t know exactly how to make social contact. They constantly have to relearn how to deal with people: how to talk, how to interpret the gestures and facial expressions of the person they are
talking to, how to react appropriately, and how to build and maintain friendships. Like other people, they feel hurt when experiencing rejection, because they want to be loved and accepted despite their disability and the resulting difficulties. Inferiority complexes, frustration, stress and offence can then lead to tantrums or other strong affective reactions such as depression, anxiety and self-harm. Skin picking is a form of self-abusive behaviour that may occur – repeatedly picking at their skin or a wound until it bleeds results in the would not healing and eventually leads to scarring.
Everyone’s pulling in the same direction: Ways out of the “behavioural vortex”
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.
When they have overcome their anger, talk to them about their outburst and reflect on the situation together. Try to find out exactly what triggered the episode. Together you can devise strategies that will allow them to better control their violent impulses during the next outburst.
Ask for help for yourself and your child if you have the impression that there are problems with anger and aggression that occur in the family home or at school that they can no longer cope with on their own. Contact the Prader-Willi Parents’ Association, visit our workshops on the subject, and talk to psychologists about your requirements.
Teenagers with PWS and their sexuality
The sexuality of disabled people is still a social taboo. They usually experience it with great limitations, which is why it is all the more important for their parents and caregivers to introduce them to this topic with sensitivity and willingness to talk. Now, our approach to sexuality is very personal. It is shaped by our upbringing and our own experience. For example, it can sometimes be difficult to find the right approach to questions about sexuality, which can sometimes be tricky. Some parents of disabled children are happy when the paediatrician finally asks the redemptive question of how their child perceives their sexuality. Some would rather ignore the whole subject and “let sleeping dogs lie”. But everyone has a right to psychosexual development. Young people with PWS also want security and understanding. They want to believe that they are beautiful, especially in relation to their body, which is now constantly at the centre of therapeutic effort. They want to make friends and exchange affections. Their interest in the opposite sex is present, but their mutual attachment behaviour cannot be compared with the activities of their healthy peers.
Young girls and boys with PWS have fewer opportunities to meet other young people because they are driven home after school and usually do not have any school friends. They often lack the necessary contact to build friendships. And if they do have the opportunity, PWS makes it difficult for them to develop the relationship and maintain it in thelong term. Their parents must support them.
Young girls and boys with PWS are often very innocent and childish. Due to their attitude towards adults in particular, parents are often afraid that their daughters will become victims of abusive behaviour. Education is the best protection against this. Parents should strengthen their disabled children by teaching them that there are things that people should not do with them. That they, too, can say “no”. And that they themselves must not do anything that may upset others. It is important to keep talking to your child about this. In addition, a trusting contact consisting of the child’s doctors and therapists normally helps.