Management

There's no cure for Prader-Willi syndrome, but your child will have support from healthcare professionals who will help you manage the condition.

Support for development will come from your local child development team, and your child will also see a hospital paediatrician or a paediatric endocrinologist.

The treatment plan will be continually reassessed as your child gets older and their needs change.

The Prader-Willi Syndrome Association UK (PWSA UK) has detailed information about how the support your child needs will change as they get older.

Treating problems in babies and children

Feeding difficulties

Babies with Prader-Willi syndrome have difficulty feeding at birth, and may need to be fed using a tube that goes into their nose and down their throat into their stomach.

After a few months, it will usually be possible to feed your child normally using breast or bottle milk, although they may be slower to feed than other babies.

Dietitians and speech and language therapists can help with advice about what feeds to give and how to encourage your baby to feed.

Undescended testicles

If you have a baby boy with undescended testicles, surgery is usually recommended to correct this in the 1st or 2nd year of life.

Treatment is recommended because your son:

• will have an increased risk of developing testicular cancer if his testicles are not corrected
• may have problems with his self-esteem and body image if he's "missing" 1 or both testicles

Read more about treating undescended testicles.

Managing weight and diet

The most important aspect of caring for someone with Prader-Willi syndrome is trying to manage their diet and prevent them gaining too much weight. It's also probably one of the biggest challenges.

People with Prader-Willi syndrome burn up less energy, and need fewer calories and less food than other people.

A dietitian can give you information about what food to give.

Advice about diet:

• start to get into good habits with a healthy diet and regular mealtimes as soon as your child starts on solid food – do not wait for them to show signs of an increased appetite
• avoid sugary items, sweets and high-calorie snacks
• give them smaller portions of carbohydrates such as potatoes, rice or pasta
• increase the amount of lower calorie foods, such as vegetables, salads and fruit
• give them a vitamin supplement

It can be difficult for your child to control their food intake themselves, so you need to:

• keep to regular mealtimes and not allow any extra portions
• stop them accessing food outside of mealtimes – you may need to lock cupboards and the fridge, or lock the kitchen, and make sure they cannot get food from the bins or freezer
• keep food out of their view
• make sure there are no times they can access food unsupervised
• make sure everyone who is in contact with them (school staff, relatives and friends) knows about the issues with food

It's also important to let relatives, friends, other parents and teachers know about the need to restrict the person's diet.

Some medicines have been used to try to suppress appetites, but have all been unsuccessful.

Weight loss surgery is not recommended for people with Prader-Willi syndrome because they are not able to stick to the strict diet required after surgery.

Exercise

Exercise plays an important part in helping your child maintain a healthy weight. Children should do at least 60 minutes of exercise a day.

Many people with Prader-Willi syndrome have reduced energy levels.

It may be a good idea to break down their exercise into 5- to 10-minute sessions throughout the day to stop them getting tired and discouraged.

Your care team should be able to recommend a suitable exercise plan.

People with Prader-Willi syndrome usually prefer individual activities to team sports, such as:

• swimming
• walking
• exercising in a gym

It's important not to promise food as a reward to encourage exercise because it can encourage unhealthy behaviour.

Read more about physical activity guidelines for young people.

Hormone treatments

Treatment with an artificial version of the human growth hormone (HGH) is usually recommended for children with Prader-Willi syndrome.

HGH also has a number of other important health benefits.

For example, it:

• increases muscle size while lowering the amount of body fat
• increases muscle strength, which helps with activities such as walking and running
• should increase energy levels, which will help your child become more physically active
• helps change facial appearance, making the distinctive facial features associated with Prader-Willi syndrome less noticeable

Starting treatment with HGH is usually recommended during early childhood, from 6 months to 2 years of age, and normally continues until the end of growth.

A test looking for breathing problems (a sleep study) is usually done before starting growth hormone.

A type of HGH called somatropin is used to treat children with Prader-Willi syndrome. Somatropin is given by daily injection. Most children tolerate somatropin well and side effects are uncommon.

It's usual to replace female sex hormones (often with the combined oral contraceptive pill) to:

• encourage development of secondary sex characteristics (breast development) and periods
• improve bone strength and prevent osteoporosis

Replacing the male sex hormone (testosterone) is more contentious. Although this will encourage development of puberty and build muscle strength, it's possible that some behavioural challenges could get worse.

Managing challenging behaviour

Structure and routine

Most people with Prader-Willi syndrome cope best if they have a very structured environment and daily routine.

For example, you should:

• establish a regular daily routine and give plenty of notice if any changes are planned
• not put pressure on a child to speed up to complete a task – give them plenty of warning if they need to complete an activity, such as getting dressed to go out
• ensure other people, such as relatives and teachers, know how to interact with your child
• avoid eating in front of your child so they do not start to think about food

Dealing with emotional distress

Parents often learn to recognise the warning signs of emotional distress, which some people call a "meltdown".

It can help if you try to understand why it's happening.

People with Prader-Willi syndrome have difficulty managing their emotions and tend to feel more anxious and stressed than others.

It's sometimes possible to manage this behaviour.

For example, you can:

• acknowledge the person's feelings by saying things like, "I can see you're upset about this"
• encourage them to go to a quiet place for a few minutes and try some deep breathing or listening to soothing music
• remain as calm as possible – if the frustration is directed at you, your partner or another carer can take over

After an episode of emotional distress, try to remain as calm as possible.

It's important to be positive afterwards, and to talk about what has happened.

You can do this once the situation has calmed down – for example, later that day.

You should avoid making them angry or frustrated again by talking about what happened too soon.

Taking food

Many children with Prader-Willi syndrome will try to take food if they get the opportunity.

This is because they are not able to control their impulses when it comes to food.

But it still needs to be managed, otherwise they could become severely obese.

Tips to prevent taking food include trying to establish a contract to reward good behaviour.

In younger children, a verbal agreement, such as: "If you stick to your diet then you can play an extra hour with your puzzles", should work.

In older children and teenagers, a written contract may be more appropriate, particularly as children with Prader-Willi syndrome respond well to clear instructions.

Controlling the urge to take food should always be highly praised and persistent good behaviour rewarded.

Prader-Willi Syndrome Association UK has more information about behaviour management in adults and children with Prader-Willi Syndrome

Treatment for skin picking

Frequent skin picking can lead to scarring and skin infections such as cellulitis, an infection of the underlying tissue.

It's very important for cellulitis to be identified early and treated with large doses of antibiotics.

Keep nails as short as possible to help minimise damage to the skin.

Try to keep any affected part of the body covered up and use clothes to limit access if possible.

Make sure you keep any areas of damaged skin as clean as possible.

Antibiotic cream may be prescribed to be used on damaged areas to prevent infection.

Treatments that are moderately effective in helping prevent people with Prader-Willi syndrome from picking their skin are cognitive behavioural therapy (CBT) and medicine.

Cognitive behavioural therapy (CBT)

Cognitive behaviooural therapy (CBT) is a type of talking therapy. It aims to modify unhelpful and unhealthy patterns of behaviour by changing the way people think.

It's thought people with Prader-Willi syndrome pick their skin as a way of coping with situations such as feeling unhappy or bored. CBT can help people understand the thought patterns that drive skin picking and encourage them to find new ways of thinking about and coping with these situations.

Medicine

A type of antidepressant – selective serotonin reuptake inhibitors (SSRIs) – or antipsychotics (medicine usually used to treat psychosis) are sometimes recommended to treat Prader-Willi syndrome.

However, these medicines can cause side effects and are not usually recommended for children under the age of 18.

Medicine is usually only considered if the symptoms of skin picking are severe enough to justify the associated risks of treatment.

Treatment for psychosis

A small number of people with Prader-Willi syndrome develop psychosis, usually during the teenage years or adulthood.

Psychosis is a mental health problem that causes people to perceive or interpret things differently from those around them.

The symptoms of psychosis usually begin abruptly and cause the person affected to suddenly become very upset and agitated, and act in ways that are uncharacteristic.

Contact your care team if you notice a sudden and unusual change in behaviour.

Psychosis can be treated with CBT or medicine such as antipsychotics.

Read more about treating psychosis.

Treatment for other related conditions

Children and young people with Prader-Willi syndrome are vulnerable to a range of related conditions as they grow up. Follow these links to read more about treating these conditions:

• treating long-sightedness
• treating sleep apnoea
• treating osteoporosis
• treating scoliosis
• treating short-sightedness

Adults with Prader-Willi syndrome

Most adults with Prader-Willi syndrome are not able to live fully independent lives, such as living in their own home and having a full-time job.

This is because their challenging behaviour and issues with food means these environments and situations are too demanding.

But adults with Prader-Willi syndrome can have active social lives and become involved in clubs or volunteering.

Adults with the syndrome who do not live with their parents may need residential care or supported living.

Prader-Willi Syndrome Association UK has more information and advice for adults with Prader-Willi syndrome