Management (1) | NICS Well

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

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 has detailed information about how the support your child needs will change as they get older.

Treating problems in babies

Poor feeding

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'll 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 first or second year of life.

Treatment is recommended because:

your son will have an increased risk of developing testicular cancer if the testicles aren't corrected
your son may have problems with his self-esteem and body image if he's "missing" one or both testicles

Read more information about treating undescended testicles.

Managing weight and diet

The most important aspect of caring for a child 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.

Your child will never learn to control their food intake themselves, so you need to do this for them. Children with Prader-Willi syndrome burn up less energy, and need fewer calories and less food than other children. A children's dietitian can give you information about what food to give your child.

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 – don't wait for them to show signs of an increased appetite
avoid sugary items, sweets and high-calorie snacks
give smaller portions of carbohydrates such as potatoes, rice or pasta
increase the amount of lower-calorie items such as vegetables, salads and fruit
give a vitamin supplement

Your child will not learn to control their food intake themselves and you need to take control of things:

keep to regular mealtimes and do 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 can't 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 the child (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 your child's diet.

Some medications have been used to try to suppress children's appetites but have all been unsuccessful.

Weight loss surgery isn't recommended for children with Prader-Willi syndrome because they don't have the willpower 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 children 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 child's care team should be able to recommend a suitable exercise plan.

Children 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 your child to participate in exercise because it can encourage unhealthy behaviour.

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 developmental progress such as walking and running
should increase energy levels, which will help your child become more physically active
helps normalise facial appearance, making the distinctive facial features associated with Prader-Willi syndrome less noticeable

It's usually recommended that treatment with HGH begins during early childhood, from 6 months to 2 years of age, and would normally carry on until the end of growth. A test looking for breathing problems (a sleep study) is normally 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 male sex hormone (testosterone) is more contentious. Although this will encourage development of puberty and build muscle strength, it's possible that some behavioural problems will be exaggerated.

Managing behavioural problems in children

Structure and routine

Most children 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, are briefed about how to interact with your child
avoid eating in front of your child so they don't start to think about food

Dealing with tantrums

Parents often learn to recognise the warning signs of a temper tantrum. It's sometimes possible to stop the tantrum before it starts using a number of approaches. For example:

try to take their mind off the situation by doing or saying something unexpected, or talking about a subject they're interested in
encourage them to go to a quiet place for a few minutes and try some deep breathing or listen to soothing music
avoid saying things like, "You'd better not have a tantrum about this"
remain as cool and calm as possible – if you feel your child's frustration is directed at you, then your partner or another carer can take over the situation

After a tantrum, try to remain as calm as possible. If your child hurts themselves or others, you may need to be taught special restraint techniques. Your child's care team will be able to advise you about this.

It's important not to give in to the demands that provoked the tantrums. While this may be tempting, it will signal to your child that tantrums are an effective way of getting what they want.

Taking food

Many children with Prader-Willi syndrome will try to take food if they get the opportunity. This isn't because they're being naughty but because they aren't able to control their impulses when it comes to food. Nevertheless, taking food is a behavioural problem that needs to be addressed, otherwise your child could become severely obese.

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

Most children with the syndrome will automatically lie about taking food, even when the evidence is overwhelming. So rather than asking: "Did you steal that food?", say something along the lines of: "I know you stole that food and we need to talk about why that's wrong."

It's important your child understands the consequences of their actions and what's considered acceptable behaviour. If they steal food or money to buy food, always insist they apologise and pay any money back.

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

The Prader-Willi Syndrome Association has also produced a leaflet about Behaviour management in Prader-Willi Syndrome (PDF, 144kb).

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.

It's recommended you keep your child's nails as short as possible. This should 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.

Keep any areas of damaged skin as clean as possible. If your child has a history of frequent skin infections, their care team may prescribe an antibiotic cream you can use on damaged areas to prevent infection.

Two types of treatment that are moderately effective in helping prevent people with Prader-Willi syndrome from picking their skin are cognitive behavioural therapy (CBT) and medication.

Cognitive behavioural 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.

Medication

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

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

Medication 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 will cause the person affected to suddenly become very upset and agitated, and act in ways that are uncharacteristic.

Contact your child's care team if you notice a sudden and unusual change in their behaviour.

Psychosis can be treated with CBT or medication 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:

Adults with Prader-Willi syndrome

Most adults with Prader-Willi syndrome are unable to live fully independent lives, such as living in their own home and having a full-time job because their behavioural issues and problems with food mean these environments and situations are too demanding.

However, adults with Prader-Willi syndrome can have active social lives and become involved in clubs or volunteering. Adults with the syndrome who don't live with their parents will probably require residential care.

Page last reviewed: Fri Jan 2021 Next review due: Wed Feb 2020

Management