This article is reviewed, corrected, and approved by: Dr. Joshua Collins M.D. | MRCP। FRCP
A normal child will play, get hurt, and will learn how to behave in what situation. Parents teach them to learn, behave, and what to eat and what not to eat. Even often, we see that children are adamant about eating. Parents are struggling to feed them.
But what if your child doesn't stop eating, and is your child always hungry but skinny? They don't understand what is wrong or them and what is not, no matter how much you make an effort to make them understand. Then you probably need to learn what it is Prader Willi Syndrome.
Children may have panic attacks or be naughty all the time, and it is not PWS at all. When they see a burning flame and put their hand on that flame, they will not understand that putting their hand on a burning flame will make their hand burn.
Also, if you hit them, they will learn that there is nothing wrong with hitting. Before judging any solution, you need to learn what is Prader Willi Syndrome PWS.
What are the symptoms like, how to deal with it, or why is this even happening? And moreover, is prader willi life expectancy short? Or can it be improved?
What is Called Prader Willi Syndrome?
An uncommon genetic condition is PWS. It leads to issues with the mind, body, and conduct. It starts from the sign of extreme hunger. Overeating is the main problem of PWS.
They feel hungry continuously, and they always feel empty. Due to this overeating problem, they tend to grow many physical and mental problems like obesity, diabetes, sleep apnea, and many more.
Life expectancy for Prader-Willi syndrome
It is sad that they have a low life expectancy. They don’t survive like others. Despite the fact that people often live into their 60s, the average life expectancy is 30 years.
Symptoms Of Prader-Willi Syndrome
There are two types of symptoms, from childhood and from adulthood.
For Infants
The following signs and symptoms may be present from birth:
- Muscle fatigue.
- Face-defining features.
- No sucking reflex.
- Low responsiveness.
- Having a weak genital system.
Early childhood to adulthood
Sometimes PWS may occur in childhood and last lifelong.
- Weight gain and food cravings.
- Slow development of sex organs.
- Physically and mentally deficient.
- Cognition impaired.
- Retards.
- Having trouble speaking.
- Behavioral problems.
- Insomnia.
Reasons for Prader-Willi Syndrome
Chromosome 15 gene mutation is responsible for PWS. There are several causes of genetic changes on chromosome 15:
- Chromosomal deletions: Chromosome 15 is absent from the father.
- Mothers who are uniparental: Both copies of chromosome 15 are inactive.Translocation: Chromosomes produce genes that aren't properly located, so they don't work effectively.
Complications
Obesity-related complications: PWS people have low calories and are less active. Because they constantly feel hungry. These create many obesity-related problems. Such as-
- High blood sugar or type 2 diabetes
- High blood pressure.
- Heart disease.
- Sleep apnea.
- High cholesterol.
- Liver disease.
- Gallbladder stones may also occur
Other complications
Other complications include:
- Binge eating effects: An abnormally enlarged stomach can result from gulping large amounts of food, which is called binge eating. Prader-Willi syndrome sufferers rarely vomit and do not report pain. Eating too much can cause choking. The stomach may rupture if a person eats too much.
- Reduced quality of life: Families, education, and social participation can be adversely affected by behavioral problems. Prader-Willi syndrome affects the overall lifestyle even when they have grown up.
- Infertility: Some Prader-Willi syndrome patients can have a child, but most cannot.
- Osteoporosis: Osteoporosis makes bones weak and flaky, which lets them break easily. In Prader-Willi syndrome, growth hormone levels are low. Even the sex hormone is also defective.
How To Handle Prader-Willi Syndrome?
By following these options, one can manage PWS.
Feeding support for babies
The feeding tube may be needed for babies with Prader-Willi syndrome. PWS babies take more than a month to learn how to drink from the breast or bottle. Dieticians and feed specialists will assist you.
Exercise
Low muscle tone exercises will be given to people with Prader-Willi syndrome. Only a few minutes of exercise can develop a better change in their life. Doctors or professionals can guide you on some exercise plans which is appropriate for PWS people.
Hormone treatments
Human growth hormones can be given to children. It will help them to grow taller and strong.
Picking & healing
To avoid skin problems, they should avoid short dresses and have to maintain hygiene, like trimming nails and hair regularly. Older children can also benefit from therapy and antidepressants.
Management of controlling weight and diet
Well, managing PWS people is not an easy job. You need to work hard. The experts and charities can offer you a lot of advice and support.
Some easy and quick tips
- Make sure they learn how to eat healthily and maintain a regular diet from their childhood.
- They should limit their intake of processed food like chocolate, sweets, or junk food.
- Carbohydrates such as pasta, rice, and bread should be served in small portions.
- Eat more vegetables, salads, and fruits.
- If necessary, lock cupboards, fridges, and bins to prevent eating food outside of mealtimes.
Living With Prader Willi Syndrome
Conclusion
With hard work and determination, people with Prader-Willi syndrome can live a normal life if they start treatment on an early basis. They need extra care. So you should select schools that can provide them extra support and care they need to cope with.
To achieve maximum independence, people with PWS require lifelong support. Consult a nutritionist if you need help managing the child's eating disorder.
Support groups or health professionals can be helpful to parents and families. These professionals will help these children in the learning of how to adjust to the modern world.
Frequently Asked Questions (FAQs)
Question 1: Can Prader-Willi syndrome be cured?
Answer 1: Prader-Willi syndrome cannot be cured. The condition is currently being researched.
Question 2: Is Prader-Willi syndrome common?
Answer 2: One in 10,000 to 30,000 people worldwide can suffer from PWS.
Question 3: Who is affected by Prader-Willi syndrome?
Answer 3: Anyone can have Prader-Willi syndrome. You could inherit it if it is familial. Prader Willi syndrome affects many celebrities.
Question 4: Can Prader-Willi syndrome patients live normal lives?
Answer 4: Keeping a healthy diet and not becoming overweight can lead to a healthy life. When PWS patients grow up, they can manage to work part-time or volunteer. So yes, they can lead a normal life if they try hard.