Little Fat Power Syndrome: Managing Excessive Appetite in Children - Rare Disease

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Little Fatty Syndrome


Hello, doctor.
My child is 1 year and 2 months old and is a twin.
Currently, he is 72 cm tall and weighs 8 kg.
It seems that he cannot control his appetite, as he has a very large food intake.
For example, at 9 AM for breakfast, he can eat a piece of radish cake that is about 1.5 cm high and 4 cm long, a small cup of soy milk (300 cc), and one egg.
An hour later, he drinks 180 cc of Pediasure.
At noon, he can eat a portion of noodles about the size of a fist, 90 cc of juice, half an orange, and 3 pieces of soda crackers.
At 4 PM, he drinks another 180 cc of Pediasure.
By 5:30 PM, he has a bowl of rice that is about 80% full, along with some vegetables and meat.
Around 7 PM, he can eat about 300 cc of noodles and some fruit.
By 8 PM, he will ask for more food, and he still cries for milk at night.
I originally wanted to control his food intake, but if I restrict him, he cries and throws tantrums, lying on the floor and crying loudly, hitting, throwing things, and even biting or licking the floor and chewing on clothes, showing signs of emotional outbursts.
I tried not to give him milk at night, but he also cries loudly.
I am concerned that he may have Prader-Willi Syndrome or other issues due to his inability to control his appetite.
What are the other characteristics of Prader-Willi Syndrome besides uncontrollable appetite, and how can I manage his proper food intake? Could you please provide some insights, doctor?

nana, 20~29 year old female. Ask Date: 2010/12/15

Dr. Lin Xuanpei reply Rare Disease


If there is a suspicion of Prader-Willi Syndrome (PWS), it is essential to take the child to a pediatric genetic specialist for a detailed evaluation and specialized genetic testing to confirm the diagnosis.
After the diagnosis is confirmed, the specialist will explain the results and guide related care matters.
It is advisable not to jump to conclusions or overly worry before the diagnosis is established.
Care guidance for Prader-Willi Syndrome (PWS) requires considerable patience and perseverance.
More importantly, effective communication with the child's primary physician and the professional healthcare team is crucial for developing a treatment and care plan, along with regular follow-up assessments (it is recommended to do this every 1 to 3 months).
While growth hormone therapy is a central aspect of PWS treatment, maintaining a regular daily routine, behavioral guidelines, and scheduled exercise, along with careful dietary calorie management, are equally important.
If necessary, consultation with a nutritionist, social worker, rehabilitation physician, child psychiatrist, or psychologist should be sought.
Once a well-structured daily routine is established, it is essential to adhere to it.
Children with PWS often exhibit strong fixation and may initially resist; therefore, patience and guidance are required to help them gradually adapt.
The PWS Association has many parents who can share valuable experiences, which often surpass theoretical knowledge and written descriptions.
Lin Hsuan-Pei, Pediatric Genetics Department, Mackay Memorial Hospital.

Reply Date: 2010/12/15

More Info


Understanding Prader-Willi Syndrome (PWS) and Managing Excessive Appetite in Children
Prader-Willi Syndrome (PWS) is a complex genetic disorder that is characterized by a range of symptoms, including insatiable appetite, obesity, intellectual disabilities, and behavioral problems. It is crucial to understand that not all children with excessive appetite have PWS, but the symptoms you describe in your child warrant a thorough evaluation by a pediatric specialist, particularly a geneticist or a pediatric endocrinologist.


Symptoms and Diagnosis
In PWS, the excessive appetite typically begins in early childhood and can lead to severe obesity if not managed properly. Other common symptoms include:
1. Hypotonia (decreased muscle tone): This can affect motor skills and lead to developmental delays.

2. Growth hormone deficiency: Children with PWS often have short stature and may benefit from growth hormone therapy.

3. Cognitive impairments: These can range from mild learning disabilities to more significant intellectual challenges.

4. Behavioral issues: Children may exhibit temper tantrums, stubbornness, and obsessive-compulsive behaviors.

Given your child's age and the described behaviors, it is essential to consult a pediatrician who specializes in genetic disorders. They may recommend genetic testing to confirm or rule out PWS.


Managing Excessive Appetite
If your child is diagnosed with PWS, managing their appetite and weight is critical. Here are some strategies that can be employed:
1. Structured Meal Plans: Work with a nutritionist to create a structured meal plan that includes balanced meals and snacks at regular intervals. This helps prevent constant grazing and teaches your child about portion control.

2. Behavioral Interventions: Implementing behavioral strategies can help manage your child's reactions to food restrictions. Positive reinforcement for appropriate behaviors and consistent consequences for inappropriate behaviors can be effective.

3. Physical Activity: Encourage regular physical activity tailored to your child's abilities. This can help manage weight and improve overall health.

4. Family Support: Involve the entire family in the management plan. Educating family members about PWS and the importance of adhering to the meal plan can create a supportive environment.

5. Professional Guidance: Regular follow-ups with a multidisciplinary team, including a pediatrician, dietitian, and psychologist, can provide comprehensive care and support.


Nutritional Considerations
Regarding your concerns about high-protein foods and their impact on your child's health, it is essential to focus on a balanced diet that includes a variety of nutrients. High-protein foods can be beneficial, but they should be part of a well-rounded diet that includes fruits, vegetables, whole grains, and healthy fats. Avoiding excessive sugar and processed foods is also crucial.


Conclusion
In summary, while your child's excessive appetite and behavioral responses may raise concerns about PWS, a definitive diagnosis can only be made through proper medical evaluation. If PWS is confirmed, a structured approach involving dietary management, behavioral strategies, and professional support will be essential in helping your child lead a healthy and fulfilling life. Early intervention and consistent management can significantly improve outcomes for children with PWS.

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