Often hungry but have eaten a lot, maybe a symptom of prader – willi

After eating, you may expect to feel full. However, this is not the case if you are diagnosed with a genetic disorder known as Prader – Willi syndrome. This disorder, whose medical name is quite difficult to pronounce, causes a person to be often hungry even though they have just eaten a lot. Here's all the information you need to know about Prader – Willi syndrome.

What is Prader – Willi syndrome?

Prader – Willi syndrome (PWS) is a genetic disorder that affects a person's appetite, growth, metabolism, as well as cognitive function and behavior. PWS is characterized by low muscle tone (weak muscle contraction), short stature due to not getting enough growth hormone, cognitive disabilities, behavioral problems, slow metabolism which can cause a person to be hungry often but never feel full.

Complaints of frequent uncontrolled hunger - whether by limiting access to food, therapy, maintaining body weight, and medications - can lead to overeating and lead to deadly obesity.

It is estimated that one in 12,000 to 15,000 people in the world has PWS. PWS can occur in any family, both male and female, and in all races around the world. But despite being considered a rare disorder, PWS is one of the most common types of eating disorders and is the most common genetic cause of obesity to date.

What causes this frequent hunger disorder?

Prader – Willi syndrome has no known cause. However, this eating disorder is a genetic mutation disorder that occurs at or near the time of conception for unknown reasons. The number of chromosomes in a normal human body is 46, but Prader – Willi Syndrome is always caused by a mutation in the 15th chromosome that is inherited from the father.

This mutation causes about seven genes on the chromosome to be lost or not fully expressed. This father-derived chromosome plays a role in regulating appetite and sensations of hunger and fullness. Therefore, gene deficiency or defects due to mutations can cause a person to be hungry frequently without ever feeling full.

PWS can also be acquired after birth if the hypothalamus part of the brain becomes damaged due to injury or surgery.

What are the signs and symptoms of Prader – Willi syndrome?

The symptoms of Prader Willi Syndrome can vary from person to person and can change with age.

Prader – Willi syndrome signs and symptoms in newborns, including:

• Has special facial characteristics - too small eyes shaped like almonds, too small forehead and thin upper lip.
• Weak sucking reflex - caused by imperfect muscle mass that makes it difficult during the breastfeeding process.
• Low response rate - marked difficulty waking from sleep and a low crying sound.
• Hypotonia - characterized by low muscle mass or the baby's body is very soft to the touch.
• Incomplete genital development - more easily recognized in men such as a penis and scrotum that is too small or a testicle that does not descend from the abdominal cavity, whereas in women the genital areas such as the clitoris and labia appear too small.

Signs and symptoms of Prader – Willi syndrome in children to adulthood, including:

Other symptoms can only be detected when the sufferer enters the age of children and persists throughout their life. This causes Prader Willi Syndrome to be treated appropriately.

• Plenty of eating and rapid weight gain - can appear as early as two years of age and cause a child to feel persistently hungry. Abnormal eating patterns such as eating raw food to things that are not supposed to be eaten can occur. A person with Prader Willi Syndrome is very at risk of obesity and its complications such as diabetes mellitus, cardiovascular disease, sleep apnea and liver disease.
• Hypogonadism - characterized by the production of sex hormones that are too little so that they do not experience normal development of sex and physical organs, late puberty to infertility.
• Incomplete physical development and growth - characterized by shorter height in adulthood, too small muscle and too much body fat.
• Cognitive disorders - such as difficulty thinking and solving problems. Even without significant cognitive impairment, learning disorders can still be experienced by people who live with it.
• Motor problems - the ability to walk, stand and sit in Prader Willi Syndrome children is slower than children their age.
• Speech disorders - such as difficulty pronouncing words and articulation - can still occur into adulthood.
• Behavioral disorders - such as showing stubbornness and anger and difficulty controlling emotions when not getting the food he wants. They also tend to have compulsive repetitive patterns of behavior.
• Other physical characteristics - having hands and feet that are too small, crooked spine (scoliosis), disorders of the waist, disorders of the salivary glands, vision problems, high tolerance to pain, skin, hair and eyes tend to be paler

Can Prader-Willi syndrome be cured?

Prader – Willi syndrome cannot be prevented and cannot be completely cured. However, the symptoms can be controlled if diagnosed as early as possible. Prader Willi syndrome can be recognized and differentiated from other genetic disorders such as down syndrome based on the person's history of symptoms.

Early detection can be done when a newborn shows symptoms of hypotonia of unknown cause and if the baby has weak reflexes when breastfeeding. Confirmation of the diagnosis requires a DNA methylation test and fluorescent in situ hybridization (FISH) to detect any genetic disorders.

If the cause of your frequent hunger is true Prader – Willi syndrome after being tested in the hospital lab, your doctor will advise you to take hormone therapy to maximize the release of growth and sexual hormones for genital development. In addition, you also need a strict diet to control your appetite and calorie intake. Social and occupational therapy as well as mental health therapy are also needed to prepare you to socialize in your surroundings more comfortably.

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