Tsoi Tan Mei shares her story about raising a child with Prader Willi Syndrome.
My son, Hoi-yau, is a patient with Prader Willi Syndrome. He is now two and a half years old and has just entered a special child care centre* in Hong Kong.
Shortly after he was born, he was hospitalized in the high dependency unit for cryptorchidism (the absence of one or both testes from the scrotum). Later on, his doctors observed that he had low muscle tone and difficulty in sucking. They suspected that he could be suffering from Prader Willi Syndrome. His blood sample were sent via a genetic clinic to the USA for lab test. The suspicion was confirmed when he turned three months old.
Although it is a rare disease, we felt lucky that his attending doctor has other patients with Prada Willi Syndrome. When the doctor noticed that Hoi-yau’s symptoms matched some of those for this disease, he suggested to have Hoi-yau’s blood tested.
The doctor did not explain much about this disease when he first told us about it. He encouraged us to find out more from the Internet. Before the diagnosis of Hoi-yau was confirmed, we have read almost everything about Prader Willi Syndrome on the web. We learnt that patients with Prader Willi love to eat and suffer from developmental delay.
We were so sad when we received an email of the blood test results confirming that Hoi-yau has Prader Willi. As there was no medicine that could treat him, we could only start from managing his diet.
Hoi-yau began physical therapy, occupational therapy and speech therapy at six months old. We were frequently in and out of the hospital for follow-up checks and therapy appointments. We also arranged some private therapy sessions for Hoi-yau, despite the burden on our family expenses. It was lucky for Hoi-yau to be diagnosed early. It did not take long for him to be enrolled in a special education programme offered by the government. This really helped much in reducing the expenses for education.
In hopes of helping Hoi-yau’s development by increasing his muscle tone, we suggested to the doctor about giving him growth hormone injections. The doctor agreed. Since a year and five months old, Hoi-yau began receiving injections of growth hormone at home every night. His muscles became stronger.
Despite his developmental delay, Hoi-yau began to reach the major milestones of a baby. Every little improvement was exciting for us. The first time he crawled, stood up, took his first step and called us mom and dad – we remembered them all.
Since Hoi-yau likes to eat, we need to control his diet by counting the number of meals and limiting the amount of each meal. Basically, that means he has to stop after finishing one bowl, with no refill. He cannot have any snacks except for some biscuits during tea time. We keep food away from where he can reach them. Food cannot be given to him as an encouragement or reward whether it is at home or at school.
He is still young now, so he cannot reach too far. However, we know that we need to be more careful about keeping the food away when he grows up. It is important to keep a diet low in sodium, oil and sugar to control his calorie intake. His food is steamed or poached. Rarely does he eat pan-fried or deep fried food. For drinks, he takes water, chinese soup, diluted fruit juice, hi-cal milk and soya milk with reduced sugar. Definitely no soda or instant sweet drinks. Since Hoi-yau is still at his growing phase, there is no dietary treatment recommended by the dietitian. We simply keep to the principle of controlling the amount of food that is low in oil and sugar.
Considering that patients with this disease have a slower metabolic rate than the rest of us, they would need to do more exercise to burn the same amount of energy. However, due to lower muscle tone, they do not have as much strength and endurance. Whenever Hoi-yau exercises, he would ask to stop after doing two or three times. It is difficult for him to control his weight by exercise. Furthermore, he wants to eat whenever he sees food, making it difficult for us to know if he is really hungry or not! All we can do is to give him the same amount of food everyday, regardless of whether he has exercised or not. By doing this, he will not become overweight and need to shed some pounds afterwards, since it is really difficult for him to exercise.
We read from the internet that there is a wide range of severity for patients with Prader Willi Syndrome. Those with mild condition will not crave for food and can finish secondary school. However, those who are severe may steal food and run away from home. We hope that after four years of special care training in the center, Hoi-yau will have the same abilities of other children his age. Most importantly, he will be able to study in a mainstream school and maintain a standard weight.
Our daughter is five years old and healthy. Since we have experienced the developmental steps of a baby as first time parents, we are in no hurry for Hoi-yau to walk or talk. This makes it easier for us to accept that Hoi-yau is a rare disease patient. We know that he is making progress. It is just a bit slower. Give him more time, a little more training and he will be able to do it too. Our daughter understands why her brother is slower than others and needs to go to the hospital often. She knows that she needs to take care of him. For example, she helps to take note of what he eats and not give him any food without our permission. We hope that when our daughter grows up, she will continue to take care of her little brother.
Although there is no medicine at this time that can treat or cure patients with Prader Willi Syndrome, we are confident that we can help him through exercise and good eating habits. We encourage Hoi-yau to project his interest in food on something else. We read many success stories of patients who remain fit when they grow up.
“They are not hungry all the time. They just don’t know when they are not hungry.” We hope that everyone can remember this. Once we understand that they are getting full, we just have to stop giving them food and their diet can be controlled.
When there is a chance, we try to meet other patients and their families. It helps a lot to exchange our experiences with one another and share tips on how to care for children with special needs. This alleviates our stress as caregivers.
We are grateful to have met a doctor who knows about Prader Willi Syndrome. The earlier the diagnosis, the sooner the therapy and training. Hand-in-hand, our family is here to support Hoi-yau. We wish that there will be medicine available for controlling his appetite in the near future.
* Special child care center – Run by the government’s Social Welfare Department, these centers provide special training and care for moderately and severely disabled children to facilitate their growth and development, helping them prepare for primary education.