Willi – Prader-walker syndrome is a disease which has no known cure. There are three forms of the disease: Willi-Willi (WVS), Spinocerebellar syndrome or Spinal muscular atrophy; and Wilson’s disease, which is also called Neuromuscular disease. Willi is caused by a mutation in the gene that causes cysts on the surface of the small intestine. Children affected by this disease may have a poor appetite and growth, weight loss and floppy hair. It can affect boys or girls. In children, it tends to be more common in boys and girls.
Wilson’s syndrome is caused by the same mutation in the cyst-producing gene but the cysts present in Willi-Willi and Wilson’s disease are not present in Willi. It is a rare genetic disease with about one percent of the general population being affected by it. A predominantly milk-fed baby is more prone to develop the syndrome than a bottle-fed baby. Treatment involves a strict diet and most children eventually become vegetarians due to deficiencies in essential vitamins.
The treatment for Willi – Prader-walker syndrome is largely limited to avoiding any nutritional deficiencies. It is advisable to avoid cow’s milk and formulas prepared to use cow’s milk as these will often aggravate the symptoms. Cereals, wheat, oatmeal and fruits are also to be avoided as much as possible. Fruits like apples, oranges, mangoes, bananas and avocados should be added to the diet and supplements should be taken if necessary.
It is important to start a strict diet at an early age to prevent Willi – Prader-walker syndrome. This disease is more common in children below five years of age and is more common in girls than boys. A proper diet helps normalize glucose levels in the body and prevents constipation, which is associated with the syndrome. It is also important to take care of intestinal health by eating a healthy balanced diet and supplementing it with adequate amounts of fibre, minerals, and vitamins.
There are a number of factors that can cause the syndrome. These include genetic disorders such as cystic fibrosis and sickle cell anemia. Environmental causes include allergies, poor nutrition, underdevelopment of the digestive system, chronic diarrhea and infection. Illness and injury to the head and neck are also frequent triggers. Psychological stress and traumatic experiences can also lead to this condition. Sudden infant death and SIDS are well known risk factors for Willi – Prader Walker syndrome.
The main objective of treating Willi – Prader Walker syndrome is to achieve normal growth and development. A specialized diet plan is designed for each individual case. It starts by evaluating the weight, the height and the head circumference of the child. In addition to taking into account the age and breed of the child, the type of milk to be fed and the duration of lactation must also be taken into consideration. Based on the evaluation of the child’s nutritional status, the doctor comes up with a customized diet plan.
There are certain criteria which serve as the basis for prescribing the unique diet plan for every patient. For instance, the diet should provide a balance between proteins and carbohydrates. Additional vitamins and minerals supplements may be added to the diet plan if necessary. The recommended amount of protein for infants with Willi – Walker syndrome is set at 20 grams per day. Milk and cereals are considered to be the ideal sources of protein for growing infants.
The usual duration of breast feeding is four months and the period of time spent on solid food can range from two to six months. Most doctors recommend that breast feeding is continued until the child reaches approximately eighteen months of age. A special formula has been developed by Dr. Willi – Prader Walker which includes all the essential amino acids and is therefore more beneficial than the standard formulas.