Treatment of ASAL

You should consult 

• Medical geneticist
• Metabolic disease specialist
• Pediatric critical care specialist
• Dietitian

*When your/a child has symptoms of ASAL Deficiency, it is advised to seek immediate treatment so as to prevent build-up of ammonia.*

Henceforth are treatment methods often recommended for children/babies diagnosed:

1.  Low-protein diet and/or special medical foods and formula

The diet that ASAL deficient children need to eat consists of very low-protein foods, special medical foods and possibly a special formula. The dietician will come up with a meal plan that contains sufficient quantity of protein, nutrients and energy (calorie). This meal plan should be continued throughout the child’s life.

A low-protein diet is the most effective treatment. Foods that need to be avoided or strictly limited include:

• milk, cheese and other dairy products

• meat and poultry, eggs, fish

• dried beans and legumes

• nuts and peanut butter

Consuming high-protein foods will cause accumulation of ammonia in the blood, harmful to the body. It is necessary to still consume protein. Examples such as vegetables and fruits can be eaten, in measured amounts.

Medical foods and formula

There are special foods that cater to people with amino acid disorders. Examples are special low-protein flours, pastas and rice. Special formula that contains the right amount of nutrients may be given as well. The above mentioned meal plan will always be adjusted under the guidance of a dietician, with the growth of a child.

2.  Medication
Most ASAL deficient children are given oral arginine supplements. Arginine helps the body to remove ammonia from the blood. There are also other medications for prevention of high ammonia levels. In the event of high blood ammonia, children should be taken to the hospital. More often, IV is one way medication is introduced into the body. Sometimes dialysis is needed to remove ammonia from the blood.

3.  Blood tests  
Regular blood tests is needed to measure amino acid and ammonia levels of the child. The child's diet and medication may need to be adjusted based on blood test results.

4.  Call the doctor at the start of any illness:
For some babies and children, even minor illness can lead to build up of ammonia. In order to prevent problems, call a doctor right away when your child has any of the following:

• loss of appetite
• low energy or extreme sleepiness
• vomiting
• fever
• infection or illness
• behavior or personality changes
• difficulty walking or balance problems

5. Liver transplantation
Liver transplant surgery has been tried in some children with ASAL who do not respond to dietary treatment. Liver transplant in children with ASAL deficiency does not cure the underlying disease and is only done when necessary.

This major surgical procedure is associated with risks, and individuals who have had a liver transplant must take medication for the rest of their lives to prevent their body from rejecting the donor liver. Many factors must be considered before surgery and this option should be discussed thoroughly with your child’s doctors.