HOW IS BIOTINIDASE DEFICIENCY TREATED?

People with biotinidase deficiency are unable to use naturally occurring biotin in their diets and are unable to recycle biotin. For successful treatment, they require free or unbound biotin supplements. The recommended dose of free biotin is 5-20 milligrams (mg) per day taken orally, which provides the body with the free biotin it needs. These large doses are 10 to 20 times more than the Recommended Dietary Allowance (RDA).

As discussed earlier, all children with biotinidase deficiency who have developed symptoms have improved following biotin treatment. Children with biotinidase deficiency who are carefully treated before symptoms occur rarely, if ever, develop any symptoms related to the disorder.

WHERE DO WE GET BIOTIN?

Your child's doctor must prescribe large doses of free biotin to treat your child. Diets containing egg yolks, liver, and certain vegetables are "rich" in biotin, but they are usually not effective in treating this condition because the biotin is protein-bound, not in a free form.

It is often difficult to obtain free biotin in adequate dosages from local pharmacies or health food stores. Your doctor can arrange to have the biotin purchased from a hospital pharmacy or pharmaceutical company that can supply the biotin in a form that your child can use.

Biotin is in the form of a crystal and is usually supplied by pharmacists in a capsule or tablet. The capsules can be opened or the tablets can be crushed to add the biotin to a baby's milk or a child's food. Biotin supplied in solution should not be used because the biotin often settles out of the liquid, possibly resulting in inappropriate doses. In addition, these solutions may be prone to grow bacteria. The biotin is usually given once a day, such as first thing in the morning, or may be divided into two doses each day, in the morning and in the evening.

The cost of biotin treatment varies, but is generally between 30 and 90 cents per capsule (5 or 10 mg) or between $100 to $350 per year. Unfortunately, some insurance companies do not cover the cost of prescriptions for vitamins including biotin. If you have had this problem with your health insurance carrier, you may wish to contact the company directly or ask your doctor to write a letter explaining the importance of biotin treatment for your child.

BIOTINIDASE DEFICIENCY REQUIRES LIFELONG BIOTIN THERAPY.

Our bodies need biotin throughout life, therefore, biotin therapy begins following the diagnosis and continues for life. No serious side effects of biotin treatment are known, although little information is available on the long term effects of biotin treatment. We do not expect that adults treated for biotinidase deficiency will have any new health problems from the disease or from the treatment. Treated biotinidase deficiency is not expected to pose a risk to mothers or their developing babies. However, because we do not yet have experience with pregnancies in women with biotinidase deficiency, research is needed in this area. Life expectancy for people with biotinidase deficiency is anticipated to be normal with proper treatment.

By following the progress of children treated for biotinidase deficiency, doctors are gaining a better understanding of the body's need for biotin.

WHAT IF WE MISS A DAY OF TREATMENT?

Missing a dose of biotin once in a while probably will not harm your child; however, not making an effort to follow the prescribed treatment may cause your child to develop symptoms of biotinidase deficiency. If at any time your child develops any of the symptoms of biotinidase deficiency, you should have the child examined by his or her doctor to determine if the dose of biotin needs to be changed.