Treatment and Sources of Biotin
Currently the only known treatment for Biotinidase Deficiency is administering Biotin.
Our parents tell us Doctors prescribe anywhere from 5 – 10 mgs of Biotin Daily for profoundly affected individuals.
Below are some sources parents have provided:
At the time this webpage was updated Hoffman La Roche was the sole U.S. distributor of Biotin. If you know other sources please e-mail us to let us know and we will post it here.
1. Mericon Industries
8819 N. Pioneer Road
Does Insurance Pay for Biotin?
Because this is a medical condition many of us have worked with our insurance companies to encourage them to pay for the Biotin. Since Biotin is very inexpensive some of us have found it to be cheaper to purchase it outside our insurance plan. Each individual should research the cost savings based on their insurance plan.
What form of Biotin do I give my child?
Tablets, capsules, pharmacy to compound.
Some parents report opening up capsules and putting them into babies milk.
Older children are sometimes given Biotin in their apple sauce or other foods.
Free biotin or biotin that is not bound to protein or other molecules is the treatment for biotinidase deficiency. Biotin in essentially all available vitamin preparations is free biotin. Biotin in many foods from animal sources is usually not free biotin and should NOT be used as a source of biotin to treat biotinidase deficiency. The biotin in yeast is almost entirely bound and is NOT useful in biotinidase deficiency. Although some or most biotin in vegetables and plants may be in the free form, the quantity is too small to be therapeutic. Therefore, to be sure that the biotin is in the free form and it is in sufficient amounts, biotin supplements are used to treat biotinidase deficiency.
A note from Dr. Wolf:
The issue of how to give the biotin seems to be a popular one and also is a very important one. Let me try to help and give the experience of over 20 years. First, biotin is a crystalline substance that makes up very little of the total of powder in a capsule or crushed tablet. Second, most of what makes up the powder in a capsule or tablet is filler. Third, biotin is considered to be a water-soluble vitamin, but it is not very easy to get it soluble in water or liquids with water especially when in combination with the powder. Fourth, when biotin is cooled or cold it is even less soluble. Therefore, when you mix the biotin material in a capsule or crushed tablet with milk, it essentially is not dissolved, but is in a suspension or mixture that tends to settle with time.
Most of the biotin preparations that we recommend have 5 or 10mg per capsule or tablet. There is no need to use many capsules of tablets of low doses. It is not good to use capsules and tablets that contain other vitamins, etc. in them either. Meribin (5 mg of biotin capsules) from Mericon Industries in Peoria, Illinois is great (I don't get any kickbacks!).
Be careful in the doses! We give 5 or 10 milligrams (mg) of biotin to kids with profound BD. Micrograms (mcg) is a very tiny dose (1/1000th of a milligram) and is not adequate. 1 mg is equal to 1000 mcgs!
For these reasons, we do not recommend that the biotin be put into a liquid or syrup and stored in a refrigerator. Much of the biotin is probably not in solution, has a tendency to settle unless thoroughly shaken, and even then likely sticks to the bottom or sides of a container. In addition, biotin sitting in syrup for long periods of time is likely prone to grow bacteria. So when biotin is added to a bottle of milk, much of the biotin may never make it to the nipple and, if it does, would have trouble getting through the nipple, unless the nipple hole is enlarged.
Fortunately, the amount of biotin in the volume for a daily dose of about 5-10 mg per day used in profound deficiency is more than enough and it is not crucial or critical that every dose is exact. However, for a consistent daily dose we recommend taking the contents of a capsule or crushed tablet in a small spoon or a medicine scoop (available at most drug stores). Then add a small volume of milk (formula or expressed) or water and make a slurry. This suspension is then placed into the mouth of the baby. A 3 or 5 cc syringe (without a needle!) can also be used to deliver the slurry into the mouth. Give it in volumes as tolerated until gone. Some babies take the whole dose at one time, whereas others take a little at a time until gone. Give some milk between administrations to help wash the biotin down. This way the dose is consistent and the child takes the whole amount regularly. As the child gets older, you can add the powder to applesause or other similar foods that allow the whole dose to be taken in a small volume (and the kids enjoy taking). Eventually, the kids are old enough to swallow the capsule or tablet whole.
There is always many other ways to get to the same end and I am here to learn from all of you. I hope this information helps. I am very grateful for the support group and everyone's suggestions and comments. I am here to help when I can.
For additional information email Dr.