Treatment Name

IVIG monthly infusions

FDA Status

Other

Company

CSL Behring

Official Description

Privigen is the first and only immunoglobulin (IVIG) therapy made with proline. Privigen is made using advanced purification and pathogen inactivation/removal technology. The manufacturing process involves multiple steps designed to help make a highly pure and safe IgG solution. In clinical research studies, Privigen helped patients with primary immunodeficiency (PI) achieve a low annual rate of serious bacterial infections.

Privigen provides the missing protective antibodies (replacement therapy). Privigen is manufactured from the pooled plasma from up to 60,000 blood donors and, therefore, includes a broad variety of antibody specificities against common pathogens to which the donor population has been exposed. Moreover, the antibodies in Privigen are structurally and functionally intact and their effector functions are fully operative.

Expanded Description

IVIG has been used for over a decade in Europe, but especially so in the past 5 years, to prevent episodes of SCLS — and the few results that have been reported, and the personal testimonials found in this RareShare SCLS site, are very encouraging. (See the list and links to scientific articles in the Disorder Resources section.)

The apparent success of IVIG suggests that SCLS may be an illness that follows from an acquired immune deficiency.

The recommended dosage is 2 grams per kilo (of patient weight) per month, infused intravenously during two sessions over a two-day period. For example, an individual who weighs 100 kilos would get a solution of 100 grams of IVIG on the first day and another 100 grams on the second day. The infusion rate is slow during the first hour and is then increased in the second hour as per the manufacturer’s recommendations. The procedure usually takes about 4-5 hours per day. The initial side effects include headaches and allergic reactions, and thus it is customary practice for patients to be given something like Tylenol and Benadryl a half hour before getting their IVIG, and to be monitored closely the first few times they are infused for any other side effects.

IVIG has been tried in several patients in the United States and Canada in the past couple of years with great success, but its use for the prevention of episodes of SCLS has not received FDA approval. It is rightly considered an experimental, off-label use of an otherwise FDA-approved medication.

There has never been anywhere in the world — and there probably never will be — the kind of well-designed, clinical trial results published in a peer-reviewed medical journal that would lead the FDA and medical insurance companies to agree to cover the (high) cost of IVIG because it has been proven to be “medically necessary” for the treatment of SCLS. Therefore, in the United States, one has to make a personal appeal, backed by doctors’ testimonials, in order for IVIG to be covered on an experimental basis — and said coverage may be denied, or granted only on appeal, or only temporarily.

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