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My son (who has PSC) and I have recently been diagnosed with ataxia

telangiectasia (a genetic disorder ) that includes immune deficiencies among

other things.I believe that his immune deficiency caused his chronic infections,

that caused his PSC. He is doing better and stable with his PSC at least since

he started IVIG infusions and vancomycin treatments.

This is very interesting info from our doctor's -Dr Melamed's web site

Braden and I get IVIG

I thought the therapeutic apheresis looked really interesting !

Treatment Therapies

IVIG—IVIG is an acronym for " intravenous immunoglobulins " . Immunoglobulins are

antibodies released into the bloodstream. These immunoglobulins are extracted

and purified from large donor pools. Following comprehensive evaluation, IVIG is

administered intravenously to patients who cannot produce sufficient amounts of

their own antibodies and whose immune systems have been compromised.

Immune Replacement with IVIG—In cases of primary immune deficiency, where

components of the immune system are missing, IVIG is used as replacement

therapy.

Immune Modulation—In the case of secondary immune deficiency, studies have shown

that IVIG can be used to restore balance to a dysregulated immune system.

Correction of the imbalance by immune modulation using IVIG is prescribed.

Therapeutic Apheresis (TA)—Therapeutic Apheresis (TA) is a procedure in which

pathogenic (disease-causing) components are removed from the bloodstream. When

the level of these components is lowered, a concomitant decrease in disease

severity is noted.

During TA, a procedure which is performed by specially trained personnel using

highly sophisticated equipment and technologies, a unit of blood is withdrawn

from the patient into the TA machine. The machine separates the blood into its

components and removes the desired fraction from the unit. Finally, it is

transfused, along with the volume of necessary replacement fluid, back into the

patient. If plasma is removed, it is called plasmapheresis or plasma exchange;

if platelets are removed, it is termed plateletpheresis or for white blood

cells, leukapheresis.

In autoimmune disorders where an increase in the level of autoantibodies is

found, TA allows for the specific removal of the antibodies related to the

disorder. TA is utilized in the acute phase of autoimmune disorders to remove

the increased amounts of circulating autoantibodies due to an exacerbation of

the disorder. Through TA, one unit of plasma exchange reduces the level of

circulating autoantibodies by 60%. Within 24 hours after TA, extravascular

antibodies travel to the vascular system, enabling another course of TA to

become effective. After 3–5 courses of plasmapheresis, a reduction of 90% in the

levels of these antibodies is noted.

Other forms of TA are indicated by the disorder. The removal of components in

the blood, either cellular or protein, that aggravate the disorder can be

accomplished through the use of TA.

Other Infusables

Remicade: Medications TNF-alpha inhibitor works by blocking the action of

TNF-alpha, substance in the body that causes inflammation. Relives the symptoms

of certain autoimmune disorders.

Xolair: Blocking the IgE Receptor. Approved for asthma.

Tysabri: Monotherapy treatment for relapsing forms of Multiple Schlerosis

Other Avenues of Immune Enhancement

In recent years, studies have established a correlation between nutrition and

stress and the increase in immune-related diseases. Part of the increase in

immune-related diseases can be attributed to escalating stressful events and

nutritional imbalances, along with many other factors. As a result of our

continuing research into this area.

We are exploring various avenues that can enhance our immune system:

* Balanced vitamins and trace elements

* Balanced nutrition

* Improved lifestyle

* Reduced stress

Lori

lucky mom blessed with triplets

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