While Nephrogenic Systemic Fibrosis (NSF) is a rare and painful degenerative disease usually affecting individuals with kidney disease, scientists are researching treatment options for these patients. To better create treatments for NSF, individuals should know what the disease is associated with. Usually those receiving MRIs or MRAs for their kidney disease are injected with a dye known as gandolinium. Scientists have linked gandolinium to NSF.



Studies have found that more than 95% of American NSF patients have been directly exposed to gadolinium. In response, the U.S. Scientists are researching the dye and its connection to NSF, in addition to that, the FDA has ordered a "black box" warning on gandolinium labels. Preventive Measures Unfortunately

Treatment of NSF is still based around prevention. The FDA has issued guidelines three times since June of 2006, each strongly suggesting that doctors avoid using gadolinium in patients with moderate to end-stage kidney disease. For patients with severe kidney failure, it is suggested that medical professionals avoid using gandolinium for MRAs and MRIs; or in patients with any level of kidney dysfunction caused by liver problems. It is advised that the dye is discontinued for use in these types of patients unless absolutely necessary. Even then, they are asked to make sure they do not use more than the recommended dosage. And the FDA has asked doctors to consider sending these patients for dialysis as soon as possible after the MRI.

Experimental Treatments Currently, there is no consistently successful treatment for NSF. In fact, the disease is so new -- it was first identified in 1997 -- that doctors are still investigating several treatments to see which give patients the best long-term relief. Treatments that have helped some patients include:

* Improving kidney function is the treatment with the most consistent good results. This can mean medical treatment (including dialysis), a kidney transplant or both. While improved kidney function has actually reversed the course of NSF in some cases, it has had no effect in other cases.

* Oral steroids are pills that have improved the skin problems of some patients. They do not work for everyone, and may cause calcium deficiency, high blood sugar and ulcers, making them inappropriate for many people with some other health problems.

* Skin creams and ointments, such as vitamin D3 and forms of cortisone, have helped some people fight their skin symptoms.

* Physical therapy, especially swimming, and deep massage have helped some patients slow down NSF's effect on their joints.

While several individuals have seen a decrease in symptoms when using thalidomide, cytoxin and pentoxifylline, the drugs have not had a widespread success that many were hoping for. Similarly, two types of blood treatments -- plasmapheresis and extracorporeal photopheresis -- and ultraviolet light therapy have been helpful for one or two patients. But in all of these cases, more research is needed.

In addition, a recent study from Wake Forest University Baptist Medical Center suggests that NSF could be stopped before it starts if doctors are able to administer drugs that inhibit a bodily enzyme called transglutaminase-2 (TG2). These researchers found high levels of TG2 in patients with NSF, which suggests that the enzyme helps activate the disease. If that is in fact the truth, medical professionals may be able to prescribe drugs inhibiting TG2 that will prevent NSF. Nephrogenic systemic fibrosis is a deadly, incurable disease that can disable and eventually kill a patient, often a patient who is already weakened by kidney failure. It is also completely preventable -- if doctors work closely with patients and their loved ones to avoid using gadolinium in kidney patients. As of late December 2006, the FDA had identified 215 patients with NSF around the world; every single one whose medical history they could review had been exposed to gadolinium. If you or a loved one has developed NSF after being given a dye with gadolinium for an MRI or MRA, you deserve answers.