Nephrogenic Systemic Fibrosis (NSF) is a rare degenerative
disease that affects patients with serious kidney problems.
It causes a buildup of scar tissue -- fibrosis -- on the
skin and on several vital internal organs, including the
heart, lungs and diaphragm. Its main symptom is the sudden
appearance of skin lesions on the arms, legs, hands and
feet, sometimes accompanied by swelling, pain and blisters.
These lesions are painful and unsightly, looking a little
like the skin of an orange -- but more importantly, they
can be crippling. Over time, the hardened skin can prevent
patients from bending joints, robbing them of the ability
to walk and perform simple tasks. Patients may be bound to
a wheelchair within a few weeks. In addition, scar tissue
can build up so far on internal organs that it interferes
with their functions, eventually causing death.
Scientists have strongly associated NSF with the use of a
chemical called gadolinium -- a dye injected to make blood
vessels easier to see in magnetic resonance imaging and
magnetic resonance angiography (MRI/MRA) tests. Studies
have found that more than 95% of American NSF patients have
been directly exposed to gadolinium. In response, the U.S.
Food and Drug Administration has already ordered a "black
box" warning on the label of the dye, and research is
underway to confirm the connection. Preventive Measures
Unfortunately, the best treatment for NSF is still
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. More specifically, the agency has strongly
suggested that doctors avoid using gadolinium for MRIs or
MRAs in patients with chronic or severe renal insufficiency
(kidney failure), with a glomerular filtration rate at or
below 30 milliliters a minute; or in patients with any
level of kidney dysfunction caused by liver problems.
Doctors are not prohibited from using the dye with these
patients, but are strongly advised against it unless they
truly need it to make a diagnosis. Even then, they are
asked to make sure they do not use more than the
recommended dosage. Also, 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.
* A few patients have improved by using the drugs
thalidomide, pentoxifylline and cytoxin, but these have not
had widespread success. 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's true, doctors may someday
be able to prevent NSF by prescribing existing drugs that
inhibit TG2.
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.
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