Nephrogenic Systemic Fibrosis (NSF) is a rare but potentially fatal degenerative disease of the skin and internal organs. First identified in 1997, it occurs only in a few patients -- those who have a serious kidney disease and have undergone magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA) tests. Many scientists believe NSF is caused by exposure to gadolinium, a chemical used as a dye in MRI/MRA tests. In fact, one study showed that more than 95 percent of NSF patients had been exposed to some type of gadolinium within three months of contracting the disease. In response, the FDA has already recommended that doctors avoid gadolinium, and MRI/MRAs in general, in patients with kidney failure. Researchers in the United States and Europe are working to determine whether the substance should be banned altogether.
Scientists believe gadolinium may cause NSF because it reacts badly to high levels of acid in the body. Patients with kidney failure susceptibility to NSF is due to the fact that they are not able to process impurities out of their blood as well as people who do not have kidney failure. As a result of this, the acid level in their bodies is much higher, a condition which is known as acidosis. Some dyes containing gadolinium have been approved in the United States for MRIs and these brand names include:
* Magnevist
* MultiHance
* Omniscan
* OptiMARK
* ProHance
Brand names of gadolinium-containing dyes used in other countries include Dotarim, Gadovist, Primovist and Vasovist.
Painful, Debilitating Symptoms
NSF was originally called nephrogenic fibrosing dermopathy, because its first and most obvious symptoms are lesions or rough patches on the patient's skin. Over a period of days to weeks, patients notice a swelling and tightening of the skin, especially the skin on the arms, hands, legs and feet. This is often symmetrical, meaning that a spot on the patient's right arm will appear in a similar place on the left arm. As a result of this disease, the skin develops areas where it gets redder or darker that itch, burn, and send sharp pains through the area. On a microscopic level, scientists have found that these lesions are caused by a buildup of too much skin and connective tissue -- essentially, scar tissue.
Over time, the lesions harden and become "woody," resembling an orange peel. Eventually, this hardening makes it impossible for patients to bend their fingers, elbows, knees or other joints -- robbing patients of their ability to walk, perform daily tasks or even care for themselves. The organs are attacked by the disease in the same way which leads to an accumulation of scar tissue which could ultimately cease internal organs from functioning, which causes death.
* In addition to these symptoms, NSF patients have also reported: Blister-like spots on the hands
* Muscle weakness
* Bone pain in the hips and ribs
* Yellow lesions on or near the eyes
* Sudden development of hypertension (high blood pressure)
Confounding Diagnoses
Because NSF is only found in patients who already have kidney failure, its symptoms are often disguised by existing symptoms of a serious disease. Indeed, some scientists believe that NSF is underdiagnosed in kidney patients. In addition, NSF is often found about two weeks after the patient underwent a serious medical problem or a surgery, such as failure of a transplanted kidney, deep-vein thrombosis, angioplasty or another traumatic event that affects the blood vessels and the heart. Researchers are currently investigating whether these traumas cause NSF or are some of its effects. Many of them would be treated or diagnosed with MRI/MRA tests, suggesting that the tests -- and their use of gadolinium -- could be the problem.
Crippling Effects
Even if it didn't affect patients' independence, NSF would be disabling because of its painful and disfiguring effects on the skin. But because it also causes muscle weakness, joint pain and physical inflexibility in patients who already have serious kidney problems, NSF can be literally crippling, putting some patients in a wheelchair within weeks of diagnosis. In severe cases, patients may need assistance just to move, care for themselves and perform the daily tasks of living. For people who have fought hard to live independent lives after renal failure, this can be emotionally devastating as well. And NSF presents a very real threat of death: directly, through buildup of scar tissue on vital internal organs, or indirectly, through falls and accidents caused by the patient's loss of mobility. That's just too high a price to pay for a simple diagnostic test.
As of December 21, 2006, the U.S. Food and Drug Administration knew of at least 215 cases of NSF worldwide. Of these, all of the patients whose medical histories were reviewed in detail had been exposed to gadolinium in an MRI or MRA. Additionally, studies have shown that test people with NSF have found gadolinium in the skin lesions. While more research is needed to confirm these findings, this very strong correlation between gadolinium and NSF has already led to lawsuits across the country. As researchers begin to confirm the link between NSF and gadolinium, observers expect many more.
Patients with existing kidney issues are at risk of contracting a rare and deteriorating disease known as Nephrogenic Systemic Fibrosis (NSF). This disease attacks the skin and causes a development of fibrosis (scar tissue) on the skin and also on several vital inner organs such as the lungs, diaphragm, and heart. Symptoms of Nephrogenic Systemic Fibrosis include the abrupt presence of skin lesions on the feet, arms, hands, and feet. These lesions can be connected to swelling, blisters, and pain. These skin lesions can become hard and in areas that prevent people afflicted with the disease from bending joints, eventually disabling their ability to walk or even perform simple daily tasks. Patients with this stage of the disease may be forced to be in a wheelchair within just a few weeks. In addition to these symptoms, the internal organs may start to malfunction because of scar tissue that can develop. This stage of the disease is fatal
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. In addition, the FDA has advised doctors who administer gadolinium to send the patients that used the chemical to dialysis as quickly as possible after receiving 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. Improving kidney function has been able to counter the progress of NSF for some cases, but unfortunately, it has had no results in other cases.
* Oral steroids are pills that have improved the skin problems of some patients. These do not work positively for everyone and side effects include high blood sugar, calcium deficiencies, and ulcers which make them improper for people who have various other health problems.
*Ointments and skin creams such as vitamin D3 and types of cortisone have aided some patients in battling their skin symptoms.
*Physical therapy, especially deep massages and swimming have been ways that some patients can slow down the effect that NSF plays 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.
Additionally, a study conducted recently by the Wake Forest University Baptist Medical Center revealed that NSF can possibly be prevented if doctors would give patients medicine that impedes a type of bodily enzyme known as 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 accurate, then doctors might be able to prevent NSF someday by prescribing certain medications that suppress 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.
Peter Kent has sinced written about articles on various topics from Exhaust, Fitness and Health. Your source for everything legal on the web can be found at LegalView.com. To find an attorney and gain entry into complete legal databases, visit us at . This sit. Peter Kent's top article generates over 40500 views. to your Favourites.