Angiogram, MRI, CT Scan Contrast Dye The FDA reports hundreds of patients with moderate to end-stage kidney disease that progressed into Nephrogenic Systemic Fibrosis or Nephrogenic Systemic Dermatopathy. NSF/NFD is often misdiagnosed as scleroderma or myxederma after having dye or contrast injected from either: An MRI scan takes a picture of a patient's internal organs and tissue. An MRA makes a photo of a patient's blood vessels and heart. A CT of CAT scan takes photos of tissues and organs. A gadolinium-based contrast agent is injected into the patient's vein which can have devastating effects on the kidneys, skin and joints. ►Click the link to See Video: How Gadolinium Can Cause NSF If the video window doesn't open, please click here. NSF/NFD, Nephrogenic Sclerosing Fibrosis and Nephrogenic Fibrosing Dermopathy occur when there is a buildup of collagen in tissues that results in thickening and hardening of the skin. NSF/NFD can result in either partially or completely immobilizing patients, but in the most severe cases can cause death. NSF/NFD is a serious and debilitating disease that most commonly affects persons who have mild, moderate, or severe kidney disease. Symptoms of this progressive disease can develop within days, months, or as late as one year after receiving an MRI or MRA. Symptoms include: - burning and itching
- swelling, tightening and thickening of the skin
- muscle weakness
- stiffness in joints
- red or dark patches on the skin
- yellow spots on the whites of the eyes
- pain in the hips or ribs
- fibrosis or scarring of body organs
Patients may experience multiple symptoms, but not necessarily all symptoms listed above. These symptoms may develop within days or months (sometimes up to 18 months) after having an MRI or MRA scan. Warning to Health Care Providers: It is important that patients be screened for kidney problems prior to undergoing an MRI or MRA scan. NFD is a fibrotic disorder of the skin which occurs in patients with kidney disease or renal failure. This disease was first reported in 1997. The only known risk factor for developing NFD is exposure to gadolinium. Since 1997, hundreds of cases of NFD have been reported and reports have shown a link between NFD and gadolinium based contrast agents used in conjunction with MRI and MRA scans. NSF/NSD FACTS
Nephrogenic systemic fibrosis, formerly known as nephrogenic fibrosing dermopathy (NFD), is a debilitating, potentially fatal disease which causes a thickening of the skin, connective tissues, muscles and internal organs throughout the body. There is no known cure for NSF, it becomes worse over time and can lead to death.
How Common is NSF?
Nephrogenic systemic fibrosis is a relatively new and rare disease. The first reported case of NSF in the world was in 1997. The NSF Registry, run by Yale University, collects and organizes information about NSF from all over the world. As of now, over 215 cases of NSF have been confirmed worldwide.
What Causes NSF?
The exact cause of NSF is still unknown, but research has confirmed that exposure to gadolinium dyes triggers the disease in certain patients. So far, NSF has only occurred in people with kidney disease. Neither the duration of kidney disease nor its underlying cause are related to the development of NSF. Patients have developed NSF in the earliest and latest stages of kidney disease.
Case studies have revealed that the following events have occurred prior to the onset of NSF in some patients: - Thrombotic episode (i.e., deep venous thrombosis)
- Recent surgery (particularly vascular surgery, i.e., angioplasty of a blood vessel);
- Coagulation abnormalities (blood clotting);
- Recent failure of a transplanted kidney;
- Sudden onset kidney disease with severe swelling of the extremities.
Researchers are currently investigating to see if any of these events could be a trigger for NSF. It is important to note that many of these events are preceded or followed by MRI or MRA exams. So the common denominator linking these events to the development of NSF may just be the gadolinium-based contrast dyes used with the MRI and MRA exams.
Who is at Risk for Developing NSF?
So far, NSF has only developed in patients with kidney impairment. Although the cause of NSF has not yet been discovered, medical research has confirmed that NSF can be triggered by exposure to gadolinium. Gadolinium is found in certain contrast dyes used with MRI and MRA exams. These dyes are collectively referred to as gadolinium-based contrast agents (GBCAs).
The following patients are believed to be at high risk for developing NSF if they are exposed to gadolinium dyes:
Patients with severe kidney insufficiency;
Patients just before or after liver transplantation, who also have kidney insufficiency; and
Patients with chronic liver disease, who also have kidney insufficiency.
FDA warnings have been issued about these life-threatening risks. So far, NSF has not occurred in patients with normal kidney functioning.
The typical patient is middle-aged and has end-stage renal disease (ESRD). Most, but not all, are on regular dialysis treatment or have undergone dialysis for kidney failure. Many patients have a history of taking immunosuppressive medications or have other diseases, such as hepatitis C. NSF appears to affect males and females in equal numbers. Although most NSF patients are middle-aged, it has occurred in children and the elderly. NSF has been confirmed in patients ranging from 8 - 87. NSF patients come from a variety of ethnic backgrounds and from various continents across the world, including North America, Europe, and Asia.
What are the Symptoms of NSF?
Some patients have experienced episodes of hypertension (high blood pressure) before the NSF symptoms appear. These patients have never had high blood pressure before and the cause of these episodes is unknown.
The typical course of NSF begins with swelling of the hands and feet (and sometimes the calves and forearms). Symmetrical darkened or reddish patches, bumps, or blisters appear on the skin. They appear most commonly on the calves and forearms, and sometimes on the hands and feet. Some patients have reported yellow bumps on or near the eyes.
This is followed in subsequent days or weeks by severe hardening of the skin in the swollen areas, which sometimes spreads to the thighs, trunk, lower abdomen, and/or buttocks. It almost never affects the head and neck. The skin may feel "woody" or like an orange peel. The skin hardening may be associated with burning, itching, or severe, sharp, constant pain in the affected areas. Loss of skin flexibility, muscle restlessness, and muscle weakness are common. Deep bone pain in the hips and ribs is sometimes reported. In some cases, the thickened skin inhibits the movement of the joints making it hard to bend and straighten the arms, legs, hands, and feet. The patient may develop contractures, which is an abnormal, sometimes permanent, shortening of the muscle or connective tissue. This can lead to serious physical disability, often necessitating the use of a wheelchair.
The skin symptoms may be followed by internal symptoms affecting the lungs, heart, liver, muscles and diaphragm. Thickening and hardening around internal organs can cause potentially fatal complications.
NSF has been linked to exposure to gadolinium dyes. So far, according to study results, NSF patients have experienced the first NSF symptoms between 2 weeks and 3 months after the MRI or MRA procedure. This time range may change as more data becomes available.
In some cases, the disease progresses rapidly, (it may run its entire course in several months), while in other cases, it progresses slowly. Many patients become dependent on a wheelchair within weeks of the first NSF symptom. When NSF has a sudden intense onset and severe rapid progression, it may be more likely to cause fatal complications.
How is NSF Diagnosed?
There is no specific test used to diagnose NSF. A diagnosis is generally made based on the clinical course, dermatologic findings, and skin histology (biopsy). The doctor will gather the medical history, conduct a physical examination, and take a skin biopsy which will be examined under a microscope.
NSF can be difficult to diagnose. It is a new and rare disease that most doctors have never seen before and many NSF symptoms are also symptoms of other conditions. When the disease progresses slowly, it may take even longer to properly diagnose.
What is the Treatment for NSF?
NSF gets worse over time and as of yet there is no cure. There is still no established treatment for NSF, but it appears that improvement of kidney function seems to slow the disease down. Many different treatments have been tried and are currently being investigated. Following are some of these treatments:
Extracorporeal photopheresis (ECP) - a medical therapy used to treat skin cancer and other conditions. The patient's blood is drawn intravenously and white blood cells are separated before the blood is returned to the body. The white blood cells are then mixed with a liquid medication that makes the cancer cells sensitive to ultraviolet light. The white blood cells are then exposed to ultraviolet light which kills the cancer cells. The blood is then returned to the body.
Plasmapheresis - a medical therapy used to treat myasthenia gravis and other autoimmune conditions. The patient's blood is drawn intravenously and the fluid part of the blood (plasma) is separated from the blood cells. The cells are returned to the body and the plasma is discarded and replaced with a plasma substitute. This has helped some NSF patients with dual liver/kidney transplants.
Oral steroids (Prednisone) - Medications used to treat a wide variety of conditions, including diseases of the skin, i.e., psoriasis, Stevens-Johnson syndrome, and severe seborrhea. These have helped some NSF patients but are not recommended because of their side effects.
Reduction of ESAs - Many kidney patients are treated with anemia drugs called erythropoiesis-stimulating agents (ESAs), i.e., Procrit, Aranesp, and Epogen. Lowering the dosage of these drugs can sometimes help NSF symptoms.
Topical Dovonex - Medicated cream or ointment used to treat psoriasis. This may improve skin lesions in the early stages of NSF.
Cytoxan - A medication used to treat different types of cancer.
Thalidomide - Medication used to treat myeloma and leprosy. This drug causes severe birth defects and is part of a special distribution program regulated by the FDA. It can only be prescribed and dispensed by doctors and pharmacists who are registered with this program. Some NSF patients have shown improvement with this, but long-term side effects may be a problem.
Pentoxifylline - A vasodilator medication used to treat chronic occlusive arterial disease of the limbs. Is supposed to aid circulation. Has helped some NSF patients.
Plaquenil - Medication used to treat malaria, lupus, and arthritis. Has helped some NSF patients, but can cause side effects in the eyes and must be closely monitored.
Minocycline or similar antibiotics - Some NSF patients have shown improvement on this type of antibiotic, but gastrointestinal and other side effects may be a problem.
High dose intravenous Ig therapy - Immunoglobulin is administered through an IV. This is used to treat leukemia, HIV in children and several other diseases.
Physical therapy - Swimming and deep massage has been reported to help relieve some NSF symptoms and help slow the progression of joint problems.
Ultraviolet therapy - A medical therapy that uses ultraviolet electromagnetic radiation. It is used to treat diseases of the skin.
Renal transplantation - Some patients' NSF has improved following kidney transplant.
What is the Prognosis for NSF?
NSF generally gets worse over time. Sometimes the disease progresses rapidly and sometimes it develops slowly. As of yet, no cure has been discovered.
There have been reports of patients experiencing a gradual improvement in mobility and a slight softening of the hardened skin over time. But complete spontaneous healing has not been reported in patients with ongoing kidney disease.
An estimated 5% of NSF patients experience sudden and severe symptoms with a rapid progression of the disease which may result in death. The cause of death is not the NSF itself, but rather the complications caused by the NSF, i.e., restricted breathing and complications from fractures after falls caused by mobility problems.
NSF is a debilitating disease. Many NSF patients could have avoided the disease if the drug companies had properly tested and researched their gadolinium dyes and warned the public about the serious risks involved. If you or someone you know has suffered from NSF symptoms following exposure to gadolinium dyes, contact our offices for a free case evaluation by one of our pharmaceutical attorneys.
Help for NSF Patients
NSF is a devastating disease that completely disrupts people's lives. Most people have never heard of it and even doctors don't know that much about it. Patients and their families need to know as much as they can about the disease to help them cope with it.
While the Food & Drug Administration approved gadolinium for use in MRIs, which visualize organs and tissues. Gadolinium has never been approved for use in Magnetic Resonance Angiography (MRA) or Angiograms which visualize the blood vessels inside the body for heart catheterizations and visualization.
Brand Names of Gadolinium Contrast Dyes that are the subject of pending lawsuits include:
· Omniscan made by GE Healthcare
· OptiMARK made by Mallinckrodt/Tyco Healthcare
· Magnevist made by Bayer/Schering AG/Berlex
· ProHance made by Bracco Diagnostics
· MultiHance made by Bracco Diagnostics
However, the use of gadolinium contrast dyes place patients with kidney disease or renal failure at risk for developing Nephrogenic Systemic Fibrosis (NSF). Typically, patients suffering from kidney disease or renal failure who undergo an MRI are subjected to much higher levels of gadolinium than a healthy patient. Patients with kidney disease or renal failure are unable to timely excrete the toxic levels of gadolinium from their system, resulting in risk for developing NSF/NFD.
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