Pulaski & Middleman Launch New Nephrogenic Systemic Fibrosis Helpcenter

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The Lawyers of Pulaski & Middleman have launched a new Nephrogenic Systemic Fibrosis (NSF) helpcenter to help the families and victims of this disease. This is due to the lack of information generally available to the public concerning the disease and its treatment options.

The lawyers of Pulaski & Middleman, in conjunction with Simmons Cooper, L.L.C., have launched NSFhelpcenter.com. This was created so that individuals would have a forum to share information and to discuss problems arising from Nephrogenic Systemic Fibrosis and to remain apprised of the most up-to-date information and medical literature regarding treatment and diagnosis. In addition, we hope that this website will raise the general public's awareness of this disease and may help to increase research efforts designed to find a cure for NSF.

In February 2007, physicians who read Renal and Urology News were alerted to an updated public health advisory, issued by the FDA, regarding the development of a disorder of skin, muscle, and body organs, in some patients with end-stage renal disease (ESRD), who had been exposed to gadolinium-based contrast agents during magnetic resonance (MR) imaging. That disorder, currently referred to as NSF or NFD, can be associated not only with pain and physical disability, but with mortality.

Now, there is concern that cases of NSF are not being properly diagnosed because of a lack of available information regarding the presentation and diagnostic criteria for this relatively newly discovered disease. The incidence of this disease now appears to be greater than previously reported. Manufacturers of the gadolinium-based contrast dyes have recently begun to inform physicians of the relationship between the dye and NSF; however, more information needs to be made available so that individuals presenting with symptoms of NSF can be assured that all physicians, including general practitioners, internists and other physicians have been made aware of what to look for.

NSF can manifest in a variety of ways. Although the literature suggests that patients typically have swelling, hardening, and tightening of skin over the extremities, some present only with skin-colored, pigmented, or slightly reddened, occasionally pruritic, papules, in the absence of pain and joint contracture. A yellowish, scleral plaque, when eyes are examined, may be an indicator of systemic involvement. Furthermore, NSF is not limited to end-stage renal patients. Some, who had an MRI with Gadolinium during a temporary period of renal insufficiency such as a Kidney Injury, do not have renal insufficiency at the time signs and symptoms become apparent. The disease may not appear for more than a year after gadolinium contrast exposure. Patients with delayed manifestations may not even recall an MR examination or may have a medical record of MR evaluation that is ambiguous with regard to use of a contrast agent. Prompt hemodialysis after gadolinium exposure does not always prevent NSF. The condition is not confined to the adult population; it does affect children.

Patients may be misdiagnosed as having cellulitis, pretibial myxedema, morphea or scleroderma. The disorder has been reported in the breast, mimicking inflammatory carcinoma, and in patients complaining only of "deep lying" pain, where it can be misdiagnosed as degenerative osteoarthropathy.

NSF appears to be related to an inability to clear gadolinium-containing contrast from the body in timely manner. Some gadolinium-containing agents are ionic, have increased osmolarity, and can actually cause acute renal injury (thereby leaving a patient susceptible to developing NSF). Gadolinium contrast is not limited to MR imaging but, in fact, can be utilized in conventional radiography--urography, pyelography, cholangiography, angiography, and CT. Radiology reports may not always document the gadolinium exposure to patients who have had MR studies.

The manufactures of the various contrast dyes are currently involved in litigation over the physical injuries and impairments sustained by NSF patients. Through discussions with our clients, we have become aware not only of the physical challenges presented to the patients, but also the problems related to finding treatment for their disease.

Treatment - or Lack Thereof

Many of the patients diagnosed with Nephrogenic Systemic Fibrosis or Nephrogenic Fibrosing Dermopathy are now faced with 2 issues - a battle for their lives against this disease and a battle to obtain appropriate treatment.

At the present time, there is no cure for NSF. In fact, most of the treatment plans/options are experimental or investigative. We hope these experimental treatments will prove to be helpful in attacking this disease. A problem exists as these treatments are viewed by health insurance carriers and Medicare/Medicaid as non-FDA approved. Often, they will not make any payments towards these unproven treatments.

Without insurance assistance, many patients are left without any hope for a treatment that may prove effective. Many find themselves unambulatory and unable to care for themselves, which now requires another family member to care for them 24/7. Research dollars need to be committed to finding treatments for this potentially fatal and certainly debilitating disease.

Current treatments are intended:

  • To prevent NSF in patients who have been exposed to gadolinium-based contrast.
  • To control progression of disease in those who have signs, symptoms, and a documented diagnosis of NSF.

Attempts to control progression of NSF (i.e., the deposition of additional fibrous tissue) have been effective only in some patients. Once NSF is clinically apparent, the fibrous tissue, that has already been deposited, is, for the most part, permanent and irreversible. Physical therapy and analgesia are considered supportive, assisting patients with mobility and pain, but have no effect on the underlying disease.

Some current treatment options include:    

  • Intravenous administration of sodium thiosulfate and oral administration of pentoxifylline
  • Plasmapheresis
  • Photopheresis
  • Steroids

The bottom line is that NSF treatments, to date, are investigational and, as such, may not be reimbursed by third-party payors. Your patients may need your assistance in gaining access to treatment and in finding ways to pay the bills. If you find yourself with a patient diagnosed with NSF/NFD and their health insurance provider is denying coverage for experimental treatment, there may be a way to get help. Allowing these patients to go without hope of obtaining medical care should not occur. There are ways to work with the insurance companies to take steps towards moving these experimental treatments onto their approved/covered list.

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Joe Devine
Pulaski & Middlemen
1 (800) 223-3784
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