In the case of the NSF Registry, it is a collection of glass microscope slides and documentation collected to support or refute a diagnosis of NSF. Each individual in the registry has a file that contains paper, electronic, and/or photographic documentation of their case. In addition, there are corresponding glass microscope slides and sometimes tissue blocks.
How does one get into the Registry?
Glass microscope slides and supporting records are sent to my attention at Yale University. I review the materials. If the glass slides are suggestive of a diagnosis of NSF, the supporting documentation is carefully reviewed for clinical information that can help exclude other disorders that resemble NSF. If the clinical and pathological information both support a diagnosis of NSF, and if no other more likely diagnosis can be made, the case is diagnosed as NSF or compatible with NSF and assigned a Registry number.
What happens once a case becomes part of the Registry?
When a case is diagnosed as NSF, an invitation is made to the patient to formally become a Registry member. This entails a pre-arranged telephone conversation during which the details of the case will be evaluated by an experienced interviewer. Additional supporting information will be sought, and the patient will be verbally consented to participate in the Registry. Exchange of detailed protected health information cannot occur until this verbal consent is obtained. A written consent is sent by mail, and once this is returned, any additional missing parts of the story will be sought from physicians and hospital records.
What do you do with the data/records?
These are stored under lock and key. Important data elements are placed in a protected computer database. The database allows data from many patients to be examined simultaneously. For instance, if the US FDA asks our Registry to provide a breakdown of the genders and ages of patients with NSF, we are able to do so. These large scale datasets allow patterns to emerge, and have been instrumental in answering questions such as:
1. Which contrast agents are more likely to trigger NSF?
2. Are peritoneal dialysis patients at a greater risk for NSF than hemodialysis patients?
3. Are children at a greater risk for NSF?
4. Does a higher cumulative dose of gadolinium lead to a higher risk?
So why is this important?
Most clinicians will never see a case of NSF. NSF is a rare disease, and our work has made it even rarer. A clinician cannot rely on his/her experience in diagnosing a case of NSF if they have never seen it. Similarly, pathologists may not be familiar with the disease. The Registry allowed hundreds of records to be compared, and for the common symptoms, signs, lab values, and pathology findings to be identified. Using this information, a clear diagnostic definition was formulated and published. The definition was meant to identify as many cases of NSF as possible while also excluding persons with other diseases that resemble NSF. This definition creates a high degree of certainty that information that comes from the pure population of NSF patients in the database is unpolluted by other disorders. This "clean" information can be used to generate new hypotheses about the disease and to suggest therapies and preventive strategies.
Why isn't every case of NSF in the Registry?
- NSF is not a disease that has mandatory reporting, like HIV and some other infectious diseases. Entry into the Registry is voluntary.
- Physicians may not know about the Registry, and will not automatically seek to have a second opinion rendered.
- Patients may not know they have the option to have their pathology slides sent to me for a second opinion. In most cases, insurance will cover the cost of this second opinion.
- Many cases diagnosed as NSF elsewhere do not reach the level of diagnostic certainty to be included in the Registry database.
- In some cases attorneys involved in litigation on their clients' behalf do not want the diagnosis of NSF "confused" by a second opinion. In many cases, however, attorneys seek to have cases of NSF reviewed for possible Registry inclusion.
How do I find out more about the International NSF Registry?
Contact us through our website (http://www.icnsfr.org)