Registry & Research

We ask you to contribute to our 4p- patient registry so we can conduct the research that is so desperately needed.  Below is a list of general information about the registry and its purpose.

The Patient Registry session from our 2014 National Conference is available to all families via YouTube to help them gather the information that will be needed to enroll, and to also help answer any commonly asked questions. The session is broken into 2 parts. Part 1 is the main informational session. Part 2 is the question and answer section which goes over the registry questionnaire. Part 2 will be vital to helping you answer the questionnaire correctly. Families are strongly encouraged to watch it prior to completing their questionnaire.

Go to: Registry Session Part 1

Go to:  Registry Session Part 2

What is a Patient Registry?

A patient registry is an online database that stores important information in a systematic way and can be queried into data reports. In order to study any medical condition, it is vital that researchers have data to conduct their studies. With a rare disorder, it is absolutely critical to have a central registry for medical data for that specific condition, since patients are so few and are geographically dispersed. The 4p- Patient Registry will make a huge impact on our community because we will not only be able to define 4p- more accurately, but also answer the questions many of us have, such as, “What percentage of affected individuals have seizures, sleep disorders, or renal failure? What treatments work best for seizures, sleep difficulties, or ADHD?” By defining the syndrome more effectively, we can better educate our families, and in turn the medical community about 4p-. The registry’s long range goal is to determine effective treatments to provide a better future for all our loved ones with 4p-.

Participating in the registry is the single most important thing families can do to help build a better future for those affected by 4p-. We need the participation of MANY to make this critical resource a real success; this is one of the most direct ways that families can contribute to scientific research and to clinical advances made on 4p-/Wolf-Hirschhorn Syndrome.

What You Need To Know

Who is on the 4p- Registry Committee? Amanda Lortz, Executive Director, 4p- Support Group, Dr. Amy Calhoun, Chair of the 4p- Scientific Advisory Board, Dr. John Carey, Co-Chair of the 4p- Scientific Advisory Board, Dr. Sarah South, 4p- Scientific Advisory Board Member, Rena Vanzo, CGC, 4p- Scientific Advisory Board Member, Mallory Sdano, CGC, 4p- Scientific Advisory Board Member, and Megan Martin, CGC, 4p- Scientific Advisory Board Member, comprise the 4p- Registry Committee, whose task it has been to evaluate the program and recommend the best way forward.

What is a patient registry? A patient registry uses observational studies to collect uniform data to evaluate specific questions for those affected by a particular disorder. The International 4p- Patient Registry will allow us to provide researchers an opportunity to study people with 4p-, and will help to educate physicians about prognosis and treatment options. Families will benefit from increased research and publication.

Who or what is CoRDs? The Coordination of Rare Diseases at Sanford (CoRDS) registry is a national and central registry for all rare diseases. The goal of CoRDS is to create a central resource of data on rare diseases to help accelerate research into rare diseases. CoRDS is headquartered in the Sanford Children’s Health Research Center at Sanford Research in Sioux Falls, SD and supervised by David Pearce, PhD.

CoRDS provides a mechanism by which data can be collected securely under an IRB-approved study, which can be made available to researchers who are studying rare diseases. CoRDS oversees participant enrollment and preforms annual follow up reminders.

Why is participation in the 4p- Patient Registry important? The 4p- Patient Registry offers anonymized data to researchers so that they can more easily increase scientific and medical knowledge about the syndrome. Each individual’s medical history is important, because 1) each additional data point adds to the aggregate to show a more complete 4p- picture, and 2) each individual is different and may tell an important part of the full 4p- story.

We hope that improved knowledge ultimately will lead to clinical advances, which will help all those with the disorder, including 4p- participants. Individuals’ involvement also will facilitate participation in any relevant clinical trials in the future.

How do I participate? Go to http://4p-supportgroup.org/registry to complete the screening form.  Once complete, CoRDS will email you a username and password that you will use to log in and complete the questionnaire.

What will I need in order to participate? In order to verify your child’s diagnosis, you will need to obtain a copy of their DNA report(s). If you do not have a copy of the results, contact your child’s doctor to ask for a copy.

Why is my child’s DNA report so important for enrollment? Your child’s report provides the interpreted result of their DNA analysis. This interpretation provides geneticists and researchers with valuable information including a “map” to pin-point the mutation and genes that may be affected in connection to the mutation.

How long does enrollment take? We anticipate families will spend 20 minutes or less completing the enrollment questionnaire. Our former BioForm took families a great deal of time to complete. In order to get as many families as possible to participate, we kept the initial questionnaire short and simple, while still allowing the collection of significant data.

What is a PI? What is his/her role? The Principal Investigator (PI) holds ultimate responsibility for the design, conduct, and management of a research study. Our Executive Director, Amanda Lortz will fill the role of PI for the 4p- Support Group. Amanda will be supported by several others who will help maximize the registry outcomes.

What is an IRB? An Institutional Review Board (IRB) is charged with protecting the rights and welfare of people participating in research.

Will the 4p- Support Group be applying for an IRB in order to conduct research with the registry data? Yes! We are currently in the process of completing our application. However, we do not need an IRB in order to use the registry data for non-research purposes, such as information needed to create patient care guidelines.

Thank you for your participation!

If you have any questions or concerns about our 4p- patient registry and/or participating in research please contact Sylvia Renfro at sylvia@4p-supportgroup.org

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