About HI Global Registry

Welcome and Introduction to the HI Global Registry (HIGR)

CHI and the HI Global Research team would like to extend our warmest welcome, and thank you for supporting this important patient-powered research initiative. We couldn’t do this without you; together we will connect the dots to advance knowledge and research for better patient outcomes.

The HI Global Registry is a patient-reported online research study that consists of a series of 13 surveys, which ask questions about the patient’s experience with hyperinsulinism over his or her lifetime. These surveys include questions about contact details and demographics (such as, age, sex and country of birth), as well as questions about diagnosis, medication management, diet & feeding, surgical procedures, other diagnoses, developmental and quality of life for the parent/guardian and participant (patient). The questions have been carefully developed by a team of HI experts, including family members of children with HI, advocates, clinicians and researchers.

More information about the registry can be found on the HI Global Registry webpage located on CHI’s website at https://congenitalhi.org/higlobalregistry/. Information includes the registry study’s objectives; members of the Registry Steering Committee; communication material, like a brochure and fact sheet; a Q&A; and best practice guidelines.

We encourage anyone who has received a diagnosis or is suspected of having congenital hyperinsulinism to complete the surveys in the registry. Patients (referred to as participants in the registry) who are eighteen years old or older should enroll themselves and may work with parents or legal guardians on answering survey questions.Parents or legal guardians of participants under the age of 18, or the legally authorized representative of participants who are 18 and over, may report on behalf of the participant.

Before joining it’s helpful to understand key words and terminology and the different roles in participating in the registry:

HIGR is the shortened name for the HI Global Registry Study.
The Participant is the patient (the person with congenital hyperinsulinism).
The Respondent is the individual who completes the surveys, and can either be the participant or his or her parent, guardian or legally authorized representative (LAR).
LAR is the shortened name for legally authorized representative who is legally authorized to input the data on behalf of the participant.
The Sponsor (CHI) is the organization responsible for the initiation, management, and financing of the HI Global Registry.
The Principal Investigator (PI) is the researcher overall in charge of conducting the HI Global Registry Study, including producing de-identified reports, evaluating requests for de-identified data from third parties (such as, researchers, advocacy partners, and biotechnology firms doing relevant work), and ensuring the safety and ethics of the HI Global Registry, in accordance with the Institutional Review Board (IRB) approved study documents.
Co-investigators are researchers who work with the PI to conduct the HI Global Registry Study, including producing de-identified reports, evaluating requests for de-identified data from third parties (such as, researchers, advocacy partners, biotechnology firms doing relevant work), and ensuring the safety and ethics of the HI Global Registry, in accordance with the Institutional Review Board (IRB) approved study documents.
The HIGR Research Team includes the Research Director, Investigators and other personnel who support the operations of the Registry.
TheRegistry Steering Committee is a collection of international HI representatives that provides oversight and expertise to the design and operation of the Registry from the patient, parent and clinical professional perspectives [https://congenitalhi.org/hi-gr-steering-committee/].
An Institutional Review Board (IRB) is an appropriately constituted group that has been formally designated to review research to assure the protection of the rights and welfare of the human subjects. To accomplish this purpose, IRBs use a group process to review research protocols and related materials (e.g., informed consent documents and investigator brochures).

In order to join the Registry, respondents will need to complete the following steps; they will see red asterisks which indicates a required field:

Register with the registry by creating an online account.
Start by clicking on the green Register button.
Fill in the required registration information, such as name, username and password.
Select contact preferences, which gives the Respondent an opportunity to choose why he or she would like to be contacted by the HIGR research team. For example, the Respondent can select to be contacted about potential clinical trials that he or she may be interested in; or being put in touch with other respondents who are reporting similar experiences.
Read Terms and Conditions of entering the NORD (National Organization of Rare Disorders) I am RareRegistry Platform, and agree to them by clicking “I have read and agree to the terms and conditions”.
Confirm registration by copying the Confirmation Token from the confirmation email that will be automatically sent after accepting the terms and conditions.
The Respondent then needs to enroll himself or herself as the participant (patient) or add someone else as the participant. If the respondent adds someone else as the participant, then the relationship to the participant needs to be established. For example, if a parent is adding his or her child’s information to the registry study, then as the Respondent the parent will need to select “parent (biological, adoptive, or step)” from the drop-down menu of responses. Or if the Respondent is another family member, such as the grandparent, then the Respondent would select “Grandparent” from the drop-down menu. Information about the participant, like his or her name and birthdate, will also be asked.
After enrolling, the respondent will need to provide consent to provide the participant’s data in HIGR study. The conditions of participating in the study will be outlined clearly in the consent form. In order to proceed to take the study surveys the Respondent will need to agree to all the conditions of consent.

After informed consent is granted the Respondent will be presented with a list of all the registry study surveys and can start taking them. The Contact survey should be completed first to set preferences regarding different units of measurement (these questions will be marked with a red asterisk indicating mandatory responses)that will be used throughout the survey. After completing the Contact survey, the Respondent can choose to take the remaining surveys in any order he or she wishes. However, please be aware the intent was to structure the questions in a way that is logical, and meant therefore to create a sense of ease when answering them. When taking a survey, the Respondent will be presented with one question at a time and either a selection of responses that can be organized in a drop-down menu, radio buttons, check all that apply, tick box, or a text box that allows the Respondent to enter free flowing text. Also, following some sections in the surveys and at the end of each survey, the Respondent will be asked the following question: “If there is any additional information you would like to add please insert here.” The purpose of this question is to give the Respondent the opportunity to share any additional information he or she believes is important to include in the registry study.

In order to ensure responses are saved we strongly encourage the Respondent to save them in “draft” format along the way. When the Respondent submits his or her responses as “final” he or she cannot go back and change the answers provided. However, if after submitting a survey as final, the Respondent realizes there is a mistake that needs correcting orfurther responses added, the Respondent may contact a member of the HIGR research team to help with updating the survey answer(s).The goal of the study is to improve the knowledge of hyperinsulinism by gathering as much detailed information about the Participant’s history as possible. Some Participant histories will be shorter and less complex than others, and the Investigators realize not every survey question will be relevant for every Participant. We ask that the Respondent complete the survey questions relevant to the Participant’s history as accurately as possible.

If the Respondent feels at any point in time he or she needs help or advice in completing the surveys the HI Global Research team will be available, and assistance can be sought from others who can help the Respondent, such as family members or medical staff that could aid in answering some of the questions.

The goal of the study is to improve the knowledge of hyperinsulinism by gathering as much detailed information about the Participant’s history as possible. Some Participant histories will be shorter and less complex than others, and the Investigators realize not every survey question will be relevant for every Participant. We ask that the Respondent complete the survey questions relevant to the Participant’s history as accurately as possible.

Participation in the registry study is purely voluntary, and therefore if the Participant (or LAR on behalf of the Participant) would like to withdraw from the study he or she can choose to do so at any point. In order to withdraw, there is a revoke consent button that can be selected under the Participant Enrollment tab. Selecting to revoke consent will deactivate the account, and the Participant’s information will be archived and no longer appear in reporting from the Registry. If the Participant, or LAR on behalf of the Participant, wishes to have the Participant’s information permanently deleted from the Registry system, he or she may contact the HIGR research teamand request that all data be deleted.

If you have questions about the HI Global Registry, feel free to contact the Research Director by email, info@higlobalregistry.org or by phone +1-973-842-7559.

Thank you so much for visiting the registry and taking an interest in this important research initiative!