CHI Blog

I received the following update from the CHOP Hyperinsulinism Center on the diazoxide that many of you have written about with concerns. This is what was shared with me:

“Yesterday a TEVA Quality Assurance Auditor gave the following explanation for the questionable diazoxide batch:

- There is no official recall of this batch, which is why people are being sent bottles from the same lot.

- According to TEVA this lot is up to specifications which state that the appearance of Proglycem can vary from very white, to light brown, to pink.

- Since most people have not ever seen Proglycem quite so white in appearance, they understand the hesitation to use it, however the company is NOT testing the bottles because they do meet the medication specification standards. They will take the bottles back and send more, but there could be issues with availability.

- There is no official shortage, however if someone sends back medication from this “different appearing” lot and that’s the only lot that their distributor has locally available, then they may not be able to get more meds for a while. So basically, the rejection of Proglycem bottles from this lot could cause local shortages in areas where this is the only available lot.”

During the Fourth Congenital Hyperinsulinism Family Conference to take place March 17th and 18th at the Children’s Hospital of Philadelphia (CHOP), tweens and teens with HI and their siblings will produce a show that will be broadcast to pediatric patients at CHOP.

A gift from the Ryan Seacrest Foundation for a recording studio known as The “Voice” is making the creation of this show possible. The” Voice” which is housed in the lobby of CHOP was installed to aid CHOP in caring for the general well being of patients, the whole child. The shows that have been created have been highly interactive and creative.

The show that will be produced during the HI conference sponsored by CHOP and Congenital Hyperinulinism International brings a new dimension to the” Voice” and to CHOP.  Many of the HI tweens and teens who will be producing the show were fairly long term patients at CHOP and at other Childrens’ hospitals. Like the current CHOP patients who will have a chance to take in the live show, the young people with HI had to endure operations, trials, and complicated medical regimes often involving medical devices hospital stays lasting for months.

The idea of the show is to give the current patients at CHOP hope, that they will go on to lead full and interesting lives just as the HI youngsters have. Some of the themes our HI tween/teens will focus on in the show are:

• Focusing on the good times and accepting there will be some hard times. Everyone has ups and downs.
•  Experiencing serious illness as a child makes you stronger!
•  Early challenges with health help us appreciate the small wonderful moments in life.
•  Friends and family can be very understanding and helpful with chronic illness management.
• The more you share, the more likely friends and relatives are to understand and be helpful.
•  Managing chronic illness is so important. Planning is key.
• Getting to know other kids dealing with similar issues is so helpful.
•  Siblings can be a great support.
•  We are here for you! Let’s stay in touch.

This whole project was the brain child of Susan Becker, Coordinator of the Pet Scan Program at CHOP. Susan has held many crucial posts at CHOP, most recently as Nurse Coordinator of the Congenital Hyperinsulinism Center at CHOP. Susan believes that the experience of producing the show will be fantastic for HI tweens and teens and that it will be very meaningful for them to give back. Teresa Dansbury, the current Coordinator of the HI Center at CHOP ran with Susan’s idea and made it happen. We are so grateful to both of them for this opportunity.

In addition to the thematic aspect of the program, the conference participants will have an opportunity to select musical offerings for the current CHOP patients.

CHI is presenting a series of blog articles on the speakers who will be addressing families attending the Fourth Congenital Hyperinsulinism Family Conference March 17 and 18, 2012, in Philadelphia, PA. In this article, we focus on advocacy issues and present Mary Dunkle, Melanie Cohen, and Kristin Crossland.

Advocacy is one of the most important elements of the work of a patient organization. When the disorder is rare, it is all the more incumbent upon affected families to get involved. If not us, who will do the work of ensuring that all people living with congenital hyperinsulinism (HI) receive: a timely diagnosis, state-of-the-art-treatment, necessary medications and devices, and the possibility of a cure?

At the Fourth Congenital Hyperinsulinism Family Conference, we will all become advocates! CHI and CHOP have asked Mary Dunkle, a seasoned professional in the field of rare disease advocacy, to guide us in this process. Mary is Vice President of Communications at NORD, the National Association of Rare Disorders, and in that capacity has a fantastic grasp of all the elements of a fine advocacy program. Mary will share information about how we can work with different governmental branches, committees, and organizations to create a better climate for patients with HI.

Mary will offer examples from different rare disease communities. She will provide us with information on how our organization can work effectively with the National Institutes of Health (NIH), the Federal and Drug Administration (FDA),the Federal Government, the U.S. Congress, local government, pharmaceutical companies, medical societies, etc. Mary understands that our concerns go far beyond U.S. borders. She will share her thoughts on how we can work internationally.

Melanie Cohen, the fantastic development officer for the Congenital Hyperinsulinism Center at CHOP who has attended many of our events, Kristin Crossland, a wonderful rare disease advocate that many of you have gotten to know on our blog, and I will be on the panel with Mary and we will be looking at advocacy issues specific to HI. We will ask Mary for guidance on how the CHI community can work to prevent future intermittent drug shortages, what we can do to help reduce the time it takes for a rare disease drug to come to market, how we can most effectively communicate with governmental representatives about newborn glucose protocols, how we can foster more research, and how we can further support the HI centers of excellence in the U.S. and around the world. Mary will also emphasize the importance of working with NORD. “Alone we are rare. Together we are Strong.”

CHI is presenting a series of blog articles on the speakers who will be addressing families attending the Fourth Congenital Hyperinsulinism Family Conference March 17 and 18, 2012, in Philadelphia, PA.  In this article, we present Teresa Dansbury.

Teresa is the Nurse Coordinator of the Congenital Hyperinsulinism Center at CHOP. She has been on the job for less than a year so many of you will be meeting her for the first time. Teresa started her nursing career as a U.S. Air Force nurse with a background in obstetrics. She went to nursing school at Penn and it was at that time that she learned all about CHOP. Even back in nursing school Teresa was aware that CHOP was an incredible place, with amazing people helping families from all over the world. Teresa left the OB world last year, seeking an opportunity to try new areas of nursing. “From my experiences in nursing school and an externship at CHOP, I knew that this was the place where I could not only be of service families but also learn from the best physicians in the world. Being new to HI, I can say that I feel lucky to be a part of this CHOP team and have learned SO MUCH in the past 10 months (from the docs and the families)! ” Teresa is not only a great administrator and nurse, she is also a musician in her spare time, singing with a band. Teresa will be leading a session at the conference on prenatal testing. In addition to being one of the speakers at the conference, Teresa has been one of the key people involved with conference planning.

CHI is presenting a series of blog articles on the speakers who will be addressing families attending the Fourth Congenital Hyperinsulinism Family Conference March 17 and 18, 2012, in Philadelphia, PA.  In this article, we present Melissa Rearson, Nurse Practitioner.

Melissa Rearson has been working at CHOP with diabetes and congenital hyperinsulinism (HI) patients since 1989.  I first met her in 1996 when my son, a newborn at the time, was transferred to the “Endocrine floor” at CHOP.  Ms. Rearson completed a master’s degree and started seeing patients at CHOP in the Division Endocrinology/Diabetes in 1993.

Ms. Rearson will be speaking at the Family Conference on diabetes secondary to HI and she will also be leading a session with the pre-teen and teen group at the Conference.   Ms. Rearson is a fantastic resource because she has an excellent understanding of blood sugar issues from a medical standpoint and is equally well-versed in day-to- day home management of hypo- and hyperglycemia.  

In her own words:

“I was initially fascinated by the endocrine specialty because I saw that there is lots of room for family support and education—all things done well by nurses!   I mainly work with patients in our division with type 1, autoimmune, diabetes, but that has changed over the years as we were able to have more and more nurse practitioners who specialized in various parts of our practice.  When I initially started in the division, I worked on the floor with all of the endocrine patients—including the congenital hyperinsulinism patients.

Now, nurse practitioners in our division have a group of families that we follow at each outpatient visit.  Nurse practitioners work to educate, evaluate health and chronic condition management, support family members with maintaining health at home, refer to various other specialists and act as a liaison for the family among the endocrine team at CHOP.

I am committed to helping kids and families manage diabetes to the best of their ability with the technology that exists. This means that I delve into what ‘real life’ looks like for each family and work with the various members to find a program that will work for the family as a whole. Managing diabetes takes energy, enthusiasm, diligence, patience, problem-solving and coping skills—and often cheerleading! I hope to provide help in all of these areas for each family in a way allows them to do their best with a sometimes overwhelming chronic condition.”