ReachhdLab (1).jpg
ReachhdLab (1).jpg

Hirschsprung's Research: Where Your Donations Go


Reach is committed to providing the most up to date information about Hirschsprung's Disease. Each year we help fund HD
research through our research grant program. Here you can see where all your donations from our t-shirt sales, fundraising events
and other fundraising efforts go towards. The results, very promising, move us forward and closer to hopefully one day
finding a cure for the disease.  

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Hirschsprung's Research: Where Your Donations Go


Reach is committed to providing the most up to date information about Hirschsprung's Disease. Each year we help fund HD
research through our research grant program. Here you can see where all your donations from our t-shirt sales, fundraising events
and other fundraising efforts go towards. The results, very promising, move us forward and closer to hopefully one day
finding a cure for the disease.  

Grant Winners


Grant Winners


2017 Reach Research Grant Winners

2017 REACH research grant winners
 
 

We are pleased to announce...

Seattle Children's Hospital and Iowa State University have been selected to receive the 2017 REACHirschsprungs Foundation Research Grant. Since we did not select a grant applicant last year, this year we were able to fund two grants. The applications were chosen from among a very competitive group of applications reviewed by the Research Grant Selection Committee which is compiled of guest doctors, researchers and Reach board members.  We are inspired by the work that these and other investigators are doing to advance our understanding of Hirschsprung’s disease and ultimately to improve the lives of those children living with the disease and their families.

Congratulations once again and we look forward to monitoring the progress over this year!

 
 

2017 Submissions


Candidate to be announced January 2017

2017 Submissions


Candidate to be announced January 2017

Reviewing the 2017 Research Grant Applicants.

Reviewing the 2017 Research Grant Applicants.

2017 Reach research grant

We are very pleased to have six submissions for our Research Grant this year! So you can see where your donations to Reach go and also to see the support we receive from medical institutions to further the study, treatments and potential cures for this rare life-long disease.

 

1. Le Bonheur Children’s Hospital

Memphis, TN

Laura V. Veras, MD

“Defining Hirschsprung’s Associated Entercolitis: A Multi-institutional Study”

2. Iowa State University Department of Genetics

Ames, IA

Dr. Julie Kuhlman

“Navigating the neural gaps in Hirschsprung Disease with polycaprolactone microfiber scaffolds”

3. Seattle Children’s Hospital

Seattle, WA

Raj K. Kapur, MD, PhD

“A Novel Explanation for Post-Pullthrough Obstructive Symptoms: Reinnervation by Abnormal Extrinsic Nerves”

4.Johns Hopkins School of Medicine

Baltimore, MD

Anish Kapoor, PhD

“Targeted high throughput variant analysis of multiple Hirschsprung Disease Genes”

5. Massachusetts General Hospital

Boston, MA

Nador Nagy, PhD

“Optimizing the isolation, propagation, and transplantation of human enteric neuronal stem cells for treating Hirschsprung Disease”

6. Emory University

Atlanta, GA

Iain Shepherd, PhD

“Assays to Define factors that promote ENS Stem Cell Proliferation/Differentiation”

Julia Ganz Judith Eisen Laboratory Institute of Neuroscience


2014 Research Grant

Julia Ganz Judith Eisen Laboratory Institute of Neuroscience


2014 Research Grant

Julia Ganz Judith Eisen Laboratory

Institute of Neuroscience, University of Oregon

Neural regulation of intestinal inflammation in a Hirschsprung disease model

Julia Ganz

Summary

This work funded by this award aimed to understand if reduction in the number of enteric neurons in a zebrafish Hirschsprung disease model leads to a change in gut motility, causing changes in gut microbiota composition and resulting in increased gut inflammation. To investigate changes in gut motility, we established a high-resolution spatio-temporal analysis of gut motility using a novel analysis method that allows us to determine six different gut motility parameters (Ganz et al., 2016). We tested this novel analysis method and found that comparing gut motility patterns in fed with unfed wildtype zebrafish siblings revealed higher wave frequency and a trend to increased wave amplitude in fed larvae compared to unfed larvae. No other parameters were affected by providing food, suggesting that feeding affects very specific aspects of gut motility. In addition, we investigated changes in gut motility in a zebrafish ret mutants, a Hirschsprung disease model, and found that the amplitude of contractions shows a trend to be reduced in compared to wildtypes. We also analyzed the composition of the gut microbiota in zebrafish larvae with fewer enteric neurons. We find that the range of bacterial colonization shows a trend to be larger in mutants compared to wildtype siblings. In contrast, the diversity of the microbiota is larger in wildtypes than in their mutant siblings. Finally, we investigated if zebrafish larvae with fewer enteric neurons have increased inflammation in the distal gut by measuring number of neutrophils present. We find no significant increase in neutrophil influx between mutants with fewer enteric neurons and their wildtype siblings. In conclusion, we developed sophisticated software to analyze motility patterns in the zebrafish gut. We find that changes in the ENS lead to changes in microbiota composition and the range of microbial colonization, however these changes do not show a clear correlation with gut inflammation.

Grant Results 2014


Grant Results 2014


Progress report for REACH Foundation Research Grant 2014

Ryo Hotta MD PhD

Pediatric Surgical Research Laboratories, Massachusetts General Hospital

Project title: Recovery of anorectal function following enteric neuronal stem cell transplantation in mice with Hirschsprung disease

 

 

Project Summary

            Hirschsprung disease (HD) is a serious congenital disorder where children are born without nerve cells (also called ganglion cells) in the lower part of their intestine. Following surgery to remove that abnormal and non-functioning part of the colon, many children experience problems with their intestinal function, including constipation, incontinence, or enterocolitis. Our laboratory at Massachusetts General Hospital in Boston is working on developing a novel method of treating Hirschsprung disease which involves isolating neuronal stem cells from the intestine, growing them in the laboratory, and then transplanting those cells into the colon of mice with Hirschsprung disease. To achieve this goal, several challenges need to be addressed, and these represented the main goals of our proposal that was support by REACH.

First, it was not known whether the part of the colon that lacks ganglion cells is able to accept transplanted neurons and allow them to divide, migrate, and form mature nerve cells. This is critical if cell therapy is going to be a real option for this disease. To address this question, we isolated neuronal stem cells from the intestine of normal mice, grew the cells in culture, and then transplanted those cells into the colon of mice with HD to determine whether the “diseased” gut environment is able to accept new nerve cells. Amazingly, the transplanted cells were able to multiply, migrate, and develop into mature nerve cells in the HD intestine. With that successful result, we moved on to the second phase of the experiment.

In the second set of experiments, we wanted to test whether neuronal stem cells could be isolated from the HD intestine and whether those cells are capable of producing large numbers of neurons following cell transplantation. This is very important since the ultimate goal is to use a patient’s own cells to treat their HD. We isolated nerve stem cells from the “normal” part of the intestine of mice with HD, grew the cells in culture, and characterized them to confirm that they made healthy neurons. We then transplanted those cells into the colon of HSCR mouse to determine if they could survive, migrate, and differentiate after transplantation. We were thrilled to find that cells derived from mice with HD still retain a normal capacity for growth and differentiation. These HD-derived neurons were able to colonize the colon of mice with HD and to populate the appropriate layers of the intestinal wall and form mature neuronal cells.

The experiments supported by REACH demonstrate that neuronal stem cells can be isolated and cultured from the normal intestine of mice with HD, grown in culture, and then successfully transplanted into the diseased bowel of HD mice to generate neuronal networks within the intsetine. These results establish the potential for using patient-derived neuronal stem cells to treat HD in humans. We are now testing whether the transplanted cells are able to restore the function of the HD colon, which represents the ultimate goal of our research.

Copyright 2016 Reach