2018 RESEACH GRANT SUCCESSFUL APPLICANTS
As our financial position improves, we are working to expand the grants we can make available to fund EB research which has an Australian component. Our priorities are aligned with those of DEBRA International. This year we have been able to increase our awards to AUD$95,000. Congratulations to all the successful recipients.
Dr Zlatko Kopecki
Researcher: Dr Zlatko Kopecki (University SA)
Research Project: Development of a simple, effective and pain-free diagnostic tool for patients with mild EB
Project Description: Approximately 70% of all EB cases are of the EB Simplex subtype, which is often mild and therefore greatly underdiagnosed or misdiagnosed. Many mild EBS patients are reticent about having large punch biopsies taken which may lead to open wounds and are worried about pain associated with current conventional sample collection. This clinical and laboratory project will develop a novel pain-free minimally invasive technology for better and earlier diagnosis of EBS which may enable patients better outcomes and access to services including the National EB Dressing Scheme.
Dr Kenneth Pang
Researcher: Dr Kenneth Pang (Murdoch Children’s Research Institute Melbourne)
Research project: Development of animal models for Epidermolysis Bullosa-related pain
Project description: Epidermolysis Bullosa (EB) is characterised by blistering, the pain of which can require long-term, high dose, opiate (e.g. methadone) analgesia from infancy. Unfortunately, the use of such analgesia is not only associated with significant side effects but is also frequently ineffective. Consequently, one of the main causes of reduced quality of life in EB is chronic pain. In this project, we seek to develop the first ever mouse models to study EB-related pain. If successful, the work will provide a foundation for future pre-clinical studies to test the effectiveness of novel analgesics in EB.
Researcher: Professor Dedee Murrell (St George Hospital Kogarah NSW)
Research project: A prospective, double-blind, cross-over, pilot study to assess safety and efficacy of topical
sirolimus 2% in the treatment of plantar blistering in patients with epidermolysis bullosa simplex (EBS)
Project description: The purpose of this clinical trial is to investigate whether sirolimus 1% cream will help reduce the calluses and blistering on the soles of the feet of people with epidermolysis bullosa simplex (EBS). Promising findings from the USA on the positive effects of topical sirolimus 1% on a similar keratin disorder have lead us to think it may be beneficial in EBS too. This grant provides additional funding to support completion of the study.
Grant for International Research Collaboration
Albert Mellick (second left, Sydney) and EB Haus Research Team
Researchers: Dr Verena Wally (EB House Austria) and Associate Professor Albert Mellick (Ingham Institute, Sydney)
Research Project: Establishment of in situ hybridizations for RNA localization in RDEB tissues
Project Description: RDEB accounts for many premature deaths in the EB patient population. Of those, nearly all are the consequence of aggressive squamous cell carcinomas (SCCs) with frequent metastases, which arise in nearly all patients in their third or fourth decade of life. Unlike normal SCCs, which are poorly malignant, the propensity of SCCs derived from EB patients to become malignant and lethal is very high. However, the mechanism leading to the development of these highly aggressive tumors is not yet fully understood. Previous work has shown that there are very precise changes in gene activity associated with the evolution of dystrophic non-malignant skin tissue to one which has developed into a malignant SCC. Of particular interest are changes in a group of small molecules referred to as small non-coding RNAs. These molecules are master regulars of gene activity and changes in levels of these small RNAs can be predictive of pathologically significant changes in disease and development. The work in Austria has identified several candidate diagnostic and therapeutic RNA targets. In Australia Associate Professor Mellick has developed novel methods in identifying and tracking the activity of these small molecules in patient samples. The project proposal is to establish collaboration between Australia and Austria to study and develop this method as a diagnostic tool in EB. This work has the aim of: (i) allowing clinicians to track the evolution of SCC in EB; and (ii) identifying patients which may benefit from improved monitoring.
2017 RESEACH GRANT SUCCESSFUL APPLICANTS
Congratulations to Dr Kenneth Pang and Dr Zlatko Kopecki who were the successful research grant recipients earlier this year. DEBRA Australia will support each project with a $30,000 grant.
Researcher: Dr Kenneth Pang (Murdoch Childrens Research Institute Melbourne)
Research project: Characterization and utilization of new mouse models for dominant dystrophic epidermolysis bullosa (DDEB)
Project description: Mouse models play a key role in the study of many human genetic diseases and the preclinical development of new therapies. Currently, there are no mouse models for dominant dystrophic epidermolysis bullosa (DDEB), which is caused by mutations in the gene encoding collagen VII and can result in severe blistering disease for which there are no effective treatments. This project seeks to provide a series of new genetic mouse models for DDEB and, by doing so, should advance understanding of the molecular mechanisms underlying DDEB and facilitate preclinical testing of novel therapies.
Dr Kenneth Pang
Researcher: Dr Zlatko Kopecki (University of South Australia Adelaide) + use photo from previous newsletter/website of Zlatko
Research project: Combating blister infection one step at a time
Project description: Skin blistering diseases (Epidermolysis Bullosa (EB)) affect 1:17,000 live births with 500,000 cases estimated worldwide. EB children experience large non-healing wounds and spontaneous skin blistering affecting 70% of their bodies. Infected wounds are a constant problem for EB patients and often lead to sepsis and death. This project aims to use novel models of bacterial wound infection to investigate the mechanism underpinning innate immune responses in fighting wound infection. Understanding how different proteins contribute to susceptibility of bacterial infections may lead to novel treatments for blistered skin in EB patients.
Dr Zlatko Kopecki
2016 TRAVEL GRANT SUCCESSFUL APPLICANTS
Congratulations to the following recipients of the April 2016 Travel Grants:
- Rebecca Saad (EB nurse, Sydney Children’s Hospital) – Attend DEBRA International Conference 2016 and training in UK ($2990)
- Lewis Citroen (Podiatrist, EB clinic, RCH Melbourne) – Attend DEBRA International Conference 2016 and network with podiatrists involved with EB patient care ($2260)
- Emma Kim (Medical student, St George Hospital, Sydney) – Attend 2016 College of Dermatology meeting in Perth and present paper on her EB research carried out under the supervision of Professor Murrell ($750).
2015 RESEARCH GRANT SUCCESSFUL APPLICANTS
Congratulations to the following applicants who received Research Grants in 2015:
Dr Zlatko Kopecki
2014 RESEARCH GRANT SUCCESSFUL APPLICANTS
Congratulations to the following successful research recipients following the 2014 research awards:
Associate Professor Orli Wargon
Dr Zlatko Kopecki
Lauren Weston – EB Nurse at Melbourne Royal Children’s Hospital
2013 RESEARCH GRANT SUCCESSFUL APPLICANTS
DEBRA Australia is pleased to announce the successful applicants for its 2013 Research Grant was Dr Zlatko Kopecki (pictured top) and Professor Allison Cowin, Centre for Regenerative Medicine, Mawson Institute, University of South Australia in Adelaide. Dr Kopecki and Prof Cowin were awarded $15,000 for their laboratory based project titled: “Development of systemic therapy for treatment of blistered areas”. Epidermolysis Bullosa (EB) is a heterogeneous group of inherited disorders characterized by skin fragility; and systemic blistering of the skin. Besides the evident skin blistering and development of chronic wounds, patients with EB also develop secondary complications resulting in pain, loss of voice, poor nutrient uptake, rectal bleeding, constipation and retarded growth. Dr Kopecki and Prof Cowin have previously showed that topical application of Flightless neutralizing antibodies (FnAb) to skin reduces the severity of skin blistering. In this pilot study Dr Kopecki and Prof Cowin will investigate the effect of systemic FnAb therapy on healing of cutaneous blistered areas. This research may open up further research avenues in systemic treatment of EB patients. Dr Kopecki commented that “we are grateful for the continual support from DEBRA-Australia and we hope that this research will lead to novel approaches to help reduce the symptoms associated with blistering observed in patients with EB.”
2013 RESEARCH & EDUCATION TRAVEL FELLOWSHIP RECIPIENTS ANNOUNCED
The first of DEBRA’s new Research & Education Travel Fellowships was allocated to two recipients. Firstly, to our Qld EB Nurse, Samantha Hay who will be using the grant to travel to the UK to attend Epidermoloysis Bullosa Introductory and Advanced study days on 2nd & 3rd December 2013 at Great Ormond Street Hospital for Children.
Secondly, a travel grant has been offered to Clement Loh, a 5th year UNSW medical student who completed Honours last year in dermatology research, who will attend the International Investigative Dermatology World Congress of Dermatology Research, held once every 5 years, to give an oral presentation. This year’s congress will be held in Edinburgh. Clement’s project involves developing the EBDASI, which is the first fully-validated objective severity scoring system for EB that will prove useful in future EB research trials. The IID conference is an excellent opportunity to formally introduce this scoring system for the first time to EB researchers from all over the world.
Congratulations to both Samantha and Clement!
(Applications for Round 1 closed on 31st March 2013 for travel between 1st May and 31st December 2013)
2012 DEBRA ANNOUNCES INAUGURAL RESEARCH GRANT AWARDS
DEBRA Australia is pleased to announce the successful applicants for its 2012 Research Grants. The principal grant of $15,000 has been awarded to Professor Allison Cowin (pictured top) and Dr Zlatko Kopecki, Centre of Regenerative Medicine, Mawson Institute, University of South Australia, in Adelaide, for their laboratory-based project “Function of Flightless protein in the development of SCC in children with fragile skin”.
Patients with Recessive Dystrophic Epidermolysis Bullosa (RDEB) develop extensive skin blistering and scarring which often lead to the development of Squamous Cell Carcinoma (SCC). No specific cure exists and the mechanism behind the development of SCC in children is poorly understood. This pilot study will help to understand the mechanisms involved in SCC tumour growth in children with skin blistering diseases and identify potential routes for preventing its formation, which may open up further research avenues.
Professor Cowin and Dr Kopecki have a strong track record in wound healing research and this grant will help them to expand their program to understand more about the processes behind the development of SCC. Professor Cowin and Dr Kopecki commented that they “were grateful for the award from DEBRA Australia and they hope that that their research will lead to new approaches to help reduce the incidence of SCC in patients with EB.”
As further research funds have become available due to a recent bequest, the Board of DEBRA Australia has also decided to make a smaller, secondary award of $10,000 to Dr Mae Ramirez and Professor Dédée Murrell (pictured below) from the Department of Dermatology, St George Hospital, University of NSW, in Sydney, to assist in the conduct of their Clinical Trial “Investigation of Micro-needling Skin Therapy for Recessive Dystrophic Epidermolysis Bullosa (RDEB).” Micro-needling has long been used in cosmetic surgery to improve scarring, but has not been used in wound treatment. This study compares the effects of micro-needling to a placebo in patients with RDEB, to see if wounds heal faster with micro-needling.
Professor Murrell explained, “This project follows on from a previous clinical trial that we completed, which had the unexpected result that healing was as good in patients receiving injections of placebo alone as those who received an experimental therapy of cultured cells called dermal fibroblasts. If micro-needling alone proves to be effective, it may offer a cheap and effective method of healing RDEB wounds.”
Dr Ramirez, who qualified as a dermatologist in the Philippines, is a dermatology research fellow with Professor Murrell who established the Australasian EB registry and the diagnostic lab for EB in Australia. Dédée has a track record of EB management and research since 1995, including collaborations with Prof Cowin and Dr Kopecki.