This week is Porphyria Awareness Week in the US, and guest blogger Mikey is discussing his experience of the impact that his EPP has had on his professional life.
Direct and indirect sunlight, from reflected light, is a real “killer” for me. If I can find a shadow, I can put exposed skin into that shadow to prevent that area getting burnt.
Unless you’ve got EPP you wouldn’t understand.
Clinuvel has invited guest blogger Mikey to discuss EPP on our blog; This is the second post in the series discussing the impact of EPP on life outdoors. We are grateful to Mikey for being able to share his experiences.
I was diagnosed with EPP when I was about 7. Australia is a great country. It is vast in its beauty and diverse in the hazards it provides! One of those, for someone with EPP is the “great outdoors”. I reckon this is the best country in the world. I’ve lived in New York, and travelled extensively around the world – I’m always pretty pleased when I return home.
Clinuvel has invited guest blogger Mikey to discuss EPP on our blog; a series we will publish in the coming weeks. We are grateful to Mikey for being able to share his experiences.
My name is Mikey and I live in Sydney, Australia. I’m 44 and have had EPP all my life. I grew up in Sydney with my parents, an older brother and a younger sister. Mum and I both have EPP, but neither of my siblings nor my father had it.
I remember, as a child prior to diagnosis, my family didn’t know what was going on with me when I’d been in the sun too long – except my mum who had experienced the same things I was complaining about.
The body consists of cells which communicate with each other via signaling molecules to govern and determine a variety of physiological functions in the body. The action and effect of these signaling molecules is mediated by ‘receptors’ which are located on the surface of (plasma membrane), or inside a cell. A molecule that ‘binds’ with a receptor is known as a ligand and can be protein such as a hormone or drug and the role of the ligand is to activate, or inactivate a particular biological activity.
In some ways receptors can be likened to switches with on and off positions, and which in turn affect the cell’s internal functioning. The action or inaction of receptors is determined by the type of ligand with which they bind, i.e. receptors are activated when an agonising ligand, an ‘agonist’ binds and left inactive when an ‘antagonist’ in some way prevents the agonist from binding. The specific biological action is dependent upon which particular ligand binds with a receptor.
“The fear of what lay ahead” is our latest webcast exploring the impact of EPP upon individuals, their families and loved ones.
Wendy and Ralph are the proud parents of two daughters, who were both diagnosed with Erythropoietic Protoporphyria (EPP), although their first daughter suffered for almost three years before the correct diagnosis of EPP was arrived at.
In this short film they discuss the fear, confusion and distress they endured as a result of grappling with EPP’s severity, invisibility and often unknown and misunderstood nature, all the while unable to treat or protect their children.
Clinuvel would like to extend its thanks and appreciation to Ralph, Wendy, Angela and Lyndal for allowing us to share their story.
As many of you may know, this week saw Clinuvel hold its Annual General Meeting in the Melbourne Town Hall. As managing director I welcome these opportunities where I am able to meet and talk to the people whom Clinuvel considers integral to the company and its existence; the Board of Directors sees Clinuvel’s investors as unique. In Clinuvel, an investor has to fundamentally believe in the premise of offering pharmaceutical photoprotection, a novel option, an innovative approach to skin disorders.
In a meeting that would be considered short by international standards I was both excited and proud to present some of the achievements and milestones our team has reached this year, and lay out our plans for the next 12 months.
The research that underpins afamelanotide began over 20 years ago in the US. At that time the dramatic increase in incidence of skin cancer and its impacts were beginning to be discovered and new solutions sought. The National Cancer Institute (NCI) sponsored researchers to explore applications for various peptides as part of its melanoma prevention strategy.
This study led them to investigate peptides that acted on melanocyte receptors. The rationale behind this being that increased melanin leads to more efficient photoprotection, as melanin is a highly successful and naturally occurring UV quencher and filter. By consistently increasing melanin in the skin, skin damage from light and UV radiation can be lessened.
A new initiative has just launched in the UK with the goal of increasing awareness and support for the more widespread use of photodynamic therapy (PDT). Compared to surgery and chemotherapy, PDT is a relatively simple, quick and effective treatment for certain types of cancer that we have covered previously on this blog.
Killingcancer.co.uk is a fundraising initiative aiming to create both a groundswell of interest and a pool of funds to support the development and access to PDT in the UK, to make it more readily available through the National Health Service (NHS). Through a mix of celebrity endorsements and patient stories they build an informative and compelling case for their cause.
Solar Urticaria (SU) is an allergic or immune reaction to the sun, UV and visible light. Although rare, it can arise in any person, at any time and diagnosis is difficult due to its superficial similarity to other allergies and conditions. For example, it is often mistaken for Polymorphous light eruption (PMLE), lupus erythematosus and even erythropoietic protoporphyria (EPP).
During more than a decade of clinical research on Clinuvel’s proprietary drug afamelanotide, our teams have specialised in, and focused on, the study and importance of light absorption for our health and well-being.
However, light is regarded as an ‘optical paradox’: too little may result in vitamin D deficiency or seasonal affective disorder (SAD), while too much may result in skin cancer. In seeking answers from related fields, our in-house scientists often remark that human biology is unfortunate to lack green chlorophyll with its ability to efficiently process light into nutrition.
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