DEBRA Christmas Greeting 2020 - DEBRA NZ
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DEBRA Christmas Greeting 2020 Seasons Greetings! Whew – the year is nearly over. What a strange, challenging time we have all lived through in the last few months with Covid, lockdowns and concerns about what the future holds for everyone. I know I’m looking forward to a summer holiday soon, and I hope all of you have a chance to relax and unwind, have fun with family and friends, and recharge your batteries for what we hope will be a great year in 2021. A brief overview of some of the highlights of 2020 for DEBRA NZ Rare Disorders NZ hosted an event at Parliament on Rare Disease Day, 28 February 2020 to call for a National Rare Disorder Framework and launch their Fair for Rare campaign. A few of us in Wellington attended this launch to support the efforts of RDNZ for all families living with rare disorders. We are closely connected to the Rare Disorders NZ organisation with Martin Hanley continuing to be a member of their board. For more about the work of RDNZ, the Fair for Rare Campaign and to sign their parliamentary petition, please see their website: www.raredisorders.org.nz The EB skin research project in Auckland is very exciting. They can grow EB skin from a small biopsy sized sample and then change the genetic make-up of the skin to remove the EB. There was a story about their work on TV 1 news on Saturday October 31, during EB Awareness week. You can search Epidermolysis Bullosa on the 1 news website to see the story. https://www.tvnz.co.nz/one-news Russell Gear’s research project on EB in NZ – Russell has nearly finished his study of the NZ EB community and the types of EB they have. Thank you to everyone who participated in his research project, funded by DEBRA International. The bandage supply system – DEBRA are now contracted to the MoH to manage this, and the first good outcome is having lifelong Special Authority numbers instead of the annual re-authorising for each person with EB. The DEBRA Nurses can now authorise access to dressings instead of this having to go through a Dermatologist. We’re still working on having a national supply system but with all the different DHBs, this is moving forward in small steps! Ski Camp for teens with EB was postponed this year due to uncertainty about travel restrictions, but this is now being planned for August 2021 in Wanaka – please see the flyer in this newsletter. 2020 is the 40th year since DEBRA NZ was started by Jillyan Hing. We will look forward to a big celebration of this next year if Covid allows us to travel easily, and we look forward to seeing as many of you as possible to get together and congratulate Jillyan for the fantastic organisation she started for the families in NZ who live with the challenges of EB, and what a difference she has made to everyone who can now be part of this big DEBRA family. Happy New Year everyone! Anna Kemble Welch, Director PAGE 1 | TOP 10 FAMILY HOTELS Greetings from Mo and Kora. After 8 years, I managed to convince my husband that we NEEDED a cat.This lovely was waiting for us at the SPCA, Wellington. We now sleep in all manner of strange angles and have an alarm that goes off at 4:30 am demanding to be fed. She has no respect for our personal space and walks over us like we are part of the furniture. And we LOVE it. I hope you are all well and happy. Due to Covid I have not been able to visit my clients as much as I would have liked to. Please know that I am always available for any concerns or questions or even just a chat. I work 3 days a week, which are not always the same due to my DHB job, but I do check my emails and phone regularly. So please feel free to get in touch, and if you do need or want a visit, let me know. Wishing you all a lovely and safe Christmas and New Year. ☺
bringing together more than 500 EB researchers, medical specialists, DEBRA representatives from over 40 countries, and some local and international families living with EB. We were able to get travel grants and financial support for accommodation and registrations from DEBRA International and DEBRA UK for three of our EB Nurse Specialists to attend, as well as Lea (standing in for Anna who wasn’t able to go). Along with a NZ family who have a child with EB (who self funded to attend) DEBRA NZ was well represented. This was an incredible experience, a lot of learning, sharing of our NZ experiences, an opportunity to contribute to developing EB Clinical Practice Guidelines, networking with EB experts and being connected to the international EB community. EB GLOBAL The abstracts from presentations are available on the congress website, along with the programme and CONGRESS IN speakers and other information about the Congress. You can find these here: ebworldcongress.org. LONDON As the world became more aware of the looming Covid crisis, our DEBRA team were still heading back to NZ and The EB 2020 Global Congress combined the research conference and thank goodness they all got home safely before travel clinical conference that usually happen every second year in different restrictions began. parts of the world, with the annual DEBRA International Congress, https://www.debra-international.org At the beginning of this year, I was privileged to travel with my colleagues, Sharon, Jacqui and Lea, to the EB Congress in Visit the DEBRA International website for all the latest in London. It was so full of information and studies that I, EB news, research, events, health care and patient personally, found it a little overwhelming. I managed to catch guides. up with some people that Deanna and I had previously met, reinforcing international friendships and working relationships. The congress covered many aspects of EB care and study. For hand therapy there has been a tool devised to assess the level of hand contracture – Assessment Contracture Evaluation (ACE). It also was found that there was a predictable course with hand contractures. This can be found on the Great Ormond Street Hospital website under EDEB. There was also a discussion about Losartan, a medicine that is used in the treatment of heart failure, decreasing the speed of mitten deformity in children with RDEB 3 – 16 years. Stanford University was recruiting to put protein back into cells where it was missing. It is not seen as a cure but more as a comfort measure. After many days of discussion and presentations, no new information about a potential cure was any closer. But there were ways to make life easier for those living with EB. One of the best moments in my time at the congress was when the Australian and New Zealand nurses were asked to help advise any people who had questions regarding care. I was with a colleague from Australia and found the interaction with a young woman who had been newly diagnosed with epidermolysis bullosa acquisita (EBA) humbling and rewarding. Though I was in a period of feeling like I understood nothing, it brought me back to why we were here. Sharing of information and listening to peoples’ stories does sink in and transpose itself into practical magic. Please feel free to hit up your local EB wizard (nurse) to talk through any questions or issues you may have. Mo Blishen EB Clinical Nurse
EB Community CALEB MORRIS LIAM BRAY-SIMPSON During the 12th and 15th of November (our Canterbury Show Weekend) my To say this year’s been hectic is quite possibly the understatement of the century. family and I, along with two other We’ve been lucky to avoid the worst of the covid craziness here in sleepy little Mot, but families (so 12 of us all up) walked the we still managed to get up to a fair bit. Our nanna, Marie, stayed for a lot longer than Abel Tasman track. We caught the water was initially planned, 7 months longer in fact, thanks to covid. It’s been absolutely taxi up to Totaranui and walked back to lovely having her here, and the parting was predictably teary. Torin recently started a Marahau, camping 3 nights along the part time job to do alongside school, so he’s growing up faster than any of us could way at Onetahuti, Bark Bay and have imagined! Mum and Dad have been working too hard as usual, and I finally got Anchorage. We walked about 43ks all up around to starting a communication degree, with Massey. from start to finish with a small pack each, which had our sleeping bags, One thing that’s shifted for me relatively recently, has been that my lifelong issue of clothes and nibbles in it. My feet got recurrent corneal abrasions finally seems to have been solved, which has been quite sore but surprisingly I didn't get indescribably amazing. We heard via Sharon about the use of bandage contact lenses any blisters! Anchorage was my favourite overseas, and after bringing it up at an ophthalmology appointment, I tried them, and place to stay and I would go back and haven’t had a single abrasion in the many many months since we first started using do it all again as it was awesome. them. I go into Nelson Hospital to have them changed every 4-6 weeks, although eventually I’d like to be able to just change them over myself. Getting them changed can be slightly uncomfortable, but once they’re in they’re almost totally invisible, and I can’t feel them on there at all. At first I was suitably nervous about putting something like that directly on my eye, but it was during a particularly bad spate of abrasions, and I was somewhat desperate for an alternative solution. Silver bullet fixes don’t come along very often, but for me, bandage contact lenses have been exactly that. I hope they’re as beneficial as they have been for me, for other people with EB, because not having the looming fear of a corneal abrasion has been incredibly freeing. We’re all doing well, or as well as we can in this crazy time, and looking forward to seeing the DEBRA family at some point in the future! THE RIORDAN Caleb, Fynn, & Wayne Morris FAMILY Well done Caleb! 43 kilometres in four Sharon Cassidy enjoyed a days! The Department of home visit with the Riordan Conservation’s difficulty rating for this family recently and loved walk is ‘Intermediate’. You can see seeing their home decked from the DOC map that the terrain is out with beautiful up and down hills and includes tidal Christmas decorations. She crossings. An achievement for captured this lovely family anyone. photo. Tanya, Caleb and Fynn’s mum was naturally very impressed with how both her boys managed the tramp. She had been worried about damage to Caleb’s feet but using Cavilon Durable Barrier Cream, quick drying socks and Caleb’s regular sneakers seems to have done the trick. Hannah, Louise, and Jon Riordan
MAY'S verrucous carcinoma right medial ankle, with BTM coverage (Biodegradable Temporising Matrix, a skin substitute).In February, a third surgery was required with a large area of split EXPERIENCE OF thickness skin graft extending over much of my heel and ankle. Skin taken from my right thigh (donor site) took over 5 months CARCINOMA & (till July 2020) to heal. Trouble with walking OTHER I have calluses under my 2nd and 3rd toes, my foot is unable to go flat - my heel doesn’t go all the way down to the ground, with CHALLENGES a 10 degree angle and significant tightness in the Achilles tendon. I consulted local podiatrists and later an EB expert podiatrist, Tariq Khan from the UK who is now based in Sydney, for problem solving. I’ve had opinions from three foot and ankle Please note, this article surgeons. The 3rd opinion from Matthew Tomlinson who contains some clinical referred me to get a scanogram which confirms a 10mm details difference in the length of the femurs and a 2mm difference in the length of the tibiae. The surgeon measured my top of talus May is in her early 50s and has RDEB, and this (large bone in the ankle) to bottom of foot measurement today story tells of her on x-rays and there is no difference. That gives us a total experience with finding difference of 12mm, which is mostly in the femur. I have seen that a chronic wound Shore Orthotics and they can do an assessment of my foot, and on her ankle had take into account the 12mm leg length discrepancy and I hope developed into skin make an orthotic, which will make it more comfortable for me to cancer and how this walk. Mr Tomlinson suggested that I stick with this conservative was managed. regime but if my symptoms persist, I will go back to him for a Although people with review. some types of EB are known to have a greatly Covid with EB increased risk of Living with EB always has developing squamous its challenges and these are cell carcinoma (SCC) as compounded when you’re they get older in lock down. Regular (particularly with severe RDEB but a lower increased risk with clinical appointments were Junctional and some other forms of Dystrophic EB), this was pushed out, for instance my May’s first experience of that happening. There has been a lot five-weekly micro suction written about EB and SCC, including DEBRA International’s Best ear appointments were cancelled and oral health Practice Guidelines. It is important to note that many EB reviews with my subtypes, especially EB Simplex, are not associated with an prosthodontist were increased SCC risk. If you want to know more about the risks of delayed 6 months out. SCC, please talk to your EB Nurse Specialist. Family This is May's personal account of her experiences: During alert level 1, my I had planned to go to the EB World Congress 2020 in January husband Coen is working of this year. It would have been great to go with the DEBRA NZ 70% from home and 30% at the office. My daughter Elizabeth team and represent both New Zealand and Malaysia. My plane started her second year at the American University of Paris, ticket and hotel was booked. My mother lives in Kuala Lumpur France, majoring in Psychology, minoring in Linguistics. She is and I was going to stopover to see her on the way. studying remotely at present whilst France has their Unfortunately, EB sometimes gets in the way, even of attending confinement. a congress of world experts in EB! I had a hyperkeratotic lesion on my right medial ankle. Three different dermatology Therapeutic painting Even though my mobility was severely limited due to my pathologists had confirmed that this was not SCC, but Professor surgeries and lock down provided social challenges, I found Dedee Murrell, head of dermatology at St Georges Hospital, painting very therapeutic and continue now that I am able to be Sydney, and conjoint professor at the University of NSW, has a more active. lot of experience with EB and SCC and she suggested that her pathologist who is familiar with EB take a look at it.In August 2019, the ADHB dermatologist referred my case to Plastics service, Counties Manukau DHB, for excision of the lesion. I was seen by Richard Wong She, Clinical Lead, Burns Specialist in October.The first surgery was in December for excision of the chronic lesion. Specimen results showed verrucous carcinoma (a well-differentiated SCC variant). The second surgery took place in January 2020 for re-excision of May and Lea chatting over Zoom
TREVICHKA I am seventy-five years old and live in a body that has the systems of Dystrophic Recessive Epidermolysis Bullosa, also known as EB; it is not a disease but a genetic disorder, genetically doing what it’s meant to do. Not wanting get into negatives about this as I’m sure all such like travellers of E.B could tell their own horrific tale of woe. Instead, I would like to pass on those things that I feel are a great positive in life. I do not have the cure for E.B but definitely believe I have the next best thing. Diet, first and foremost, is of utmost importance. I became a vegan many years back because of my belief in not killing. After several months of being vegan the improvement to E.B was plainly obvious. Certain types of wounds were not reoccurring. Some of the deeper wounds, the uglier deep seated scabs. No more. When I was 57 it was suggested to me to try flaxseed every day. Just a level soupspoon in a glass covered with hot water left to soak overnight and consumed in the morning. Simple, no taste, dirt-cheap. Put it on cereal, porridge or just eat as is. Bingo, after three or four months of taking daily, the benefits started to kick in. Subtly at first, like less blisters, less open wounds and as time has passed by, the improvement has been much more obvious. Just over 18 years ago, I was practically fully bandaged, hands, arms and legs. These days I’m down to the occasional bandage and most periods with none. The blister factor has dropped significantly. Major really, and what’s more, the improvement is still continuing. And of course, that which is worth mentioning is the drop in pain factor. From huge amounts of pain every or most days to now some days and weeks are pain free. I want to pass this positive message on to fellow individuals who live with E.B. I hope that others will benefit also. I understand that this is not the cure and as a realist have not yearned for what is or isn’t a cure, but have always looked to improving the scenario whenever the opportunity has presented itself. Tough journey, yes, but life’s an experience. Experience it! I hope that the years of living with the E.B factor will not be a wasted journey and whatever one may glean from it in life, in the manner of positives, I’m all for it. I wear no bandages now for months on end and the body displays no open wounds. Up until age 57 the body was bandaged every day that one can remember except maybe the odd day. Now thanks to diet and flaxseed it is months bandage free and even then, it’s just an odd one. Bandage free means pain free. Please see next page for Trevichka's recent poetry SOPHIA Time is flying! This most difficult year is nearly gone, hopefully everything will become better in 2021! & Although many things were unimaginable in 2020, we still did a lot of pleasant things, especially during the Lockdown period. We are four members and had a wonderful family leisure time at HILTON home, James has accomplished his deck and fence, I was immersing myself in cuisine creation process and my English language training online, Lucy has some fulfillment in her painting, and Hilton enjoying everything in his amazing world, please see the photos. Of course, Hilton keeps growing well, and is continuously supported by the government and DEBRA as well. So much appreciated! I heard that 2020 is the fortieth anniversary of DEBRA, but the celebration will be held next year, right? We all looking forward to this wonderful moment, and looking forward to seeing you all again! Merry Christmas is coming soon, all the best to you all! Sophia
BABY 2020 CAMP SPIRIT RHINO BORN WITH COLORADO EB In February this year I attended a Winter camp in Colorado for people with RDEB in During lock down we read about an America. There was a great group of 10 kids and lots of staff to make the camp one to extraordinary occurrence – a baby white remember. rhinoceros was born in Hamilton zoo and she has EB. Her name is Zahra, and We had an amazing time despite being the coldest week of the winter with she is doing very well. The EB research temperatures in the minus’s. Days were jam packed with winter adventures including scientists in Auckland have been in touch with the zoo offering their expert skiing, dog sled riding, snowmobiling, and a horse and sleigh ride, finishing off with help to do the genetic diagnosis. We’d smores of course. love to have an event in Hamilton for DEBRA families and we had hoped to With busy days of fun and adventure, dressings and skin care was done in the arrange something for EB awareness afternoon/evenings. Then everyone would get together to share a meal and play some week in late October, but Covid meant games, do crafts and hang out. I loved seeing everyone have a boogie on our last night this wasn’t realistic so we’ll organise a after a large game of cards won by one of the campers. get together there next year for our EB children to meet baby Zahra. I look forward to using what I learnt to help run our Winter Adventure Camp for teens with EB in August 2021 down in the South Island in Wanaka. We also have a team of Deanna Forsyth and her children visited interested helpers from America that would love to join us one day when the borders Hamilton Zoo in September to meet open again! Zahra. The children found them to be very stinky and sleepy in the heat of the I am very grateful to DEBRA NZ for supporting/funding my attendance and to Camp day. Unfortunately, they were not able to do the usual encounter trip due to Spirit for welcoming me onto the team. Covid. Deanna Forsyth EB Clinical Nurse
Fundraising DEBRA MASKS CUPCAKE SALE Masks are increasingly becoming part of our daily lives, and probably will be for some time to come. Emily Mason and her family’s success at holding a butterfly cupcake sale at Luckily, DEBRA NZ has a selection of stylish, Emily’s school last year inspired Sharon Cassidy to organise something reusable, reversible masks, made from quality similar this year, during International EB Awareness Week. Sharon set up a cotton fabric. Patterned on one side for when you stall at Christchurch Hospital and sold cupcakes and butterfly masks (see the want to make a statement, plain on the other pictures of our DEBRA NZ reusable face masks). when you don’t. The perfect stocking filler for that Thanks so much Sharon, this is a wonderful way of raising awareness of EB person that has everything except Covid 19 and and DEBRA NZ. wishes to keep it that way. Please keep it in mind for something similar in your local area next year. Get in touch with your nurse or the office if you would like some sent out to you, either for your own use or as a DEBRA fundraiser with your local NEWTOWN community groups, friends and family. Suggested donation of $10 per mask would be appreciated but not essential. FESTIVAL SUNDAY, 7 MARCH 2021 In early March every year the Newtown Festival is a major event in Wellington, and an opportunity to fundraise for DEBRA NZ. The festival is huge with over 450 stalls and 14 stages with music, dance and performance, and over 80,000 people come from around Wellington and around the country to enjoy the day with their family and friends. The festival is organised by Martin, Anna, Rhona Carson (Anna’s sister and Humphrey’s aunty), Emi (who does much of the DEBRA graphics stuff), and a cast of many others doing small bits of this complex multifaceted event. DEBRA benefits by having lots of friends and relations volunteer, from turning up in the early morning to be marshals, or looking after the performers on a stage, or helping at a chill-out zone where people can have a rest and a cup of tea, or many other opportunities. If anyone is going to be in Wellington, available on the day and interested in helping for an hour or 2, please get in touch with Anna – 027 2717 084. It’s really fun to be part of making such an awesome event happen, then spend the rest of the day enjoying the music, food, and fun atmosphere, and the DEBRA volunteers help contribute to a significant donation to DEBRA NZ each year. Newtown Festival was the last big event in Wellington before lock-down. Our Prime Minister Jacinda Ardern was there with her family, then a week Bob Mitchell wearing his new butterfly mask. Bob later she was announcing the country was going to shut borders, stop all is selling our masks to raise funds and awareness travel, and we all had to go home, stay home. for DEBRA NZ through his Tauranga Sunrise Lions Club.
Donors & DONORS & SPONSORS Support Dr Mike Kearsley Grateful thanks to Dr Mike Kearsley, who has recently returned from the UK to his home in Papamoa. During his time in the UK he was the Masonic Grand Orator for Middlesex and Prestonian lecturer. He supported DEBRA UK with donations from the many Masonic Lodges he made presentations to, and now he is back in New Zealand he is very generously supporting DEBRA NZ in the same way when he speaks to Masonic Lodges here. We really value this support Bob Mitchell Thanks also to Bob Mitchell, a member and long time supporter of DEBRA NZ who has encouraged his Sunrise Lions Club in Tauranga to help DEBRA NZ with a generous donation. We really value the kind and Margy Bliss generous donations from friends, family, clubs and businesses that help towards our goals of making life A special thank you to the generous and multi that little bit more comfortable and less of a challenge talented Margy Bliss. for people with EB and their families. Margy has been pumping out our fundraising DEBRA Thank you facemasks and is also one of the songbirds in Mo’s crew for the Ditti’s for DEBRA concerts. Thank you to everyone who has helped out in the past year, and those who plan to support DEBRA NZ in Although Margy doesn’t have a family connection to 2021. Our supporters are wonderful people and we EB, she is a great supporter of DEBRA NZ using her really appreciate you and everything you do for talents to fundraise and her social media profile to DEBRA! raise awareness of EB. DONATIONS TO DEBRA NZ We really appreciate any and all donations & grants to help provide services & activities like the Family Camp and Adventure Camp for people living with EB. Online — www.givealittle.co.nz/org/debranz Bank transfer — DEBRA NZ Trust ANZ, Bay Road Branch 06 0513 0192618 25 Cheque — DEBRA NZ PO Box 7316 Newtown Wellington 6242 New Zealand Payroll Ask your employer if they offer payroll giving. Your donation to DEBRA NZ is taken out of your regular pay before it goes into your bank account. If your donation is more than $5, you receive a third of the donation amount as an immediate tax rebate. More information at IRD. Donations are tax deductible. To receive a receipt from DEBRA NZ, please provide your name and contact details with your cheque or via email to debra@debra.org.nz with Ask us for a copy of the code for your next the amount and date of the donation. fundraising event!
Keeping in Touch Did you know that there is a DEBRA NZ OFFICE LOCK DOWN Facebook page and a DEBRA NZ Members private group page? Anna Kemble Welch Director If you haven’t joined, you might find it 027 2717 084 What were your experiences with lock an interesting way to keep more in down? touch. There are over 420 people Lea Prujean & Anna KW following the DEBRA NZ page which is debra@debra.org.nz It was nerve wracking trying to get PPE open to the wider community, and gear for the DEBRA nurses, make sure currently 16 members in the DEBRA NZ NURSES everyone who needed them had back- Members group which is a private group up bandage supplies in case imports for people living with EB and those Jacqui Finnigan were delayed and supplies not directly involved in EB care. EB Clinical Nurse Specialist available, and to try and find out if Covid 0211 960 960 was likely to have any extra impact on jacqui.finnigan@debra.org.nz people with EB that was worse than for others. As far as we could find out Sharon Cassidy internationally, it was no worse a risk EB Clinical Nurse Specialist except for our concern about the risk of 021 2410 447 being in a hospital environment sharon.cassidy@debra.org.nz dedicated to Covid, isolated from EB specialist dressings and woundcare, or Mo Blishen risking intubation with ventilators. No- EB Clinical Nurse one knew what to expect. We did get off 021 0233 0935 very lightly in NZ, thankfully. mo.blishen@debra.org.nz Deanna Forsyth EB Clinical Nurse 021 2442 992 deanna.forsyth@debra.org.nz Christmas New Year Hours The DEBRA office will be closed 21 December to 11 January, but the email address debra@debra.org.nz will be checked intermittently. If you need urgent attention and have not been able to contact one of our EB nurses please contact Martin Hanley, 027 4169 731. Newsletters are for you, our DEBRA family. We love to hear your stories, achievements, sorrows and joys, big and small, your day to day experiences. The lot :) ! All contributions are very welcome. Feel free to send items or make suggestions about things that would interest you to Anna or Lea.
Teen Camp, Wanaka, New Zealand WINTER 2021 Join the DEBRA NZ team, nurses and others with EB - for a 5 day camp full of fun winter adventure activities! Dates: (4 nights) Costs: Saturday the 21st - Wednesday the 25th TBC - some fundraising/self funding of August 2021 will be required Age: Activities: 15-20ish years - campers 2 day’s skiing at Cardrona 20-30ish years - camp helper / mentor Visit to Puzzle World, Toy Museum, Accomodation: Horse ride or Quad Biking Zula lodge, Albert Town, Wanaka Team building, games and crafts Please register your interest with DEBRA NZ: debra@debra.org.nz Get in quick as LIMITED SPACES available! [1]
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