I had not heard of Krabbe disease until 2011, when a relative was diagnosed with it. Krabbe is also called Globoid cell leukodystrophy; Galactosylcerebrosidase deficiency; Galactosylceramidase deficiency. It is a rare genetic disorder of the nervous system and is a type of leukodystrophy. It is named after the Danish neurologist Knud Haraldsen Krabbe who discovered it.
Krabbe disease is caused by a defect in the GALC gene. People with this gene defect don’t make enough of a substance called galactocerebroside beta-galactosidase (galactosylceramidase).
This is needed by the body to make myelin, the material that surrounds and protects nerve fibers. Myelin breaks down without it, brain cells die, and nerves in the brain and other body areas do not work properly.
There are two forms of Krabbe disease.
Early-onset Krabbe disease appears in the first months of life.
Late-onset Krabbe disease begins in late childhood or early adolescence.
Krabbe disease is inherited, which means that it runs in families. To get this disease, each of your parents must pass you a copy of the faulty GALC gene.
This condition is very rare, Affecting 1 in 100,000 births.
Symptoms
Changing muscle tone from floppy to rigid.
Hearing loss that leads to deafness
Feeding difficulties
Irritability and sensitivity to loud sounds
Severe seizures
Unexplained fevers
Vision loss that leads to blindness
Vomiting
The condition is often misdiagnosed as cerebral palsy.
There is no specific treatment for Krabbe disease.It is possible to have a bone marrow transplant in the early stages of the disease, but this treatment has risks.The outcome of Krabbe is usually not good. On average, infants with Krabbe die before age 2. People who develop the disease at a later age have survived into adulthood with nervous system disease.
A blood test can be done to see if you carry the gene for Krabbe disease and prenatal tests can be done to screen a developing baby for this condition.
Coldplay and Noel Gallagher have already signed up to the new currency at www.bluedotworld.com, which gives fans access to live shows and exclusive material by artists, authors and movie stars in return for donations, volunteer work and media sharing.
Blue Dot is the new social currency given to people who do good things for their favourite charities by sharing on social media websites, volunteering and donating to any one of the 100k non-profit organisations globally. The idea also influences young people to get involved with charities, as a recent survey conducted by SAGA showed that people over the age of 75 are the most likely generation to give to charity… yet Blue Dot is hoping to overturn these figures by rewarding youngsters with amazing prizes for ‘doing good’.
Within moments of the site going live, Noel Gallagher offered last chance tickets to his debut UK dates exclusively to Blue Dot citizens. Within 48hrs, thousands of positive actions had been taken for causes, including cash donations to over 70 UK different charities. Coldplay also used Blue Dot in the UK and US, to offer the chance to see them live exclusively to fans of Blue Dot.
Blue Dot is rapidly claiming recognition and credibility… founder of Blue Dot, Chris Ward, filmed exclusive backstage footage for Blue Dot with artists at BBC’s Children in Need, including with Ed Sheeran, Rizzle Kicks, Coldplay, JLS, One Direction, Snow Patrol, The Saturdays, Elbow and James Morrison.
One Direction have commented: “We’re thrilled to be part of Blue Dot; It’s a brilliant concept and a great cause. We really hope that our exclusive dressing room acoustic performance of ‘What Makes You Beautiful’ encourages people to donate.”
Nichole Scherzinger, Professor Green, Katherine Jenkins, Rebecca Ferguson, Anton Du Beke and Joe McElderry are amongst others that have donated singed albums and tour tickets.
Blue Dot Founder, Chris Ward said: “I was previously Creative Director of the UK’s Comic Relief, where we work first hand with major stars and brands to create attention-grabbing moments that engage millions of people in volunteering and charitable giving.
I created Blue Dot, as a genuinely new & innovative way to enable every single Cause to create that same impact, every single day of the year.”
World Bank sits on 90% of Unspent Funds for Forest Projects
Vivienne Westwood & Frank Field MP Launch Campaign to Expose Failings
Dame Vivienne Westwood is investing 1 million of her own funds to launch a 7 million pound fundraising campaign supporting the rainforest charity Cool Earth. The action is to highlight World Bank’s expenditure failings as a staggering 90% of funds pledged to halt deforestation remains unspent. (See bottom of press release for details). The designer and environmental campaigner, coupled with Frank Field MP (founder of Cool Earth) aim to demonstrate how rapidly money can be deployed into rainforest programmes.
The campaign called “No Fun Being Extinct”, (nofunbeingextinct.org) supported by fashion’s biggest names, such as Kate Moss, Lily Cole and Sadie Frost will run for 18 months during which time Westwood will aim to help Cool Earth secure three of the world’s most endangered forest.
Former Minister, Frank Field founded the rainforest charity Cool Earth in 2007 as a vehicle to allow ordinary people to leapfrog governments and take immediate action in the fight against climate change by protecting rainforests. The conservation method is a ground up approach, which works with indigenous communities to make rainforest trees of greater economic value left standing than cut down.
Despite a growing number of research papers highlighting community led management as the most effective way to keep rainforest standing over traditional reserves, many communities are not receiving pledged funds.1, 2
The Climate Investment Fund dedicated 600 million dollars (£390 million) to the Forest Investment Programme (FIP) to “tackle drivers of deforestation” with UK tax payers providing the most generous contribution – almost four years on, only 15 million dollars (£10 million) has been spent, all of which has gone on administration and advisors.3 Vivienne Westwood will demonstrate how taxpayers money should be spent when it comes to saving rainforest for the benefit of the nations future.
Frank Field founded Cool Earth as a result of his utter despair at governments’ failure to solve the problem of deforestation, “The lack of action in spite of such generous funding is a real disgrace. It shows that political will is just as important as money.”
Fashion designer and environmental campaigner, Vivienne Westwood, has been working closely with Cool Earth over the last 3 years, “Cool Earth has a plan to save the rainforest. If we don’t save the rainforest forget it! I am personally supporting Cool Earth and investing in our future. I’m inviting anyone interested in saving our beautiful world to join me.”
Frank and Vivienne are calling on the government to invest in community-led forestry management, which has been proven to be the most effective way to halt deforestation. Vivienne is presenting a report to No. 10 to showing how her funds have been spent to show that an effective mechanism to halt deforestation does exist.
Cool Earth has protected over 2.5 million acres of vulnerable rainforest since its launch in 2007, which has cost just £1.75 million with less than 10% spent on administration. This is because the charity has worked from the ground-up with local communities who have a clear interest in keeping the forest standing. This works out as costing just a half of a percent of the FIP’s budget.
Vivienne will also be calling on the public to help protect trees at Cool Earth’s new website launched on a November 28th called ‘No Fun Being Extinct’. Individuals can save as little as 3 trees for £3 on the website at www.nofunbeingextinct.org. Every tree makes a difference.
U.S. supermodel Caprice and American InterContinental University celebrate Thanksgiving with a meal for the homeless for second year running
Supermodel Caprice, along with students from American InterContinental University London, celebrated their heritage and the American holiday of Thanksgiving yesterday, 23rd November by providing a meal for homeless women for the second year running.
Supermodel Caprice, who is renowned for being an ‘all-American’ girl joined in the festivities by helping to carve the giant turkey and serve out the dinner – waving American flags all the way. AIU London students were also on hand to serve out the traditional Thanksgiving meal of turkey, mashed potatoes, sweet potato, stuffing, and gravy. American flags and pumpkins decorated the room as the women enjoyed the seasonal feast.
Caprice said: “I’m so delighted to be sharing the spirit of Thanksgiving with the women of the Marylebone Project and the students of AIU London for the second year in a row. An American Thanksgiving is all about bringing people together, so to be able to celebrate with these inspiring women is a real honour.”
The roast turkey meal was provided to more than 60 homeless women at the Marylebone Project, based on Cosway Street, in Marylebone London. The Marylebone Project works with vulnerable homeless women to help empower them towards independent living through making informed choices.
Dr. Randolf Cooper, Campus Director and VP of Academic Affairs at AIU London, which is also based in Marylebone, said: “Thanksgiving arrived in America with the Pilgrims in the autumn of 1621, when the 53 surviving Pilgrims celebrated their successful harvest, as was the English custom. Today Americans celebrate Thanksgiving without a religious connotation; the emphasis is on abundance and family. We are happy to share that spirit with the community and delighted that our students can be involved in supporting a local charity such as the Marylebone Project.”
Established in 1978, AIU London offers a wide range of U.S. associate, bachelor’s and master’s degrees in Fashion, Visual Communication, Interior design and Business. AIU London design programs are validated by the University for the Creative Arts www.ucreative.ac.uk to award BA (Hons) degrees in Fashion Design, Fashion Marketing, Fashion Marketing and Design, Interior Design and Visual Communication. The AIU London business program is validated by Buckinghamshire New University www.bucks.ac.uk to award BA (Hons) degrees in Business Administration.”
AIU is accredited in the United States by The Higher Learning Commission and a member of the North Central Association, a regional accrediting body for U.S. degrees.
Let the bells ring out for every child in the world this Christmas.
This festive season, forget the latest iPhone, toy or gadget and give your family and friends a
truly inspirational present by treating them to a UNICEF Inspired gift, which will be delivered
to a vulnerable child around the world on their behalf.
UNICEF’s Inspired Gift range is unique, suitable for all budgets and easy to buy online.
All the inspired gifts are real lifesaving supplies delivered to children, some living in desperate
conditions around the world. They include items such as medicines, foods, water containers
and education materials. Your friend or family member will receive a gift card, which tells
them how their gift is making a real difference to children’s lives.
Now, with the ‘children’s famine’ affecting nearly 2 million children in Somalia alone and its
impact likely to last for years to come, there is no better time to add a UNICEF Inspired Gift to this year’s Christmas shopping list.
There is a gift for every budget;
• For £13 you can purchase life-saving therapeutic milk to help a child suffering from
severe malnutrition to survive another day.
• You can help five families protect themselves from malaria with mosquito nets for just
£20
• For £12 you could brighten a child’s day with five story books
• For those with a slightly bigger budget, £150 will enable you to deliver an entire
‘school in a box’ to children caught up in an emergency so they get back to learning
as soon as possible
UNICEF is the world’s leading children’s organisation, responding to more than 200
emergencies each year and working in every country to make sure the world’s most
vulnerable children are reached. Every year, more than 8 million children die before their fifth
birthday, mostly from preventable causes, that’s almost one child every four seconds. This is
wrong but by purchasing one of UNICEF’s inspired gifts this Christmas, you can help to put it
right.
For those who want to wrap up something to go under the tree then UNICEF also offers a
more traditional selection of cards and gifts including gorgeous handmade leather bags and
ethical jewellery: www.unicef.org.uk/shop
Our top 10 Inspired Gifts
All of the photos can be provided as high-resolution images on request. Product photos also
available. Prices effective as of 1 September 2011
Emergency water kit for a family £8.50
Give a UNICEF Inspired Gift that will enable a family caught up in an emergency or natural
disaster to collect, store and even purify water.
Deliver a baby £27
Provide all the equipment and medicines needed for the safe delivery of a new baby.
Life-saving milk £13
Give life-saving, therapeutic milk for the treatment of severe child malnutrition.
Water pump £320
Buy a water pump and help provide clean, safe drinking water for a whole community.
Peanut paste to save a child from malnutrition £23.50
Help malnourished children with this life-saving therapeutic food. It’s a high-protein, peanutbased
paste that comes in a ready-to-use sachet.
Five mosquito nets £20
Mosquito nets for five families to protect them from malaria. Malaria kills one African child
every 30 seconds.
Three months HIV medicines for a mum and a baby £12
Provide life-saving anti-retroviral medicines for a mother and baby for three months.
Polio vaccines £9.50
Protect 100 children from this highly contagious viral infection.
School-in-a-box £150
This emergency education kit provides the school supplies that children need to continue their
lessons as soon as possible after a disaster.
Story books £12
Open up the delight of story-time for whole groups of children with five story books.
UNICEF’s full range of Cards, Gifts and Inspired Gifts* are available exclusively online at www.unicef.org.uk/shop or by calling 0844 888 5505.
The recent X Factor ChildLine Ball at the Savoy raised more than £600,000.
Presenter and writer Esther Rantzen said: “It was a fantastic evening, a really thrilling way for ChildLine to celebrate this very important year, its 25th. Dermot O’ Leary was his usual charming self. Mary Byrne and Rebecca Ferguson were wonderful as they always are. And we raised a staggering amount of money, thanks to Johnny Gould and Peter Dickson. This means we can answer all the desperate children who ring ChildLine and contact us online, giving them hope, advice, and a lifeline.
“Today is also the publication date of my book Running Out Of Tears; telling the story of young adults who rang ChildLine when they were children, and how those phone calls transformed their lives. SO this is a landmark year in ChildLine’s life and in mine.”
The National Autistic Society (NAS), the UK’s leading charity for people affected by autism, is asking friends, families and colleagues to get together during the festive season and raise money for charity by throwing a ‘FestiviTea Party’. Organising your very own bash couldn’t be easier and requires just three very simple ingredients: tea, cake and friends!
FestiviTea Parties are a chance to roll up your sleeves, don your aprons and bake up a storm with your nearest and dearest, whilst at the same time raising vital funds for the National Autistic Society. You can hold your party anywhere and at anytime. Whether it’s at home, work or out in the local community, you can make your FestiviTea Party as elaborate or as laid back as you like. How you choose to raise the money is also up to you – ask guests to buy tickets, make a donation, or pay for the cakes and biscuits you bake!
Christmas can be a stressful time for many families, but it can be extremely difficult for the half a million people affected by autism, who can often become confused and frightened by sudden changes of routine, unusual foods and new social activities. We need your help to continue to provide vital support and services for individuals and families affected by autism across the UK.
Jane Asher, President of The National Autistic Society and famous for her cake baking said: “I love baking, especially at Christmas time, and if you have children FestiviTea is a great activity to get them involved in too. But you don’t have to be an expert baker or spend lots of time putting together your own tea party: you can keep things simple and easy to organise by asking your friends to help out – or by buying ready-made supplies from the shops.”
“This is the time of year when we all like to get together with our loved ones – but it’s also the perfect time to think about helping those who find Christmas celebrations frightening and confusing. FestiviTea lets you do both at the same time: you’ll have fun with family and friends and every penny you raise will go towards helping the half a million people with autism in the UK.”
Last year, FestiviTea Party organisers across the country raised a fantastic £16,000 and this year, we want to get everyone in the Christmas spirit and raise even more. Every penny raised makes a difference and helps ensure that people with autism across the UK have the support they need to live fulfilling and rewarding lives.
To find out how to organise your own FestiviTea Party, visit: www.festivitea.org.uk or order a pack by calling 0845 180 0426 or emailing festivitea@nas.org.uk.
Too Many Women is an incredible initiative, which aims to raise £1million for the pioneering work of the Breakthrough Breast Cancer Research Centre. It is supported by a number of high profile celebrities and ambassadors, including Scarlett Johansson, Sienna Miller, Gwyneth Paltrow, Pippa Middleton and Matthew Williamson.
GUESS Watches is delighted to be working alongside Too Many Women and are launching a limited edition Sparkling Pink watch to raise money and help Too Many Women reach their goal of £1million.
The Sparkling Pink watch features with a polished silver bracelet and multifunction dial, which are both elegant and functional. With the addition of twelve pink accent crystals around the facia and the iconic pink Breast Cancer ribbon logo, this striking design supports a great cause and aims to be a chic wardrobe staple.
GUESS Watches is committed to raising funds and awareness to help fight the battle against breast cancer and is excited to be among the many supporters joining forces to assist Too Many Women and Breakthrough Breast Cancer.
