Wall Street Journal Reports on Cyclodextrin, NPC, and HIV-AIDs

Dear British Media – Feel Free To Call or Email!

Dear British Media:

Thank you so much for your interest in our daughters, Addi and Cassi, and their fight against Niemann Pick Type C disease, a fatal cholesterol disorder that is often referred to as the “childhood Alzheimer’s.”  There have been two stories that were written today regarding Addi and Cassi’s cyclodextrin sugar  treatment and we can’t thank you enough for bringing this important health story to the attention of people in the UK.

Unfortunately, there are some incorrect facts in the stories (see below) that are now appearing online.  It also would have been courteous to contact us and conduct fact checking.  We realize that it’s a competitive media environment and you are in a world of meeting daily deadlines but we do not appreciate being quoted in stories when words did not come directly from us. We are more than happy to tell our story to the world (we are trying to do this all the time through our website!) and you can call or email us any time day or night as our contact information is on our website.

Again British media, thank you for getting the word out on Niemann Pick Type C disease.   While considered a “rare disease,” Niemann Pick Type C disease could have implications for millions of people.  The Niemann Pick Type C gene on Chromosome 18 is a very important gene in the human body – we all have this gene and it appears to regulate people’s cholesterol at a cellular level.  We hope that more discoveries regarding the NPC gene will come out soon.

The Evening Standard:

http://www.thisislondon.co.uk/standard/article-23667508-details/The+twins+aged+five+who+suffer+from+Alzheimer+s/article.do

Correct facts:

• Our names are Chris and Hugh Hempel. Chris is Mom, not Dad, as you have reported in your article

• Our children’s names are Addison and Cassidy, we refer to them as Addi and Cassi – no “e’s” at the end of their names

• The US FDA did not give the approval to us today to start these treatments on our children – this was approved a few weeks ago and we are in the process of gearing up for the treatments

• Children’s Hospital of Oakland California is not conducting the actual infusions – mediports for the sugar infusions were surgically installed at this hospital. Cyclodextrin infusions will take place at Renown Regional Medical Center in Reno, Nevada, USA. We are working towards the infusions right now and hope to start in a week or so

• We have no idea if cyclodextrin can help Alzheimer’s patients as was quoted in your story. However, this is an interesting idea and cyclodextrins amazing properties should be looked into further

• Doctors did not discover cyclodextrin, researchers at UT Southwestern University in the United States have reported on cyclodextrins effects in NPC mice

Telegraph:

http://www.telegraph.co.uk/health/healthnews/5055351/World-first-treatment-may-offer-hope-to-twins-with-childhood-Alzheimers..html

Correct facts:

• Niemann Pick Type C does not only impact children, it impacts adults too and there are a number of adult cases in the UK.  Children experience dementia, tangles and other symptoms that are similar symptoms seen in Alzheimer’s disease.  Possibly the two diseases are related to disruptions in lysosomal function in the cell?  Why are Alzheimer’s researchers not looking more at NPC and Lysosomal Storage diseases??

• The proper spelling for the disease is Niemann Pick Type C, not Niemann Pick’s

• Did you find quotes from Chris from previous videos on our website?  Feel free to contact us and we will be happy to give direct quotes that are current

• This is not the world’s first treatment – we actually provided all of the cyclodextrin information we submitted to the US FDA to a family in India and Lebanon. Cyclodextin infusions have been started on two children in these countries based on our ground work.  Due to the fact that these countries do not have the same types of restrictions that we have in the United States, these families have been able to start over 1 month ago!  This will be the first time in the US this will be tried

Again, thank you for starting the education process on Niemann Pick Type C disease.   Please pray for all who have this horrible and devastating illness that is stealing their minds.

Addi & Cassi Appear On November Cover of “The Scientist”

 

Our sincere thanks to Alison McCook along with the editors, designers and publisher of The Scientist for this incredibly well written story that takes an in-depth look at Niemann Pick Type C disease and explores the possibility that NPC research could provide insights into the mechanisms and potential therapies for Alzheimer’s disease.  You can read the entire story on The Scientist website (free registration). To download a PDF version of the November 2008 issue of The Scientist article, choose one of the following links:

• High Resolution good for printing (10mb)
• Medium Resolution good for reading (3mb)
• Low Resolution good for dial-up connections (900kb)

 

Dr. Phil’s “The Doctors” Show To Bring Childhood Alzheimer’s Into National Spotlight

 

On Monday, September 22nd, 2008, our family will featured on "The Doctor’s," a new Dr. Phil spin-off television show that is in its first season.  Addi and Cassi’s battle against Niemann Pick Type C, also known as the "Childhood Alzheimer’s" disease, will be discussed and we will appear as guests on the show.   Addi and Cassi will also make an appearance towards the end of the segment — you can see the girls getting into all the apples on the set!   Watch us now.

Thank you so much to The Doctor’s and to Dr. Phil for helping create this new show to bring reliable medical and health advice to our nation.   We hope this new show will help generate national awareness of our fight to beat this fatal illness that destroys the brain and kills children in childhood.  Please take a moment to read our story and make a wish for us today!

Rare Disease Takes Priority Over Vote

Reno Gazette Journal
Rare disease takes priority over vote
January 20, 2008

While most Americans are focusing on narrowing the list of candidates for the office of the presidency, I have found myself preoccupied with another serious issue. My friends Chris and Hugh Hempel discovered that their identical twin daughters, Addi and Cassi, have a rare and incurable disease called Neimann Pick Type C (NPC). The family has begun a long, hard journey to find a cure. A group has come together to do a fund-raiser in May that will help with the research on this illness. While I believe that the election is of utmost importance, and I will certainly participate in the political process, I hope that this community will join us in the fight to save these little girls’ lives. I recently found myself disenchanted with the slate of candidates for the office of presidency. I criticized every presidential candidate while I conversed with my husband. I then realized that I have not felt at ease since hearing the devastating news from our friends.

I know that the office of the presidency is the most important one in the country, and I hope that the best person wins the election, but I will be working with a group of friends to understand this disease, plan a fundraiser for research, and help Addi and Cassi while they undergo multiple treatments. Two years ago, the girls had enlarged spleens. When their health problems continued, their parents took them to Stanford and it took some time to isolate the problem. Yet no one was prepared to hear the news that the girls had a severe disease. We all cried. Neimann Pick is a group of inherited metabolic disorders referred to as leukodystrophies or a lipid storage disease in which harmful amounts of fatty substances or cholesterol are accumulated in the liver, spleen, lungs, bone marrow and the brain. Some of the progressive symptoms include lack of muscle coordination, learning problems, loss of muscle tone, slurred speech and swallowing problems. The children can also develop a childhood form of Alzheimer’s. 

While there is no cure, there is research being conducted that may help the girls and in the end will help others who have problems with cholesterol and Alzheimer’s. The National Institutes of Health is following the girls. There are many research projects under way to find a cure for NPC. The girls are receiving a medication that normally would cost $160,000 a year for both of them. There are children with NPC who do not have access to these treatments. Thus, a group has come together under the direction of one of our neighbors, Maria Muzea, to put together a fund-raiser to be held May 10 in Reno. Interested participants can contact The Hempels at www.AddiandCassi.com.  So, as you move forward with the election, please keep these children in mind as well as the need to find new treatments for many diseases.

Ellie Lopez-Bowlan is a regular contributor to Voices

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