Quarterly Advocate Winter 2002 In This Issue: BCCNS Life Support Network Western Regional Conference San Francisco AAOMS Convention Chicago Gorlin Group UK Meeting First meeting of a Gorlin Support Group in the Netherlands Band Aid Bear Recall Clinical Trials Update
BCCNS Life Support Network Western Regional Conference San Francisco
On October 26th, 2002 the BCCNS Life Support Network hosted our first Northwestern USA Regional
Conference at the University of California San Francisco. We were very fortunate to be the guests of Dr.
Over 40 attendees were treated to a full day of learning, sharing & caring. The sessions started off with
introductions and expectations of the day stimulated by Kristi Schmitt Burr. An explanation of our
organization and our mission was presented by Bud Caruso.
Attendees enjoyed a very thorough explanation of Basal Cells and prevention by Dr. Ervin Epstein Jr. Dr.
Epstein also explained their current study using Celecoxib (Celebrex) a Cox II inhibitor to prevent the
growth of BCCs. The Celecoxib study is in the Clinical Trial stage and the results are very promising. Dr.
Epstein also invited his staff to attend the meeting. As a result his team members left with a greater
sense of purpose for their work and higher level of compassion and understanding for those affected by
BCCNS. We all owe huge thanks Dr. Epstein. http://bccns.org/celebrexupdate.htm
Mohs Surgeon, Dr. Christopher Zachary (http://www.ucsfhealth.org/adult/cgi-
bin/prd.cgi?action=DISPLAYDOCTOR&doctorid=1389 ) provided a very detailed presentation on how
Mohs surgeons deal with BCCs that have developed aggressively and reconstruction of wounds created
by their removal. Many important points were brought forward by Dr. Zachary’s presentation including:
• Electro desiccation & curettage (ED&C), Mohs or laser as treatment options for surgical removal
• Recurrent BCC vs. new BCC in the same location. • Healing by secondary intention vs. closing the wound. • Harvest sites, “spare parts” and when to use them. • Use of hydrogen peroxide & antibiotics after surgery (or not)
After Dr. Zachary’s presentation our members agreed that prevention and diligent maintenance are
paramount in dealing with BCCs. Thank you Dr. Zachary
Oral & Maxillofacial Surgeon Dr. Brian L. Schmitt also of UCSF Medical Center (www.ucsf.edu/omfs)
enlightened us as to the various methods of removing BCCs. Dr. Schmitt was extremely informative and
helpful to our members. Some of the topics covered were:
• Marsupialization (Draining the cysts over a period of time to promote bone growth) • Carnoy’s Solution (An acid based solution used to kill off residual OKC cells and bacteria) • Chryotherapy (Use of liquid nitrogen to destroy residual cells and sister cysts) • Origin of Odontogenic Keratocysts (OKCs) (From bacteria)
• Digital Imagery (3D images of the cysts are now available to the surgeon) • OKCs as neoplasms • Biopsy of cysts
Dr. Schmitt was an absolutely wonderful presenter who expressed genuine caring for his patients and a
passion for his work & research. We are very thankful to announce that Dr. Schmitt has agreed to be an
active member of our Medical Advisory Board. Thank you Dr. Schmitt.
Some of our members and new found friends gathered for an evening of fine dining at Yabbie’s, one of
San Francisco’s many wonderful restaurants. If you are fortunate enough to have the BCCNS Life
Support Network host a regional conference in your region you’ll want to make every effort to attend.
AAOMS Convention Chicago
On October 2nd – 5th 2002 Jennifer Werkmeister, Kristi Schmitt Burr and Bud Caruso represented the BCCNS Life Support Network with an exhibition booth for the American Association of Oral & Maxillofacial Surgeons 84th annual meeting, scientific sessions and exhibition, at the beautiful Lakeside Convention Center in McCormick Place, Chicago.
The trio were exhausted after days of setting up displays and distributing information creating awareness of BCCNS and our organization. The organization and information were very well received. Many surgeons sought out our booth to obtain information for their patients. We were informed of many families in need of our services. We look forward to assisting them.
Dr. James Hanna was instrumental in obtaining the booth space and encouraging our attendance. We also had the opportunity to sit in on some of the scientific sessions where various methods of removing Odontogenic Keratocysts (OKCs) were presented debated. The sessions helped us in better understanding conservative methods of removal and continuing our mission of keeping up with the latest available information to our members.
On Saturday evening we joined with some of our members in Chicagoland for a social event held at Ed Debevic’s restaurant. A good time was had by all.
Be sure not to miss this awesome opportunity to meet & bond with your fellow BCCNS patients and the
ability to become informed on the syndrome by some of the world’s foremost authorities on the
subject. We look forward to seeing all of you there.
We have attached a registration form in MS Word Format. Please complete the form and mail it to out
office as soon as possible. If you have any questions or trouble downloading / printing the form please
Gorlin Group UK Meeting
On November 9th, 2002 the Gorlin Group UK hosted its 10th annual meeting. Over 70 of their members
were in attendance. The group was treated to a full day of information sessions. http://www.gorlin-
Professor Peter Farndon being the keynote speaker described in great detail the genetics responsible for
the syndrome. Professor Farndon also described the crucial role of the Costello family whose DNA was
used in discovering the defective gene responsible for the
disorder.http://www.bham.ac.uk/ICH/staff.htm#farndon
Dr. Ernest Allan & Dr. James V. Moore addressed the group on the advancements in Photodynamic
Therapy (PDT) in the UK. The team announced that trials with Photofrin II have started. Dr’s Allan &
Moore were kind enough to bring the actual devices used in the procedure. The Paterson Lamp used in
PDT was developed at the Paterson Institute for Cancer Research. Attendees had the opportunity to see
first hand the potential benefits of PDT and to ask questions regarding the therapy from two of the
pioneers in the field of PDT. The group expressed a tremendous interest in the
treatment.http://www.christie.nhs.uk/default.htm - http://www.paterson.man.ac.uk
Margaret Costello and Sally Webster provided the group with an update on the organization. A move
toward charitable status is being pursued.
The BCCNS Life Support Network was represented by Bud Caruso who addressed the group on the
progress of our organization and the importance of participating in clinical trials and various studies. We
also took the opportunity to promote our conference in April. We are happy to report that, to date,
three families will be attending our meeting as a result.
Our good friend and valued member Bill Costa also attended the meeting. I know all of the children now
hold a warm place in their hearts for him. Having the opportunity to witness the interaction with Bill and
the UK group members, I am certain many new friendships were also solidified. Atta Boy Bill!
While in the UK we had the opportunity to socialize with our good friend Jim Costello the founding
member and driving force behind the Gorlin Group UK. Jim was instrumental in helping us form the
BCCNS Life Support Network. With time we all became very close to Jim and the Costello Family. We feel
Jim is a Brother to us all. As many of you know by now, Jim passed on just a short time after our visit.
We are all devastated by his passing. Our deepest condolences go out to his many friends and his family.
The BCCNS Life Support Network was represented at his funeral by Kristi Schmitt Burr who expressed all
of our sympathy. To our Brother Jim we say “Au Revoir”, for we will meet again.
Here is a note from Margaret Costello Jim’s wonderful wife and soul mate:
It is with regret and a deep sadness that I write to inform you of the death of Jim Costello. Jim passed
away peacefully, surrounded by his family at St Catherine's Hospice, Lostock Hall, Preston on Monday,
Jim was a loving, caring husband and a devoted Dad to Jane, Helen, Anna (deceased) and Richard. Since
his retirement in 1989 Jim dedicated much of his life to raising awareness of Gorlin Syndrome and
supporting many fellow sufferers. I know Jim valued the friendship and support of you all. On behalf of
Despite personal difficulties Jim retained his dignity and never lost his sense of humor. I am sure you
will agree, he was an inspiration to all. Jim will be sadly missed but remembered with affection.
It was Jim's wish that donations in his memory be divided equally to benefit St Catherine’s Hospice and
the Genetic Research Trust Fund F06062 to aid research into Gorlin Syndrome. Cheques made payable
to preferred charity can be forwarded to Mrs. M Costello, 11 Blackberry Way, Penwortham, Preston,
First Meeting of Netherlands Group
While en route to the Gorlin Group UK meeting, Bud Caruso was fortunate enough to attend the first meeting ever of a support group being formalized in the Netherlands. Willem Breurken who has been providing services to people in Holland, Belgium and Germany for almost three years now, hosted the first meeting of people he has been helping and communicating with. http://members1.chello.nl/~wf.breurken/Persoonlijke%20webpagina.htm
Willem was kind enough to organize a meeting hosted in his home in Almere, Holland. Together with his wife Betty and their entire family, Willem provided the experience of a lifetime for his group. Those of us who have met in person, others with the syndrome are aware just how emotionally overwhelming the experience can be. After a lifetime of suffering from the disorder to have the ability to share with others who have walked in your shoes is a wonderful experience. Willem & Betty made everybody feel welcome. Willem distributed information on the disorder and played the BBC programme on Gorlin Syndrome for those who had not previously viewed it.
The group is off to a great start and I am certain the right man is at the helm. Willem recruited several members to assist in formalizing the group and their future activities.
In the days that followed Willem was an awesome ambassador for his country. He showed my Mother (Jean Thompson) and I a wonderful time with sightseeing and a private tour of the countryside. Willem was also keen enough to arrange a visit for us to the University of
Amsterdam’s Medical Museum. This was an event I will remember for life. Here medicine has been studied since the early 1300s. Their collection goes back just as far. Actual examples of bifid ribs and skeletal remains of persons with enlarged cranium, bifid ribs and scoliosis were observed. The tour was given to us by the Museum’s director who is studying the origins of medicine. He was even kind enough to take us in the basement storage area to show us even more examples that were not currently being displayed. A wonderful experience! Many thanks to the Breurken family.
Band Aid Bear Recall
I am very sorry to say that I have some hard news to tell you. The Big Bear that has been sent out from
the BCCNS Life Support Network has been recalled by the Build A Bear Company. They are no longer
making it and it has been/will be taken off the shelves. I received a call today from the Build-a-Bear
company informing me of the recall. You can read about the recall here at this
link: http://www.buildabear.com/GuestServices/SafetyMessage/
I am going to be sending you a letter from the company instructing you what to do with the bear. The
company asks that I send these to you. If you have a store near you, you will be able to go to the store
and exchange or you will need to follow the instructions that I will send to you. You can see if there is a
build-a-bear store near you by going to:
I am sorry for any problems that this may cause.
If you have any questions please feel free to contact me.
AAD Convention San Francisco
The BCCNS Life Support Network will be attending the American Academy of Dermatology 61st Annual
Meeting March 21-26th, 2003 in San Francisco. The BCCNS Life Support Network will have its brochures
and reprints available to attending physicians in the Coalition of Patient Advocates for Skin Disease
Research (CPASDR) booth. This was a very beneficial event four our organization last year. We
anticipate each year will provide new contacts and opportunities. We are thankful to be participating at
Clinical Trials Update
QLT Industries (formerly Quadra Logic Technologies) the company that originally developed Photofrin, one of the drugs used for PDT has moved into the clinical trials stage for their study of a new sensitizing drug Verteporfrin. The drug promises to be as effective at removing BCCs while the residual photosensitivity is dramatically reduced. For more information please visit http://www.mbccstudy.com
Using the Cornell late blight simulator to test biological questions: a case study on the influence of the source of irrigation water on Phytophthora infestans development O Frenkel1, D Shtienberg1 and U Yirmiyahu2 Computer simulation models, if properly validated, can be used as research tools. The models can be used for testing biological hypotheses by conducting simulation experim
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