|
|
 |
Newsletter 1996
VHL Family Forum, Volume 4, Number 1 - March 1996
Section 1
|
This newsletter is distributed to members of the VHL Family Alliance. It is supported by dues, fundraising, and advertising. We welcome your comments, suggestions, ideas and submissions. Copyrighted works or their modifications must be accompanied by the copyright notice. Opinion(s) expressed by the authors are not necessarily those of VHLFA.
Further information is available from the VHL Family Alliance, info@vhl.org
by Robert B. Welch, M.D., Associate Professor of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland
Cryotherapy is the therapeutic use of cold. The name is derived from the Greek word for cold, kryos. Its use is based upon the principle that extreme cold damages tissue by freezing, thus creating an inflammation that destroys an area and replaces it with a scar. As early as 1918 experiments with cold using carbon dioxide snow had shown that inflammation could be produced in the eye. In 1933 this led Professors Deutschmann and Bietti in Europe to apply this clinically in the treatment of detached retinas, using the scar as a fastener, to "tack" the retina in place. However, since instrumentation for applying cold was crude at that time, cryotherapy was soon abandoned in favor of diathermy (heat) introduced by Professor Weve in the late 1930's.
Interest in cryogenics, however, continued in various fields, and in this country Fay in 1936 attempted to induce low temperatures in the brain to destroy malignant tumors. With continued research in this field Cooper in 1961 was able to report successful cryogenic neurosurgical procedures. With new technology now available to produce instrumentation using liquid nitrogen, carbon dioxide, or freon as the source of the cold (freeze), its use in the treatment of retinal detachments was reborn and soon became the most prevalent form of treatment of the retina.
One of the advantages of cryotherapy was that the cold probe could be applied to the outer wall of the eye and the freeze delivered through the coats of the eye to the retina without significantly damaging those layers (conjunctiva and sclera). Thus retinal tears could now be treated without cutting surgery which made it similar in that respect to photocoagulation (laser surgery). With the realization that freezing destroys tissue, various investigators soon tried this technique on various tumors of the eye with some success in retinoblastoma. However, treatment of von Hippel angiomas was reported to be unsuccessful in cases reported in 1967 and 1968, which led Dr. Shea to state in 1967 that cryotherapy "cannot be used to sclerose vascular lesions such as angiomatosis retinae."1 However, in 1966 I had begun to treat angiomas with cryosurgery using a repetitive freeze-thaw cycle (two or three cycles), based on the observations of Cahan that repetition of the cycles of freezing and thawing is one of the criteria for producing cryonecrosis of tissues.2 The cases treated were those where the angiomas were anterior (in the far periphery of the retina) and they were treated transconjunctivally.3 The angiomas responded favorably, shrinking and being replaced by scar tissue.
Thus I added cryotherapy to the use of laser and diathermy in the treatment of angiomas. The procedure is performed with local anesthesia and the post-operative course is similar to that of laser. I use it predominantly on large anteriorly placed lesions that I feel will not respond well to laser or as a subsequent treatment to an angioma that has not responded well to laser. It is planned to treat all cases on several occasions since overtreatment should be avoided to reduce complications.
Whether it be cryo or laser therapy, I always anticipate several treatment sessions. Over the years I have found that this conservative approach has considerably lessened complications from treatment.
1. sclerose as a verb means to cause them to harden; angiomatosis retinae is the Latin name for von Hippel-Lindau in the eye, literally a disease of vascular tumors of the retina. 2. cryonecrosis, causing the cells to die by freezing them. Just as a closed bottle of liquid will burst when frozen, the cell bursts and dies when frozen. 3.
transconjunctivally, the probe directs the cold through the conjunctiva to the retinal lesion, without damaging the conjunctiva. See illustration. Excerpted from "Von Hippel-Lindau Disease: The Recognition and Treatment of Early Angiomatosis Retinae and the Use of Cryosurgery as an Adjunct to Therapy," Transactions of the American Ophthalmological Society, 1970 68:367-424.
After the Procedure
Follow carefully the instructions of your physician. Even though you don't feel sick, you have had a surgical procedure, and you have a wound in the back of your eye. It is important not to cause this wound to bleed or enlarge, but to allow it to heal naturally. Pamper yourself for 3-7 days to avoid bleeding. Bleeding in the eye can take months to clear, so an investment of these few days is very important.
- Basically, don't do anything that will cause additional pressure in your eye.
- Don't take aspirin. Tell your ophthalmologist about all your prescription medications.
- Keep your head above your heart, or
- Keep your fanny below your head (squat, don't bend over)
- Take your blood pressure medicine (if any) as prescribed
- No heavy lifting
- No jumping or jarring movements
- Take a mini-vacation from your exercise routine (no jogging, push-ups, tennis, etc.)
Learning from VHL
by Mary P., Utah
I suppose my story is no different from the rest. I, too, am a member
of the “doctor of the month club” and am affectionately
referred to as "Slash" by my husband and family in honor
of my many VHL scars. I, too, have cared for loved ones who have undergone
excruciating and horrendous procedures, and then stood by and watched
as they passed on alone to the world beyond. I, too, look into the
faces of my children and wonder.
When I was first diagnosed with VHL, I saw it as a thief robbing
me of my future. You, too, would understand. In desperation and fear
I fought hard. Every scan and checkup that came back without change
was my victory, and we celebrated. For a time, the battle consumed
much of my energy and thought.
I'm not sure when it happened or how, but one day when my strength
was nearly spent, I stopped fighting long enough to view my adversary.
My foe presented itself and I recognized it as a part of me and a
part of who I am. Together now we visit the doctor and have our scans,
and I ask VHL, "What is it you are going to teach me about myself
this time?" It has taught me much. You, too, would understand.
I cannot help but believe there will be much VHL will teach us about
all in our human family. I join with you in this great pursuit.
We Thank our VHL Family
-- Don and Peggy M., Mississippi
Peggy and I send our most heartfelt thanks to our VHL Family. Your
prayers, concerns, and encouragement and special support have made
the weeks since Peggy's brain surgery very special for both of us.
We have received messages, cards, and calls from all over the world.
We want to give a special "Thank You" to Joyce Graff's family.
They called and visited every day and brought love and encouragement
to both of us. We receive love and support from our family all the
time, and especially during our VHL episodes, but the additional support
of our "VHL Family" is very special to us both.
Peggy is on the road to full recovery and expects to be back in action
by the end of March. She personally wants to thank members of the
Mid-South Chapter for their efforts in raising funds for the Research
Fund. They were able to raise more than $5000 of the Challenge Grant
through raffles, letter writing, mailings and one on one contacts.
We look forward to seeing you at future meetings and would like to
meet everyone in Hawaii in June.
We're Going to . . .
The VHL Symposium
Hawaii, June 17-21, 1996
For hotel reservations, call the
Pacific Beach Hotel, (800) 367-6060 or (808) 922-1233
For flight arrangements, call your local travel agent or call in
Pennsylvania: Jacki Hunsberger at 610-489-0896 or 800-829-0022, Fax:
215-361-8008.
in Hawaii: Rod & Vanessa Belen, +1 (808) 623-3633
Families: Learn the latest advances in managing your health
Doctors: Share ideas with other doctors and researchers to
advance diagnosis and treatment for people with VHL.
To Register:
See pages 19-20 of this issue.
advertisement
Commissions for VHL
-- from Pat Stepper, Virginia
Real Estate Brokers earn commissions not just from sales, but also
from listings, rentals, and referrals. Pat Stepper, who is a member
of the Alliance, is also a Real Estate Broker. Since she is affiliated
with a national company, Coldwell Banker, she can earn money from
referrals all over the country. She has pledged to donate one-half
of the commission from any referral, sale or listing where the customer
mentions the Alliance. If you, or anyone you know, is planning on
buying or selling a house, anywhere in the United States, call Pat
at (703) 212-800 or (800) 825-7794. You can fax information to (703)
212-0730. She will be glad to refer you to someone from a Coldwell
Banker affiliate in your area. Make sure to tell her that you are
contacting her because of your association with the Alliance so the
VHLFA will get half the referral fees!
VHL and Pregnancy
Before and After Pictures Needed
The National Institutes of Health is investigating the effects of
pregnancy on tumor growth in VHL women. They are looking for a number
of volunteers who have imaging studies from before and after their
pregnancies who would be willing to participate in their study, or
women with good pre-pregnancy studies who are currently pregnant and
would be willing to come to NIH for post-pregnancy studies.
If you are interested, please contact Dr. McClellan Walther, Senior
Investigator, +1 (301) 402-2251.
Handbook in French !
VHL France is pleased to announce the publication of the first edition
of the VHL Handbook in French! To obtain copies, write to VHL Family
Alliance, 2001 Beacon St, Suite 208, Boston, MA 02135-7787 USA or download from the
website.
Congratulations to Mireille and Michel Proux and the team
of physicians and doctors who translated and revised this text. [Update
2005: The Third Edition of the Handbook is now available
in French. Thanks to Paul Bonneau of Canada for translating the English
into French. It is available
for viewing on the internet, or download.]
There are many interesting and rewarding jobs to be done to make the VHL Family Alliance succeed and grow. We are looking for committee members for the committees listed on page 18, chapter chairs and willing workers in states and regions, internet surfers, and new Board members. Please call or write and offer your time and talent. We are growing and meeting new people every day . . .
We Need Your Help!
Question: I have a blood clotting problem for which my doctor has prescribed Coumadin. Should I be concerned about the effects of Coumadin on tumor growth in VHL? -- Clark C., Washington
Answer: Coumadin (warfarin) is a "blood thinner" that predisposes those taking it to bleed. It is used in people who have artificial heart valves, thrombophlebitis, pulmonary embolus, myocardial infarction, atrial fibrillation, etc. Coumadin interacts with many other drugs and has several side effects. The primary side effect of Coumadin is the predisposition to bleed. Coumadin should be used when its benefit outweighs its risk of side effects.
VHL tumors are generally highly vascular (hemangioblastoma, angiomas, renal carcinoma) but they usually do not spontaneously bleed. The predisposition to bleed caused by Coumadin could be a problem with VHL tumors, but it is hard to know exactly how much. As usual, one would have to carefully weigh risks versus benefits, and act accordingly. I personally do not want to use Coumadin in a VHL patient unless there was a very good reason to do so, that is, there were unequivocal benefits. -- Col. J. M. Lamiell, M.D., Chief, Clinical Investigation Regulatory Office, U.S. Army Medical Education Department, Fort Sam Houston, Texas.
by Altheada Johnson, New York
SupportWorks is a service which allows eight people to "meet" via a conference call. The VHL Family Alliance has arranged five meetings to take place over the weeks and months ahead. VHLFA members in three states -- Idaho, Michigan and Iowa -- will be able to meet via teleconference for one hour on the following dates. We will also have a support group meeting for the visually impaired, and one on the highlights on the Hawaii meeting.
If you are interested in joining any of these meetings, please call the hotline number at 800-767-4845 to reserve a space and get the number to call on the date of the meeting. It's first come, first served, since the system can only connect 8 people.
Saturday - Idaho Chapter with Peggy L. Rudd
April 20 at 3:00 pm Mountain Time
Saturday - Vision Support Group with Betty
May 18 Beebe at 3:00 PM Central Time
Sunday - Michigan Chapter with Fran Mott
May 19 at 3:00 PM Central Time
Saturday - Iowa Chapter with Diane Mansheim,
June 8 R.N., at 3:00 PM Central Time
Sunday - Highlights of the Hawaii Meeting
June 30 with Altheada Johnson at 3:00 PM
Eastern Time.
Assistance is available for telephone charges.
by Richard Harbud, Denmark
Like the VHL Family Alliance in the USA, the Danish Association was started in 1993, and for pretty much the same reasons. Originally the association comprised solely of the family members of our chair, Vibeke Harbud, herself a VHL patient, and one doctor. We too felt there was a lack of coordinated information on VHL, and that if we wanted more information on the subject then we had better find it ourselves. Today we have a membership of eight families and contact with some thirty doctors.
It has been our experience that the need for clear information, for patients and doctors, is of paramount importance. General practitioners and hospital doctors have not always been aware of the necessity of regular and systematic check-ups, and it follows that patients have also been unaware of the benefits. Fortunately, today we have the cooperation of hospitals, who are kind enough to inform VHL patients of our Association, and who in turn can inform their doctors.
However, establishing a contact with VHL patients is not easy. It is estimated that there are perhaps 30 to 50 families in Denmark with VHL. Few of these will have been correctly diagnosed, and fewer still are known to our Association. Despite the fact that Denmark is a rather small country, with some five million people, our Association could still be regarded as regional. Currently most members live in and around the city of Aarhus, as it was there the association was started, and there many VHL patients have their check-ups. Fortunately, Dr. Eigil Kjeldsen of Odense University Hospital (eikjeld@biobase.dk) who specializes in human genetics, hopes to instigate a nation-wide study of VHL patients. Thus a more complete picture of the extent of VHL in Denmark could be available within the next few years. Hopefully Dr. Kjeldsen will be attending the VHL conference in Hawaii in 1996.
As I stated before, our Association is very small. My wife and I take care of the few administrative tasks there are; Vibeke (pronounced vee-b-ka, in case you are curious) keeps in touch with doctors and I take care of letter writing, forwarding of brochures and so on. Generally speaking very little happens. Occasionally, however, the phone rings and a complete stranger introduces themselves as a VHL patient. More often than not we discover that the person on the other end of the line has just been told that they have VHL. Just as frequently we learn that they could have been helped at an earlier stage, had their families not tried to keep the disease a secret from their relatives and general practitioners. It is no easy thing, talking to a complete stranger on the phone about a potentially dangerous disease, and despite the fact that we immediately refer them back to their own doctor and offer to supply them with copies of all the material we have on VHL, we find that we end up administering first-aid. The chain of events is almost always the same: the patient, following treatment for previously undiagnosed symptoms, is informed by the hospital that they have VHL, at which point in time the patient is ill-equipped to deal with the problem and therefore understands little of what is being said to them. The patient's family concedes that there is "something in the family;" the doctor does his or her best to explain things. The patient, by this time thoroughly confused, finds the brochure given to them by the hospital, phones us, and the battle against ignorance commences.
It has not been easy, starting and running an active association for VHL patients in Denmark, and it has been inspiring to establish contact with the VHL Family Alliance. Both my wife and I were astounded by the speed and quality of response following our first contact, and to describe the VHL Family Alliance as "dynamic" might be bordering on understatement. We know for sure that since making contact we have helped to increase the range of inter-professional resources available to doctors in Denmark, which in turn will give long term benefits to patients. We would like to take the opportunity to thank the VHL Family Alliance and wish all those involved all the best for the future.
Foreningen af Von Hippel-Lindau Patienter, Vibeke Harbud, Fiskervejen 10, 4000 Roskilde, Denmark. Tlf: +45 46 76 70 33
Rare Should Not Mean Alone, a 35-minute video about individuals who have Treacher Collins Syndrome and their families, the physicians who provide treatment, the researchers conducting studies across the country, and the Treacher Collins Foundation. This closed caption video addresses issues faced by families at various times during the life cycle of an affected individual and is a comprehensive educational tool for families and professionals.
While this is not directly about VHL, Treacher Collins is a related disorder (another of the phakomatoses), and the information about living with a rare disease is quite pertinent. Local chapters may find it useful. To purchase a copy, contact the Treacher Collins Foundation, P.O. Box 683, Norwich, VT 05055. Tel: +1 (802) 649-3050
The VHLFA office in Massachusetts has one copy which may be borrowed for chapter meetings.
The National Association of Hospital Hospitality Houses, Inc., is an association of residences for outpatients and family accompanying patients being treated for serious illnesses away from home. Contact Shirley LaBerteaux, NAHHH, 4013 W. Jackson Street, Muncie, IN 47302, (317) 288-3226 or (800) 542-9730; fax: (317) 287-0321.
The National Parent-to-Parent Network of Mothers United for Moral Support, Inc., (MUMS), connects families with similar conditions, esp. children with special needs. Free newsletter. Julie Gordon, MUMS, 150 Custer Court, Green Bay, WI 54301-1243, Tel: 414-336-5333; Fax: 414-339-0995.
When your mate can no longer be your social partner, parenting partner, financial partner, emotional partner, or sexual partner, you are a Well Spouse. Support meetings for husbands, wives, and partners of the chronically ill and/or disabled, U.S. and Canada. Contact Well Spouse Foundation, http://www.wellspouse.org, or send to wellspouse@aol.com (updated 4/2000)
Long Distance Love is a national non-profit support network that links individuals with similar health problems for telephone support and correspondence. Contact Roxanne Black, P.O. Box 114, New Brunswick, NJ 08903, +1 (908) 418-1811 or http://www.njin.net/~ldl/
Cancer Care Counseling Line is a toll-free telephone counseling service of Cancer Care, a non-sectarian social service agency founded in 1944 to help cancer patients, their families and friends cope with the impact of cancer. (800) 813-HOPE.
VHL Family Forum On-line ©1995 by the VHL Family
Alliance. All rights reserved. Reproduction for publication requires
written permission in advance. We will be glad to supply additional
copies for health professionals to distribute to their patients.
VHL Family Forum, Newsletter of the VHL Family Alliance
Editor: Joyce Wilcox Graff, 1-617-277-5667 (eve)
Adviser: Debra L. Collins, M.S., U. Kansas Med. Center, 1-913-588-6043
|
|
|
