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Newsletter of the national gaucher foundation; summer, 2000

Gaucher Disease Newsletter
Summer, 2000
A Publication of the National Gaucher Foundation
Founded by Rubin Bakin
Dear Doctor
Q: I am a 35 year old woman who was diagnosed 10 years ago with Gaucher Disease. I have received
ERT for 5 years and I feel great. I am going to Israel for 3 months. Can I stop my infusions while I
am in Israel?

A: There are various theories about this. I can tell you that there is a definite “reservoir” effect
that takes place. During the three months, if you do not take enzyme, there will be a slow build up
of glucocerebroside, but if you begin right after returning, it is likely that you will not suffer any
negative effects. It is also possible to continue your therapy in Israel. Contact Dr. Ari Zimran,
Director of the Shaare Zedek Gaucher Clinic at 972-2-655-5111 to discuss treatment options with
him.

Q: My son is 18 months old and has Type 1 Gaucher Disease. He will start treatment soon. I am
curious about the effect Gaucher has (if any) on the immune system. This is the second time he has
been on Augmentin and it takes at least a week or two before he gets back to normal after finishing the
medication. He has terrible diarrhea each time. I know that this is a side effect of the antibiotic, but it
seems abnormal for it to continue to affect him so long after finishing the Augmentin.

A: It is unlikely that your son’s infections are related to Gaucher Disease. He is probably allergic
to something he is eating. Swollen tissues from allergic reactions frequently become “infected” due
to lack of healthy drainage. The diarrhea is related to the amoxcillin. Give him ¼ teaspoon of
powdered acidophilus in water twice daily for two weeks to replace the normal intestinal bacteria.
This should eliminate the diarrhea. Please feel free to email me his normal week’s diet and I will

Lenny’s Legal Lines – Q & A
By Lenny Van Pelt, Founder and Director of the International Patient Advocacy Association
Q: I have been reading a lot about genetic privacy. As an individual with Gaucher, is informed
consent a state requirement?

A: Medical information is presumed confidential, but increasing capabilities to store and rapidly
transfer data escalate the challenge of protecting privacy. Laws in many states restrict access to
medical records. At issue is whether genetic information should be protected generally, as another
component of individual medical records, or by specific genetic privacy laws. Laws in 13 states
require individual informed consent to perform a genetic test or obtain genetic information.
Nineteen states require consent to disclose genetic information. Colorado, Florida, Georgia,
Louisiana, and Oregon explicitly define genetic information as personal property. Pending federal
action may set minimum standards or preempt state laws on medical record privacy.

Q: I read an article you wrote regarding senior advocacy. The research about prescription usage and
errors was shocking. Does this apply to Gaucher seniors?

A: No…Advocacy for seniors with Gaucher has always been strong due to the support of the
National Gaucher Foundation, Genzyme’s Reimbursement Dept., and the IPAA. Due to patient
meetings, newsletters, etc., a senior is more informed and can avoid prescription errors you read
about. For our readers, the research found 25% of all hospital and nursing home admissions result
from seniors who do not take their medications properly. By the end of this year, there will be 35
million U.S. seniors who will take more than half of all prescriptions dispensed.

How to reach the IPAA:
Office: 425-402-4037; Facsimile: 425-462-9532
Letters to the Editor
Dear Friends,
Every year, at Holiday time, members of the extended Cleverdon family create a
small family fund. We do this to honor the spirit of the season as well as the
memory of David W. Cleverdon. We all called him Grampa and remember his quiet
passion, his abiding sense of justice, compassion and fair play. We each contribute a
modest amount to the fund while one of us chooses a cause or organization to receive
its proceeds. This choice reflects a specific concern of the chooser as well as the
general interests of the entire family.
This year, David Booth chose the National Gaucher Foundation. Joshua Booth,

Grampa's first great grandchild and David's son, has Gaucher Disease. We all pray
that during Joshua's lifetime a cure will be found for this disease. I cannot tell you
how grateful we are that the National Gaucher Foundation exists -to promote
research to discover treatments and a cure as well as to help patients and their
families deal with the disease on a day to day basis.
On behalf of the Cleverdon family, I am glad to be able to send you this contribution

to further your good work.
Peace and Godspeed,

D. Cleverdon

Dear NGF:
I have enclosed a $35 check to cover my membership fee. I was a member many
years ago under the name of Michelle Jones. I have moved and married since that
time. I am on Cerezyme and I am living an active life. What your Foundation does is
absolutely wonderful. I don’t think I would have had such a wonderful life without
the work of your Foundation.
Thank you,
Michelle

Dear NGF:
I’d like to thank you for assisting me in my project. I received the materials on
Monday and I’m very happy with both their quality and their visual appeal. You were
a tremendous help to me. I only wish that there were more kind and helpful people
like you out there. Thank you again.
Sincerely,
The Gendisease-J Listserv Discussion
By Samuel Shponka
Making full use of the technology revolution, those of us in the Gaucher community can now
rapidly share information and feel less isolated. We have available to us a free, automated, e-mail
program known as a listserv. E-mail sent to the listserv by a subscriber (someone on the listserv’s
mailing list) is automatically sent to every other subscriber on that listserv, and delivered as
ordinary e-mail. Thousands of listservs covering a wide range of topics exist, some with only a few
subscribers and some with well over ten thousand subscribers.

In 1994, Wayne Rosenfield started a listserv for people who have Gaucher Disease or one of the
other rare inherited metabolic diseases disproportionately affecting people of Jewish descent.
Gendisease-J, as the new discussion was called, now has well over two hundred subscribers
throughout the world. It includes people with Gaucher Disease, family members, doctors, nurses,
researchers and others interested in Gaucher Disease or other inherited metabolic disorders. It’s
accessible twenty-four hours a day and it’s independent in that neither Genzyme nor NGF controls
its contents. In fact, the participants themselves determine the content, with occasional input from
the Gendisease-J listserv discussion.
On this discussion list:
We get important Gaucher news faster than any other way. In 1997, when black specks
were found in my shipment of Cerezyme®, I notified Genzyme and then posted the report to
the listserv. This was the only place where we could have shared information so rapidly
about the specks, Genzyme’s response, and what we needed to do.

We learned it was important to prevent the enzyme from foaming while preparing it for
infusion because foaming could cause it to degrade.
We found out about plans for the 1998 Gaucher conference in Memphis and the 1999
Gaucher conference in Arlington, long before we heard about it from any other source. If
not for this listserv, it would have been almost impossible for the Gaucher Conference
Committee, an independent organization not connected with either Genzyme or NGF, to put
on these conferences.

We read about Gaucher treatments under development by companies other than Genzyme.
We can get advice and support from people living with Gaucher Disease and can offer
others our support.
We have read about people who, thanks to what they learned here, avoided unnecessary
surgery urged on them by doctors who had limited experience with Gaucher Disease.
We have been able to share and compare experiences with other members of our worldwide
community on a daily basis, without regard for the miles that separate us.
Many top doctors and researchers in Gaucher Disease are on this listserv. Their expertise is
confirmed by years of experience treating hundreds of people who have Gaucher Disease and by
their extensive research and numerous publications on Gaucher Disease. By sharing in our
day-to-day struggles and triumphs, they become even more attuned to our needs, and become even
better providers.

The privacy of individual participants is respected here. Most of the over two hundred subscribers
to the listserv just read the postings and never post anything themselves. Unless you post a message
to the listserv, no one except Wayne will know you subscribe. You don’t have to worry about
computer viruses either, because messages long enough to carry viruses as well as all types of
attachments are rejected by the listserver.

We are fortunate to have a resource like the Gendisease-J listserv, and the connection it provides
to us as a worldwide community. This listserv is a blessing to the Gaucher community and to those
involved with the other genetic diseases represented there.

EARLY RESEARCH ON ORAL THERAPY
HOLDS PROMISE
By Deborah Elstein and Ari Zimran, M.D., Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem,
Gaucher disease, the most common glycolipid lysosomal storage disorder, is especially prevalent
among Ashkenazi Jews, and hence, of importance in Israel. It has become a model for other

inherited disorders particularly since effective enzyme replacement therapy has been introduced.
Indeed, the enzymatic treatment has revolutionized the management of symptomatic patients; it
leads to reduction of massive organomegaly, improvement in hypersplenism, and often
amelioration of bone pain. However, the administration of the enzyme poses a significant hardship
to the patient as it involves intravenous infusions, usually once every 2 weeks, for life. In addition,
the high cost of enzyme treatment (between $100,000 to $400,000 per year of treatment for a 70 Kg
adult patient, depending on the dosage regimen) limits the number of patients that can avail
themselves of this treatment worldwide. Too, the current formulation of enzyme replacement is
incapable of crossing the blood brain barrier, thereby limiting its value to patients with
neurological manifestations. It is in this context, that a group of scientists and physicians from 4
European centers [including Zimran and Elstein from Shaare-Zedek Medical Center in Jerusalem]
have recently published the results of a new clinical trial using a novel oral substrate inhibitor (the
imino sugar N-butyl-deoxynojirimycin; OGT-918), which inhibits glucosyltransferase, the first step
in the biosynthesis of glycolipids. This biological rationale, the successful outcome in animal
models, and the safety data from an earlier clinical trial involving 130 patients with HIV, have
suggested OGT-918 as a suitable candidate for oral treatment of glycolipid storage disorders.
The authors report the results of a one-year open-label trial, wherein 28 adult patients with
symptomatic Gaucher disease were enrolled (7 patients were post-splenectomy). All patients were
started on 100mg OGT-918, taken orally 3 times a day. The safety profile of the drug was good,
with the only significant side effects being diarrhea and abdominal discomfort, which occurred in
two thirds of the patients, but disappeared within the first few weeks of treatment. Two patients
withdrew from the trial because of these side effects; 4 other patients withdrew at various points
during the trial for reasons unrelated to adverse effects. The report mentions 2 of 21 patients who
continued beyond 12 months, who developed a peripheral neuropathy, which was reversible with
stopping the drug. The treatment resulted in a dramatic reduction in spleen and liver volumes,
19% and 12% respectively, which is comparable to that seen in patients on enzyme replacement
therapy. Blood counts, including hemoglobin and platelets, as well as chitotriosidase levels, also
improved during the course of the trial, albeit at a less satisfactory rate.
Based on the safety and efficacy demonstrated in this study and after further clinical research,
OGT-918 may become a viable treatment for symptomatic patients without severe hypersplenism
or for those who have developed side effects with enzyme. In addition, since OGT-918 as a small
molecule crosses the blood-brain barrier, it is a candidate drug for the neuronopathic forms of
Gaucher disease and may be the harbinger of an effective treatment in other lysosomal storage
disorders as well. Finally, the article implies that OGT-918 is the first generation of similar-acting
compounds already being developed.
MED.ED.NOTE: Further studies are in progress to investigate the peripheral neuropathy issue, to
explore varying dosages, and to develop protocols utilizing Cerezyme and OGT-918 together for
improved therapeutic gains and decreased costs.

YOUR ACHING BACK . HOW TO
CHOOSE THE PROPER EXERCISE
PROGRAM FOR YOU
(Second article of a three part series)
by Suzanne Krupskas
Please refer to the Spring 2000 Newsletter for the first set of exercises. Before you begin any
exercise program consult your physician first.

Your exercise routine should be painfree. Find your painfree position either in a sitting or standing
position. Are you more comfortable in an arched position (called lordosis) or are you more
comfortable bent slightly flattening the lower back (called kyphosis)? Arching your back is
considered extension bias and flattening your low back is considered flexion bias.

You always want to exercise within your painfree range. So find your most comfortable bias
position and follow these guidelines. First, warm your back with a heating pad (preferably moist).
The temperature should be gentle warmth - not too hot! Positioning is important so go by these
guidelines:

A) Flexion bias warmup - (AN ALTERNATIVE POSITION TO LYING ON YOUR BACK . see
previous newsletter) Lie on your painfree side with 1-2 pillows under your head and 1-2 pillows
between your legs. Place the heating pad on your back . rest comfortably for 15-20 minutes.

B) Extension bias warmup - Lie on your stomach with a pillow under your stomach/pelvis area and
a pillow under your shins. Place the heating pad on your lower back.rest comfortably for 15-20
minutes.

After the warmup, perform the following exercises that are appropriate for your bias. You may
want to exercise on the floor or the bed. If you choose the floor, make certain you are capable of
getting on & off the floor without assistance and without any pain or strain.
Throughout your exercise routine proper breathing is so important. Always exhale through your
mouth during the exerting part of the exercise and inhale through your nose during the relaxation.

The following flexion and extension bias exercises will ultimately strengthen the trunk stabilizers
(stomach and back muscles).

FLEXION BIAS EXERCISES:
1) Pelvic Tilt: (this exercise is repetitive from the first series, but so important) Lie on your back
with yours knees bent. Tighten your stomach as you squeeze the buttocks, flattening your lower
back into the bed or floor. Hold position for 3 seconds - 10-20 repetitions.

2) Alternate Arm Raise: (use 1-3 lb. arm weights, the weight should feel comfortable - Not Too
Heavy) Holding the weights in each hand, perform a pelvic tilt first (see above exercise). Maintain
the tilt, keep the elbows straight and palms down. Raise 1 arm up towards the ceiling (90 degrees
from starting position), then lower and relax the tilt. Alternate arms and always perform the tilt
first. 10-20 repetitions for each arm

3) Both Arm Raise: (use 1-3 1b. arm weights) Hold the weights in each hand. Keep your elbows
straight and palms down. Perform the tilt and maintain it as you raise both arms towards the
ceiling (90 degrees from the starting position), then lower the arms. Relax the pelvis and repeat
10-20 repetitions.

4) Touch Opposite Knee: (use 1-3 1b. weights for arms & legs) Same position as above with your
arms by your sides. Perform a pelvic tilt and maintain the tilt. Slowly bring your right knee
towards the chest as your left hand (with the weight) touches the knee. Bring the same arm up
towards the ceiling simultaneously as you straighten the leg (parallel to the floor). Do not thrust the
leg as the movement is very gentle. Perform 10 times on both sides.

EXTENSION BIAS EXERCISES
1) Prop on Elbows: Lie on your stomach, place your elbows under your shoulders so that you are
leaning on your forearms. In this position relax your low back muscles and breath naturally.
Remain in this position for 3-4 minutes.

2) Press-Ups: (this exercise is repetitive from the First Series, but so important) Lie on your
stomach with your palms down, with your elbows bent 90 degrees. Arms are shoulder width apart.
Straighten your elbows as you press your palms into the bed or floor. Sink your pelvis into the bed
or floor and hold position for 3 seconds then lower yourself as your face goes towards the bed/floor.
Rest and perform 10-20 repetitions.

3) Buttock Squeeze: (use1-3 lb. ankle weights) Lie on your stomach, resting your head in your
arms. Legs are together. Squeeze your buttocks together, as you bend your knees approximately 20
degrees off the bed/floor. Hold the position for 3 seconds then relax. Perform 10-20 repetitions.

4) Extension in Standing: Stand upright with your feet apart, knees are straight, place your hands
in the small of your back. Bend your trunk backward at the waist as far as you can using your
hands as a fulcrum. Hold position for 3 seconds. Perform 10-20 repetitions.

This entire exercise regimen (first and second series) should be performed 4-5 times per week. Do
not give up on these exercises as they are advantageous in preparing your body for daily activities.
The third series will discuss ways to interlude both flexion and extension exercises into your
exercise program.
Good Luck! Here's to a healthier back!

(This article is written by Suzanne L. Krupskas, a full-time Physical Therapist and Gaucher
spokesperson. Any questions or comments, please contact Suzanne directly at:

Scoop on the Groups
Chicago Chapter
Please join the Chapter for their Second Annual Gaucher Disease Divot Classic on Thursday, July
20, 2000. The outing will be held at Midlane Golf Course in Wadsworth, Illinois. Various
sponsorships are available. If you know of an individual, business or corporation who might be
interested in participating, please call Cynthia Yannias at (847) 581-1855.

Delaware Valley Chapter
The Fifth Annual Golf Fore Gaucher Disease Classic is scheduled for Friday, September 22, 2000
at Philmont Country Club in Philmont, PA. Save the date and plan to join us for big prizes, great
food and drinks, and a fun-filled day of golf on one of the best courses in the area. Please call
Larry Kaliner at (610) 649-6766 to reserve your spot!

Washington, DC Area
Our national office is busy preparing for the First Union Golf Classic on Monday, August 7, 2000,
at the Tournament Players Club at Avenel in Potomac, Maryland. We are very excited to have the
support of such sponsors as First Union, Capital One, TheraCom, and Nova Factor. The golf
invitations were mailed the beginning of June and registrations are rolling in! We hope to have a
sold-out golf tournament again this year. If you would like to participate as a golfer, sponsor or
volunteer, please call Sharon Adams at (800) 925-8885. We would greatly appreciate your
support!

Tune In….
For readers in the NY City area, we would like to ask for your help. In an effort to educate the
local community about Gaucher Disease, an NGF member has asked the NY television station,
WNET, to run a segment on the disease. It appears that they determine programming based on
the number of requests per month they receive on particular topics. We would like to ask our
readers in the New York area to write a letter to the station requesting a segment on Gaucher
Disease. We feel that if enough requests are submitted that there is a good chance the station will
include an informational piece on Gaucher Disease. The contact information for WNET is,
through the headquarters, at: NOVA-WGHB, 125 Western Avenue, Boston, MA 02134. We
appreciate your help!

Special Appreciation
The NGF would like to send a special Thank You to Claudia and Suzi Goodman for the wonderful
arts and crafts fund-raiser they held this Spring. We realize that they donated their time and
effort making the beautiful craft items that were sold. All the proceeds were given to the NGF for
research and education on Gaucher Disease. We truly appreciate your help, Claudia and Suzi!

Click on Dr. Gaucher to return to the Gaucher Disease Homepage.
2000 by The National Gaucher Foundation
Last Revised: August 12, 2000

Source: http://www.gaucherdisease.org/newsletters/ngfnewsnew2000c.pdf

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