VHL ALLIANCE VHLA StrAtegic PLAn creAted for 2018-2020
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NEWSLETTER OF THE
VHL ALLIANCE
VHLA Strategic Plan Created for
2018-2020
By Karen Ramsey, VHLA Board of their committees to work on each include: launching an innovative
Directors, Immediate Past-Chair initiative and report progress patient natural history study to
every month. The plan is a living, capture information about VHL
D ream…think…brainstorm…
create…modify…calculate…
negotiate… adjust…solidify. In
breathing document and subject
to change as some ideas get
provided directly by the patient;
awarding four grants to fund
initial research focused on finding
a nutshell, that describes the a cure for VHL and improving
strategic planning process diagnosis; additional services
that the VHL Alliance Board of Please consider joining like monthly patient and parent
Directors and Staff engaged calls and a coaching program for
in over the last 90 days. It is a
the growing number
Clinical Care Centers.
dynamic process that takes a of volunteers and
The 2018-2020 strategic plan
great deal of time, energy, and stakeholders helping supports the VHLA’s mission
effort to complete. to bring VHLA’s new of “research, education, and
This is the third time the VHLA strategic plan to life. The support to improve awareness,
Board and Staff have undertaken commitment of people diagnosis, treatment, and quality
this difficult and important of life for those affected by VHL.”
just like you makes sure
task. With the results so fruitful, Each component, or “strategy,” is
everyone enthusiastically a cure for VHL is found!
outlined. Just as in the past plans,
participated in developing the Go to vhl.org/volunteer there are LOTS of moving parts.
2018-2020 plan. to volunteer or make a As board member Camron King
The truth is many organzations donation. mentioned in the recent Annual
undertake strategic planning Report, ALL of us need to roll up
because they “should.” Those our sleeves and contribute our
plans generally collect dust, only time, treasure, and talent to make
to be looked at when it’s time to this plan as successful as the last!
put together a new plan. That is scrapped and new opportunities
NOT the case with VHLA. The present themselves. The 2018-2020 Strategic
plan truly serves as a road map Just a few of the amazing
for the Board and Staff to follow.
Plan can be found at:
initiatives undertaken and
Board members take individual completed during the most vhl.org/strategic-plan.
responsibility to partner with recent strategic plan (2016-2018)
CONTENT
VHLA Strategic Plan Created Dealing with VHL as a Patient Upcoming Clincal Trial
for 2018-2020 and a Parent MyVHL
Are You Ready for a CCC Corner Happenings
Blame-ectomy? 2017 VHLA Annual Meeting Save the Dates
Summary
Winter 2018
LIVING WITH VHL
aRE YOU rEADY FOR A
bLAME-ECTOMY?
By Gary L. Wood, PsyD, VHL Patient, Parent, and will take time to understand your concerns. You
Grandparent collaborate with your therapist and set goals
together. You’ll learn coping skills you can use for
I ’m a psychologist and a VHL patient in my early
70s. When I found out that I had passed along
von Hippel-Lindau to my daughter, and later, my
the rest of your life. This is very empowering.”
Two helpful techniques
When you are experiencing guilt, it’s helpful to
grandson, I had to learn how to perform a “blame-
put those thoughts into words, Dr. McBride says.
ectomy” on myself.
“Then, like a scientist, you can
This term does not appear in
test how true the statement is.
psychology literature. I use it to
For example, you may have the
describe the process of removing
blame from our thinking. When {{ thought ‘I did a terrible thing
by passing this disease on to
we have – or care for – someone
I spent years blaming myself my child.” Write it down. Ask
with a chronic disease, it’s human
yourself, ‘Is this something I did
to want to cast blame on ourselves and thinking some of those intentionally?” You’ll likely answer
or others, with thoughts like “I
thoughts [of blame]. It took ‘no’. Ask ‘is there any benefit to
should have done this…” and “I
having VHL?’ Your first response
ought to have known…”. These
me [that] I was in my late 50s may be ‘no,’ but maybe you can
thoughts can cause us to be angry
see that living with the disease
or anxious much of the time. until I was able to reduce my allows greater personal growth
I spent years blaming myself
feelings of guilt. and empathy for others with
and thinking some of those
chronic diseases.
thoughts. It took me until I was
Another technique is turning
in my late 50s that I was able to
reduce my feelings of guilt. Even
|| attention away from yourself
and toward others who are going
with my years of advanced study
through something similar. “There
in psychology, I knew I couldn’t
is no downside to helping other
do it alone. A process called cognitive behavioral
people,” Dr. McBride says. “In fact, it’s one of the
therapy (CBT) helped me shift my negative thinking
things that has been consistently shown to have a
to a more realistic mindset. The process has been
good effect on our mood.”
physically and emotionally freeing. It is also helping
me be a better father and grandfather. Self-help or professional help?
Two excellent self-help books based on cognitive
Changing your perspective
behavioral principles are “Mind Over Mood,” by
Professionals may use a combination of
Dennis Greenberger and Christine A. Padesky; and
approaches including CBT to help individuals see
“Feeling Good” by David D. Burns.
a troubling diagnosis or chronic illness in a more
With a willingness to make changes, you can
rational way.
reduce thoughts of self-blame. If you find you are
Licensed clinical psychologist Angela M. McBride,
not making progress on your own – especially if
PhD explains it this way: “Cognitive behavioral
the intensity of your negative emotions about VHL
therapy tends to be short-term and focused. You’re
interferes with your daily life – that’s the time to
not signing up for ten years on the couch. Rather
seek professional help.
than telling you what’s wrong with you, the therapist
MyVHL: Help find the Cure (vhl.org/MyVHL)
2 | Winter 2018 | VHL ALLIANCEdealing with VHL as a patient
and A Parent
By Bobby K, VHL Patient and Parent up slowly with that glazed look of a college student
who has had one too many. His request: can we get
T
2
oday I took flex time from my job as a dental some food in the cafeteria? He knows a good thing.
school professor to meet my wife for lunch. As a patient and a parent dealing with VHL,
It’s not a date I really wanted to have, but it’s a I’ve learned to roll with the ups and downs while
necessary part of being a VHL parent. I drove being diligent and positive. It’s a big job. Our son
from Midwestern University to Phoenix Children’s has known of his diagnosis since he was 6. I found
Hospital where our seventeen-year-old son has out at 44. As a patient, I cope by only dwelling on
been laying in an MRI tube for the last 90 minutes this disease when I have to: the routine exams
under general anesthesia. There was time to and follow up appointments, waiting anxiously
grab a bite to eat before he was finished. We for the results of the last scan, scheduling surgery
walked past the Christmas trees in the hospital when necessary and convenient. I’ve had two
lobby, all decorated spinal surgeries,
beautifully but past renal cryoablation
their welcome, as that did not work
volunteers were busy and a subsequent
VHLA Programs for Parents of VHLers
packing the ornaments nephrectomy on my
in boxes. The cafeteria • Telephone Discussion Groups left kidney, as well
here has particularly as multiple retinal
good Mexican food. • Facebook Group for parents of children with
hemagioblastoma
(I’ve even rated it on laser ablations. But
VHL: facebook.com/groups/parents2parents
TripAdvisor, their today I feel pretty
huevos rancheros • Better Together Peer Mentoring good! We’re watching
rock!) I shouldn’t know lesions on my brain,
that fact but I do. I Visit vhl.org/support or contact the office at
right kidney, and
know it because we lumbar spine. My
info@vhl.org or 617.277.5667 x4.
are regulars here at right hand and foot
PCH. Scans, follow up are experiencing
appointments with the paresthesia
oncology team and our (numbness) which
neurosurgeon from makes trail runing
Barrows Neurological a challenge. Otherwise you would never guess I
Institute. They provide excellent care and we are have anything going on. Fortunately, I have a great
blessed to have fantastic health insurance from career at a fantastic institution, supportive family
my employer, which they accept. But I would much and friends. I just move forward because that’s
rather be somewhere else, like the beach or even how I deal with this. I would give my life if my child
work. This is the life we have with VHL. I spent would not have to live with this. But he does. And
Tuesday evening in the MRI tube myself. Oh, what so does my wife of 30 years this August, bless her
fun it is to get the IV, be strapped onto the board heart! Being the parent of a teenager with VHL is
inside a tube and to feel the contrast dye move unique. I want him to look forward to his adult life
through your body. The myriad clanks and clicks but he has begun to question the role VHL will play
interrupted by breathing instructions: take a deep in it. He wonders why he should work so hard in
breath in, exhale, hold your breath, over and over. his AP classes and extracurricular activities if VHL
At least Gunnar is unconscious for his 2-hour ordeal could take it all away. I do my best to reassure him
today. This time I went for the fajitas…very tasty! I’ll that time and medical advances are in his corner,
add them to my reviews later. Susie and I returned but it can be a difficult debate to win. I try to be
to the radiology waiting area to be notified when the best role model that I can be by my actions
Gunnar was in the recovery room. We sat next to and words. We have reached out to counselors for
him and watched his vitals on the screen. He woke help and have dealt with teen depression. We’ve
continued on next page
VHL ALLIANCE | Winter 2018 | A
3dealing with VHL as a patient and a parent
continued from page 3
adjusted medications, schedules, and attitudes. It here at PCH and Barrows. I have placed my trust in
can be a challenging time for any parent, let alone them before and again today. Thank goodness for
VHL parents. At a time when Gunnar should be the advances in medicine we have now and may
getting excited about college tours and eventual gain in the future. There are support groups for VHL
matriculation, he is not. A basket full of letters from patients and parents, which I encourage you to join.
interested schools sits unopened. He is a bright kid I have hope. You can have hope. I have a positive
with top 10% PSAT scores and a bright rewarding outlook. I hope that you can too. And sometimes I
future in any field he chooses. The lack of interest realize that my fingers are often crossed, wishing for
and excitement stems from his worries about a cure, just a little too often.
VHL. It’s a tough sell. Today’s MRI is just another The nurse just told us that Gunnar is free to leave
appointment in his VHL calendar. We meet with his the recovery area. We are off to the cafeteria once
team next week to see if the lesion on his brain stem again. This time it’s a family function! Bon apetit!
has enlarged. Our fingers will be crossed until then, Writing this article today while we endure
as growth would move him closer to a second round another follow up appointment has helped me cope
of brain stem surgery (his first was 5 years ago). once again. I hope that you can find a personal way
There is no feeling more helpless than watching to cope as well! Peace.
your child being rolled out on a gurney, his life in
the hands of a neurosurgeon. We have a great team
CCC CORNER
T he VHL Alliance is pleased to announce the University of Colorado – Denver as the newest VHL Clinical
Care Center. The VHL team at UC-Denver is led by Dr. Nicholas Cost. Dr. Cost is an experienced
urologist, with a specialty in urologic oncology and pediatric urology. He dedicates a large portion of his time
to adolescents, young adults, and adults with hereditary kidney cancer predisposition syndromes.
The VHL team at UC-Denver has the latest technology and an experienced staff, comprised of a wide
range of specialists, who meet regularly and are well-versed in treating patients with VHL. They are
experienced, excited and well-equipped to address any and all needs of the VHL community in the Colorado
area.
If you are interested in pursuing care at the University of Colorado – Denver, you can reach out to the
team’s Point of Contact, Heather Thompson, by phone at: 720.848.8570 or by email at:
heather.thompson@uchealth.org.
VHLA NEEDS YOUR FEEDBACK GIVE BACK TO THE VHL COMMUNITY
D o you or your family member with VHL
receive care at a VHL Clinical Care Center?
If so, we want to hear from you! We invite you
T ake on a leadership role. The VHL Alliance
is currently looking for VHL patients and
caregivers to help us work with each Clinical Care
to submit any feedback that you have about Center as Patient Liaisons. This volunteer role
your experience at: vhl.org/cccfeedback. You are requires a minimal time commitment, but can have
welcome to remain anonymous. Your feedback is a significant impact on shaping the relationship
vital to helping us improve the quality of care and between provider and patient. If you are interested
experience for all. in learning more about the CCC Patient Liaison
program, please email Josh at:
josh.mann@vhl.org or call: 617.277.5667 x4.
4 | Winter 2018 | VHL ALLIANCEVHLA UPDATE 2017 VHLA ANNUAL
MEETING SUMMARY
T he 2017 VHL Alliance Annual Meeting was held
in Tampa, Florida on Saturday, October 14. The
agenda included 10 scientific presentations, patient
head of the pancreas
• The tumor doubling time is less than 500 days
• There is a mutation in exon 3 of the VHL gene
and caregiver break-out sessions focusing on the In the case of locally advanced, or metastatic
emotional aspects of VHL, and a panel discussion disease, surgical management is indicated, if
with patients. Below we highlight two presentations feasible and if greater than 90% of the entire tumor
about VHL in the adrenal glands and retina. can be removed.
For presentation slides, video recordings, and
The full summary can be found at: vhl.org/about/
written summaries of the presentations from the
resources/annual-meeting-presentations.
annual meeting visit: vhl.org/about/resources/annual-
meeting-presentations. Endocrinological Advances in the
Management of VHL
Liver and Pancreatic Manifestations of VHL John Tourtelot, MD, a specialist in the
Mokenge Malafa, MD, a specialist in the endocrinology program at Moffitt Cancer Center,
Department of Genitourinary discussed the endocrine
Oncology at Moffitt Cancer manifestations of VHL and
Center, discussed the liver and current updates. VHL is an
pancreatic manifestations of autosomal dominant disorder
VHL. Seventy-seven percent of
In the case of localized [VHL
caused by mutations in the
VHL patients have pancreatic pNET] disease, surgical VHL gene and is characterized
lesions. Most have simple managment is indicated when: by the occurrence of multiple
cysts, while 9% have serous endocrine and non-endocrine
cystadenomas (SCA) and 9%
• pNETs are greater than or
lesions.
have pNETs. Survival rates equal to 3cm in the body or tail A pheo is a tumor arising
and quality of life in patients of the pancreas from specific cells in the
with VHL can be improved adrenal glands that commonly
by better understanding of
• pNETs are greater than or
produces one or more specific
the biology of VHL-associated equal to 2 cm in the head of types of hormones. These
pancreatic and liver tumors. the pancreas tumors are rarely biochemically
This can be accomplished silent, producing hormones
through improved diagnostics
• The tumor doubling time is less
known as catecholamines
and surveillance, as well as than 500 days (dopamine, norepinephrine,
individualized treatments. The • There is a mutation in exon 3 and epinephrine). A para is
management of pancreatic of the VHL gene another tumor derived from
cystic lesions in VHL consists specific cells in the thorax,
of observation, until the abdomen, or pelvis. Evidence
lesions become symptomatic. has demonstrated that
For VHL-related pNETs, hereditary pheos and paras
surveillance is indicated because 40% of patients are characterized by a distinct clinical presentation
with pNETs will have no tumor growth or decrease and differences in biological behavior. About 80
in tumor size over 4 years. Surveillance includes to 85% of these tumors are pheos. Paras are rarer
annual CT and MRI scans. In addition, less than and represent about 15 to 20% of these tumors.
20% of these tumors are malignant. In the case of Paras can also arise along certain nerves in the neck
localized disease, surgical management is indicated and at the base of the skull. These tumors do not
when: produce hormones.
• pNETs are greater than or equal to 3cm in the It is important to note that not all pheos
body or tail of the pancreas and paras are alike. Recognizing the distinct
• pNETs are greater than or equal to 2cm in the
continued on next page
VHL ALLIANCE | Winter 2018 | 5AANNUAL MEETING SUMMARY
continued from page 5
presentations of hereditary pheos and paras, it is Studies show that adrenergic receptor blockers
recommended to take a personalized approach should be started at least 7 days prior to surgery to
to patient management. This normalize blood pressure. Taking
includes biochemical testing, phenoxybenzamine, an alpha
imaging, surgery, and follow-up. blocker, intravenously for 5 hours
Initial biochemical testing for per day for 3 days before surgery,
All VHL patients should
pheos and paras should include has been reported as one effective
blood and 24-hour urine tests. be tested for a pheo approach. Studies have also found
There are different types of VHL that initiation of a high-sodium diet a
mutations that can increase the prior to surgery few days after the start of adrenergic
chances of having a pheo. receptor blockers can prevent low
Dr. Tourtelot described the blood pressure upon standing from
importance of preparing for any a seated position, before surgery.
surgery. All VHL patients should The full summary can be found at: vhl.org/about/
be tested for pheos prior to surgery. Patients with resources/annual-meeting-presentations.
a hormonally functional pheo or para should take
medication to prevent cardiovascular complications.
CLINICAL TRIAL RECRUITMENT
Multiple sites to open throughout the US. First site to open in
March 2018.
PT2977-202: An open-label phase 2 study to evaluate PT2977 for the treatment
of von-Hippel Lindau disease-associated renal cell carcinoma
More information can be found at:
VHLA website: vhl.org/clinical-trials or clinicaltrials.gov (Study Number
NCT03401788)
VHL Alliance: josh.mann@vhl.org, 617.277.5667 x4
Study sponsor: Peloton Therapeutics, Inc.
BOARD OF DIRECTORS STAFF
Officers: Directors: Ilene Sussman, PhD, Executive Director
Manuel Greco ilene.sussman@vhl.org
Steven Angotti, JD Seth Horwitz
Chairman of the Board Emily Billcheck Heidi A. Leone, Director of Advancement
Camron King
Doug Karle heidi.leone@vhl.org
Gordon Cooke Bettina Micheli
Vice-Chairman of the Board Joshua Mann, MPH, Director of Engagement
Barbara Correll Mark Pallansch & Outreach
Andrea Rafael, Treasurer
Jennifer Galenkamp Karen Ramsey josh.mann@vhl.org
Anna Waller, Secretary
Eric Jonasch, MD Soniya Sapre Diamond Tarwoe, Office Manager
diamond.tarwoe@vhl.org
6 | Winter 2018 | VHL ALLIANCEMyVHL
The VHL Patient Natural History Study
Do you want to help find a cure?
DISCOVER how your unique journey can
help others
Help find a CURE for cancer through VHL
Be a part of progress in RESEARCH
PARTICIPATE NOW!!!
Register for MyVHL today at vhl.org/MyVHL! Learn more about your VHL and
contribute to finding a cure. And, when you complete the survey and upload your
scans (or submit a records release form), a $50 donation will be made to VHLA.
HAPPENINGS
MAY AWARENESS EVENTS AND FUNDRAISERS:
Team VHL Yard Sale Fundraiser LuLaRoe Sale benefiting Michigan Chapter VHL
Saturday, February 3rd, Thousand the VHL Alliance Thursday, Meeting, Saturday, May
Oaks, CA May 3rd 12th, Clinton Township, MI
Lunch Event, Honoring Dr. Jonasch, VHL Pancake Breakfast, Painting with a Purpose,
March 23rd or 24th, Houston, TX Saturday, May 5th, Sterling Tuesday, May 22nd,
Mom’s Day Run Heights, MI Rochester, MI
Sunday, May 13th, Ventura Beach, CA Comedy Festival, May TBD,
Mom’s Day Event Durango, CO
Sunday, May 13th, Lynn, MA
What are you doing in May to promote VHL Awareness?
Need ideas or help? Please contact Heidi at heidi.leone@vhl.org.
VHL ALLIANCE | Winter 2018 | 7ANewsletter of the VHL Alliance
1208 VFW Parkway Suite 303
Boston, MA 02132
ADDRESS SERVICE REQUESTED
SAVE THE DATEs!
Young Adult Retreat (VHL.ORG/YAR)
JUNE 8–10, 2018 — bOSTON, mA
vhl mEDICAL/rESEARCH sYMPOSIUM
oCTOBER 4–6, 2018 — hOUSTON, tx
Please note NEW dATE & Location
VHLA ANNuAL FAmILY WEEKENd
oCTOBER 19–21, 2018 — Denver, Co
8 | Winter 2018 | VHL ALLIANCEYou can also read
