PHACE Syndrome: the Families, the Physicians, and the Facts

PHACE Syndrome: the Families, the Physicians, and the Facts

 

September 16^th & 17^th, 2010

Milwaukee, WI

 

This conference is sponsored by: Children’s Hospital of Wisconsin, Medical College of Wisconsin, the National Organization of Vascular Anomalies, and HIG (Hemangioma Investigator Group)

 

Schedule for the Family Conference

• Thursday, September 16, 2010
  • 12:30 Registration/Event Check-In
  • 1:00-5:00 Family Conference
  • Following the conference: Family dinner and social event
• Friday, September 17, 2010
  • 8:30 Continental breakfast
  • 9:30-3:30 Family Conference (includes luncheon)

 

Event Highlights

2 day Educational Conference:

• Overview and update on PHACE syndrome
• Radiology imaging in PHACE syndrome
• Neurological issues that can be a challenge to individuals with PHACE
• Treatment and management guidelines
• Social implications of facial differences
• Laser therapy for hemangiomas
• Research update

Social Event Thursday Evening with dinner

(Reception, Dinner and Entertainment tentatively set at Betty Brinn Children’s Museum)

 

Registration

• Cost: Free for PHACE families and includes everything listed in this section
• How to register: To register electronically E-mail: jlehman@mcw.edu and request a registration form or call Jennefer at 414-955-2818 and you can register over the phone. Registration deadline is August 13^th, 2010.
• All educational sessions on Thursday & Friday
• Written educational materials about PHACE syndrome
• Childcare for children 2-8 years old during educational sessions
• Reception, dinner and entertainment on Thursday evening
• Light breakfast on Friday morning from 8:30-9:30
• Lunch on Friday
• There is no charge to the families for anything listed in this section

 

Why Attend?

There are many reasons to attend the PHACE conference. Here are just a few of them:

• Attendees will leave with a wealth of knowledge about PHACE syndrome
• Opportunities to meet other families facing similar challenges
• Exchange ideas, support and advice

 

Who Should Attend?

This conference is designed for families of children with PHACE syndrome or anyone that is interested in the care of children with PHACE syndrome from newly diagnosed to the latest research available.

 

Speakers & Panelists

Denise Adams, MD - University of Cincinnati College of Medicine, Cincinnati Children’s Hospital

Beth Drolet, MD – Medical College of Wisconsin, Children’s Hospital of Wisconsin

Jen Duncan, Director/Founder at PHACE Syndrome Community

Ilona Frieden, MD - University of California, San Francisco

Maria Garzon, MD – Columbia University, Morgan Stanley Hospital

Karla Hall – Executive Director NOVA

Christopher Hess, MD, PhD – University of California, San Francisco

Jennifer Hoag, PhD - Medical College of Wisconsin, Children’s Hospital of Wisconsin

Jensen, John, MD - Medical College of Wisconsin, Children’s Hospital of Wisconsin

Craig Johnson, DO – Medical College of Wisconsin, Children’s Hospital of Wisconsin

Patricia Marik, Psy D - Medical College of Wisconsin, Children’s Hospital of Wisconsin

Denise Metry, MD - Baylor College of Medicine, Texas Children’s Hospital

Orbach, Darren, MD, PhD – Harvard Medical School, Children’s Hospital Boston

Dawn Siegel, MD – Medical College of Wisconsin, Children’s Hospital of Wisconsin

Other Speakers TBD

 

Conference location

The conference takes place at the Children’s Health Education Center located at

1533 N. RiverCenter Dr.

Milwaukee, WI  53212

(414)765-9355 phone

Map available at this website

http://www.chw.org/display/PPF/DocID/20530/router.asp

 

Where to Stay?

Comfort Inn Downtown Lakeshore: Rooms $79 plus tax. Has a shuttle to/from Children’s Health Education Center for families. No pool. There is a block of rooms set aside. Address:  916 East State Street, Milwaukee, WI, US, 53202 Phone: (414) 276-8800. Mention PHACE conference for discount room rate. Deadline for this rate is August 15, 2010.

There are many other hotels in the area also.

 

We have also secured discounted passes for the Paradise Landing Indoor Waterpark for $10 each, at the nearby Hilton Milwaukee City Center downtown. The passes are available for use that Friday from 3-10pm and Saturday from 9am-10pm. Just mention that you are with the PHACE/Children’s Hospital Group when you purchase the passes at the water park.

 

 

 

PHACE Meeting

Approx dates and times:
Thur, Sept 16 (1PM - 4:30PM). Family Social Event and Dinner in the evening.
Fri, Sept 17 (9AM - 4PM) All day sessions - continental breakfast and lunch provided.
…
The location is going to be at Children’s Health Education Center in downtown Milwaukee. http://www.chw.org/display/PPF/DocID/20530/router.asp

The topics and speakers are still being finalized. Once they have been confirmed we will be able to publish the schedule. Looks like we are going to have a great group of Physicians on hand as many will be attending the scientific sessions Wed and Thurs morning.

This will be a KNOW NOVA event.

Vascular Birthmark Center of NC

The Vascular Birthmarks Foundation (VBF), the National Organization of Vascular Anomalies (NOVA), the former MSN Vascular Birthmarks Support Group, and Birthmarks.com, are excited to announce the launch of the newest vascular birthmark and anomalies treatment center in Greensboro, North Carolina on July 19^th, 2010.

 

The Vascular Birthmark Center of North Carolina is led by Dr. Gregory Levitin, an internationally recognized expert in the diagnosis and treatment of all types of vascular birthmarks and malformations, including hemangiomas, port-wine stains, venous and lymphatic malformations, and arteriovenous malformations.  Dr. Levitin accepts most major insurances and is accepting appointments for new and returning patients for consultation beginning July 19^th.  You can schedule an appointment either by emailing Dr. Levitin at drlevitin@gmail.com or visiting www.carolinabirthmark.com. 

 

Contact information for this new clinic will also appear on the websites of the Vascular Birthmarks Foundation at www.birthmark.org, NOVA at www.novanews.org and at www.birthmarks.com.  

Cost of treatment

Each year thousands of families search for medical care to treat a vascular anomaly.  Some have to travel out of state for care, this creates complications with health care insurance.  The few fortunate ones are able to get  authorization from the insurance companies for out of network care at in-network benefits because of an exclusion policy that allows for out of network care when in-network care is not available for a specific treatment.  However, most are not so lucky.  They may get some out of network benefit but in the end most families have to search of other options to pay for care.  Many of the NOVA family of physicians offer reduced fees of service for families that do not have insurance or when the insurance is not paying the in-network benefits.  The generosity of our physicians is above and beyond what any one expects.   I can not even count all the patients NOVA has referred that have been treated at various Vascular Anomaly Centers with minimum or no fee charged by the doctor.    While most of the doctors will work out payment plans and clearly outline fees for services some offices give verbal indication that there will be no out of pocket cost to the family when in reality this is not the case.  NOVA is advising ALL patients and their families to ask for a written estimate of charges for care from the doctor in advance of any treatment and for an explanation of the payment plan that is available.  NOVA is also advising ALL patietns and families to read the fine print of contracts of service with the doctor, the hospital and any associates of either.  While doctors may waive or reduce fees hospitals are not typically included in this agreement!

Here at NOVA we want to protect our families from any hardship that may occur when unexpected medical costs arise.  We have had recent concerns voiced to us about billing practices and unexpected charges by some offices. Upfront communication and clear expectations by patients and physicians can prevent financial heartaches later.

Please feel free to share your stories and experiences with us here.

CLOVES Syndrome

Up until a month ago I had not heard of CLOVES Syndrome. In doing my monthly literature search I found a reference to a 2007 publication by Sapp and associates to a newly defined syndrome. This little known syndrome was identified in 7 patients in this article. Many of these patients were previously thought to have a variant of Proteus or other overgrowth disorders- but CLOVES is different.

Facebook allowed NOVA to connect with CLOVES families and now it is our joy to welcome these individuals to the NOVA Family!

A brief description of CLOVES with references to the journal articles can be found under INFORMATION.
Karla

ISSVA-2010

The 18^th meeting of the general assembly of the International Society for the Study of Vascular Anomalies (ISSVA) was held in Brussels April 20-24, 2010.  The meeting was nearly canceled due to the eruption of a volcano that literally shut down  air travel throughout Europe/Asia for nearly a week.  Fortunately the dedicated and hardworking members of the ISSVA conference team utilized modern day technology to pull together the first Trans-Atlantic Web-Conference for  Vascular Anomalies.  Live-stream web broadcasting was coordinated with Montreal and Brussels with internet viewing available for those that could not travel to either location.  Dr. Mikka Vikkula, the entire Brussels team and  Dr. Josie DeBois, of Montreal must be commended for the success of this important scientific meeting that at least appeared to be planned for web broadcasting!

 

Nearly 100 presentations from the spectrum of vascular anomalies diseases were made by centers across the globe.  These presentations outline the dedication by clinicians and scientists to better understand and manage the complex spectrum of vascular anomalies. 

 

What started as an idea to advance vascular anomalies, by Dr. John Mulliken nearly 20 years ago has grown into ISSVA today!  Everyone affected by vascular anomalies owes these dedicated researchers our gratitude.

 

Karla Hall

Executive Director NOVA

Migraines, Malformations and More

Migraines, malformations and more….

 

 

There is a lot of new information coming out about Migraine headaches.  Much of this information has to do with the mechanism of migraine and the occurrence of migraine.  There is still not clear answers to these questions but the research is ongoing.  We are hoping that some of the information about migraines will help us better understand the migraines that children with PHACE often experience. 

 

 

Summary of information:

 

Migraines

 

• Migraine headaches have a female predominance with a peak occurring in the 30’s and 40’s.  Migraines can be divided into two major groups: migraine with aura and migraine without aura.  Migraine without aura is the most common type of migraine headache.  In both groups the headacahes are unilateral, moderate in intensisty, often pulsate, aggravate with routine physical activity, associated with nausea, photophobia and or phono-phobia.  Each migraine lasts from 4-72 hours.  Migraine with aura occurs in 37% of the women with reported migraine.  This type of migraine is associated by the presence of a neurological symptom that develops over 5-20 minutes and lasts for less then 60 minutes.  The aura precedes the headache in most cases.

 

• The most widely accepted mechanism for the development of migraine headache with aura is excitability of the neuronoal cells membranes of the occipital cortex of the brain.  The excitation spreads through the cortex leading to activation of the trigeminal nerve pathway causing pain. 

 

• In the absence of aura the mechanism of migraine pain is debated.  The main area of dispute is whether the migraine pain originates from the activation of the trigeminal nerve pathway due to a release in vasoactive chemicals from blood vessels or does this originate in the center of the trigeminal pathway in the brainstem.  Regardless of the origination pathway of the migraine the underlying cause appeases to be due to dilation of the large cranial vessels beneath the surface of the brain, (dura mater).  These vessels are interwoven in the trigeminal pathway.  Research has identified several vasoactive peptides in the trigeminal nerve cells including calcitonin gene related peptide, (CGRP).  This peptide seems to be the most important identified peptide in the development of a migraine headache.

 

 

• Pregnancy has a variable effect on both the intensity and the frequency of migraine without aura headaches.  A literature search shows that most women seem to have either some improvement or no change in migraine during pregnancy.  No data can be found establishing and objective set of criteria that will predict which patients will see improved symptoms of migraine and which patients will see no improvement.  Women with migraine with aura headaches are less likely to see improvement of symptoms during pregnancy. 

 

• Ovulation also seems to have an effect on migraine occurrence.  In one study reported in the literature women with history of migraine, undergoing induced ovulation had a higher incidence of headaches then those without history of migraine.  During ovulation levels of estrogen fluctuate.  Similarly in the perimenopausla state estrogen levels fluctuate.  The observation that migraine attacks increase during the 30’s – 40’s supports the suggestion that fluctuations and abrupt changes in estrogen levels triggers migraine headaches.

 

 

• Migraines headaches do not seem to directly affect the outcome of pregnancy.  There are no outcome studies that report an increase in the preterm labor, low birth weights or congenital anomalies.  There is a noted indirect effect.  Patients with migraine headaches have a higher risk of developing pre-eclampsia then those without migraine.  Migraines that develop during pregnancy or delivery must be evaluated and considered for other complications associated with headache such as pre-ecalmapsia and post-epidural headache.

 

 

• At the 14^th International Headache Congress it was reported that abdominal migraine represents %15 of gastrointestinal pain in the pediatric population.  If you track children with gastrointestinal migraine a significant number of these children will go on to have migraine headaches later in life.  Abdominal migraine is characterized by episodes of moderate to severe midline, periumbilical, or poorly localized pain lasting for 1 to 72 hours with nausea, anorexia, pallor, and occasionally headache. “But headache is not one of the cardinal features. It’s episodes of belly pain,” reports Dr. Donald Lewis Professor of Pediatrics Eastern Virginia Medical School.  In a retrospective study it was found that there is a large subset of children who have had a vague diagnosis of GI pain who most likely have abdominal migraine.  Making the correct diagnosis says Dr. Lewis, “ means the spectrum of migraine medications will become available to children.” 

 

Rare Vascular Tumors

 

• Verrucous hemangioma is an uncommon, localized vascular malformation associated with reactive epidermal acanthosis, papillomatosis and hyperkeratosis on histological examination.^[1] The lesions are bluish-red, well demarcated, soft, and compressible. In time, verrucous hemangiomas gradually enlarge and satellite nodules may arise. 

 

• It as a congenital vascular malformation comprising a capillary or cavernous hemangioma in the dermis and subcutaneous tissue associated with reactive epidermal acanthosis, papillomatosis, and hyperkeratosis, distinguishing it from angiokeratoma.^[4]

 

• Most verrucous hemangiomas are located on the lower extremities and involvement is generally unilateral. Lesions have usually been present since birth or have occurred in early childhood, although they may appear later or even in adult life.

 

• Verrucous hemangiomas do not resolve spontaneously and have a tendency to relapse. Early diagnosis is important to get a better cosmetic result. Verrucous hemangioma requires a large, deep excision. Incomplete excision leads to recurrence because of deeper components. Cryosurgery, electrocautery, and laser therapy can be used for smaller lesions.  It must be confirmed by histopathological examination.

 

 

Treatment:

 

• A recent published report in Pediatrics supports the opinion that Propranolol is a safe and effective treatment for severe infantile hemangioma.  Use of propranolol can markedly shorten the disease course according to the small study published in the September 2009 issue of Pediatrics. Authors of the study note that within a few hours of treatment with the beta-blocker Propranolol the color of the hemangioma lightens and the growth is reduced.  The authors conclude that if the results are confirmed by a large comparative study, propranolol could be come the first line treatment for infantile hemangioma.

NOVA provides this information as a resource.  It is not intended to engage in the practice of medicine or to replace the physician.  NOVA does not claim to have medical knowlege.  NOVA does not endorse any particular physician, treating facility or treatment protocol.  In all cases NOVA, the NOVA Board of Directors and associates recommends that you seek the opinion of a physician experienced in the diagnosis and treatment of hemangioma and vascular malformations.

 

 

 

 

 

photo page

We are adding photos of vascular anomalies to the web site.  They can be found on the drop down menu of the Information Page or http://www.novanews.org/information/photos-of-vascular-anomalies.

National Geographic Show

If you missed the National Geographic Channel’s special on Rare Anatomy: Blood,  Featuring  Friends of NOVA Son Pham and Michale Politzer you can view it now online at:  http://channel.nationalgeographic.com/series/extraordinary-humans/4214/Overview#tab-Videos/07899_00

Additional Broadcast dates have not been announced.  We are hoping for updates soon.

ISSVA- 2010 Brussels

18th International Worskhop on Vascular Anomalies

21-24 April 2010, Brussels, BELGIUM

Visit the ISSVA website for more information: www.issva.org