The Sudden Arrhythmia Death Syndromes Foundation


SADS HEART to HEARTH



Summer Newsletter
Heart Disease on the QT
1996




"Heart to Hearth" is a newsletter
mailed three times yearly designed to bring
you information on the programs, events
and philanthropic activities of the SADS Foundation
as well as Medical Updates on the Long QT Syndrome
and other related cardiac disorders.





INSIDE




The SADS Foundation
540 Arapeen Drive, Suite 207
Salt Lake City, Utah 84108
Toll free: 800-786-7723
Fax: 801-582-1941






PRESIDENT / MEDICAL DIRECTOR'S REPORT
G. Michael Vincent, M.D.

We are delighted to announce the appointment of Linda Shockley, M.D. to the SADSFoundation Board of Trustees. Dr. Shockley brings to the Foundation a career in Pediatrics, aswell as considerable insights into the issues faced by families with the long QT syndrome andsudden death. She will be a wonderful addition to the SADS Foundation, and we express ourwarmest welcome to her. Please see the highlighted section on Dr. Shockley for moreinformation.

We have been getting a number of questions about gene therapy for long QT syndrome. This very exciting concept is just in its infancy, and gene therapy is still somewhat of ahypothetical concept. A number of clinical trials are underway in other disease processes, withsome short term but promising results. It is probable that sometime in the future gene therapymay be a treatment for the long QT syndrome, and particularly advantageous because it couldbe essentially a cure for the individual receiving the therapy. However, much work remains tobe done both in the principals and application of gene therapy and in further elucidating thegenetics of the long QT syndrome. Thus, I wouldn't look for gene therapy of the long QTsyndrome in the very near future, but perhaps within several years to a decade.

We are excited about the SADS home page on the Internet and it is generating quite a bit ofactivity already, even in its infancy. The Internet will give us enormous opportunities tocommunicate with individuals and groups throughout the world. This is certainly an excitingtime for the SADS Foundation, and with our increasing opportunities to be of help to so manypeople. Thanks to all of you for your contributions and the great work that you perform.



SADS ON THE WEB

The SADS Foundation has hopped onto the information highway and we are now on theInternet! You can visit our page at http://www.sads.orgfor up-to-date information about the Long QT Syndrome, SADS Foundation activities andpublications, including our brochure and this newsletter. You may send us E-mail at<sads@mail.aros.net>. :)



NEW BOARD OF TRUSTEES MEMBER

LINDA SHOCKLEY, M.D., CHAPEL HILL, NORTH CAROLINA
Linda, Bill and Lindsey Shockley came into contact with the SADS Foundation last year afterLinda read the small article about the long QT syndrome in Good Housekeeping magazine. Linda and Bill's, daughter, Casey, died at age 16 in August of unknown causes. They immediately had Casey's ECG screened, and, indeed, she did have LQTS. Thankfully, Lindsey,Casey's sister, does not have the syndrome.

In addition to her personal experiences, Linda brings a great deal of medical knowledgeto the SADS Board. She graduated from Indiana University Medical School in 1976 inPediatrics and through the course of her career she served in the private and publichealth sectors as well as practicing academic medicine. In 1990, Linda retired to becomea "full-time mom" to Casey and Lindsey- in her words, the best decision she ever made.

Linda is doing a great deal to spread the word about LQTS. She has spoken at DukeUniversity, had a newspaper article published about Casey and LQTS, is helping Casey'sfriends organize a "grass roots" awareness effort by visiting pediatricians' offices,and many, many more activities. Thank you, Linda and Welcome!






AWARENESS


READER'S DIGEST ARTICLE

This month we had the wonderful opportunity to reach 51 MILLION PEOPLE when the long QT syndrome was featured in the June 1996 issueof Reader's Digest. The article will also appear in several versions of the July Reader's Digest in other countries and we will reach many moremillions of people. The article profiles our very own President and Medical Director, Dr. G. Michael Vincent, and his long QT research overthe last 25 years. We have already received over 3,000 telephone calls and four extended families have already been diagnosed with LQTS asa result of this article. We are expecting that hundreds more life-saving diagnoses will occur over the next several months.



JANE, STONE AND OPRAH... HERE WE COME!

The overwhelming response to the recent LQTS articles in Good Housekeeping and Reader's Digest re-emphasizes the effectiveness of nationalmedia to educate families about the long QT syndrome. The SADS office as well as several LQTS families have already contacted many of thesesources and we are asking YOU to join us in our awareness efforts by participating in a "letter writing campaign" to two national programs. Please send your personal letters chronicling your family's experience to the following addresses. Please mention in your letter that you arewriting in conjunction with an effort by the SADS Foundation to get on their program. If you found out about LQTS from a media source (ratherthan your physician or another family member), please describe your experience. As many of you know first hand, public awareness about LQTSsaves lives.

DATELINE NBC
Story Editor, Room 408
30 Rockefeller Plaza
New York, NY 10112

OPRAH WINFREY
Story Editor
P.O. Box 909715
Chicago, IL 60690

THANK YOU. Please send us copies of your letters, and in our next newsletterwe will feature highlights.



CALL YOUR LOCAL PAPER & SPREAD THE WORD ABOUT LQTS

Thousands of people can be reached by stories in local newspapers or on local television news programs. Reporters are often surprisingly opento publishing a story about LQTS. Thank you to the following individuals and families who were instrumental in getting their local newspapersor television stations to do a feature on the long QT syndrome.

Would you like to help get a story on LQTS printed in your local paper? Call the SADS Foundation and we will coach you through contactingthe media as well as send you a media kit and sample articles that you can supply to local reporters.



SADS AWARENESS SUPERSTARS

ELLEN MITCHELL
Miami, Florida

Ellen's daughter, Stacey, died in June of 1995 from the Long QTSyndrome. Soon after Stacey's death, Ellen began fervently raisingawareness about LQTS by helping put together a 5K Run/Walk inmemory of Stacy. She has also written nearly 100 letters to the media,labor unions, health care groups, the military, and even the WhiteHouse (yes, she did get a response from the President)! She speakswith anyone she can to help her educate the world about LQTS. Sheis also starting a Foundation Support Group with the help of Dr. MingL. Young. She has truly made a tremendous difference in more livesthan she will ever know. Our hats go off to you, Ellen, and youramazing attitude and determination. Your victories are proof that oneperson can make a difference.

RITA EUKEN
Cumberland, Iowa

Since the death of her daughter, Melissa, three years ago, Rita hasbeen dedicated in her mission to help stop other families fromexperiencing the tragedy of losing a child to LQTS. She has beenextremely diligent in contacting her local media, the school system,and local physicians about LQTS. She also scans the papers andtelevision news for reports on young sudden deaths and tries to makecontact with the deceased individual's family to give them informationabout LQTS. Like many parents who have lost a child to LQTS, Ritais extremely frustrated by the lack of knowledge and understandingabout the disorder. She is taking her frustration "by the horns" and ismaking a decisive impact on the ignorance that can be so disastrous.


What is an "Awareness Superstar"? Someone who has been extremely effective in taking the LQTS message to all avenues of publicinformation. Our Superstars doggedly pursue the ideal that every person know the signs & symptoms of LQTS.






SADS FOUNDATION SUPPORT GROUPS


NEW FOUNDATION SUPPORT GROUP FORMAT

We find that our Support Groups and families with the long QT syndrome are truly our emissariesto the public and the medical profession. In an effort to streamline the efforts of SADS and thesegroups, we have developed a new format that reflects our goals. The new group format includesa steering committee consisting of a Chairperson, a Secretary/Treasurer, and a Chairperson fromeach of the following four subcommittees: Public Awareness, Medical Provider Education, FamilySupport, and Fundraising. We have had great success with this format at our meetings in Orlando,Washington, D.C. and Seattle and look forward to all of our groups achieving great things!



NEW SADS FOUNDATION SUPPORT GROUPS

In order to save funds yet maximize our efforts in educating families and physicians about LQTS,we schedule Foundation Support Group meetings in cities to which our President, Dr. Vincent,is traveling. The last few months he has attended and spoken at several conferences and as aresult, we were able to visit three wonderful cities. We have met many fantastic families with theLong QT Syndrome and everyone whom we have met along the way has been kind and hospitableand has opened their homes (Thanks Bernadette and Cal Driscoll) and hearts to us. THANKYOU!

ORLANDO: March 24, 1996
Several families from all over Florida gathered at the Children's Hospital in Orlando and metwith Dr. Vincent, Katherine Timothy, Katie Roberts and Dr. Richard Crampton. A specialthanks to Christine and George Badame for making the meeting arrangements and supplyingthe refreshments. From this one meeting, four groups were formed (see telephone numbersbelow):

WASHINGTON, D.C.: April 21, 1996
Families from the Washington D.C. area as well as loyal supporters from the Philadelphiagroup attended this wonderful meeting at the Hyatt Regency in downtown Washington. RayWoosley, M.D., and several of his colleagues from Georgetown University graciouslyanswered family questions with Dr. Vincent. Dr. Woosley is the Chairperson for this groupand he is anticipating several exciting activities in the area. A very big thank you to RalphHardy, a SADS Board Member who lives near Washington, for funding this beneficial event.

SEATTLE: May 18, 1996
SADS welcomed several families from the Seattle area and Canada for a very informativemeeting at the Seattle Westin Hotel. Thank you to everyone for their kind input and toChristine Nieuwenhuis for volunteering to be Chairperson. The group is already working onsome wonderful projects for raising awareness in their lovely city.



OLDIES NIGHT!!

On March 23, 1996, The Philadelphia SADS Foundation Support Group, led by Dorothy and JimRenner, put together a fun-filled evening of information, good food, dancing, prizes and greatfriends. (What more could you want?). Thanks to everyone who so kindly gave time, "chance"items and donations to support the SADS Foundation. What a great night!



PLEASE JOIN US...

"Sudden Cardiac Death and the Long QT Syndrome"
August 17th, 1996
9AM - 11:30 am
8th Floor, Mailman Center, University of Miami
Parking: NW 12 Ave (North to NW 16 St.) parking is $1.50 over the weekend
Speaker: Ming L. Young, M.D.
Please RSVP or for more information call: Ellen Mitchell (305) 271-2826
There is no cost to attend this informative meeting.

The Philadelphia SADS group is planning a picnic get-together in early September. Please call Shirley McDonald (610)358-5131, for more information and specifics.






SADS NEWS & VIEWS


DONORS TO THE SADS FOUNDATION

January 1, 1996 - June 1, 1996

$24 and under

$25 - $49

$50 - $99

$100 - $999

$1000- $4,999

$5,000

$20,000 and up




THANKS TO ALL OF YOU
WHO ARE HELPING US REACH FAMILIES
IN NEED OF INFORMATIONAND SUPPORT.

TOGETHER, we are SAVING LIVES!




SADS HAS A NEW LOGO!

After months of design and deliberation, our new logo is complete and ready to hit the nation! We arevery pleased with it and feel that it nicely symbolizes our activities and goals as a Foundation. We offerour supreme gratitude to Tom Olson, a graphic designer in Salt Lake City who has donated hundredsof hours to produce this work of art. THANKS, TOM, we love it! (And if you think this looks great,you should see it in color!)



...AND A NEW LQTS BROCHURE!

We have updated the long QT syndrome brochure to include up-to-date findings on diagnosticprocedures, treatmnt concerns and genetic findings, as well as a current copy of the Drugs to Avoidlist. SADS produced the brochure with the intention of reprinting it often in an effort to keep it ascurrent as possible in this rapidly changing environment.

Please let us know if you would like copies of this new brochure to send to family members (a goodopportunity to encourage family members who have not yet been screened for LQTS), or if you wouldlike a package of them with a Raising Public Awareness booklet that you can use to educate yourcommunity about LQTS. Call us at (800) 786-7723.



HEALTH INSURANCE BILL

In the last issue of Heart to Hearth, weoffered some tips in acquiring andkeeping health insurance for yourfamily members with the long QTsyndrome.

As you may know, both the UnitedStates House of Representatives and theSenate have now passed legislationreforming health insurance policies,particularly to make health insuranceportable and to eliminate non-coverageof pre-existing conditions. Both billsprevent companies from discriminatingon the basis of pre-existing conditions. The Senate bill is stronger because itprotects both group and individualcoverage, while the House bill onlyhelps those with group coverage. Additionally, both bills contain awaiting period of 12 months afterenrollment for the pre-existingcondition to be covered. Although thenew legislation may not be ideal forthose individuals and families withhealth insurance problems, it will helpa great number of people in their fightfor coverage. We hope this will ensurethat families will not experience "job-lock", when they cannot leave jobsbecause they will lose coverage, whichis a reality for many families withLQTS.

Thanks to The Genome ActionCoalition (TGAC), of which SADS is asponsor and member, both bills wereamended to prohibit discriminationagainst persons on the basis of geneticinformation. The final bill will be thefirst time the United States Congresshas recognized, legislatively, theprotections that are needed as we enterthe age of molecular medicine.

Action on this legislation is possiblein the very near future. We lookforward with hope to a victory forpatients, families and researchers whowill benefit from the final bill.



SADS CYBERCHAT

You are cordially invited to join otherfamilies and interested friends in a"chat room" to talk about issues that arise with having cardiac arrhythmia patients in your family. The sessions are unstructured and open to anyone who has interest in meeting other familieswith similar concerns.

Where: Enter America-Online. Click on Go To. Click on Lobby. Click on Go To Rooms.Click on Private Room. Type in SADS and you should arrive!

When: The third Saturday of every month at 9:00 p.m. EST/6:00 pm PST.

(The next session will be on July 20th.)

DON'T MISS OUT - SIGN ON!




SO YOU SAY YOU HAVE NO TIME TO EDUCATE PEOPLE ABOUT LQTS?

HERE IS WHAT YOU CAN DO WITH 10 MINUTES...
Call your school board and speak to the school nurse administrator. Ask him/herif they will feature an article about LQTS in the School Nurse Newsletter.After they say yes, you can send them our brochure and a pre-written articlefor their letter (supplied by SADS).

WITH 20 MINUTES...
After you drop the kids off or on your way to lunch, stop by aphysician's office (any Pediatrician, Cardiologist, General Practitioner or Neurologist will do) and ask tospeak to the doctor or a nurse and give him/her a bundle of our brochures. Tell him/her that you have a child(or a friend, grandchild, etc.) that is affected by LQTS and that most doctors you have met don't know muchabout it. Smile a lot, be very nice and they will listen and take heed!

WITH ONE DAY...
While you are calling about the School Nurse newsletter, ask the sameperson what you need to do to participate in the school system's athletic health screening. You can then setup a "booth" during the screening (most school systems have all the athletes come on the same morning inJuly or August) where you can ask each athlete if he/she has ever fainted or had a seizure, or if he/she hasany relatives who died at a young age of unknown causes. If the student answers yes, give him/her a formletter (that you and SADS compile together) and our brochure.

IF YOU WANT TO DO ANY OF THESE QUICK LIFE-SAVERS, OR FOR MORE IDEAS,CALL SADS AND WE WILL SUPPLY THE MATERIALS AND INFORMATION.



NEW ADVISORY COMMITTEE MEMBER:
CHRISTINE BADAME

Christine and her husband, George, live in Leesburg, Florida with their two beautiful boys,Conner, age 3, and Carson, 8 months. Conner has the long QT syndrome and Christine wasrecently diagnosed with it as well. Christine has been active with the Foundation for 3 years, andis always full of spectacular ideas. She started SADS CYBERCHAT as well as planning the firstSADS meeting in Florida on May 18th. Thanks, Christine for all of your hard work, dedicationand input. We are expecting lots more!



THANK YOU!!

Three thousand thanks (one for each call we have received) to our Salt Lake City Group for thetremendous help they have given during the Reader's Digest phone call "frenzy". Special thanksto Nancy Brown and Burma Skinner for the hundreds of hours they have each donated. Manyother local families have volunteered to take off voice mail, do data entry and stuff envelopes. Your kindness and generosity with your time helps us get the information out much more quicklyto families still looking for answers. THANK YOU!



MENDING HEARTS

Shirley Mann McDonald, of Aston, PA, began working on thisquilt to commemorate LQTS survival 3 years ago. Her goal isto create a symbol of the growing number of lives that are beinglived to the fullest despite a diagnosis of LQTS. The firstsquare of the quilt is dedicated to our loved ones who have losttheir lives to LQTS. However, all of the other squares ofShirley's quilt represent the living LQTS families withindividual hand prints for each surviving member of the family. Through these interlocking hands, the quilt depicts the manylives being saved through increased awareness, diagnosis andtreatment of LQTS. If you have not already done so, pleasecontact Shirley with your family information and she will createa quiltpatch foryourfamilyandincludeyou inher quiltwith itsspiritandmessageofoptimism andhope forourfuturegenerations.PleasecallShirleyMcDonald at610-358-5131.






SADS BLUE RIBBON AWARDS

Cynthia Voisin CYNTHIA VOISIN
Since Cynthia was diagnosedwith LQTS, she has workeddiligently to educate herextended family who maybe at risk with this disorderand has even located "lost"family members in an effort tosave lives. The family hasdeveloped their own pedigreeand is participating in LQTSmedical studies.

Cynthia has graduated fromcollege and now works from herhome office. She was married in1994 to John Sehl, who takes aspecial interest in LQTS as afamily doctor. Their first childwas born in January/96--she is athriving daughter, Olivia Claire.

Being aware of the syndromeand dealing with it in a positivelight has been emphasized inCynthia's family. After all,Cynthia believes that one'sattitude will determine one'squality of life. Cynthia issupportive to her sisters,extended family members, andothers with LQTS whom she hasmet through the Canadian SADSFoundation. She inspireseveryone she meets with herwarmth, her strength, and herpositive attitude.

Submitted by: Pam Kotsilidis


Karly Hazellief KARLY HAZELLIEF
Karly is a talented, funny 14 yearold who excels in dance, singingand acting. She attends theAmerican Stage Workshopfor one month in the summerto "sharpen" her acting skills. She is doing lots of babysitting tohelp pay for a car when shebegins driving. She has a greatmany passions and interests butbelieves that she would like tofocus her efforts and eventuallybecome a pediatric cardiologist.

Karly's mother died suddenlywhen Karly was an infant, andseveral years later her aunt alsodied under similar circumstances.Shortly after the death of heraunt, another of Karly's aunts,Michelle, was correctlydiagnosed with the Long QTSyndrome.

Despite people close to Karlyhaving died from LQTS andbeing diagnosed with thedisorder herself, Karly is aloving, kind, giving, beautiful-hearted young woman. She is"just like her mother" accordingto her aunt Michelle.

She brings a great deal of joy toher Oma (Grandma) and herPoppop (Grandpa). Her family isextremely thankful for her andloves her very much.

Submitted by Michelle Lundy,Karly's aunt


Kerry Williams Sheets KERRI WILLIAMS SHEETS
When Kerri Williams Sheetsof Jackson, Alabama was 18years old, she wasdiagnosed with a seizuredisorder following a seriousautomobile accident.

Then, in February of 1995, heraunt was diagnosed with theLong QT Syndrome. Kerri waschecked and LQTS showed onher ECG. A pacemaker wasimplanted in March 1995. Thenin April she had another LQTSepisode at work. Her doctorsdecided to do a Left CardiacSympathetic Denervation(LCSD). (See Heart to HearthWinter 1995 for information onLCSD).

Five months after her surgery,on her 24th birthday, Kerri wasmarried to Ben Sheets. She and"hubby" Ben are buying a homeand things are going very well forthem. Kerri loves her job asoffice manager for theBirmingham Bulls Hockey Cluband her attitude remains positiveand upbeat despite an extremelytumultuous and busy year.

Please call SADS if you areinterested in speaking with Kerriabout the LCSD.

Submitted by Edwina Williams, Kerri's mom


Michelle Dillione MICHELLE DILLIONE & WILLHEMINA TAYLOR
"I can still see Michelle's face whenwe met at the SADS Gala in Conshohoken, Pennsylvania, in 1994. With hurt still in her own heart from the loss of her sisters, she offered me support for my own losses. Michelle is a full time wife, mother and corporate lawyer with time left over for support to others. She also found time to chair the SADS Philadelphia Gala in 1994 and helps the Philadelphia area support group for SADS. She is indeed a busy and caring young woman with LQTS who deserves a pat on the back."

"I would also like to nominate Michelle's mother, Willhemina Taylor. She is a full time nurse, chief baby sitter for Michelle's little A.J., and a student at Neumann College, striving to get her Bachelors degree in nursing. She also worked diligently on the Philadelphia Gala and the SADS support group. Having lost her husband and two daughters in a short period of time, she still continues to support others like myself."

"What wonderful and strong people these women are!"

Submitted by: Shirley McDonald


Is a child that you know with the Long QT Syndrome doing something exceptional? Did he win the school spelling bee? Was she victorious in her race for school president? Or is your kid just all-around great? If so, we want to give this super person a BLUE RIBBON! Please send a brief description of what makes the child in your life a winner and a picture to the SADS office. Your champion will receive a blue ribbon package from the SADS Foundation and may well see him/herself in our next newsletter.






SCIENTIFIC ADVISORY BOARD


THE SADS FOUNDATION SCIENTIFIC ADVISORS

Peter J.Schwartz, M.D., Chairman
Milan, Italy

Jeffrey L. Anderson, M.D.
Salt Lake City, Utah

Charles Antzelevitch, Ph.D.
Utica, New York

Richard S. Crampton, M.D.
Charlottesville, Virginia

Arthur Garson, Jr., M.D.
Houston, Texas

Kunitake Hashiba, M.D.
Nagasaki, Japan

Mark T. Keating, M.D.
Salt Lake City, Utah

Thomas S. Klitzner, M.D.
Los Angeles, California

William J. Mandel, M.D.
Los Angeles, California

Arthur J. Moss, M.D.
Rochester, New York

Dan M. Roden, M.D.
Nashville, Tennessee

Jeffrey A. Towbin, M.D.
Houston, Texas

Dan Tzivoni, M.D.
Jerusalem, Israel

Victoria L. Vetter, M.D.
Philadelphia, Pennsylvania

Raymond L. Woosley, M.D., Ph.D.
Washington, D.C.




NEW SCIENTIFIC ADVISOR

Raymond L. Woosley, M.D., Ph.D.
Dr. Woosley is the Chairman of the Department of Pharmacology at Georgetown University. Dr. Woosley has been involved with the SADS Foundation for nearly a year and has helped us tremendously with our Drugs to Avoid List. In the words of a colleague, "studying drug-induced prolongation of the QT interval is his life's work."

We are delighted to welcome Dr. Woosley as a Scientific Advisor to the SADS Foundation.



LONG QT SYNDROME (LQTS):
THERAPEUTIC DECISIONS BASED ON GENE FINDINGS

Jeffrey A. Towbin, M.D.

During the past year, the genes for three of the four known loci (i.e., positions on a chromosome) for LQTS have been discovered by a team of collaborators from the SADS Foundation Scientific board led by Dr. Mark Keating. These genes, KVLQT1 for the LQT1 chromosome 11-linked locus, HERG for the LQT2 chromosome 7-linked locus and SCN5A for the LQT3 chromosome 3-linked locus, all encode different ion channels of the heart. KVLQT1 and HERG encode potassium channel proteins while SCN5A encodes a sodium channel gene. Mutations (or mistakes in these genes) are responsible for the development of clinical LQTS. Whether the specific gene or certain specific mutations in these genes cause more severe or less severe symptoms is not yet known.

Therapy for LQTS has relied on ß-blockers such as propranolol (Inderal) and atenolol (Tenormin). This therapeutic regime successfully treats the vast majority of LQTS patients. However, a small but significant number of patients continue to be symptomatic on this therapy. These patients commonly proceed to other medication, pacemakers, defibrillators, or surgical sympathectomy. Although these therapies clearly succeed in treating most affected individuals, the mechanisms of action are either nonspecific or uncertain.

One of the benefits of understanding the underlying cause of LQTS in individual patients is the potential to develop ion channel-specific therapies which may be used after the gene-specific diagnosis is made in that patient. Dr. Arthur Moss, in a collaborative effort, recently described the potential ability to diagnose the gene-specific type of LQTS by electrocardiographic (ECG) analysis. This, in conjunction with the molecular genetic "blood test" that allows for direct gene mutation analysis, may allow for a more specific diagnosis based on mechanism. Using the information obtained from these diagnostic methods, Dr. Peter Schwartz and collaborators selected the sodium channel blocker Mexiletine to use in patients with defects in the sodium channel gene SCN5A (LQT3), demonstrating shortening of the QT interval on an ECG. Although not enough patients have been studied and not enough long-term information from this type of study is known the hope is that this type of therapy could allow for better long-term caring of patients with LQT3, as well as improving the prognosis for high risk patients that fail conventional (i.e., ß-blocker) therapy. It is certainly possible that many of those patients who fail conventional therapy are those with specific ion-channel defects, such as those with SCN5A mutations. Similar studies of medications that work directly on potassium channels are underway. It is possible that new developments in therapy will rapidly occur based on the new found knowledge gained from understanding the responsible genes. Hence, a brighter horizon may be in view for patients with LQTS, particularly in the areas of diagnosis and therapy.



SADS SCIENTIFIC ADVISORY COMMITTEE MEETING

A SADS Scientific Advisory Committee Dinner Meeting was held at the Westin Hotel in Seattle on May 16th, in conjunction with the NASPE meetings. The group enjoyed a lively round table discussion regarding SADS Foundation activities, physician education and emerging issues and special concerns about LQTS.



ADDITION TO DRUGS TO AVOID LIST

Please make note of the addition of the antihistamine Benadryl (chemical name: Diphenhydramine) to our Drugs to Avoid list. This applies to the oral medication: the topical ointment is safe to use. This finding can serve as a reminder for those with the long QT syndrome to avoid over-the-counter (OTC) medications if possible. Many drugs have not yet been tested for prolongation of the QT interval and may not be safe to take. Always inform any physicians you see as well as your pharmacist about your condition and check our Drugs to Avoid list every Heart to Hearth issue for updates.






DRUGS TO AVOID IF YOU HAVE THE LONG QT SYNDROME

Drugs which prolong the QT interval and would be best avoided if you have the Long QT Syndrome. Discuss with your doctor.


DRUG NAME CHEMICAL NAME GENERAL USE


ANESTHETICS / ASTHMA

Adrenaline

Epinephrine

Local anesthetics (for dental, minor surgery), or as an asthma medication


ANTIHISTAMINES

Seldane

Terfenedine

Allergies

Hismanol

Astemizole

Allergies

Benadryl

Diphenhydramine

Allergies


ANTIBIOTICS

E-Mycin, EES, EryPeds, PCE, etc.

Erythromycin

Infections; lung, ear, throat

Bactrim, Septra

Trimethoprim & Sulfamethoxazole

Infections; urinary, ear, lung

Pentam intravenous

Pentamidine

Lung infections


HEART MEDICATIONS

Heart rhythm drugs

Quinidine, Quinidex, Duraquin, Quiniqlute, etc.

Quinidine

Heart rhythm abnormalities

Pronestyl

Procainamide

Heart rhythm abnormalities

Norpace

Disopyramide

Heart rhythm abnormalities

Betapace

Sotalol

Heart rhythm abnormalities

Lipid lowering drugs

Lorelco

Probucol

High triglycerides, cholesterol

Antianginal drugs

Vascor

Bepridil

Chest pain (angina)


GASTROINTESTINAL

Propulsid

Cisapride

For esophageal reflux, acid


ANTIFUNGAL DRUGS

Nizoral

Ketoconazole

Fungal infections

Diflucan

Fluconazole

Fungal infections

Sporanox

Itraconazole

Fungal infections


PSYCHOTROPIC DRUGS

Elavil, Norpramine, Viractil

Amitriptyline (Tricyclics)

Depression

Compazine, Stelazine, Thorazine, Mellaril, Etrafon, Trilafon, others

Phenothiazine derivatives

Mental disorders

Haldol

Haloperidol

Mental disorders

Risperdal

Risperidone

Mental disorders

ORAP

Pimozide

Mental disorders


DIURETICS

Lozol

Indapamide

Water loss, edema



POTASSIUM LOSS
In addition, many diuretics cause loss of potassium, as does extensive vomiting and diarrhea, and low potassium levels in the blood can aggravate the symptoms of the Long QT Syndrome.


The Sudden Arrhythmia Death Syndrome Foundation (SADS)

Heart to Hearth is a publication of the SADS Foundation, a not-for-profit public charitable foundation. The primary purpose of this publication is for general information only and is not to be used as medical advice, diagnoses or treatment. Readers are reminded to seek the advice of their respective physicians.

If you appreciate the Heart to Hearth Newsletter, send your comments to Katie Roberts at the SADS Foundation and indicate you saw the newsletter on the Internet. This will help us to know what medium to use to best contact people. If you are not on our mailing list to receive this and other information about the SADS Foundation and the Long QT syndrome, please call the Foundation on our 1-800-stop-sad toll free number and ask to be put on the mailing list. If the Internet seems to be the most effective means of communication, we will attempt to put more of our materials here.

Best wishes and good luck.

G. Michael Vincent, M..D.