By Frances Benham

The conference held at the Tremont Plaza Hotel in downtown Baltimore in July 2003, was judged by participants as one of NAPE's best in terms of information provided.  We are indebted to Jean Feeney for organizing it.  She was ably assisted by Rosemary Atallian who invited Dr. Robert P. Murphy to discuss feeder vessel therapy.  Deborah Clark also was successful in bringing cardiologist Jonathan Plehn to discuss PXE's impact on the heart.  Deborah also brought nurse/practitioner Sara Miller Plehn who shared information on eating for a healthy heart.  Fran Benham invited Bill Paul, a long-term PXE patient, who shared his views on living fully with PXE.  Mr. Paul's talk is included in this issue.  Dr. Kenneth Neldner updated participants on a wide range of PXE issues. 

PXE and the Heart

Jonathan F. Plehn, M.D., FACC, cardiologist for the National Institutes of Health spoke on his review of the available research reports on the impact of PXE on the heart.  He noted that he found no such research and attempted to determine why.  He began with the estimated number of those who have PXE per thousand population and estimated the number to be about 1400 people in the USA.  These potential study subjects would be scattered across the nation.  Certainly NAPE's membership roster affirms his observation.  Only a few zip codes, even in the largest cities, show ten members.  Most medical doctors seldom if ever meet a person who has been diagnosed with it.  Thus it would be quite difficult to gather PXE subjects in large enough numbers to conduct a study to determine that impact.

Dr. Plehn noted that there are so many high impact disorders which affect larger numbers of persons that funding agencies tend to spend limited resources where they may help the most people.

The only broad-based study of PXE patients was conducted in the early 1980's by Dr. Kenneth Neldner.  Dr. Plehn's review of the medical literature and his observation suggests it to be unlikely that another such study will be done, except by another dedicated individual.  So while we know those who suffer from PXE may experience cardiovascular problems, doctors do not have scientific data that clearly delineates the impact.  Dr. Plehn expressed interest in determining that relationship but expressed doubt that a study would be funded any time soon.

Eating for a Healthy Heart

         Sara Miller Plehn, nurse/practitioner spoke about the role of diet in maintaining a healthy heart.  Recommendations for fruit, vegetables, fish, poultry and less sugar, fat and red meats came as no surprise.  Her emphasis on the role of exercise also surprised no-one.  NAPE members have learned the truths Sara Plehn shared - now if we can just follow them.

Feeder Vessel Therapy

         Robert P. Murphy, M.D., retinologist at the Retina Group of Washington, D.C. shared his experience in his work on Age Related Macular Degeneration and other disorders which behave in the eye as does ARMD. He has treated a small number of PXE patients and finds that the slow developing smaller bleeds typical of PXE (as compared to those typical of ARMD) can result in a better outcome for the PXE patient, especially if feeder vessel treatment is given very early in the bleed.  Dr. Murphy was introduced by NAPE member Rosemary Atallian who was treated by Dr. Murphy early in her bleed.  She reported that she enjoys 20/20 vision as a result of her treatment.  After his talk Dr. Murphy met individually with all PXE patients who wanted to talk with him.  Later a number of them made appointments for an evaluation by Dr. Murphy.  For those interested Rosemary Atallian and Frances Benham wrote an article about their experience with feeder vessel treatment.  It was published in the May 2003 issue of PXE Awareness and can also be found on the NAPE website. 

A Continuum of Care

         Bill Paul, an executive for a major corporation, was diagnosed a number of years ago with PXE.  He continued to work, even receiving a promotion while seeking the care he needed to live as fully as possible with vision loss.  Mr. Paul has spoken to physicians about patient needs and has published in medical journals to encourage doctors to assist patients in obtaining ongoing care and coping skills when faced with vision loss.  He inspired his audience to tenaciously seek the care they need and to invest in helpful aids.  His article, published in several journals, appears in this issue.  Mr. Paul stayed for individual discussions with all who wanted his guidance.

PXE Update

         At each annual meeting for many years Dr. Kenneth Neldner has conducted a session on basic information about PXE and living with it.  This meeting was no exception.  Dr. Neldner provided information and responded to questions.  He focused on the most current information available about PXE.  The NAPE website carries two articles that include the basics of PXE.  Tap into www.napxe.org to find them.

Back to Table of Contents

Home | Current Article | What is PXE? | Contact NAPE |

Membership Form | PXE Pals | Q & A | Amsler Grid