Skip to main content

Dr. Gahl honored by NORD

Those of us in the Hermansky-Pudlak Syndrome community know not only what a great doctor, but also what a great person, Dr. William Gahl is - so it's no surprise to us that he was honored this year by the National Organization for Rare Disorders (NORD). Below is the announcement. I know many of you are anxious to hear how the American Thoracic Society meeting went, and I'll be posting more about that shortly.

One of the things that was truly amazing about this week, however, is that the HPS Network was making those little steps towards the cure on both coasts this last week. While we were at ATS, the Network was represented by Ryan (my brother), and Kristie and her mom Jackie on the East Coast. They went to the dinner in honor of Dr. Gahl and the other award recipients. Ryan and crew got to also meet Dr. Gahl's wife and two of his four children. I didn't know this, but Dr. Gahl has a son serving in Iraq. There's another thing to add to our prayer list!

Honorees Announced for 2007 Tribute Banquet


A National Institutes of Health researcher, two members of Congress, the European Organization for Rare Disorders, and four companies will be honored at the 2007 NORD Tribute Banquet in Washington, DC, on Monday, May 21. This is the annual event at which NORD honors individuals and companies that have made significant contributions improving the lives of those affected by rare diseases.


For information about reserving a seat or table for the 2007 NORD Tribute Banquet, or space for an ad in the commemorative journal, contact Donna B. Jackson in the NORD Development Office at (203) 744-0100 (or djackson@rarediseases.org). Hotel reservations for rooms at the Capital Hilton in Washington, DC, can be made by calling (202) 393-1000. (Ask for the NORD room rate, which will be available until April 20.)


The 2007 Tribute Banquet honorees are:
NORD Leadership Awards


Representative Judy Biggert (R-IL), U.S. Congress

Representative Louise Slaughter (D-NY), U.S. Congress

NORD Public Health Leadership Award

William Gahl, MD, PhD, National Institutes of Health

NORD International Leadership Award
The European Organization for Rare Disorders (EURORDIS)

NORD Corporate Leadership Awards
Alexion Pharmaceuticals for the development of Soliris to treat individuals with paroxysmal nocturnal hemoglobinuria (PNH)

Genzyme Corporation, for the development of Myozyme to treat Pompe disease

Karl Storz Endoscopy-America, Inc., for the development of the Karl Storz Semi-Rigid TTTS Fetoscopy Instrument Set for twin-twin transfusion syndrome

Shire Human Genetics, for the development of Elaprase to treat mucopolysaccharidosis II (Hunter syndrome)

Congresswoman BiggertRepresenting a suburban Chicago district in the U.S. House of Representatives, Congresswoman Biggert is an attorney and elected official with intense interest in education, health, and women’s issues. She and Congresswoman Louise Slaughter are being recognized by NORD for their co-sponsorship of the Genetic Information Non-Discrimination Act. This is an important piece of legislation aimed at preventing discrimination through misuse of the genetic profiles of individuals. The Act would prohibit the use of genetic information in decisions related to employment or health insurance. Congresswoman Biggert has taken a leading role in women’s health research, issues related to homelessness and domestic violence, and she helped to craft the No Child Left Behind Act.

Congresswoman Slaughter Currently in her 11th term in the U.S. House of Representatives, Congresswoman Slaughter is the first woman in history to chair the influential House Committee on Rules. She holds a bachelor’s degree in microbiology and a master’s degree in public health, and she began her career as a bacteriologist for the Department of Health in Kentucky. Now, her home district is in New York State, covering Rochester, Buffalo and Niagara Falls. With her scientific background, Congresswoman Slaughter has taken a leadership role on issues related to science, patient protections, and biomedical research in the U.S. Congress. The Genetic Information Non-Discrimination Act that she and Congresswoman Biggert co-sponsor is expected to be passed by the Congress this year.


William Gahl, MD, PhDDr. Gahl is an internationally known expert on cystinosis, Hermansky-Pudlak syndrome, alkaptonuria and disorders of sialic acid metabolism. He is a dedicated public servant with a profound allegiance to the study of rare diseases. He currently serves as clinical director of the National Human Genome Research Institute (NHGRI), head of the Section on Human Biochemical Genetics in the Medical Genetics Branch of NIH, and director of the Intramural Research Program of the NIH Office of Rare Diseases. In particular, he is being honored for his support of the rare-disease community through his work with the Office of Rare Diseases at NIH and the National Human Genome Research Institute. Dr. Gahl serves on the medical advisory boards of the Cystinosis Foundation, the Cystinosis Research Network, and the HPS (Hermansky-Pudlak syndrome) Network.


EURORDISThe European Organization for Rare Disorders (EURORDIS) is being honored on the occasion of its 10th anniversary. EURORDIS represents more than 260 rare disease organizations in 29 European countries, covering more than 1,000 rare disorders. The European Union defines a rare disease as one that affects fewer than one person in 2,000 European citizens. Therefore, EURORDIS is the voice for 30 million patients. It was initially modeled on NORD, and NORD’s president, Abbey Meyers, has served as honorary president of EURORDIS since its founding. Today, EURORDIS provides important advocacy for patients and families, and promotes awareness, research, and the development of new treatments.


Alexion PharmaceuticalsAlexion is being honored for the development of Soliris, the first treatment to be approved by the U.S. Food and Drug Administration (FDA) for the rare blood disorder known as paroxysmal nocturnal hemoglobinuaria (PNH). This disorder affects approximately one in a million people. It is characterized by abnormal development of red blood cells. Components of the body’s immune system attack and destroy these abnormal cells, causing anemia and debilitating weakness. In severe cases, the disease may lead to life-threatening heart attacks and strokes.


Shire Human Genetics TherapiesShire is being honored for the development of Elaprase, an enzyme replacement therapy approved by the U.S. Food and Drug Administration as a treatment for MPS II in 2006. MPS II (or Hunter syndrome) is a genetic disorder that primarily affects males. Signs and symptoms are generally not apparent at birth but may become noticeable during the first year of life. They include inguinal hernias, ear infections, and frequent colds. Later manifestations may include a decline in cardiac function, breathing problems, characteristic facial features, and joint stiffness.


Genzyme PharmaceuticalsGenzyme is being honored for the development of Myozyme, an enzyme replacement therapy for Pompe disease. This disease occurs as the result of a deficiency or lack of an enzyme necessary for normal muscle development. There are different forms, including an infantile form and later-onset forms. Myozyme has been approved by FDA for treating the infantile form. It continues to be evaluated as a treatment for the other forms. About 40,000 people have Pompe disease, and the disease can result in life-threatening respiratory problems. The infantile form is the most serious.


Karl Storz Endoscopy-AmericaThis company is being honored for the development of the Karl Storz Fetoscopy Instrument Sets, which are surgical tools used in the treatment of twin-to-twin transfusion syndrome (TTTS). This is a rare condition in which there is an imbalance in the amniotic fluid of some identical twins. The imbalance is caused by an uneven flow of blood between the twins through shared blood vessels that are present when the twins share one placenta. The fetoscope is a telescopic camera used to view a fetus. After it identifies the shared blood vessels, a laser is used to destroy the shared blood vessels with heat and normalize the flow of blood between the twins.

Comments

Popular posts from this blog

Ratner's Cheesecake

Here's another recipe from Toby! Thanks Toby......and I'll get the others posted soon! Ratner's Cheesecake and plain cookies Ratner's was a Jewish dairy restaurant in the lower East Side of Manhattan. This recipe, from my disintegrating, no longer in print Ratner's cookbook, is the closest I've ever gotten to reproducing the rich, heavy cheesecake my mother made when I was a kid. It's worth the time it takes to prepare and every last calorie. Dough Can be prepared in advance. Makes enough for two cakes. Can be frozen or used to make cookies – see recipe below. 1 cup sugar 1 teaspoon vanilla extract 1 cup shortening 1 teaspoon lemon extract 1 cup butter 2 eggs 3 cups sifted cake flour ½ teaspoon salt 2 cups all purpose flour 1 teaspoon baking powder 1. In a bowl, combine all ingredients with hands. Refrigerate 3 -4 hours, or preferably overnight. Filling (for

The next generation with Hermansky-Pudlak Syndrome

I'm so behind on posting about the trip to Puerto Rico. Since the episode of Mystery Diagnosis on Hermansky-Pudlak Syndrome ran right after we got home, it's been a little busy. These, however, are my favorite pictures from Puerto Rico. I know, not pretty senery etc - but these little guys and gals inspire me. They are the next generation of folks with HPS, and if we keep up the hard work, they will live better lives because of it. They motivate me.

Some good news about Pirfenidone

Below is a press release from Intermune, the company that makes Pirfenidone. They have essentially reviewed the various clinical trials going on, and decided that Pirfenidone is safe and well tolerated. That would pretty much go along with what we've observed in the HPS community as well. We have a few folks that have been on the drug since the late 90s and continue to do well. Of course, as a journalist, I do have to say consider the source - but at the same time, as someone in a Pirfenidone trial, it's good to know. Results of Comprehensive Safety Analysis of pirfenidone In IPF Patients Presented At European Respiratory Meeting - Analysis shows safety and tolerability of pirfenidone across four clinical trials - VIENNA, Sept. 14 /PRNewswire-FirstCall/ -- InterMune, Inc. (Nasdaq: ITMN ) today announced that the results of a comprehensive review of safety data from four clinical studies were presented at the 2009 European Respiratory Society Annual Congress in Vienna, Austria