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Providence Hospital and Medical Centers

April 2005 

Monthly Cystic Fibrosis Newsletter

Julie Feldman MPH RD, Clinical Dietitian

   

Back Online 

You may have been thinking, where is that CF newsletter that I so love to read each month! Well, it has been on maternity leave. But we are happy to publish the first, in a while, CF newsletter. Back and better than ever, we promise to continue to keep you updated and abreast of all new information that pertains to your health, along with other fun tidbits and of course good recipes. If you have any questions about upcoming CF news or information contained in this newsletter, please contact Julie at julbethy@yahoo.com.
 
   

Fast Track

PTC Therapeutics, a biopharmaceutical company, focused on the development of drugs targeting DNA, has announced that the company has been granted fast track designation from the United States Food and Drug Administration (FDA) for the development of its novel drug, PTC124, for the treatment of cystic fibrosis(CF). Development programs receiving fast track designations typically receive FDA priority review (6-month vs. standard 10-month review). PTC124 targets the nonsense mutation in the cystic fibrosis transmembrane regulator (CFTR) gene. Nonsense mutations are single-point alterations in the genetic code that prematurely halt the translation process, producing a shortened, non-functional protein. PTC124 allows the cellular machinery to bypass the nonsense mutation and continue the translation process, restoring the production of full-length, functional proteins. It is estimated that 10% of the cases of cystic fibrosis (CF) and 15% of the cases of Duchenne muscular dystrophy (DMD) are due to nonsense mutations. PTC has catalogued over 1,800 distinct genetic disorders where nonsense mutations are the cause of the disease in an appreciable percentage of patients. In addition to CF and DMD, other potential indications under consideration for PTC124 include hemophilia, neurofibromatosis, retinitis pigmentosa, bullous Skin diseases, and lysosomal storage disorders.
   

Technology a Cure

Alnylam and Cystic Fibrosis Foundation Therapeutics, announced on March 16, 2005 that they are developing RNA therapeutics, targeting the defective Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein, for the potential treatment of cystic fibrosis. Under the agreement, the companies will use Alnylam's RNA technology that restores protein function.
   

Speed Screening

Ambion Diagnostics, announced the adoption of its first molecular genetic test test, Signature CF 2.0 ASR. The Signature CF 2.0 ASR detects mutations in the gene associated with cystic fibrosis (CF), and represents a new generation of molecular diagnostic tests that brings simplicity and speed to genetic testing. Cystic Fibrosis is one of the most common genetic disorders in Caucasian populations, carried by about 1 in 29 individuals. CF is much less frequent in Asian Americans (carried by 1 in 90), in African Americans (1 in 65), and in Hispanic Americans (1 in 46). The American College of Obstetrics and Gynecology (ACOG) now recommends that obstetrician-gynecologists make DNA screening for cystic fibrosis available to all couples seeking preconception or prenatal care - not just those with a personal or family history of carrying the CF gene, as previously recommended. The Signature CF 2.0 ASR, can be used to identify 25 mutations and 6 variants of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

   

CF Clinic Location

While most of you have been to our new office location, many of our patients are always looking for our office as its location changes frequently. We are currently located in Suite 203 of the Providence Medical Building. Just take the elevator to the 2 nd floor, and turn right . Our office is the first on the right. On that same note, if any patients wish to be enrolled in the Axcan Scandipharm Comprehensive Care Program which allows patients to receive free ADEK vitamins, flutter, and Scandishakes with Ultrase prescription need to contact Julie at 248-464-0076.

   
Nutrition News

20 Minute Skillet Salmon

Fish is the perfect source of omega-3 fatty acids which help reduce inflammation throughout the body. Enjoy this wonderful Salmon Recipe for a delicious spring dinner.

Prep Time: 5 min
Total Time: 20 min
Makes: 4 servings

 

1 Tbsp. oil

4 salmon fillets (about 4 oz. each)

1 cup whole milk

1/2 cup (1/2 of 8-oz. tub) PHILADELPHIA Cream Cheese Spread

1/2 cup chopped cucumber

2 Tbsp. chopped fresh dill weed or 2 tsp. dried dill weed

 

HEAT oil in large skillet on medium-high heat. Add salmon; cook 5 min. on each side or until fish flakes easily with fork. Remove from skillet; cover with foil.

MIX milk and cream cheese spread in skillet until well blended. Stir in cucumber and dill. Return salmon to skillet. Cook 2 min. or until heated through.


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