January 2004 

Julie Feldman MPH RD, Clinical Dietitian

2004 brings us another opportunity to pledge our dedication to our health.  For some of you, your health is the focus of the majority of your daily activities, and for others days may go by without acknowledging how lucky you are to be able to do what you do.  Working in the healthcare field gives me the opportunity to be thankful for my health every day.  We look forward to helping you achieve your optimal health in 2004 as well.  Please do not hesitate to let us know how we can assist you in your efforts to feel your best!

Johns Hopkins Research

The researchers at Johns Hopkins discussed their research on the CF gene at the annual meeting of the American Society for Human Genetics in Los Angeles this month.  Their findings include the identification of short repeats in a particular genetic code in the gene responsible for CF.  The researchers were also able to illustrate how the repetitive nature of the mutation maybe responsible for predicting the severity of CF.  Roughly 10% of the US population carries one portion of the gene mutation that causes CF.  This research will hopefully allow physicians to predict the severity of the disease in their patients.

     Inologic, Inc. announced December 2nd of last year that it has achieved its first milestone in the creation of INO-4995 for CF treatment.  This new therapy, funded by a $1.5 million award from the CF foundation.  This new drug has completed its toxicology examinations and has received orphan drug status from the FDA.  The focus of this drug is to restore the sodium and chloride balance in the lungs of patients with CF.  Most therapies are designed to treat symptoms of CF.  INO-4995 is designed to prevent the flare-ups from happening.

Phase II of CF drug

Pharmagene PLC announced in late December that they have started the Phase II clinical trial  of a CF disease-modifying drug.  The drug is being investigated by 3 CF centers in the UK.  The drug being researched is a synthetic form of human secretin, which affects mucous production.

New Orphan Drug

Dyax corporation has received orphan drug status from the FDA for their protein inhibitor of the human protein elastase (hNE).  The airways of CF patients are known for their persistent and excessive neutrophil production.  By controlling the release of these proteins including  hNE, this new therapy may be able to limit the damaging effects of excessive inflammation of the lung tissue.

Watching Your Sugars

As time goes on there is an increased likelihood of developing CF related diabetes.  Although I have talked with several of you privately about the risk and warning signs of high blood sugars, I wanted to review certain markers that we can all be looking for.  The first signs or symptoms of diabetes include frequent urination, excessive thirst, inability to gain weight despite normal intake, and lethargy following large meals.  For many of you, people have been telling you to eat anything and everything that you can to keep weight on.  This often includes candy bars, regular pop, cakes, cookies, etc...

If you are noticing any of the above signs or symptoms, it is important to let us know quickly.  For those of you who do not see any of these typical symptoms, keep them in the back of your mind.

When patients with CF do develop CFRD (cystic fibrosis related diabetes), it does not mean that they need to go on a diet.  Typically we just re-work their usual intake to avoid eating concentrated sweets like jelly beans, coke and added sugars and replace them with higher protein foods.