June 2005 

Julie Feldman MPH RD, Clinical Dietitian

Are You Listening?

Reading and learning about CF doesn’t always have to be boring. Through the support of the Boomer Esiason Foundation, you can log on and download MP3’s of interviews with CF patients, family members and caregivers which offer enlightening looks into the world that you all share, and battles that you all face day to day. Jerry Cahill's Cystic Fibrosis Podcast is presented by the Boomer Esiason Foundation. Jerry Cahill is a 49 year old male with CF who has been able to thrive with a disease that has an average life span of 31 years. Visit Jerry at www.jerrycahill.com.  

New Standards

Hannover medical school has announced new criteria for screening for diabetes in CF patients. Their results were published in the European Respiratory Journal this past May. CFRD is of obvious concern as it is the most common comorditiy in CF patients. Current guidelines recommend an Oral Glucose Tolerance Test only when a fasting glucose test is elevated. The researchers at Hannover found when looking at 108 patients, that several patients slipped through the cracks of these testing requirements. They now recommend an OGTT be performed annually for all CF patients to ensure early detection of CFRD.

Better than a Scale

As you all now, every visit to Dr. Pichurko begins with being weighed. This is done in order to provide us with information regarding trends in your body weight, which is often linked to abdominal issues including obstruction, possible onset of CFRD, and generalized decrease in health. Researchers are recommending using BMI and body fat percentage as markers of nutritional status instead of relying solely on the scale. Clinical indicators of malnutrition include a BMI less than 18.5 and a body fat percentage of less than 10% for men or less than 20% for women. In certain patient groups BMI is a poor representation of body fat. The researchers of the present study investigated whether the simple use of body fat percentage estimated from BMI would accurately assess nutritional status in cystic fibrosis (CF) patients. Whole-body bioimpedance was used as the reference method. This study found that BMI was a good indicator of nutritional Status. This means that a simple calculation of BMI can be used to detect patients at risk for malnourishment.  

More Effective Test

AMBRY GENETICS, a worldwide leader in genetic testing, announced it has introduced a valuable enhancement to its Cystic Fibrosis molecular testing menu. The Ambry Test: CF AMPLIFIED can now detect gross deletions, increasing the detection rates of genetic abnormalities that can cause CF. Gross deletions for CF have been commercially undetectable until now. The tests provide the most comprehensive analysis of the CFTR gene currently available, scanning for the more than 1,300 known mutations rather than simply identifying 25 to 100 specific mutations. Gross deletion testing can aid in those cases when full gene sequence analysis has not resulted in a conclusive diagnosis. Genetics new deletion assay may solve more patient dilemmas by providing a complete explanation, Ambry Genetics is offering to retest individuals, when appropriate, at a reduced cost. From now through the end of August, patients who have previously had their DNA analyzed by the Ambry Test: CF can be tested for gross deletions for $250. Clients who have submitted DNA within the past thirty days do not have to submit another sample.

Get Dressed for Good Health

The LifeShirt System from VivoMetrics will be used in a multisite Clinical trial supported by the Cystic Fibrosis Foundation to measure and gather data on the breathing patterns of children with cystic fibrosis (CF) between the ages of 3 and 6 years. The LifeShirt is a noninvasive, light-weight garment with embedded sensors that collect pulmonary, cardiac, posture and activity signals. It is used to monitor respiratory and cardiopulmonary function. The LifeShirt System is based on a miniaturized, ambulatory version of respiratory inductance plethysmography -- a technique for measuring breathing. Breathing is measured via wires embedded in the garment that surround the rib cage and abdomen. The LifeShirt collects respiratory data while the patient sits quietly reading a book or watching a video. Preschool children may be more easily able to comply with the LifeShirt measurement than with other measures that involve breathing into a mouthpiece while wearing a nose clip. The study objectives include obtaining pulmonary function data that can be used for planning future multicenter clinical trials in preschool children with CF, and better characterizing the natural history of CF lung disease.