Cystic Fibrosis Update (January 2004)

Care of cystic fibrosis patients has been steadily improving, with nationwide statistics showing an increase in patient survival and lung function. Additionally, new treatments are being implemented that actually improve lung function rather than merely slowing its deterioration. These new treatments include:

  • Pulmozyme®
  • TOBI®
  • Zithromax® MWF

While these changes are wonderful news for patients and families, all centers need to continue improving care. To this end, the Cystic Fibrosis Foundation has a new emphasis: individual center quality improvements. At California Pacific, our Pulmonary Team, led by Karen Hardy, MD, is directing their energy in this direction with the goal of improved patient outcomes.

The following are specific developments in the research and treatment of cystic fibrosis patients:

Gene Therapy: In 1989, the CF gene was mapped, thus accelerating a race for gene therapy and a better understanding of the cystic fibrosis transmembrane conductance regulator (CFTR) gene product. Currently, research projects are addressing all phases of the disease process. The Adeno-associated virus has proved to be a safe vector during phase 1 and 2 trials, and is now moving to a larger patient study. At California Pacific Medical Center, new research being led by Dr. Dieter Guenter, is evaluating liposomal vectors. New projects to facilitate moving CFTR to the cell surface are also underway. Ion channel modifiers are meant to alter chloride flow and thus return the airway lining fluid to normal levels so that cilia can move to propel mucus forward. Many of these types of products are currently in phase III studies.

Advances in Airway Clearance: Airway clearance is very important for patients and a new recognition of the significance of cardiopulmonary physical therapy has emerged. Now, in addition to moving mucus through pounding, other methods are becoming more commonplace (i.e. high frequency chest wall oscillation vests, PEP therapy devices and active cycle of breathing techniques). Also, there is a new appreciation of the value of exercise, thoracic mobility and strength, posture and a patient’s active participation in CF care. A key factor is individualizing therapy so the patient can master and adhere to it. New methods for making therapy more fun have also surfaced along with this work. At California Pacific, our team includes Ruby Ng, MPT, who uses biofeedback as a tool for showing patients how the various methods of airway clearance work for them.

Antibiotic therapy: New antibiotics to treat infections by oral, IV and inhaled routes are surfacing. Linezolid is a new oral antibiotic that treats MRSA. Chiron is developing a dry powder form of TOBI, which will enable patients to take their treatment in a matter of seconds versus 20 minutes.

Infection Control Issues: Some of the most common bacteria grown in the sputum of CF patients include Hemophilous Influenzae (HI), Staph Aureus (SA) and Pseudomonas Aeruginosa (PA). Generally, SA is the first bacteria seen in young children with CF. PA is the bacteria most commonly associated with infection in people with CF. This bacteria has been associated with worsening disease and some strains are becoming more resistant to the usual antibiotics.

Bacteria found less commonly in the general CF population include Burkholderia Cepacia (BC), Stenotrophomonas Maltophilia (SM) and Xylosoxidans Alcaligenes (XA). The BC bacteria is very rare in people with CF (<5%). There are nine different strains of BC, one of which is associated with rapid deterioration and possibly death within two years of acquisition. BC is very resistant to antibiotics and can be spread from person to person. The effect of SM and XA has not yet been determined. It is not clear whether these two bacteria attribute to worsening disease, nor is it clear if they are more resistant to antibiotics. We know they tend to emerge with aggressive antibiotic treatment and do not seem to spread as easily as BC or SA.

Infection Control Guidelines:
Following are standard precautions for all patients with CF:


  • Quarterly sputum C&S and frequent handwashing
  • Maintain a 3-foot zone between patients
  • Provide easily accessible covered waste receptacles for disposal of used tissues
  • Use toys that can be easily wiped down with alcohol-based solution
  • Provide hand washing facilities (alcohol-based gels are acceptable)
  • No shared equipment and no cohorting of patients


Isolation issues: Patients with multiple resistant Pseudomonas, MRSA, BC or VRE
should wear a mask when coming to clinic and be put into a room immediately. Inpatients should have a private room and wear a mask whenever leaving the room, and staff should wear gown/gloves/mask during patient contact.

This information was also shared with families in November 2003 during an annual educational forum at California Pacific. We also mail monthly newsletters to patients and families. California Pacific’s Cystic Fibrosis team continues to grow and strengthen, and has many years of experience with CF patients of all ages. Dr. Brown continues to lead care for adult patients and Dr. Hardy works with Drs. Wen, Alpert and Ofreneo, and Nurse Practitioners Sarah Boushey and Maria Villanueva, to provide care for pediatric patients. DJ Kaley is our new nurse educator and case manager. She has made a big difference in patient care and support this past year. Our team is known for being “the best in the Bay” for those times when admission is needed. If you have any questions regarding patients with CF or patients who might have CF, be sure to call and talk with any of us.

Pediatric Pulmonary and Cystic Fibrosis Program
Tel. (510) 428-3305 (main number). Select Option 1 for Physician Line
Tel. (510) 428-3885 x2135: DJ Kaley, RN, MSN, AE-C

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