CF Care Center Corner

Maria Fareri Children’s Hospital at Westchester Medical Center and New York Medical College

I’ve been caring for patients with Cystic Fibrosis for over 35 years. Just writing that sentence amazes me.  It has been an extraordinary adventure, and I am enormously grateful to all the patients and families that have placed their trust in me and our center here at the Maria Fareri Children’s Hospital at Westchester Medical Center and New York Medical College. How did I get “hooked” on CF?  One reason is that I was blessed with terrific role models. I just happened to train at great CF Centers, where I was lucky enough (and now old enough) to watch and learn from the generation of CF docs that preceded me. I want to say a few words about the physicians that cared for children for the two or three decades before I arrived on the scene. 

Before I started out, caring for children with CF was a lonely profession Almost all patients with CF back then were children and would never make it past childhood.  Much of today’s routine treatments for CF were not available, and pediatricians that dedicated their professional lives to CF were special people drawn to their calling.  Being a CF doctor was not for the faint of heart. They were fighting against a ferocious, relentless disease that was emotionally devastating; and only the most driven physicians chose to stay in the fight.  I was fortunate to know and admire Jack Gorvoy at LIJ, Harry Schwachman at Boston Children’s, Mandy Mascia here at Westchester Medical Center, and most importantly for me, Pureza Gaerlan and Carolyn Denning at St. Vincent’s.  These caring and loving men and women embodied for me what it means to be complete physicians: hard-working, single-minded, not afraid to become emotionally involved with their patients and their families, sitting by their bedside for nights on end, focusing their intelligence on figuring out what works best for each individual patient. I want to particularly single out Carolyn Denning, who taught me so much.  She was one of the most amazing doctors I’ve ever met.  Her focus, dedication, and grit were inspirational. 

The lives of patients with CF have dramatically changed since then, and more than half are now adults. Just this past month, I spent a few minutes with a former patient of mine, now 70 years old, who has been cared for by our adult CF center for a long time now. Far too many patients still lose their battle with CF, but the future grows ever brighter. 

What I learned back then from that amazing generation of CF docs remains just as true today. The key to success lies not in any specific medication or combination of therapies.  Success is much more fundamental. In no particular order, here are some of my principals of care:  

All patients should go to an accredited CF Center. No matter where you live, get to a CF Foundation-accredited center.  The accreditation process is very rigorous and you can be assured you will be find a dedicated team and state-of-the-art care. All accredited CF centers in the New York metropolitan area are terrific.  I’m particularly proud of our center’s dramatic increase in research including clinical trials testing new medications. Today, patients often move many times through life, sometimes all over the country. This is a wonderful sign of how life has changed for people with CF. Wherever you move, find an accredited center. Simply put, patients receive better care at centers dedicated to teaching and research.  This makes a huge difference.

Find a doctor you like and trust.  Find someone who listens to you, who truly believes in the concept of “shared-decision making,” that you are a team.  When you meet a doctor who is convinced they are always right, run from that doctor. If they don’t call you back, or are not willing to spend the time required to advocate on your behalf, try someone else.
Plan on frequent scheduled visits to your CF Center, even when you feel perfectly well; especially when you feel well.  Organize your life around these visits, and insist on them. The CF Foundation recommends visits at least 4 times per year, and that should be considered a minimum. I know this isn’t easy, especially as patients grow older and have competing demands on their time, such as school or work. Even if this means missing school or work at least 4 times a year, this single step makes all the difference.

Close monitoring is essential, watching for any subtle changes.  This includes not just frequent clinic visits but performing pulmonary function tests and obtaining respiratory cultures as often as possible. I know many patients tire of doing spirometry, or don’t want to see the numbers.  Sometimes I don’t either.  But this simple test detects mild changes long before patients are aware or before a physician will hear anything different with a stethoscope or see something different on an X-ray. And now, many centers, including ours, are able to measure lung function very accurately and easily even in infants, toddlers, and preschool children.

The overreaching attitude and philosophy of your CF physician and their team is critical.  Be aggressive, never give up.   If either you or your doctor detects a change such is increased cough, trouble maintaining or gaining weight, decreased appetite or energy level, do something.  Don’t give in and watch your health slowly slip.  Don’t ever assume you can’t beat CF. Fight it all the way.

Focus on nutrition. One of the most important discoveries contributing to the dramatic improvements in longevity for our patients is related to our approach to nutrition.  When patients eat many more calories than anyone else their age, this extra intake actually slows the lung disease of CF.  30 to 50% extra calories is more effective than all the antibiotics in the world, actually killing germs on contact.  For many patients, this additional caloric intake cannot be accomplished with just oral supplements, and therefore tube feeding must be considered.  Don’t be afraid of tube feeding.  It really works! 

Live life to the fullest.  If you are parent of a child with CF, teach them early it is possible to devote a significant portion of every day to taking care of their health, adhering fully to their medical regimen, and still find time to follow their dreams.

 I hope to keep learning from our patients and their families for years to come.   

Allen J. Dozor
May 2014