The Atypical Cystic Fibrosis Nightmare
Having atypical cystic fibrosis is a nightmare! With just plain CF, there is no indecision or second guesses. You have a dedicated care regiment, organized therapy and policies put into place to help you during times of flareups and infections. But with aCF, there is no help...no dedication or policies to keep you healthy and safe while in the hospital. This typically leads to a shortening of life expectancy for aCF patients; just because the rules have not changed.
All my life, I was diagnosed with asthma. Whoever, when my asthma became unmanageable and I was not responding to typical treatments, a study was done that found out that I don’t have asthma. So you’d think they’d correct their diagnosis...
For years, physicians stood by and watched my health decline. They tried drug after drug, therapy after therapy with no resolve. When they ran out of ideas or caved in; it was your fault that they failed and either dismissed you from their care or just ignored you.
I had one pulmonologist back in 2014 who loaded me up with drugs. His philosophy was that if he could not control the symptoms, then he’d just overdose your medication. At that time, I was on Advair, Qvar, Asmanex, Spiriva, Theocron, Prednisone Regiment, Breo, Incruse, Albuterol, DuoNeb, Singular.
It wasn’t until 2016, when I got a new pulmonologist, did he realize that this might not be just asthma. With digestive complications, metabolic issues, lung disease and a long history; he agreed that the disease sounded like cystic fibrosis.
Without the aid of a positive genetic test and sweat test; my access to many programs was nonexistent. To correct the weight loss and muscle loss we added creon to my regiment and that stopped the muscle loss and balanced my minerals and vitamins. With the constant lung infections, we decided to add CPT to remove the mucus and in a year, he cut infection rate in half. Pulmonozyme and hypertonic saline was added to the regiment.
For the first time in my medical history, I was actually improving.
When my healthcare was noticed by the CF Center at Multicare, they were very hesitant with my care as they did not know much about atypical cystic fibrosis; however, the symptoms I was having matched that of a CF patient and I was only responding to CF-style treatment. In November of 2018, I was suppose to go to the CF Center for a work-up, but it was delayed after hospitalization.
I was told over-and-over by pulmonologist that I did not have CF, yet they could not say what was causing these symptoms. The hospital doctors said that everything looked like CF and when they tried to treat it only as asthma, my health rapidly failed. With all that I’ve been told, I expected to be notified that my new CF doctor was dropping me from his care.
That call never came, however, only 24 hours before my CF clinic visit, they canceled it. However, it was canceled due to my hospitalization and rescheduled one month out.
Is this typical of a CF center? Typically, a pulmonologist wants to see you after a hospitalization, so I was surprised to be delayed. I checked my diagnosis codes and still saw that I was covered by the CF Clinic and seen for non-classic cystic fibrosis. A day after my canceled appointment, there was an appointment for the CF Nutrition service scheduled.
I decided that I was no longer being followed by the MultiCare CF Clinic and was glad that I did not drop my CHI Pulmonologist. About a week later, the MultiCare CF Clinic called to reschedule a new appointment that will be my first CF Clinical appointment. They asked me to mask up and expect a long meeting with the doctor, nutritionist, social work and nursing.
Where exactly this is going? I don’t know!
Do I call my CF Doctor, really my doctor? Mostly with what I’ve heard from his colleagues. I am highly doubtful that they will take my case serious. And why should they? No one can solve this puzzle.