|Lynn's Story: Answers Can't Come Soon Enough|
Like many people with primary ciliary dyskinesia (PCD), I was born with reversed abdominal and thoracic organs, (a condition called situs inversus totalis), but it wasn't until I was 9 (in 1979) that I was diagnosed with Kartagener syndrome, which is the name for PCD when reversed organs are present. Lung collapse of my left lung (anatomical right) after I fell on the playground showed bronchiectasis and I underwent lobectomy. This all happened before 'PCD' had been officially recognized as the cause for Kartagener syndrome er and no one knew much about it.
My parents and I were told I would be lucky to live a year. I lived with that misinformation all through childhood and some of my adult life. I was scared and felt alone for a long time. There was a period of time that I was afraid to go to sleep when I was sick for fear of never waking up.
The biggest turning point for me was connecting with Michele Manion who would go on to start the PCD Foundation (PCDF). I found current, accurate information, connected with other patients and had the opportunity to participate in research studies. I learned that PCD is progressive and that daily treatment compliance is required to maintain my health.
Sometimes that is not enough. I have lost PCD friends, some post transplant. It motivates me to be compliant. Through the years I've had numerous hospitalizations, infections of the ears, sinuses and lungs and sinus surgery. My treatments (airway clearance, nasal irrigations, nebulizers and various meds) take 2 hours on a good day, 4 or more during exacerbation. Fertility and pregnancy are also affected with PCD. It took 12 years and 3 pregnancies to have my wonderful son, Michael.
Social situations can be uncomfortable because I sound like a smoker and try not to cough. I don't look sick when I have infections, which makes it hard for friends and employers to appreciate the seriousness of PCD. I have a small circle of very close friends that I share the ups and downs of PCD with and my husband is an amazing support for me. Currently I have mild bronchiectasis in both lungs.
One of the biggest challenges I and others with PCD face is the lack of awareness about this disorder in the medical community. We have no published standards of care and it is difficult to find doctors who know how to treat it. While there are exciting developments in research all time for patients living with the disorder every day answers can't come soon enough.