Kartageners Syndrome, PCD, Immotile cilia syndrome

[lynnie25]

Hi,

My name is Lynn. I'm 34 with Kartagener's syndrome(KS). KS is a subset of PCD(primary ciliary dyskinesia). PCD is an inherited cilia disorder. My cilia do not work properly to clear secretions causing chronic lung, ear & sinus infections. When you have PCD with reversed organs it's called KS. I have Dextrocardia with complete situs inversus.

I have mild bronchiectasis in both lungs. I had the left middle lobe removed at age 9 after a lung collapse. I'm getting ready for sinus surgery. My sinuses are pretty bad and painful.

I take Advair. I use the vest for chest pt(airway clearance)

Just wanted to introduce myself, say hello and find out if anyone else here has KS, PCD or immotile cilia?
Take Care
Lynn

[usrmmrn2]

Hello,

My name is Mark Robertson I am 40 years old and I too have Kartageners Syndrome. I have had to take medication (maintenance anti-biotic) for most of my life. I do have two beatiful adopted children. Obviously I could not have children. I do have the situs invertis (reveral of organs). I have a great Doctor that keeps a touch on me. To my understanding as long as you keep infection down and when an infection arises handle with a sense of urgency with “high powered” antibiotics. Please correspond with me and I will provide more information.

You had mentioned your lung had collasped. Please tell me more...how are you doing? How old are you? What do the Doctors say? Do you take anti biotics

Sincerely,

Mark Robertson

[jerrie97]

Hi my name is Rachel. I had a bronchoscopy on Halloween for a bronchial biopsy to rule out PCD. I've had recurrent bronchitis since January which grows only Haemophilus influenzae, Streptococcus pneumoniae, or both at times. I too have had quite a time trying to get rid of the Haemophilus. I've been on two different kinds of Augmentin (875mg and the big 1000mg), Biaxin/Zithromax (which does not help at all), and Vantin. I think Vantin helps the most. I've had CT scans done which ruled out situs invertus, so I may not have PCD, but we'll see. I don't have bronchiectasis, but I do have right middle lobe atelectasis and some other scarring. About how long did it take you to be diagnosed from a bronchoscopy? My doctor sent the biopsy to the Mayo Clinic.

[lynnie25]

Hi Rachael!

It's so nice to e-meet you. I'm not sure how long it took to get diagnosis from the bronch because I was a kid. I did go UNC Chapel Hill (They are the leading researchers on PCD in the US) last year to participate in the research and had the diagnosis reconfirmed through a nasal scrape and found out right away. That is only because it didn't need to be sent anywhere. It was kinda cool. They put my sample right under the scope I was able to view it. Then they showed me a video of normal cilia...yikes what a difference. The part that took several weeks was finding out my cilia defect. They actually slice the cilia like a cucumber(cross section) , photograph it, blow it up and can see what the defect is.
You can have PCD without situs inversus. PCD(primary ciliary dyskinesia) is the main disorder. There are subsets of PCD like Kartageners Syndrome,which is PCD with situs inversus. I wasn't surprised to hear about the atelectasis in the middle lobe. The scarring is from recurrent infections. Have you been tested for CF? Have you had any other respiritory issues prior to January. Any sinus or ear troubles?

I've cultured out the same two things as you. More often than not it's the h-flu for me. I'm on yet another round of antibiotics. I usually do not have such a hard time kicking these little buggers but this one is just being a pain in the behind)

Hope I haven't bored you to tears) If you have any questions please feel free to ask. I hope your biopsy comes back negative.

Sending many positive thoughts your way,
Lynn

[lynnie25]

Originally Posted by swimfast989

Hi my name is Rachel. I had a bronchoscopy on Halloween for a bronchial biopsy to rule out PCD. I've had recurrent bronchitis since January which grows only Haemophilus influenzae, Streptococcus pneumoniae, or both at times. I too have had quite a time trying to get rid of the Haemophilus. I've been on two different kinds of Augmentin (875mg and the big 1000mg), Biaxin/Zithromax (which does not help at all), and Vantin. I think Vantin helps the most. I've had CT scans done which ruled out situs invertus, so I may not have PCD, but we'll see. I don't have bronchiectasis, but I do have right middle lobe atelectasis and some other scarring. About how long did it take you to be diagnosed from a bronchoscopy? My doctor sent the biopsy to the Mayo Clinic.

Hi Rachael,

I'm just wondering how you made out with the biopsy? Did you get the results yet? I know it can take 6 weeks. Hope you're doing well.

Many Smiles
Lynn