Primary Ciliary Dyskinesia?

It seems like I’m always chasing An Answer haha

I have recently been in hospital with sputum showing P. aeruginosa and S. aureus. Sputum sample #2 went missing, and I couldn’t produce a 3rd sample but my inflammatory markers — which I assume were raised when I was admitted — came down. I have only been diagnosed with severe asthma, and although a lot has changed lung-wise since my chest CT in early 2016, it was decided that as that was clear, I didn’t need an updated one. So Tazocin was stopped after 5 days instead of the 2 weeks they were originally going to do, and despite there having been the same amount of growth of staph and pseudomonas on the one sputum sample they had, they determined that the staph was causing my symptoms but that the pseudomonas was probably contamination. But how can they determine that when there was the same amount of growth of both, and they only have one sputum sample? So anyway, they reasoned that Tazocin is a strong antibiotic — which is fair — and that, if I don’t have underlying bronchiectasis or COPD, we don’t need to do the full 2 weeks as long as my infection markers are down.

But bronchiectasis can start to develop in 3 years, can it not? I also make up my own (sterile) hypertonic saline solution at home which really helps, but didn’t have any in hospital, so honestly my lungs felt like they had concrete sitting in them. I coughed up some little green hard pearls of mucus but really couldn’t shift anything. I’m home now and have been using my hypertonic solution, and now I can clear my chest better but I’m bringing up the same sticky slightly-fluorescent green stuff I was bringing up before I went into hospital. My peak flows have steadily declined over the past few months and although they increased a bit in hospital — presumably due to my asthma getting a helping hand and the main infection being quashed — they’re still struggling.

My only thought now is that I can feel something isn’t right and disagree with their choice not to repeat my CT. Now I’m home with my hypertonic saline, I can get an updated sample and drop it into clinic on Tuesday to be sent off to see where that’s at. I have had repeated ear infections since early childhood, most requiring antibiotics, and chronic sinus issues, as well as what has been considered to be refractory atypical asthma, and wonder if it is worth asking my consultant to rule out PCD? He did test for CF which came back negative, but I know someone who was recently diagnosed with PCD at 25, with only very mild bronchiectasis which showed on a CT.

Any thoughts? Sorry this is so rambling!

[Hidden]

I’m glad that you finally discovered that you don’t have cf

Pseudomonas shows up florescent on the lab plate and when the numbers are high you can see it when you cough it up -interesting fact.

I really don’t know what they are playing at. They have sent you home still poorly and with symptoms of a chest infection and pseudomonas.

We don’t know who read the last ct and if they were trained propery to look for bronchiectasis.

Yes, when there have been repeated infections bronchiectasis can develop in three years. It can also have been brewing for years undiscovered in a person with asthma and missed on the last ct scan.

Does anyone in the group you have been seeing actually have any speciality and experience in bronchiectasis? They sound to me as if they are playing in the dark, doing a bit of this and a bit of that with no real idea of what they are dealing with.

Having a test for PCD could be a good idea but it will only indicate why you have developed the lung condition and have the infection problems. The lack of cillia means that the secretions in the lungs are not naturally cleared by the tiny hairs, infection gets into them and the cycle goes on. It can lead to bronchiectasis over time.

You need to see a proper bronchiectasis specialist who can do the breathing tests, read a ct scan properly and decide what it is that you are struggling with.

If I was you I would find the name of a bronch specialist in your area who is not with the people whom you have been seeing. Take it to your GP. Tell the GP that you are getting nowhere with the people whom you are seeing and want to start again with a bronchiectasis specialist. Do not take no for an answer. This is your health we are talking about.

[Hidden in reply to Hidden]

Oops I didn't quite give all the information. Before I went in I was a fair bit worse. I was having night fevers and intense body aches which were stopping me sleeping. This has stopped with the Tazocin, and my inflammatory markers went down. I was told to go straight back in if the night fevers came back or I got any worse. I also have a clinic appointment on the 19th with my consultant, where I will be able to discuss my concerns: I have also asked for his email address in the meantime so I can send him my concerns and give him a chance to look over those and consider them. I am also dropping in a sputum sample on Tuesday.

My current consultants are an asthma+ILD specialist, and an asthma+CF+non-CF bronchiectasis specialist, with the latter being my main consultant. He is very good and I am confident that, if I explain to him in person how much things have changed since that last CT, and mention PCD to him, he will be more than happy to take it all into account and work with me to find answers. He was the one who sorted the CF tests for me, and Leeds has a PCD centre with which I know he has contact. I don't have any qualms with him, or with the nurses who looked after me, but I think the doctors did not take everything into account. They did have a senior radiologist look over my CT from 2016 and it was perfectly clear, but I need to impress upon them just how much things have changed since then.

In fairness to them, my x rays were clear, my inflammatory markers went down, and my fever and body aches went away, and if I 'only' had asthma (although they did stress that there's nothing 'only' about severe asthma) then the longer course of Tazocin would not be suitable once infectious markers and signs are down due to the risk of resistance forming. With asthma alone, cough, chest tightness, chest ache etc. take time to clear after an infection, and I have the appointment on the 19th to look into things further, so for someone with solely asthma, it would make sense to send them home once IV antibiotics are no longer indicated (as they would not be indicated for the full 2-week clean-out unless there's bronchiectasis, PCD, or COPD as well) to rest at home, away from infections we could easily pick up in hospital. So I do understand that.

But I agree that it doesn't make sense for them to conclude that the pseudomonas was probably contamination and the staph was infectious when both were 2+ on the one sputum sample they had, and granted it was a senior microbiologist who looked over those but I'm not sure how he came to that decision based on one sputum sample only. My anti-pseudomonas antibodies were negative but that doesn't always mean it's not there. I also feel annoyed that they didn't try and elicit a sputum sample to check up on either of the things grown on that original one. I understand hesitancy giving hypertonic saline to someone who has asthma alone, especially severe asthma, especially when ill because it often causes bronchospasm and I should have been honest with them and said I use hypertonic at home..but they still should have taken another sputum sample, even if they still discharged me after taking it.

Hopefully I'll get my consultant's email address soon but if I don't have it by Tuesday, I will write up my questions and my email address and take those in with my sputum sample and ask the desk clerk to pass it on to him.

[cofdrop-UK]

Absolutely agree with littlepom and feel you are being sort changed by your present medics.

Once you see a consutant with a special interest in cf/bronchiectasis they should do all the tests including for PCD. PCD is inherited, as I am sure you will know, and can incude more problems than bronch, sinus and ear problems. I do know some with PCD who are infertile and have situs inversus. Saying that people who have bronciectasis which is not PCD related also have problems with their ears and sinuses.

Please find a good proactive consultant. Your treatment has been unnaceptable.

Cx

[Hidden in reply to cofdrop-UK]

I have situs solitus but then again only 50% of PCDers have situs inversus. With that said, an awful lot of medical literature still uses PCD and Kartagener Syndrome interchangeably, which just goes to show how misunderstood PCD is!

My main consultant is actually a severe asthma specialist, but also one of the lead CF doctors at the Leeds Adult CF unit, and a non-CF bronchiectasis specialist. Leeds also has one of the UK's four PCD centres, and I know my consultant has contact with them. I have mentioned my sinus issues to him, which was part of the reason he organised CF testing, but I haven't mentioned my ear issues, and I haven't impressed upon anyone really just how dramatically things have changed since that last CT scan. I also wouldn't know if I'm infertile because I am actually female-to-male transgender so I don't have sperm to see if their flagella work properly, and I haven't ever tried to get pregnant so have no history of tubal pregnancies which would be an indicator. So my only indicators are chronic lung issues that don't quite fit with asthma but aren't CF, repeated bronchitis throughout childhood (but that's common in asthmatics anyway), ear issues since early childhood -- currently itchy and on/off ache, dammit -- often requiring antibiotics and drainage, and ongoing sinus issues. But again, the latter is common in asthmatics as well. I may also have pancreas issues, and these have been linked to ciliaopathies in some recent medical literature but all we have for that are some symptoms, and an incidental finding on an otherwise-clear CT of my kidneys.

Please also read my reply to littlepom as I have written more there to clarify things! But my consultant is very good so I have every faith that he will understand my position and work with me once I explain everything to him but it irks me that the hospital didn't take a repeat sputum sample.

[cofdrop-UK in reply to Hidden]

Hi again mainsdour,

Having read your replies giving a much fuller explanation I have certainly changed my mind about the service you are getting. You seem to know a lot already, especially as you have had asthma for some time. Am I right in thinking you were on the general respiratory ward on level 7 and that your 2 concerns are that 1. They didn’t do another sample and 2. You are querying if you might have PCD. Things don’t always happen as you hope they will on the resp ward. It does make sense to get you out of the bug ridden hospital. I am lucky enough to do my own IVs at home over the holidays, which not only saves the hospital and community money but allowed me to spend time with my family instead of hospital. Good to hear your organs are all in the right position and that fertility has not been an issue for you.

I absolutely take it all back re your consultants now you have explained things. I, along with some other pals on here see the fabulous cf/bronchiectasis team. My consultant is Lead Clinician, although I see the consultant I am sure you are talking about within the Bronchiectasis Service who is amazing too. They are a very proactive, caring team and will not leave any stone unturned.

Hope you have a good consultation on the 19th.

Cx

[Hidden in reply to cofdrop-UK]

Ah if we're on about the same doc, he is really good and I have every faith he'll understand that I just need to be able to get better and not be held back, especially now I'm a student.

With regards to your questions, yeah I was on J9 and I'm frustrated that they didn't take another sputum sample. However, I do feel I ought to have been 100% honest with them and told them I use hypertonic saline solution at home and that it helps with no issues regarding my asthma. I may have gotten myself a metaphorical slap on the wrist for a) DIYing nebuliser solution (although I am incredibly careful about sterility) and b) using hypertonic saline unsupervised with being a severe asthmatic, as I could have triggered an attack alone without access to help I would have needed. It felt like I had concrete in my lungs but I was daft enough to be worried that they would think I was a complete idiot! haha

My chest aches pretty bad and I'm still struggling somewhat but I left a sputum sample yesterday so hopefully that won't go missing or get forgotten or anything and the results will be available by the time I see Dr C on Tuesday morning. I also left a message with his secretary and she emailed me back saying I could email my questions to her and she would ensure they are passed on to him, which I wanted to do to give him a chance to see things ahead of my appointment rather than be bombarded with things during the appointment which would probably be poorly worded and unhelpfully structured, knowing me!

I intend to outline my question regarding why the doctors on J9 thought it would take many years to develop bronchiectasis from a clear CT, so there wouldn't be any point doing an updated one as there wouldn't be any change like that in a 3-year time frame. I'll also outline my issues in general and ask about PCD, and note the effect things are having on my studies and general QOL (I haven't been able to play ice hockey for months, for example).

I guess I just constantly worry that I'll seem overly pushy, like I'm trying to get some horrible diagnosis, especially as tests often come back normal. For example, I had a CT scan which showed a 'plump, featureless pancreas' which is consistent with pretty much just one condition, Autoimmune Pancreatitis. I had every symptom of AIP with exocrine pancreatic insufficiency, with the exception of weight loss. But my faecal elastase, serum amylase, and IgG antibodies (defining marker of AIP) were all smack bang in normal range. Or it seems like I'm coming up with answers for everything e.g. my pulse oxygen sats are almost always 97%+ no matter how my lungs are, but this is irrelevant as a marker of my clinical status (and has been deemed such by various doctors) as I compensate well. Or my FEV1 is in the normal range (80-90% predicted)..but I'm a lifelong classically-trained singer and ice hockey player used to blowing 120% predicted, and my peak flows are definitely a better indicator as I have been tracking those daily for years so have clear 'zones' as it were.

Agh essay over. If you've read this far then thank you haha!

[cofdrop-UK in reply to Hidden]

Yes I have read it!

I do think you should mention that you make up your own hypertonic and wonder how you guage the percentage and if you use a bronchodilator prior to nubulising it. They should know so they can get a fuller picture of your condition, but you already know that.

Hope all goes well with your consulation.

Cx