PCD Adult Service in Cumbria?

[windle]

My son is 23 and was diagnosed with PCD at 13. Last year was his best as he was on Azithromycin 3x a week. As soon as he stopped he is having a dificult time since 30th of december: 4 different antibiotics amoxycilin, Doxycicline, and after founding pseudomonas in sputum took Ciprofloxacin and afterwards Ceftazidime IV, for 2 weeks, which he had to wait about 10 days to be organized.

Even during the IV his sputum started to change colour and taste, he his poorly again, but the result of sputum was negative due to the antibiotic. Had to repeat the sputum test and all takes time and worry, as his condition is not improving in the meanwhile

Great difficulty in response from the consultant in Carlisle. Does anyone know the best way to have a quicker response for this situation in this area of the Country?

[toothfairy44]

Hi, we live in Kendal, our son Josh has Situs inversus & PCD he is now eight.

Josh was prem and when his lungs collapsed at three days old a chest x-ray revealed situs inversus. We were really lucky that our paediatric consultant in Lancaster was aware of sitis inversus and the link with PCD, so from three days old he was treated as if he had PCD with AB & physio. Nasal brushing at Leicester later confirmed that he has totally imotile cillia. We still have the same consultant and were also under the management of the North of England PCD specialist Team based in Leeds ( we meet them at Alder Hey at leas once a year)

Unfortunately I am a aware that adult service are far and few between so it may be worth contacting the PCD support group direct for up to date info on adult services as they have been battling to gain an equal care pathway for all age groups.

[windle in reply to toothfairy44]

Hi, thank you very much for your advise and sharing your experience!

We confirmed that he had PCD in Leicester as well. During these last 4 years, since we moved to Penrith, we had 3 different consultants in Carlisle so far and these constant changes makes one feel a bit anxious, specially, when my son is in a crisis and needs a quick intervention/response from the specialist. GPs and nurses have been helpfull but this is a rare situation for them.

Hope your son is well!

[toothfairy44 in reply to windle]

Josh is well at the moment thank you, we continue with Azithromycin 3 x weekly until the spring, and are advised monthly following sputum sampling if there are additional Ab's required. We have daily nebuliser, acapella and physio.

We are just grateful that his condition was suspected so young as the condition seems more difficult to manage with later diagnosis. You must have fought for years for answers as PCD sufferers are often just classed as sickly children...always got a runny nose!

The PCD days are always really useful, the main one is in Milton Keynes 1st July 2017.

Have you spoken to any of the consultants to put you in touch with specialist respiratory and physio services at the RVI in Newcastle. I am aware that they have CF clinics, as PCD parents we are fully aware it is not the same condition but the services are often linked as we have found at Alder Hey.

I work in Penrith and unfortunately the Cumberland Infirmary is suffering alongside a lot of hospital with staffing and funding issues.

[windle in reply to toothfairy44]

HI,

So grateful for the support.

Pity we live so far from these centers.

Has been a long fight, thankfully his doctors even without a diagnosis treated the situation well, thats why he has only mild bronchiectasis. Avoiding further damage of the lungs as much as we can is the main goal.

We try to avoid food that contributes for inflamation such as gluten , milk , sugar and red meat as much as we can and increase the consumption of vegetables and fruit . We do as well suplements of vitamins, all to help the body fight the infections.

Thanks again for all the information. It all helps.

[fionac66]

Hi Sorry to hear that your son isn't very well at the moment. We are in the process of trying to set up a proper funding adult service to help improve adult care. In the meantime I would try and get him a referral to one of our PCD centres, either at Leeds (Dr. Daniel Peckham), Leicester (Dr. Simon Range), the Royal Brompton (Dr. Michael Loebinger) or Southampton (Dr. Mary Carroll).

Has he been given nebulised antibiotics for the pseudomonas? I would at least ask about that.

Alternatively if you are not able to travel to one of these sites - you could ask the consultant in your local hospital to contact the Brompton for advice ... .Dr. Loebinger or Dr. Rob Wilson will both be happy to offer advise about patient treatment.

Let us know how you get on. Happy to answer direct questions if you want to email me [e-mail]

Hope to see you at PCD Day at some point too!

Fiona

[windle in reply to fionac66]

Hi Fiona,

Thank you very much for your help, so gratefull for having support.

Yesterday received confirmation of pseudomonas again, restarted Ciprofloxacine tablets. He never had nebulised antibiotics, we will ask about that.

I will keep in contact.

[fionac66]

Normal treatment is 6 months to a year of nebulised colomycin for Psuedomonas so definitely worth asking. Good luck!

[windle in reply to fionac66]

We are concerned about the nebulizer because when he was

younger he used without antibiotic and it used to increase the production of mucus and made him feel

worse, that's why he was hoping to solve the problem without it.

But he has been another week on Ciprofloxacine (750mg 2x day) with little improvement, and feeling so exausted physically and mentally. He has been unable to go to college for a long time.

We will contact the consultant on monday and ask about the nebulised antibiotic which I suppose can be done at home?

Thank you so much for the precious information and support.

[fionac66 in reply to windle]

Definitely worth asking about. Good luck and keep me posted x