What to do when your concerns are “unremarkable”

[paul-met-debbie]

Well in a way it is good news that the xray is unremarkable. That means there is no excess fluid in the area (or not much). Still, it could very well be pleurisy because without effusion it can’t be detected with an xray. Think about this: a bruised rib doesn’t show up on an xray (sometimes not even a fractured rib) and boy, does it Hurt!

If you have a stethoscope you can hear the pleura rub against eachother at the end of an inhalation and start of exhalation. Any GP could hear that for you, you don’t need a lung specialist for that.

Many pains are not too be made visual even with modern imaging techniques and an xray isn’t even modern. So an answer about the cause of pains is often outside the realm of modern medicine.

I would go on trying to diminish the inflammation. I have been fighting inflammation in my airways and joints/ligaments for a long time and for me, these things work best:
– anti inflammatory drugs, like nsaids. I found continuous low dose work better than incidental normal dose. And diclofenac works best of all, and longer (up to 12 hours) so it is easiest to dose continually. Check with your doctor about using omeprazole to protect the stomach.
– anti inflammatory diet. Mediterranean style works best for me. Check “Foods that fight inflammation” on Harvard Health Publishing. It is at least equally important to avoid foods that trigger inflammation. Sure before trikafta you also ate, but things might have changed that made your body more sensitive and prone to inflammation. And even without trikafta your body would change all the time too, as do environmental influences.
– protect your airways against cold and dehydration. Make sure that inside the house air humidity is 50-60% at room temp. In winter most heated houses are much too dry. Outside in these days when temps are below 10 C / 50 F, the air is almost always too dry even at 90% humidity because air of that low temp cannot hold enough water. So cover your mouth and nose with a double layer woolen shawl and breath into a pocket of fabric, so that your inhaled air passes through that warm and moist pocket and warms up before it enters your airways. Perhaps a covid mask under the shawl would work fine as well. Don’t go out in the cold for prolonged times even with this protection. Better to do several short activities outside (up to 30 minutes) than one long one. If on a long car ride, use aircon as little as possible.

I think it is not strange to assume that on Trikafta, airways become more sensitive to outside triggers of inflammation like cold/dehydration and viruses (hence the increase of sinusitis, common cold and flu as mentioned in the leaflet) because the thick layer of cf mucus acted as sort of an insulation/protection that is now diminished or gone, leaving the airways much more exposed. They still have CF of course and are over-reactive, and they are not (yet) used to being less protected and may react with rage (inflammation). In response we should protect our airways better ourselves. Just a theory of me.

Good luck hope this helps.

[jenny-livingston]

Thanks for this, Paul. Something you mentioned here that I could definitely work on is diet. ‘Tis the season of holiday cooking and endless sugary treats at my house, and I know that sugar and inflammation go hand in hand.

You make a very interesting point about Trikafta and the loss of that layer of “protective” mucus. I truly believe this is the reason for my severe allergies this year — allergies that I never had before Trikafta! Your theory makes so much sense, and even with my allergy experience, I hadn’t applied that theory to other environmental irritants.

You’ve certainly given me some things to think about!

[tim-blowfield]

Your experience is unremarkable. Pleurisy as Paul says is often not visible on XRay. With CF pleurisy is not unexpected.
Unfortunately CF’ers often have symptoms and problems dismissed as such, not just chest pain but many of the comorbidities that go with CF. My wife who has persisted with CF now for 78 years often experiences such dismissive attitudes – often put in the ‘too hard basket’. She would not have been diagnosed as having CF if we had rolled over and just accepted what some Doctors said. Still fighting to getting her Cardiomyopathy treated, her nasal problems treated, what is the best way to treat her hyperparathyroidism and so on. We find the extreme fragmentation of medical services a serious threat. Specialists often have no understanding beyond their narrow specialty (and often don’t want to think outside their box).
CF Physicians need to be good general physicians – Yes with expert understanding of the lungs – but much more as every cell in the body is affected by the faulty CFTR gene.

[paul-met-debbie]

Tim, you’re absolutely right. CF should ideally not be treated by a pulmonologist, but by a dedicated CF-specialist. Like children are treated by a cf-specialised pediatrician. Unfortunately, these do not exist for adults and as soon as we reach the age of 18, fragmentation takes hold of us. At least then current specialties should work together as a real team, not just as the “cf-teams” we know today that are only pretending to be one system, but when the questions arising exceed the box of the pulmonologist, the patient is shipped off to the other Kingdoms where he has to start all over again (including the waiting list.) Many times to find no answers either because the other specialist doesn’t know enough about cf to be of any real service and doesn’t really want to get into the subject at all, thinking that he knows it all and cf is only of minor relevance.

Regarding heart problems and cf I recently stumbled upon a research paper called “Heart involvement in cystic fibrosis” by Fabien Labombarda et al, a group of French researchers. You probably know it already, but I mention it just to be sure. What I sometimes do in these cases is find out the e-mail adres of such researchers (and the ones mentioned in footnotes) and start up a conversation with them myself to get an answer to some questions that my doctor doesn’t have. In many cases there is no answer, but sometimes I was pleasantly surprised and got a lot out of it.

[tim-blowfield]

Subclinical Left Ventricular Dysfunction is Influenced by Genotype Severity in Patients with Cystic Fibrosis<
Michael Pallin, Dominic Keating, David M Kaye, Tom Kotsimbos,1 and John W Wilson
CF Unit, Alfred Hospital, Melbourne, VIC Australia

Clin Med Insights Circ Respir Pulm Med. 2018;.
Published online 2018 Aug 19.

Abstract
Background and objective:
Over 2000 genotypes in the cystic fibrosis (CF) gene have been described. These genotypic differences result in variable clinical manifestations of CF, with severity of disease dependent on CF transmembrane conductance (CFTR) protein function. CFTR is widely distributed in nucleated cells, including cardiac myocytes, but the effect of genotype on cardiac function is not known.

Methods:
This retrospective review of echocardiographic data is from a single adult CF centre between 2000 and 2015. Patients were cohorted based on the functional classification of genotype. ‘Severe’ patients had both CF genes from functional classification groups 1-3; ‘mild’ patients had one or no gene from these groups, or in the event of the second gene being unknown were pancreatic sufficient.

Results:
Genotype and echocardiography were recorded during the inclusion period in 100 patients, 79 of whom were classified as having severe genotypes. Although the severe group were younger they had a lower fractional shortening (33.66 ± 6.6 vs 36.9 ± 6.3, P < .05), left atrial area (14.9 ± 3.6 versus 18.0 ± 4.2 cm2; P < .01) and volume (39.9 ± 18.7 versus 51.0 ± 18.7 mL; P < .05) and showed a trend to lower left ventricular ejection fraction.

Conclusions:
This study is the first to show that in CF, severity of genotype (functional classification) is associated with cardiac impairment. Patients with severe CF genotype and cardiac dysfunction should be identified to evaluate cardiac response to gene-modifying treatments prior to consideration for lung transplantation.