helen-louise (baratron) wrote,

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apparently, I am a freak of nature

Thanks to all who commented on my last post. Visit to the doctor yesterday [0] went extremely well, far better than I could have imagined. I have an actual diagnosis!

One of the reasons I like my GP is that he's ridiculously experienced in all sorts of areas of medicine. He has a "special interest" in psychiatry and knows more about psych meds than any of the psychiatrists I've ever seen, but he's also worked on other things as well. When he does eventually retire completely, it will be impossible for me to ever find someone else that good. He's involved in the training of the new kiddy doctors that come into the practice, but there are certain things that it's hard for a person to teach. He's probably the only doctor I've ever met who is able to totally adjust his way of relating to patients based on his perception of the patient's intelligence and knowledge. I managed to train him out of patronising me years ago, and we have interesting conversations about biochemical modes of action of medicines rather than the "this is how your lungs work" type explanation that I get from everyone else. (Sometimes, even after I explain to them that I have a chemistry degree and tell them everything they were about to tell me. Grrr.)

Anyway, it turns out that I have a really rare form of asthma. h-l is weird - we all knew that already, right? Turns out my lungs are weird too. What I have is called bronchorrhea (with optional extra o in British English), which means extreme overproduction of snot, where "too much" is defined as more than 100 ml per day. I laughed a lot when I read that online and looked at a fifth of my 500 ml bottle of water. That's about the amount I produce from cycling up a steep hill.

Bronchorrhea is a common symptom for 70% of asthmatics but usually only in severe asthma attacks that you're hospitalised for. Generally, the only people who get it on a daily basis have serious lung diseases like emphysema, chronic bronchitis, and basal cell carcinoma. Something that causes damage to the cells of the lungs. I'm sure I don't have lung cancer because I've been snotty like this since I was a baby. So I am just a freak.

There are actually very few papers about bronchorrhea specifically in asthmatics on Google or PubMed. PubMed has 14 links, many of which are too old to be online. At least one of the abstracts even claims that bronchorrhea does not exist in mild asthma, which I found quite hilarious. It is so incredibly rare that it did not exist as a diagnosis on the GP computer system, despite my GP trying all conceivable variants on the name.

The classic paper appears to be Chemical properties of bronchorrhea sputum in bronchial asthma (full text available for free), which points out the differences between saliva, nose snot (or mucoid sputum, if you prefer) and lung snot (bronchorrhea sputum); thus "proving" (if you think their feeble number of samples was enough) that lung snot is not nose snot that's dripped down the back of the throat, nor saliva being mistaken for snot. Ha! Take that, stupid nurse practitioner, who thought I was crazy for feeling like 1/3 of my lungs are filled with fluid during an allergy attack, and eventually decided it must just be nose snot plus saliva. Humph.

Also rather interesting is Chronic Cough With a History of Excessive Sputum Production (also available for free - I *heart* the Chest Journal. Not only are all their recent papers free online, but someone has taken the time to scan in ancient papers - from the 1950s and the like!). This specifically describes how bronchorrhea in asthma can be treated with inhaled β2-agonist bronchodilators with corticosteroids. It probably won't surprise you to learn that those are the regular inhalers given to the usual airways-closing-up type of asthmatic.

This is just an abstract, and not fantastically translated either: A Case Report of Asthma. But it's rather interesting because these doctors concluded that the dose of steroids given for bronchial asthma was insufficient as a therapeutic dose for bronchorrhea. "This case indicates that in the treatment of bronchorrhea, it is necessary to administer appropriate dose of steroids, and that after the sputum is under the control, the possibility of recurrence is extremely small and the prognosis is fine."

So yes, it's not surprising that I now have big notes on my medical records saying STOP TRYING TO REDUCE HER INHALERS, k'thx'bye. Now for some research on my part to find out what other drugs exist that no one's ever thought to give me due to not having heard of the condition. Several not-available-for-free papers (such as this one) include reviews of mucoregulatory agents. "Mucoregulatory agents reduce the volume of airway mucus secretion and appear to be especially effective in hypersecretory states such as bronchorrhea, diffuse panbronchiolitis, and some forms of asthma. Mucoregulatory agents include anti-inflammatory agents (indomethacin, glucocorticosteroids), anticholinergic agents, and some macrolide antibiotics." I already have the glucocorticosteroid [1], but what else is there?

I am also currently intrigued by finding a paper stating that excessive mucus production in asthmatics is linked to goblet cell degranulation, whatever that is (the mast cell page on Wikipedia explains it slightly better, but still $too_much_biological_jargon_error), caused by a list of things that started with smoke and sulphur dioxide. Hmm. Haven't I been going to doctors for years complaining how other people's smoking causes me SEVERE overproduction of snot?

And Neurogenic Switching: A Hypothesis for a Mechanism for Shifting the Site of Inflammation in Allergy and Chemical Sensitivity may well be some sort of Theory of Everything to explain why my body is so utterly broken.

Finally, amusing title of the night must go to: Regulation of secretion from mucous and serous cells in the excised ferret trachea, but yes, I'm easily amused at 3 am.

[0] Tuesday. I started writing this in the early hours of Wednesday morning and am finishing it now.

[1] Mine has the awesome name of S-(fluoromethyl) (6S,8S,9R,10S,11S,13S,14S,16R,17R)-6,9-difluoro-11,17-dihydroxy-10,13,16-trimethyl-3-oxo-6,7,8,11,12,14,15,16-octahydrocyclopenta[α]phenanthrene-17-carbothioate, which actually beats yesterday's drug of the day for the sheer number of chiral centres. I can't believe I've been on fluticasone for several years and not looked its structure up before! Nor can I believe it didn't occur to me that fluticasone probably had fluorine in. It has three fluorines for the win!
Tags: allergies suck, better living with modern pharmacology, h-l is weird, my weird medical stuff

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