Feb 28th is National Rare Disease Awareness Day!
While asthma is not considered a rare disease, my other lung condition is. So, I thought this might be a good time to write a little bit about my “other” condition.
If you follow my blog, it’s asthma, asthma, asthma, and how crazy mine can get. Well, it’s now believed that one of the reasons my asthma exacerbations get so bad, is because of an underlining lung condition that I’ve developed over the years. You see, I not only have severe asthma, which affects primarily the larger airways, but I also have a condition called ” Idiopathic Obliterative Bronchiolitis “, also known as Bronchiolitis Obliterans or “BO” for short, which affects my smaller airways and is probably the main reason why I always have symptoms, even when my asthma is presumably under control.
Classified a rare disease by the National Institutes of Health (NIH), Bronchiolitis Obliterans is a condition where inflammation, fibrosis and scarring in and around the smaller airways causes them to become narrowed, occluded or totally obliterated, hence the name Obliterans. It’s often difficult to pin down the exact cause of BO, though exposure to certain toxins appears to be high on the list.
I don’t really know how I contracted the condition or how long I’ve actually had it. Its only after years of observations by the medical staff at UCSF and my Pulmonologists about how my asthma behaves, along with various tests, that a diagnosis was formed. I was exposed to a lot of insecticide sprays and other harmful chemicals during my teenage years, but that was a long time ago and no coloration thus far has been made. More than likely, it has something do with my immune system, but who knows? This is why the condition is labeled “Idiopathic”.
What makes “BO” so challenging to manage, at least in my case, is that the symptoms are very similar to those of asthma. This makes it difficult to tell which of the two is the driving force behind many of my exacerbations. Over the years Ive learned to distinguish some of the subtle variations in symptoms between the two conditions. The bronchiolitis part of my lung condition is always there in the background, if you will, or chronic in nature and manifests itself primarily in the form of air trapping, frequent bouts of dyspnea (breathlessness) and occasional non-productive cough. My asthma on the other hand, manifests itself mainly as sudden or acute chest tightness and/or wheezing and the inability to exhale, which comes and goes. Much like Asthma /COPD overlap syndrome, when either of my two conditions flares up, it tends to exacerbate or worsen the other… what they refer to in the biz as “Acute on Chronic lung disease”. This is probably why I end up in the hospital so often. Fortunately for me, considering my age, my IBO has been relatively stable these past few years with my PFTs worsening only slightly. My baseline FEV1 is currently hovering around 30% and my FVC about 53%. My O2 sats are normal when I’m not flaring.
The treatment for both diseases is essentially the same, ie bronchodilators and steroids. But while asthma is generally reversible to some degree with proper treatment, constrictive bronchiolitis is not. For now, the best I can do is to try to keep my asthma in check the best I can, so as not to exacerbate the other condition. For me this means, continuing use of asthma biologics, macrolide therapy, steroids and tons of Albuterol.
There’s limited information on the web about “BO” for the general public, so as I learn more about my “Other” condition, I’ll be sure to revisit this topic.
Thank you for sharing your story with us. You have certainly traveled a difficult road. I want to ask you a couple of questions: Do you cook in your kitchen and can cooking smoke trigger an asthmatic event? I have asthmatic friends that won’t cook in their kitchen because cooktop exhaust systems rarely get rid of all the smoke. Let me know how you deal with it.