Living With Emphysema or Chronic Bronchitis (COPD)? What Happens Next?

I'm living with emphysema.  What should I do now?  That's what a reader recently found out:
I recently found out that I have emphysema. I have never smoked, not one single time, unless you count second-hand smoke. I don't do drugs.  I apparently have had this for decades (I'm 57 now). I read some paper that suggests doing a big work-up on nonsmokers with emphysema.  You must deal with this situation every day. Here's my question. Under what conditions is it worth doing a work up to find out the cause of this? I have insurance but I am still cost sensitive, and if it isn't going to affect my treatment, does it really matter?
I am scheduled to see a pulmonologist next month and would like to be prepared to make decisions he's going to ask me to make.
Thanks for your question.  This is not advice for you but my opinions on this scenario.  Obviously, if you've never smoked, emphysema is probably the last thing you would have ever expected.  Smoking is the most common cause of emphysema.  Smokers should expect to live many years with emphysema.  

Emphysema is a chronic lung disease in which the lung tissue dies away, making it unable to exchange the gases necessary for life.  You breath in oxygen and breath out carbon dioxide.  This exchange occurs on a microscopic level at the interaction of your capillary blood vessels and the alveoli air sacs.  When you're living with emphysema, these alveoli air sacs disappear, the lining of the sacs thin out and they cease to function normally. This makes it hard for oxygen to get into the capillaries and carbon dioxide to get out of the blood and into your lung tissue for you to exhale.

That's the layman explanation for emphysema.  Now, you're saying "I have emphysema.  What should I do now?"  I'm curious as to how that diagnosis was made.  I was always taught that emphysema  is a tissue diagnosis based on a biopsy result.  Now obviously,  that isn't how we diagnose it clinically.  Nobody gets a lung biopsy to diagnose emphysema. 

Here's usually what happens.  Someone comes in short of breath.  They've smoked all their life and have always felt fine.  Their body gets used to the daily abuse of tobacco.  Their body compensates little by little every year.  The patient compensates little by little by doing less and less every year.  That's the classic clinical scenario of living with emphysema.  

They swear they've tried everything to quit smoking for good, from the nicotine patches to the free Chantix they got from their  family medicine physician.   Nothing worked.  Then they get their first hospital admission.  They get a chest xray that shows some classic changes of emphysema/chronic bronchitis  It shows  hyper expanded lung fields, a barrel chested appearance, flattened diaphragms and  large emphysematous blebs .  They get started on routine therapies for acute COPD exacerbation, spend a couple days in the hospital being taken care of by a hospitalist and then get sent out on antibiotics, a steroid taper and an assortment of inhalers or nebulizers.

Now, as an outpatient, for someone without acute decompensated COPD, making the diagnosis of emphysema almost certainly happened because someone got a chest x ray that looked like emphysema.  (The terms COPD and emphysema are often used interchangeably.  Chronic bronchitis and emphysema together make up the disease spectrum known as chronic obstructive pulmonary disease (COPD).  Chronic bronchitis  occur in  the large airways down to the bronchioles  and parenchyma while emphysema is usually reserved for destruction of the distal lung parenchyma itself.) 

So the question first is, how was your  diagnosis of living with emphysema  made?  Were you short of breath?  Did you have dyspnea on exertion?   Did you have any complaint at all?   I suspect it's because someone got a chest x ray based on something you said.  I am assuming your diagnosis of emphysema is real. 

There are several types of emphysema.  And which type you have depends on what your x ray looks like.  Obviously, smoking is the biggest cause by far of  living with emphysema.  Since you aren't a smoker, what are the other causes that can help explain why you have emphysema?  Chronic air pollution exposure can cause emphysema as well.  Chronic exposure to grain dust (farmers)  and other air born irritants can do it as well.  I'm sure there are a number of occupational hazards and exposures that can lead to  a life with COPD.  Exposure to other inhalants such as silicon dust or asbestos cause their own lung problems that  have their own natural history and progression.   

For someone who's never smoked, testing for the genetic condition alpha-1- antitrypsin deficiency should be done.  A deficiency of this enzyme allows other proteins to destroy the tissues of the lung and liver  leading to emphysema and liver cirrhosis.  If you have this condition, treatment with enzyme replacement can help.  

What are all the possible studies that can be done in someone living with emphysema?

1)  Blood work.
  • Arterial blood gas helps to clarify the amount carbon dioxide trapping
  • Alpha one antitrypsin level to screen for this genetic condition.
  • Both of these are reasonable, although one can get a generally good idea of how much air trapping is going on by looking at a basic metabolic panel (BMP) and seeing what the bicarbonate level is.  An ABG can be uncomfortable (it's an arterial blood draw out of the wrist), while a venous draw rarely is painful, unless it results in a hematoma.
 2)  Chest x ray
  • I assume one has already been done if you are getting referred to a pulmonologist for emphysema.  If you haven't had one yet and you are getting referred by your primary care doctor to a pulmonologist for emphysema, find a new primary care doctor.  Yours really sucks.
3)  Pulmonary Function Tests (PFTs)
  • There are different degrees of this test that can give valuable information about how much air trapping is going on and how well your lungs respond to inhaler therapy.  Often times an ABG is ordered if this study is going to be done.  You sit inside a giant glass box and blow into a tube.  A less in depth (and much cheaper) version called spirometry can be done (and often is in a primary care doctor's office).   But it can't tell you anything about how much air you're actually trapping in your lungs or  give you any information about how big your lung volumes are.   While PFTs help to stage the degree of emphysema and help the doctor screen for  how well a patient will respond to bronchodilator therapy (the mechanism of action for Spiriva), what I see clinically is that it doesn't really matter.  Because medications are prescribed based on the degree of symptoms, rather than the level of pathology.  Whether the patient has early or late emphysema, the patients I see as a hospitalist, whether their PFTs show a reversible component or not with medications, always seem to get the same medication therapy.  One could argue that staging the disease process offers no advantage in treatment except to offer the patient statistical prognosis.  If you don't want to know how bad they are, don't get PFTs.  PFTs can also clarify the diagnosis by suggesting a restrictive instead of an obstructive  lung disease pattern.  But I am assuming you have emphysema, and the diagnosis is not in question.
4)  CT scan
  • Sometimes doctors order a CT scan, or even a high resolution CT (HRCT) in patients with emphysema.  Some CT scans can give you enough radiation as 30 years of living with background radiation.  Given what we know these days about CT scan radiation exposure, I'm not sure this test should be routinely done in a new diagnosis of COPD.  I just don't see how it will change management, enough to warrant the expense and cancer risk down the road.     If the doctor ordering the CT scan also owns the CT scan, an inherent financial conflict of interest can always cloud a physicians judgment, no matter how unbiased a physician thinks they are being.   We already know that insurance status affects physician self referral patterns for the procedures they employ.  Ask them if they own the CT scanner you'll be getting your CT in.  If the answer is yes, you'll always wonder if you really needed that CT scan.  This is one reason why I am a strong believer in bundled care.  It would really tell us how much we do as doctors is really necessary.
5)   Cardiac Echo.
  • While this study offers nothing in terms of pulmonary function, it can help quantify the degree of pulmonary hypertension that results from the loss of lung tissue.  As lung tissue dies, the small capillary blood vessels die too.  If you're trying to pump the same volume of blood through a smaller network of blood vessels in the lung, the back pressure is going to rise.  This is the right sided pressure of the heart and it can be measured indirectly on a cardiac echo.  Will it change management at all?  No.  Except to say that anyone with shortness of breath will likely end up with a cardiac echo (to look at the wall motion and evaluation for evidence of valvular stenosis or regurgitation) and maybe even a cardiac stress test at some point in the near future after that symptom is described to a physician. These things have nothing to do with emphysema but are part of the basket of tests that are  routinely done in the evaluation of "shortness of breath".  Would I do an echo in someone with shortness of breath?  Yes, I would, if I didn't have a baseline echo in my system.  Would I do one in the evaluation of someone with emphysema?  No.  Because nothing would change management wise.  It would confirm pulmonary hypertension.  Great.  So what.
6)  Surgery
  • There are surgical lung resection options for COPD.  I have no experience with this and can't give you any insight into when it's indicated and when it isn't.  
7)  Oxygen needs determination.
  • I think this is self explanatory.  They will check your oxygen needs, probably at rest and with activity.
8)  Pulmonary Rehabilitation
  • Thinking back to my residency days I was taught that the only two things that are known to prolong life in a patient with COPD is oxygen and pulmonary rehab.  If they recommend pulmonary rehabilitation, do it.
 What are you going to get treated with?  You'll get bronchodilators.  You might get the cheap ones (albuterol, atrovent) or a combination  duoneb or combivent.  You might get a long acting bronchodilator with a steroid component (Advair or Symbicort).  You might get a long acting atrovent (Spiriva).    That's probably where you'll start.  Other drugs can be offered but I doubt they will be first line, at least not from what I see in hard core COPDers that get admitted.

What happens next?  You'll probably get all the tests they recommend, get started on the above  medications with a  "follow up chest x ray and PFTs" once a year, with appointments to see  the physician assistant or nurse practitioner in clinic every three months for the next 20 years, more often if you have really good insurance.

That's just the way it is.

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