My most recent posts stress the importance of understanding ‘the big picture’. For patients with a chronic, progressive disease, a critical component of this ‘big picture’  is an understanding of the natural history of that condition.

but unknowingly, providers don’t always explicitly share that information.  

Mr X, 65 yrs old, had been dealing with progressive interstitial lung disease for several years.  His last hospitalization was 6mo ago and ever since, he has required supplemental oxygen 24hr/day.   Over the past few months, his health has continued to decline.  And now, Mr X is again hospitalized for an acute exacerbation.  Despite aggressive treatments, including the shift to oxygen delivery by high flow nasal cannula, he has made little improvement; and his breathing continues to be labored.

His providers worry that he is heading towards requiring intubation and ventilatory support to stay alive.  But because of the severity of his disease at baseline, there is real concern that once intubated, Mr X will never be successfully weaned of the ventilator- i.e., he would remain dependent on a ventilator for the rest of his life.

Palliative medicine was consulted to discuss this with Mr X and his family and see ‘what he wanted to do’.

While talking with Mr X and his family, I asked if he had ever discussed the possibility of being ventilator dependent with any of his providers, be it his pulmonologist or primary care provider or perhaps even a respiratory therapist.  I was quite surprised when his response was a succinct “No”.

Now, I like to think that this possibility had at least been mentioned in the past, because it’s no surprise for a patient with this diagnosis to be faced with this exact situation.  In fact, unless Mr X were to suddenly die from another acute issue (such as an accident or a heart attack or stroke) the natural progression of interstitial lung disease is worsening respiratory function with the possible permanent need for intubation/vent support to stay alive.

But this is not something that can be discussed during one routine visit; this information must be addressed over time.  During my surgical training, we called it ‘laying the crepe’- meaning giving patients information about ‘badness’ that could be in their future.  And it’s not just because patient visit slots are often pathetically short that these discussions don’t happen.  It’s also because it’s far easier- for provider and patient alike- to concentrate on the ‘here and now’ than to talk about the future.  But this has important treatment implications, because the eventuality will likely come at some point, and a discussion about what to do when that time comes is better to explore during a time of stability, rather than during a crisis.

Sadly, the typical discussion about intubation takes place during a crisis, when a patient is struggling to breathe.  Often the patient gets intubated because either-

• it’s such an emergency that the providers go ahead and do it to save the patient’s life,
• or if there’s time during an urgent situation, the patient/family get asked “what do you want done?” and because it’s so chaotic and their loved one is struggling to breathe their default answer is ‘everything!’.

And then, when the patient is unable to be weaned off of the ventilator the discussions start about ‘what to do next’.  Not really a great situation.

Patient autonomy depends on the patient having all appropriate information needed for decision-making. And assuring the patient understands the natural course of their disease is critical.   Although ethically there is little difference between not starting a treatment and withdrawing it when it is not beneficial, the reality is that for patients/families it can be a wrenching decision to withdraw a treatment once started.  For many, not starting a treatment in the first-place results in less suffering (for patient and family, and I would add for providers as well), than to withdraw it.  This is particularly true when someone is nearing end of life.

For patients like Mr X, the discussion needs to include an explanation of the gradual progression of his lung disease and the eventuality of requiring a ventilator to stay alive.  Would a life stuck on a machine, likely in a nursing facility be one worth living?  Thinking about this during a period of relative stability gives the patient time to reflect and talk with family.  Maybe even visit a nursing facility to see what it’s like.

For some patients, the answer is “yes”.  I’ve had patients who felt that as long as they were alive and could interact with their surroundings- that that would be a good life.  They’d want at minimum a trial of this treatment.  And if the burdens of this treatment were too great, then the decision to change to comfort care would be appropriate.

For others, this type of life would be unacceptable.  For these patients, as their symptoms worsen, their providers would know that instead of adding increasingly invasive interventions, it would be time to shift the focus to comfort oriented care, perhaps including hospice services, and allowing them to die free from suffering, with their family by their side.

The truth is that for much of the time when someone has a chronic illness, the treatment goal is ‘to get better’.  But there comes a time when people with advanced disease get to the end of the line- in terms of effective treatments.  For patients with these types of chronic conditions, conversations must take place before crises develop- allowing the patient time to explore their overall treatment goals with themselves and their families.  And it’s up to the provider to broach this topic– because it’s the provider who has this information.  This typically takes a series of discussions, over time, between providers and patients/families, separately and as a team to discuss this eventuality.

– so decisions can be made before the inevitable crisis arrives.