Caring for patients with Pulmonary Fibrosis requires not just caring for their lung disease but also the impact that their lung disease has on them overall. With the approval of Esbreit (pirfenidone) and OFEV (nintedanib), we finally have medicines that slow the progression of IPF. However, these drugs are not a cure. Despite our best efforts, most patients with IPF will still die from their lung disease. We are very optimistic that over the next few years we will have more effective medicines to offer our IPF patients. However, today, caring for IPF patients still means that we have to confront the reality that much as we plan for our patients to live with IPF we need to prepare them to die from IPF. This does not mean that we should dwell on end of life matters. It does mean that part of the discussion around IPF and disease progression must involve end of life planning.
I encourage my patients to consider end of life planning early in their disease course so that once they have addressed the important issues they don’t need to focus on those issues. Some patients are reluctant to consider end of life planning as they feel that it represents acceptance of their disease process progressing or they are afraid to discuss matters with their family. Your physician should be comfortable helping initiate the discussion. Key steps in end of life planning are as follows:
1. Every adult IPF patient should have an advanced directive.
This is a straightforward form that may be downloaded from the Internet. It expresses your wishes with regards to the types of interventions you would and would not like if you become critically ill. If you do not have an advanced directive, then your doctors will provide maximum support and life-sustaining measures until told to stop by your power of attorney (see below) or until your health care team reaches a point of futility. The major areas of an advanced directive are listed below.
a. Life support: Would you want to be maintained on a mechanical ventilator?
b. CPR: Would you want your healthcare team to do chest compression and shock your heart if it stops?
c. Artificial nutrition: Would you want to be sustained with a feeding tube in your stomach?
2. Power of Attorney for health care
This is a legal document that assigns a surrogate decision maker or decision makers if you are unable to make decisions about your healthcare.
Think carefully and chose someone who will be able to make decisions the way you would want them to. Some family members may not be able to carry through with a plan to limit interventions at the end of life even if you had so advised them. Most commonly power of attorney is your spouse, parent or grown children. However, it may be any other adult that you chose. If you do not have a power of attorney for healthcare, then each state has formal laws that designate a surrogate. Most states recognize your spouse first then your adult children and then your parents in descending order of priority.
3. A Will or Trust is a legal document that specifies what happens to your possessions and assets.
Every adult should have a basic will. A trust is often a more complex document that is very useful if you have substantial assets or a complex family structure.
Planning ahead does not signify that you are ready to die or that you are somehow pulling back from an aggressive treatment course. Leaving end of life decisions until you are very sick places additional burden on you and your family when you are less well equipped to deal with them.