Background/Purpose: Relapsing polychondritis (RP) is an inflammatory condition with a variable phenotype thought to be due to autoimmunity towards cartilage, particularly, that found in the airway.  It can be challenging to diagnose and lead to symptoms that can have a profound effect on quality of life (QOL) and is one of the leading causes of morbidity and mortality for this condition.  I was diagnosed with RP after over a decade with “challenging asthma.”  In addition to progressive diffuse airway malacia (figure 1), I had auricular chondritis, migratory polyarthritis, episcleritis, and sicca syndrome.  After an extended period of steroids and multiple medications, partial remission was achieved with low dose prednisone, IV tocilizumab, methotrexate, and hydroxychloroquine.  Despite adequate immune control, the residual airway damage resulted in nearly constant air hunger, dyspnea, and cough.  After 1 year, I was barely able to walk, talk, or work requiring intermittent positive airway pressure (PAP) and cough suppressants during the day.

Intervention:

Once on immunotherapy, the persistent air hunger led to poor mental health and fear.  As a physician involved in the care of very complex diseases, I work alongside palliative care (PC) teams, and I thought they might be able to help.  Against the advice of my primary doctor, I sought consultation with my local PC program.  After assessment, we worked to establish goals and a plan.; specifically, time free from air hunger, reduced cough, and to be able to continue working. 

Maintenance:

Unfortunately, things were more complicated than I was prepared for which led to the first and most important intervention:  acknowledging the severity of my illness.  In addition to the airway symptoms, I was obese with low muscle mass from steroids and the situation was untenable.  Working was unrealistic, especially in my chosen career, and continuing likely increased risk of complications and death.  I was totally disabled.  Step one, I had to quit working.  Next, though I was skeptical, I start extended release morphine for air hunger.  Despite my skepticism, this proved to be highly effective and I was able to breathe comfortably resulting in an immediate and significant improvement in my QOL. 

Quality of Life:

After an adjustment period, I began trauma and grief counseling and refocused on my QOL with the goal to get back to work.  I began moving some every day.  This wasn’t simple or easy requiring mobility aids and portable PAP (See figure 2).  Over time, I was able to take walks and travel.  After 2+ years, I lost 125 lbs and increased my strength (See figure 3).  After 4 years, I was finally able to go back to work.  I have kept off the weight and have strategies in place to focus on living day to day.  Without PC’s unique expertise directing focus toward goals and QOL, I would be more likely to be a statistic than writing this abstract.

Takeaway:

In conclusion, airway involvement from RP leads to symptoms of severe cough, dyspnea, and air hunger compounded by the debilitating effects of steroids.  These symptoms are complex and challenging and can persist despite remission of the disease.  PC provides unique expertise in the treatment of respiratory symptoms and poor QOL commonly found in advanced stages of disease.  Though traditionally associated with the end of life, when a PC team is utilized to focus on a patient’s symptom control and QOL, it can help to optimize rehabilitation and maintenance of overall health thereby improving outcomes for patients with advanced airway involvement from RP.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-role-of-a-palliative-care-support-team-to-optimize-outcomes-for-patients-with-airway-involvement-from-relapsing-polychondritis/