A Joint Perspective on End-of-Life Care in Pediatric Pulmonary Hypertension

Rebecca Kameny, Steve Van Wormer, Nidhy P. Varghese

https://doi.org/10.1002/pul2.70181 

Although there are defined outpatient and inpatient spaces, when end of life occurs in the intensive care unit (ICU) setting, there is an opportunity for collaboration for chronic and acute medical teams to work for a “good death.”

In this commentary, we share our perspectives on collaborating for pulmonary hypertension patients as they approach end-of-life.

1 Steve Van Wormer
Pediatric PH Parent, Co-Founder of Phaware Global Association

PH is a disease that affects not only the patient but the entire family. The emotional and mental burden of caring for a pediatric PH patient is hard to capture. It begins with a nagging sense that something isn't right with your child, followed by the shock of a rare, chronic diagnosis. From there, caregivers walk a tightrope as they navigate the complexities of school, insurance, escalating therapies, and the inevitable progression of symptoms.

2 Nidhy Varghese, MD
Pediatric Pulmonologist and PH Physician, Texas Children's Hospital, Baylor College of Medicine

As an outpatient physician managing chronic illness, my role extends through the patient's journey. This means celebrating victories and offering support during struggles. Our relationships are built on trust, familiarity, and the shared goal of beneficence. However, this bond is not confined to the outpatient clinic.

For rare and complex diseases like PH, outpatient physicians are uniquely positioned to provide continuity of care, and this is particularly true during hospitalization. These settings are often unfamiliar to patients, who may face complex decisions and unsettling independence. The outpatient physician/provider can offer clinical context to aid in this medical decision-making. When the outpatient team trusts and collaborates with the inpatient team, patients and caregivers feel reassured, minimizing the vulnerability that may accompany an ICU stay.

While outpatient physicians/providers may feel intimidated by the high-stakes environment of the ICU, they should never hesitate to do a “social visit” and support their patients or a “continuity visit” and support their inpatient colleagues. As pediatricians, our role goes beyond providing medical care—we are there to support, encourage, and guide families through some of the most challenging moments of their lives. To work with the inpatient team for the patient's comfort through death, to support the family through the transition, and to truly be present for one of my chronic patients is both my profound privilege and humbling responsibility.

Read the full letter to the editor