‘I feel very blessed to have the perspectives of caregiver and medical professional in the PH community because I know how much I can push to advocate for my daughter.’

The Liu family was vacationing in the Netherlands in 2012 when their 2-year-old daughter, Esther, had a heart attack. Two days later, she was diagnosed with pulmonary arterial hypertension (PAH), with an unknown cause. Since then, she has received a double lung transplant and travels all over the world with her mother Michelle, a physician and Pulmonary Hypertension Association (PHA) board member, to share their PH journey. Dr. Liu, M.D., M.P.H., FAAOA, shares that story as part of PHA’s fall fundraising campaign.

By Michelle Liu
On the last day of our vacation, Esther was jumping on a trampoline when she suddenly collapsed. I initially thought she was throwing a tantrum, but I realized something was wrong when she wouldn’t get up and wasn’t breathing.

I performed CPR with another bystander until an ambulance could take Esther to urgent care. There, she was transferred to a small, pediatric hospital. Two days later, we found out that Esther’s cardiac arrest was due to idiopathic PAH. Through a cardiac ultrasound, doctors found that Esther’s right heart was the size of her whole chest, leaving no room for her left side. The doctors determined she was in the end stage of PAH, so it wasn’t a fuzzy diagnosis.

We spent the next three months in Holland while Esther received acute care for her PAH. Because she had CPR after her cardiac arrest, she was put into an induced coma and onto life support. We didn’t know what her brain function would be when she woke up.

Despite my medical background, I was uninformed about pulmonary hypertension (PH). When I looked online to find out more information, I discovered the Pulmonary Hypertension Association (PHA).

I relied heavily on PHA as my primary source of information while my family and I navigated the first three months of Esther’s intensive care. Through its resources, PHA gave me a “crash course” on PH that helped me learn more about Esther’s diagnosis. I also found the PHA Facebook support groups, which helped me find support for myself and my family.

Seeking care
After her diagnosis, Esther was transitioned from the pediatric hospital to a hospital where the top PH specialist for Europe practiced. I felt so blessed that Esther could receive care from the specialist. She remained in critical care and begun taking IV Flonan. Though he was due to travel to California at the time Esther was transferred, the specialist rearranged his plans to care for her. His care saved Esther’s life.

While under this specialist’s care, Esther suffered another cardiac arrest. The cardiac arrest lasted 40 minutes. That night, in the middle of the night, the hospital called to let us know of the incident and told us to say our goodbyes. This was the most memorable moment for me in all of Esther’s PH journey. I never thought I would have to say goodbye to my two-year-old child. Let alone at her hospital bedside at 1 a.m.

Miraculously, Esther survived. The doctors told us it was nothing short of a miracle. Esther remained on life support and IV Flonan.

In December 2012, Esther was stable enough to leave the hospital. My husband stayed with Esther in the Netherlands as she recovered. My two sons and I moved back to Italy where I was stationed with the Navy.

Initial treatment

In January 2013, our family moved back to the United States for Esther’s care. During the move, she was taken off IV Flonan, but we started the process to resume that treatment when we returned.

At the time, Flonan wasn’t commonly used in the United States. After discussions with Esther’s specialist, we ended up treating Esther with IV Remodulin. Having a two-year-old receiving IV treatment through a central line was a challenge. Despite combining oral and IV therapies, Esther continued to get worse.

We tried to give Esther as normal a life as possible. She went to day care and preschool but continued to be unstable. She was hospitalized every couple of months due to PH related incidents. Our specialist told us he was not sure there was anything else to do to treat Esther. When I heard that, I asked myself “Is that true?”

Exploring options
I started to explore other options and other medical centers, mainly through asking my connections on Facebook. Once I found a new facility, my family and I started to explore lung transplants.

Within a few weeks, we met our new specialist. He had a formula to determine whether someone was eligible for a transplant. Based on his formula, Ester wasn’t sick enough. We still went through the transplant evaluation process. Three months later and after another hospitalization, Esther was deemed eligible for a lung transplant.

Esther was listed for a double lung transplant in May 2014. In June, we went to PHA 2014 International PH Conference and Scientific Sessions. To do so, Esther had to be removed from the transplant list. She went back on the list after the conference and received her double lung transplant in July. She was on the transplant list for only two months.

Transplant journey
Esther’s recovery from her transplant was rocky, as it is for many transplant recipients. Two days after her transplant, Esther suffered her third cardiac arrest due to a mucus plug. Fortunately, she recovered and was hospitalized only for a month. Over the next year, Esther stayed at home with an au pair as she was immunocompromised.

In September 2014, my family and I wrote a letter to Esther’s donor family through the Gift of Life Foundation. We wanted to thank them for donating and saving Esther’s life. In November 2014, we received a response from the grandmother of the donor. A year later, we connected with the mother and two years later we met the donor family in person. We’ve stayed in touch through Facebook and look forward to connecting with them again Indianapolis during the PHA 2024 conference.

Spreading hope
Since recovering from her double lung transplant, Esther and I have traveled to international and national conferences to speak to physicians and families about her transplant experience.

I became the leader of the PHA Washington, D.C., Pediatric PH Support Group. I became a support group leader so I could advocate for children with PH and their families. The connection that PHA support groups provide to other patients and families is critical.

I feel very blessed to have the perspectives of caregiver and medical professional in the PH community because I know how much I can push to advocate for my daughter. There are so many challenges when it comes to communicating for your health; I believe that medically challenged families become experts at pushing to get the care that they need.

I support PHA because of its resources and quality programs dedicated to advancing PH education, research and clinical care. I’ve met some wonderful PHA leaders who have given me a voice. Sometimes I question whether I should be in the transplant community and transition out of PHA, but I feel like this is where we belong.

Sharing pictures of Esther and bringing her to conferences is a joy because families look at her in amazement. Everyone’s course is different, but seeing someone who has faced the obstacles that they face and be here 10 years later is amazing.

Esther’s story is more than a miracle, it is navigation of a course. I let people know there is a lot they can do with the information they receive. It all comes down to decision-making and exploring available resources.

Because she was so young, Esther doesn’t have many memories of her battle with PH. Now that she’s older, hearing the story of how she handled so much adversity has given her a special spice that has turned her into the person she is today.

I am very proud of her. While I don’t fully understand the anxieties she faces, I try my best to empathize and support her and try to push her to live her best life.

Inspired by Michelle and Esther’s story? Donate to PHA’s fall fundraising campaign. Or share your story with PHA’s Right Heart Blog.