September 12, 2000.  I get a call from the Emergency Room at The University of Tennessee Medical Center in Knoxville where my 20-year-old daughter Christen was taken when she fainted at the UT Library.  It’s a doctor who is telling me that he thinks she might have primary pulmonary hypertension and that this is a very serious situation.  I tell him I’ll be on the next flight down.

Is this related to the shortness of breath she’s been having for a couple of years, I wonder? Her doctor at home told her she should lose some weight and that would probably solve that problem. Lately she’d been having trouble climbing stairs, but still the doctor didn’t seem very concerned. Lose weight, he said.

But now it turns out she might have a fatal disease that seemed to have snuck up on us without warning. I mean, if her doctor wasn’t concerned about the shortness of breath, why should we be worried, right?

When I get to Knoxville, Christen is in Intensive Care. The doctors tell me they have done a right heart catheterization and have confirmed the diagnosis: it’s pulmonary hypertension and they don’t know what caused it.  They tell me to get her to an academic medical center where they can care for her. And they tell me the illness she has is often fatal within two years of diagnosis.

I call my cousin who is a pulmonologist and tell him what’s going on. Take Christen to an academic medical center, he says. She’s at an academic medical center now, I say, and they’re telling me to take her someplace else. My cousin says to take her wherever they suggest because he’s not familiar with the options in the south. The UT doctors suggest two places and we pick the one closest to Christen’s home.

We go there and she is placed on a drug called Flolan – a drug administered by a small pump through a tube in her chest. They scare the hell out of us by saying that if the pump fails or if the tube pulls out, she’s likely to die in minutes. We are reeling from the devastating diagnosis.

After a week in the hospital she goes home and later goes back to school, although her doctor tells her not to. She wants to finish her final semester and get married. It’s unclear whether this is a bad idea or not, and we’re not getting very much in the way of answers.

June, 2001:  After being very unhappy with the follow up care and the lack of information, Christen asks to be taken to the other option we were given in Knoxville, namely The Vanderbilt University Medical Center in Nashville.

It is now about two and a half years since Christen first began experiencing shortness of breath and nine months since her diagnosis, and we still have not regained our balance. We’re still reeling. We’re in denial about how bad this can be. We’re scared and confused.

The doctors and nurses at Vanderbilt seem to know what they’re doing and we’re feeling more confident. Then they tell Christen that her disease is advanced and that she needs a heart-lung transplant. We look into what this is about and learn that while it is the only ultimate solution for pulmonary hypertension, it comes with its own dire consequences, including death during surgery and a poor prognosis after that. She decides to wait. We want to support her as best we can, but have no idea what the best thing is to do or where to find out. This is before we knew of the Pulmonary Hypertension Association.

May 2002:  It’s been almost a year since Christen went to Vanderbilt and she seems to be doing okay – not great, but okay.  She calls to tell me she has a scholarship to attend the Pulmonary Hypertension Association’s Conference in California and asks if I’ll go with her.

In June at the Conference in California it becomes clearer to Christen and me just how sick she really is. I had just seen her in April and now a couple of months later she is clearly doing much worse. I still can’t believe in my heart what my head is telling me: Christen is dying. I get the feeling she knows this, but she doesn’t say it.

Through the summer and into the fall I speak with her almost daily and with her mother weekly. I can tell things are not going well. Her nurse at Vanderbilt says there are no other options other than a transplant. Christen agrees to go on the transplant list, but there are examinations and interviews that have to happen before that can be finalized.

October 19, 2002: Christen flies to New Jersey to visit us and, when I pick her up at the airport she can barely walk. I say this trip was not a good idea and wish I had not agreed to it. She stays a week and spends most of the time sitting on the sofa. Her hands and feet are cold and she aches all over. I begin to suspect that the end is coming, but push the thought out of my mind whenever it intrudes. Christen says don’t worry, Dad. I’m seeing my doctor in Nashville next week and we’ll figure something out.

So off she goes to Nashville for what she thought was a routine visit.  She arrived at the hospital on Tuesday with her mother. She was unexpectedly admitted to the hospital. I arrived on Thursday and stayed with Christen in her room. On Friday morning her doctor told us that her heart was dangerously enlarged and that there was nothing that could be done. He told her she had weeks, maybe days to live.

By Saturday, November 2 all of her family was gathered around her, and after she has spent a little time with everyone she lost consciousness

Christen died just after midnight on Sunday, November 3, 2002 – 782 days after being diagnosed and God only knows how long after experiencing her first symptoms of pulmonary hypertension.