In memory of Christen
June 17, 2017
Christen White Cranford (1979-2002)
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- again, and again.
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. We are reeling from the devastating news.
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 admitted to the ICU and is put 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. Now we are reeling from devastating information about this life-altering treatment.
After a week in the hospital she goes home and later goes back to school, although her doctor tells her not to. But she is determined to finish her final semester and get married in February. It’s unclear whether to us whether going back to school is a bad idea or not. We want her to feel in control of her life when it seems to be spinning out of control. And we’re not getting very much in the way of answers. Although we can't put our finger on why, we’re not confident Christen is getting the best care, but are not sure where to turn.
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 are great and seem to know what they’re doing and we’re finally 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 a high probability of 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. But, of course, people with PH can look okay even when they’re not. 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 we learn more about PH than we’ve ever known, and 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. So 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 tell her I think maybe 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 something very bad is happening, 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 we thought was a routine visit. She arrives at the hospital on Tuesday with her mother. And before you know it she’s admitted to the hospital. I arrive on Thursday and stay with Christen in her room, only leaving to get coffee or something to eat. On Friday morning her doctor tells us that her heart is dangerously enlarged and that there is nothing that can be done. He tells her she has weeks, maybe days to live.
By Saturday, November 2 all of her family is gathered around her, and after she has spent a little time with everyone she looses consciousness. Christen died just after midnight on Sunday, November 3, 2002 – 37 days shy of her 23rd birthday, 782 days after being diagnosed, and God only knows how long after experiencing her first symptoms of pulmonary hypertension.
Christen’s fight is over and she’s at peace.
But we’re not at peace. Our fight goes on. We will not be at peace and will not give up the fight until PH is cured so that nobody ever has to tell a story like this ever again.
Christen’s story shines a bright light on two common realities about PH. The first is the lag between onset of symptoms and definitive diagnosis and treatment. The other is the lack of knowledge among the public and the medical community where are the best centers of care for PH patients.
So our struggle is about more than a cure, although that remains our ultimate and most important goal. Along the way to a cure we must assure that symptoms of PH are recognized early by primary care professionals. And we must assure that newly diagnosed patients can quickly get connected to the very best centers of care and the most knowledgeable clinicians who can initiate the best possible treatment program to extend life and assure a good quality of life.
We need your help to make this a reality. Please be generous.
~ Steve White (Christen's Dad)