Marie is too young to have a fatal diagnosis. She has Primary Pulmonary Hypertension. This condition is called “Primary” because we have no idea of the cause. Medical lingo has a variety of ways of labeling unknown causes such as “Idiopathic” or “Essential”, but in Marie’s case, it’s “Primary” – as if that label somehow shows some understanding.
Actually, I do understand that this is a devastating diagnosis which tends to affect adult women about three times as often as men. Her family physician sends her to me for puzzling shortness of breath. Marie is a delightful soft spoken woman of Asian descent, born in Guam but moving to the USA many years ago. She has raised three children, been healthy, and helps out in her husband’s business as a bookkeeper. She has a beautiful smile and tends to downplay her symptoms.
A non-smoker, her lung functions are normal, but she has some swelling of her ankles. Her heart tones are normal except for a split second heart sound, a sign of delayed closure of the valve leading from the heart to the lungs – the pulmonic valve. The EKG shows strain in the right ventricle. All this points to possible pulmonary hypertension which is confirmed later by heart catheterization and cardiac ultrasound.
We rule out things like fen-phen or blood clots and begin some drug treatment to try to dilate the vessels going to her lungs. Essentially all the blood returning from the body needs to be pumped out to the lungs where it gives off carbon dioxide and picks up oxygen. It’s normally a low pressure system, but not in Marie’s case.
Over the next few years she begins to fail. Other drugs are tried even a battery powered continuous IV. Oxygen is now needed. Marie somehow arranges an annual trip to Guam, her birthplace where many relatives still reside. Between office visits, she hardly ever calls and we have to reach out to see what’s going on.
“Marie, how are you doing?”
“Oh, just fine. A little limited but I can’t complain.”
I have her come in. There’s no doubt things are worsening and that more drastic measures need to be considered.
“Marie, we need to have a talk about next steps. Your cardiac echo is showing very high pulmonary artery pressure. This is becoming life threatening. The drugs are no longer working well. I’d like to refer you to the University Hospital to consider the possibility of lung transplantation.”
“Oh, doctor. I’d never want anything like that.”
“Why not?”
“Well you see, I’m at peace with God. What will be will be. I don’t expect any miracles. Surgery seems so risky to me. I think I’d rather just go on.”
I ask Marie to bring in her husband Gerald who is hardly ever in the exam room with her. I want to make sure he and the children understand what the situation is. She smiles at him when I bring up lung transplantation. Gerald smiles back at her and doesn’t show frustration, “Look doctor, we’ve already talked about this. She doesn’t want surgery. She knows her time is short, and we’re flying back to Guam next week for a month’s visit.”
After more conversation, I begin to understand that they are further along in accepting her impending death than I am. They are Catholic, having a strong faith along with a kind of fatalism that I don’t often see in my practice. They have accepted her destiny, while I’m still ready to fight on!
I OK the oxygen use on the airline and they have arranged for oxygen in Guam. During that month I receive a postcard basically saying, “Don’t worry, I’m OK.”
But on return to Seattle, Marie is worse. She can no longer walk across the room, has episodes of severe shortness of breath, and is becoming bed-bound. Marie and Gerald agree to a hospice referral. Hospice steps in with their usual grace and provides amazing support to Marie and the family.
From that point on I receive mainly electronic progress reports from the hospice nurses and an occasional call for medications. Small doses of palliative medications are keeping her comfortable.
Then the hospice nurse calls me, “Doctor deMaine, we’re wondering if you could visit Marie at home. She and the family would really like to see you.”
I don’t know what to expect when I leave the office at 6PM to stop by their Bellevue home. The house is in a pleasant well groomed neighborhood. A child shyly answers the door and calls for her Grandpa, Gerald. The scene surprisingly seems festive to me. Food abounds on the large dining room table, music is playing, and kids are running around playing games. The living room has been transformed into a bed room. There in the middle of it all is Marie. Her hospital bed’s head is propped up a bit so she can see. A sleepy smile appears on her face when she sees me.
“Why don’t you get something to eat doctor, you must be tired and hungry.”
I sit with her for awhile, listen to her chest with my stethoscope and hold her hand. The warmth and love surrounding her and her family is palpable. I feel tremendous respect for her emotional strength.
Within a week Marie is gone from this life. She has left a beautiful legacy of love and acceptance of death – a blessing for her family, and me too. I come away with the hope that I can some day face my own death with such equanimity, and that I can have my loved ones close by.