Pulmonary Hypertension

Gina is 43. She's been with us for around eight weeks. Gina can hardly go to the bathroom or eat without seriously desaturating-- like from a normal oxygen sat of 95% right down to 68%, in just a few seconds. Recovery is slow each time, made up of many quiet slow deep breaths from the nonrebreather mask that gives her as close to 100% oxygen as we can. These days, when she's going to engage in any activity at all, we just crank her on up to 100%. She desats anyway.

She spent her first month with us on Flolan, a powerful drug given IV that relaxes the pulmonary blood vessels and affects blood clotting to help improve blood flow in the lungs. Not only does this help with oxygenation, but it reduces the burden on the right side of her heart, which must pump against all that resistance day in and day out. The drug has a very short half-life: just a few minutes. The infusion must not ever be interrupted. We must always have a back up pump and tubing ready to go, as well as a second (or third) IV access in case her peripheral falls out or her newly inserted PICC line decides to explode spontaneously. If her Flolan pump alarms, everyone comes running. Naturally, Gina proves to be a nervous type. Who wouldn't?

We titrate her dose up slowly and steadily, horrific side effects our only limitation. It is difficult to tell whether she's getting much benefit from the Flolan: her oxygen requirements are still super high, and her severe shortness of breath with any activity almost completely disabling. However, presuming that she would be worse off without the drug, and with very few things to offer, the decision is made to transition her to Remodulin. This is a drug that works very similarly, but is given subcutaneously via a tiny needle, much like insulin pumps some diabetics use. It has a longer half life (measured in hours instead of minutes) which means it is a lot safer, especially for home use. There is less risk of infection than with a central IV, and less risk of death from pump malfunction or operator error.

Home use is what Gina is looking at, if we can get her oxygen requirement down to a more reasonable level. Really, what she needs is a new set of lungs.

One morning, I am sitting by her bed after one of our frequent pump checks (she does most of it while we RNs watch, because she needs to learn to manage it all by herself). We’re looking at a brochure the transplant coordinator has brought by. It talks all about life before, during, and after in excruciating detail.

Right now, she’s stuck in the before-before zone. She’s three hours from home because we’re the only place around for this therapy. Sometimes, we talk about her kids. She hates that she is going to miss her daughter’s first day of school. She worries about them even more when her husband is here and they're staying with relatives, but she feels so much better when he sleeps on the fold-out chair next to her.

Gina is in limbo. Will she get new lungs? When will they show up? Will she be ready? What will the surgery be like? Will her body accept these foreigners? Will she ever be able to live without a mask on her face? Will she ever make it home again? She tells me she was never claustrophobic before, but now, with the mask always covering her, little things like a closed curtain can be too much to take.

She's "stable" on the Remodulin now, though she has seen minimal functional improvement. It is time for her to leave the ICU and go to a floor. We just can't justify her staying with us any longer. At this point for Gina, it's mostly about the waiting, and small tweaks to therapy that may help a little here and there.

It’s time for her to face another unknown, the first in a long line. As she’s wheeling away in her new bed, I dash across the unit for a quick hug. Then, she’s gone.