The Hard Road - Part II

It was a lot to take in on October 31, 2012, barely five weeks home from China. 

We had our follow-up appointment with our cardiologist to check on the occluder and talk about how Joy was feeling post-cath.

We had some questions about the medicine she was put on to combat her pulmonary hypertension and the 24/7 oxygen that we were learning to manage. Would we increase? How necessary was the oxygen? Was any of it helping? Because giving medicine every six hours around the clock and keeping an active six year old tethered to oxygen is rugged.

During the appointment, Joy had an echo (ultrasound of the heart) showing elevated pressures in her heart which were unexplained. Basically, pre-cath, Joy's pressures were equal. Yours and mine have about a 30% differential. After the occluder was put in, Joy's pressures went down to about 65% which was a big improvement. The plan was to try to medically manage the residual pressure and hope to lower it with oxygen and medications. Because 65% is still in the moderate to serious range of pulmonary hypertension, we were willing to do anything in the short term that would give Joy a better chance to have a symptom free life. 

The echo on October 31 showed very high pressures perhaps even higher than pre-cath, which if left alone would cause right ventricular heart failure in a very short amount of time. The doctor was very concerned. 

"So, what you are saying is that by plugging her PDA, or closing the hole in her heart, we made it worse?" 

Based on all the measurements in the cath lab before and after they put in the occluder, the spike in pressure was counter to all the data and statistics that pointed to this procedure as a successful way to close a PDA. 

It shouldn't have happened. But it did. And our cardiologist threw himself under the bus a bit because no doctor likes to make their patient worse. 

Once we heard about the echo, we had to talk about what a lifetime of pulmonary vascular resistance (PH) was going to mean for Joy. 

What the hard road might look like. 

I think we listened to the rest but we didn't really "hear" what was said. I pushed the panic down and tried to focus on the details and ask intelligent questions. 

Because the alternative was.....to cry. Hard.

A lifetime of medicine. No pregnancies. Quality of life? Shortened life span.

Being told your child's life would be cut short by heart disease is surreal, especially when you have a daughter who is active, vibrant, and seemingly as healthy as anyone else. Honestly, except for a slight pigeon chest and breathing that is a bit faster than normal, you would never know how "sick" she is. 

In order to deal with the information overload, we needed a plan of action. Because having a plan and moving forward always helps in these situations. 

Our cardiologist was going to call the PH doctors in Boston to set up an appointment as soon as possible. We have the advantage of a cardiologist who trained in Boston and when he calls Boston, he is talking to people who are personal friends and colleagues with whom he worked for many years. We know we are getting the best! 

We left the office in a bit of shock, not quite believing that this precious happy girl in the back seat was destined to suffer with PH for the rest of her life.

Part 3: In which I talk to more doctors from my home phone than I have ever spoken to in my lifetime!