I really wish I had better news to share. I really believed that things were going to go better. I certainly feared what I actually heard, but didn't think it would go that way.
Her Fontan pressures are too high. That is causing more shunting, which is why her oxygen saturation (sats) are low. This explains the oxygen requirement. And the Lasix (diuretic) requirement.
I tried so hard to not cry when her doctor was telling me everything. But, I couldn't help it. I am very sad, scared and hugely disappointed. I know enough to know this is very bad. And the harder I tried to hold it together, the worse it got. I don't cry often. Very rarely, in fact, which is probably pretty hard to believe.
Prior to her Fontan operation, which was done in July 2010, her pressures measured 18 (20 is considered high). Today, her pressures were 24. So, there was nothing that could be done in the cath lab to help her, which was our hope.
What does this mean?
Well, it's complicated. I know that high pressures with a Fontan is bad. I think the simple answer is that it means her heart will not last as long as it would if her pressures were better. So, a heart transplant would be needed sooner versus later.
I also know that we can't leave things alone. We are going to have to spend some time and energy to try and find out the cause (which doesn't always present itself, meaning that we may never know why). I also know that we need to get with pulmonary to see if her lung disease is a factor, and if anything can be done from that angle.
Here are more questions/issues:
1. Who should oversee this, doctor-wise? Her docs in Richmond? Or her docs here in DC? There are pros and cons either way. But, I foresee hospitalizations in her future (some meds need to be trialed in the hospital to make sure they work for her), and quite frankly, I think I'd rather be an inpatient here than in Richmond. I've been trying to maintain local docs when I could. But that means that her care is still wide spread. At this point, I'm afraid I need to consolidate and focus on receiving care in one place (or in less places than we are currently gracing with our presence). This is not as easy as one might think. The pediatric medical community is small, I've spent years building relationships, and I don't want to hurt any feelings or burn any bridges. All I want is to give my daughter the best chance I can to have the longest, highest quality life I can possibly give her. And spreading her care all over the place is making that more difficult.
2. Does this mean she has to stay on oxygen? I don't know exactly. She tolerates low sats, meaning that she doesn't pass out, or become highly symptomatic (seems to be fine). So, do we take away the oxygen and allow her sats to be low during the day (buying her freedom from the tanks) and give her oxygen when she sleeps? I don't know. At this point we don't really know how low she gets because I don't let her get low. We need to trial her on low to no oxygen while she's monitored so we can see what happens.
3. Could the cause of her high pressures be obstructive sleep apnea? Apparently, if she suffers from this, her CO2 (carbon dioxide) levels could be high, which can raise pressures. She needs a sleep study to rule this out. It can be a wait of weeks to months for date. We have attempted to do a sleep study several times in the past and it has never happened because she either she got sick, or circumstances changed and the study was no longer needed. I do not look forward to this process. At all.
4. What does it mean if she has sleep apnea? Will she need help breathing while she sleeps? I don't know the answer to either question. I suppose we will have to cross that bridge when we get there.
5. What if she doesn't have sleep apnea? What happens then? I think we try meds. Which they have already started to tweak. Are there meds we could add to help? Well, in kids with pulmonary hypertension, they try Sildenafil (Viagra). But, this is not pulmonary hypertension. I don't know exactly what it's called (a dysfunctional Fontan? A Fontan with challenges? I don't know). The Fontan is a completely different circulation than the way your heart and lungs are supposed to work. Apparently Children's Hospital of Philadelphia is conducting a study on kids with Fontans with higher pressures and Sildenafil to see if it has the same affects (possible lowering of pressures) as some kids with pulmonary hypertension. So, this is fairly new I guess. Awesome.
6. How will we know if something works to lower her pressures? A heart cath is the only way to measure pressures. So, we can look forward to more in the future. More awesomeness.
7. What if it isn't sleep apnea, and meds don't work? I don't know.
8. Will her pressures continue to get worse over time if they don't get better with intervention? I don't know. Apparently there can be time periods of better and of worse over time.
9. How does this affect her quality of life? I don't know.
Earlier, when I spoke to her cardiologist, we discussed that it would be nice for me to meet with the pulmonolgist here (which won't happen over the weekend) and they could try some things while she was inpatient. At first, I said, sure. While we are here, let's see what we can find out. But shortly thereafter I spoke with Tom and we decided that we just can't do this now. It's almost Christmas and Harlie has already had one helluva year. I'm not prepared to stay into next week. And we aren't going to be able to answer the big questions now anyway.
So, in the morning, I'm going to have the doc in charge call her cardiologist to make sure that we aren't taking a huge risk if I take her home tomorrow (Saturday) and wait until after the first of the year to meet with pulmonary, and address the issues as an outpatient.
We are heavy hearted right now. I don't want any of this. And I am so tired of having to do so many things I simply don't want to do. We need some recovery time. We need to regroup and find our hope again.
When I went on Facebook to post an update earlier, I saw a picture that my dear friend Ann (who I was in San Diego with in October) shared. It simply said, "we can do hard things." Funny timing. I suppose we can. But I can tell you for certain that we don't want to!
It's late, and I am beat. Thank you for all the love, thoughts, prayers and comments today. I mean it when I say we couldn't survive this life without your unwavering support.