A Rough Few Days

This is going to be a long post as I need to synthesize. Before I get into all of the details, I'll provide Madeleine's status right now: She's stable and resting in the Cardiac ICU at Children's. As the title indicates, she's had a rough few days but we're hopeful that we've gotten through the worst and that Madeleine will continue to recover.

If you've read the last few weeks, you're probably scratching your head after all of the hopeful postings and we're right there with you. Here's what has happened, what we've learned, and the plan for going forward:

This past Wednesday, September 17th, the doctors were preparing to discharge Madeleine from the hospital. She was gaining weight, was quite alert, and we were fairly confident that she was stable and ready to get back to the Bushey casa. The one sticking point was that the doctors were concerned about Madeleine's pulmonary hypertension and questioned whether they should pursue more testing now or later. The argument for testing immediately focused on Madeleine's health stability and theorized that, if Madeleine does have pulmonary hypertension, now was as good a time as ever to test for it. The other side of the coin argued that additional growth would make Madeleine more stable (assuming she can stay healthy out of the hospital). In the end, we agreed with the doctors that it made sense to go forward with a heart catheterization-- the invasive procedure that provides the most reliable data regarding Madeleine's hypertension. We theorized that if we waited for Madeleine to grow, we ran the risk of getting sick again (especially if her hypertension went untreated) and we could gain valuable insight by going into the lab. (NOTE: Despite Madeleine's setbacks, we think this was still the correct decision.)

The decision to go forward with the heart catheter procedure was made on Wednesday and Madeleine was scheduled in the lab on Thursday afternoon. The procedure itself was successful and provided some helpful, if not entirely positive, outcomes. Here's what we learned:

- The catheter confirmed that Madeleine does have persistent/chronic hypertension that requires medical intervention. The level of hypertension isn't major but if there is a scale of 1-3 (with one being low and three being high), her level of hypertension is a high 2.
- Here's the nitty-gritty medical details (feel free to skip over if this confuses you.) Madeleine's right atrial pressure was about 2/3 of her systemic body pressure-- it should be less than 1/4 or 25%. Additionally, pulmonary resistance was gauged at a level 10-- resistance in a normal body is between 1-2.
- In the cath lab, they also try "treating" the hypertension with different drugs to determine possible courses of long-term treatment. One of the most common treatments for acute hypertension is nitric oxide. Madeleine's hypertension was largely unaffected by the nitric oxide. This isn't terrible news but it would be better if she reacted positively to nitric oxide as it would indicate a higher likelihood to being medically manageable. Still, there is a reasonable expectation that her hypertension can be managed medically.
- The first drug that they ordered for Madeleine's hypertension is sildenafil. If you're in the medical profession or know drug names, you'll know that the medical term for sildenafil is Viagra. (Yes, you read that correctly.) Viagra was developed as a hypertension medicine and its highly marketable and publicized side effect was identified in preliminary testing.
- The Cath lab showed some good news: it showed that Madeleine's heart patch is doing very well and there is no noticeable shunting between the left and right sides of the heart. This is good for three reasons: First, it indicates that Madeleine's hypertension should be relatively unaffected by her heart condition. Previous damage may have occurred before the correction but further damage should be minimal. Additionally, there was a concern on the echocardiagram that the readings were less trustworthy as shunting can make the echo less reliable. This means that we don't have to rely on invasive hearth catheter procedures all the time. (though we will have heart caths in the future.) Finally, the heart condition shows that it is less likely that she'll need further heart surgery in the future.

That's about all we leaned in the lab tests. At the end of the tests, Madeleine was transported back to the heart floor but she was moved from the regular heart and kidney unit (HKU) to the Cardiac Intensive Care Unit (CICU). They made this move for two reasons. First, she was still intubated (on a ventilator) requiring her to be on the CICU floor and they could also monitor her more closely. Unfortunately, this proved to be a wise decision as Madeleine's condition worsened throughout the night on Thursday night/early Friday morning. Kirsten and I had gone home to sleep at about 9PM and were awakened at 2AM. We were told that Madeleine was requiring higher levels of oxygen support and showed signs of destablilizing. We decided to come to the hospital immediately and when we arrived, Madeleine was in the middle of a pulmonary hypertension "crisis." In such a crisis, the hypertension increases to a level where it is difficult for any blood to go from the heart to the lungs. Since the lungs don't receive blood, no blood receives oxygen and the body begins to turn blue while the systemic blood pressure drops quickly. Madeleine's case was exacerbated because the best drug to treat it is nitric oxide and the test proved earlier that she her hypertension is resistant to this drug. Ultimately, the crisis was resolved by administering dopamine and epinephrine. The actual crisis itself didn't last too long and the doctors are not concerned that any long-term damage occurred. That being said, they stressed that any hypertension attack is extremely serious and can be fatal. (yup-- we were thoroughly scared.)

While we were obviously scared by the crisis in the early morning hours, we're feeling much more confident about the long-term pulmonary hypertension issues. I'm tired now and will conclude with just a quick synopsis: right now, Madeleine is on the ventilator and she is going to be weaned slowly (likely over a 72 hour period-- potentially longer.) Once she is off the ventilator and stable, she will be moved back to the HKU floor (just down the hall from the CICU) and they'll monitor her hypertension's reaction to the sildenafil. If she reacts well, she will be released and we'll monitor the hypertension closely in the next few weeks and months. If that doesn't occur, we'll add other drugs to the mix. PH (the abbreviation for pulmonary hypertension) is extremely complicated and relatively unknown in the medical community and we're still trying to synthesize everything we've learned. The short answer on Madeleine's diagnosis is that we don't know how bad her case is and how much it will affect her. Let me sleep on this thought tonight and I'll provide further insight tomorrow on the next steps and how we'll move forward in treating her hypertension and determining its long-term impact.

Thanks-- have a good night. Kirsten and I simply can't say enough thank yous. Between dinners, thoughtful gifts, nice comments, and simple generosity of spirit, we're continually thankful for all of our blessings. yes-- we're scared right now but still confident in our daughter's ability to rebound and we look forward to reporting Madeleine's progress and accomplishments. Take care,

Brent and Kirsten