Glacial Movement

I said yesterday that any type of forward progress is all we want... and that's what we have. Madeleine had a teeter-totter night last night as she'd be doing well and would get weaned a bit and then would fall back. At the end of the ups and downs, she down a little bit on her oxygen support and for now, it seems like the doctors are happy just keeping her stable. Assuming she can maintain her stability-- and we're pretty convinced she can-- they'll consider weaning in the evening or possibly wait until tomorrow. They've identified a low-level infection that they're treating right now with antibiotic and that helps explain some of her struggles-- especially last night's teeter-totter.

That's pretty much it on the update front. We don't expect any major changes and are just collectively holding our breath and praying that the progress continues. We're starting to feel more confident but also realize that she could be intubated for weeks. We'd obviously prefer this to go faster but we're digging in for the long haul. We spoke with the attending doctor last night and he was re-assured when I reminded him that we've been through similar lengthy episodes in the NICU. It isn't fun but we know Madeleine puts up a good fight.

Thanks again for your support. Don't expect further updates today as we're not expecting anything to change. Thanks,

Brent

Tuesday Update: A Step Forward

Madeleine has made slow progress since being moved to the oscillating ventilator on Saturday morning and today, the doctors switched her back to the more traditional ventilator. This is a major step in the right direction as we're hoping that Madeleine can continue to be weaned. With that being said, we have a long way to go as she's on a high level on oxygen and nitric oxide support as well as getting blood pressure support and a high level of sedation. All of these drugs will need to be weaned in the days (possibly weeks) ahead before we can start discussing extubation procedures.

That being said, this is a step forward and Kirsten and I are feeling much better than just a few hours ago. We're prepared for a long, slow weaning process and we're confident in Madeleine's full recovery. (we're not so sure if our stomachs and/or hearts will ever be the same...) We still don't have a timeframe and/or any other goals other than we want to see Madeleine making forward progress each day. Once we start to see her move forward, we may set goals for when we want to extubate but right now, forward progress-- no matter how small-- is the key.

On a lighter, more positive note, I hope folks enjoy the September pictures at the top of the blog. (To see larger versions, you can visit http://picasaweb.google.com/brentbushey) My mom took these pictures less than two weeks ago when she was visiting. Madeleine was hospitalized and had been diagnosed with Pulmonary Hypertension but the pictures show how great she was doing. We're hopeful that we can take similar pictures in a few weeks as Madeleine continues on the road to recovery.

That's all for now. Thanks again for everyone's support. Given this large change today, we don't expect much weaning to occur as the doctors just want her to stabilize. I'll report back tomorrow once we have some time under our belt and expect to report that Madeleine is moving forward.

Brent and Kirsten

Monday's Update

Hi all,

The weekend was a rough one for Madeleine so I apologize for the lack of updates. I'll provide a quick update right now and will update when we get more information. When I posted on Friday, we were looking toward the long-term future with balancing Madeleine's pulmonary hypertension. This view is important but it failed to take into account the seriousness of Madeleine's current situation. On Saturday morning, we learned some troubling news: despite being on high settings on the ventilator, Madeleine's lungs weren't responding and were actually sliding backwards a bit. This was especially confusing to the doctors as a follow-up echocardiagram showed that Madeleine's pulmonary hypertension (PH) was at the same levels as she was diagnosed on Thursday-- i.e., her PH is significant but manageable.

Given this disturbing news, the doctors decided to start a round of steroids to help her lungs and also switched her from the traditional ventilator to an oscillating ventilator. (a more aggressive form of ventilation that opens up Madeleine's lungs more effectively.) Since these changes, Madeleine has responded well and they've been able to wean some of her support-- she's gone down on oxygen as well as on nitric oxide. While this progress is encouraging, we have a long ways to go. In the next 24 hours, the doctors hope to further wean her support and, if Madeleine responds well enough, they'd like to wean off the oscillator and back onto the traditonal ventilator.

That's all we know right now. We'd like to estimate time tables on her recovery but right now, we're taking it hour by hour and day by day. I'll try to update as we make progress but in the lieu of updates, operate on the assumption that no news is good news. Thanks again to everyone for your support.

Brent and Kirsten

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

Still at Hospital

We're still at the hospital in the Heart and Kidney unit at Children's hospital but it seems like our time is nearing an end. The doctors have been extremely cautious during this stay as they are hoping to prevent any further hospitalizations. Additionally, they've been a little confused by Madeleine and want to make sure that they don't miss anything. Here's a quick run-down of where we are:

1) Cardiology: We are in HKU because Madeleine was diagnosed with pulmonary hypertension two Fridays ago (9/12). We are getting another echocardiagram today and we're hoping that it continues to show that the hypertension is resolved. If this is the case, we'll likely be discharged and will return to the hospital in about a month for additional tests. Madeleiene's initial hypertension readings were very high so the doctors intend to monitor her closely for the time being. I'll report more once the echo results come in today.

2) GI: Madeleine is not feeding by bottle right now and we intend to keep it that way for at least a few months. She is on a continuous feed pump and her feeding tube goes past her stomach into her small intestines. The goal right now is to get her to grow (she's nearing 8 lbs) and we'll reassess in a few months. We'd prefer to feed some via bottle but given the likelihood that Madeleine would aspirate formula into her lungs, we're not taking that chance. This is quite important as Madeleine's lung issues likely caused her high hypertension readings and further trauma could lead to this condition once again.

3) Pulmonary: Madeleine had a CT scan yesterday and it largely confirmed what we already knew: her lungs show signs of BPD (premature lung condition) and resolving pneumonia. The good news is that none of the damage/issues appear to be life long problems. If we can just keep her healthy and get her to grow, her lung condition will improve. Madeleine has also developed a new issue in the hospital: her oxygen saturation rate drops when she is laying down in certain positions (called positional desatting). We're not entirely sure why this is happening and we're trying to come up with a solution. The good news is that once she is re-positioned, her saturation rates go up quickly so it doesn't appear to be a long-term issue. Its likely that she obstructs (like sleep apnea) and this issue arises. This is the most acute issue we're facing right now and ideally we'll have a solution before she is discharged.

That's about all we're focused on right now (think that's enough). Once we resolve all issues at the hospital-- namely, the echocardiagram and the positional desatting-- we need to determine where Madeleine will be discharged to. We have the option of bringing Madeleine home and this is our preference. However, we have had some discussions about moving Madeleine into a rehabilitation hospital for a short-term stay. We feel that we're fully capable of handling all of Madeleine's day-to-day medical issues but we're a little uneasy about the positional desats and by Madeleine's general instability. Given these issues, we may decide it is beneficial to place her in a rehab hospital for a few weeks to allow her to grow and ideally eliminate the positional desatting. When we bring her home, we'd like to be confident that she's strong and won't relapse and end back in the hospital for another stay. The jury is still out on this decision and its one we hope to resolve in the next few days.

Thanks again for everyone's support. This is a long, frustrating road but we're making the best of the situation and we're confident in Madeleine's ability to overcome all of these challenges. Take care,

Kirsten and Brent

Slow Progress at the Hospital

If you've ever been hospitalized or have had a family member hospitalized, you'll be familiar with what I'm about to say. We are confident that Madeleine is getting better as the meetings and discussions with doctors are getting later and later every day. Last night, I didn't speak with Madeleine's attending doctor until 8:30 PM. While this waiting can be frustrating, we've learned that the doctors deal with their most difficult cases first so it is also a bit re-assuring.

The big news yesterday was focused on results of the most recent echocardiagram. The echo was a little different than Mondays as it showed slightly elevated pressures in her heart. The doctor explained that if this was her first echo, they wouldn't be concerned as the echo test isn't a perfect diagnostic for pulmonary hypertension (PH). However, given her results last Friday, this raises a small amount of concern and muddies the water a bit.

With this muddy picture in mind, the focus for the doctors is to identify a viable explanation for Madeleiene's PH last week. The assumption right now is that her GI issues-- reflux, aspiration, and pneumonias-- have caused lung trauma and this repeated trauma led to the PH they identified last week. While this is a logical explanation, the cardiologists want "proof" from GI that this is correct and, so far, we don't have any test results that demonstrate how/when the reflux and aspirations are occurring. For the next few days, the cardiologists have asked GI to re-examine Madeleine to see if any further "proof" can be identified.

If GI can identify a plausible cause of the PH, we may be in the clear and Madeleine could be released without any further tests. However, since it will be difficult to identify a plausible cause, it is likely that the cardiologists will schedule a heart catheterization (heart cath) in the next few weeks. The heart cath can provide additional data including:
- More accurate readings of Madeleine's pressure. The echo is an indirect measurement tool while the cath can directly measure Madeleine's heart pressures. This is especially important in Madeleine's case as she has small (medically insignificant) "holes" in her heart related to her previous heart surgery. While the holes are medically insignificant, they make reading her pressures via an echocardiagram much more complicated and potentially inaccurate.
- Additionally, the cath can take pictures of the blood vessels in the pulmonary veins leading to the lungs. PH can be diagnosed by identifying inappropriate pressures via echo-- like last Friday for Madeleine-- but the disease itself is characterized by changes in the blood vessels in the pulmonary veins. If enlarged veins aren't apparent, the doctors can be much more confident in attributing last week's PH to the treatable lung trauma (aspirations/pneumonia).

The downside to a heart cath is that it is invasive and the doctors don't want to conduct it unless they feel that it is medically necessary. We'd also prefer to avoid the procedure but if it provides helpful information, we'll obviously support the doctor's decision.

I hope this posting makes sense to you. PH is complicated stuff and if I'm the only one that understands it, that is fine with me-- it really helps me to type all of this stuff out. (I wish her doctors would read it and clarify where I'm making mistakes.) If all of the other technical stuff flew by you, here's what it means for the short-term: Madeleine is going to be in the hospital through the weekend and we don't expect to learn much more until results from the various GI tests-- sleep study (conducted last night) and any other tests ordered by GI today-- are made available. Anything is possible but its likely that we won't know much more until Friday afternoon or even next week. (Remember what I said at the top: good service in the hospital equates with poor health so we're fine waiting until Monday for the results... I'll keep repeating this mantra as the waiting takes its toll this weekend...)

We'd obviously prefer to get Madeleine home but she's in a very comfortable room-- we can watch the ND-UM football game on a flat panel television-- and honestly, she seems to enjoy the audience she gets in the hospital. As one example: she woke up at 4:45AM this morning as the nurse was removing the leads from her body associated with the sleep test. You'd think Madeleine would be angry with such a disruption of her sleep but no, she just flashed her tongue and dimples at the nurse... good stuff.

That's all for now. I hope things are going well for everyone out there. Thanks for your interest and support.

Brent

The Charmer at the Hospital

Quick update this morning before I head to work: Madeleine had another good day at the hospital and she's becoming quite the charmer. As she continues to feel good, she's beginning to realize that she's the star of the show and whenever a new person enters the room, she flashes her new smile where she sticks out her tongue and smiles showing off dimples on both sides. It only lasts for a second so we have yet to catch the smile on camera but yesterday, there was a group of student nurses that were dispersed across the cardiac floor and all of them came by to practice with the "little baby that likes getting her vitals taken." Kirsten and I could have our hands full when we get home and Madeleine realizes that she's the star but only of a three person house.

As you can probably tell, things are going quite well. We're in the hospital right now primarily because we really can't explain the pulmonary hypertension and the doctors want to keep a close eye on Madeleine while conducting various tests. Yesterday, the focus was on Madeleine's GI tract and everything they looked at seemed normal. Today, there will be another echocardiagram to ensure that the pulmonary hypertension remains under control and tonight, Madeleine will participate in a sleep study where they will monitor her esophagus and air passage way at night to determine if she has any blocked apnea issues. Depending on the results, the sleep study could help us determine a path moving forward regarding feeding her by mouth.

Currently, Madeleine is getting nothing by mouth (they call it NPO in the hospital) and all of her medicines and nutrition go into her "J" tube. For the time being, we're comfortable with maintaining this course of action as it is clear that Madeleine is putting on weight and isn't suffering from reflux. Obviously, down the road we hope to be able to feed her by mouth but until she grows a significant amount, there's ample evidence indicating that her reflux presents a potential problem with aspirations. Since it is likely that the hypertension identified last week was related to aspirations and/or pneumonia, it is extremely important that we avoid any further aspirations and/or trauma to the lungs.

In any case, I just wanted to provide a quick update to let you know that things are going exceedingly well here. Once we get through the echo today and sleep test tonight, we're hoping to get a better idea of what the next steps are and possibly when Madeleine could be discharged. Thanks again for everyone's support.