Baby Steps

Madeleine has had another positive day today. She's maintained her low level of oxygen support and was successfully weaned from Vecuronium (VEC), the paralytic drug that they used to keep Madeleine calm. This was a fairly large step forward today as the doctors weren't certain that Madeleine could remain calm after the VEC was weaned. As with everything else, there is no guarantee that we won't have to go back on the VEC but we're cautiously optimistic.

The next step is to slowly begin weaning the nitric oxide-- they're not sure if we'll start this tonight or if we'll wait until tomorrow to move forward. As with everything else, our mantra is simple: slow and steady wins the race. Madeleine is doing great and we don't want to push her to the point that she falls backward. Its still possible that we'll fall backward anyway but the hope is that by going slowly, we can avoid further crises days.

That's all from our end. We're hopeful for slow and steady-- dare we say boring?-- days. Thanks again,

Brent

PS-- I forgot the most important point: for the first time in well over a week, Madeleine held my hand. She's still heavily sedated so she probably thought I was a smurf or a pink elephant but I'll take that over seeing my daughter paralyzed...

Madeleine: The Roller Coaster

This will likely be a long post, so let me get the important details up front: After some extremely difficult days, Madeleine has stabilized and is "doing well." I've used the quotation marks because she's still on a ventilator in the CICU but she has come a long ways in the past few days and we're feeling much better about her short and long-term prognosis.

That's the short story. Here's the long one: Starting on Thursday afternoon, Madeleine began to struggle with her oxygen saturation levels and her oxygen dependence grew rapidly. She finally stabilized Thursday evening and the doctors told us that our "day of crisis" appeared to be over. Friday was a relatively easier day as Madeleine appeared to stabilize despite being very reliant on oxygen and other ventilator support. We hoped that this stable trend would continue through the weekend but Saturday turned into another day of instability. In addition to struggling to maintain her oxygen saturation levels, on Saturday, Madeleine's blood pressure fluctuated from very high pressures to dangerously low pressures. Madeleine's nurse had never met Madeleine before Saturday and by the afternoon she looked at Kirsten and I and exclaimed "she's just really frustrating and doesn't make any sense." I think you can categorize such a statement as "preaching to the choir."

Fortunately, starting on Saturday night, Madeleine finally appeared to get her act together and in the past 24 hours, she's made major progress. She has greatly reduced her oxygen dependence to 50%, has stabilized her blood pressure allowing them to wean her dopamine level (down to 8 mcgs from 12) and the doctors are beginning to make plans for tomorrow's weaning. (They don't make plans when they're concerned about another crisis.) Assuming Madeleine stays on course, tomorrow they hope to have her dopamine down to 5 mcgs (a low level) and then will turn to reducing the nitric oxide-- she's currently at 20mcgs (a high level). In the next few days, we hope to slowly wean the nitric oxide, the dopamine, the oxygen and other ventilator settings. If all goes well, the doctors would possibly talk about extubating Madeleine by the end of the week.

We'd like to be optimistic about such a short timeframe but honestly we're dug in for the long haul as we've learned (or re-learned) these lessons in the past few days:
1) Pulmonary hypertension is extremely complicated and when you are on a ventilator and sick, you can expect to have challenges-- weaning is never simple and just when you think things are going well, they can all fall apart.
2) The ups and downs are extremely frustrating and can be dangerous. While this reality is difficult to accept, we learned this fact on Saturday and it at least helped us understand why Madeleine is having such a difficult time-- understanding helps us keep things in perspective.
3) Madeleine is an extremely frustrating and difficult kid but is also tough as nails. If you're a frequent visitor to this blog, you already know this fact. Kirsten and I have had some difficult days and we've honestly questioned whether Madeleine would make it through these challenges. We're not out of the woods yet but we've been amazed by Madeleine's will to live and are confident that she's going to make it through these challenges. She just doesn't quit and I love that about her. (Though I'd love her if she could find a way to get healthy and stay that way.)
4) We have an astonishing network of friends and family. Here's a few examples of the support that we've been amazed by:
- During our difficult day on Saturday, two of Madeleine's long-time caregivers came to Madeleine's hospital room and talked to us, explaining how pulmonary hypertension helps explain the ups and downs. Their ability to put our experience in perspective and to empathize (I can never remember if its empathy or sympathy?) with us was truly remarkable and helped us unwind and sleep well on Saturday night. (The important of sleeping well can't be overstated.)
- Kirsten's co-workers, sensing that we were down, put together an amazing basket of goodies including food for the week, lotions, magazines, and tons of other items.
- Our parents have agreed to travel out here in the next few weeks to help support us as Kirsten makes the transition back to work (starting next Monday). Given the uncertainty surrounding Madeleine's situation, this flexibility makes it much easier for Kirsten and I to continue working, knowing that Madeleine will have an advocate in the hospital.
- One friend of ours demanded my keys so that she could go to our house and clean our kitchen. She'd heard Kirsten complain about not having enough time to clean and she practically backed me against the wall telling me that I needed to give her my keys so that Kirsten wouldn't have to go home to a dirty kitchen. (true story)
- Our close friends and neighbors allowed us to stay over last night to watch the Penn State game despite being so tired, they both fell asleep in the third quarter. We didn't want to leave as VA. Tech was on and pre-empted PSU in our house. (They have the Game Day package.) This is just one example of this couple's constant role as our "surrogate DC area family" as they're the ones that help us pick up the pieces on a daily-- and sometimes hourly-- basis.

In short: Kirsten and I continue to be amazed by the outpouring of support that we've received. (These stories don't take into account the countless number of people who have listened to us vent about Madeleine and the difficulties we've faced.) We're humbled and honestly don't feel worthy of such kind acts. We simply hope that folks know how appreciative we are even if we can't get thank you notes out in a timely fashion.

I'm sure my brothers are reading this and right about now are thinking "Geez Brent, cut it with sappy crap" so I won't continue any further. I'll report back this week as the hospital sagas continue and hope to repot that the Madeleine roller coaster continues on this smooth section. Have a good start to the week and thanks again for your support.

Brent and Kirsten

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.

What a Day!

Today was definitely a difficult day with a potentially very happy ending. We learned this morning that based on additional review of last Friday's echocardiagram, the doctors were quite concerned that she had a chronic form of pulmonary hypertension that could impact the quality and even the length of her life. They believed that Madeleine's recent pulmonary issues-- recurrent pneumonia, aspirations related to her reflux, and her overall health-- could contribute to the hypertension readings that they got last week but they felt that her premature birth, her Down's syndrome, and her history of heart disease made it highly likely that her hypertension would prove to be a chronic form that would require medical intervention and could prove extremely serious.

As you can tell from the tone of this post, we learned later today that it is possible that Madeleine's pulmonary hypertension is already under control and quite possibly may be related primarily to her recent bouts with pneumonia and aspiration. We still have lots of tests to conduct and a lot of praying and crossing our fingers but today's echocardiagram failed to demonstrate any form of pulmonary hypertension. In fact, the results were so shocking that before the doctors told us the news, they ordered a second echocardiagram explaining that they didn't get good pictures of some views of the heart during the first test and wanted to see further pictures before providing a diagnosis. Both echocardiagrams showed the same picture-- that Madeleine's hypertension is resolved for the time being-- and we're cautiously optimistic that the worst is behind us.

You might expect the "but" that is going to follow and you're right on. Here's the caveat about today's findings: they're so shocking that no one is convinced that they're one hundred percent accurate and its fully possible that Madeleine has some form of hypertension and/or could develop it down the road. That being said, this was the best possible news we could get today and we're allowing ourselves a little bit of relaxation and enjoyment.

Our game plan for the week is as follows:

1) The focus for tomorrow and likely the rest of the week is to determine how major a role Madeleine's GI system plays in its relation to the lungs. They will conduct an upper GI test tomorrow where they feed Madeleine barium fluid and then X-Ray her to see the condition of the GI tract. Any abnormalities in the esophagus, stomach, and X-Rays should be noticeable on the X-Ray as the barium contrast fluid shows up very well. Additionally, it will be very apparent on the X-Ray if Madeleine aspirates any of the fluid. We'll also do a sleep study to check for blocked apnea and will potentially also re-do the milk scan and swallow studies that were done a few months ago. The reason for all of these tests is to identify madeleine's reflux issues and/or other abnormalities that can potentially cause Madeleine to aspirate fluid into her lungs. The ultimate goal is to avoid any further aspirations as Madeleine's recovery shows that her lungs can be drastically impacted by external trauma.

2) We'll continue to have echo cardiagrams taken every few days. The basic goal of these X-rays will be to esure that the pulmonary hypertension remains dormant.

3) We'll consult with the pulmonary team as well as with complex care to ensure that our path going forward takes into account her issues with pulmonary hypertension as well as the rest of her complex medical challenges.

In short: we have a long road to haul but we feel extremely fortunate today and we're hoping for a similar good day tomorrow. Thanks!

Brent and Kirsten

A Better Day

After the difficult news yesterday regarding pulmonary hypertension, we're happy to report that we've had a very good day in the cardiac unit at Children's hospital. We're by no means out of the woods but Madeleine has as good a day as possible. When Madeleine was transferred to the cardiac unit last night, the goal was very simple: stabilize Madeleine's lung issues, administer oxygen to help treat the hypertension, provide steroids and additional nebulizers to help her lungs recover, and monitor her to see how she responds. We're happy to report that Madeleine has had a very good day and we believe that her status has improved throughout the day. She's by no means back to behaving 100% normal but we've played with her quite a bit and she's much more stable and happy than she has been for the past two weeks. This positive reaction doesn't mean that the hypertension is improving since the increased oxygen can mask some of the symptoms but it is a very good sign. (if her condition had worsened today, there would be more concern that her condition was being caused by the hypertension.)

The goal for the rest of the weekend remains the same and we're hoping that very little changes. As far as diagnosing the hypertension and its extent, our next update should come on Monday when another echocardiagram is conducted. There are two possible outcomes that we can expect on Monday:

1) The preferable alternative is that the echocardiagram demonstrates that the pulmonary pressure has decreased. If this is the outcome, we'll likely continue with the same course of action and will hope to wean Madeleine slowly from oxygen. The assumption will be that Madeleine's hypertension has resulted primarily from the trauma to her lungs caused by the pneumonia and related reflux/microaspirations.

2) The other alternative is that the hypertension will be largely unchanged (or possibly increased.) If this is the case, there are a number of courses of action that will be considered. First, the doctors will likely schedule a cardiac catheterizaion. This is a procedure where a catheter is inserted in Madeleine's leg and goes into her heart. The procedure is helpful for two reasons: first it is much more accurate in diagnosing the extent/severity of the hypertension than the echocardiagram. Additionally, during the procedure, the doctors can experiment with possible courses of treatment and can judge how effective the treatments are in reducing the hypertension. Additional courses of action would be determined based on the results of the catheter procedure.

That's about all we know right now but I'd like to add a couple of quick personal notes. First, we're still scared but we're feeling a heckuva lot better than we did 24 hours ago. One of our good friends suggested a month ago that Madeleine deserves a pair of pink boxing gloves as she continues to show that she's a fighter. Today is yet another example of Madeleine's fighting spirit.

Second, and much more importantly, we're floored by all of the support that we've received since receiving the difficult news yesterday. We can't say thank you enough to all of the folks who have read the blog, sent us text messages, called with concerns, prayed, the list goes on and on. Additionally, we don't say this nearly enough but the health care professionals that have helped with Madeleine are truly great and passionate people and we're eternally grateful for the fact that we live so close to such a great facility. We've obviously had some difficult days here at Children's and there have been days when we've been frustrated with the staff. Still, the vast majority of our experiences-- despite all of Madeleine's medical challenges-- have been positive and we can't say thank you enough to those that have aided in Madeleine's health challenges.

That's all for tonight-- we'll continue to provide reports as we learn more about pulmonary hypertension and Madeleine's condition. Thanks again for your concern and support.

Kirsten and Brent



Madeleine was transferred to the cardiac unit last night and the plan is very simple: Madeleine will get 2 liters of oxygen for the next two days and will continue her IV steroid treatments as well as a high amount of nebulizer treatment for her lungs. The goal is to ensure that Madeleine's lungs get cleared out as much as possible in the next few days. Further ensuring that Madeleine remain stable and grow, she is being fed via the "J" tube that extends into her small intestines and is receiving all of her food as well as her standard medicine for reflux and indigestion via the tube.

As far as the hypertension is concerned, we're not going to learn much more until Monday as the goal right now is to stabilize her, treat her lungs, and by Monday, enough time will have elapsed to compare Monday's echo results with the results from yesterday (Friday).

Really Frustating and Concerning Situation

Madeleine was re-admitted to Children's Hospital yesterday after four days of us struggling at home with her oxygen saturation levels. Using the pulse oximeter (pulse ox) that we brought home with her last Friday, we began monitoring her blood oxygen levels this past Monday when she just didn't seem right to us. We consulted with our pediatrician as well as with the pulmonary folks at Children's DC and after Kirsten took Madeleine to the pulmonary clinic yesterday, they decided to admit her as they were concerned with her labile (highly fluctuating) oxygen saturation levels.

Our initial frustration focused on everyone's inability to explain why her saturation levels were so low and fluctuating. The doctors took two different chest X-Rays, checked her blood levels, her electrolytes, and a host of other issues. Finally, after exhausting most other potential causes, they ordered an echocardiagram to examine her heart in an effort to rule out one more possible cause. The doctors weren't overly concerned with the heart given the success of her previous surgery confirmed by follow-up echo cardiagrams while Madeleine was still in the NICU. Unfortunately, the echocardiagram unearthed some concerning information: it diagnosed pulmonary hypertension.

For those unaware of pulmonary hypertension-- a group that included Kirsten and I until about 3 hours ago-- this is a diagnosis that is made when the lungs have higher blood pressure than the rest of the body. In a healthy human, the lungs have slightly lower blood pressure than the rest of the body making it easy for the heart to send blue blood back to the lungs for re-oxygenation. Hypertension is dangerous because the higher pressure makes it more difficult for the heart to send blue blood to the lungs. We learned that there are two types of hypertension: primary and secondary. Secondary hypertension is more common and is the treatable form of hypertension and results when someone has significant trauma (pneumonia being the most common form of trauma) and/or infections that can cause a temporary increase in the lung's blood pressure. Given Madeleine's recent bouts with pneumonia, it is entirely possible that this is the form of hypertension that she has but since we did not have echocardiagrams performed during her previous three stays in the hospital, it makes it hard to determine if the hypertension has been caused by her bouts with pneumonia or if the hypertension is one of the primary causes. Primary hypertension is the less common form of hypertension that cannot be treated and is ultimately fatal. (For those from the medical profession that are reading this-- I know I'm oversimplifying the issue but this is how it was explained to us in the hospital.)

At this point, there is no way of knowing what type of hypertension we're faced with and the doctors must gather a bunch of data before we'll know anything certain. For right now, our focus is on treating her lungs and ideally eliminating the high pressure. The good news is that the treatment that was started when she was admitted on Thursday is largely the course of treatment that is recommended for hypertension. The best "drug" for treating hypertension is oxygen, so the doctors are going to keep Madeleine at a higher oxygen level (at least 2 liters) for the next three days. In addition, Madeleine was started on a round of IV steroids and this will continue through the weekend. There is no evidence of infection at this time but Madeleine will be closely monitored and if any is identified, they will start a round of IV antibiotics as well. After three days, Madeleine will undergo another echocardiagram (for those unaware, this is a non-evasise procedure that uses the exact same technology as an ultrasound used on pregnant women.) If the echocardiagram shows a decreased level of blood pressure-- this will be really good news as it will likely indicate that the high pressure is caused by the lung trauma and the treatment-- oxygen and medicine-- is helping to resolve the issue. If it doesn't show a decrease in pressure, this won't be terrible news but will likely indicate the need for additional medical intervention including some drugs that I know nothing about. The worst diagnosis-- primary/persistent hypertension-- will only be confirmed after all of these different forms of intervention have been exhausted.

Kirsten and I are obviously frustrated/scared/angry/worried and lots of other emotions that we were unaware existed just over 6 months ago. That being said, we're confident knowing that Madeleine has come through plenty of other difficult diagnoses and we're fully expecting her to clear this hurdle as well. We'll report back further information as it comes to us-- just wanted everyone to be aware of the issues we're faced with right now. Thanks for your support.

Brent and Kirsten

6 Months!

Madeleine is 6 months old today! We can hardly believe it. In some ways, we can hardly remember a time before her birth. In other ways, it has been the craziest, hardest, scariest, happiest, and fastest six months of our lives. Regardless: it hasn't been boring.

For those that haven't heard-- Madeleine came home from the hospital last Friday and we spent a relaxing weekend in the DC area. We've struggled a bit with Madeleine's feeding as the pump can be cumbersome when you're moving her around. Additionally, Madeleine started displaying some signs of reflux over the weekend and we've decided to stop bottle feeding for the time being. Instead, we'll supplement additional hours on the feeding pump and we'll re-try bottle feeding in a few weeks once we've seen significant growth and feel that Madeleine will be better equiped to control her reflux.

In the meantime, we're focused primarily on keeping Madeleine comfortable and hope that she'll begin to put on weight. Her stay in the hospital caused her to lose weight and she's now about back to her previous weight of 7 lbs 11 oz. Once we've ironed out all of the feeding issues-- Kirsten is meeting with our pediatrician today-- we're hopeful that Madeleine will begin to gain 1 oz/day again. More importantly, we're hopeful that the feeding changes we've made will ensure that we avoid the hospital for at least a few months.

That's really about all that's on our plate right now. I've been reluctant to post in the last few days as I've been rather frustrated with all of the changes we've made with Madeleine and the associated concerns about these changes. In the long run, Kirsten and I are confident that Madeleine will overcome all of the challenges. On a day-to-day basis, it gets a little bothersome and frustrating when you're constantly worrying about the health of your child. I'm starting to get more confident that we're out of the woods associated with reflux, pneumonia, and hospital visits and I'm hoping this continues.

Thanks again for everyone's support. Here's to a much healthier next 6 months.