Monday December 17, 2007

I am sorry for keeping you hanging, but yesterday turned out to be a long day, not because of Maddie, but other stuff. Don’t laugh, we do have other stuff in our lives! In case we haven’t said it in a while, we truly appreciate all of you faithful souls who tune in daily (some of you hourly) to hear the latest news. When emergent situations arise Cindy & I pray first, and then put the word out on the web ASAP, so if a bit of silent time goes by, you should take it as good news or at least the absence of bad news.

We didn't really get a formal debrief for the failed trial yesterday, but I think she just wasn't optimized very well for it. She had a lot of things working against her. The trial, which was originally scheduled for Monday morning had to be done a day early, because the circuit was too cruddy to last another day. It was kind of a double whammy because she failed the trial, but she also had to have the entire ECMO circuit changed.

Today was fairly uneventful, as Maddie equilibrated to the newly changed ECMO circuit. The plan is to trial her off again tomorrow morning. We are hoping that she comes off tomorrow, but we are praying to see and understand God’s timing. Bed time now. G’nite.

Tuesday December 18, 2007

Well it is 1:15 PM and Madeline has been trialed off of ECMO since 9:30 this morning. She is doing incredibly well! She is nearly weaned down to the baseline of 60% ventilator oxygen and her vitals are all rock solid. Somehow her saturations are higher now than they were on ECMO. She is in the high 90’s and we have even seen the occasional 100%!!! The word on the block is that the trial is basically complete, and she WILL COME OFF ECMO TODAY!!! God be praised for answering our prayers and giving Maddie the ultimate crew to save her (again).

The cannulas still need to be removed surgically, which is no small hurdle. Her demonstration this morning has only earned her eligibility to face the next surgery. She is still in total renal failure, having not made any pee pee for a week now, so she needs dialysis. The ECMO circuit made it easy to filter her blood, but now she will have lost that ability. We know she will require dialysis, but we are not sure how it will happen. Most likely she will need a venous catheter. We are praying that her kidneys recover quickly and fully, so that her veins can be protected. She will need some good venous access points for the rest of her life, so we really need to safeguard them. She has lost most of her primary veins.

4:30 PM
The decision was made to have a dialysis catheter inserted in Maddie’s groin tonight, because we need the ability to dialyze her if necessary and there could be an immediate need. Everything will start happening soon and quickly. She will be separated from ECMO and de-cannulated, and then the catheter will be inserted. Please pray that all goes well. We will rejoice when ECMO is gone, but it will be short-lived, because once she is back to her old Fontan physiology, she will have the potential for forming casts in her lungs again. She needs a heart – period. We are praying for that now more than ever.

Later that evening . . .


Wednesday December 19, 2007

Yesterday’s celebration was cut short last night when Maddie’s blood pressure and sats began falling through the floor. It was that kind of night where the hospital had to call us and say, “You might want to come in.” I was heading back to the hospital around 10:00 PM, anticipating a night of uninterrupted sleep, when the call came. It was pretty obvious right away that it was going to be a restless night. I so badly wanted to avoid making the call to Cindy, because just the sound of the phone ringing is sickening. When you realize it is the hospital, it is even worse. When the voice on the other end doesn't immediately say, “She’s fine,” it takes your breath away. After about 30 minutes of watching Maddie dip into the danger zone I had to make that call.

Early on, we were afraid she might not make it through the night. I could have lived with less than optimal vitals, but when they suctioned out a cast, my heart sank. Everyone was hoping against hope that it was not a newly developed cast, but rather an old one that just rose to the surface. It might have been wishful thinking, but we were all banking on it. She was tenuous through the night, but survived. As of this morning she is still extremely fragile and continues to walk a fine line, but so far there have been no more casts that we know of.

It has become so hard to know precisely what to pray for. We know she needs a heart, but it could be best for her if she could take the next week healing up and getting her kidneys back to center. However, if she waits too long the plastic bronchitis could be an issue. On the other hand, the longer she is able to wait, the better her chance could be of finding a more suitable heart. Receiving a heart today may not be in her best interest at all. We just don’t know. What a perfect opportunity for God to flex His muscles and show us what His plan is. Please pray for her kidneys to recover, her lungs to clear, the casts to keep away, and a heart to arrive at just the right time. Also pray for peace for the doctors and staff to chart the proper course.

Thursday December 20, 2007

Well Madeline had a much better night last night and a better day today than yesterday. She is still brittle. She is on 90% vent oxygen support and her own oxygen sats have leveled off in the mid 80’s. More oxygen does not help raise her levels, so we probably have to conclude that it is not a pulmonary gas exchange function, but rather a blood flow problem. We could pump all the oxygen in the world into her lungs, but if the blood is not flowing there to make the pick up it just doesn't matter. Because of all the trauma and lung disease, she has developed a high pulmonary vascular resistance, and without a pumping ventricle to overcome this resistance, pressure builds up on the venous side and migrates to the path of least resistance, which in Maddie’s case is her liver. Guess what? Her liver is enlarged (no great surprise). Another path the pressure could potentially take is through a lymphatic duct, which is precisely where it goes in the case of plastic bronchitis. The lymph is supposed to flow from the lungs (and everywhere else) into the venous system, and then off to the kidneys (like taking out the trash), but if the venous pressure is too high the lymph flows backwards.  You know what happens when your septic system flows backwards into your house? Yeah, same thing pretty much with Maddie’s pressure. Understand? There’s your anatomy lesson for the day.

Now the good stuff. Somehow she has managed to find her smile again under all of the tape and tubes about her face. Her blood pressure and sats are not perfectly where we want them, but at least they seem to have stabilized, unlike the night she came off ECMO, where if she sneezed we were afraid we might lose her, or have to put her back on ECMO. At the moment the order for ECMO is on stand down. Praise God. Praise God. Praise God.

Friday December 21, 2007

Madeline never ceases to amaze us. I was hopeful that last night would be a quiet uneventful night of peaceful sleep, but you just cannot count on Maddie to behave for too long. She usually goes to sleep after dark, but then wakes up around 10:00 PM to play for awhile. As the nurse and I were putting her to bed around midnight we notice she was kind of cranky. I heard the distinct sound of her passing a little gas, but we checked her diaper and it was clean. Maybe I was hearing an air leak around her breathing tube? No. As we rolled her on her side to reposition her for the night, we found a dime-size hole in her side that was oozing air and a lot of old blood!!!

I can’t remember if we mentioned this or not, but the first time Maddie was on ECMO and she had that bed-side thorocotomy, the doctors had to remove a section of rib. Three weeks ago a small lump appeared on Maddie’s side and it continued to grow. It was first thought to be subcutaneous air and was dismissed, but when it grew to the size of a baseball it was determined to be her right lung herniated through the missing rib area. The doctors pushed it back in and wrapped her in a pressure dressing, like the kind you wear for a cracked rib. I may not have mentioned this before because a herniated lung and missing rib are minor nuisances for Madeline. There’s some perspective for you!

So, as we rolled her over there was a tremendous amount of blood and air oozing out from under the pressure dressing. We removed the dressing and discovered that a round scar from one of her chest tube sites had reopened, most likely from the skin being stretched. That site had been healed for almost two months, which shows you how fragile her skin is. It has not been fully confirmed, but the current thought is that the blood was more junk that was gathered in her right pleural space (recall the loculations and clotted material from the last ICU tour). Whatever it was and wherever it came from, we think it is better out than in. Maddie must think so too, because she is breathing much better now with far less support.

The rest of today was good. Everyone is hoping she will continue to heal and get stronger each day leading up to the heart transplant. We are praying specifically for two major things. 1) that her kidneys will start working well enough to take her off dialysis and 2) that she would stave off any infection, as that would make her ineligible for a donor heart.

Saturday December 22, 2007

It was a calm day for the most part.  I cannot remember the last time Maddie slept so much during the day.  The dialysis machine had some trouble through the night, so she is retaining some fluid, but hopefully that will improve now that we are back up and running.  Her abdomen is HUGE and her skin is stretched taunt across her belly.  We are praying tonight for that swelling to subside before her skin starts splitting apart.  I would not have thought it possible until it happened yesterday.

Her numbers are okay, but she continues to hover at the minimally acceptable oxygen level, occasionally dropping below the 80% threshold and regularly receiving suction.

Cindy found our verse for the day.  For all of her life, we will ever look at Madeline and see the goodness of God.  That is a promise.

"Let me live that I may praise You . . ."
Ps 119:175

Sunday December 23, 2007

Cindy writes:

"They will call Him Emmanuel, which means God with us."
Matthew 1:23

If there is one thing we have not doubted in all of this is that God is with us. Has been with us every step of the way. And as Christmas quickly approaches we find even more comfort in that fact. God has not forgotten nor abandoned Madeline or us in this journey, instead He has proven His everlasting faithfulness. God is indeed with us! And the more I think about things, the more I hope that a heart does not come for her at Christmas. For as wonderful as it would be for us, how I would hate for someone else to have "the sting" of such heartache associated with the beauty of Christmas. How I would hate for another family to somehow think that God was NOT with them because of the day. Anyhow, enough with the "deep thoughts."

So for today, little Miss has done very well. We've been able to go down on her oxygen a bit. Her sat's are better, a bit. Her lungs seem to be clearing a bit. And the hole in her side is not leaking as much . . . Oh, but then there is the issue of her tooth. Yes, now it is her front tooth that seems to have gotten knocked loose by her breathing tube. Oh well, it's only a tooth. Kinda like, ah well, it's only a rib.

Tuesday December 25, 2007

We woke up this morning in a very non-traditional way, in a hotel suite on a hide-a-bed.  This will be the second time I spend Christmas morning in a hotel.  It's not optimal, but as Anna and Sam reminded us, the important thing is that we are together, and that next year Maddie will be with us.  I hope to make a better update later today, but just wanted to get the word out early that Maddie had a restful night and is doing fine.

What a blessed Christmas we had.  With the exception of Maddie not being with us, this day was perfection. -- a gift from God.  I had prayed so hard that Maddie would behave, and that there would be no midnight call on Christmas Eve.  For the sake of Anna and Sam, we wanted to make this day as magical and memorable as we could.  Everyone, both near and far chipped in to make that dream happen.  Our kids know that Christmas is a birthday celebration and it is not measured in the presents we get, but just to give you an idea of the magnitude of the, "Chipping in," take a look at this picture from our room at the McDonald House!

These are gifts we received from the house, the hospital, families, friends and dear people whom we have never met.  This Christmas has demonstrated to us more than any other what giving and sacrifice is all about.

Speaking of sacrifice and giving, there are two unsung heroes who have blessed us beyond measure.  Never looking for recognition and always quick to try and convince us that their gifts to us are not sacrifices (even though we know better), it was Nana & Poppa once again to the rescue to postpone their Christmas and allow us to have ours, by spending the night at the hospital.  It is hard enough to walk this road with Maddie, but there are two things, that without either one would make this journey infinitely more difficult.  First we know that Anna and Sam are safe and sound and surrounded by loving family.  Second, we have never missed a paycheck from work.  Walt & Bonita are responsible for both.  Many families here in the hospital do not have that and it makes us realize how truly blessed we are!

Thank you Nana, Thank you Poppa for loving us and your grand-kids.  Your hearts are solid gold.

So for Christmas we . . . had a feast at the McDonald House, checked into a hotel suite, played on the elevators, swam in the pool and hot tub, opened a present, had too much chocolate fondue at the Melting Pot, played on the hotel elevators some more, stayed up too late, opened another present, had a pillow fight, watched TV, passed out, woke up on Christmas morning, another pillow fight, hotel brunch, more swimming, went to the McDonald House to play, opened a ton of presents, had another huge feast, sat around in disbelief about how much we had eaten, had some pie and cupcakes, Anna won the, "Guess how many candy bars are in the jar," contest, and won the jar of candy bars, so we ate some of that too!!!  It even snowed for a few minutes today!  Finally we made it over to the hospital to play with Maddie for awhile, before saying goodbye to the kids and Nana & Poppa.  We have some presents for her to open, but decided to wait until tomorrow.  Hey we all stretch our own birthdays out for several days, why not Jesus' too?!

She really loved her candy cane . . .

. . . and playing Rudolph with JoJo

Merry Christmas Little One.  - a great gift for many.

Wednesday December 26, 2007

Good night.  Good day.  The tentative plan for tomorrow is to take a peek down Maddie’s airways with a flexible bronchoscope to see if there be any casts, cast formations, bits of cast material, or in general any signs of casts.  Hopefully it will be a quick in – look left – look right – get out.  Sometime in the afternoon she is supposed to have a Doppler study done to map out her vascular remains and find out what major access areas she has left.  We know of a few big veins that are no longer functioning and access will be critical to her follow up care after the transplant, especially for long-term dialysis.  So far no one is saying that her kidneys will not recover, but they are saying it could take several weeks or months even.  While we wait for the heart call, the plan is to continue moving towards extubation.

We pray for no casts tomorrow, for her kidneys to heal and for a heart to come.

Thursday December 27, 2007

Rumors of Madeline’s demise have been greatly exaggerated. If you subscribe to the Madeline Update list, you received a reminder to tune in to the one-hour heart special that a local station (KING 5) ran, featuring Maddie. For those of you not in the Seattle area, you may have tried to catch the story at http://www.King5.com. If you read the story there, it may have upset you, because they announced that after fighting for several months in the ICU, Madeline, “Lost the battle.” We spoke to the station and they have since corrected the story. We got a few calls from the support net, but fortunately we caught it before it got out of control. The only thing Maddie has lost in the ICU so far is a tooth!

The bronchoscopy could not have gone better today. It was just as we had hoped, quick in & out with no visible signs of casts or appreciable inflammation. The Doppler study (which was actually performed yesterday while I was sleeping) revealed some good access ports in Maddie’s lower extremities, so we remain hopeful that dialysis will be a good solution while Maddie’s kidneys heal.

She did spike a significant fever today. Tylenol was administered to reduce her temperature and cultures were drawn to check for infection. No results yet.

Angry Airway from Plastic Bronchitis

Happy Airway Today

The video on the left was recorded during one of the rigid bronchoscopies several weeks ago.  Over a five day period TPA was administered and casts were expressed.  Notice the suction catheter removing plugs like a Hoover vacuum.  The video on the right still shows some mild inflammation, but is remarkably better than before.  I never would have thought that something like this could bring a tear to my eye, but it does.

Saturday December 29, 2007

Cindy writes:

Hello there. The word of the day is "weaning". Madeline is weaning down on her pressure support and weaning off some of her medications and weaning down on her nitric oxide (thankfully, since that's the one that costs about $8,500 a day) - all of this as a means to trying to get our little one extubated here sometime in the near future. Oh how good it will be to hear her once again say, "I don't like your breath!"

One very exciting thing for me today was when Dr. Law (the transplant doctor) came in to check on Maddie. After his daily examination (which in the past has been met with grimaces and somberness) he looked at me and said, "She looks amazing! I just can't believe how great she's doing!" I could have done a back flip! You know when Dr. Law is sounding optimistic that things are certainly looking up. Praise God! May she continue to just blow their minds. God is good, all the time.

Kidneys! Pray big - pray huge - pray mightily, that those pesky little things will jump start here soon!

Monday December 31, 2007

The past 48 hours have been mostly concerned with getting Madeline to sleep without dropping her blood pressure.  When she is awake and alert her pressures are normal, but as soon as she nods off the pressure plunges to an uncomfortable level.  Today we saw 45/26.  If you know what that means, then you know how nerve-racking it is.  If you don't, then trust me it is way too low for comfort.  It is incredibly difficult to treat with medicine or fluids because the moment she wakes up she returns to normal.  Yeah that's right, its weird, strange, blah, blah, blah.  One of her surgeons admitted today that she is (and I quote), "Not human.  . . . probably from Krypton."

She has had regular fever spikes for the past week and has had every conceivable point of entry tested for bugs.  So far we have received no positives from the lab.  Her antibiotic coverage has been widened as a measure to kill any infection that may be hanging around.  She continues to have a fever.  Today we sent samples to the lab to test for viruses, which means we had to switch to isolation mode (gloves, gowns and masks).  The results just came back negative, so we only had to endure isolation for part of the day.  There's a big praise God for ya!

On a good note, she has been weaning down on the vent settings.  This morning she trialed on CPAP for an hour and a half and never blinked.  There is talk of extubation soon!  How awesome will that be?!  My folks are coming next week and I would love for Maddie to be talking by then.  (just a little side prayer for yours truly).