We just received word that Aurelia has successfully underwent surgery. Dr. Spray repaired both her holes and augmented her pulmonary artery. He says that although her mitral valve appears slightly misshapen there is no leaking or backflush of blood across the valve, which is good, and it appears that whatever muscle bundles might have been in the way of her VSD repair have been cut away. Apparently, Aurelia's heart has become a little larger to compensate for the resistance placed against blood flow due to the PA band she received in October. As a result, the increase muscle tissue in her heart might have obstructed those areas the surgeons needed to access when repairing the VSDs.
The echo cardiogram taken immediately after surgery shows no leaking across the two chambers of the heart and no leaking across the valve, as I mentioned above. It also appears that her heart is successfully handling the full blood flow into and out of the lungs, and through her body. Whether or not her heart could handle pumping by itself is a concern, primarily due to the fact that her left ventricle sits right on the border line of a functioning/non-functioning ventricle (which is based on its capacity and ability to maintain adequate pressures and flow).
Currently, Aurelia is off the heart lung machine, being extubated, and undergoing an x-ray. We will be watching her carefully over the next day or two for effusion, or increased fluid around the heart, and any change in her heart's ability to handle blood flow to the lungs and body. Again, due to her heart's increased musculature the trick is going to be getting her heart to relax substantially, so that it can fill to complete capacity, and subsequently pump blood to and from the lungs to complete capacity. In the past her heart built up its strength and did not pump to capacity because of the restrictive PA band. Hopefully, though, given the medicine she is on we will see improvements over the next few days as her heart begins to fully manage the appropriate blood flow of her body.
Thank you to everyone for their prayers and well-wishes. These next 24 hours will be crucial in Aurelia's recovery, so we will keep you informed. For now Aurelia is out of the dark heart of the forest and onto the path, but the way is steep and overgrown, and fraught with briers and other dangers, so keep those prayers and positive thoughts coming.
Showing posts with label HLHS. Show all posts
Showing posts with label HLHS. Show all posts
Wednesday, July 13, 2011
Monday, November 1, 2010
Simmer down now!
It's been an interesting weekend. After Jillian's father arrived in the wee hours of Saturday morning, unexpectedly, Jillian and I hoped for a nice quiet weekend. For the most part we got what we wanted, but there are a number of unanswered questions as our sixth week begins.
Aurelia's heart continues to do well. We smile some at the irony of our stay here as her heart is doing so wonderfully that our discharge could have been weeks ago! To date there are no problems with her urinary tract and the VCUG she underwent showed no back-flush into her dead kidney at all. We are still too far out to do anything about her cleft palette and the latest brain ultrasound showed that her clot is still resolving itself and shrinking every day. These are very good things!
Her genetic evaluations have come back with nothing significant that requires any immediate therapy (if any at all) and Occupational Therapy liked her status as of last Thursday (we won't see them for some time). The Peanut's weight continues to increase and even though feeding has been a disaster she's still holding her own with only a potassium supplement to her daily nutritional fluids.
The PICC line is stable and uninfected, her peripheral line is still functioning and not painful, and the latest cardiac data on the pressures in each ventricle is good, as is the ability of her mitral valve to hold its own. The pulmonary band continues to be effective and the aortic arch reconstruction has not been rejected in any way. In fact, all of that surgery has been a success.
The treatment for her NEC has been bumped to ten days from seven, but that isn't necessarily a bad thing; her daily films show no advance of the infection, but they also do not show it lessening. Even if we *do* get the OK to start feeds again in ten days we probably won't until this airway business is completely sorted out.
The current issue on the table is Aurelia's upper and lower airway. She has had three airway collapses since she's made the move back to the CICU last Friday. She generally has one every twelve hours and only when she becomes particularly worked up. This usually follows a diaper change or a bout of hunger pain. Only one of these episodes, other than the very first which got us back into the CICU, required any kind of sedative to calm her and restore breathing function. The typical pattern for these collapses is a progressive desaturation of oxygen in her blood when she's having a fit. Then we'll see a sudden drop in her saturation levels as her airway (described as 'floppy') is unable to remain open. At this point they will slap the oxygen mask on her and pump her with anywhere between 2 to 4 liters of room air to push the airway open. They will then slowly ween her from the room air over the next few hours and she will be fine.
In the intervening time the staff suctions her nose and mouth for secretions every one to two hours. Because of her severe acid reflux (which is still being medically treated) and because they have a sump running into her stomach to keep everything out of her intestines, and because of her cleft palette, our daughter produces far more secretions in her mouth and esophagus than normal. This goop is usually withdrawn via a tube inserted through the nose into the back of the throat, or directly into the mouth and down the throat into the upper airway.
Dr. Naim is our attending again (yay!) and she'll be setting up a time for Ear/Nose/Throat to examine Aurelia's airway sometime this week. They will (or maybe not) intubate her - something that is not good for her vocal cord paresis or future eating ability - depending on how far down they have to examine her airway. Treatment can be anything from C-Pap, respirators, to temporary or lifetime tracheotomy. If we do have to go with either of the last two issues we will be here for another two months or so, give or take. Feeding at that point will be done via g-tube. This test coming up is a bit more invasive so it counts as yet another surgical procedure down in the operating room.
So that's where we are. My superintendent let me be absent from a professional development day today so that I could be here for rounds and the E/N/T consult/evaluation. We'll do our best to keep people updated.
Aurelia's heart continues to do well. We smile some at the irony of our stay here as her heart is doing so wonderfully that our discharge could have been weeks ago! To date there are no problems with her urinary tract and the VCUG she underwent showed no back-flush into her dead kidney at all. We are still too far out to do anything about her cleft palette and the latest brain ultrasound showed that her clot is still resolving itself and shrinking every day. These are very good things!
Her genetic evaluations have come back with nothing significant that requires any immediate therapy (if any at all) and Occupational Therapy liked her status as of last Thursday (we won't see them for some time). The Peanut's weight continues to increase and even though feeding has been a disaster she's still holding her own with only a potassium supplement to her daily nutritional fluids.
The PICC line is stable and uninfected, her peripheral line is still functioning and not painful, and the latest cardiac data on the pressures in each ventricle is good, as is the ability of her mitral valve to hold its own. The pulmonary band continues to be effective and the aortic arch reconstruction has not been rejected in any way. In fact, all of that surgery has been a success.
The treatment for her NEC has been bumped to ten days from seven, but that isn't necessarily a bad thing; her daily films show no advance of the infection, but they also do not show it lessening. Even if we *do* get the OK to start feeds again in ten days we probably won't until this airway business is completely sorted out.
The current issue on the table is Aurelia's upper and lower airway. She has had three airway collapses since she's made the move back to the CICU last Friday. She generally has one every twelve hours and only when she becomes particularly worked up. This usually follows a diaper change or a bout of hunger pain. Only one of these episodes, other than the very first which got us back into the CICU, required any kind of sedative to calm her and restore breathing function. The typical pattern for these collapses is a progressive desaturation of oxygen in her blood when she's having a fit. Then we'll see a sudden drop in her saturation levels as her airway (described as 'floppy') is unable to remain open. At this point they will slap the oxygen mask on her and pump her with anywhere between 2 to 4 liters of room air to push the airway open. They will then slowly ween her from the room air over the next few hours and she will be fine.
In the intervening time the staff suctions her nose and mouth for secretions every one to two hours. Because of her severe acid reflux (which is still being medically treated) and because they have a sump running into her stomach to keep everything out of her intestines, and because of her cleft palette, our daughter produces far more secretions in her mouth and esophagus than normal. This goop is usually withdrawn via a tube inserted through the nose into the back of the throat, or directly into the mouth and down the throat into the upper airway.
Dr. Naim is our attending again (yay!) and she'll be setting up a time for Ear/Nose/Throat to examine Aurelia's airway sometime this week. They will (or maybe not) intubate her - something that is not good for her vocal cord paresis or future eating ability - depending on how far down they have to examine her airway. Treatment can be anything from C-Pap, respirators, to temporary or lifetime tracheotomy. If we do have to go with either of the last two issues we will be here for another two months or so, give or take. Feeding at that point will be done via g-tube. This test coming up is a bit more invasive so it counts as yet another surgical procedure down in the operating room.
So that's where we are. My superintendent let me be absent from a professional development day today so that I could be here for rounds and the E/N/T consult/evaluation. We'll do our best to keep people updated.
Tuesday, October 5, 2010
Her next heartbeat
Jillian and I are the sort of tired happy that comes after a long car trip - happy to be at your destination, unsure of exactly how you got there, and worried about what else could possibly go wrong before the day is over.
Aurelia is amazing. I am astounded at her resiliency and fortitude. This girl was born with so many complications, so many problems, endured invasive bowel surgery during her first day and followed up that performance with open heart surgery at age eleven days. Just yesterday her sternum was cracked, her ribs spread wide, and her body frozen into submission. Today she is resting, with a half-smile on her face and covered in residual orange dye; a silly sort of October pumpkin.
The surgery yesterday took far longer than we had hoped or even considered. Aurelia was out of our hands and actually in surgery from seven-thirty in the morning to three in the afternoon. In surgery the doctors changed their game plan after they saw the situation in her heart. The initial plan included putting Aurelia on bypass and then banding her pulmonary artery before making any incisions into the actual heart (excluding the sack that exists around the muscle). When they got in to her chest, however, they recognized that the aortic arch was far more narrowed than anticipated and required their skilled attention first. Thankfully, Dr. Gruber insisted on going in her chest and his decision made sure that they widened all of the narrowed parts of`Aurelia's arch; had they done the previously mentioned side incision they would have missed part of her narrowed aorta and would have likely needed to do a second surgery.
While I am on the topic of Dr. Gruber, I want to make sure I record just how amazing it was to see him in action yesterday morning. On Sunday all of the cardiac surgeons & fellows, some fifteen in all, met and discussed Aurelia's case. Dr. Gruber chaired that meeting from Cleveland (he was at a speaking engagement) and apparently a number of the team disagreed with his assessment that we enter Aurelia's chest cavity from the front.
When we met with Dr. Pillai that Sunday afternoon the message we received was that of the dissenting team members - that Aurelia's surgery would be from the side, most likely - and we were happy with the decision to go with a less invasive procedure. At crib-side Monday morning Dr. Gruber gave us one final debriefing on the procedure and mentioned going in from the front. Jillian noticed the discrepancy immediately and made mention of the plans we heard the day before to go in through her ribcage.
Dr. Gruber underwent some sort of Fellow Transformation, reserved for only the experts in his field. First he ordered one nurse (or doctor, for that matter - nobody outranked him during those next few moments, no matter their title) to retrieve Aurelia's paperwork. He concisely, but not rudely, discredited the fellow who conferenced with us Sunday by indicating his utter displeasure with our receiving incorrect information. His words to us included, "I'm not sure what that Fellow heard or said, but there clearly was a miscommunication. The plan was never to go in through the side. There was some questioning of what our final decision would be, but I clearly ruled out going in from the side."
After he directed the many staff to clean and re-stamp Aurelia on the front of her chest he called surgery and seemed pleased that everyone in the operating room had been planning to go in through Aurelia's chest. Thankfully the repair to Aurelia's arch, facilitated by that decision to open her chest, went without incident. I have such a hard time trying to imagine my daughter laying on an operating table with her chest open wide and blue from the cold that has shut down all the major systems of her body. I just can't fathom what it looked like to watch her heart slow and slow one beat at a time until it sat silent and still. I cry thinking about her there, her body dead and a machine carrying precious oxygen and blood to her brain and limbs.
The surgical team moved quickly after that, repairing the band and slowly moving Aurelia off bypass. At this point the first of the challenges facing Aurelia (as if all that came before was easy!) manifested itself in the form of excessive bleeding. Throughout any complicated surgery like this many nicks and cuts are made in the body, along veins and organs and muscle and tissue, but very rarely is bleeding seen by the surgical team. A body in Aurelia's state is so cold that you can't see the amount of bleeding until they begin to take her off bypass and warm her up, waking her heart from its slumber.
Aurelia underwent a series of cooling procedures over two and a half hours to slow the excessive bleeding inside her chest cavity. These procedures amounted to literally filling Aurelia with ice, waiting fifteen minutes, warming her up again, checking for bleeding and re-applying ice if needed. Eventually Aurelia's leaks sealed themselves and the doctors made sure that all the vents and lines into her firmly lay in place. At that point Aurelia had a drainage line installed, two pacemaker wires, an IV directly to her chest cavity and heart, another IV in her foot, and an arterial line into her belly button.
We met with Dr. Gruber after the surgery for his recounting of the surgery and he showed enthusiasm and pleasure with Aurelia's progress. Other than her bleeding he felt confident that she would emerge from the procedure with much improved breathing and heart function. Dr. Gruber decided to set the pulmonary artery function at 90% its original and he avoided looking for other potential problems with Aurelia's mitral valve and her existing muscular VSD (the large hole between her left and right ventricle). Jillian and I were happy with the outcome and Dr. Gruber's decision to avoid looking for further problems with Aurelia's valve and hole, because both may improve themselves over time and because the valve may actually not have much of a problem once her heart regains strength.
When we first saw Aurelia we were thrilled. As scary and difficult as it was to look at her I will never forget the joy in my heart at that moment. All that afternoon and evening I felt like running and hugging and kissing everyone I saw, from Dr. Ince to the admitting orderly out by the entrance to the CICU. Jillian felt more than mild embarrassment at my excitement and expression. I don't blame her. It's quite possible that I mortified not only Jillian, but Aurelia, too.
After about one hour the bleeding from Aurelia's chest cavity slowed to barely a trickle and we started to face the second and far worse concern with her surgery, this time revolving around urine. When put on bypass and our temperatures lowered our systems shut down one by one. As we come back to life our body slowly regains heat (Aurelia's temperature still hadn't reached normal two hours after her surgery finished) in controlled increments and the body's systems begin to reboot one by one. We skipped over any trouble with Aurelia's respiratory system by having her on the machine that took care of that process for her, her bleeding in her circulatory system had slowly come under control, her nervous system seemed to be intact, she hadn't suffered any strokes, and brain function appeared normal.
Unfortunately, prior to, during, and after surgery Aurelia hadn't passed any urine through her catheter. In any normal person this wouldn't raise much of a concern, but with only one kidney the chance of system shock and failure meant life or death for our baby girl. Dr. Ince, whose praises I sang before, kept a measured response and reassured Jillian and I that this sort of delay did not mean anything out of the ordinary. Stressed nonetheless we felt increasing tension as the minutes went by and as the staff began discussing their response to a kidney failure. The sheer mention of 'what if' scenarios had me in near panic. All I wanted for her was to pee. I have never, ever, wanted anyone to pee so badly in my life.
She kept us waiting nearly two hours, the cut off point for 'no concern' we later found out, until we saw a few drops in her catheter. I about jumped for joy at the sight of a yellow droplet in her tube. This seems silly, I suppose, but looking at her bruised chest, swollen face and arms, and knowing that all that fluid she had been infused with during surgery had to escape, I think I actually felt more fear than I did when they wheeled her away for the operation earlier that morning.
We stayed late last evening with her and left her happy that she once again faced down tough odds and beat them with courage and tenacity. Though a machine was breathing for her and her chest still bled some, and despite the low urine output, Aurelia inspired strength and hope in us that we needed to fight back the worry and concern we felt all day.
I confess that at points during the day I let my mind drift away to places dark and sad, wondering what it would be like to lose her. We agreed to participate in a genetic study before Aurelia's surgery, permitting the CHOP staff to keep tissue and blood samples from Aurelia's heart permanently. Dr. Gruber and his team will use the sample (amongst many others) to try and identify the genetic cause behind HLHS. It is suspected that the disease is genetic, but much more work needs to be done before a specific cause is identified. I found a minuscule measure of comfort knowing that even if she died, Aurelia's heart and blood could still help someone else at risk of HLHS. I even felt good knowing that if Aurelia's DNA showed anything even remotely useful that Jillian and I could then give our own DNA and blood to support scholarship and research into the defect.
As for today, other than one minor scare, Aurelia continues to do well. I'll give a more detailed post a little later, but I know folks have been waiting for details so I wanted to write this before my memory lost pieces of Aurelia's journey.
Thank you everyone, for all your positive thoughts and prayers and love. Your support has resulted in immeasurable strength needed and used by Jillian and me. We love you.
Aurelia is amazing. I am astounded at her resiliency and fortitude. This girl was born with so many complications, so many problems, endured invasive bowel surgery during her first day and followed up that performance with open heart surgery at age eleven days. Just yesterday her sternum was cracked, her ribs spread wide, and her body frozen into submission. Today she is resting, with a half-smile on her face and covered in residual orange dye; a silly sort of October pumpkin.
The surgery yesterday took far longer than we had hoped or even considered. Aurelia was out of our hands and actually in surgery from seven-thirty in the morning to three in the afternoon. In surgery the doctors changed their game plan after they saw the situation in her heart. The initial plan included putting Aurelia on bypass and then banding her pulmonary artery before making any incisions into the actual heart (excluding the sack that exists around the muscle). When they got in to her chest, however, they recognized that the aortic arch was far more narrowed than anticipated and required their skilled attention first. Thankfully, Dr. Gruber insisted on going in her chest and his decision made sure that they widened all of the narrowed parts of`Aurelia's arch; had they done the previously mentioned side incision they would have missed part of her narrowed aorta and would have likely needed to do a second surgery.
While I am on the topic of Dr. Gruber, I want to make sure I record just how amazing it was to see him in action yesterday morning. On Sunday all of the cardiac surgeons & fellows, some fifteen in all, met and discussed Aurelia's case. Dr. Gruber chaired that meeting from Cleveland (he was at a speaking engagement) and apparently a number of the team disagreed with his assessment that we enter Aurelia's chest cavity from the front.
When we met with Dr. Pillai that Sunday afternoon the message we received was that of the dissenting team members - that Aurelia's surgery would be from the side, most likely - and we were happy with the decision to go with a less invasive procedure. At crib-side Monday morning Dr. Gruber gave us one final debriefing on the procedure and mentioned going in from the front. Jillian noticed the discrepancy immediately and made mention of the plans we heard the day before to go in through her ribcage.
Dr. Gruber underwent some sort of Fellow Transformation, reserved for only the experts in his field. First he ordered one nurse (or doctor, for that matter - nobody outranked him during those next few moments, no matter their title) to retrieve Aurelia's paperwork. He concisely, but not rudely, discredited the fellow who conferenced with us Sunday by indicating his utter displeasure with our receiving incorrect information. His words to us included, "I'm not sure what that Fellow heard or said, but there clearly was a miscommunication. The plan was never to go in through the side. There was some questioning of what our final decision would be, but I clearly ruled out going in from the side."
After he directed the many staff to clean and re-stamp Aurelia on the front of her chest he called surgery and seemed pleased that everyone in the operating room had been planning to go in through Aurelia's chest. Thankfully the repair to Aurelia's arch, facilitated by that decision to open her chest, went without incident. I have such a hard time trying to imagine my daughter laying on an operating table with her chest open wide and blue from the cold that has shut down all the major systems of her body. I just can't fathom what it looked like to watch her heart slow and slow one beat at a time until it sat silent and still. I cry thinking about her there, her body dead and a machine carrying precious oxygen and blood to her brain and limbs.
The surgical team moved quickly after that, repairing the band and slowly moving Aurelia off bypass. At this point the first of the challenges facing Aurelia (as if all that came before was easy!) manifested itself in the form of excessive bleeding. Throughout any complicated surgery like this many nicks and cuts are made in the body, along veins and organs and muscle and tissue, but very rarely is bleeding seen by the surgical team. A body in Aurelia's state is so cold that you can't see the amount of bleeding until they begin to take her off bypass and warm her up, waking her heart from its slumber.
Aurelia underwent a series of cooling procedures over two and a half hours to slow the excessive bleeding inside her chest cavity. These procedures amounted to literally filling Aurelia with ice, waiting fifteen minutes, warming her up again, checking for bleeding and re-applying ice if needed. Eventually Aurelia's leaks sealed themselves and the doctors made sure that all the vents and lines into her firmly lay in place. At that point Aurelia had a drainage line installed, two pacemaker wires, an IV directly to her chest cavity and heart, another IV in her foot, and an arterial line into her belly button.
We met with Dr. Gruber after the surgery for his recounting of the surgery and he showed enthusiasm and pleasure with Aurelia's progress. Other than her bleeding he felt confident that she would emerge from the procedure with much improved breathing and heart function. Dr. Gruber decided to set the pulmonary artery function at 90% its original and he avoided looking for other potential problems with Aurelia's mitral valve and her existing muscular VSD (the large hole between her left and right ventricle). Jillian and I were happy with the outcome and Dr. Gruber's decision to avoid looking for further problems with Aurelia's valve and hole, because both may improve themselves over time and because the valve may actually not have much of a problem once her heart regains strength.
When we first saw Aurelia we were thrilled. As scary and difficult as it was to look at her I will never forget the joy in my heart at that moment. All that afternoon and evening I felt like running and hugging and kissing everyone I saw, from Dr. Ince to the admitting orderly out by the entrance to the CICU. Jillian felt more than mild embarrassment at my excitement and expression. I don't blame her. It's quite possible that I mortified not only Jillian, but Aurelia, too.
After about one hour the bleeding from Aurelia's chest cavity slowed to barely a trickle and we started to face the second and far worse concern with her surgery, this time revolving around urine. When put on bypass and our temperatures lowered our systems shut down one by one. As we come back to life our body slowly regains heat (Aurelia's temperature still hadn't reached normal two hours after her surgery finished) in controlled increments and the body's systems begin to reboot one by one. We skipped over any trouble with Aurelia's respiratory system by having her on the machine that took care of that process for her, her bleeding in her circulatory system had slowly come under control, her nervous system seemed to be intact, she hadn't suffered any strokes, and brain function appeared normal.
Unfortunately, prior to, during, and after surgery Aurelia hadn't passed any urine through her catheter. In any normal person this wouldn't raise much of a concern, but with only one kidney the chance of system shock and failure meant life or death for our baby girl. Dr. Ince, whose praises I sang before, kept a measured response and reassured Jillian and I that this sort of delay did not mean anything out of the ordinary. Stressed nonetheless we felt increasing tension as the minutes went by and as the staff began discussing their response to a kidney failure. The sheer mention of 'what if' scenarios had me in near panic. All I wanted for her was to pee. I have never, ever, wanted anyone to pee so badly in my life.
She kept us waiting nearly two hours, the cut off point for 'no concern' we later found out, until we saw a few drops in her catheter. I about jumped for joy at the sight of a yellow droplet in her tube. This seems silly, I suppose, but looking at her bruised chest, swollen face and arms, and knowing that all that fluid she had been infused with during surgery had to escape, I think I actually felt more fear than I did when they wheeled her away for the operation earlier that morning.
We stayed late last evening with her and left her happy that she once again faced down tough odds and beat them with courage and tenacity. Though a machine was breathing for her and her chest still bled some, and despite the low urine output, Aurelia inspired strength and hope in us that we needed to fight back the worry and concern we felt all day.
I confess that at points during the day I let my mind drift away to places dark and sad, wondering what it would be like to lose her. We agreed to participate in a genetic study before Aurelia's surgery, permitting the CHOP staff to keep tissue and blood samples from Aurelia's heart permanently. Dr. Gruber and his team will use the sample (amongst many others) to try and identify the genetic cause behind HLHS. It is suspected that the disease is genetic, but much more work needs to be done before a specific cause is identified. I found a minuscule measure of comfort knowing that even if she died, Aurelia's heart and blood could still help someone else at risk of HLHS. I even felt good knowing that if Aurelia's DNA showed anything even remotely useful that Jillian and I could then give our own DNA and blood to support scholarship and research into the defect.
As for today, other than one minor scare, Aurelia continues to do well. I'll give a more detailed post a little later, but I know folks have been waiting for details so I wanted to write this before my memory lost pieces of Aurelia's journey.
Thank you everyone, for all your positive thoughts and prayers and love. Your support has resulted in immeasurable strength needed and used by Jillian and me. We love you.
Monday, October 4, 2010
Mid-surgery update
Just a quick mid-surgery update:
Pulmonary band is in place. Aortic bridge is sliced and enlarged with the transplanted material. All the lines are in place on her.
Her chest is still open and they are waiting for the bleeding to slow. Her bleeding rate right now is 'normal' as far as that goes, but it still worries the sh*t out of me. Yes, I said sh*t. When you do this type of surgery with this many sutures, then there is a good bit of 'leaking'. Once the bleeding slows they will close her up.
More news as we get it from our nurse.
Pulmonary band is in place. Aortic bridge is sliced and enlarged with the transplanted material. All the lines are in place on her.
Her chest is still open and they are waiting for the bleeding to slow. Her bleeding rate right now is 'normal' as far as that goes, but it still worries the sh*t out of me. Yes, I said sh*t. When you do this type of surgery with this many sutures, then there is a good bit of 'leaking'. Once the bleeding slows they will close her up.
More news as we get it from our nurse.
I'm not even sure...
I'm not even sure what to write about today. Aurelia has gone in for her surgery and is very likely just now undergoing her first incisions. We stayed the night here at the hospital, cramming ourselves into a single bed so that we could be here for our girl. Jillian pumped crib-side and I managed to get all the materials finished for today's, tomorrow's and Wednesday's lessons so when the early morning rose we could spend as much time with her as possible.
Our morning was quiet and tense, nervous and upsetting, and full of loving moments. We whispered affections to Aurelia and held her tight in our hands. I felt her hand on my face, kissed her little lips and nose, and caressed her cheeks and forehead. I cried at her smile and smiled at her little pout. Jillian and I split our time this morning holding her and covering her with kisses.
Her surgery has changed somewhat since we last conferred with cardiology. Today they will go in through her chest (we thought as of yesterday that the incision would be in her side), they will insert a variety of tubes and lines and wires into her before they begin the surgery. She will have several wires laid upon her chest to monitor any potential disrhythmia. She will also have a line placed into her wrist to function as her IV, and a drain inserted into her chest cavity. There is a possibility that they will insert a line directly into her heart to function as an IV if it is needed. She also had an arterial line put into her belly button and of course a catheter.
The plan is to place a band around her pulmonary artery to restrict the amount of blood flowing into her lungs (which leads to fluid build up and problems or troubles breathing and eating) and then make an incision along her tiny aortic bridge, spread it apart and graph it together with donated tissue (called a homeograph). If that tissue doesn't take then they will use a synthetic material. Of course before any of this can be done she has to be put on full bypass and transfused with a variety of blood products to thin her blood, then help it clot later in the surgery.
Essentially they stop all blood flow to the heart, drop her temperature to about 17 degrees celsius, or 62 degrees Fahrenheit, so that her heart slows to what is in essence a stop. Once her heart is ready for the first incision they will work on the aorta. They will also be collecting small parts of her heart that are typically discarded during surgery and using them in a study which we will talk about at a later time.
I forgot to mention that they will have to open up the sack that normally surrounds the heart and they will actually leave that open and insert what amount to several man made valves into her heart that they will then keep there to use in future surgeries or her recovery, should they need them. Ideally, Aurelia's sternum will still be more cartilage than bone so that they will be able to pack it with what amounts to a paste to help the bone meld together again. If her sternum is all bone they will then have to wire the bone with a non-magnetic wire that they will then remove for her second surgery.
Our nurse just came in for our second update since she's been gone, and Aurelia has been undergoing the actual repair for about twenty minutes. Things are going according to plan. That's about all we have for now, aside from red-eyes and headaches. We'll keep you all updated as we can.
Our morning was quiet and tense, nervous and upsetting, and full of loving moments. We whispered affections to Aurelia and held her tight in our hands. I felt her hand on my face, kissed her little lips and nose, and caressed her cheeks and forehead. I cried at her smile and smiled at her little pout. Jillian and I split our time this morning holding her and covering her with kisses.
Her surgery has changed somewhat since we last conferred with cardiology. Today they will go in through her chest (we thought as of yesterday that the incision would be in her side), they will insert a variety of tubes and lines and wires into her before they begin the surgery. She will have several wires laid upon her chest to monitor any potential disrhythmia. She will also have a line placed into her wrist to function as her IV, and a drain inserted into her chest cavity. There is a possibility that they will insert a line directly into her heart to function as an IV if it is needed. She also had an arterial line put into her belly button and of course a catheter.
The plan is to place a band around her pulmonary artery to restrict the amount of blood flowing into her lungs (which leads to fluid build up and problems or troubles breathing and eating) and then make an incision along her tiny aortic bridge, spread it apart and graph it together with donated tissue (called a homeograph). If that tissue doesn't take then they will use a synthetic material. Of course before any of this can be done she has to be put on full bypass and transfused with a variety of blood products to thin her blood, then help it clot later in the surgery.
Essentially they stop all blood flow to the heart, drop her temperature to about 17 degrees celsius, or 62 degrees Fahrenheit, so that her heart slows to what is in essence a stop. Once her heart is ready for the first incision they will work on the aorta. They will also be collecting small parts of her heart that are typically discarded during surgery and using them in a study which we will talk about at a later time.
I forgot to mention that they will have to open up the sack that normally surrounds the heart and they will actually leave that open and insert what amount to several man made valves into her heart that they will then keep there to use in future surgeries or her recovery, should they need them. Ideally, Aurelia's sternum will still be more cartilage than bone so that they will be able to pack it with what amounts to a paste to help the bone meld together again. If her sternum is all bone they will then have to wire the bone with a non-magnetic wire that they will then remove for her second surgery.
Our nurse just came in for our second update since she's been gone, and Aurelia has been undergoing the actual repair for about twenty minutes. Things are going according to plan. That's about all we have for now, aside from red-eyes and headaches. We'll keep you all updated as we can.
Sunday, September 26, 2010
A Whirlwind Day
I'm not even sure where to begin with today's post. I know people are expecting some updates, and I know that it's not required of me to post every day, or multiple times a day, but something just compels me to expunge some of this into writing to help me sleep or relax or whatever.
Aurelia is doing well today. The funny thing is I can't even remember what I've passed on and what I haven't to everyone that wants to know about her story. It's absolutely exhausting when you're working to keep tabs on what feels like a million different departments, specialists, lab results, doctors, and changes in your baby's status. I'll try to give a comprehensive update of where Aurelia is with all her issues, hoping that I don't dull your senses with things I've said already, but give you the new information that hasn't made it out of my head yet.
Aurelia's blood clot has disappeared for now and this is a wonderful wonderful thing. It will allow her to receive blood thinners and be put on bypass for her heart surgery and it means one less course of action we have to take to correct her cardiac function. The neurologists will consult with us again tomorrow about the status of her clot (as well as any impact her enlarged #2 ventricle will have on her development, if any) and I wouldn't be surprised if they schedule her for another MRI before she goes into surgery for her heart. The neurologists will also be working with the ophthalmologists and the geneticists to determine what, if any, genetic abnormality or syndrome is at the root cause of all these troubles.
To that end, the ophthalmologists and the geneticists will likely consult tomorrow as well. They've done some preliminary work, but I wouldn't be surprised if they do more work tomorrow, or even schedule Aurelia for tests to try and get at what might be causing all these troubles. It's quite plausible that Aurelia has several defects among her chromosomes and that combined they each result in the many problems she faces. This 'syndrome' would have no name per say, but would explain why so many things are going wrong in her physical development.
The urologists consulted on Aurelia this morning and between them and the kidney specific teams they have decided to leave well enough alone and let her kidney stay inside her at the moment. It's quite possible that her kidney will die and shrivel up inside her. Either way, those kidneys are prone to infection and as a result she will be on antibiotics for her entire life (or as long as the surgical team decides to leave the kidney in place, I suppose. There is a test they will perform at about one month of age, and that will clarify the course of action they choose to take, but that is still weeks off, of course.
Aurelia continues to recover well from her surgery the other day. She has shown no signs of internal bleeding and she has already had several bowel movements. This is key, as anytime your intestines are operated upon they often shut down for a period of time (up to a week!), but in Aurelia's case they are up and running again. They are still running suction into her stomach to ease the burden on her newly reorganized and re-routed digestive track and they will keep her off food for one week as they prepare her body for surgery. Thankfully, she has not yet been fed any food (other than nutrients through her umbilical lines) so she does not yet know what it means to be full or hungry.
Her heart surgery is scheduled for October 4 (her original due date) and it will be a modified Norwood procedure. In children with HLHS the first procedure, called a Norwood, widens the pulmonary artery, separating the main pulmonary artery from the left and right portions and joining it with the upper portion of the (typically smaller) aorta. This allows a mixture of oxygenated and non-oxygenated blood to move through the body, pumped by the one remaining good ventricle.
A man-made shunt (think of a canal connecting two existing rivers) is then installed on the subclavian artery to connect it to the pulmonary artery. This allows the single ventricle to move the non-oxygenated blood into and out of the lungs and into the body's circulatory system. I've included an image below to help make some sense of the medical jargon.
In Aurelia's case the left ventricle is still present (a victory, as in most HLHS kids the ventricle is not functioning) and her mitral valve and aortic valve are smaller, but still considered adequate for life. In most HLHS kids, these valves are not functioning, or will not function normally.
This means that Aurelia will have a more hybrid procedure, which will place a stent in the aortic bridge and a band around the enlarged pulmonary artery to prevent over-circulation to her lungs. It's also hoped that this band will help trick her heart into repairing and closing the existing hole in her heart between her right and left ventricles.
If all goes according to plan, Aurelia will do well and hopefully begin to eat and put on weight during her recovery.
To that end, I should mention that Aurelia's cleft palette is not a concern for her eating at this point, and it is so far down the list of issues that we are facing that we haven't even talked about getting a consult on what to do to fix the problem. I suppose that will simply be a bridge we cross when we get there.
Jillian and I also agreed to be part of a study at CHOP for parents. It is called, C.O.P.E. and it is designed to scaffold parents into care for their congenital heart defect baby. It's a new program designed to show parents the many similarities (and of course the differences) between a baby born prematurely or a long-term NICU baby and those babies with cardiac difficulties similar to Aurelia's. For instance, both share some very different sensory experiences and both may experience developmental delays as they age. We'll probably write more about this program soon, but we're very excited to be a part of our first study at CHOP - as parents. To us, this is one of the things that makes CHOP different; it isn't just about the patients, but also their families as they struggle to adapt to life with a congenital heart defect child.
Similarly we also have arranged for Madison to meet with a life counselor who will work with her in the coming weeks to handle the transition to having a younger sister. While often difficult enough for older siblings who have a newly arrived younger brother and sister, having a new arrival in the house that has very specific needs and requires additional attention can be even more challenging. Above all we want to be sure that Madison's emotions and experiences are validated, that no unfair expectations are placed on her, and that she is permitted to create a relationship with her little sister that is genuine, and not hurried or pushed by us or medical concerns.
It's going to be a tough road, but we'll get there!
Aurelia is doing well today. The funny thing is I can't even remember what I've passed on and what I haven't to everyone that wants to know about her story. It's absolutely exhausting when you're working to keep tabs on what feels like a million different departments, specialists, lab results, doctors, and changes in your baby's status. I'll try to give a comprehensive update of where Aurelia is with all her issues, hoping that I don't dull your senses with things I've said already, but give you the new information that hasn't made it out of my head yet.
Aurelia's blood clot has disappeared for now and this is a wonderful wonderful thing. It will allow her to receive blood thinners and be put on bypass for her heart surgery and it means one less course of action we have to take to correct her cardiac function. The neurologists will consult with us again tomorrow about the status of her clot (as well as any impact her enlarged #2 ventricle will have on her development, if any) and I wouldn't be surprised if they schedule her for another MRI before she goes into surgery for her heart. The neurologists will also be working with the ophthalmologists and the geneticists to determine what, if any, genetic abnormality or syndrome is at the root cause of all these troubles.
To that end, the ophthalmologists and the geneticists will likely consult tomorrow as well. They've done some preliminary work, but I wouldn't be surprised if they do more work tomorrow, or even schedule Aurelia for tests to try and get at what might be causing all these troubles. It's quite plausible that Aurelia has several defects among her chromosomes and that combined they each result in the many problems she faces. This 'syndrome' would have no name per say, but would explain why so many things are going wrong in her physical development.
The urologists consulted on Aurelia this morning and between them and the kidney specific teams they have decided to leave well enough alone and let her kidney stay inside her at the moment. It's quite possible that her kidney will die and shrivel up inside her. Either way, those kidneys are prone to infection and as a result she will be on antibiotics for her entire life (or as long as the surgical team decides to leave the kidney in place, I suppose. There is a test they will perform at about one month of age, and that will clarify the course of action they choose to take, but that is still weeks off, of course.
Aurelia continues to recover well from her surgery the other day. She has shown no signs of internal bleeding and she has already had several bowel movements. This is key, as anytime your intestines are operated upon they often shut down for a period of time (up to a week!), but in Aurelia's case they are up and running again. They are still running suction into her stomach to ease the burden on her newly reorganized and re-routed digestive track and they will keep her off food for one week as they prepare her body for surgery. Thankfully, she has not yet been fed any food (other than nutrients through her umbilical lines) so she does not yet know what it means to be full or hungry.
Her heart surgery is scheduled for October 4 (her original due date) and it will be a modified Norwood procedure. In children with HLHS the first procedure, called a Norwood, widens the pulmonary artery, separating the main pulmonary artery from the left and right portions and joining it with the upper portion of the (typically smaller) aorta. This allows a mixture of oxygenated and non-oxygenated blood to move through the body, pumped by the one remaining good ventricle.
A man-made shunt (think of a canal connecting two existing rivers) is then installed on the subclavian artery to connect it to the pulmonary artery. This allows the single ventricle to move the non-oxygenated blood into and out of the lungs and into the body's circulatory system. I've included an image below to help make some sense of the medical jargon.
This means that Aurelia will have a more hybrid procedure, which will place a stent in the aortic bridge and a band around the enlarged pulmonary artery to prevent over-circulation to her lungs. It's also hoped that this band will help trick her heart into repairing and closing the existing hole in her heart between her right and left ventricles.
If all goes according to plan, Aurelia will do well and hopefully begin to eat and put on weight during her recovery.
To that end, I should mention that Aurelia's cleft palette is not a concern for her eating at this point, and it is so far down the list of issues that we are facing that we haven't even talked about getting a consult on what to do to fix the problem. I suppose that will simply be a bridge we cross when we get there.
Jillian and I also agreed to be part of a study at CHOP for parents. It is called, C.O.P.E. and it is designed to scaffold parents into care for their congenital heart defect baby. It's a new program designed to show parents the many similarities (and of course the differences) between a baby born prematurely or a long-term NICU baby and those babies with cardiac difficulties similar to Aurelia's. For instance, both share some very different sensory experiences and both may experience developmental delays as they age. We'll probably write more about this program soon, but we're very excited to be a part of our first study at CHOP - as parents. To us, this is one of the things that makes CHOP different; it isn't just about the patients, but also their families as they struggle to adapt to life with a congenital heart defect child.
Similarly we also have arranged for Madison to meet with a life counselor who will work with her in the coming weeks to handle the transition to having a younger sister. While often difficult enough for older siblings who have a newly arrived younger brother and sister, having a new arrival in the house that has very specific needs and requires additional attention can be even more challenging. Above all we want to be sure that Madison's emotions and experiences are validated, that no unfair expectations are placed on her, and that she is permitted to create a relationship with her little sister that is genuine, and not hurried or pushed by us or medical concerns.
It's going to be a tough road, but we'll get there!
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