Tuesday, December 15, 2009


"A moment in our arms, forever in our hearts"

Gabriel Lee Aguilar
July 1, 2009
November 16, 2009

Join us as we remember and celebrate our precious Super G, who so deeply touched and blessed our lives.

Saturday, January 16, 2010
~Memorial Service 11:00am
~Reception immediately following

Twin Lakes Church
2701 Cabrillo College Drive
Aptos, CA 95003

Friday, December 11, 2009

December 11, 2009

Breathe in...breathe out...one foot infront of the other...one day at a time...

It has been almost one month since we walked out the front doors of the LPCH NICU, without our Gabriel. It's hard to put into words how we made it out of the hospital that night, towing behind us a wagon full of Gabe's stuff, but no Gabe. I always knew it was going to take a miracle to fix G's body, but I always, always hoped it was possible. I had too, how can you ever give up on your child?

I will always remember November 16, 2009 as the most horrible and most beautiful day of my life. We took Gabe up to the roof of the Hospital. We found a spot under a tree with lovely purple flowers, and there, with Jesse by my side, I cradled my son in my arms. It was the first time I had ever held my son without tubes and wires inbetween us. It was the first time I could stare into his beautiful face without anything blocking my view. It was only then, seeing Gabe more at peace and more content then I'd ever seen him before, that I realized how much he had gone through. I've always known that we pushed Gabe and his body, but it wasn't until that moment, on the roof, that I saw the real Gabriel. He wasn't in pain anymore, and I knew we had made the right decision. We snuggled Gabe close and told him how much we loved him; how proud of him we were for the battle he had fought; and that it was ok to go now. Gabriel took several breaths on his own, and then passed quickly, quietly, and peacefully.

How do you go forward in life after loosing a piece of your heart? We haven't figured this one out yet? I'm not sure that the pain ever goes away, I think you learn how to live with it. Some days are worse than others, and sometimes there are tears, and sometimes there are smiles, when we think about this beautiful little boy that forever changed us. Because of Gabe, I will never be able to look at life the same again. In Gabe's short time on this earth, he opened my eyes to what is truly important, and for that I will be forever greatful. He will always be our son; we will always talk about him; his pictures will always be scattered about our house; and his stocking will always hang with ours at Christmas time, just as it is right now.

Gabriel will forever be my Super G and my inspiration for life.

Thursday, November 19, 2009

Gabriel Lee Aguilar
July 1, 2009
November 16, 2009

Saturday, November 14, 2009

Our Angel

In a few days our precious son Gabriel will have his Angel wings.

After receiving devistating news on Friday, about Gabe's brain, we have decided it's time to let our son go. This is the most difficult decision we have ever made. The thought of saying good bye to our baby, leaves an excruciating pain ripping through our hearts. Jesse and I must do what is best for Gabriel though, not us, and we feel we have made the right decision.

Thank you for respecting our space, as we try to collect ourselves and figure out how to move forward in life, with a piece of our hearts forever missing.

Gabriel, you are Mommy and Daddy's hero!

Monday, November 9, 2009

November 9, 2009

I like to think of myself as an optimistic person. My cup is usually always at least half full. Over the last four months I have some how been able to stay rather upbeat about everything going on with Gabe. Always realistic and aware of the worst case scenerio, but able to stay strong and hope for the best. The last few weeks have been very dark for me. I am having trouble looking at everything regarding Gabe, and feel much hope. I know this sounds absolutely horrible. Especially coming from a mom, but this is where I am right now. Jesse and I have always been, and will continue to be, Gabe's biggest and loudest cheerleaders. I will never give up on him. He is a piece of my heart, and my heart aches non-stop 24/7 for my son. The cruel reality of all this is, Gabe's body did not form in a way that allows it to be self-sustaining. If it wasn't for modern medicide and all of the fabulous machines out there, Gabe would not have made it out of the delivery room alive. We are working against mother nature and trying to fix this little body, which quite honestly, might not be fixable. We are not ready to throw the towel in yet, but this battle is getting really really scary, and we are getting to the point where we may need to start trying some of the last resort medical interventions to see if they can heal Gabriel. His body does not seem to be able to heal on its own.

On Saturday, 400mls of fluid drained from Gabe's chest tubes (mostly the left side), and on Sunday 300mls came out. If G's lymphatic system was capable of healing itself we should be seeing much smaller numbers coming out at this point. With 100's of cc's of fluid coming out a day we need to start talking about pleurodesis (chemical introduced into the pleural space causing irritation and scaring of the sack around the lungs and the chest wall, which makes them stick together closing off the space between them, which should prevent fluid from building up between them. Of course the fluid could begin leaking somewhere else in the body). We first want to find out if there are any other imaging options for the lymphatic system. It is an extremely small system running throughout the body and is almost impossible to see. If we could some how get a look at it perhaps we could see where the leak(s) is coming from. I was Googling the other day and found a study where a group in Japan used MRL (magnetic resonance lymphography) to do some studies on the thoracic duct. This is a new form of MR imaging. I told of couple of G's surgeons about it and they mentioned it to radiology. Apparently it peaked the interest of one of the radiologists, so we will see what happens here. This new imaging could definitely put Lucile Packard Children's Hospital on the map, and we would most certainly be open to Gabe being the first one to try it out. If it worked, it could be a win win for us and LPCH.

On Friday, Gabe was in a good enough place to try CPAP again. The transition went much smoother then it did two weeks ago, so it was a good start. Gabe started out breathing a little fast, but then settled down. His CO2 rose, but then started dropping back down. Jesse and I stood by Gabe's side, holding his hands, and cheering him on. As time passed Gabe began to breath faster and faster. A blood gas was taken, and his CO2 wasn't much higher than it had been when he was on the vent. He was having to work really hard, but was able to blow off enough CO2 on his own. I cannot even begin to describe how exciting and scary this experience was. I will never again take a single breath for granted. We watched while the hours crept past. There was one point when we thought he was going out, but Nurse "L" jumped in a worked her magic and G was up and going again. There were several times when Gabe started to get really agitated. When this happened his O2 saturation dropped and his CO2 levels went up. Jesse, Nurse "L," and I worked hard to calm him back down. After about 3-1/2 hours deminished breaths sounds were noted on the left side and an x-ray was taken. It showed a compromised left lung. Whether it was collapse or fluid building up in the left pleural space, we weren't sure? At change of shift we stepped out to grab a quick bite to eat. When we got back Gabe was still huffing and puffing, but seemed to be resting comfortably. More hours ticked by, and at 10pm we headed home to relieve Grandma Reynolds from toddler duty. Shortly after we left, Gabe had a major desat and went out. Nurse "S" got him back quickly. The team wanted to reintibate at this point, but Nurse "S" said no. She got him all fixed up and he was back at it. At 5am I received a call from Nurse "S" letting us know that Gabe was reintibated at 4:30am. Gabe had gone for 14 hours and 10 minutes. We are very proud our little guy. This was a huge battle for Gabe. We are so very appreciative of Nurse "L" and Nurse "S!" If it wasn't for them and all of the work they did, Gabe would not have made it as long as he did.

Gabe walked away from his 14 hours and 10 minutes of breathing with a collaped left lung, high CO2 levels and complete exhaustion. He has spent most of the last three days sleeping and letting the vent do all of the work for him. We are excited about this marathon Gabe ran, but it did not bring us much relief. Gabe is four months old, and has lungs that can barely support him for 14 hours of breathing, and unfortunately we cannot give his lungs all the credit for the work. He had high pressures of air being forced through his nose and very high levels of sedation on board to keep him calm. It is taking him several days to recover from breathing. His left lung has reinflated, his CO2 levels are back down, they have been able chip away at the higher vent settings he required after CPAP, and start weaning on his sedation again. This experience, of breathing, took such a tole of Gabe his electrolytes are now all out of wack, and overall it pushed him backwards. He is recovering, but breathing, the most basic of life's necessities, is not an easy task for Gabe. He has small lungs, and now, more than ever, we wonder if they will ever be able to work on their own. If they are capable, it is going to take many months to get them going. A trach has once again been laid out of the table, as a stepping stone in this journey of breathing.

Jesse and I will sit down with Dr "B" this week to have a very serious heart-to-heart about Gabe. Like I said before, we are not ready to surrender in this battle for our son. We want to exhaust all options and give Gabe the best chance possible at life, but if machines are the only way we can keep him alive, and he has to spend his life in a care facility...that is not living. More than anything in this world I want to put Gabe in his car seat and bring him home. I want to take him outside to hear the birds and the wind blowing through the tress. I want Kayla to have her little brother to torture with tea parties and share a lifetime of memories with. Just the other day, out of nowhere, she said: "I want to hold Gabriel." My heart has never ached so badly.

Everyone is always saying they wish there was something they could do for us. Here is what you can do: never take another day for granted; if you love someone, tell them; if you are holding onto a grudge, let it go; don't put off until tomorrow, what can be done today; and always, always, take the time to stop and smell the roses. Life is short and unpredictable. Please take time to enjoy every breath you are blessed with.

Thursday, November 5, 2009

November 4-5, 2009

November 4: Gabe spent the first part of the morning awake and hanging out with Nurse "N." He started dosing off for a nap around 10am, which was of course just when I arrived. I was looking forward to "playing" with Gabe when he woke up from his late morning nap, but apparently it was a sleepy day, becasue G spent most of the day napping. There were only very short periods where I got to see him awake. That's ok, if I had the option of sleeping all day, I just might. Since Gabe has been pretty stable for the last few days they weaned his Versed, Hydracortisone and his vent settings. He handled all of these changes well, and had a good night.

November 5: When Nurse "L" arrived this morning, Gabe was quickly becoming a wild man. His temp was up, and he was not happy. She removed the blanket covering him, applied a cool cloth to his head, and any other tricks she had up her sleeve. Not much seemed to be helping Gabe. Since it had been 24 hours since his last PRN, she decided these might be withdrawl symptoms and decided to give him a extra dose of Morphine. Thank goodness Nurse "L" knows Gabe so well. This PRN did the trick. G calmed down and was a happy little boy. When Jesse and I arrived Gabe had once again dosed off for his late morning nap, which again turned into an all day nap. Since Gabe handled his weans so well yesterday they decided to wean a bit more on his Versed and made a pretty big step on his vent. They turned the rate setting on the vent down from 16 to 12. This means he is now receiving four less breaths from the vent. Super G showed us his super powers today, and thus far, has done just find with less breaths from the vent. I spent some time watching the vent screen today and was very excited to see my Bubba taking so many breaths on his own.

This afternoon we spoke with Dr "M" from the pediatric surgery team. With the amount of fluid Gabe's chest tubes have been putting out, the surgery team was called in to consult once again. Dr "M" spoke with us about two possible surgeries to help resolve the pleural effusion issue. One is putting a powerdy substance into his pleural cavity. This powder roughs up the outer lining of the sack around the lungs and the chest wall. The hope is the two scared surfaces will stick together and there will no longer be space for the fluid to collect. Without space to collect the idea is that it was force it to stay in the vessel it belongs in. Of course, the fluid could just find another open space, like the abdominal cavity, to flow into. The other surgery is one where they go in and tie off the thoracic duct in hopes of stopping the leak. With this one is it somewhat of a shot in the dark, becuase you are hoping to tie it off in the right area and there is the risk of actually causing more holes. There is of course much more to both of the surgeries, and they do have names but I can't remember them right now, however I will not get into all of the little details becuase we are hoping to not have to take the path of surgery. Over the last week Gabe's right side still hasn't put much fluid out, and over the last two days the fluid on the left side has gone down drastically. Becuase of the new trend (fingers crossed this is a trend and it won't start flowing again) the surgery teams feels, and we agree, that it's best to wait a few more weeks and see if the fluid is slowing down and if the lymphatic system is starting to heal. If the fluid keeps coming, we will try and collect more information about G's lymphatic system so we know which of the above mentioned surgeries might be his best chance and healing. Dr "M" is going to speak with radiology and find out if there are any other imaging options that might provide a better look at his lymphatic system. Neither of these surgical procedures are very pleasant, but Gabe can't leave the NICU with chest tubes. Fingers crossed his lymphatic systems really is starting to heal and this slow down in the chest tube output, is really a slow down.

We also sat down and spoke with Dr "R" today. We quickly went over the discussion with Dr "M" and the pleural effusions. If his lymphatic system is going to heal on it's own, the chest tubes are the best form of treatment and they are in place, so for the next few weeks we will watch closely and revisit this issue if things don't start getting any better. The main focus of today's meeting were Gabe's lungs. They have to start working on their own!!! A vent is ment as a temporary fix, not a permanent one. The longer he is on the vent, the more damage is going to occur. Dr "R" is happy with the progress Gabe has made on the vent over the last few weeks, since his last CPAP trial, and thinks he is ready to try again. If Gabe is in a good place tomorrow it's marathon time for his lungs. I already have butterflies in my stomach just thinking about tomorrow. I know Gabe can do it, and I just hope tomorrow is the day. If he doesn't start making some impressive progress with breathing in the next few weeks and it looks like it is going to take several more months to get him off the vent, we will have to start discussing a trach tube. Go lungs go!!! We don't want to have to put in a trach. That is opening up a whole new can of worms. We are very appreciative of Dr "R" and the team taking the time to sit down with us, away from the bedside, to discuss Gabe and how we can help him move forward.

At this point I truly think Gabe is in the best condition he has ever been in, but is still far from being healed, and is still in a scary place. Let's hope Gabe's lungs are ready to start doing all of the work on their own, and he can break through this wall he has been up against for so long now.

I will be sending Bubba ALL of my Mommy Super Powers tomorrow!

Tuesday, November 3, 2009

November 2-3, 2009

So what has Super G been up to for the last few days?

Gabe spent yesterday hanging out with Mommy and Nurse "L." He was a bit agitated first thing in the morning, but after a little happy-juice all was good, and he spent several hours wide awake and quite content. Of course as soon as I showed up he drifted off. I enjoyed just sitting with him; holding his hand and watching him sleep, and making sure the crib vibrator never stopped shakin.' Since his right chest tube hadn't put anything out in almost a week, the plan was to remove it yesterday. He had an ultra sound in the morning to make sure there was no fluid before taking the tube out. Unfortunately the ultra sound did show a "moderate" fluid pocket on the right side. Dr "R" tried to get the fluid flowing through the tube, but nothing seemed to work. Since the tube has been in place since September 10, it was decided it would be removed today and a new one placed. Noone wanted to see another tube going in, but perhaps if the fluid was gone, Gabe would be able to get down to the vent settings Dr "R" wants to see him on. Another thing I thought about was that it took almost a week for a "moderate" amount of fluid to build up, while the left side is putting out around 200 mls a day. Maybe the right side is slowing down? Other then waiting to find out if the right chest tube could be removed, and trying to help Gabe get rid of some nasty gas with some tummy massages by Nurse "L," Gabe had a pretty mellow day yesterday.

Gabe had a pretty good night, but was quite agitated, by more gas, when Jesse arrived this morning. The poor little guy was trying to sleep but kept waking up to those darn gas pains. Jesse gave him a little tummy massage and was able to help him work a little out. Jesse helping Gabe pass gas...true Father/Son bonding. In rounds the two big issues discussed were the upcoming chest tube removal and reinsertion, and his low albumin levels. With the amount of fluid his left chest tube has been putting out, we were not at all surprised by the low albumin levels. Gabe received a dose of albumin to help bring his levels back up. The rest of the day was spent waiting for an ultra sound machine to come avaiable for the chest tube "project." Finally around 3:00 the ultra sound machine, and radiologist arrived. The old tube was used as a marker, and the new tube was inserted just below it. They spent a little over an hour trying to get the new tube situated in the pocket of fluid, but were not having any luck. They finally decided to stop becuase the pocket of fluid was so low and so close to Gabe's liver they were affraid to mess with it anymore and cause damage to the lung or liver. The new tube was removed. They pulled back a bit on the old tube and a little fluid came out. Dr "R" and the radiologist decided it was best to leave the old tube, in it's new position, in over night. If no more fluid comes out over night they will remove it tomorrow and watch closely. If more fluid builds up, maybe a larger pocket of fluid will be easier to drain. Poor little Gabe. Even with extra sedation, I can't imagine it's very comfortable to have someone manipulating a chest tube around in your pleural cavity.

Gabe and his chylous pleural effusions, will be discussed at the weekly pediatic surgery meeting tomorrow. We are eager to hear what they have to say about this leaky lymphatic system that doesn't seem to want to heal on its own.

Gabe's current goal is to work those lungs out, to start taking more breaths on his own, and to hopefully be ready to try CPAP again in the near future. At last week's Care Conference a tracheotomy was mentioned, and we just don't want to go there. Come on Bubba! Time to get those lungs working on their own!!!

Sunday, November 1, 2009

November 1, 2009 - Happy 4 Months!

Happy 4 Months Gabriel!

What an amazing and crazy four months it has been. Amazing becuase we didn't know if Gabe would make it through the first hours of his life, and here he is 4 months later still fighting. Crazy because the last four months have been the most intense roller coaster we have ever been on. The ups and downs of daily life have been draining some days, and exhilarating others. When I think back on the obstacles Gabe has over come, I am in complelte awe of my son, and am so pleased by the progress he has struggled so hard to make. At the same time the fact that he is 4 months old, still has several hundred mls of fluid building up around his lungs a day, and still doesn't have lungs strong enough to function on their own, really really scares me.

Every day Gabe is on a vent more damage is done to his lungs. He has been doing well on his new big boy vent, but still has times when he doesn't breath at all and the machine does all of the work. G's lungs are obvisouly not ready to do all of the work on their own, and we don't know if they ever will be. We are hoping to try CPAP again in the near future, and to start getting more aggressive with trying to get his lungs going.

Gabe's right chest tube has put out extremely little fluid in a little over a week now. An ultra sound last Monday showed no fluid around the right lung. If tomorrow's ultra sound shows the same, then his right chest tube will be removed. We are all praying this lack of fluid build up means the part of the lymphatic system that was leaking into the right pleural cavity has healed. Now we just need the left side to follow and stop putting out several hundred mls of fluid a day.

Another issue, which I believe I mentioned in a blog not too long ago, is Gabe's nutrition. Gabe is not growing becuase he is not getting enough calories. There is only so much TPN can do for him, and with the leaky lymphatic system he really can't eat. His size doesn't seem like something we should be worrying about at this point, with the larger, more life threatening issues at hand, but he is fighting a huge battle and his body needs proper nutrients and calories to be in tip-top battle condition. Dr "R" is trying to figure out how to work on getting Gabe more calories.

We are trying to stay strong as we battle along side our son, but some days are harder than others when it comes to staying positive. We know Gabe is a fighter and has this cute little personality, but what we don't know is if we can heal him. Every day we have with Gabe is a blessing, and all we can hope is that these days and months we have had with him thus far, turn into more months and into years.

Thursday, October 29, 2009

October 28-29, 2009

Zoooom! Where have the last two days gone???

Yesterday we dropped Kayla off with Grandma and Grandpa Aguilar and headed up to Palo Alto to spend the day with Gabe. It was the first time in quite a while that Jesse and I have had the opportunity to visit with Gabe together. Thank you G & G Aguilar! Gabe had a good day yesterday and spent most of the time sleeping. We decked him out is his super cute pumpkin/Halloween garb and did a little photo shoot in the first part of the day. We would have loved to have left him in his costume, but the beenie, booties and bib were sure to have overheated Bubba, so we removed them promptly after the shoot. Yesterday we discussed moving G back to the big boy vent. It has a pressure support setting that will support each breath he takes, which in turns strengthens his lungs. This morning Gabe was switched to this vent. He slept right through the switcheroo and has done well on it all day. The most talked about issue yesterday and today was, as usual, his chest tube output. I believe between Tuesday night and this morning he had put out a little over 300 ml. Most was from the left side. My heart sank this morning when I heard this number. The right side really isn't putting out much anymore and I was hoping the left side would be following close behind. Gabe hasn't put this much fluid out in a really long time. We have no idea why this happened. I guess we just have to wait and see if it was a one time thing or if the flow is going to start picking up. With this large chest tube output, Dr "R" is interested is getting the pediatric surgery team involved to find out if a surgery that ties off a section of the lymphatic system, in hopes of stopping the leak, is something that could be considered

This surgery has been discussed in the past, and at this point it is not something Jesse and I think should be considered yet. If memory serves, I believe the cardiothurasic team was consulted a little over a month ago, and I don't believe this was a surgery they were in favor of. We will see how they feel about it again. Jesse is going to have a care conference with Dr "R" tomorrow and I'm sure Gabe's chest tube output and this surgery will be at the fore front of the converstaion.

As the day progressed today, Gabe wasn't putting much out of his chest tubes. This seemed extremely suspicious, especially considering how much came out in the last 48 hours. Gabe began to get more and more agitated. They aspirated his chest tubes this afternoon and were able to get things flowing again. The release of fluid did the job and Gabe settled back down. I would get upset too if there was fluid filling up the space around my lungs.

We spent yesterday with Gabe, so we took the day "off'" from the NICU today, and spent the entire day with Kayla. We headed up the coast to a fabulous pumpkin patch with pony, train and tractor rides; a playground; jumpy house; a huge maze, which we didn't attempt; a petting zoo; and lots of other great stuff. It was a beautiful, crystal clear day, and we thuroughly enjoyed every minute of our time with Kayla, and with eachother. It is tucked in a little valley, and if you stopped and listened, you could hear nothing but the sound of the wind and Kayla laughing. It was such a difference from the loud beeps and other noises of the NICU. I had a really great time running around with my husband and daughter, but I have to say that in some ways, it was rather bitter sweet for me. The whole time we were there I had a slight ache in my heart because one thing was missing. This year when we visited the pumpkin patch, I was supposed to have Gabe strapped to the front of me, snuggled safely in the Baby Bjorn.

How I long for the day I can scoop Gabe up and put him in the Bjorn. How I long for the day I can hear him cry; see him smile; see him sit up; see him in clothes; see him without wires and tubes. I welcome the day I want to pull my hair out and lock myself in the bathroom because I'm feeling overwhelmed by my children, both of which are infront of me, in our home, both of which are needing different things at the same time. I feel cheated out of the pregnancy experience, the childbirth experience, the baby experience. Gabe has been cheated too.

We are handed what we are handed in life, and although it doesn't always seem fair, I believe there is a lesson to be learned in every path we are sent down or choose to take. Gabriel has reminded me how very precious life is, and how we must embrace every day because you just don't know what tomorrow holds.

Tuesday, October 27, 2009

October 27, 2009

Overall Gabe had a good night last night and a good day today. He had his usual fidgety moments, but Nurse "J" last night, and Nurse "S," today, know him well, and were able to calm him down. We have found that quite often a "simple" repositioning is all that is needed to get Gabe back into his happy place. He is a big boy now and is not happy laying in the same position all day. If it wasn't for that darn ET tube and those darn chest tubes we could pick G up and bounce him around the room. This is what he really needs. Gabe is a true boy and prefers constant movement. Last week just purchased a crib vibrator (yes they actually sell such an item), and we have all been doing the happy-dance ever since. Now us and the nurses don't have to take turns whacking Gabe's bed, we just push and button and G instantly calms to the soothing vibrations of his new crib gadget. That thing is by far the best $30 we have ever spent!

Jesse made it to the hospital in time for rounds this morning. There had been some talk of trying CPAP again, but Dr "R" doesn't feel G is quite ready for that yet, and would rather spend the next few days weaning the Versed, picking back up with the very slow Hydracortisone wean, and continuing to chip away at the vent settings. Gabe is down pretty low on his vent settings now, but they did have to go up a bit today because his CO2 levels have been slowly rising over the last couple of days. We have already noticed that G seems less twitchy. Maybe it's all in our minds or maybe the Versed really is the culprit? Either way it's nice to see Gabe's eyes track better and move together. Another topic of discussion this morning was G's nutrition. Becuase his body has never handled fluid volume well (it causes him to puff) they have never been able to give him enough calories. The result of this is Gabe is not really growing. His measurements are not much different from those taken at birth. Hopefully Gabe can start tolerating more calories and we can start improving his nutrition. This is a concern, but really in the whole scheme of things, not the most pressing issue at hand. We can always put him on his big sisters diet of dairy products, strawberries, and Pirates Booty and get him fattened up in no time once everything else is working properly.

For the last couple days Gabe's right chest tube hasn't been putting anything out. An ultra sound yesterday showed there were no fluid pockets on the right side. I knew better than to get too excited about this information. I crossed my fingers and hoped the right side had finally dried up, then alas, last night it started draining. Bummer! The left side has been producing a lot, but didn't put any out last night. Then today they switched again and the right stopped and the left started up again. Who knows what the heck is going on with those pleural effusions??? I just want them to go away!

Noonan Syndrome update: the results are back from the genetics testing for Noonan's Syndrome and..."no mutation detected" are the results. However only "approximately 50% of Noonan syndrome cases are due to mutations in the PTPN11 gene" and "these negative results neither confirm nor rule out the diagnosis of Noonan syndrome. Mutations in other genes related to Noonan syndrome can not be ruled out." Ok??? Wow! That was really helpful. Our take on this whole Noonan Syndrome thing is "whatever!" If he has it, he has it, and if he doesn't, he doesn't. It is not going to change the course of his treatment, it is not going to change how much we love him, so really Noonan syndrome or no Noonan's syndrome..."whatever!"

Kayla went to bed early tonight, the daily chores are done, so I think I'll go park my butt on the coach next to my husband and enjoy a couple scoops of pumpkin ice cream.

Thursday, October 22, 2009

October 26, 2009

Wow! What a crazy couple of weeks it has been! Just as we're starting to get used to one routine, a new one pops up. With Jesse back at work now, it's added a whole new level of chaos to our lives. We are so very appreciative of all of the firefighters for devoting so much of their personal time to covering Jesse's shifts for a little over 4 months. I don't know how we would have made it through that time if Jesse had been working. The new routine is: we rotate with the kids on the days Jesse is off; on the days Jesse works Kayla spends time with the Grandparents (if someone is available) and I spend the day with Gabe; we try to take one day a 4-day "off" from the NICU to hang out at home and do family stuff with Kayla. We haven't spent many nights at the Ronald McDonald house in the last month, which means there has been lots and lots of driving! I am starting to feel like I live out of my Yukon. With all of the back and forth it hasn't left us much down time, which means we haven't had a chance to sit down and blog. I have started several times in the last week, but with Kayla only napping about 50% of the time now, and the fact that we can hardly keep our eyes open once she's down at night, we just haven't found an hour (this is about how long it takes to compose one entry) to sit down and blog.

So what's been going on with Gabe?

MRI: Gabe's MRI went well, and it was worth the trip down and back becuase we did learn some new info about his Liver. Turns out he does have one mass like they orignially thought. Not eight. It is part of both lobes and measures something like 5 x 4.5 x 7 cm. Gabe is the size of a new born, so this is a ginormous mass in his itty bitty body. MRI is the best way to view the mass, therefore these are probably the most accurate measurements we've ever had of it. They were able to get a pretty good look at it during Gabe's CT scan at the beginning of August, and the mass does not appear to have grown much since then. This was a huge relief because, per the ultra sounds, it looked as though the mass had grown quite a bit in the last month. Since the mass is not blocking any veins, and is not keeping the liver or any other part of his body from functioning properly, we are going to leave it be. There is one surgeon who feels it is operable, however it would be a very intense surgery, and would be very hard on Gabe's little body. If the mass does start to grow and cause problems, then we know it can possibly be removed and we will revisit it at that point. Our hope is that it stays its current size and as Gabe gets larger it just becomes this small mass in his liver.

Care Conference: Last week we sat down with Dr "B" for a heart-to-heart about our little guy. Knowing that Gabe's chylous effusions aren't diminishing (still putting out around 150-200 ml a day) and he was 16 weeks old, we weren't sure what kind of a conversation we would be having. The main focus of our conversation was of course the chylothorax (leaky lymphatic system causing the fluid build up around G's lungs). Dr "B" shed a new light on this frustrating and discouraging situation. Even though Gabe is 16 weeks old, we only discovered the source of the pleural effusions 5 weeks ago. It was only at that point that feeds were stopped and his lymphatic system no longer had a certain chain of fats going through it, which keeps it from being able to heal. Of those 5 weeks, Gabe spent 2 of them fighting a UTI. When the body is fighting an infection the lymphatic system becomes overloaded and leaky, so really Gabe has only had 3 weeks (4 now) of proper conditions for his lymphatic system to repair itself. Hearing this made our hearts skip a beat and brought with it a whole new feeling of hope. Gabe's chylothorax has really had less then a month to heal, not 4 months. It is still a huge concern for us all, but hopefully we will start seeing less fluid output/build-up in the near future. Come on lymphatic system! The other big issue we discussed were Gabe's lungs. They are still not as healthy as we would like them to be, but have gotten better in the last 5 weeks. So much so, actually, that Dr "B" wanted to try Gabe on CPAP (breathing on his own with a constant flow of air being pushed down his nose) the next day. Dr "B" said it's easy to get caught up in only focusing on the pleural effusions and thinking that Gabe is not making any progress forward, but that was not the case. In the last five weeks Gabe's nutrition has improved, making him stronger; his lungs are slowly making progress forward; and he just looks better. Dr "B" said that if we were going to push Gabe's body to get better, and he was just going to lay there when he got home, we would be having a very different discussion, but...Gabe has proven he's a fighter; he is fiesty; and he has a little personality, which is obvious by the number of people that are drawn to him. There is a little person in there, and Gabe is trying his hardest to get better. Overall Jesse and I were very happy with our care conference and the direction it took. We still don't know how this NICU experience is going to end, but we are "cautiously optimistic" it will go the way we are all hoping and praying it will. We had been feeling so discouraged, this talk with Dr "B" was much needed. We were able to re-group and now we can charge forward and keep fighting along side Gabriel.

CPAP: Last Wednesday was the big day. Gabe was outfitted with the ever-so-attractive CPAP head-gear, the vent mode was changed to CPAP (pushes a constant flow/pressure of air down his nose), he was extibated (breathing tube removed), and...Gabe was PISSED! Could you imagine being soud asleep, letting some machine do most the breathing for you and all of a sudden, BAM! You are woken from your sleep with plastic prongs shoving air down your nose and your lungs are suddenly responsible for doing all of the work on their own. I would be upset too. Gabe recovered pretty well, his CO2 levels, which were high at first, started to go down, and he was off and running. Jesse and I took turns whacking Gabe's bed (the kid needs constant movement), and he slowly started to drift off to sleep. Since Gabe appeared to be adjusting pretty well to CPAP, we headed out get grab a bite to eat. We called in about 45 minutes later and learned he was sound asleep and everyone was sitting back out of fear of waking him. With this news we decided to lolly-gag a bit longer. Upon arriving back at the hospital I received a call from a nurse (not Gabe's, hmmm?) letting us know they were going to reintibate Gabe. We picked up the pace and when we got to the room our hearts about stopped when we saw the large group of people standing around Gabe. We suddenly realized the doctors and nurses hadn't decided it was time to put the breathing tube back in, Gabe had. When adults start to get tired they begin to slow down and put less energy and effort into it. Kids however, give it 120% and then just stop. Yes, Gabe's lungs said "that's enough, we are tired," and just stopped. Nurse "N" was right there and got the team together and got Gabe up and running again. Thank you Nurse "N!" Turns out Gabe's CO2 levels were much higher than the machine was showing, fluid was building up around his lungs becuase his chest tubes weren't draining properly, he wasn't taking strong enough breaths to clear the goobers out of his lungs so they were building up, and his lungs just got tired from their work out. Dr "B" we shouldn't look at this experience as a failure. We should view it as a huge step forward that Gabe and his lungs were in good enough condition to even consider trying CPAP. Way to go Gabe! We will try again in a few weeks when his lungs get stronger.

It took Gabe less then 24 hours to recover from his CPAP experience, and he has spent most of the last few days just chilling out. He did have one rough night, but Nurse "S" got him through it, and he has been behaving himself pretty well ever since. They have decided to start weaning G off of the Versed because it can become toxic after long term use, and can cause twitches and contractures. We are hoping that once he is off of the versed his eyes will not be as twitchy and maybe his arms won't be as contracted. We will see what happens.

I had a wonderful bonding day with Gabe this last Saturday. He was awake, alert and happy for quite a while. I situated myself over Bubba and started making stupid ooooing and goooing noises and dorky facial expressions, and he LOVED it! I swear, if he could have smiled he would have. G got a really happy/excited expression on his face, his eye brows went up and he started kicking and moving his arm and legs. I couldn't have been happier. I felt like I was having a "normal" interaction with my son. Then to make it even better, Nurse "L" took on the huge task of getting Gabe out of bed so I could hold him. After a few fidgesty moments Gabe calmed down and actually fell asleep in my arms. I held him for several hours as he snoozed away. Thank you Nurse "L!" What an awesome day!!!

Jesse was supposed to spend the morning with Gabe on Sunday, but got called away to a fire in the Santa Cruz mountains. He was going to be gone for 3-7 days. "Oh crap!" was all I could come up with. How the heck was I going to be mom and dad in two places at once, for a week. Then, this morning, I got the good news that his team was being sent home. We are all VERY excited that Daddy will be coming home tomorrow!!!

Sunday, October 18, 2009

October 16-18, 2009

Super G did super well during his MRI on Friday. The trip down and back from the MRI unit was uneventful, which is what we were hoping for, and they were able to get all of the images they needed. Upon return to his room in the NICU Gabe was being absolutely adorable and per Jesse was "high as a kite." He was under anesthesia for the MRI. Jesse got some really great pics of Gabe Friday afternoon. I don't think I've ever seen him looking so cute. Jesse will post them for ya'll tomorrow along with a more thorough description of the MRI results. Jesse spoke with Dr "H" this afternoon regarding the MRI, but of course Jesse and I have yet to have a chance to sit down and talk today. Maybe Kayla will actually go to bed on time tonight and we'll have time to sit down together. Fingers crossed!

Overall Gabe has had a good weekend. He behaved himself most of the time and didn't pull any crazy stuff. He has done well with the vent change to pressure support, hasn't required many PRN's, and has just been hanging out enjoying this lovely fall weekend. Unfortunately his chest tubes have still been putting out quite a bit. We have a Care Conference with Dr "B" (the Neonatology Guru) on Tuesday, and we are anxious to hear what he has to say about Gabe. About a month ago he said he was "frustrated but not discouraged" by Gabe. We'll see if he still feels that way, and find out what his thoughts are on how we may be able to help G move forward.

Gabe would like to thank Auntie Meghan, Grandma Reynolds, Grandpa Reynolds, Grandma Aguilar, Grandpa Aguilar, and especially his Big Sister Kayla, for visiting with him this weekend.

Thursday, October 15, 2009

October 15, 2009

Another day has zoomed past, and here I sit trying to remember all of the details. By the end of the day, and quite often all day, my head feels fuzzy. There is so much going on all the time my brain feels like a ticking time bomb. The other day I told Jesse that I feel like we are leading double lives. We go from being Mom and Dad to Kayla at home, to Mom and Dad to Gabe in the NICU. While at home I feel somewhat normal. There are chores to be done, bills to be paid, playgroups and gymnastics for Kayla and all of the usual stuff we did pre-Gabe. Then there is the NICU life that feels crazy, confusing, scary and frustrating. I long for the day we can be Mom and Dad to Kayla and Gabe in the same place at the same time. Every day that passes with no improvement in the amount of fluid draining from Gabe's pleural effusions, I wonder if we are ever going to have that dream of having our family together. The harsh reality of this horrible NICU life we are currently living, is we don't know the outcome. I don't know if Gabe will ever get to come home; if he will ever get to play with his big sister, or go to the pumpkin patch, or open a present from Santa. My heart aches constantly with this knowledge sitting in the back of my head, that Gabe may not ever get better. I don't know why this is happening to this amazing little boy. Today he was watching his mobile and kicking his legs with excitment and I thought "Please God, please! Please heal my little boy. Please give us a miracle, becuase I can't imagine waking up a single day without him." I don't care if Gabe comes home with special needs. I don't care if he has to live with us forever. I just want him better and home with us where he belongs!

A lot of fluid drained from Gabe's chest tubes today. Every time I looked down at the tubes, there was more that had collected. I felt like crying every time there was more. I was really hoping this Octreotide would work, but it looks like it may not be the answer for Gabe. Everyone keeps saying "time," but you would think if the Octreotide was going to work we would start to see less and less coming out each day. Tomorrow Gabe is going in for an MRI. The MRI will focus on his liver. I pray we will learn something new about Gabe's liver and the masses. Something that will help us get him through this wall he is now up against. The ultrasound from earlier in the week showed the masses were growing. I don't know what this means and what can be done about the masses? If they were in one lobe of the liver, that lobe could be removed, but they are in both. At this point they are benign, but as the masses grow his liver grows and takes up more space in his body, which means there is less space for everything else. Gabe's potassium was still low today, so he was given more to try and bring that level up. He also received another blood transfusion. RT (Respiratory Therapist) "K" thought Gabe might breath a little better on the pressure support setting, so they made some changes to the vent and so far he seems to be doing well with the changes. After an echocardiogram this afternoon, we got the good news that Gabe's heart is still looking normal and going strong. Phew! There is a lot of strain on a really sick babies' heart, so it was a huge relief to hear Gabe's is doing well.

Tomorrow is a new day, and hopefully it will bring with it new information about Gabe.

We love you Bubba!

Wednesday, October 14, 2009

October 14, 2009

You know you've been in the NICU too long when you call the front desk and the conversation goes as follows:

"NICU this is ____"
"Hi ____ it's Shannon Aguilar."
"Hey Shannon, how are you?"
"Doing well. How about you?"
"Great. Did you want to speak with Gabe's nurse?"
"Please. Thanks."

When all of the NICU staff know you and your child by first name, it's time to go home! Most babies around the NICU are known by "Baby" and then whatever their last name is. For example, Gabe would be "Baby Aguilar." But that is not the case with us. Not only is G called by his first name but everyone calls him "Gabe" and not "Gabriel." A couple of the nurses even call him "Bubba," which is my nickname for Gabe. After 106 days (yes, that is how old Gabe is) in the NICU, and no signs of going home anytime soon, I think it's safe to say we have become somewhat of a permanent fixture there. Kind of like, you can expect to see several things when you visit the LPCH NICU: babies, doctors, nurses, and the Aguilars. In the words of The Beach Boys' "Sloop John B:" "...I feel so broke up I wanna go home, let me go home...this is the worst trip I've ever been on...why don't you let me go home."

In yesterday's blog I reported there wasn't much out of G's chest tubes. Apparently I spoke to soon because over night he dumped quite a bit. Bringing his daily total, for yesterday, to over 100mls. Grrrrr! I just spoke with Nurse "C" and so far today there hasn't been much, but we'll see what happens as the day/night progresses. I'm not getting excited about diminished chest tube output anymore until we go at least a week or more with big changes. They spent all day yesterday trying to lower Gabe's potassium levels and it was successful. Too successful actually, becuase they got too low and are now having to bring them back up. Gabe's hematocrit (red blood cells) level was too low this morning as well, so he has already received one "dose" of packed red blood cells (blood transfusion) and may require another tonight. Regardless of these issues, Gabe as been sitting comfortably all day, and has been a very good boy for Nurse "C." They have been able to make some changes to his vent settings, and so far he is tollerating the changes.

For, oh probably 106 days now, I have been asking if Gabe's large tumor filled liver could be the cause of the Chylothorax and/or other issues he battles with. It's so large that it is displacing his intestines to the left side and pushing up on his diaphram. It makes sense to me that it could be causing pressure somewhere on the lymphatic system causing a leak or maybe even a blockage, which has caused the fluid to find an alternate route (leaking out) around. Even though this makes perfect sense to me, I have been told that on a "pathophysiological level" this would not make sense. Enter Dr "H" who just cannot get this large tumor filled liver off her mind, and is on the same page as me (in the sense that these masses must have a bigger role in Gabe and his health then we think/assume). Yes, she agrees that on a pathophysiological level the large liver would not be causing Gabe's issues, but then again, who's to say it isn't? She is currently arranging for an MRI of the abdomen that will focus on the liver and the masses. The goal is to collect as much information as possible regarding these masses and his liver and not write it off as a culprit until we know everything about them; exactly how big the masses are and what exactly they are pushing against. Thank you Dr "H"!!!!!!!!!!!

At this point Gabe has hit a wall. His chylous effusions don't seem to be getting any better, and he cannot live with them. His lungs cannot get better (if they even have the ability to get better) with the effusions, and he cannot live without healthy functioning lungs. Quite honestly, I feel that many days/weeks are spent maintaining Gabe. Don't get me wrong, this in itself is a huge all-consuming-mind-racking task, but if we don't dig deeper and learn everything we possibly can about Gabe and his body, then we can't help him move forward through this wall he is now up against. I am very excited about Gabe's upcoming MRI and the more indepth research of his liver and the masses.

We are extremely appreciative of all of the Doctors, Nurses, and everyone else involved with Gabe, for taking such great care of him, and putting so much of their heart and soul into trying to figure out our little mystery man. Becuase of these amazing people, our son has been given a fighting chance at life.

Tuesday, October 13, 2009

October 13, 2009

Today is Jesse's thrid day back at work. The original plan was to drop Kayla off at Grandma and Grandpa Aguilar's house in the morning and then head over to spend the day with Gabe. After looking out the window this morning at the pouring rain and trees bent over sideways from the wind, I decided to stay home with Kayla. As we lounged around the house in our pj's I was aware of a growing feeling of guilt for not visiting Gabe today, so...I packed Kayla up, dropped her off to play with Grandma and Grandpa and braved the crazy, stormy conditions to see my little boy. I'm really glad I decided to stick with my original plan becuase I got to see all three (Jesse, Kayla and Gabe) of my favorite people today. What a treat!

Last night was a good night for Gabe. He required only two PRN's, which is a huge improvement compared to the last two weeks, and other than some vent adjustments that had to be done early this morning, because he started to get tachypenic (very rapid breathing), it's my understanding that G and Nurse "B" had a pretty mellow evening. This morning his labs showed his K (potassium) levels were quite high, and that he was too dry intravascularly. I'm having a complete Mommy-Brain moment and can't remember why you don't want high levels of K, but you don't, so they jumped on trying to lower them right away. They stopped his TPN (nutrients) drip, becuase it contains potassium, and changed his vent settings to over ventillate him a bit to get the stuff out of his system. I think they did a few other things as well, but I just can't remember what they were. All of this was a success, and his K levels got lower through the day and as of 6pm tonight were back down in the normal range. As for being too dry intravascularly, they stopped one of his diuretics, changed the other to every twelve hours instead of every six, and went back to using FFP instead of Albumin (which they switched to yesterday). Hopefully all of these changes will help and Gabe won't be as dry tomorrow. They are purposely keeping G pretty darn dry intravascularly becuase studies show this helps with hydrops and chylous babies. If there isn't anything to leak out, then hopefully the edema will get better and the chylothorax will heal. Of course you can't keep someone completely "dry" intravascularly, so there is a fine line that has to be walked in this area and today his was just too far on the "dry" side. So are you completely confused now? You probably didn't need all of these details. All you really wanted to know was how Gabe's day was. It sounds crazy and hectic, but all it really came down too was a lot of tweaking to get his K levels and dryness under control, and Gabe was completely oblivious to it all. He spent most of this rainy day snoozing. I wish I could have climbed in bed with him for a little nap myself. I wonder if I would fit in his bed with him? I bet I could make it work. I wonder if anyone would notice???

Overall Gabe had a good day. He was awake for about an hour while I was there, and we spent that time chatting, holding hands and just hanging out. His Albumin levels are back up to 2.6 (want them to be at least 2.4), he was breathing well all day, didn't require any PRN's, hardly needed any suctioning (this is usually needed every 1-2 hours), and as of 4pm this afternoon only had 40 mls out of his chest tubes. We don't know what this means yet? Is he drying up or is the fluid hiding? On Sunday his tubes drained a little over 200 mls; on Monday he had around 130 that came out; and so far today, even after the tubes were checked for clots, has only had 40. They did go up on his Octreotide today to 10, so it is now at its highest dose. Is it starting to work??? I guess we'll just have to wait and see...

October 9-12, 2009

Here we go again, leaving you all hanging for days. We've had a couple of busy days and just haven't put the time into sitting down and updating you all. Our apologies for that.

I'm happy to announce Gabe's UTI is finally gone, and he seems to be returning to the happier little guy we had become accustomed to before this evil UTI took him over. In the last few days Gabe has had his usual ups and downs of numbers being off here and there, and the vent needing to be adjusted, sometimes several times a day, but for the most part it was a pretty uneventful weekend for Bubba. They have already gone up on the Octreotide (medication we are hoping will heal the Chylothorax) several times, becuase so far it has not made a difference, and Gabe continues to put out rather large amounts of fluid from his chest tubes. We are praying this medication starts working soon.

We are going to sit down with one of Gabe's doctors next week for a serious chat about where we go from here if the Octreotide doesn't work. Does he think with time Gabe can heal on his own? The scarriest part of this whole "adventure" is no one can say for sure what the outcome is going to be. We know that Gabe is a fighter, he has most certainly proven that, but if this Chylothorax does not heal, it's not something you can live with. The other big question we have is about his lungs. They have been compromised by the pleural effusions (fluid around his lungs) and his thick edenemous chest walls for so long that they have not been able to grow into healthy fully functioning lungs. Also, being on a vent for almost 15 weeks, and who knows how many more, causes damage too. It breaks my heart to think about these things and I try to just enjoy my son and not worry to much about the things I have no control over, but they are always in the back of my mind. I can't imagine life without my Bubba, but I know we are still not in the clear. I hope and pray that Gabe will continue to fight, and that with time his body will heal and we will be able to get him out of that NICU and home with us, where he belongs.

Thank you all for your love, support and prayers. We are blessed to have so many amazing people in our lives.

Thursday, October 8, 2009

October 8, 2009

Today was Jesse's second day back at work, and may I just say, I have a whole new respect for working parents. How the heck anyone gets out the door looking presentably disheveled (let's face it, once you enter parenthood you never quite look the same again), gets their kid(s) dropped off at daycare, and makes it to work by 8:00 am, is completely beyond me. By some miracle Kayla and I actually made it out the door on time this morning. However, after hitting traffic on Hwy 9, getting gas in my beast of a vehicle, dropping Kayla off at Grandma and Grandpa Reynolds' house, getting stuck in traffic on 280 (took me 30 minutes to go 1-1/2 miles), stopping by the fire station to get the hospital parking pass from Jesse, circling the itty bitty hospital parking lot looking for a place to park my Yukon XL, and giving up and heading to valet parking, I FINALLY made it to Gabe's bedside by 11:00 am. Holy Crap! Talk about feeling disheveled. Seeing the cute little completely-wide-awake-and-happy face that greeted me as I walked through the door to NICU room 4, made everything better.

Although Gabe did get another fever last night, for the most part he had a pretty good evening. When I arrived this morning (practically afternoon) Bubba was chilling out with Nurse "G" staring at his mobile. He still looked puffy, but better than he had earlier in the week and I was so releaved to see my happy little boy instead of the wild man that had taken him over for the last week. Soon after arriving, G nodded off, so we just sat in silence together. G snoozing and me holding his hand and head. He had one desat episode this afternoon, where his numbers got pretty low, but he recovered quickly and went back to sleep. Gabe still has a lot of secretions which cause him to desat if you don't get them cleared out of his lungs (via the ET tube) quick enough.

This afternoon we had a Care Conference. This is a time for us to sit down, away from the bedside, with a Dr, NNP, nurse and our social worker to discuss Gabe. The main focus of today's conference was his Chylothorax and the fact that it does not seem to be healing on its own. Gabe has been NPO (medical instruction to withhold oral food and fluids from the patient) for five weeks, and although at one point we thought his pleural effusions (fluid around his lungs) were beginning to dry up, they aren't and his chest tubes have actually been draining a lot of fluid over the last two weeks. The doctors think it is time to intervene and try a drug called Octreotide to try and heal Gabe's leaking lymphatic system. This drug has been used successfully with other Chylothrox neonates, and there are even reports of it working within three days for some patients and taking up to a month for others. The reason we did not try this right away is that it is a drug, and all drugs come with side effects, so they felt it was best to give his body a shot at healing on its own first. Even though there are success stories with Octreotide, there are also reports of it not working at all for other kids. I'm really hoping Gabe can be added to the success story list. We also discussed his "detour" this last week, and the Dr feels it was all associated with the UTI. I really hope she is right becuase that means once the UTI is gone Gabe will start moving forward again. They are still slowly chipping away at his vent settings, and once he is feeling better will go back to the sedation wean. Once the Chylothorax is healed, and Gabe no longer requires FFP to replenish the Albumin lost from his "leaking," the hope is that he will begin to slim down even more and his chest walls will get thinner allowing his lungs to get stronger and then...we can start talking extibation. Also, once we are sure he is no longer leaking Gabe can start eating again. We are anxious to see how this Octreotide works. If it is successful and heals the Chylothorax (leaky lymphathic system causing the pleural effusions) then I think we will start down a very exciting path. One that hopefully only moves forward. We still have a long way to go and as we've been told several times "Christmas is nice in the NICU." It's a little depressing to think about still being there come the New Year, but it means Bubba is still with us, and he is most certainly worth the wait.

Wednesday, October 7, 2009

October 6-7, 2009

It's a bit of an understatement to say G had a rough day yesterday. Wow! What a day it was...

After a somewhat peaceful night, and a little bit of an agitated early morning, Gabe was resting pretty comfortably when I arrived around 9:30am. Then all hell broke loose. I can't remember exactly what time it all started, but I can tell you that the madness lasted for hours and didn't end until about 6:30 pm. So Gabe woke up from his morning nap, in a not so fabulous mood. He wasn't happy awake and he wasn't happy asleep. Nurse "B" and I tried all of the usual calming tricks, but none of them seemed to work. We had some success wacking the side of the Boppy (Gabe like's motion, it calms him), but pretty soon even that didn't work. He got fussier and fussier until he had himself worked up into such a tizzy that even morphine and ativan didn't help. Arms and legs were flailing every which way; at times he was screaming (silent of course); and then the fever started. Gabe runs warm and is known to get fevers quite often. It's just one of his many quirks, or Gabe-isims, as Nurse "A" refers to them. If you notice him heating up you better get the blankets striped off and start cooling him, or else. We did just that, but didn't seem to make a difference and the fever kept climbing. He reached 39.2c, which I believe is around 102ish F. Whatever was bothering Gabe before was not worsened by the high fever, and now he was agitated becasue he was hot and was getting hotter because he was agitated and flailing. The final results were still not in, but it did look as though he had a UTI. If this was truly the case then this horrible day was probably due to the pain associated to the UTI. If it turns out he does not have a UTI, then I don't know what the heck was going on. I so wish I could have scooped Bubba up in my arms and bounced him around the room, mommy's always make everything better, but I couldn't. I just had to sit there and watch him go through all of this and it was heart breaking! I'm not sure what finally calmed him, but around 6:30pm Gabe finally relaxed. I was physically and mentally exhausted from the day, so I can't imagine what G was feeling like. I stepped out to get some dinner and when I returned G was snoozing comfortably. Thank God!!!

Gabe had a good night, and so far has had a good day. He is once again dumping a ton of fluid out of his chest tubes, which has caused his albumin levels to plummet. Yesterday they were 2.5 and this morning they were 1.9. Dr "B" wants to see him at 2.4 or over. That seems to be the magic albumin number for hydrops kiddos. Because of this drop they have upped his FFP to every six hours, instead of every twelve. Fingers crossed this does not make him puff. He has looked puffy over the last few days. NNP "N" did say that the puffiness could be associated with the UTI. It could be part of the infectious process. Let's hope that's what it is and will start to go away with the infection. Gabe has had good blood gasses today and they have been able to wean a little on his vent settings. Over the last week G seems to have taken a detour off the "right" path that he finally found a few weeks ago. Let's hope that detour is over and Gabe will start moving forward again.

I'm still exhausted from yesterday! What an adventure we are on...

Monday, October 5, 2009

October 4-5, 2009

After a rough week, Gabe had a pretty darn good weekend. No major issues or agitations, like earlier in the week. Justs lots of sleep and happy awake times. Unfortunately....this "happy" trend did not last. Late Sunday night Bubba became a wild man! He started his crazy retracting (taking lots of fast shallow breaths), apendages were flaling, blood gasses were out of wack, he had a fever that reached somewhere around 103 degrees, all of his PRN's (extra doses of sedation) were used plus some, and still nothing could be done to calm him down. X-rays were taken of his lungs and abdomen, but they all looked fine. A septic work-up was done to see if he might have a bug of some sort, and although the official results are not yet in, it looks like he has a UTI (urinary tract infection). I have had several of these in the past, and let me just say, OUCH!!! My poor Bubba! As if it's not bad enough to have two chest tubes, and ET tube and everything else, he now has to deal with the excruciating pain of a UTI. Antibiotics were started right away. When adults receive antibiotics for a UTI they are laced with a numbing agent, which takes effect quickly and makes the pain go away. This however, is not the case when it comes to UTI treatment in children, and this is why Gabe has completely maxed out all of his pain management options in the last 24 hours.

Gabe has been agitated all day. Not as much of a wild man as he was late last night and into the wee hours of the morning, but obvisously not a happy camper. He didn't want to be touched, required higher vent settings, and was having a "terrible horrible no good very bad day." To top things off, Jesse reported that G looks puffier, and his right chest tube doesn't seem to be working. In hopes of de-puffing him, they are going to up his Lasix dose, and as for the chest tube, it may end up getting pulled and replaced tonight.

It breaks my heart to know my son is going through all of this, and there isn't a darn thing I can do about it. There I was snoozing away last night and my child was writhing in pain. It wasn't until I received a call at 6:30 this morning that I knew what was going on. When I left last night Gabe was wide awake and totally content watching his rattle that we have suspended over his head. It's such a helpless feeling.

Here's to hoping tomorrow is a better day for G. I am sending him all of my super mommy powers.
FYI, the above picture was taken yesterday when he was a Happy Boy.

Saturday, October 3, 2009

October 3, 2009

I am happy to report, today was a much better day for Super G. After a rough week Gabe spent a mellow day hanging out with Nurse "G." We popped in to visit for a bit this afternoon and Gabe got to meet some very dear friends of ours.

Bubba was doing so well today they were able to wean on his vent settings. His potassium was on the low side this morning so he received two potassium boluses throughout the day. His Albumin levels are going back up and his puffing from the FFP is going down.

It was great to see Gabe looking like himself today, and we are so happy he had such a nice quiet, uneventful day.

Friday, October 2, 2009

October 2, 2009

Shortly after blogging last night I called to check on Gabe and learned that he was still not doing very well. The improved condition he was in when I left, apparently didn't last for too long. He was extremely agitated, was retracting (lots of quick shallow breaths) and his numbers were not sitting as stable again. Nurse "S" pulled all the tricks out of the bag, but nothing seemed to be working. After a good CPT (chest physical therapy) and some PRN's (extra doses of morphine and/or adevan) he finally started to calm, but was still not himself. Earlier in the day Nurse "G" was having some trouble with Gabe's PICC line. Come to find out the line had become clotted, meaning Gabe was not receiving any of the drips running through that line, and the drips going through that line were his Morphine and Versed. This means that the majority of G's issues over the last few days can most likely be attributed to the fact that all of his sedation and painkillers were cut off and he was having major withdrawls. Poor baby! The PICC line was pulled and an IV started to temporarily run his drips through until a new PICC line could be placed in the morning.

When Nurse "A" arrived this morning Gabe was doing much better and was resting comfortably. Unfortuantely he did look puffier then the day before, so it was decided during rounds that his FFP would be changed to every tweleve hours, instead of every six, and it would be chased by Diurel (diaretic). This is the concoction Dr "B" came up with, which successfually kept Gabe from puffing when he needed FFP. Unfortunately they were not able to get a new PICC line in, however they were able to place a deep IV, which will work for now to run his drips through. Gabe had several good blood gases so they were able to wean on his vent settings. They are still not down as low as they were at the beginning of the week, but they are lower than yesterday. I think the combination of stopping G's hydracortizone wean (and actually going up a bit), cleaning out his chest tubes, tweaking his vent settings and running a new line for his drips, has finally helped Gabe to get back on track. He spent the day hanging out with Nurse "A," snoozing and checking out his surroundings. We took today "off" and enjoyed hanging out with some friends that came into town. We look forward to seeing Gabe tomorrow. We miss him so much if we don't see him, but it sure does feel good to spend a day at home.

Thursday, October 1, 2009

October 1, 2009 - Happy 3 Month Birthday

I wish I could say Gabe's 3 Month Birthday was a nice quite day, but unfortunately I cannot. Poor little Gabe did not have a very good day.

For the last three weeks Gabe has been pretty stable, and having many more good than bad days. Starting Tuesday afternoon, G started to show a few signs of instability. We weren't sure if he was just having a funky afternoon (quite often he is agitated in the afternoons. We call it his witching hour) or if something bigger was going on. When I arrived to see Gabe around 10am on Wednesday he was retracting (taking lots of quick shallow breaths) a lot, his respitory rate was high until late afternoon, he was requiring extra oxygen to keep his O2 sats where they usually are on room air, was desating more often (like he used too) and was taking longer to recover when he did desat. Gabe just wasn't himeslf although, other than his little desat episodes, all of his numbers looked pretty good. Today however was a whole different story.

When I arrived this morning, Gabe just didn't look as good as he ususally does. He was very squirmy, was requiring more oxygen then yesterday and his numbers were not very stable. He kept desatting and had trouble recovering without assistance. I also thought that it looked like Gabe was puffing a bit. His Albumin levels have been low so he has been receiving FFP (blood product) every six hours. This has been known to make him puff in the past. In rounds I learned that his Albumin was actually up since yesterday, although still low, so the FFP treatments would continue. I suggested chasing his FFP with Diurel (a diaretic). A concoction Dr. B found worked very well and kept G from puffing. Dr V said she would try this if he continued to puff (luckily, thus far he was not). His chest tubes hadn't put much of anything out and the morning x-ray looked a little hazy and did show a bit of fluid on his left side. In rounds we mostly discussed what could be causing G's bad day. Clogged chest tubes could be one thing; the hydracortizone wean could be part of it (in the past hydracortizone weans have brought his spiraling backwards); and there was one other factor they thought could be involved in his changes over the last couple of days but I am so fried from the craziness of today I can't remember what it was. They were able to get the chest tubes cleared out and pulled off 20 cc's from his right side; they stopped his hydracortizone wean and actually went up on his dose a bit; they went up on his vent settings; and he received some blood because his numbers were a little low there too. After all of these things were done he was doing a little bit better, but still not Gabe, and still not looking that great numbers wise. At this point I was starting to get really worried that there was something larger going on that we were missing. I stepped out for about 30 minutes to pump, and when I came back in Gabe was on his tummy, his numbers looked fabulous, he was barely requiring any extra oxygen and he was sound asleep. He was letting the vent do all of the work, but that was ok considering how hard Gabe has been working the last couple of days. What a relief to have Gabe back. Phew! What a day!

Thank you Nurse G for working so hard to help Gabe yesterday and today. Let's hope we never have another crazy diaper changing/desatting experience like we did today.

We can only hope that whatever caused Gabe to go backwards for the last 2-1/2 days has been fixed and we can start going forward again.

Thank you to all of our loved ones for your endless support over the last three months. We could not get through this without you.

Wednesday, September 30, 2009

September 30, 2009

Sorry to update this blog so slowly lately. Shannon and I are burning the candle at both ends and we are extremely tired. Gabe has been doing very well and he has just been having little ups and downs. He has been doing very well with his ventilator, requiring less and less pressure as well as a slower respiratory rate. He has also been breathing on room air and not requiring any supplemental oxygen. Woohoo! Every once in a while he will get worked up and require a little more oxygen and pressure. Then he will relax and we will be able to turn it down. Gabe's Albumin level has gotten a little low lately which is requiring them to give him some more blood products to replenish what is lost. His chest tubes were slowing down substantially, down to about 10cc per side in a 24 hour period. That was down from almost 150cc per side last week. Unfortunately his chest tubes have begun to put out a little more, he's up to about 100cc total today. They told us this was going to take months and we would be going up and down, so this is to be expected.
Gabe has been spending more and more time on his tummy lately, which he actually likes. We love seeing his little back and his cute little no butt. His arm stretches are getting easier and easier each time we do them. Yesterday Gabe was upset and was moving his arms and legs more than I have ever seen them move. Even though he was upset, I was happy to see him be moving so much. Nurse "L" made my day when she let me hold him for three whole hours! It was so nice to hold my little man and be so close to him. He is even cuter when you hold him. I can't wait until we can just walk into the NICU and just pick him up and hold him like the other parents get to do. All in time.
Gabe had an eye exam today to check out the structure of his eyes. He definately did not enjoy this procedure. First they dialate his eyes using eyedrops. Then they use some sort of torture tool to hold his eyelids open as a guy with a crazy microscope on a helmet looks into his eyes. Poor little guy! The good news is that everything looked normal and they didn't find any of the signs that they can see with kids that have Noonans Syndrome. Which we will take as a good thing.
Please continue to keep us in your prayers and thoughts.

Monday, September 28, 2009

September 28, 2009

As we have learned, after almost 13 weeks in the NICU, there are always ups and downs. Gabe's chest tubes have been putting out only about 10cc's a day for the last few days. Last night, however, they started putting out quite a bit more. I believe one put out something like 70 and the other around 55. This was very disappointing for us to hear because we thought he might finally be starting to dry up. Along with the extra fluid, his numbers weren't quite as stable, yesterday and today, as they have been over the last week or so. His Alubmin levels and IGG levels were low, so he received FFP in hopes of bringing those levels back up. There were a couple other levels that were lower then they should be, but for the life of me, I cannot remember what they are. Dr "B" dropped by to say hello and said it's just going to take "time." We have seen many ups and downs over the last three months, and know that Dr "B" is right, it's just so hard sometimes. More than anything in the world, we just want to bring our little boy home!

Along with his FFP, Gabe also had an abdominal ultrasound, and a full body skeletal x-ray today. Needless to say, it was a very busy morning for Bubba and Nurse "L." The abdomenal ultrasound will be repeated every two weeks to keep an eye on G's liver and the masses. GI wants to see if the masses are growing, and if so, at what rate. The hope is of course that they are not growing. There is a possability they could shrink, so fingers crossed this is the course they choose to take. The full body skeletal x-ray was done to get a good look at all of G's bones and how they are growing. Any time a genetic disease/syndrome is suspected, one of these is done. Gabe would have had this done many weeks ago, however it's only been in the last few weeks that he has been able to be turned and manipulated, and there is much of that involved in this x-ray. I think they took about 19 x-rays of him today. If any of these show abnormal growth/development of bones, it could be another piece to the puzzle, and possibly help with a diagnosis. Gabe is going to be a glow-in-the-dark kid by the time he leaves the NICU. I guess it could be cool party trick in college.

Despite everything going on, Gabe still looks great and is continuing to shrink. The smaller he gets the more aware I become of just how small he should have been when he was born at only 32 weeks. For the most part, he has been pretty mellow and enjoys spending long periods of time looking around. He has been kicking his legs, wiggling his feet and toes, and I actually saw him move is left arm down a bit today. I was really excited to see this because it gives us hope he will not spend the rest of his life in the muslce man pose. G is beginning to have better control of his eyes now, and we rarely see them go different directions anymore. That was always a little freaky. Today they weaned a bit on one of his sedation drips and on his vent. Both very exciting things.

Regarding the possible Noonan Syndrome diagnosis; blood was drawn today to be sent off for genetics testing. It could take approximately 6 weeks to get the results. Waiting...waiting...waiting...so much waiting....

Sunday, September 27, 2009

September 27, 2009

We are finally spending a long overdue weekend at home. Shannon and I have been bouncing back and forth between the hospital and home and only seeing each other in passing and it starts to take its toll. We decided that since Gabe has been doing so well we would have a little "stay-cation" at home with Kayla. Trying to do what normal families do on the weekend and we are very glad we did.

Gabe has been doing very well. In fact, he has been improving each and everyday with only little adjustments to his fluid input and output. They have also been bringing down his ventilator pressures and also bringing down his pain medication drips, all good things. His chest tubes have been putting out less and less each day, getting closer and closer to finally having a dry chest. We even got a special visit from Dr. B, saying Gabe's lungs are the best he has ever seen. If we keep up on this pace of a dry chest and decreased ventilator pressures, we are getting closer to taking his breathing tube out.

Gabe has been enjoying spending time on his stomach and sitting upright in his boppy pillow. We have been working with Gabe's arms each day trying to loosen them up and allow them to rest at his side. What used to take two hours to do is now only taking about twenty minutes. Its absolutely amazing. I actually massaged them down by his side and he fell asleep and kept them down for three hours. His hands are even starting to straighten out, using massage and his new hand splints. He has been having longer periods of awake time and is not fussing, rather he is taking in his surroundings and loves watching his mobile. Even the spa music seems to be helping that he listens to on his custom iPod. The nurses have been seeing a decreased heart rate when they put on his music when he's agitated. That means they don't have to use sedation and that's what we want to hear.

Gabe doesn't know it yet but he has his very own 49ers autographed fire helmet waiting for him at home. The 49ers came over and visited the kids at Ronald McDonald House this past week. They played games with the kids, signed autographs and even BBQ'ed everyone dinner. It was awesome to watch them come and make these kids day. They were surprised at the fire helmet I presented them to sign, saying that this was indeed a first for them.

We are very excited with Gabe's progress and we continue to be amazed at what this little guy can do. He just never gives up and always pushes forward with absolutely incredible strength and determination, something we can all learn from. Thank you for your continued prayers and thoughts. Talk to you again soon.

Wednesday, September 23, 2009

September 23, 2009 - 12 Weeks Old

Just when we thought things might finally be falling into place...along comes genetics.

Jesse and Gabe were hanging out this afternoon, minding their own business, when in walks the genetics team. They have been following Gabe from day one since he is a complete mystery. They did a full genetics work up on him in the first month and everything looked as it should. I guess I should say, all the usual genes they look at, looked fine. There are billions so it's hard to get a good look at every one of them. Since adding Chylothorax to Gabe's list of "things," genetics now feels he may have something called Noonan Syndrome. Apparently they feel that G's eyes are a little further apart then "normal;" his legs and arms are shorter then they should be; along with a couple other things they rattled off; and with the now diagnosed plural effusions being Chylothorax; these are all characteristics of Noonan Syndrome. They drew some blood for further genetics testing. There are three specific genes they will be looking at now. It will take about two weeks to get the results back and even those are only something like 70% accurate. Jesse and I have spent A LOT of time staring at Gabe over the last 12 weeks, and never once have I noticed any of these things genetics is talking about. I know I'm not a professional or anything, but I was really surprised when they started talking about some of his measurements not being "normal." So what is Noonan Syndrome you ask???

Per the Noonan Syndrome Support Group it is:

"* It is believed that 1 in 1,000 to 1 in 2,500 children worldwide are born with this condition.
* Each day a child is born with the condition.
* It’s possible that people carry the gene yet are undiagnosed.
* Once affected, their is a 50/50 chance of passing the gene on to one or more of their children.
* It can also occur sporadically, presumably due to a new mutation.
Often called a “hidden” condition, the children affected may have no obvious casual signs to the onlooker, but the problems may be many and complex. A clinical test is available, in some cases. As of 12-03-06, 3 genes have been identified. This is a genetic condition that can affect the heart, growth, blood clotting, mental and physical development. Affected individuals may have behavior problems, learning difficulties and many other anomalies. Noonan Syndrome is one of the most common of those conditions associated with congenital heart abnormality.
Still its exact cause remains unknown….."

Good old Wikipedia has a pretty thurough description of the Syndrome along with a list of manifestations. There were things in almost every category that applied to Gabe, however a lot of the things were pretty general. I bet most of us could find a few things from the list that could be applied to ourselves.

Instead of freaking out about this Noonan Syndrome, Jesse and I have decided to stash it away in the back of our minds, as much as we can, and wait for the results. No since in worrying too much about something we have no control over. He is still our son regardless; we won't love him any less; and should he have special needs because of this syndrome, then we will deal with them as they come. Gabe seems to finally be on a good path and we don't want to let this new information get in the way of us enjoying our son. Yes, genetics definately took the wind out of our sails this afternoon, with this information and possible diagnosis, but we are recovering, and just want to continue loving our Gabriel for the precious little person he is.

Tuesday, September 22, 2009

September 21-22, 2009

I'm sitting here staring at the computer and my mind has completely blanked out. I just spent the last few days with Gabe and I cannot think of a singal thing to tell you all about him. Oh my gosh...It's offical...I've lost it!!!


It used to be that we'd sit down to blog and we didn't even know where to begin. There was just so much going on with Gabe, but lately things have been pretty mellow. This is of course a good thing. Other than waiting for his plural effusions to dry up; his lungs to get stronger so he can be extibated; and weaning him off of his happy juices; there really aren't any other major things to report. Wow! This is awesome!!! We are of course still concerned about his chylothorax drying up on its own. If the lymphatic system can heal on its own, this usually happens 4-6 weeks after all feeds have been stopped. I believe Gabe is now going on about 2 weeks of starvation (he is getting TPN and Lipids intravenously but that just isn't the same as real food), so fingers crossed we should start to see some signs of improvement in the next 2 to 4 weeks. If his plural effusions are still present after 6 weeks of no feeds then we may need to look into a medication called octreotide. I really hope we don't have to go down this path. The poor little guy has had so much junk pumped into him over the last 12 weeks, I really don't want his body to have to process one more thing. Gabe is starting to have more "good" days then bad. We are beyond excited about this, but still have our guard up at the same time. We have seen him go backward so many times, we are still a little cautious when things are going well.

Since Gabe is no longer being poked and prodded all day long, he now spends his time snoozing; watching his mobile; jamming to his spa music; getting massages to help with his tight muscles; taking sponge baths; trying new ways to lay in bed, like on his tummy and his sides; looking around at the boring NICU room; and listening to mommy and daddy blabber on and on about how adorable he is. I know I've said this before, but Bubba is seriously cutest little boy I have ever laid my eyes on. Now that he is on less sedation he has more awake time, and it's so awesome to see him with his eyes open. He is starting to do more "normal" baby things and this is really exciting! We know that there are still many hurtles he must overcome, but considering what the little dude has conquered thus far is his 12 weeks of life, I have faith that Gabriel can handle any obstacle thrown his way.

Go Super G go!!!