Back On High Frequency Vent

Well we tried, but Sofia wanted her high frequency vent back. Over this evening, her CO2 started rising again and got up to the 70's, so they switched her back about 30 minutes ago. Her CO2 is already better and back in the low 60's. Hopefully this will make her much happier. Dr. H said some babies just like the high frequency better, and if they need to, they can wean her on the high frequency until she is ready to be extubated. So it looks like we probably won't be able to hold her quite as soon as we had hoped, but as long as she is doing better that's ok.

Sofie's Mommy

Celebrating CDH Awareness Day On The Conventional Vent!

Little Sofia decided the best way to celebrate CDH Awareness Day was to switch to a conventional vent. Yeah Sofia!! She's been on the conventional vent for about an hour now and is doing well on only 50% oxygen.

Results from her echo at noon came back really good. Her pressure is now at 41 down from 49 on the last echo, so her pulmonary hypertension is getting better. They will stop her milrinone today, so that's one less med. They are still weaning down her dopamine. It's at 8 now and her pressures are stable around 50. Her hr has been in the 140's today, so much better than 170's!

The swelling in her face is a little better than it was this morning. She could actually get her eyes open a little. Her poor little ears are still so swollen, they look like they are almost swollen shut. The preliminary reports from her SVC ultrasound this morning all look good too, so that is a big relief. Dr. H thinks now that she is back on the conventional vent and she can move around more, her swelling will continue to get better. She also got a dose of Lasix and is peeing up a storm right now, so that will help a lot too.

So now she needs to continue to do well on the conventional vent. Then they can start to wean her nitric oxide in the next day or so. Hopefully she'll get rid of the dopamine soon, and that will be another med gone. That would just leave her maintenance fluid, TPN, lipids, vancomicin, gentamycin, morphine, and versed.

She'll continue the antibiotics for a few days to be sure her infection is gone. She did have one positive blood culture in addition to the positive culture from her right chest tube site. They are repeating her blood culture today and also sending a trach culture from her ET tube.

Once she no longer needs her chest tubes (hopefully in the next few days), they can be removed and we could hold her while she's on the conventional vent. We can't wait!!!

By the way, keep the turquoise pictures coming, they are great!! I also got an email from our friend, Megan, in Lawrence. She took 4 coworkers (all wearing turquoise), and they all donated blood today in Sofia's honor! Several of Kenny's coworkers have also gone to the Community Blood Center and donated blood in Sofia's honor as well. Thanks guys for spreading CDH Awareness and for donating blood - you guys are awesome!!

Sofia's Mommy

Might Be Switching To Conventional Vent Today!

Sofia is doing pretty well today. They weaned her MAP (mean airway pressure) down again on her vent to 13 and are planning to try her on the conventional vent today! She has always had a big air leak around her ET tube, even though it's an appropriate size. They even changed her to a 4.0 when she was on ECMO and it still leaks. So it appears she has the world's largest airway. This is one reason she likes the high frequency vent better, because it reduces her air leak, so we'll see how she tolerates the conventional. She is getting her post-op echo today to see if her PPHN is better so they can hopefully d/c the milrinone and start weaning the nitric oxide.

Her blood pressures and heartrate are better today now that she's had 24 hours of antibiotics, and she is peeing more since her albumin replacements. She'll get a dose of Lasix today to help her get rid of some more fluid too. Her chest and head are still so swollen! She had a positive culture (she has an infection) already last night, so I'm sure that had a lot to do with her low b/p's and high hr's the last couple of days. Turning her yesterday afternoon released a bunch of fluid from that left side of the chest, and she immediately started doing better with everything, so it's kind of a combination of things that's really helped her.

At any rate, it looks like she's trying to turn the corner. She's still really sensitive to position changes and desats to 70's-80's occasionally when she's on her right side. On her chest xray this morning, there is more fluid accumulation and less air around the left lung, so hopefully that air leak is resolving, and now some of of the fluid will start to reabsorb and that will get better.

She also had an ultrasound of her superior vena cava (SVC - large vein that carries deoxygenated blood back to the heart from the upper body) this morning to rule out the possiblity that an obstruction of her SVC could be causing all the swelling in her chest and head. This is a scary condition that could be caused by a number of things. We may have results back late this evening or tomorrow. I'll post more if anything develops with that, but we'll hope this isn't the case.

I'm so excited because in rounds today, they started talking about when to do her post-op MRI. She's never had one because she's been so "hooked up" her whole life, so she couldn't make the transport down to radiology to go through the machine with all of it. They want to do it when she is about ready to come off the conventional vent, so they can sedate her more easily while she is still intubated. Then after they do her MRI, she might be extubated. Dr. S threw out possible MRI on Thursday today, so that would mean they think she might possibly be ready to be extubated as early as Thursday or Friday of this week!!!! We might get to hold her in a few days!! I'm so excited. She's 3 weeks old today, and she's never been held. It makes me so sad for her. Hopefully she'll continue to behave and take big strides toward being tube free!!

Sofia's Mommy

Turning The Corner

Sofia is continuing to improve this afternoon and tonight. We all haven't decided what did it for sure. Dr. J repositioned her chest tube around noon today. Then around 4:00 pm or so, her nurse Sunny turned her to her back and within a half hour, 16cc of fluid had drained from that left chest tube. Then Dr. H came by for a visit, and Sofie must have thought she better shape up for Dr. H. But I really think Sofie just finally breathed a sigh of relief when Davidson missed their final shot at the buzzer! Whatever it was, she definitely turned a corner this afternoon and has improved a lot.

They were able to wean her oxygen setting on her vent down to 34% late this afternoon. Her blood pressure has improved and is back up to 45-50, right where they want it to be. Her heartrate has come down from the 170's where it's been the last couple of days to the 150's. And her O2 sats are 97-100%. She has been doing so well, Dr. J was finally able to start weaning her high frequency settings a little tonight. Her MAP (mean airway pressure) was able to come down to a setting of 14 from 16. This just means she is needing less support from the high frequency ventilator. Her oxygen only had to be increased to 44% with this change to keep her sats in the upper 90's. If she continues at this rate, she may be ready for the conventional vent in a day or two! Go Sofie!! She wants to be off high frequency so she doesn't have to be so sedated for the Final Four next weekend.

Don't forget-tomorrow is the first annual Congenital Diaphragmatic Hernia Awareness Day! Missouri, Kansas, and Kansas City, MO have all signed official proclamations, along with 32 other states and 13 cities. Several more are pending. Mexico is the first country to sign a national proclamation and the U.S. national proclamation is in the works. This is amazing considering Breath of Hope just started this campaign for awareness 6 months ago! Thanks to the determination and hard work of parents, grandparents, friends, and family members affected by CDH, this awareness campaign has really caught fire, and more people are learning about CDH every day. Raising awareness will help to increase research that might find the cause of CDH and someday may lead to a cure or even better - prevention!! Wear your turquoise tomorrow to support this cause, and try to tell at least one person about CDH. It will make a difference! Don't forget to email me a picture of you wearing turquoise to show your support for Baby Sofia. Please send them to jtmku@yahoo.com and I'll post them to the blog. Here is a little preview of some of Sofia's pals getting ready for CDH Day...

Sofia's Mommy

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Sweet Little Feet

Sofia had a better night last night. Today she has been stable. Her blood pressure is still a little on the low side, and her pulse is still high, but her blood gases have all been very good. She's still on the high frequency vent with her oxygen setting at 65%. They repositioned her chest tube on the left side around noon to try to get some more of the air and fluid around that left lung to come out. She just had a chest xray at 4:00 pm, and it showed a little bit of an improvement in getting that left lung reexpanded after that big air leak yesterday morning.

She seems very comfortable today and looks like she has good pain control. They seem to have found a good level for now with her morphine and versed drips, so they haven't changed them from yesterday. Her morphine is still at 140, and her versed is still at 110. She hasn't needed as many extra doses of pain medicine today.

Her incision is a lot less red, and her white count has gone down since yesterday with the antibiotics on board. She has a low grade temperature again this afternoon. It's still early, but her cultures so far have shown no growth of bacteria, so that's good news. We won't know for sure on those for another day probably.

Her chest and head are still very swollen. They are replacing the fluid volume that drains out of her chest tubes. She gets 1/2 cc of albumin (albumin is a large protein found in the blood) for every 1 cc of fluid that drains from her chest tubes. She's also getting 2-3 additional boluses of albumin per day to help draw some of the fluid that's causing her swelling back into her vascular system, so she can pee it off. This should help not only her swelling, but also her blood pressure and heartrate.

Yesterday afternoon they removed the dressing and drain from her ECMO cannula incision site. The incision is about an inch long on the right side of her neck, and it's already healing so well! I think you'll barely be able to see where it was once it is all healed. She already has 5 major battle wounds, so hopefully they will all heal as well as that one.

We actually ventured out of the hospital yesterday afternoon for a couple of hours to try to find Sofia some furniture, so she has a place to rest her cute little head when we take her home. We didn't find anything we liked yesterday, so we're still looking, but Daddy did pick out some little socks to keep her feet warm. And of course I had to make a little scrapbook of her first socks :-) . . .

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Superstar!

I was so excited about the chest xray earlier, I just realized I forgot to post an update about last night and today. Last night Sofia's heartrate was a little high, and she seemed more agitated and in more pain. Last night her little fists were clenched so tight. I could tell she was in pain. Her nurse was giving her pain meds, but they just weren't quite enough to catch up with her. Over the night and this morning, her morphine and versed drips were also increased a few times to help manage her pain better. Her morphine is now at 140 and her versed is at 110. She seems to be more comfortable and relaxed today.

Her blood gases continued to look good all night until around 5:30 this morning, when her pH was a little acidotic, and her CO2 had risen from the 40's to mid 60's. Her heartrate got up to the 190's, and her blood pressure was in the 30's. To help raise her blood pressure, they increased her dopamine drip to 12. They did a chest xray and found that she had a large air leak on the left side, and her chest tube on that side needed to be repositioned as well. They air leak had caused her heart to shift back over to the right, compressing her good lung, which is what caused the change in her blood gases. They inserted a needle into her chest to withdraw a large amount of air and fluid and then adjusted her chest tube, and she quickly got better. Her gases all day have continued to be very good and stable. Because she lost quite a bit of fluid from her chest last night, they needed to replace that volume, so she is getting a unit of blood right now.

Her temperature was a little unstable overnight and today, and she had some green drainage on the dressing around her chest tube site on the right side. They changed the dressing, and her skin wasn't reddened at all around the area. The site itself didn't really seem to have any green drainage, so that was good. As the day went on, her heartrate remained elevated in the 180's, and her blood pressure was running a little lower in the upper 30's - lower 40's. Her white count was also elevating, so they sent some cultures to see if she might have an infection. It will be 24 hours before the culture results are back to know for sure, but they went ahead and started her on some antibiotics - gentamicin and vancomycin. They will repeat her labs in the morning, and hopefully she will start getting better after about 24 hours.

Sofia's Mommy

Sofia Continues To Amaze Us!

We were able to see Sofia's chest xrays this morning from just before her hernia repair surgery and then her most recent one after the surgery taken at 11:30 am today. A lot of CDH babies have only the one good lung on the unaffected side and just a little lung bud that develops on the affected side. Because so many organs and so much bowel is herniated into the chest cavity, it keeps the lung on the affected side from developing. Sofia's right lung on her unaffected side grew very well to a normal size, even though her heart was shifted over into its space. Sofia had most of her bowel, her stomach, spleen, and part of her liver herniated. On the before xray, you can see she has bowel almost up to her clavicle! But still she continues to amaze everyone. On her "after" xray you can see her left lung beginning to inflate and expand, and it looks almost full sized!!! Way to go Sofia! To us, this is truly a miracle!

Sofie's Mommy

Before Surgery

After Surgery

Rock Chalk Sofia!

Sofia is doing great today, even better than expected! Her blood gases have all been really good throughout the night and today. She is on low settings on the high frequency ventilator, and she still has her nitric oxide, but she is close to being able to go back to the conventional vent soon. She is only needing 46% oxygen right now on her vent, and is keeping her O2 sats between 95-99%. She is keeping her CO2 levels way down today and seems to be ventilating much better. They have increased her pain medication over the night and some more today to keep her comfortable. Her morphine is now at 100 and her versed is at 70. She gets extra doses of morphine, versed, and fentanyl when she needs them. She is less swollen today than she was yesterday, and her incision is already starting to look better. She hadn't had a wet diaper in a few hours last night, which they weren't too surprised about since she was so heavily sedated. So she got her foley catheter back and her urine output is ok. Overall she is doing fantastic and has been very stable since her surgery. We couldn't ask for more!

Sofia is just resting and healing and getting ready for the big Jayhawks game tonight. Rock Chalk!

Sofie's Mommy

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Still doing good

While it is still early and things could change at any time this is one proud poppa. It is almost 12 hours after her insides were on the outside and her numbers look great considering everything she went through. I stand over her and would trade places in a minute, but I don't think I would be 1/2 as tough as she is. It's almost like she is telling me "don't worry dad, I got this under control." We still got a long way to go but I think she will be able to handle anything in front of her. Keep the good vibes coming her way to get her smoothly through these tricky hours after surgery.

Sofia's Dad

Surgery Details

At 17 days old, after 12 days of ECMO, and 2 days off ECMO, Sofia had her hernia repair surgery. The surgery lasted about 3 hours and 15 minutes. 90% of the left side of her diaphragm was missing. She had just a small anterior rim of diaphragm, and her stomach, intestines, spleen, and part of her liver had herniated. Her right lung is a normal size, and the surgeons did say they could see some lung tissue on the left side, but it will be a few days before we know how much is there.

She did amazingly well during her surgery and continues to be stable this evening. She didn't need to receive any blood or blood products during her surgery. She received one unit of blood after surgery, mostly as a precaution, but her blood loss was minimal considering how invasive her surgery was. Her abdomen is pretty swollen after surgery and will likely be much more swollen over the next couple of days. After being removed from her chest, her herniated organs were placed outside of her body while the surgeons placed a large patch over the area where her diaphragm was missing. Her organs were then placed in her abdomen once the surgeons made sure the patch was secured. Because of how much those organs were handled, particularly her intestines, they will swell after surgery. This is normal and will resolve in a few days. We are expecting the next 24-48 hours to be pretty rocky and have a few setbacks, but she should pull through it just fine and really begin her healing process.

Because her defect was so large, she does have a patch. Her patch is an AlloDerm patch, which is made from donated human skin tissue. It provides a biological matrix for her own tissue to grow through. The original patch material eventually dissolves, leaving her new tissue behind. This type of patch will hopefully lessen the chance for reherniation later. However, reherniation is always a possibility, especially with the size of her defect.

Before the surgery, her heart and lungs were shifted significantly to the right side of her chest. Now that the herniated organs are in their proper place and there is room in the left side of her chest, her heart and lungs will slowly shift back into the proper position. The body doesn't like to have an empty space, so the space where the organs were in the chest is filled with air and fluid right now. For this reason, they placed a second chest tube on her left side to help remove a small amount of that air. They don't want to quickly or completely drain the fluid off that space, because it could cause the heart and lungs to shift drastically over too far to the left. By leaving that fluid in there, it allows a gradual shift that is less traumatic, and the fluid will slowly reabsorb into the body on its own.

Her incision is large, about 6 inches in length, and goes about 2/3 of the way across her abdomen. As her Daddy says, that's one big owie! Her skin is closed with sutures in the layers beneath the surface of the skin. These are called subcutaneous sutures and will dissolve. The part of the incision on the surface of the skin is closed with a type of clear gel called DuoDerm that's kind of like superglue.

We are so glad to have this big hurdle behind us, so Sofia can start to heal and get ready to come home with us. We still have a long road ahead, but this is one major step we have finally been able to get past. We pray for her continued strength and healing, that she is comfortable, and feels her Mommy & Daddy are here with her. It's so hard to see your little baby laying all by herself on an OR table in a big room with a big incision that looks so painful, and not be able to pick her up and hold her and make her feel better. Hopefully it won't be long until she is better, and we can finally hold her. Here are some pictures from her big day and her battle wound. Just a warning, if you have sensitive eyes, you may not want to look! Thank you, everyone, for your amazing prayers today. God has been with Sofia protecting her before she was even born, and she is truly a miracle baby.

Sofia's Mommy

Good morning, Sweet Sofia!

Sugarbear is fixing Sofie's hair

Seriously, Sugarbear, a fauxhawk?

Getting ready for the big surgery

One of the Respiratory Therapists, Dot (Sofie's nurse), Dr. P (anesthesia), & Dr. H

Sofie's pretty little tummy before & after the surgery

The Big Owie! (Don't worry, it'll get better)

Surgery Went Great!!

Sofia's repair surgery went as well as the doctors could have hoped. We got in to see her just a few minutes after they finished. They finished around 4:45 pm, so the surgery lasted about 3 hours and 15 minutes. She really did great, and her first blood gas after the surgery was very good. She just had another gas done that was also very good. She looks very swollen, and her color is not as pink, but that is very normal after surgery, especially a surgery as major as the one she had. Her incision is pretty large and looks pretty painful, but Dr. H and her nurse, Dot, are keeping her sedated and comfortable. We'll post more details later about the surgery. Just wanted to let everyone know she is doing well, and came through her surgery with flying colors thanks to the skills of her doctors, nurses, and RTs. And especially because of all your extra prayers. Thank you!!

Sofia's Mommy

Surgery Update #3

We just got another good update from Dr. H. Sofie is continuing to do really well during surgery. They are just about 2 hours and 15 minutes into it. Her patch is in, and they are just making sure it's all secure. They also took out her appendix while they were in there just to avoid having to possibly take it out later if there were other complications. They turned up her oxygen at the beginning of her surgery to 100% to provide extra support with all the stress of surgery. But her O2 sats have been 100%, so they started weaning down her oxygen on the ventilator. It's now at about 60%, which is really good. That leaves somewhere for her to go afterward when things get a little rocky. Things are going really well so far, and they are getting closer to being done. Hopefully the next update we get will be that they are finished, and we can see her soon!

Sofia's Mommy

Surgery Update #2

Dr. H just came in and told us Sofia is doing really well. The surgery actually started right around 1:30pm, so they are about 45 minutes into it. Her O2 sats have been 95-100% during surgery. Dr. H said all of her intestines have been moved down into her abdomen, and the surgeons have said that her defect appears to be pretty large. That's all we know for now. They will come and talk with us in more detail when the surgery is finished, but I wanted to let everyone know that Sofia is doing well.

Sofia's Mommy

Surgery Update #1

We have kissed her goodbye and told here to behave for the surgeons before they kicked us out. They should be starting anytime now. Dr. H is going to stay with her through the surgery so she can come and give us updates. So when we get some news we will try and let everyone know.

Sofie's Dad

Surgery Day!

Sofia had a really good night last night. Her ABG's have been very good, and she's still nice and stable for surgery. She is getting a unit of platelets this morning, so she'll be good and ready for surgery. She's on the schedule for 1:00 pm, so please send extra prayers her way at that time. Here is a video of her big move yesterday back to the OR. (Move #3) Sorry it's a little long! If you fast forward to the end there are some closer shots of Sofie! We also made a little scrapbook of her adventure yesterday, see it below.

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CDH Blood Drive Success!

Thank you to all who donated at the CDH Awareness Blood Drive yesterday at Menorah, including Grandma Cheryl. They were expecting 20 units and ended up getting 31 units of blood! Way to go everyone! I also know of a few others who had donated recently and were just a few days shy of being able to donate again, so they are planning to donate next week in Sofia's honor. Several others were unable to make it to Menorah to donate, so they donated at other sites, so thank you to you as well! Every unit donated makes a big difference!

The first annual CDH Awareness Day is Monday, March 31st. Be sure to wear your turquoise in support of all CDH babies, including little Sofia! If you're looking for official CDH Awareness Day gear, click here. If you would like to order CDH silicone bracelets, you can find them here. Or click on the pictures below. Also, if you wear turquoise on Monday, send me your photo at jtmku@yahoo.com and I'll put you on the blog next week!



Thank you so much to everyone for your support and prayers, it really lifts us up during these rough times. We are so lucky to have such a strong support system. Sofia even got an email from her friend, Garrett, who can't wait to meet her when she's all better. Garrett is my friend Melissa's sweet little boy, and he had his Mommy write a special email from him to Sofie. Here it is...

Dear Baby Sofie -
I love you and hope you get better so you can snuggle with your mommy. I told the Easter Bunny to bring you a new book and a whistle like mine. I like your pink bow, but I want you to have a blue bow like Cinderella. I'm going to make you a blue bow, and a card for your room. I want you to take the tube of your mouth because I can see your mouth. When you get better I want to see you. ( Exact dictation from a 4 year old )

Surgery Not Happening Today

Sofia gave us a bit of a scare this afternoon while we have been waiting to hear from the surgeons. Around 1:00pm she started to get more acidotic, and her CO2 started rising again. This has been the trend when she has taken bad turns in the past, so we were very scared since she's off EMCO now. She continued to oxygenate well, but was just having problems blowing off her CO2 again. She has a fairly large air leak around her ET tube still, and they think that's what was causing this change. So they switched her back to the high frequency vent at 100% oxygen, and her numbers improved very quickly. They gave her more sedation and increased her morphine and versed drips to 70 and 60. Her blood pressure was creeping back up to the high 50's with the dopamine at 10, so they dropped down to 9 on it.

She seems to be doing much better now, but we are very nervous we will miss her very narrow window of stability for her repair surgery. The most upsetting news this afternoon is that the surgeons will not have time to do her surgery today. We feel such a sense of urgency to get it done while she's doing ok, so this has been really upsetting. As of now, her surgery is scheduled for around noon tomorrow. Please pray that Sofia continues to hold steady throughout the rest of today and tonight while she is waiting for her surgery to be done tomorrow.

Sofia's Mommy

Knock on wood

Sofie and I have had a pretty boring first night off ECMO. She has been keeping her sats in the upper 90's and her BP in the mid to upper 40's. She just got her latest blood gases and her CO2 level was 49, which means her lungs are doing a pretty good job keeping up. Her only real issue tonight has been that her heart rate and temperature are running a little high. But we have been told that the higher temp is pretty common with babies just coming off ECMO. And one of Sofie's favorite nurses Sara, who is taking care of her tonight, thinks the higher HR may be linked to the high temp. So she has been getting doses of Morphine to relax her and keep her HR down and it seems to be working.

I think she is trying to tell everyone she is ready to get all fixed up today. So let's just hope she continues to behave and the Doctors agree. When we hear a more definite time for surgery we will let everyone know.

Sofie's Dad

Sofie Continues to Rock

Sofia is continuing to do really well off ECMO! She didn't need to go back to the high frequency ventilator. She's still on her conventional vent with a little nitric oxide. They restarted the nitric today at noon at half of what she'd been on before ECMO just to optimize her lung function. Her vent settings have weaned way down in the last hour and her ABG's have been really good. Her B/P is holding steady in the low 40's with O2 sats 97-99%. What a rockstar!! As of now, her repair surgery is scheduled for tomorrow, possibly sometime around 2:00 or 3:00. We will know more tomorrow morning. Here are some pictures of before and after ECMO decannulation.

So far so Good!!

We haven't got to go back and see her yet, but the Doctors just came and told us that she is off the ECMO and is doing good. If she continues to be a little superstar through the night they will most likely do her repair tomorrow afternoon. Thanks for all the prayers and good vibes. I think she is hearing them. We will update later when we know more.

Sofie's Dad

Day 12 (Last Day!) of ECMO

We just talked to one of Sofia's surgeons (Dr. Pablo), and her decannulation surgery is set for between 3:00 and 4:00 this afternoon. We'll be glad to see the cannulas and the ECMO machine gone, but at the same time it's so scary. We hope she can do it on her own! Please pray hard for her today that she'll have the strength to fight even harder and get those lungs functioning better!

Sofie's Mommy

Sofia Passed Her Clamp Test!

Well the original plan today was to challenge Sofie by weaning her to a low setting and let her sit there overnight. And then depending on how she did, wean her completely tomorrow. Once they weaned her down completely tomorrow, they would attempt to clamp her off ECMO for a couple hours to see how she does without it and then take out her cannulas and be completely off if she does ok clamped.

The plan changed a little this afternoon. She did so well with her challenge today, that they decided around 4:45 this afternoon to go ahead and try the clamp test. She did ok clamped for 2 hours, so they've decided that she will be stable enough to come off ECMO for her surgery! Her decannulation surgery is set for tomorrow. We are still unsure of the exact time, but she will be officially off ECMO sometime tomorrow. After the clamp test this afternoon, they unclamped her and turned her flow back up to maintain her through the night. They don't want to do the decannulation surgery during the night, so they will hold tight and wait until tomorrow when the full crew is here. This is really exciting, but at the same time very scary. We just hope she is truly stable enough to withstand everything off ECMO, because there's no going back once it's gone. On the other hand, surgery is much less risky off ECMO, so it's good that it looks like we're proceeding in that direction. They also repeated her echo this morning and her PPHN is better. Her pressure was 49, much better than the last echo on Friday which was 89-90!

If all continues to go as planned, her repair surgery will be sometime around noon on Wednesday. So hopefully Sofia will cooperate and stick to the plan this time!! It's going to be rocky for the next few days, so Sofia really needs some extra prayers to get through it. Please pray that Sofie continues to be stable off ECMO, through her surgery, and in the days afterward.

Sofia's Mommy & Daddy

Day 11 of ECMO - Weaning a Little More Today

Sofia did really well over night weaning down to 300 on her ECMO flow, so they've continued to wean her down some more. When we got here this morning, her flow was at 200 and they are taking her down to 100 pretty quickly to see how she does. If she does well, they will try to wean her completely tonight and take her off tomorrow, with possible surgery Wednesday. As she goes down on her ECMO settings, they're increasing her ventilator settings, since she will have to be taking over and doing the work of oxygenating without so much ECMO support. Her oxygen challenge test was over 300 today! They repeated her echo at 1000 this morning to check her pulmonary hypertension again, which is hopefully getting better. They are keeping her much more sedated now, so she doesn't waste her energy moving around and being awake. Also, the more she is awake and moving around and trying to breathe over the vent, the more her stomach and intestines fill with air, which makes it harder for her good lung to work. Her Morphine and Versed dosages have increased from 50 and 40 to 60 and 50 to keep her asleep today.

Rock Chalk!

CDH Awareness Blood Drive Monday 3.24!!!

Just a reminder that tomorrow is the CDH Awareness Blood Drive at Menorah Medical Center in honor of Sofia Rose! Menorah Medical Center is located at 5721 W. 119th St. in Overland Park, which is at the corner of 119th and Nall. (See map below) The blood drive is in Conference Room 1 off the main lobby at "A" Entrance from 11:00am-3:00pm. You can make an appointment to donate here. Click on "Find a Drive", then enter sponsor code menorahmedctr. There are still appointment times available between 1:15 and 2:45. I know many of you have expressed interest in donating, but are unable to make it to the blood drive tomorrow. If you want to donate and can't make it, you may contact the Community Blood Center by calling 1.888.647.4040. For those that already donated at another location in Sofia's name, thank you for saving a life!! Little Sofia is getting 2-3 platelet transfusions per day, in addition to blood transfusions, that save her life. Your donation makes a difference!!

I also want to thank my friends, Julia & Melissa, for helping to prepare so much for the blood drive, for coordinating with Menorah for me, and for being there tomorrow. You guys are such a huge help, there's no way I could have done this without you!!

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Happy Easter!

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Day 10 of ECMO

Sofia is holding steady and had a pretty good night. Her oxygen challenge test was 276 this morning, and everything else has been stable. They are going to try to wean the flow down on the ECMO circuit from 450 to about 300 over the rest of today and tonight and let her sit at 300 all day tomorrow to see how she tolerates it. Depending on how she does with that, they will talk about possibly weaning again on Tuesday. If she doesn't tolerate it, they will talk about going ahead with the repair surgery on ECMO. One way or another, she will most likely have her repair surgery sometime this week. We're still hoping she can come off ECMO before surgery, but if not we're ready to get the surgery behind us and try to move forward.

She has been sleeping well since we got here this morning, so she doesn't have her "Easter outfit" on yet. Never fear, there will be pictures later today!!

Sofia's Mommy

Photos From Sofia's 1st Week

Photo and video editing at www.OneTrueMedia.com

Sofie's First Smile - 3.17.08

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Sofie's First Movie

Day 9 of ECMO has been a little better. Sofia is responding really well to the Milrinone for her blood pressure. Her blood pressure came down last night shortly after they started the medication from 70-75 to 58 and today it's been upper 40's-mid 50's all day. Much better Sofie! She is keeping her O2 sats around 97%-100% too. Her oxygen challenge test was a lot better this morning. Yesterday she only got 88 and today she got 297!! Yeah, overachiever! Her ABG's are also stable and looking good today with great CO2 levels in the 40's. Her chest xray was also better today, showing less fluid on that right lung and better expansion. Her urine output after the 2 doses of Lasix was very good, so she's back on track fluid-wise. She slept more last night and today with the increased dosages of Morphine and Versed. No new plans as of now. They are just letting her rest and hoping her PPHN resolves some before attempting to wean off ECMO again.

And as promised, here is Sofie's first movie! This was from yesterday when she was so alert and awake. She's so cute!

Photo and video editing at www.OneTrueMedia.com

Setbacks

We are on the CDH roller coaster, that's for sure. Sofia's echo came back this afternoon showing her pulmonary hypertension (PPHN) is getting worse again. Her pressures are now the highest they have been at 89-90, so that is really discouraging. They are starting her on Milrinone, a drug to help lower her blood pressure. They are continuing to increase her vent settings just a little bit to help that right lung expand a little better. She got a second dose of Lasix this afternoon to help her get rid of more of her fluid to see if that helps as well. She's also been very alert and awake most of the day today, so they are increasing her sedation for the first time since she got here. Her Morphine and Versed have been at 40 and 30 and are going up to 50 and 40 now. She doesn't seem to be in any pain, but the increased sedation will help her rest a little better, and the Morphine will also help to lower her blood pressure a little bit.

The plans for weaning off ECMO have changed again, as you might imagine. Every time we make a plan, little Miss Sofia decides to change it for us! The plan was to try to wean again tomorrow night and into Sunday morning, possibly removing her cannulas on Sunday and surgery on Monday or Tuesday. Now we will let her rest over the weekend. They'll reevaluate her on Sunday and possibly try to wean again Sunday or Monday if she is stable enough. If not, or if the wean isn't successful, we'll discuss going ahead with the repair surgery on ECMO.

Repairing on ECMO has many risks, but mainly bleeding is the biggest risk. Her blood has to be so thinned while on ECMO to prevent clots from forming that she's at a very high risk for hemorrhage during surgery, particularly for bleeding in the brain as well. Both of these risks are very scary, but if she's not stable enough to come off ECMO that will be our only option.

Please continue to pray that her PPHN resolves, so she can truly be stable enough to come off and stay off ECMO!!

Sofie's Mommy

Day 8 of ECMO - Not a Fan of Thursdays

Well, little Sofia just doesn't seem to like Thursdays very much. Last Thursday was when she had to go on ECMO, and last night she had a pretty rough night again. Her oxygen levels weren't as good, and she was having problems getting rid of her CO2. Her sats were all over from 77%-97%, and they had to increase her flow on ECMO to 390 where she had been running 280-290 last week. The increased flow has caused her blood pressure to be a little higher, but not so high that she needs medication yet. They repeated her echocardiogram this morning to see if her PPHN is getting worse again, which could be the cause of her problems.

This morning she only got 88 on her oxygen challenge test after performing like a rockstar yesterday and getting 383. Her chest xray this morning showed some more fluid collection in that right lung again, so they have adjusted her vent settings to try to help that. She also mistakenly received more IV fluids last night than she was supposed to. Her TPN ran in too quickly, so she is a bit more swollen today and getting some lasix to help her get rid of that extra fluid. Thankfully her blood sugar and electrolyte levels weren't affected by this. She really seems to have handled it pretty well.

Despite all that she is doing better today. Her O2 sats have been 96%-100% today and she has been awake looking around for us, stretching her legs, and looking pretty content to be awake snuggling her Sugarbear even though we tell her to go to sleep and rest. She is stubborn!! Wonder where she gets that from? We got some cute video today of her looking around at us that I'm sure is only amusing to us, but I will post it to the blog for everyone's enjoyment just in case. She is such a cutie!!! I can't wait to be able to hold her!

Sofia's Mommy

Day 7 of ECMO

Sofia had to move out of her big OR suite and back to her old spot in the ECMO room this afternoon. She doesn't tolerate moves very well and her sats are down a bit after the move, but overall she is holding steady. The plan will be to move her back to the OR suite sometime on Saturday. They will attempt to wean her off ECMO again Saturday night into Sunday morning, with possible cannula removal Sunday and then repair surgery Monday or Tuesday. She is still on the conventional vent without nitric oxide, so that leaves somewhere to go if she needs it at a later time. She also never needed to go back on any blood pressure medications, since she initially went on ECMO, so she'll have those to add on if she needs them too. We are still very hopeful she'll be able to come off ECMO for her surgery.

Sofia's new (old) room

Mommy gets to change Sofia's diaper for the first time (Tuesday night 3/18)

Daddy gets to put on Sofia's lipgloss (Tuesday night)

Not Quite Ready

Well we tried and little Sofia just wasn't quite ready to come off the
ECMO today. Everything was going pretty good, but she just wasn't able
to keep her CO2 levels down in a good range once they clamped her off.
The doctors said there were drugs they could have tried to help her
out, but they felt that since we were getting close to our time limit
for being clamped off that it was best to just turn it back on and
give her a few more days to rest.

They replaced her breathing tube with a larger one to help with her
with her air leak during the time they were trying to clamp her off.
So while she doesn't sound like a duck anymore Dr. H thought
this might have been a little more than she could handle during that
time and she just didn't have enough time to recover before they had
to make the decision.

So while we are disappointed it is better to give her some more time
than to take her off before she is good and ready. Because once we are
off there is no going back. I think the real reason she didn't come
off today is she wanted to stay nice and stable over the next couple
days so daddy could sneak off and catch a few minutes of the Jayhawks'
games without worrying about her too much.

We will make it next time.

Sofie's Dad

Doing Good

It was a long night, but our tough little girl made it through with no problem. I think she was much more comfortable than her parents. The doctors just got finished rounding on her and are very pleased with how she responded to the weaning last night. Her blood pressures and oxygen levels are great and she is doing it without any meds for help and her vent is only at 50%. Her lung is expanded nicely and she isn't showing any signs of the earlier air leak. Dr. H said if there is such a thing as a perfect ECMO run she did it.

So the plan is to clamp her off the ECMO in a little while and see how she responds to doing all the work on her own for a couple hours. If that goes well she will have the surgery to remove the canulas from her neck with the thought being to do her repair surgery on Friday.

The doctors say that since she has done so well without much additional help that we still have a lot of tools in the bag if she should need it. But let's just pray we don't need them. These next few days will be a little rocky, but when we know more Jennifer or myself will try to update.

Sofia's Dad

A Long Night

We are underway with the weaning. She got off to a bit of a rocky start. Before they even started the weaning process, her night nurses were assessing her and noticed a leak around her chest tube sight and she had been laying in about 22cc of serous drainage from it. They cleaned her up and repositioned her towards her right side, which she's really never been on. Her pressures went down pretty quickly from 70 to 45 and her sats dropped to about 88% for a while. After about 30 minutes, they moved her more to her back and turned up her vent settings a little. Her sats are low 90's and her pressure is 45-50. They are turning her ECMO flow down every hour and drawing ABG's every 2 hours on the evens, so the next one will be at 2:00 am. We are in for a long night I think. I'm working on photo slideshows to try to keep from looking at the monitor every 2 seconds. It's not working!!

In trying to keep myself distracted, I put together a slideshow of the day Sofia was born. I also wanted to thank everyone at OPR for the wonderful care that Sofia and I received while we were there. Thank you to Dr. E for a great delivery, to Dr. H and the rest of the NICU team for getting our sweet little girl off to a good start, and to Julia and Melissa for taking extra special care of me. All things considered, we had a wonderful experience, and I couldn't have asked for an easier delivery.

As you all know, we didn't find out whether Sofia was a boy or girl while I was pregnant. We wanted to be surprised at her delivery, so through all the ultrasounds and the amniocentesis, we made it to our induction date without finding out her gender. Early in the day, some of the NICU nurses came in to set up a few things for delivery. They had been told the baby's gender and had prepared some gender specific things for the baby in the NICU, but they had also been told that we didn't know and not to tell us. When they came in to set up, one of the nurses accidentally looked over and said to us, "so, you're having a boy?!" We thought the surprise had been ruined. We called our family in and told them all, "it's a boy!" We also told them his name would be Liam, and wrote Happy Birthday Liam on the board in the room. You can imagine our surprise when Liam came out - a girl!! Turns out we were even more surprised to meet Sofia! The nurse felt so bad for spilling the beans, but it turned out she was mistaken and it made for a good story that we'll laugh about for a long time!

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Weaning Off ECMO Tonight!!

Even though we hit a small snag this afternoon, the plan is still to try to wean Sofia off ECMO tonight. This afternoon she had an echocardiogram to see how her PPHN is doing. We expected it to be better than it was before ECMO, showing signs that it was beginning to resolve a little bit. Her last echo pressure before ECMO was 55, and today it was 78, so quite a bit higher. That was discouraging, but the plan is still to attempt to wean her off. Some CDH babies continue to have PPHN for weeks or months, even after their repair surgery. Some even go home with pressures around 60 and take medication for a while, so we'll forge ahead and hope she shows us her tremendous strength again in the coming days.

The plan is to begin weaning her ECMO settings at 9:00 pm tonight. The process will take several hours. They hope to be down on a very low flow of ECMO by 6:00 tomorrow morning and then leave her on that setting for a couple of hours to see how well she tolerates it. If she does okay with that, they will clamp her ECMO cannulas for 3-4 hours. This is called "trialing off ECMO", and it's a preview of how she'll do when she is taken completely off ECMO. If she does well with the trial, they are scheduled to remove the ECMO cannulas sometime between 12:00 and 2:00 pm tomorrow. From that point, they want to give her 36 hours to stabilize herself before proceeding with the repair surgery. If all goes well, her surgery will be around noon on Friday. So that is the plan as of now. These next few days may be pretty rough for Sofia, so please pray extra hard for her !!

If she doesn't tolerate the wean tonight, they will turn her ECMO settings back up and let her rest a couple more days before trying to wean again. There is also a good possibility that because of her PPHN, she may need to go back on the high frequency ventilator tonight and receive INO therapy (inhaled nitric oxide to dilate the blood vessels in the lungs) again to help with better oxygen exchange. Dr. H is also starting her on a new blood pressure medication tonight called milrinone to help with the PPHN. Dr. H is a great doctor and she'll here will Sofia all night tonight during the weaning process. We are so thankful for that!!

Kenny and I are staying here at the hospital again tonight and probably for the next several nights, until Sofie gets through this part of her recovery. We tried to power nap this afternoon for the all-nighter tonight. I'm sure we'll have no problem staying awake!

Sofia's Mommy

Day 5 of ECMO - Morning Update

Sofia had a restful night last night. She is continuing to do well and we are so thankful for that. She did very well on her oxygen challenge test this morning, with her oxygen level reaching 314, up from 246 yesterday. Yeah for Super Sofie!! We are still waiting for her to have her echocardiogram sometime this morning. That will give us a better idea as to how her PPHN is resolving. She only needed 1 unit of platelets last night and no blood, but it's been about 24 hours since her last transfusion, so she'll probably need some blood sometime this morning or this afternoon. She looks great, so pink and so cute!!! I'll update again after the doctors make rounds and let everyone know when she may starting weaning off ECMO today. Go Sofie!!!!!

Sofia's Mommy

Sofia's Big Week

This will be a big week for Sofia. The team made rounds late in the afternoon today. They say she is doing very well on ECMO and they are confident she'll be able to wean off for surgery. There are risks and benefits for doing the surgery on ECMO and off ECMO, but the least risky seems to be to do it off ECMO as long as she is stable enough to handle it. Once they take her off, they cannot put her back on, so she'll be on her own from that point. ECMO is a one-time thing for the most part. So the risk with taking her off is that if she becomes unstable again after the surgery, they can't put her back on again. There is a chance that if that were to happen, they could attempt VV ECMO, which is a little different and not quite as beneficial as it doesn't allow the heart to rest. She is currently on the most common type of ECMO, which is VA ECMO. VA means it involves both an artery and a vein. VV ECMO only involves the vein. But that is a last resort, and they don't think she'll need it. If they did the surgery on ECMO, she'd have the "safety net" of it still being there for her afterward. However, the risks of bleeding would be enormous and outweigh that "safety net" benefit.

The main factor in how she will tolerate coming off ECMO is her lung function. If her PPHN is resolving, there is little risk that she would rebound to the point she was at before going on ECMO. They will do an echocardiogram in the morning to check her progress on this.

If all looks well in the morning, the plan is to start weaning her off ECMO sometime tomorrow afternoon. They'll do this over a period of 12 hours or so and then trial her off the ECMO circuit by clamping the tubes for around 2 hours to see how she would tolerate being completely off. If that all goes well, they will remove the ECMO tubes (cannulas) on Wednesday. If she remains stable, her repair surgery could be done on Thursday or Friday of this week.

The doctors say she may be very critical coming off ECMO and then again for the first 24-48 hours after surgery. After that point, everything will hopefully be much smoother for her and she'll be on the road to recovery!!! She has been amazingly strong all along, so we have faith that she'll do really well with all of this. She needs lots of prayers this week to get her through the rough patches that await!

Kenny and I finally went home for the first time on Saturday night since Sofia was born. It was so hard to leave her here!! But we knew these last few days would be her most stable for a while, so we needed to rest while we could. Tonight will be our last night at home for a while and then we'll be living at the hospital for a few days again. It's so nice that there are sleep rooms and a shower for us downstairs in the Ronald McDonald Family Room. We stayed there all last week and it was great knowing she was just a few steps away and we could visit her during the night. Everyone has been so great here to help us with anything we need. We are also really happy that Dr. H will be on all night tomorrow night, during Sofia's weaning process to watch over her. She has been taking care of Sofia since her arrival here at Children's and she is fantastic!! Sofia is receiving excellent care here from all of her doctors, nurses, and RT's. Thank you to all of them!

Sofia's Mommy

THANK YOU!!!!!!!

While we have a chance, Jennifer, myself and especially Sofie would like to thank everybody who has gone out of their way to help us get through this difficult time. We have received so many cards and gifts, many of which come from people who we have never met, that we have just been blown away. It is truly amazing how kind people can be and words cannot express just how grateful we are. Just knowing that there are so many people out there pulling for our little girl is so comforting and gives us even more hope that we are going to make it through this.

To our parents who have kept watch in the sometimes crazy waiting room and to Kristi for all the things you have done to keep Jennifer sane, we appreciate it.

I would also like to thank everyone at my job for everything they have done to help me out personally. I knew before this that I worked at a pretty great place, but this has really solidified it. The gifts you have given and all that you are doing to try and raise awareness have been amazing. But to have people giving up their personal vacation time to me so that I will be able to spend more time focusing on Sofie may be may be the best gift I could have received. I know you are probably expecting me to say this, but you have done too much. If you are trying to make a lifer out of me you may have sealed the deal.

To our friends Amandrew, we will never be able to repay you for all that you have done.

I wish I could mention everyone by name but I don't want to leave anyone out. Just know that we are keeping a list and when she is up to it Sofie will be hand delivering thank you notes.

Continue to keep us in you prayers and Thank You.

Sofie's Dad

Happy St. Patrick's Day!!

Wearin' My Green!

Sofia's First Smile

Day 4 of ECMO - Morning Update

Sofia had another good night last night. She got another blood transfusion and another platelet transfusion and had to have a small amount of pain medication so she could rest a little better. Her oxygen challenge test this morning was great! Her level was in the mid 200's today, up from 151 yesterday so that's really good. Yeah Sofie!!!

She got to have a few visitors over the weekend, while she is a little more stable. We'd love for more people to be able to come see her, but she is still too critical for much activity and her risk for infection will continue to be an issue for many months to a year. The risk for infection is why we won't be able to take her out in public places or to daycare for a very long time, and she can't be exposed to other children during that time either. But Sofie thanks you all for your wonderful prayers and good wishes!!!! Please continue to pray for Sofie to rest and regain her strength to fight her next fight. They plan to try to wean her off ECMO early this week and that will be a very critical time for her. After that, the next major hurdle will be her repair surgery, possibly at the end of this week or beginning of next week. She is such a strong baby, and your prayers have helped to carry her through some very rough times!

Sofia's Mommy

Day 3 of ECMO

Sofia is still doing well on ECMO. She was stable throughout the night and didn't need any blood or blood products last night or today until around 5:00 pm, when she got 1 unit of platelets. Every morning they do an oxygen challenge test on her to see how well her lungs are resting and how much lung function is returning since she's been on ECMO. They turn her oxygen up to 100% and then check the oxygen level in her blood. It should be above 200 with good function. Yesterday her oxygen level after the challenge was 80. Today it jumped all the way up to 151!! Big improvement!

The nurses also say they really think the Narcan drip (from the study she is pioneering) is really helping her. Her morphine and versed dosages have not needed to be increased since she arrived. Her dosages are 40 of morphine and 30 of versed. Some babies that are on narcotics for a very long time get up to dosages of 300 of morphine and 200 of versed. So she is on a very low level and still scoring 0-1 on her pain levels. She is a little more active and sometimes wakes up, but she doesn't seem to be in any pain and she tolerates the increased activity very well with no change in vital signs. They are hopeful when she finally comes off of the narcotics, her withdrawal symptoms may not be as severe since she had the Narcan drip. We hope so too!!

Her sodium levels decreased after they changed her TPN yesterday and she started having lots more wet diapers, which is great. She is a lot less swollen today and her urine output is very good.

Yesterday was the first time she woke up and opened her eyes since right after she was born. I'm so glad we were there at her side to see it! We took a few pictures too (for a child that sure doesn't do much, we have 500 pictures of her already). She also was sporting her CDH Awareness Bow yesterday, courtesy of Grandma Linda.

Mommy putting my first bow in

My first hairdo

Littlest KU fan - Go Hawks!!!

Here's a quick picture from today. I'm working on putting together some more slideshows. Hope to have them up tomorrow.

The 5 Day Plan

The team finished making rounds around noon. Overall they are very pleased with how Sofia is doing. The plan is to continue to rest her on ECMO for a few more days and then possibly try to start weaning her off ECMO Tuesday or Wednesday of next week. If she is able to wean off ECMO, they would probably do her repair surgery 2-3 says later. If she isn't able to wean off, they'll wait 2-3 days and try to wean her off again. They will usually try to wean off 2-3 times before they would consider doing the surgery on ECMO. However, they are very optimistic that she'll be able to come off before her surgery, since she did pretty well for so many days before going on. They said she never had any really bad episodes and her PPHN never got really bad. Her pressures only got up to mid 50's at their worst and they say 65 is pretty bad and 45 would have been really really good.


On Tuesday or Wednesday of this week, they put a catheter in because she hadn't had a wet diaper for almost 8 hours. Once they put it in, she emptied tons of urine, so it was just the morphine she's on that was making her retain urine. They took her catheter out yesterday to see if she would go again on her own. The last couple of days her urine output has decreased a little, but is still adequate. They think the reason for this is she might have too much sodium in her TPN, so they are reducing that to see if she'll have more wet diapers to get rid of some of her fluid. This may help some with her swelling. They also check often to see how thin her blood is, since she has to be on a lot of heparin with the ECMO machine to keep the blood from clotting while it's running through the circuit and back to her. Her levels were all good last night and she didn't need any blood products at all last night.


This morning while we were waiting for the team to make rounds, her nurse helped us get footprints and handprints for her baby book. See how cute!!!

Day 2 of ECMO - Morning Update

Sofia did well last night, she has remained stable, so we are thankful for that. We are waiting for the team to make rounds this morning and see what the plan is for today. Hopefully we are in for an uneventful weekend, so Sofie can rest a little. They may start to wean her off the ECMO machine early next week if she continues to do well. We're hoping she'll come off strong and ready for surgery. I'm hoping to post some more pictures later today. She was the littlest KU fan last night with her KU bear next to her during the game! Sofie says, "GO HAWKS!!!!"

Sofia's Mommy

Day 1 of ECMO

Sorry for the delay in updates! We had a rocky day yesterday and didn't have an opportunity to post anything. Also, I'm sorry we haven't been returning calls or emails. We have been at Sofia's side and haven't been home since she was born. We can't use our cell phones in the NICU and haven't had computer access until today, so our only contact with the "outside world" has been our wonderful parents who have been staying at our house the few hours that they aren't here with us and taking care of our 2 little furry babies, Gracie & Lola. Thank you so much to them for bringing us clean clothes and anything else we ask for from the house!! We couldn't make it without you.

We started out yesterday on such a high note. We thought we'd be through with the repair surgery by now and on the road to recovery. They decided to move her to the OR suite which is attached to her room at 1:00 pm yesterday to see how she tolerated it. The move took 30 minutes and 9 people. I think Kenny and I held our breath the entire time. It was very stressful to watch!! She tolerated the move very well and continued to be stable. So around 3:00, they scheduled her surgery to be today starting between 10:00am-12:00pm.

That was the plan until about 5:30, when her O2 sats suddenly dropped to the 80's. Her heartrate started climbing and she began to be more and more unstable. At 6:30 her sats were hanging at 67% and the doctors started discussing ECMO with us. She had quite a team of people assembled to take care of her. Her doctors and nurses have been fantastic here! They are taking such good care of her, and we are always reassured that she's in good hands. Finally at 10:00 pm, the decision was made to place her on ECMO. In the hour before they made the decision to do ECMO, she was trying so hard to fight. She raised her sats up to 77% very slowly, with her Daddy cheering her on. This wasn't the scenario we were hoping for, but it was clear that she was getting tired and really struggling. Being on ECMO will give her a chance to rest a little and regain her strength so she'll be stronger for surgery.

They started her ECMO procedure to place the cannulas around 10:30 pm and finished around midnight. Then they slowly changed all of her settings over to accomodate her being on ECMO. They took her off the high frequency oscillator vent and placed her on a conventional vent. They will not need to oxygenate her with the vent as much since she will be oxygenated by the ECMO circuit. This will allow her lungs and heart to rest.

We finally got back in to see her around 1:00 am. She tolerated the procedure very well and immediately brought her O2 sats and blood gases back up to a normal range. She is so strong!!

Today was a much more stable and restful day for everyone. We can see that she is resting easier and not struggling as much, so that's good. She also doesn't have to be quite as sedated now, so once in a while she'll move her toes and feet and move her little hands. She is pretty swollen from all the fluids and blood products she has gotten so she can't open her eyes because her eyelids are swollen. Since the ECMO circuit keeps her stable, she isn't stressed as much by lights or noise or being touched, so we have been able to touch her a little bit today and talk to her. She even got her first bath early this morning! Her hair is strawberry blond and it's wild!!

I'm sure we'll sleep better tonight too, and we may even go home a little tomorrow if we can tear ourselves away from our sweet baby!

Prayers are Working!

Sofia had a good night overall last night and seemed to get better as the night progressed. She only had one spell where she needed a fluid bolus during the night, where she needed 5 boluses during the day yesterday. When we visited her at 4:15am she was moving her arms and feet quite a bit. However she was tolerating it really well and her vitals remained very good. Her b/p has resettled in the mid to upper 40's and her hr and O2 sats remain very good. Her blood gases (ABG's) were better during the night and continuing to improve this morning. They were able to wean her back down a bit on the vent to 33.

This morning during rounds the team was very encouraged by how much she had improved and feel today or tomorrow may be a good time to do her surgery while she remains in this stable window. We are waiting for the surgeons to come by and see her now and there is talk that they may actually do it today. The goal is to get it done while she is as stable as possible to avoid having to go on ECMO before the surgery, because doing the surgery on ECMO is much more risky. There is still a chance even if she did have her surgery today that she might need ECMO afterward.

After her 9:00am ABG her levels were so good they weaned her vent again down to 32. Yeah Sofie! You can do it! Her 11:00 ABG was also rock solid so they did another echo to check her pulmonary pressures more accurately. This is kind of the final criteria that needs to be good for surgery to happen. Will update again when we hear anything about surgery. Please continue to pray for Sofie to stay strong and stable!!

Sofia's Mommy

A Day of Highs and Lows

We started off the day with Dr. H (neonatologist) removing that first chest tube because it was no longer functioning and didn't need to be there. She was still stable with no further problems. Her team made morning rounds around 1030. It was the first time I had been there to hear what they all had to say and ask questions. Sofie seemed to do really well through the night after the 2nd chest tube was placed, and the team was pleased with her progress. Her labs from the night before were all reassuring and her vitals still stable this morning. Her primary problem is pulmonary hypertension (PPHN) and it seems as though her PPHN is mild to moderate with the mean pressure hanging in the low to mid 40's. That seems to be where she keeps her O2 sats the best (95-100%) and her heart rate steady in the 120's. Dr. S (neonatologist) told us a couple of the surgeons would be by to see her soon and they are saying possible repair surgery in 1-2 days. We were very encouraged to hear this! However around noon she had her first of several setbacks. She spontaneously dropped her blood pressure to the 30's and her heartrate went up. During the morning she had slowly been rising her b/p to the mid 50's in order to keep her sats and hr stable. So this was a pretty significant drop. They were concerned maybe her chest tube wasn't working properly so they took another chest xray. It showed no changes which was good. They gave her a fluid bolus to help her recover which worked quickly. They drew an ABG (arterial blood gas) which was excellent, so they actually decided to wean her vent settings even more from 33 to 31. This was her biggest jump down, so we were very excited. Not long after that, Dr. S told us her repair surgery was scheduled for 1pm tomorrow (Thursday). We were thrilled but also very nervous.

Around midafternoon, she had another spell like she did in the morning. They gave another NS fluid bolus and also gave 3 of hydrocortisone to try and help her blood pressure, which has settled in the 50's, making her PPHN a little worse overall. Through the afternoon her b/p's were labile and then around 4:45 pm she dropped her sats to the upper 80's and had another spell. Her ABG was not as good and her PPHN was worsening. So she probably won't be having her surgery Thursday, which is okay with us because we want her to be more stable when she goes in. After that last spell, they increased her dopamine and dobutamine and jumped way up on her vent settings from 31 to 36. Since she doesn't have much further to go on the vent or with her blood pressure medicines and her PPHN is getting worse, they are now considering putting her on ECMO. So after starting the day at the highest of good news, we were ending with some of the worst news.

We went down to rest in our sleep room and pray that Sofie stabilizes and doesn't need ECMO. It's now around 3:15am and Kenny just went to check on her and said since her last spell around 5:00 pm she has been pretty stable with sats 95-96%, b/p steady in the 50's and hr steady in the 120's. No further spells, her ABG's have been good, and her vent settings were weaned again down to 34 from 36. Her 1:00am chest xray showed her pneumothorax was slightly worse and her urine output is down a little. They are okay with her urine being less because she had been putting out tons, which means her kidneys are being perfused and working well. So that is where we stand heading into Thursday. I pray that we can get back on the right track and avoid ECMO and that her PPHN improves. Please pray extra hard for Sofie today, she really needs it!

Super Sofie!!!!

It was a busy day yesterday. We are sorry about slacking on the update. I tend to ramble a bit so forgive me if this isn't as good as what you are used to getting from Jennifer.

I want to start by thanking everybody for their thoughts and prayers. Whatever you are doing keep it up because our little girl is as good as we can hope for at this point. She is such a strong little girl.

Mom got released late yesterday morning from the hospital so she was able to spend a lot of time with Sofie yesterday. She probably overdid it because she was in a lot of pain last night. Thankfully they have Ronald McDonald Family Rooms here at the hospital so she was able to lay down last night and get some much needed rest.

Well I better get to the star of the show. While I don't think they could find room for another wire or tube on her little body and she has enough drugs going in her to make everybody on Celebrity Rehab very jealous, she is doing really good. The main problem right now is there is air leaking from her right lung that they are trying to get under control. They put a chest tube in not too long after she got here to help relieve the pressure. After several rounds of Xrays throughout the day they thought it wasn't in the best location to be getting everything so they put in another tube last night and her blood pressure numbers are the best they have been since we got here. The doctors have even said that if continues to progress at this rate that they're optimistic that we will be able to make to surgery without having to go on ECMO. Which may be the best news yet.

I think I should start leaving her alone more often because everytime I went in to see her last night they were lowering her settings on the ventilator. The Doctors told us we are on the low end of the range of settings, but it's still good to see everytime they go even lower.

The surgens were in to check on her this morning, which is good becuase the doctors told me when we got here we wouldn't see them too much untill her vitals got into a range that is safer for surgery. They told me we aren't quite there but we are getting closer.

Some of her doctors have begun a study that is trying to help babies that have to take a lot a pain killers. With babies that are on pain killers for a long period of time they begin to develop a tolerance which means the drugs levels have to continue to increase. This also means they have a harder time with withdrawls when they start to come off the drugs. So her doctors want to try a drug that has been used on adults and has been effective but hasn't been studied on babies yet to try and limit the the need to increase her pain medication levels. Our little girl is the study's first participant. So the doctors say if this works she will be legendary to a lot of babies in the future.

Well while it seems like I have been writing forever I sure I am leaving out so much. I need to go down and get Mom so I will sign off for now. Keep those prayers coming. More updates later. Hopfully from Jennifer who is better at this stuff.

Sofie's Dad.

Sent from my iPhone

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Pictures of Sofia

Sofia is doing well so far! I'm pretty exhausted and not thinking very clearly so this post may not make much sense. She was transferred to Children's Mercy around 4:00 and did well during the transfer. After being settled in at Children's, they placed a chest tube and central line. She is pretty sedated now, but holding steady with oxygen saturation 90's - 100% (doesn't get any better than that!) She's on the oscillating vent and getting inhaled nitric oxide, which helps with pulmonary hypertension. Kenny is staying at her bedside at Children's and I should be able to be discharged tomorrow so I can join them. Here is a quick slideshow with some of Sofia's first pictures!

Photo and video editing at www.OneTrueMedia.com

It's a Girl!!

It is an honor, as my first duty as a Grandpa, to announce that Sofia Rose Miller arrived at 11:43 a.m. this morning. She was 19 inches and weighed 6 lbs. 4 1/2 oz.

Jennifer is doing well, as is Kenny. The NICU is preparing Sofia for transport to Children's Mercy Hospital in Kansas City, MO. Early reports of Sofia's condition sound positive. Jennifer will be providing updates regarding Sofia's progress in the days to come.

On behalf of Kenny's parents (Grandma Cheryl and Grandpa Kenny) and Jennifer's parents (Grandma Linda and Grandpa Steve), we want to thank all you that have helped Jennifer and Kenny in so many ways. A special thank you for all of the prayers for Sofia. Please continue to pray for miracles in the days and weeks to come.

The Countdown has Begun

Just a few hours left to go until we head in to the hospital - 7 hours to be exact! Baby Miller will be here by this time tomorrow and will finally have a name other than Baby Miller!!! We are excited to meet this little one and see what the next phase of this journey brings. We are so blessed to have a great team of people working tomorrow to take great care of our baby and lots of friends and family to support us. Thank you to everyone for your thoughts and prayers, phone calls, emails, and cards. We really appreciate everything. We'll post an update just as soon as we can with all the big news and hopefully some baby pictures!

KS Proclaims the Entire Month of March CDH Awareness Month!

Another victory in the CDH Awareness campaign came this weekend. Initially Kansas denied the proclamation for CDH Awareness Day, because the Governor's office determined that it was not of "local or regional interest." After 2 letters to Governor Sebelius, my parents took another route and contacted Senator Schmidt. The work paid off and Governor Sebelius signed the proclamation declaring March CDH Awareness Month! See the official proclamation below!