Thanks to my new weekend night shift hours at work, this will be the first year in five years that I don't either have to work the day of, before, or after the holiday. Now I only work holidays that fall on the weekend. Nice, huh?! Had I stayed in my full-time day position, this would have been my year to work Christmas, so I would have missed out on Sofie's first Christmas.
I work Sunday night, so I'll come home Monday and try to get some sleep before we head out Monday afternoon sometime. Sofie and I will get to stay until Friday, and then we'll have to come home so I can work Friday night. It will be so nice to be able to spend a little more time with our families instead of running around like crazy people trying to fit everything in before we have to rush back home.
Then Saturday will be the big KU vs. MU football game in KC. We always plan a big tailgate for this and will have some friends in town for the big game. It will be a lot of fun, but I'm sure I'll crash after the game, since I will have been awake since Friday before work.
Grandma Linda and Grandpa Steve are coming back with us on Friday so they can watch Sofie while we are at the game on Saturday. I'll have to train them on Sofie's new feeding pump the week we are down visiting, since it's a new addition from the last time they took care of Sofie for us. Or Sofie could just be a rockstar and bottle feed all day for them. ;-)
I'm sure I'll be taking lots of pictures of my little turkey this week to post!
I initially set up our blog as "unlisted" on blogger.com and all search engines. Therefore, the only way it could be found was by word of mouth. Once we reached 60,000 visitors, I figured we weren't so elusive ;-) anymore, so I allowed it to be listed on blogger.com and on search engines.
Here are some of our most interesting stats:
148,787 - The total number of times Sofie's blog has been looked at.
100,182 - The total number of times someone was actually spending time reading Sofie's blog (not just clicking back after following one of our links).
27,808 - The number of individuals that read Sofie's blog once and never returned.
72,374 - The number of times Sofie's blog has been read by someone who continues to check the blog.
454 - Average number of times Sofie's blog is looked at each day.
221 - The number of individuals that continue to read Sofie's blog daily.
We have readers that follow the blog regularly from the following countries in addition to the U.S.: Canada, Australia, New Zealand, Sweden, Belgium, United Kingdom. I've also noticed readers from several other countries on the feedjit live traffic feed.
I'm just amazed at how word has spread about Sofie and how big the CDH community is. We keep meeting new CDH families all the time and from all over the world. I hope we have reached people that had never heard of CDH before, as well as those facing a diagnosis that may have thought they were alone. I know when we got our diagnosis we felt very alone and hopeless. What a blessing it has been for us to find other families going through the same things. I hope we can be that link for many others.
That being said, it's also important to note that her stomach is no longer in her chest and that has a lot to do with her improved feeding as well. It's not all related to the structural differences, though. I'd say the reason her PO feeding went downhill so much was about 80:20 with regard to hunger:reherniation. I know this because once she was allowed to get hungry by being NPO for her CT she ate like a champ (just like she is now), and this was before her repair surgery. Once she was repaired, she was again willing to eat when she was hungry, but once we starting pushing the volume again with NG feeds, she went straight back to her old eating habits of refusing the bottle. Undoubtedly the structural problem of a reherniation comes into play, but I feel like it didn't have as big of an affect on her feeding as the hunger piece of the puzzle does.
The only thing that is strikingly different since her repair surgery is NO THROWING UP. She had gotten to the point that she was throwing up after almost every NG feed (never after a PO feed, but those were almost nonexistent by the time she went to surgery). She starting throwing up with NG feeds way back in mid-July when we first put the NG back in. Then her 1st recurrence was discovered. However, after that recurrence repair in July, she still threw up after NG feeds. This part of the reason I believe she's been reherniated all along since early July. I think she did reherniate again just a day or so after her hernia repair in July. She's just now been fixed properly after all this time, so that's why she's finally stopped throwing up.
In hindsight, I'd have to say the only symptom of recurrence she had was throwing up after NG feeds. And since she did it all summer, even after her repair in July when she was supposed to be fixed, we didn't make the connection. If she truly has been reherniated since the first or second week of July until her surgery last week, it completely coincides with her throwing up. No throwing up before that time frame, no throwing up after. To me it really reinforces my suspicion that she was reherniated all this time. Poor Sofie!
She was always SO happy though. You would've never known anything was wrong with her. Other than throwing up she was completely asymptomatic. No increased respiratory rate, no increased work of breathing, spot checks on sats 97-100%, nothing else out of the ordinary at all. With her first recurrence in July, she also developed a bowel obstruction and did not have a poopie diaper for the 3 days leading up to surgery, so she did become symptomatic for the bowel obstruction.
She had several chest xrays during the summer, including at least 3 that I can think of after her repair in July. Those xrays were incredibly deceiving. They didn't show her stomach or any of the bowel that was up in her chest. Her diaphragm appeared intact, when in fact it had pulled away around the rim cleanly, leaving the edge to appear perfect and smooth on the xray. Xrays don't show the whole picture. She was reherniated for a long time and the xrays didn't show it.
She is such a trooper. Now she's finally fixed and she can move forward much easier. Lots of good things are about to happen for her. I'm very optimistic about our NG weaning plan!!
In other good news, her sniffles are much better today, so I think we've dodged the cold bullet and she just had a benign reaction to her RSV shot.
The circle it's mounted in is painted on the wall. Thanks to our friend, Kate, for the brilliant idea of how to paint a perfect circle! We put a nail in the center of where we wanted the circle, tied a string from the nail to a pencil and traced the circle on the wall. Then we covered the pencil line with painter's tape. Next we replaced the pencil on the string with an exact-o knife and cut the painter's tape to get the circle guide. I painted inside the circle (4 coats) and then carefully removed the painter's tape, leaving behind the perfect circle.
The smaller circles around the framed photo are stick on decals from Target that match the bedding.
I made ceramic impressions of Sofie's footprints and handprints after she came home from the NICU and color matched the paint to her bedding. I ordered the wording on the wall from wordsanywhere.com. You can customize it lots of different ways, and it's priced really well too ($12 total for all my lettering).
The furniture is by Argington, designers with KC roots and KU as an alma mater - go Hawks! - that now live in NY.
When I took the BandAid off to shower on Monday, it was HORRIBLE looking. It was raised and open and oozing and there was a hardened area underneath the size of a nickel. It was really red and warm around it, with the redness extending about an inch and a half or so out around the open part. It was totally disgusting. Knowing it could be MRSA I went to straight to my Dr. for a culture. She started me on Levaquin (an antibiotic that covers MRSA) while we waited for the culture to come back.
Today the culture came back positive for MRSA. MRSA is really contagious, so I've been keeping the site covered and washing my hands like crazy to keep from giving it to Sofie. It's starting to get better today. It's less red and sore and a huge chunk of of it has fallen out. Yeah, gross, I know. It looks like I got shot. I'd post a picture, but it's far too gross, and I'm too embarassed to show it off. Trust me, you're not missing out.
I was worried that I wouldn't be able to go to work with it, so I called my boss and she talked to infection control and as long as it's covered, it's okay for me to work. I've also been on a strong antibiotic for 4 days now. I'm going to scrub around it really good, cover it with 2x2 gauze and then cover that with a big tegaderm, so I won't expose anyone else to it. At least I won't be missing any more work!
With this lovely new development and the fact that my milk supply has been greatly diminishing over the last several weeks, this dairy cow has officially been put out to pasture. As Kenny says, I put up a good fight pumping for over 8 months. I have to say I honestly hated every minute of it too. I don't miss pumping one little bit, but I am feeling a guilty about not providing any more milk for Sofie. I do still have about 3 months worth of breast milk in the deep freeze, so she should be able to get it until she's almost a year old. Exclusive pumping is For. The. Birds. Next time around I will do everything I can to try to get our baby to breastfeed, so I only have to pump while I'm at work.
Sofie's cold-like symptoms are slightly better today, so while I had my lovely Dr. on the phone (you rock, Dr. B.!) I asked her if her symptoms could be a possible side effect of getting the RSV shot yesterday. She said since it was her first shot, it's possible that she's just developing immunity to the antibodies in the shot and that could be causing her symptoms. I REALLY hope that's all it is and not a cold!!! I asked the nurse that gave the shot if I should watch for any side effects, and she said she didn't know of any, but didn't seem too sure of herself. I will be celebrating bigtime if she's just having a mild reaction to the shot and is not really getting a cold. Keeping my fingers crossed!
As far as the NG hindering oral feeding...
It's been our experience that it absolutely does. Sofie has always seemed more "gaggy", more likely to throw up, and less likely to PO feed with the NG in versus when it's out. It's a foreign object in the back of her throat, so it definitely makes it more uncomfortable for her to eat. I always say I imagine it would be like laughing a spaghetti noodle out of your nose 24/7.
Some people say you get used to them after a while. When it's first put down, it's bothersome for a while, but then you get used to it and don't notice it as much. Then if you take it out, it's a little strange again until you get used to nothing being there. If you put it back down again, same thing - it bothers you until you get used to it. I can't speak from personal experience on this (sorry, Sofie, Mommy hasn't taken that one for the team, yet), and last time I asked Sofie she didn't have anything too specific to say about it. But I understand that's the way it is for adults, so they (meaning health care professionals that I've talked with) assume it's the same for babies. However, chronic use of an NG for feeding can affect oromotor skills in a negative way, more so, I would imagine, for someone who is already having difficulty coordinating their swallow.
NG vs. G-Tube...
Chronic use of NG feeding can cause scar tissue to build up in the back of the throat, furthering oral aversion. Since it also passes through the esophageal sphincter, over time it can cause incompetency of the sphincter, making reflux worse. In turn this can cause esophageal inflammation, erosion, or ulceration. NG use also poses a higher risk for aspiration of gastric contents into the lungs, which can lead to aspiration pneumonia. These are a few of the reasons why I think the g-tube is a better option for tube feeding than an NG if tube feeding is going to be long-term.
G-tubes have their drawbacks too. They have to be surgically placed. Surgery to place the g-tube can increase the severity of reflux. They carry a higher risk for infection and leakage of gastric contents if they become dislodged. They can also produce granulation tissue around the site, which needs to be removed. But overall, g-tubes tend to be better for long-term tube feeding. There is no oromotor involvement with a g-tube and no more intervention around the face, which can contribute to oral aversion. They don't carry an increased risk for aspiration or pneumonia. It's relatively unnoticeable underneath clothing and allows for better mobility. (Imagine learning to roll over or crawl with a long spaghetti noodle hanging out of your nose, dragging on the floor.)
If Sofie isn't able to wean from the NG to PO feeds, she will need to have a g-tube placed. Some kids only have the g-tube for a few months. But our docs tend to leave g-tubes in for 2-3 years once they're placed. Their reasoning is they don't want to take them out prematurely and then have to surgically replace them. Knowing that if Sofie gets a g-tube she'll have it for a long time, we want to give her every opportunity to avoid it if possible, especially since she was 100% PO fed before all this reherniation business started this summer. Our thinking is she's been a normal PO fed baby before. We know she can do it again.
If we just take out the NG, will she start eating better?...
I'd like to think if we just take out Sofie's NG and leave it out, she'll feel better and take off with oral feeding. And I think she will - eventually (hence, Phase 3 of Peetie Unplugged). But we have a little work to do first. We need to recondition her oral skills to set her up to succeed.
Her PO feeding has declined so dramatically after having the NG in. She was 100% PO fed right after she came home from the NICU. When we did finally put her NG back in in mid July (she began refusing bottles because she had reherniated), her PO feeding immediately began to plummet. PO feeding continued to decline to the point of being 100% NG fed just prior to her surgery last week. Since her surgery we have been encouraging more PO feeding and not pushing the volume with the NG as much (Phase 2 of Peetie Unplugged). We've found that with the return of hunger from not pushing so much volume, her PO feeding skills are returning.
We have had the NG out for the day a couple of times since the end of the summer for one reason or another (didn't have a replacement, just wanted to see what would happen, etc.). On those days she'd take about 7 ounces from the bottle. Better than nothing, but still not nearly enough, so we'd always put the NG back in. This time when we pull her NG, we'll be leaving it out to give her a chance to improve on her own (Phase 3 of Peetie Unplugged). It won't be an overnight change at all. She still has a long way to go, but we are really optimistic that she can do it!
Hunger mechanism - the main player...
It's not just the physical presence of the NG that causes problems with PO feeding. I think the primary problem with chronic NG use has more to do with the extinction of the hunger mechanism. When an NG is used for a long period of time to deliver feedings, especially on a set schedule, the brain doesn't really need to tell the body to be hungry anymore. On the most basic level, hunger exists at motivation for us to consume the nutrients we need to live. When we bypass this mechanism it slowly disappears. There is no cause and effect relationship anymore with regard to hunger being satisfied by eating. It's basic behavior extinction. When that's coupled with lots of medical intervention around the face and negative experiences with feeding, the problem is greatly compounded.
There's SO MUCH that goes into oral feeding problems. It's a multifaceted issue to say the very least, and my very limited knowledge of it only spans the tip of the iceberg. We're so lucky to have the help of Sherry and Dana and to be able to talk with other CDH parents to see what has worked for them. Every situation is a little different, but we can draw from the similarities and build upon them.
We affectionately call Sofie "Peetie", short for Sweetie Peetie. And I've dubbed the our NG weaning plan "Peetie Unplugged". The plan covers about three months, so we won't see any quick changes. In fact, it may take longer than three months. There's no quick fix when it comes to tube feedings, and we aren't sure this will even work, but it's our last ditch effort before putting in a g-tube.
And as far as g-tubes go, if you have to be tube fed, I think the g-tube is a far better option than the NG. The reason for staying with an NG over getting a g-tube is that we hope the tube feeding is short term. Getting a g-tube means tube feeding will most likely be a long-term thing, usually spanning the first 2-3 years of life, maybe even longer. I've been able to get the opinions of other CDH parents on their experiences with g-tubes and feel like if we can't get Sofie transitioned to oral feeding, the g-tube is definitely the way to go. I'm just not quite ready to throw in the towel yet. If Sofie hadn't had these two recurrences, we would have started this weaning plan long ago. So we want to give it a fair shot, before we take the g-tube route.
Peetie Unplugged has three phases:
Phase 1: Fatten Up (Peetie in Training)
Phase 2: Wean to increase oral feeding skills (A.K.A. The Kinley Melcher Strategy)
Phase 3: Withdraw all NG support (The Big Leap)
We've been in Phase 1 (Peetie in Training), since mid-October. Phase 1 is simply getting as many calories as possible in each day to boost Sofie's weight so she has some to lose and still not dip below the 5th percentile for weight. During the weaning phase and especially when we withdraw all NG support, we are prepared for Sofie to lose a lot of weight, maybe even a pound. That's a big hit for such a tiny person, so we are trying to "pad" her numbers (no pun intended) before we start aggressively weaning. Phase 1 also includes lots of positive associations with food, so when we start to wean, she's not aversive to food. Here's Phase 1 of Peetie Unplugged...
I talked to her about trialing off oxygen and if we could start the process at her next office visit. She said that seemed very reasonable!! Even more good news! Her next appointment isn't for 4 weeks, so as long as she stays healthy and steady with her weight gain, she can trial off oxygen. I'm assuming they'll do a short trial in the office and if it looks good, they'll have us use a sat monitor at home for 48 hours to see how she does. Dr. R. said that even if she does come off oxygen, she'll still have us keep an emergency oxygen tank at our house. That way if she gets sick or has an emergency, we'll have oxygen readily available, which makes me feel reassured. What great news that Sofie may be off oxygen by Christmas!!!
Now for the "Thumbs Down" part. Our discussion about feedings was discouraging. I told them we were working her back up on volume and that she's tolerating 5 ounce feedings again with no throwing up. The last few days she hasn't come close to getting her goal volume, just because she's been gaining weight really well, so we didn't feel like stuffing her like a Thanksgiving Butterball. We're working back up to it slowly. She's getting about 25 ounces per day. I mentioned that she's willing to take the bottle again if we allow her to get hungry, and that stuffing her with 30-32 ounces per day just completely zaps her hunger.
They think that her oral skills are unrelated to hunger, and that 4 hours after a feeding she should be hungry enough to want to eat if hunger is the issue. They also said that for her age she should be taking 6 ounces at each feeding. I feel like that is a ton of volume, since I know plenty of healthy babies that never took more than 3 ounces at a time from the bottle.
Then they asked how we're currently fortifying her milk. The last time we saw our CMH dietitian, she gave me the recipe for fortifying to 28 cal/oz, so that's what we've been doing for the unthickened milk, since she's tolerating 28 cal/oz now. I calculated how to make it with the thickener, so I add a little more when thickening to make it 30 cal/oz and then once the thickener is added, it reconsitutes to almost 27 cal/oz. Everyone in the room was like, "30 cal - oh my - that's a lot - we need to refigure..." even though it's NOT truly 30 cal/oz, once the thickener is added. They said it would be a couple of days until they could get the correct recipes to me. For the life of me, I cannot understand why the thickener displacing the calories continues to be such a difficult issue. When I got home there was a message giving me the "correct" recipes to use, which happened to be exactly what I told them I've been doing all along. Shocker.
So with all the positive reinforcement, I failed to mention our plan to wean the NG. I think we'll just use our best judgement, with the aid of our FirstSteps OT and dietitian, and do what we want to do. We are fine with Sofie hanging at the 5th percentile. I think she's just a small girl genetically, and it's okay if she doesn't want to eat a set amount each day. As long as she eats by mouth and continues to grow and thrive, we will be very happy. She's bigger than I was at her age, and I was perfectly healthy.
So enough of that. Sofie gets to trial off oxygen next month!!!!!!!!!!!!!!!!!!!!!!!1
There should be no doubt that all CDH babies qualify for Synagis due to their chronic lung insufficiency, but unfortunately many insurance companies deny it. The shot is extremely expensive. I've heard it ranges from $900 to $1800 per month, depending on the weight and age of the child. So insurance companies don't hand out reimbursement for it very readily.
I'm so glad my insurance company approved it (our pediatrician started the pile of paperwork for it this summer) and we didn't have to deal with appealing a denial to get it. Many CDH families are facing this headache right now, and I feel fortunate that we aren't. I know of a family who were retroactively denied last year after their child received all the shots and were then socked with a bill for over $10,000. When insurance companies consider the medical histories of CDH babies and the cost of a hospitalization and treatment for an RSV infection, it should be a no-brainer to cover these kids.
I will call Sofie's pediatrician today to see when she can get her first shot. I'm hoping she may possibly be able to get them via a home health nurse, so we don't have to take her into the pediatrician's office so much during cold/flu season. I know you have to have special approval to get them through home health, so that may be a pipe dream, but we'll see.
She has several tiny incisions 3-5mm in length from her surgery. Most of them actually are just little holes. They are small enough that none of them needed sutures. They all just had steri-strips over them, which I removed tonight. One of the incisions in her back had the JP drain in it, so it's a little bigger and it seems to be the one that bothers her the most. We removed the dressing from that site yesterday and it looks okay. It's not the prettiest little incision, but it looks like it is healing well. We are careful to pick her up by supporting her bottom and not under her arms or around her back where her incisions are. I will take some pictures of her incisions soon and post them.
We were prepared for her to lose weight during all this, maybe even half a pound. But she did great and has not only held her weight steady, but gained three ounces since we took her into the hospital last Tuesday. Tonight she weighed 15 lbs, 3 oz!! This actually puts her between the 5th and 10th percentile for weight, so she has gained on her growth curve!
In other great news, she hasn't thrown up once since her surgery! She had gotten to the point where she was 100% NG dependent before surgery and would get so distended after NG feeds, she threw up after almost every feeding. It was horrible watching her do this. We felt so helpless. So it's such a huge relief that she is tolerating feeds really well again. She can even handle 5 ounce feeds again with no problem, even when they are NG feeds. So far we haven't run them faster than 120cc/hr, but we are working on increasing that to 150cc/hr.
With the NG supplementation and the continuous night feeds, her hunger is zapped. She refuses most bottles, but occasionally will get hungry. Tonight before bed she was hungry and took 3 ounces by bottle for me! I was so excited!! She has always been a better eater as the day goes on. She usually refuses the bottle in the morning and may be willing to take more by mouth later in the day. She promised her Grandmas and Grandpas she'd start eating better from the bottle, and I'm pretty sure they'll hold her to it.
Sofie is snuggled in bed now getting her night feeding. Tomorrow morning she has an appointment at the Special Care Clinic with Dr. R. I'm going to bring up her oxygen status, echo results, b/p changes, and the possibility of a trial off oxygen. I think it's a little soon after surgery at this point to expect a trial right now, but hopefully she will consider it in the near future.
Thursday @ noon (1st post-op day) in the PICU
Napping on Grandpa Steve's lap Saturday morning
My Little Blue Wagon Ride out on Saturday morning
So Sofie was getting very little, if any, oxygen all day. NO WONDER her sats were 88-91% on 1/8 L. At that low of flow, I'm sure she wasn't getting any oxygen. Plus she still had the cannulas in her nose, so they probably occluded her nose as well. Once they had it working correctly, they set it on 1/8 L and she's been satting 95-98%.
So we totally could have gone home today. Oh well, I'd rather stay a day extra than leave too early and have to come back again like we did last time. Plus now we know that she sats 88-91% off oxygen, maybe better if she weren't occluded with the cannulas. Very promising, considering she's only post-op day #2! Once she is fully recovered, I know she won't need her oxygen. Now I just have to convince her doctor of that. ;-)
Sofie was on 1/2L oxygen yesterday, last night, and this morning. Her sats were running mid to upper 90's with that. This morning they said to wean back to 1/8 L since that was what she came in on from home and if she could keep her sats 92% and above on 1/8 L she could go home today. She tolerates 1/4 L just fine, but she's so sensitive. If it's sitting between 1/4L and 1/8L, her sats are 92-97%, but when we turn her down to 1/8L she sats between 88-91%. It's such a small difference. Stinker!
Last night we finally got to her room on the floor around 5:00 pm. It took all day to get the order written to transfer her out of the PICU. Then they had to get a room ready for her. The good part of that is that they were full on the post-surgical floor she's usually on. That floor doesn't usually have sick kids, because they are all post-op, but once in a while they do have a sick one. Our new room now is on the Burn Unit and there are only burn patients here, no sick kids, which I'm super happy about. The less exposure to germs, particularly RSV while she's here, the better.
Once we got to the room I gave her a bath and put on her footie pj's. She was still yucky from surgery and still Naked Baby. Once she was clean and in her own pj's, she looked so much better. They weighed her last night and she was so heavy from still being swollen and full o' poo. She weighed 15 lbs when we brought her in on Tuesday. Last night she weighed 15 lbs 8 oz! I wish that were a true weight, but I know it's so not.
Last night I was a little frustrated, because she's been here since Tuesday and hasn't ever gotten any of her usual meds she takes at home. Her abdomen was getting really hard and distended and she hadn't had a poopie diaper since Tuesday afternoon. She's used to getting medicine to help her have regular stools and she hadn't had it since Tuesday morning. Plus she had surgery with manipulation of her bowels and narcotic pain meds, which are constipating. I had requested she get her meds several times following surgery Wednesday evening and all day Thursday. I even gave out a list I had brought with all her home meds and their dosages written down for the docs to write the orders for them. I brought all her home meds with us and asked if I could just give her our own until we got the orders in, but of course that's not "legal", so they said they'd give it once they got orders.
She finally got her first doses of Zantac and Erythromycin last night around 9:00 pm, but before I gave them I checked to make sure they were right. The Zantac was right, so I gave it, but the Erythromycin was way off. She usually gets 16 mg and the dose was for 60 mg. She gets the Erythromycin for intestinal motility. We want her to have a poopie diaper, but not that big of a poopie diaper! So the nurse paged the doc to change the order and also to get an order for a glycerine suppository to help her have a stool so her tummy wouldn't be so hard and distended (and painful, I'm sure.) Two hours later, we still had no new orders. So I just got out our own home meds and medicated her myself. But the Erythromycin takes a few days to take affect, so she was still uncomfortable. She finally went to sleep after getting her Toradol for pain.
We gave her first overnight continuous feeding last night. She got 9 ounces over 6 hours and tolerated it just fine. So she got a total of 17 ounces of milk yesterday. Not bad for post-op day #1.
We were up with her a couple of times through the night. Then she went for a chest xray around 5:00 am. Her docs made rounds around 6:00 am. It was the surgery fellow on service, some residents, etc. Basically a bunch of people we don't really know do rounds in the mornings. Plus I had my glasses off and was totally blind, so I couldn't tell you how many people were even in here! We asked about going home, her chest xray, her echo results (we'd asked several times and couldn't get any info on the results), and for the glycerine suppository. They said to wean the oxygen, as I talked about above, as far as going home. They said they'd write an order for a suppository (there was so still order written). And they'd look at the chest xray and echo results and let us know.
Another doc came in at 11:30 am and took out her JP drain, because it was hardly draining and it was all serous drainage (non-bloody, kinda clear fluid). After that she FINALLY got her glycerine suppository.
Dr. S.P. rounded on her around 12:30 and talked to Kenny (I was napping, thinking I'd be going to work tonight). He said her chest xray looks good, same as yesterday. Her hiatal hernia is still present, which he expected. He asked about her reflux and if she's thrown up at all since surgery. She hasn't seemed to be bothered by reflux so far and no throwing up - yay! At that time we thought we were going home today, so he said we'd see him again for follow up in clinic in about 2 weeks.
Sofie napped from about noon to 2:30 this afternoon. She's back to her old habits with bottle feedings. Her PO feeding tapered off this morning at her 9:30 feeding. She's refused all bottles since. I feel like it's no coincidence that the steep drop in PO feeding happened just after we went back to her old feeding schedule and using the NG to increase her volume with each feed and give a night feeding. There's a very strong inverse relationship with tube feeding and PO feeding. The more we tube feed, the less she PO feeds. We've known this for quite some time, but had to keep it up so she'd be healthy for surgery. Once she's healed, we're planning to withdraw NG support and require her to feel hungry so she can learn to associate hunger with the need to PO feed. We'll be doing this with the assistance of Sofie's OT and Dietitian. She'll probably lose a lot of weight, but we are prepared to take drastic measures try to get her off tube feeding. The only other alternative at this point is to put in a g-tube. If she fails our trial off NG support and doesn't take off with PO feeds, she'll have to get a g-tube.
The good news with feedings today is that she's tolerating 5 ounce NG feedings just fine, which is a big improvement since her surgery. Prior to surgery she would throw up after NG feedings, even 4 ounce feedings given over an hour or more. So this is a definite improvement as far as tolerating an increased volume.
About 2 hours after Sofie got her suppository, she had a poopie diaper - yay! Her tummy feels much softer now and I'm sure she feels better. Her Erythromycin dose still hasn't been reordered correctly, so I gave her ours again at 2:30 this afternoon. She did get her correct Prilosec dose this morning (first one since Tuesday morning) and again at 5:00 pm. I also gave her our own multivitamin at 2:30. Her normal home med schedule is:
9:00 am - 16mg Erythromycin and 6.2 mg Prilosec
12:00 pm - 1 ml Multivitamin with Iron
3:00 pm - 6.2 mg Prilosec
9:00 pm - 16 mg Erythromycin and 30 mg Zantac
We still couldn't get any answers on the echo results. It seemed like no one really knew anything about it, so our nurse finally printed off the report from yesterday and the report from her echo in May and let me read them. Her PFO is the same as it was at 2.5mm, which is very small. Her right ventricular pressure in May was 35-40 mm Hg. If I'm calculating right and I'm not nuts (both of which are possible), that would mean her estimated mean pulmonary arterial pressure was between 23 and 26. Pulmonary hypertension is present when mean pulmonary artery pressure exceeds 25 mm Hg at rest or 30 mm Hg with exercise. So it was actually pretty good back in May. Yesterday's echo was inconclusive for estimating her right ventricular pressure, so we can't calculate her estimated pulmonary pressure. But at least I have copies of both reports now, so I can take them to Dr. R. and possibly request a repeat echo to determine if her pulmonary hypertension is completely resolved if need be. I strongly think it has resolved and that's why her b/p has normalized over the last couple of months or so. If it has resolved that's a good indication that she's ready for a trial off oxygen.
Sofie sat up and played for a quite a while this afternoon. We got several smiles and some babbling today, so she's feeling much better.
She got her last dose of IV Toradol around 4:00 or so. Her left (the one with the IV) hand and arm were looking a little puffy yesterday, but she was so puffy everywhere that it was hard to tell if it was just from the tape on that arm or from her IV possibly starting to infiltrate. But the skin around her IV site wasn't cool or red and she didn't act like it was tender. Her IV fluids were stopped yesterday afternoon. But last night and today she would cry when she got her IV Toradol. I thought it just bothered her to feel the coolness of the flush and Toradol burns when it goes in to. But throughout today her overall swelling went down quite a bit, but her left hand/arm were still puffier, so we just had the nurse take out her IV a little while ago and discontinue her Toradol. If she needs something for pain she can take an oral pain med instead.
Her weight tonight was 15 lbs 2 oz, a little closer to realistic. I'm sure her weight tomorrow will be more accurate and she will have lost a few ounces throughout her stay, but hopefully she'll bounce back really quickly. Thanks for all your prayers - they have obviously helped so much! We should be going home tomorrow for sure. We are anxious to get back home!
P.S. Yes, Kris, we are starting her early on becoming a true fan. For the record, it was my idea, not Kenny's!
Around 5:00 am she had a chest xray and they said it looks good. We saw the chest xray from yesterday afternoon last night and her left lung is nice and inflated well. Her left diaphragm is much lower, now only a half a rib to 1 rib higher on the left than the right, as opposed to the way it was before. It was about 2 ribs or so higher before, I think. The tip of her NG tube is still a little high, so it looks like she still has her hiatal hernia. Bummer. But Dr. O. (one of the surgeons) went in during her surgery yesterday and said both he and Dr. S.P. could see all around her diaphragm and the rest of it was intact, so we feel good that nothing was missed and it truly just is her hiatal hernia still there.
She took 1/2 oz bottle at 10:30 pm and another 1/2 oz at 3:10 am. She took 1 oz at 9:30 this morning and I gave her an additional 3 oz through the NG with it. That was her first "full" feeding since she's been here and so far she's keeping it all down just fine and doesn't seem uncomfortable. If she's taking in enough fluids with feedings today and tolerating it well, they will stop her IV fluids and possibly remove the IV tonight.
She got her scheduled Toradol for pain around 5:00 or 6:00 this morning and hasn't needed anything since for pain. She seems better today. She sat up while I was holding her a while ago and didn't cry or seem to be in pain. She's still kind of sleepy and pouty, but she's been awake more and watching some cartoons. She's taking her morning nap right now, and the doctors just gave orders for her to go out to the floor. No talk of going home today, but we are still hoping maybe tomorrow. If she can tolerate 4oz feedings I think that will get us out of here faster.
She had her echo around 9:00 or so this morning and her PFO is still there but very very small and won't affect her. It appears to be closing and can take up to a year or even 2 years to completely close. It may not ever completely close, but it shouldn't cause any problems. Many adults have PFO's and don't even know it. So that was good news. I haven't heard the rest of the echo results as far as her pulmonary pressure yet. I hope it looks good, so Dr. Robertsen will consider letting her get rid of the oxygen. That would lighten our load so much!!
She sits at the table with us for 2-3 meals per day and then has "snacks" with her other NG feedings. These exposures to food focus primarily on solids and we are just putting things out for her to explore and play and develop positive associations with food. We incorporate some feeding OT into these times as well. When I'm making dinner in the evening, I bring her to the kitchen to "help" so she can watch me cooking and preparing food and try some samples. I also put different foods on her tray to explore. Here's the typical Helping Mommy Make Dinner Snacktime...
I fed her a bottle around 6:30 pm and she took the half ounce I put in the bottle in about 5 quick gulps and was mad that it was gone. I wanted to start her off slow just in case it made her feel sick to eat. But since she did so well with her first feeding post-op, we've offered her whatever she wants to take since then. She took an ounce and half at 7:45 and then another half ounce at 10:30. She got some morphine then too and went to sleep, so she kind of peetered out on her feeding, but that's okay. She is totally ad lib with feedings, so we are just doing whatever we want and letting them know so they can document it.
She has an NG tube in, but we haven't given her a tube feeding since she's been in the hospital. We are just kind of letting her do her own thing. What a novel concept - feed the baby when she's hungry! Brilliant! It's a real breath of fresh air to not have such a rigorous feeding schedule with such pressure for caloric intake. We have been planning a drastic change in her feeding regimen for the last month or so, but wanted to keep her healthy and strong to get her through surgery first. We wanted her fat and sassy for surgery. Once she has healed appropriately, we'll be taking a much different approach with feeding.
But back to today... She improved SO MUCH just from 3:00 pm to 6:00 pm. She started out extremely sleepy and irritable, desating to the 70's-80's when she was upset. By 6:00 she had her eyes open, watching a little tv without too much of a fuss, and her vitals have been stable. Her oxygen is at 3/8 L by nasal cannula and she's keeping her sats 96-100%. Lots of sad faces and pouty lips though. Hopefully tomorrow she'll be feeling much better.
Kathy, the surgery nurse practitioner we know, is following Sofie tonight and tomorrow. She said that she'll just be staying in the PICU for tonight and then tomorrow will either go out to the floor or even possibly GO HOME! Can you believe it?! I'm not getting my hopes up, because I think tomorrow is a lofty goal, but I'm very optimistic that we'll be going home on Friday.
Sofie had an arterial line put in her right foot just before surgery started to monitor her blood pressure continuously. It's a pretty routine thing for her situation and the kind of surgery she had. Manipulation in the chest can cause quick changes in blood pressure, so they like to be able to keep close track of it. She's had an art line for all of her other surgeries. The good news is that she's very stable. She has some AM labs due and then she'll be losing the art line. That will leave her with just the regular peripheral IV in her left hand for hydration and pain meds if needed.
She's getting scheduled Toradol (an IV NSAID sort of like ibuprofen - works great for post-op pain) and Morphine PRN (as needed). She seems to be very comfortable now. She's resting in her bed. We held her from the time she got to the PICU until about 11:30 pm, so she got lots of good snuggles. Last time I checked my Davis Drug Guide you couldn't O.D. on snuggles!
She's had lots of wet diapers, so she's mobilizing her excess fluid well and her kidneys are working great. Just being in surgery for a couple of hours getting IV fluids, I was amazed at how puffy she had gotten. It just doesn't take much fluid at all with babies to become overloaded. The good news is that it's not settling in her lungs, but she's peeing it off instead. Once she feels good enough to move around that will help mobilize that fluid better too.
Her JP drain in her old chest tube site has been draining some, but not excessively. I'm guessing it will come out early tomorrow.
Her PICU doc came by a couple of times to check on her. He seems very nice. He discussed some of her history with me to get a better sense for all she's been through, since her chart is, um, fairly thick at this point. We got to talking about her blood pressure and her last echo and I said her last echo was done in May just before discharge from the NICU. It showed some residual pulmonary hypertension (I can't remember what her ventricular pressures were). At that time her resting systemic blood pressure ran high, like 110's/70's - 120's/80's, which is pretty high for a 2 1/2 month old. Some CDH babies tend to run high b/p's. I figured it was due to the residual pulmonary hypertension. Since the systemic b/p needs to be higher than the b/p in the lungs for the blood to be able to flow into the lungs and pick up oxygen, it makes sense that a higher pulmonary pressure would, in turn, increase the systemic b/p in response to compensate.
Her b/p's continued to run high post-discharge when she was checked in clinic until about August when they started declining to where they are now, which is 80's/50's generally, sometimes lower. I wondered at the time if her pulmonary hypertension had resolved and that's why her b/p had lowered. I asked her PICU doc about it and he said that could be the case. He asked about her home oxygen regimen and I told him she's on 1/8 L continuously, but we feel it's unnecessary at this point. I told him I thought Dr. R. was keeping her on, for a few reasons:
1) She's on the low end of the growth curve and Dr. R. doesn't want her burning extra calories breathing harder.
2) It's cold/flu season - maybe not the best time to come off, and Dr. R. tends to be very conservative with discontinuing oxygen.
3) She's had that residual atelectasis in her left lung pretty much since June or July.
I also told him we feel like once she's healed from this surgery, she really doesn't need it anymore. He said he'd like to repeat her echo tomorrow to see if her pulmonary hypertension has resolved or improved and, if so, that could be good evidence for Dr. R. to consider discontinuing her oxygen. That would be great!
I also told him that her last echo showed a PFO or Patent Foramen Ovale. This is a normal hole in the heart that all babies have in utero, but normally closes after birth when babies use their lungs to breathe. In utero the pressure in the lungs is higher than the rest of the body, because babies don't use their lungs to breathe until they're born. When they are born, they adjust to extrauterine life by taking that first breathe and the dynamics in the body change fairly rapidly so the pressure in the lungs is less than the pressure in the body. This allows blood flow through the lungs to pick up oxygen. This change in pressure allows the Foramen Ovale to close. Since CDH babies frequently have pulmonary hypertension this keeps the Foramen Ovale open, which is why it's called a Patent Foramen Ovale. It's not considered a normal finding, but if the baby is asymptomatic (i.e. not having any problems from it), it's okay. I'm also interested to see if her pulmonary hypertension has resolved if her PFO has closed. Guess we will see tomorrow...
Well there's probably more to add, but this is getting to be the longest post in history. Back to working on fun videos..
I took some videos of Sofie playing with her shape sorter. The video is a little on the long side. I think it's about a minute and a half, but it gets a lot better about halfway through if you stick it out.
The first half she was distracted, because Moose and Zee came on TV and they are her absolute FAVORITE. She actually loves Zee the best, but Zee doesn't talk so it's Moose's voice that gets her attention. She heard Moose and Zee come on tv the other night when we were at the dinner table (you can't see the tv from our dining room), and she was looking all around trying to find them. She got so upset and started crying because she could hear Moose, but she couldn't see him anywhere. It was so cute!
The good news was that her bowel was still completely contained in the peritoneal sac, so he could easily push it all down and be sure that all the bowel was contained within the sac. The peritoneal sac is the normal "container" for abdominal organs to keep them where they are supposed to be.
He pushed the stomach down and the hole came together perfectly with sutures. Just to add extra reinforcement, he also placed a Surgisis (SIS) patch over it and sutured that down and then put a layer of glue in. We want this one to stick! The SIS patch is another bioabsorbable type of patch, similar to Alloderm.
He didn't need to open her abdomen at all or need a thoracotomy. He just used 5 "poke holes" to do the surgery. Two are around her left side, one is through an old chest tube scar on the right side, one is near the right scapula on her back and I think the 5th was also somewhere on her back. The incisions were all 3-5mm long. He put a drain in the 3mm hole where her old chest tube scar was when he was done, just in case she had some excess fluid build up, but he doesn't anticipate she will need it more than a day or two.
She should come off the vent during her recovery in PACU - yay! She may still go to the PICU for observation for a while. He said he watched her lung reexpand on the vent completely before closing to make sure it's not collapsed like it was after her last repair.
He also did a nerve block underneath her ribs to help with pain over the next several hours. She'll also have pain meds ordered if she needs them. He said she should recover much faster than she did with her last repair, and he is hopeful that she'll feel much better than she has in quite a while.
He did an amazing job of explaining literally everything he did. It was quite complex and amazing what all goes into the surgery. I couldn't repeat half of it to save my life, but I definitely feel informed, which we greatly appreciate!! I now have a really good sense for how she's put back together in there. It helps to know that and be able to sort of picture it, when considering whether or not she becomes symptomatic in the future.
Knowing now what she has been like inside, we have a better understanding about why she has had some of the feeding behaviors and issues she's been having for quite some time. We REALLY hope feeding improves. We always hope that it improves. It will take time to know if there's improvement, but we are hopeful.
Thanks for all your comments! I'll keep everyone posted on any new developments. We should be able to go back to see her very soon.
Here's our tiny dancer on October 30th dancing to the Backyardigans (her current favorite show). She always dances when there's music playing. She's a much more animated dancer these days, so I'll have to get a newer video of her dancing as soon as she's feeling better.
Sofie is back in the O.R. now. Surgery had a cancellation this morning, so they came early to get her around 9:15 am. We just gave her lots of pre-op hugs and kisses and she fell asleep for her usual morning nap. They are getting her settled in and off to sleep under general anesthesia right now. She will be intubated and on the vent for surgery and likely for a period afterward. Dr. S.P. said that he will first attempt the repair thoracoscopically through a few small incisions in her chest. If the hole is very small, he will do another primary repair by just suturing the hole closed. If the hole is larger, he will do the RLD flap repair. There's always a chance that he won't be able to work appropriately from the chest and will have to open her abdomen again through the old scar, but that would be the last resort.
Dr. S.P. said right now it looks like her entire stomach is in her chest along with some bowel, so I'm guessing she will need the RLD flap repair. She has been completely asymptomatic though. She is one tough cookie!
We are just waiting here with both sets of Grandmas and Grandpas to hear updates. I'll post as soon as we hear anything new. Until then, I'm going to try to keep myself occupied by posting some pictures and videos that I've been meaning to get on the blog for a while.
Thank you so much for all your support and prayers. When I sat down in the surgery waiting room and checked my email, I had so many wonderful blog comments and emails to read. It helps so much to know you're thinking of us.
They gave the fentanyl first, because sometimes that's enough all by itself to drift them off to sleep. Sofie immediately got glassy eyed and had pinpoint pupils with it. The nurse said she'd stare off in space for a few minutes and then a lot of babies will just drift off to sleep, but sometimes, they'll fight out of it and need the versed. Of course, our little fighter fought right through it, quite vigorously I might add. So they gave the versed. And stubborn, little tough-as-nails Sofie was still kicking wildly. So they gave the nembutal and it took a minute or so, but she finally did drift off to sleep.
The CT itself only takes about 5 minutes, but it takes about 45 minutes for the sedation to wear off. We had to leave during the CT, but they let us back when she was waking up about 40 minutes later.
She came back up to her room and Dr. P., one of the surgeons that we know, came up to discuss the results. He said the CT did show another recurrence of her diaphragmatic hernia. She does have some bowel up in her chest, though it did not show any bowel obstruction. We were so crushed to hear she's had another recurrence. We really had our fingers crossed that the CT would show no recurrence and we'd find that she had a paralyzed left diaphragm instead. Unfortunately that wasn't the case. Since her original defect was so large (90% of her left diaphragm was missing at birth) and she's already reherniated through her patch once and now she's reherniated through a primary repair (just sewing the hole closed without a patch), they recommended a different fix this time.
They plan to do a reversed latissimus dorsi flap repair (RLD flap) this time. That means they'll use her own latissimus dorsi muscle to repair her diaphragm. They will do the surgery thoracoscopically by making a few small incision in her chest, rather than going back in through her old scar. Since it will be the third major surgery around her diaphragm there will be lots of adhesions/scar tissue to take apart before they can get down to the repair part of the surgery. Longer surgery and more adhesions means an increased risk of excessive blood loss, damage to surrounding organs or nerves and those sorts of lovely complications. However, the risk for those things happening is still relatively small.
As of now (11:30 pm) her surgery is scheduled for mid to late morning tomorrow, and will take several hours. She'll be intubated and on the ventilator for the surgery and probably for at least 24 hours post-op. Because she'll be on the vent, she'll probably be going to the PICU following surgery.
Hopefully everything will go as smoothly as possible and she'll be extubated within 24 hours. I don't know of too many babies that have had to have the RLD flap repair, but from what I've read about it, it seems to be a good method of repair with a low rate of recurrence and several benefits like decreased work of breathing and improvement of chest wall deformity,such as pectus, which she has.
Thanks for all your wonderful comments, thoughts and prayers. It means so much to know you are thinking of us and praying for Sofie. We'll update as soon as we can.
Grandma Cheryl and Grandpa Kenny are here and Daddy will be coming before her CT. We'll update as soon as we know something.
It was so nice outside, and I had her in the sling like I usually do when we go out, so we walked around Brookside and window shopped for a while after we voted. The Dime Store wasn't too busy, so we stopped in and got some new ribbon for hair bows.
I have some cute videos to post when I get the chance to do it. I bought her some new toys last week and she's loving them.
She's getting so big, she officially graduated to the next size of diapers (2) and is fitting into her 6-9 month clothes well. I only buy one size at a time because I never know how long she'll fit into that season's clothes. She just got to the point that her 3-6 month pants fit her around the waist without the legs being too short, so I stocked up on every color Target had. Then the little stinker got taller, so she's bordering on high waters again. They really need to make baby clothes in talls too. ;-) She's 26 1/2" tall, so she's around the 45th-50th percentile for height.
Her weight is currently still at the 5th percentile, but we're hoping to beef her up over the next month. She isn't putting on weight quite as quickly as she did a week or so ago. She's still hanging right around 14 lbs, 10 oz. She tends to grow several ounces at a time and then not gain too well for a week or so, then gain several more ounces. So I hope she continues this trend and gains well this week before she goes back into the hospital next Tuesday. Chances are she'll be having surgery on Wednesday, and I hope she doesn't lose too much weight by the time it's all said and done.
She's still waiting to start her RSV shots. Dr. Y. doesn't have them yet and neither does Children's Mercy. I'm nervous for her to be hospitalized this time of year without having an RSV shot first. It's the worse possible place she could be as far as RSV exposure goes. We'll definitely insist that she be quarantined away from any sick kids!
Tomorrow she has her usual OT appointment with Sherry and her student, Shelly. We always look forward to Wednesday mornings. Sofie also has her first OT appointment at Children's tomorrow afternoon. I'm not sure what to expect, but at least we'll give it a try and see how it goes. We've been so pleased with our FirstSteps experience, not to mention the wonderful bonus that it's done right here at home!
Sofie was baptized by a priest at Overland Park Regional shortly after birth, but we have always planned on having the regular ceremonial baptism for her as well. We had hoped to have her off oxygen by the end of the summer and do the baptism sometime in October, but it didn't work out. Grandma Cheryl made her a beautiful Christening gown that I'm anxious to see her in.
We still haven't decided where we will have her baptism. We'll either do it here at our church or back in Kenny's hometown. Some of you know that we "church hopped" a bit during the last half of my pregnancy. We had attended a church near our house since we moved. We'd been attending for almost 4 years and then decided to officially join after Sofie's diagnosis to plan for her emergency baptism at birth and seek the counsel of our priest. We were shocked to receive a form letter a week later from the parish office stating that we lived outside the church boundaries and could not join. (We live 10 blocks from this church.) We were very offended and did not return to the church. We visited a few others in the area and really liked St. Francis. We joined the parish just before Sofie was born and feel very at home there. Being back at church this Sunday, I was reminded of how much I really like Father Matt and how St. Francis is such a better fit for us than the church we had been attending. At the time, I was so upset and angry that we'd been turned away in our greatest time of need. But it turned out for the best.
Instead, she got to go to the RMT Halloween Party at Daddy's office. She had such a good time seeing everyone. It was SO nice outside though and she was actually really hot in her monkey costume, so we had to keep taking her monkey head off to keep her from sweating. And for the record, the banana/monkey duo was DADDY'S idea! He took home the Funniest Costume award, although there were several good ones! I'd have to say I got the biggest laugh from David's costume. David has been Kenny's partner in crime on the big Houston project over the last couple of years.
Sofie also got to see her cutie pie boyfriend, Luke. I hadn't seen him in a couple of weeks and he's grown so much!! I think she'll clearly be thanking us for our choice of future husband in a few years, because he is the cutest! Luke dressed up as a cowboy - my absolute favorite part is the cowboy hat. What a handsome dude.
Sofie also had to have a "homemade" costume, because I remember my mom always making my Halloween costumes and they were the best. The Wonderwoman onesie came from Luke's Aunt Jenny. He has a matching Superman onesie too. We just added tall red socks for the boots and I sewed her a cape. It was the tiniest cape ever!
She had so much fun dressing up and so did Gracie & Lola, amazingly enough. I can't believe they didn't roll around and try to get their costumes off, but they didn't at all. Gracie actually really enjoyed posing for pictures. It was hysterical!
Here's the Miller Halloween 2008 Scrapbook
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