Official Maia Diagnosis

For those of you wondering what, exactly, ended up being wrong with Maia, here it is. She has three problems/potential problems. The first two, congenital cutis aplasia and possible tethering of the spinal cord, is what brought us to CHOC. The third one, extreme sensitivity to propofol (the "Michael Jackson" drug) is what has kept us here longer than expected.

She has Congenital Cutis Aplasia, meaning there is an absence of skin from birth. It isn't common (approx. 3 in 10,000 births) and so there isn't a lot of info online about it, but it is actually most commonly found on the scalp. The condition may be because of the absence of underlying bone structures, or it may be that everything else is fine (i.e. it is just cosmetic, her vertebrae are intact underneath). Maia's is cosmetic. We've been talking with various doctors to understand her problems and it's taken a few days to come together in our minds. Maia will likely undergo a minor surgery in the next few months to correct this skin problem. My understanding is that although it is cosmetic, because it is "indented," the skin defect is lying very, very close to the spine. As it heals it will develop scar tissue, and that scar tissue could form attachments to the spine, which may cause some pulling. So on the one hand, surgery to correct it would be cosmetic, but it would also be preventative of potential future problems.

The skin trouble is what we noticed when she was born. Because it can be caused by absense of underlying bone structure, they wanted to look at the spine underneath by MRI to ensure that the vertebrae were intact and there were no spinal cord issues. Saddleback does MRI's on adults but not infants, which is what brought us to CHOC.

The mild sedation, propofol (despite its infamy from causing Michael Jackson's death, it is a VERY safe sedative and the most common used on infants, complications from its use are very rare), was used in the MRI to keep Maia still. Although there is always some risk in sedating a baby, there was more risk in letting her go home with a potentially exposed spinal cord. Her negative reaction to propofol is what led to the scariest day of my life.

The results of the MRI show that her spinal cord ends just a millimeter or so lower than it should. This means that her spinal cord might be tethered to something that is pulling it down lower than usual. They couldn't confirm this for sure in the MRI, which is why she has "possible spinal cord tethering." There is no immediate risk to this, as she grows it would continue to stretch and probably not cause acutal problems for another decade, however we will become good friends with CHOC's pediatric neurologist over the next couple of years as she has regular checkups, ultrasounds, and another MRI somewhere down the road to see what is happening to her spine as she grows. If it is tethered, untethering it is a very simple corrective procedure. Spinal cord tethering is on the neural tube disorder spectrum, but is on the very, very, very mild end of that spectrum. She has no movement or reflex issues, i.e. she has full use of her body and no immediate consequence to the condition.

The official medical language - Reason for MRI: "There is a tract in the superficial skin/soft tissues of the lower back at the approximate L3-L4 level which connects to the spinal cord." Findings: "There is no evidence of an associated lipoma of myelomeningocele. Low-lying conus terminates at L3-L4, raising concern for possible tethered cord."

So all the news is very good. Getting here was a rough journey, but at the end of the day we can breathe a huge sigh of relief and thank God for a healthy little girl that will grow and develop normally and never even remember that she had any problems as a baby. And when she cries or destroys things or makes me want to pull my hair out with her stubborn personality, I'll just be glad that she is healthy and alive. :-)

Last Night

Last night was the my final time sleeping at the hospital (hopefully for the rest of my life.....) as tonight I will be at home with Josh. :-) Maia's nursed convinced me that she could take care of my baby girl just fine, and that I should go get some sleep, as much as I could, since a full night's sleep is something I won't have for a while. I surprised myself by sleeping a full 8 hours and woke up feeling incredibly well rested. (And yes, Maia did seem to be taken care of by her nurse just fine, although I'm sure not quite as well as mommy could have!).

I can call and confirm in about an hour, but unless there are any big surprises, we can pick up our son any time today. We're planning to spend several hours with Maia Jean and then pick him up mid-afternoon. I can't wait to take him out of the hospital - and this time I get to carry him (no wheelchair) since I'm not a patient anymore. Well, I'll let Ryan carry him, he's a bit heavy! Another week of healing for me and then I'll be lifting him just fine.

Our First Relaxing Evening

This is the first evening we could really sit back and enjoy both babies (not together quite yet). I spent about 4 hours with Josh this afternoon and then Ryan and I spent the subsequent 4 hours with Maia. 2 of which were spent trying to keep her eating - she had 2 feedings we'd call successful today and one that was ok, another 4 that weren't very good. But that's better than yesterday when she wasn't eating anything, so we're moving in the right direction. Josh is still on track to come home tomorrow afternoon and depending on how Maia's eating goes, probably Sunday.

Sleeping Josh - we've taken to calling him little linebacker instead of little peanut because he's kind of huge....

His feeding tube came out today but after his second successful feeding with mom, but I wasn't able to stick around to get any pictures with it out yet. They hadn't used it for a couple of days but had left it in just in case.

Meeting Aunt Jen

Burrito Baby - I thought he might not like to be swaddled since he spent his first 5 days naked under heat but apparently he's just fine with it

Then back from Saddleback to the CHOC floor at Mission to cuddle with Maia Jean

All of Maia's nurses love her...they made her name sign. Cute! :-)

Mommy trying to hold her up for a picture....if I look uncomfortable it's because holding them still hurts my incision a bit, I usually lay them down in front of me on a pillow. But that's not as cute for pictures.

Steps Toward Going Home

We just graduated from our warmer-bed....

Back to a regluar crib. :-)

It's encouraging to see that things can turn around for the better just as quickly as they turned around for the worse a couple of days ago. Thanks everyone for your prayers. I don't have current pictures of Josh right now, but his daddy is at Saddleback for a car set inspection in anticipation of being released tomorrow. And Maia's PICU doctor said Josh is welcome to join us in her room during the day tomorrow - so it is possible that brother and sister will be reunited in about 24 hours! I think Maia will improve even faster when her brother is by her side.

Waiting

The main thing we are waiting on from Maia is re-established feeding. She was a good little eater before her poor reactions to the sedative used in the MRI, and now we are going on close to 48 hours of very little eating (she is being supported by an IV with essential nutrients and fluids). She had her last good feeding at 10 am on Wednesday. We tried re-introducing feeding by bottle on Thursday around 4 pm. She has had 4 bottle feedings since then and she shows interest in eating and signs of being hungry, but can't seem to suck hard enough to actually get anything to swallow in her mouth. She isn't appearing to get very frustrated, but she is also very lethargic so maybe she just doesn't have the energy to get worked up. We need her to get back to her regular good sucking/swallowing. It took me 45 minutes to get 10 mL down her at her 3 am feeding, and I used to get 40 mL in about 10 minutes.

Great Day

The highs and lows of this week are astonishing - didn't know we could handle this much in one LONG week. Josh and Maia will be one week old tomorrow. Today was a great day. I'm off to get some sleep soon so won't write much - still exhausted from yesterady - but just wanted to say both babies are doing great.

Maia - came off the ventalator at 1, heat lamp off at 2, took 1 oz at 5 and slightly less than 1 oz at 9 (both bottle feeds). This is a bit low and her latch/sucking isn't very strong, but she's low on energy from a very taxing 48 hours and just trying to sleep and recover, she'll be back to her regular eating soon. She needs to stay for about 48 hours after re-establishing regular feedings, so if she gets that back tomorrow we can have her SUNDAY! :-) Also got the results of her MRIs which I'll post about in more detail later, but suffice it to say for now that she does not need any type of immediate surgery and has plenty of time to grow strong and big before needing any corrections. She does not have ANY spina bifida and on the spectrum of possible neural tube disorders her problem is very minor and very easily corrected.

Josh - came out of his isolet this morning and has been off EVERYTHING since then and is doing great. He did a pretty good job and breastfeeding his first try at his 6 pm feeding tonight and gets 3 more tries tomorrow. But he is already doing enough at feeding that he can come home SATURDAY! :-) So by the time this week and month are over we will all be home as a family. Josh is stocky and really mellow - difficult to agitate - and drank 80 CCs of my milk (bottle) at his 3 pm feeding, which is twice what Maia was drinking before her Wednesday fiasco. He was born with a double chin and I think he is going to be a linebacker. :-) Maia is small but she's a little pistol and seems pretty high-strung. Personalites seem to match the parent with the matching gender. :-)

Ok now I'm just going to post all my favorite pictures I came across while we were looking at the past week tonight and just breathing big sighs of relief......

Josh being born

Josh and Maia :-)

Cousin Audrey bringing gifts Cousin Audrey trying to take gift back!
Little Miss Maia Jean, 1 day old

Josh and Daddy
Thursday's Goal: Excubate

Daddy wanted to be the first to hold Maia after she got her ventalator excubated Our sweet little girl recovering

Josh watching over Maia while she was very sick

Maia Jean is back :-)

Daddy says "Don't scare us like that again, ok?"


Maia Jean says "Don't sedate me for an MRI again, ok?"


Ok! :-)

Quick Maia Update

Woke up for a middle-of-night feeding and came in the PICU to be with Maia during this time, so thought I'd post a quick update that she is improving. Her pediatric neurologist was here after 11 pm last night going over her MRI and EEG results and says her brain looks perfect. We are still waiting on lab results for a variety of potential infections but in the mean time she has made some improvements:

- She is starting to breathe on her own. They are weaning her from the ventalator, turning it down little by little, and she is starting to take independent breaths in between.

- She is getting her little personality back; making those little faces with her lips especially and getting a huge scowl on her face if I touch her too much while she's trying to sleep ("leave me alone mom, I'm trying to rest!!!"). She's getting a little fidgety, which is much nicer than her lifeless body yesterday. Things are looking good for removing the ventalator in the next 12-24 hours and letting her breathe on her own again.

This was a picture of us leaving Saddleback with her on Tuesday, on our way to CHOC. Hoping to get back to this picture soon, and to add Josh to it soon as well:



There was a time last night when I started doubting whether we would ever bring Maia home or not because of what she was doing through. It was the first time I'd thought in terms of "if" instead of "when" and that was a really hard thing for me. My heart really goes out to the parents who exist in this type of sustained crisis mode for months and live with constant daily "ifs" instead of "whens".

Ronald McDonald House

23 years ago I spent a lot of time with my parents and little sister at a Ronald McDonald house, so that we could be close to my twin brothers, who were in an NICU several hours away from our home. I was only 5 and I didn't get what was going on, I thought it was fun, like going on vacation.

Tonight for the first time I am a parent at the Ronald McDonald house. It's so awesome that we can stay here, even though we only live 30 minutes away, that's an extra hour a day I can spend with my babies. Plus I am not ready to go home and be there without them, so it's nice to be here at the hospital. It's a great facility, they have beds and a nice bathroom and snacks and a kitchen. But it is certainly not fun and nothing even remotely close to a vacation.

I think my parents are amazing and such strong people for getting through their difficulties having children and it's interesting how you can never really glimpse how hard someone else's life must be, until you've experienced a little of it yourself.

Wednesday, April 27 - good day for Josh

Josh drank his first bottle - my milk - today at noon. His nurse told me he "sucked his first bottle down in nothing flat." This is great news because it means his sucking/breathing/swallowing instincts are already working. It also means I should be able to start working on breast-feeding him with a lactation nurse by Friday or Saturday.

Ryan just left to go see him, I'm just not up to going anywhere tonight so I hope the little guy understands. I did send him milk, a blanket, and a little onesie since his nurse said he could wear clothes now. He will be likely be moved completely out of his isolet (sp?) and into a normal crib tomorrow - no heat lamps, no oxygen, no IV. :-)

Also as I was going through some things at the hospital today I stumbled across my necklace with some of Josh Newman's ashes on the inside - it's a little silver heart. I found it while I was unpacking from our move out of the regular room into the PICU, and putting it on made me feel really strong. Now I have Josh Newman here with me, I feel like I have a little bit of my baby Josh here with me since that's his namesake, and they are both here with Maia too.

Wednesday, Apr 27 - rocky road today with Maia

Maia's big brother watching over her - I don't know what this feels like but he does. They haven't seen each other since Friday and I can't wait to get the two of them together in real life instead of just a picture. I think the will have a very special bond

Today had a lot of ups and downs. After not feeding Maia from midnight to 8 am to prepare for a possible sedated MRI of her spine and head first thing in the morning, a few emergency pediatric cases took priority and we had to wait until 3. They let us feed her around 11 and that seemed to satisfy her enough to sleep until the 3 pm MRI. I was able to go over to Ryan's mom's house, sleep for 3 hours, take a shower, and change clothes for the first time since Sunday I think? It was a good thing I did because I felt like a new person and ended up needing that energy to cope with the rest of the day.

MRI good news - They were able to get a good picture of Maia's spine and the pediatric neurologist has concluded spinal issue is only related to spinal tethering, which is a relatively easy correction that doesn't require actually touching the spinal cord. Our concern with timing of the procedure was due to the skin issue, which is actually more pressing than the tethering - however her body appears to be healing the skin (it's starting to develop scabbing) so it looks like we will probably be able to hold of on the surgery for a few months. We have not had a formal meeting with the neurologist but looks like that's where it is headed.

MRI bad news - Our poor little sweet girl started having problems breathing while in the MRI. They pulled her out early. Assuming everything was fine I had gone back up to our room to rest while Ryan stayed in the waiting room. He said it ended up being a good thing because it was incredibly traumatizing to see her rushed back up to the PICU (pediatric ICU, similar to the neonatal ICU Josh is in at Saddleback) with a team of doctors surrounding her bed and using a manual pump to breathe for her. We knew reduced capacity to breathe was a possible outcome to the sedation (and almost definitely the outcome to any full anesthesia for surgery, which is why for the surgery breathing tubes would have to be put in), but seeing it is different than hearing it. When Ryan, the nurse, the doctor and the anesthesiologist walked into our room I pretty much stopped breathing too and thought I was going to throw up before they even started talking and telling me what was going on.

To summarize the rest of the day, she also had a slight temperature drop (after maintaining perfect body temperature for 5 days) and what appeared could have been 2 minor seizures. Within 60 seconds of the 2nd seizure they had her hooked up to an EEG to monitor her brain and determine why she was having them, but she hasn't had anymore, so hopefully it was just her body's reaction to coming out of the sedation.

Hopefully the temperature drop and breathing difficulties are just related to the whole procedure as well. It's very possible all these symptoms are just due to stress on her little 5 pound body, which we were somewhat prepared for. In the meantime, instead of holding my little baby and rocking her to sleep right now, she looks like her brother did a few days ago- she's under a heat lamp, on a ventilator and an IV drip, and having her heart rate and brain waves monitored. They are running several lab tests to rule out possible infections (some of her symptoms could indicate infection), the list of which I will not even start to describe here because no use wasting energy on negative things.

We have moved from our pediatric room. There is a small Ronald McDonald House apartment on the CHOC floor, we have a room with a twin bed and access to a really nice kitchen and bathroom. Maia also has her own room in the PICU, with a fold out bed for one of us. So we'll probably alternate sleep tonight, each of us spending a few hours in each place.

It looks like we will be here a little longer than expected.

I am typing this while Maia is getting a 2nd MRI for her head since it wasn't possible to do it the first time around. The head is easier to keep still than the body so they are doing it this time with no sedation and just securing her head. Plus she is exhausted and probably won't even open her eyes for another day.

Apparently these babies were not ready to come yet. Mommy is feeling very badly that her liver wasn't able to hold out long enough to carry them longer. Damn cholestasis.

Tuesday - quick update

Tuesday day was uneventful aside from moving Maia from Saddleback to CHOC (CHOC is wonderful and we are very comfortable, we met our pediatric nerologist and it was great to speak with him face to face), Tuesday night was rough. Found out Josh was not able to start eating yesterday (so he is still being fed through a tube) because although his breathing is still improving, it wasn't stable enough for the dr to feel comfortable trying to feed. Not the end of the world, just a little disappointing as we had really hoped to hear he was doing well with real food in his stomach. On the bright side he was more alert than I'd ever seen him (wish we had brought the camera!) and it was like he was making eye contact with us which was really cool.

Ryan's mom watched Maia so we could both go see him together, when we got back to CHOC around 10:15 they had taken her to give her an IV. We knew they would probably be doing this; we couldn't get her to hold still enough for yesterday's MRI so we are trying again this morning with a little sedative. We initially thought she would not be able to eat for 4 hours, which isn't a big deal, but the anesthesiologist decided it really needed to be 8. She has been much better than I thought she would be after being hungry, but has definitely been more fussy than usual and I've been up with her about a dozen times in addition to Ryan's 3 or 4 times (only because I can't help but get up with her most of the time whether Ryan does or not, so I tell him to just put on headphones and sleep since I can't sleep through her cries anyway) in the past 6 hours. I.e. no sleep for mom. My pain levels from the C-section are definitely up, probably from anxiety and lack of sleep. We are going to leave the hospital to sleep some this morning after such a long night.

I'll try and post an update when we get MRI results.

My little baby being quite the trooper:

Monday, April 25th

Monday was a good day. First several good news items about Josh:


We got to hold him for the first time! Before this we have only been allowed to touch his hand or rest a hand on his back. On Monday, Josh spent the whole day with no breathing tubes, meaning he was breathing room air naturally rather than having oxygen delivered to his lungs.


Also, on Sunday, he had started being fed through a feeding tube (rather than just having nutrients delivered through an IV). I had provided about 6 milliliters of colostrum at that point, but his nutrition needs are about 120 milliliters a day, so the majority of what he was given was formula. He did not digest it very well, which caused his breathing to be more stressed out - his breaths per minute increased from the food, which is not what we want. On Monday, which is only the 3rd day, I was able to give him about 60 milliliters of pumped milk, so half of what they gave him was from me. At each feeding they would mix half my milk with half of a different type of formula meant for sensitive babies, and he digested that just fine with no stress to his breathing.


As long as he tolerated his 9 pm feeding well (he's fed every 3 hours), we were told we could hold him. We got there at about 9 (thank you family for coming to watch Maia for us!) and sat with him for a half hour while he digested that feeding, and since all went well we were able to hold our little boy for the first time. He is so much bigger than Maia but also very calm just like his sister:





The plan on Tuesday, so long as he has continued to tolerate feedings well, is to bottle feed him. I would actually be allowed to try to nurse him at this point, but since we have a big day of tests and meetings with doctors at CHOC with Maia and I am not getting a lot of sleep, we decided we will continue to deliver as much pumped milk as possible but hold off on adding one more thing to our schedule (learning to breast-feed him) until we have things worked out with Maia. Holding him was the best part of my day but at the same time it will make it a lot harder to leave him in the NICU when we discharge on Tuesday. Something about holding him increases my attachment to him more than words can describe.


Maia also had a good day. She actually gained a little weight back (very good for only day 3, many babies are still losing) which will be a huge blessing if she ends up having surgery this week. Every little ounce counts when you are that small. She had dropped down to 4 lbs 15 oz on Sunday and was back up to 5 lbs 1 oz on Monday (original weight was 5 lbs 4 oz). She also had a couple of feedings where she exclusively breast-fed with no formula supplements, which I really do think is better for her at this point as she needs every immunity benefit she can get from my milk because of her size and her potential surgery.

Because we won't get to take them home, we put her in her little take-home outfit, it's newborn size but she swims in it! She's such a little doll.

Also we had two different friends who have connections/experience in the Orange County medical arena ask around about our pediatric neurologist. We referred by Josh and Maia's pediatrician, whom we obviously don't know very well yet, so just wanted to find out who the best of the best is and anything we should be aware of about the CHOC system. Both friends came back with similar information that makes us feel very confident about the doctor we will be meeting today and also gave us some good ideas of questions and concerns to ask/express. We have had very little time to do any of our own research or learning and really appreciate others jumping in and doing it for us, if nothing else to give us peace of mind.

Holding Up

We are both nervous about having two babies in two different hospitals, and mommy is anticipating being an emotional wreck walking into the house on Tuesday with no babies. But we are blessed that both Josh and Maia are incredibly strong and doing very well, and both of their problems are very treatable. We are in the hands of fantastic doctors, and have had tons of help, support, love and prayers from family these past few days that will continue these next few weeks. Josh an Maia have a lot of people that love them and are pulling for them! We're hoping to have the whole family at home and healthy by Mother's Day. :-)

Maia

Maia had her radiologist appointment at 4 pm the day of her birth, when she was only 3 hours old, and was referred to a pediatric neurologist because she appears to have something called "cord tethering." It is not life-threatening and is fixable especially when caught so early (sometime a baby without external symptoms such as her skin issue can grow into young adulthood before symptoms develop that allow diagnosis, at which point treatment options are more limited). Mommy and Daddy need to do a lot more research but have been very busy, our basic understanding is that the spinal cord, which has many fibers that should be free so that it can grow with the person and also move and expand with movement, is not free in Maia's body near the L4 and L5 vertebrae. It is "tethered" at the L4 and L5 vertebrae, somehow the fibers are attached to each other and also to her skin, and therefore will not be able to move freely as she grows (for now it is not an immediate problem). The skin covering over the vertebrae is very thin - i.e. she only has a few layers of tissue over the spinal cord at that point:




Because there is not immediate danger caused by the problem, she is staying with us in the hospital until we are discharged on Tuesday. She is nursing like a champ and is very strong and has her daddy wrapped around her little finger! But immediately after we leave on Tuesday we will go strait to Childrens' Hospital of Orange County and check her in there. She will have an MRI and we will develop a game plan with her pediatric neurologist, which will potentially involve surgery later this week to release the tethering. If the surgery is successful, she will need follow-up ultrasounds and possibly MRIs as she grows to ensure that there is no missed tethering and that none of it coming back, but potentially the surgery would fix the problem to the point it would never be an issue again.

No spinal surgery, and no surgery on a 5 pound infant for that matter, is ever "minor". But of all the things that could be wrong with a spinal cord, we feel thankful that it is a minor problem, relatively speaking. I was very emotional when they told me we wouldn't be taking her home, but I am dealing with it a lot better now.



Mommy, Daddy, and grandparents have enjoyed every moment of getting to know Maia over the past few days. She has been getting a lot of love and we are just spending every minute we can together so that she'll know how many people love her when she has to be left alone more and possibly operated on later this week: