on the move...

A few days after our last cardiology appointment Sophia decided that all this laying around just wasn't her thing. I turned my back on her for two minutes and the next thing I knew she had decided to start rolling over and had rolled her way across the living room and was under the coffee table sucking on the phone line. Yes, we are going to have to finally baby proof our place.
Sophia is doing well. We still struggle with her to take a bottle but, she seems to be happy with solid food especially oatmeal and bananas. The ENT doctor mentioned that she might have an easier time eating solids than drinking because of her vocal cord, looks like he is right.
On the medical front we have had more shots. We are also trying to get her Synagis shots (these protect at-risk infants from respiratory syncytial virus (RSV)).
Other than that Sophia is a very happy little girl with a sweet nature and a good sense of humor. Here are a couple recent pictures (supposedly we can put videos on the blog so we will try to get one posted).

latest cardiology appointment

Sophia is now a whopping 7 months old! She has started eating solids, her current favorites are bananas and oatmeal. Just recently she has decided that eating is okay. All of a sudden her milk consumption jumped up by ~150 mL (5 ounces) per day and she stopped the tight lip act when it came to spoon feeding. Now, she gets hungry which also means she gets mad when food isn't there fast enough and exercises her one vocal cord....it might be a good thing for our ears that both aren't working (we have to keep our humor in all of this). All of her eating is paying off and she now weighs 13.5 lbs (6.1 kg).
On Monday we had another follow up echocardiogram and check up with Dr. Lewin. Christopher and I were very nervous before the appointment, always that lingering fear of bad news. Sophia was amazing during the echo and stayed still other than squeezing the technicians thumb till it turned blue.
The good news is that her echo looked great! She still has a pinhole residual VSD and some minor valve leakage as well as a little turbulence in one area but, Dr. Lewin said all this was very minor and not anything to worry about. The best part was that he declared us officially "out of the woods". But, they do need to keep an eye on her to make sure everything is growing especially her aortic arch and that no obstructions form. So, we will go back in four months. Sophia celebrated the good news by first trying to pull the stethoscope out of Dr. Lewin's ears and then by giggling hysterically while tearing up the paper on the exam table.

back to the ENT

Now that we are posting again I will try to keep it up. We went to see Dr. Ingles (the ENT) this morning. We noticed that Sophia has once again picked up a bit of a strider (making squeaking noises as she sleeps) and that her voice quality isn't always the best. She has also stagnated in her feeding progress when it comes to taking the bottle. Dr. Ingles scoped her and said that her left vocal cord (which we thought was working) is not moving much, if at all. But, her right one is doing a great job compensating which is why she has a voice and can feed as well as she does. So, all we can do is wait and see if this changes. For now we need to find feeding strategies to help her continue compensating - we really don't want to put the NG tube back in.
So, now we start the next round of appointments - feeding therapist, 6 month well-baby + shots and cardiology. The positive thing this morning was running into other members of the CHD support group - I hope your appointments went well!

pictures from last week

an update...finally...

We apologize for this long silence - somehow time has just flown by. Happily, we can say that Sophia is doing well and has grown a lot in the past couple months.
We don't need to go back to see her cardiologist (Dr. Lewin) till Oct 29th. Our last echo in July showed that her heart was growing normally. They noted some turbulence in one area and a very small hole left where the VSD patch is but we were assured that these were nothing to worry about. For now they will keep monitoring her - the main thing to check for is that her reconstructed aorotic arch grows as she does.
We have also made some progress on the feeding front. For the past 25 days Sophia has had her feeding tube out! The first week was hard - she just barely took in enough milk to keep herself hydrated. Now she is staying hydrated but not taking enough in to grow as fast as she had been when she had the tube....so we are not sure what to do and getting her to take the bottle can be pretty stressful some days. Hopefully this will improve because we really don't want to go back to a feeding tube. Also, we are still feeding her 3 times a night so it would be great if she could get more in during the day (then Christopher and I could also sleep more). We have also started her on solid foods - she loves bananas and in general enjoys eating from a spoon which is great.
Overall though, we are finally settling into parenthood and enjoying it. Sophia is a very happy little girl with a ready smile. Right now her favorite activities are playing with her stuffed horsey, laughing with daddy and taking walks in the garden - especially if she can get her hands on a flower and shred it).

Thank you for all your wonderful support

Sophia is 2 months as of today. We are amazed how well she keeps recovering - in part due to all the positive thoughts we have received from all of you.





Thank you so much for all the wonderful messages, phone calls and cards. You have all been so wonderfully supportive and helpful. If Sophia could, she would tell each and every of you this but for now she just blows spit bubbles and smiles.

Her incision has healed (see her chest) and she is exploring everything around her. While we continue to work on feeding issues she is growing fast. Her weight is now 3385 g (approx 7.5 pounds). Way to go baby!
We will continue to post and give a monthly update with new pictures for those who are interested.

pictures from this week

Sophia spent a little time without the NG tube - it was nice to see her all of her adorable little face.

Sophia napping on the couch.

after two weeks home

Time goes by so fast! We have been home for two weeks now and Sophia is 5 weeks old. We are trying to settle into a routine which primarily involves feeding Sophia. The feeding thing is still an issue and we are still trying to figure something out. She is still getting almost all of her milk though the NG tube. We have been trying various bottle things with little success and she still has this weird thing of swallowing lots of air. So far we have seen our pediatrician and an occupational therapist about this. Next week we finally have our appointment with the ENT doctor plus follow up cardiologist and pediatrician appointments. So, hopefully we will learn something so that we can get her bottle/breast feeding.
Otherwise, Sophia is a happy baby with a beautiful smile. She loves to be danced with or sung to and naps in mommy or daddy's arms.

first days home

We made it back home! The last day in Palo Alto was hectic - packing, feeding Sophia, packing, feeding Sophia, diaper change, more packing...but we made it to the airport and Sophia was an amazing little traveler. We were very grateful to be picked up from the airport by friends and once home settled in for the night.
Sophia is doing well overall but now we are dealing with some feeding issues. She has started this weird thing of gulping air - she does this regardless of whether she is trying to eat or just laying there. We are not sure if this has something to do with the paralyzed vocal cord. We do know that she now has a major problem with bubbles in her tummy and is highly uncomfortable. It also means that she has been spitting up her feeds even when given via the NG tube. On Monday we have our first set of appointments here with the cardiologist and pediatrician and hopefully they can help or get us a referral to an ENT doctor (ears, nose, throat) so we can understand what is happening with her suck - swallow reflex.

first day on our own

Well, we made it though our first day on our own with Sophia. While she had a great first day her parents were a little stressed to organize her feedings and always checking if she was ok. Now we are settling in and enjoying ourselves. We even gave Sophia her first sponge bath this afternoon.
She ended up being discharged from the hospital with an NG tube for feeding, she is just not strong enough yet to suck down what she needs (she weighed 5 lbs 8 oz leaving the hospital). Also, cardiac babies have a hard time gaining weight so she has been put on a weight gain plan where the breast milk is slightly fortified with formula. Christopher and I are learning to manage her NG tube and at the same time keep offering her the bottle/breast so she can gain stamina and eventually eat on her own. We have also managed to give her medications - she is taking Zantac (to keep reflux from being a problem with her NG tube) and also the diuretic Lasix (to keep her lungs from accumulating fluid, a response to the surgery). The hospital was very organized with our discharge and has already set up appointments for us on Monday with the cardiologist at Children's and our pediatrician.
Our next big adventure is the flight home tomorrow night. Once home, we need to keep Sophia somewhat isolated initially until she heals more but after that we are really looking forward to introducing her to everyone.

leaving the hospital

We were discharged from the hospital today! It is quite daunting to be on our own with Sophia with no monitors or doctors around. But, at the same time it feels so wonderful to have all three of us together. Before leaving the hospital they did some last checks on Sophia including a hearing test and also took out the last lines connected to her heart - the pacer wires. They also did one last echocardiogram and removed all of her stitches.
We are very excited about coming back home in a couple of days. Initially, we are supposed to lay low and not take Sophia out for a while but over time we are looking forward to introducing her to all of you!

almost home...

The last couple of days have been quite eventful. Besides finding out that the post-operative echocardiogram showed a successful repair, we were also moved upstairs to the I/ICU on Friday afternoon. This is different atmosphere than the CV/ICU. It is a lot quieter and now we have a chance to fully participate in Sophia's care and do all the wonderful things parents do - feeding, lots of cuddling and of course diaper changing.
On Friday we also found out that Sophia's left vocal chord is not moving. The nerves controlling our vocal cords run near the heart and this is a very common complication when there has been extensive surgery on the aortic arch. Dr. Hanley was not surprised about this because he had to put some tension on the nerve during surgery however he says that the nerve was not severed. The interesting thing about these nerves is that even if they are severed they can grow back. The doctors expect Sophia's to start working again in 1-3 months. Right now her right vocal cord is compensating which is why she can still swallow and vocalize/cry and her voice gets stronger everyday - apparently we can get by with only one vocal cord.
The big news is that the doctors are planning to discharge us from the hospital on Tuesday! We are both excited and a little scared. Tomorrow they will remove the last of the lines on Sophia and run the last couple tests. They will also teach us how to handle her feeding - she will still have an NG tube for a while. We are planning to spend a couple days down here and then head back home. We can't believe that we are going to be home soon and that Sophia's surgery was just two weeks ago! We are really looking forward to having our little family home.

The operation that Dr. Hanley's team performed

This is Sophia's little heart before (top) and after the operation (bottom). Both vessels come out of the right ventricle (hear on the left). The aorta is smaller than the pulmonary artery and moved further to the right (left in drawing). The ventricular septal defect is big. In utero this worked, but once the ductus arteriosus closes (connection between vessels) Sophia would have run out of oxygen.

Below you see all the changes Dr. Hanley's surgical team performed during the very long operation. Can you see them? You should count 6 changes. These changes are massive. But here is the good news, today they performed another echocardiagram and she is doing excellent. Isn't that wonderful. We are so, so thankful and relieved.

swallowing and breathing

Today was a nice mellow day for Sophia. She spent the morning sleeping and playing with grandma. She is a really sweet, mellow baby and smiles easily. They did the swallow test in the afternoon which involved wheeling her (bed, monitors and all) down to x-ray where we fed her a strawberry flavored barium solution. As long as they used a low flow bottle nipple she did just fine - she can swallow and isn't aspirating into her lungs which is good. Tomorrow they will check her vocal cords since the doctors are still worried about the little gasping noises she makes. They will also do a full post-operative echocardiogram tomorrow. Our doctors are making sure that Sophia is doing well before moving us upstairs to the I/ICU. If things go well with these tests we can get moved as soon as tomorrow afternoon.
We also wanted to say thank you to all of you who have left us phone messages. We hope to start returning calls soon but right now we are always in the ICU where no cell phones are allowed or catching a couple hours of sleep. Once we have moved out of the ICU we hope to resurface a little.

vocal cords

The occupational therapist (OT) came by yesterday and again today to check Sophia. She definitely can suck on to the bottle, or anything else that gets close to her mouth (fingers, blanket edges and even an IV line). But, she can't tell how well she swallows. Sophia's voice is also still really week. These two combined makes the doctors worried that there is a problem with her vocal cords or maybe she doesn't have the swallowing process down. During heart surgery the nerves controlling the vocal cords can get damaged. Also, intubation can really be hard on vocal cords, especially in a small baby. The nerves and the vocal cords can recover from this, but it takes time. So, tomorrow they will check her vocal cords. To see if she is swallowing properly they will also do an x-ray tracer study by giving her barium to swallow. When she will get moved to the intermediate ICU (I/ICU) will depend on these results. She is getting all of her milk though an NG tube (it goes from her nose into her stomach) and is up to 25 ml every two hours! Otherwise, Sophia is enjoying being held and rocked - she was even held by her grandparents for the first time yesterday!

more lines out

Sophia had another big couple of days and is steadily getting better. Yesterday they pulled the line out of her right atrium. This was a little scary since she bled more than expected and there was some concern about her having blood trapped around her heart which would require re-surgery. Thankfully, a follow up echocardiogram showed that there was no blood trapped and everything looked good. Today all three of her chest drainage tubes were removed. Now we have a baby that is pretty much disconnected from most tubes and lines. We are now enjoying every minute holding and rocking her! The next big step will be to get Sophia feeding and gaining weight. Tomorrow she will get evaluated for her ability to suck-swallow-breath, that all important newborn reflex. There is also a rumor around the ICU that we might get moved to the critical care nursery in the next day or two. We can't believe that her surgery was just 8 days ago, we are in complete amazement of her and how wonderfully she has come through all of this!

Sophia on her way...

If you thought bottle feeding is hard...(Sophia's life-support lines last week)

And Sophia today (Monday)... she is taking peaceful long naps.
And so are we...

breathing tube is out

They took Sophia's breathing tube out this afternoon! She had no problems breathing after they extubated her. It was hard watching her fight the tube the last couple of days, she was miserable and inconsolable. But now she is looking comfy and is completely alert and looking around. The next big step is to get the second and last line out of her heart. This line goes into her right atrium and monitors the pressure. The tentative plan is to take it out tomorrow or early Tuesday. After that the chest drainage tubes can come out. Then, we can finally hold her again and also start working on breastfeeding (right now she is getting milk through a tube that goes from her nose to her stomach). Christopher and I are so happy to see Sophia feeling better and are enjoying talking and singing to her.

recovery update

Sorry that we have not kept up - the last few days have been busy. Christopher and I are alternating shifts so that Sophia isn't ever without one of us. She is recovering well although the last couple of days have been hard.
Thursday they closed her chest and then were able to take her off a couple of medications (her IV poles, three of them, were laden with all the various medicines she needed). They were going to close on Wed but Dr. Hanley said to wait one more day to get more fluid out of her system. On Th she finally started looking like our lanky little one again.
Friday they removed another line, this one went into her left atrium and was there to monitor the pressure. They also decreased (e.g. dopamine) or stopped (e.g. epinephrine) some of the other medications and stopped her morphine drip. Friday night was when things became more difficult, Sophia, especially for a new born is very alert. Without the heavy medication she now realizes that she, besides being in a lot of pain, has a breathing tube and so she has been trying to fight it. We are doing our best to calm her. They don't want to give her any sedatives or morphine because that will depress her respiratory system and that is what we need to stimulate to get the tube out.
Yesterday they had planned to take out the breathing tube but when they turned the ventilator down to zero she had some apnea which is common for new borns esp ones that have been sedated. So, they decided to wait another day.
This morning we had the scare that there was some air in her chest cavity. It turns out was a small bubble and they were able to remove most of it through one of her chest tubes (she has three tubes still in her chest to keep draining excess fluid from). So now we are waiting and watching to see how she does when the turn the ventilator down...hopefully there will be no problems and they can take the breathing tube out. It is really hard to watch her struggle against it. Christopher and I are amazed at Sophia's strength and resiliency plus she is still the sweetest baby. I will try and post again to say if they were able to get her breathing tube out...

first 36 hours

Sophia is recovering well. Christopher and I are taking shifts to sit with her around the clock. She is sometimes reactive and will open her eyes so we try to talk to her and hold her hand. The first night they expected her to be fairly unstable since the surgery was so major and she was on the heart-lung machine so long, but she showed them. She stabilized quickly and many of her stats (e.g. lactic acid and blood gases) have come back to normal. They were able to take her off some of the medications yesterday and also start her on a diuretic. She is very swollen so this will help remove excess fluid. Her heart is still beating on its own, has a good rhythm and conduction signals. She also has good blood flow to all her extremities showing that the arch repair went well. They were considering closing her chest this afternoon but Dr. Hanley wants to wait till tomorrow to avoid any problems compressing her heart. We will post how the chest closure goes tomorrow.

surgery went well

Dr. Hanley just finished talking to us. He said that the surgery was major but went well. They were able to do the arterial switch and patch the VSD. Fixing the coarctation in the aorta was much more difficult than anticipated and they had to put a patch in along the entire aortic arch. Besides this, they removed some sub-aortic conus muscle, removed the PDA and fixed a small hole between the atriums. Overall, everything looks good and he said Sophia has a good strong heart muscle. The next few days will be critical - she will need to stabilize and some of the swelling will have to go down. She will likely stay on a ventilator for the next week and will be kept heavily sedated the entire time.
Christopher and I can both honestly say this has been the hardest and longest day of our lives. We thank all of you who have been sending your thoughts and prayers to us. We hope to finally put a long posting about how the last few days have gone that is more detailed, in the mean times, we (or one of Kenia's sisters) will continue to post a daily update on how Sophia is recovering.

Her face is changing so quick...

Pictures, Pictures

Here are a few of pictures of Sophia from the last couple days. Enjoy!!

All is going well ...

Baby girl Krembs finally has a name!! We are pleased to introduce Sophia Luz Krembs! Sofia is doing well in the NICU. She is stable, active, and blowing bubbles to her visitors. She is eating well, and even gaining a little weight. Her surgery is scheduled for Monday, April 2nd. The surgery will last for about 8 hours and more information will be posted as that day unfolds.

Kenia and Christopher are doing well, now that they got some sleep. Kenia was released today from the hospital and both are enjoying every moment with their daughter. Kenia was able to hold Sophia yesterday for a while and will be able to continue doing that all weekend long. All three of them appreciate you well wishes and prayers.

Baby Krembs Is Here!

We are happy to announce that the baby came early this morning, March 29th, at approximately 12:52 AM! She is beautiful, with dark hair and dark grey eyes! We are also happy that she is a healthy weight and size: 6 pounds, 1 ounce, and 46 cm. (about 19 inches) long. Kenia put up a hard fight for almost 20 hours, and now we are settling into recovery rooms. The baby is currently in the NICU. More news to come! Thanks everyone for all your support and phone calls.

Baby Almost Here!

We are still in the middle of the induction -- but no baby yet! Keep posted!

induction date

On Thursday we had another monitoring (baby looks good) and then met with our OB, Dr. Smith. We now have an induction date! They will admit Kenia to the hospital Tuesday evening and start the process. Assuming the usual progression, our little girl will be born on Wed, March 28th. We vacillate between being totally excited and also completely scared but more than anything we really can't wait to finally see and hold her.
This weekend we are back at Kenia's sisters place to enjoy some fun family time before the big event. We will definitely get a birth announcement posted as soon as she is born to let you all know how things went.

she is getting bigger

We moved into the Ronald McDonald house last Wed. It is really great to be so close to the hospital and things at the house are well organized and really designed to support families with hospitalized children.

Today we had the growth ultrasound. The great news is that she is growing! Our last measurement here (2 weeks ago) put her at 4 lbs 5 oz (1.9 kg). Today she measured 5 lbs 4 oz (2.4 kg)! The OB says she is in the 5-10 percentile range for weight.

When we met with Dr. Hanley (surgeon) he said that the survival rate is much greater for babies above 2 kg, below this they have a harder time recovering from surgery. So, we are very, very happy with her growth since this puts her above the 2kg mark...hopefully she will keep growing. Kenia will diligently continue eating for two including twice the amount of ice cream.

Now the plan is to wait till next week. According to our OB, waiting the additional week also has the added benefit (besides growth potential) of allowing her immune system to mature even further. So, we are looking at an induction next week unless she decides to come on her own.

more appointments

On Monday we had another round of appointments. The first was with Dr. Susan Hintz, the neonatologist. She spoke with us a long time about how they will stabilize the baby once she is born and what to expect from birth to surgery. She also gave us a tour of the neonatal intensive care unit (NICU). This was once again very difficult but it is good to see and gives us an idea of what to expect.
We then had another heart rate monitoring for the baby and she looked great. Our last appointment was with the OB. The current plan is to wait till Monday when they will do another ultrasound to check if the baby is growing. If she is not growing, they will induce next week. If she is growing they will wait till the week of March 26th and induce. Either way, she will be born in the next two weeks!
The other news that we received this morning is that we now have a room at the Ronald McDonald house a few blocks from the the hospital. We will move there tomorrow.

meetings and tours

On Thursday we met with Dr. Hanley, the cardiothoracic surgeon. We learned a lot during the meeting and feel very confident in his ability to do the repair the baby will need. The surgical plan is to do an arterial switch (literally cutting the pulmonary artery and aorta and switching them), patch the VSD and fix the coarctation of the aorta. This is a big surgery, with Dr. Hanley rating this as an 8-9 on a 10 point scale of difficulty (with 10 bring the highest) but the survival rate it 97%. The good thing is that it is a one-stage repair with kids doing very well afterwards and having a good quality of life (no need for life long medication and a normal healthy activity level) if things go well.

We also meet with the social worker assigned to facilitate our stay who gave us a tour of the cardiovascular intensive care unit (CVICU) and the critical care nursery. This was the hardest part of the day to see that both units were very full. We really felt for the babies and their families and also realized we would be there soon. On the other hand, it is also a place of hope where these children are given a chance at life.

Our last stop was for checking on the baby at our bi-weekly monitoring of her heart rate and amniotic fluid levels (AFI). Both looked great. We then headed back to Kenia's sisters place. Our next appointment series will be on Monday so we are enjoying the weekend and the California sunshine till then.

first appointments

Right now we are staying with Kenia's sister which is really great but it is not very close to the hospital. We are waiting to get a place closer, hopefully at the Ronald McDonald House which is a few blocks from the hospital. We had our first two days of appointments but before we describe the appointments, we have to complain about bay area traffic - it's horrible. It took us over 2 hours to get to the hospital...Kenia better not go into labor during rush hour. Anyways, here's how our first two days went:

Day 1: The Lucille Packard Children's Hospital (part of Stanford Medical Ctr) is very nice. The ultrasound told us what we already knew - she is very small - between 4-5 lbs. The accuracy of baby sizing at this point is +/- 12 ounces. Her heart rate and movement was good. We then met the high-risk OB (Dr. Jim Smith) who was very personable, took a lot of time with us and is a very good OB. The current plan is to wait till next week (we are currently at 36.5 weeks next week will make 37 weeks, technically full term, the baby is due April 2nd). They will likely do amniocentesis next week to check if the baby's lungs are mature. If they are, then an induction date will be set probably for sometime during week 38 - unless she decides to come on her own before then. They fully expect that the baby will go through labor just fine so it looks like we won't need a c-section. We then started driving back home...another 2 hours. At this point we had dinner and passed out.

Day 2: Wake up, drive two hours. Our only appointment is an echocardiogram. First we started with a great technician. Then Dr. Silverman came in - he is a pediatric cardiologist and specializes in fetal echocardiography. He was great! He is an excellent doctor and is gregarious with a good sense of humor. They were able to get the best images of her heart that we have seen so far! This is really amazing especially considering that imaging her is more difficult now because her ribs are more ossified and cast shadows when trying to view the heart. Needless to say we were very impressed. The echo didn't show anything new and confirmed what has been our understanding of her condition so far - double outlet right ventricle, large VSD and a coartation. He also told us they just did surgery yesterday on another baby with a similar condition who weighed only 2.4 lbs! This baby is doing just fine so far. Amazing.

Today we have the day off and are trying to catch up on things and rest. Tomorrow we go back for another full day. Overall, after our initial appointments, seeing everything and meeting the doctors we are very satisfied and happy that we traveled down here.

on our way

Today I (Kenia) head down to the bay area, Christopher will follow in two days. We figured that its best to get the "mother-ship" down there and not risk anything happening here. This week we had a growth ultrasound and they estimate her weight at 4 lbs 10 ozs...in other words she is going to be a tiny little nut. Next week we have several appointments at Stanford - we have been impressed with the level of organization they have shown in coordinating our logistics. We will post something again next week once we go through these appointments. Thanks again to everyone for all of the support you have given us - we can't stop thinking these words (thank you) because all of your efforts in helping us has meant so much to both of us.

History

Christopher and I decided to put a blog together in the hopes of keeping everyone updated. Firstly, we want to thank all of you who have in so many ways supported us these last few months - we are immeasurably thankful for all the help and support that has been given to us. We definitely would not have managed without you.

Now, just an abbreviated history to make sure everyone is on the same page:

Aug. 2006 - Kenia is pregnant, Christopher thinks he looks pregnant

Nov. 2nd - we found out we are having a little girl! We also learned that she has a congenital heart defect (CHD).

Nov.-Jan. - lots of ultrasounds and echocardiograms (specialized ultrasounds to look at the heart anatomy). Our little girl is diagnosed with a large ventricular septal defect (VSD), double outlet right ventrical (DORV) and a coartation (narrowing) of the aorta. We also join a CHD parent support group where we get valuable advice and also meet several courageous families that give us lots of hope.

Jan. 17th - Kenia gets admitted to the hospital. Ultrasounds show that the baby isn't growing properly.

Feb. 14th - We have our last surgical consult (we had three total). This last one is with Dr. Hanley at Stanford Medical Center and we decide to go there for the delivery and baby's surgery.

Feb. 15th - After 4 weeks of hospital bedrest, Kenia is released to go home for bedrest, baby is doing fine and growing again.

Ok, that should catch everyone up for now.