Choppy Waters

It seems the past week or so has been very uneven. She may be doing great in the morning and then struggling in the evening. The last few MAP points are a struggle. Yesterday afternoon she started in the 40's and by the time I left was in the high 60's. Afternoons like that are very draining. She has strong opinions on what she likes and does not like. The first major disruption was a change from being on her stomach to her back. She would not settle for hours until the nurse relented and put her back on her belly. The last big drop was typical Natalie-big desat for no apparent reason. Then we check her diaper and find that she is wet. There are things this girl is just NOT going to tolerate. I always say more power to her. She should give 'em heck. They always tell me how stubborn she is but I tend to think that is in her favor. A person would have to be very strong willed and stubborn to fight as hard as she does.

Well, she seemed to master a MAP of 15 at one point but it was short lived. They tried it again yesterday and she tolerated it for most of the day but climbed up in Os overnight. This morning she was at 74% and the neonatologist said "she's obviously trying to tell me something." The MAP went back up to 16 and hopefully she will be able to recover the ground she lost.

The neonatologist met with the pulmonologists today to get their perspective. They may attempt to move her to a conventional vent without having reached the low MAP that was the original goal. I sometimes wonder if, as a big baby, she is tired of the oscillator and wants to breath in a normal fashion rather than at 600 breaths a minute. There are some instances where changing a baby back to the conventional results in an unexpected improvement and I suppose that is what we are hoping for. At the meeting today they were going to discuss if there would be any value in doing a round of steroids to help her make the vent change. We will talk that over tomorrow before any final decisions are made.

Going back to the conventional vent means she will be less vulnerable to atelectasis (areas of collapse in the lung), she will be more mobile, will be able to wear clothes again AND she can be held when she is stable. This is of course the part that makes me jittery with excitement. It sounds crass, but I can only imagine this is what a junkie feels when they are about to make a score. My body has been in extreme baby withdrawal and I am going a little buggy waiting for my next "hit." I am trying to not get ahead of myself with excitement until the moment is close at hand.

Going back to the regular vent also means her lungs are more vulnerable to the damage that causes BPD, especially if they can not get her settings under control quickly.

It's a gamble, just like so many decisions we have made a long the way. In my heart I believe that if we can get Natalie to settings low enough for her to have a trach she will improve much more quickly. Being comfortable, being held, having her mouth free for normal baby things can only help her thrive. We just have to get there and the wait is torture.

In non-respiratory news, (drum roll please) Natalie resumed feedings today!!!! She had a new ND (nasodoudenal) tube placed and they are running very small amount of pedialyte through it. If she tolerates that then tomorrow she may start elemental formula (it is predigested-yum) or breast milk.

This is the time of year that new residents and fellows enter the hospital and I have to say it makes for some interesting conversations. I do feel for these people as they advance in their medical training but at the same time I really do not want people practicing on my daughter. Today on rounds there was a great collection of newbies and so the whole process was understandably slower as people are learning as they go. I give the neonatologist a lot of credit for her patience. When it came time to decide Natalie's new feeding protocol the fellow immediately started calculating large feedings as she is a "big baby." The attending said deliberately "she had surgical drains....she had NEC.....we are moving slooooowly." Thank God that the newbies are not allowed to run around by themselves until they have earned their independence.

Annie is playing blocks with Daddy and making a "foot castle." I believe that is a castle you can put your foot into. She continues to love school and is making friends. It is a blessing to see her doing normal little kid things and having a life outside of the hospital. I realized the other day that she has been going to the hospital nearly every day for over 5 months now. That is not normal for a toddler but she has adjusted beautifully. As long as Natalie is there Strong continues to be a second home for all of us.

We ask for prayers that Natalie recovers from the MAP change and that she handles any changes in the next week as smoothly as possible. As always we also ask for prayers of strength and courage.

Natty resting comfortably. The bandage is gone now and plastics has signed off on her pressure sore. It is healing beautifully.


One of Natalie's favorite nurses bought her some fancy socks. They are too darn sweet.


Natalie's new ND tube just placed today. Hooray for eating!!!! She loves to keep her hand up by her ET tube, a habit that makes us all very nervous. Normally her arm is positioned in a way to prevent her "helping."


This onesie was passed on by another little girl who battled the NICU and won. I think "one brave chick" sums up Natters pretty well! Thank you Emma!

A little update

Natalie did get down to 16 on her MAP but then started to struggle. Wednesday and Thursday her O2 requirements climbed and ultimately reached 88% Thursday afternoon. This was a huge disappointment and very frustrating. We met with her doctor and the theory is that they moved on the MAP a little too quickly. In addition to that, not all the nurses were calling respiratory therapy for suctioning and position changes. The RT's can increase her MAP for a short bit during those times so that she does not lose ground. Any time she loses alveoli during stress/suctioning it takes a good amount of time for those alveoli to be "rerecruited" and useful again.

An order was written for RT's to be present for suctioning and position changes. In addition to that they asked that she not be moved more than every 8 hours.

Her MAP was increased to 17 and she recovered slowly. Saturday morning it was reduced to 16 again and she seemed to be maintaining her O2 levels. Today she is up a little bit again but I am hoping this is just due to agitation. She is quickly annoyed when she wakes up and notices the ET tube. It will SUCH a blessing when that can be replaced with a trach.

Tummy wise she is doing well. She continues to impress us with her dirty diapers. Seeing as she has not eaten in weeks it is a wonder where she was hiding it all. The standard protocol is to wait ten days following the first good X-ray before starting feeding. She is on day 6 or 7 so if all continues to go well she will start feeds later this week.

We have reached a time where even more patience is needed. The doctors do not say "when" she gets off the oscillator but "if." Living in this limbo is draining in ways I can not explain. We have not been able to hold Natalie in weeks and I am really feeling the loss of all the normal mothering I would be doing right now. I just want my baby.

I am trying to find new ways to approach this wait and new ways to manage the stress. If anyone has some good advice for living life in a prolonged state of survival mode I am all ears.

Annie is doing well. She started preschool last week and loves it. I am grateful for her to have a place to go that is just for her. There are two little girls coming to sibling watch at the NICU now and she is really starting to open up around other little children. It is so sweet to watch her play and make tentative steps toward friendship.

Thank you again for your continued support. We certainly can use all the good thoughts and prayers we can get.

News news and more news

Friday we had a bit of a rough day. Natalie had some bilious secretions come out of her stomach. It is so hard to know when something is a minor blip or the start of something significant. In addition to that the plastic surgery team had to be consulted for Natalie's pressure sore and the whole situation broke my heart. Friday was just one of those days when I could not hold up any longer and had to break down. There was a great deal of crying and exhaustion. The acting attending was very kind and sat with me for a long time, even risking me getting tears and whatnot on her blouse. I think that kind of compassion is the mark of a great physician and I am so grateful that Natalie was able to be a part of Dr. Riccio's training.

Since then things have picked up a bit. We have had several days of small bumps (such as her erratic CO2 levels) but many improvements that override the negative.

As for her tummy, all her drainage tubes are now out and the openings are healing nicely. When we arrived Sunday she was fast asleep on her stomach. This is her favorite position and she hasn't been on her stomach since the NEC was first diagnosed. Her replogle tube (this runs down into her stomach to suction out the contents and air) was turned from suction to gravity. On Monday the replogle was replaced with an NG tube but not much has been coming out. If her abdominal u/s tomorrow looks good she may start feeds again, but it will be a very slow process. It may be weeks before we know if her intestines are truly healthy. Strictures can form over a long period of time and we will not know unless she starts showing symptoms.

The big news on Sunday was that Natalie made two massive poopy diapers. I practically jumped up and down when the nurse told us. I saw the stool and am very glad that she cleared it-I have never seen anything like that come out of a baby and I can not imagine it made her intestines feel so great. The stools still tested positive for blood but it has been in there since the NEC so that is to be expected. She had one more stool today while I was visiting. SO thrilling to see her body doing what it should!!!

The other big news is her MAP (mean airway pressure). So when she had the "really bad day" and was put back on iNO her MAP jumped from 20 to 22. There it sat for the longest time. It is now an incredible 16!!!!!! Just from yesterday it went from 19 to 16. That is craziness. She seems to be tolerating the decreased MAP well. Her O2 requirements are in the 50s, which is not great but it also has not gotten any worse with the vent changes.

The new attending (Dr. Pryhuber, back again after only 3 weeks off) seems determined to get her back on the conventional vent ASAP. That means many things. including being able to hold her again. I can not find words for what it will mean to have her in my arms again. Ahhhhhhhhhhh.........

The great progress has brought great anxiety. Today was her last day of antibiotics for the NEC and I am bracing myself for another infection. It seems I do not know how to cope on good days-I am always waiting for the next hit to strike. At the same time, we are celebrating the lower MAP and the poopy messes all the same.

Anyone interested in passing along the "Are You My Mother?" story is welcome to. Natalie has really been enjoying her books on tape and listens several times a day.

I know there is probably more to report and I have a lot of pictures to post but I am about as tired as I can be. Thank you all for your support and kind words. It is such a boost to our spirits!

Finally some pictures!

Pictures in no sort of order whatsoever. You can see in some that Natalie was not feeling well.
The lighting is not great in most of our pictures because we try to keep her room dim.

Most recent picture of Natalie....she looks much healthier now that her abdomen is better. This is her "upside down diaper look." It is much easier to change her this way than flip her.


Annie's 3rd visit with her little sister. She liked touching her head but was not ready to kiss her (she has me kiss Natalie for her). She pointed at the milk in the syringe and asked where Natalie's intestines were. I told her and she asked if the milk went to her intestines. Little did we know that her intestines were about to become a very big deal in just a few hours.

Natalie the night she was diagnosed with NEC. In retrospect it is obvious that something was going on with her abdomen (this is the same night as Annie's visit).

Her diaper over the head look. She likes to be protected from light.

SO glad that she has a central line and is no longer sporting multiple peripheral IVs. She was incredibly alert for someone recovering from surgery and on a great deal of sedation.

Natter's crying face-she has very expressive eyebrows.

Natalie post surgery with her bandages. That beast with the numbers is the oscillator and the iNO tank/dispenser is behind it.

Natalie enjoying her books on tape...love that bald head! This is from Friday and you can see she is already doing so much better.


Annie's first day of preschool was today. She is growing up way too fast.

Tooting

Wednesday was another care team meeting. The surgeon came in briefly to describe his plan for Natalie's belly recovery. He started backing out some of the tubes. When they are no longer draining fluid the tubes will be removed and ideally the incisions will heal on their own without stitches. She will remain on the antibiotics until at least 24 hours after the tubes are removed.

After that, they will watch for signs that her stomach is ready for food again. The suction on her stomach (the tube that runs from her belly and out her nose) will be moved from suction to just gravity. If she tolerates that the tube will be removed for some time and we will see how she does. At some point someone will have the guts to try feeding her again. The process will be veeeeery slow. There is a chance that she has developed strictures or blockages from the NEC and we will not know until feeding is resumed. In the meantime, we wait for signs that her belly is waking up. Ideally she will grace us with a bowel movement but that may not happen until she is eating again. Another complication is that she remains on high levels of sedation and that slows the intestines. Hopefully she will continue to need less and less medication to be comfortable.

She has, however, passed gas three times in the past two days (well, three times that I was present for). I take that as a very good sign that her belly is starting to move again. I have never been so happy to hear a toot in all my life!

The rest of the care team meeting was run of the mill. In general people feel she is recovering from the NEC much better than anticipated (although I hesitate to put that in print and tempt fate). Basically from here we wait and see what Natalie does and then respond. One doctor commented that she is "writing her own book." No one dares predict what the child will do. They did spend a few minutes thinking of clever adjectives for her.

All the necessary paperwork is compiled for the second opinion. The doctor at CHOP should receive it early next week. Praying and hoping that he has some insight into our little girl.

After the meeting we returned to her room to find her very uncomfortable. She was requiring A LOT of suctioning and was miserable. Her CO2 had been high again all day for no good reason. That is such a scary feeling.

We left the hospital feeling terrible. Sometimes it is just a matter of getting through the night and hoping the morning brings better news or a new attitude. I kept reminding myself that we have had terrifying times before and come through to the other side.

Today was, in fact, better. Her morning CO2 was normal and she tolerated another MAP wean (now down from 22 to 20!). I spent the afternoon with her and enjoyed a lot of awake time. Her O2 requirements were better than they had been this week (as low as 38%) and she was much more comfortable than the previous night.

I was able to help with weighing her and changing her linen (always nice to have a fresh bed). While we were getting ready I noticed a dark spot on the back of her head. I looked closer and to my horror discovered a pressure sore the size of a dime. It looks terrible. She is very limited in positions so I understand that she is vulnerable to sores but I do not understand how one could go unnoticed this long. It is being treated with ointment and I asked the occupational therapist to come in a take a look at her positioning. She is supposed to have a gel pad under her head but somehow it keeps disappearing. Yet one more reason why I can not wait to have her receive her trach and be able to move more like a normal baby.

In other news she had a double lumen PICC line placed Wednesday so she no longer needs peripheral IVs. Hallelujah! Once again it required a cut down. The area above the incision is red and swollen so she is on a 3 day course of...wait for it....vancomycin to ward of infection. I think this may be her 10th course of vanco. It is incredible.

I finally was able to record the books for her tonight. When she developed NEC it became a low priority and she was honestly too drugged to know what was going on around her. I am glad to finally have it done so hopefully she will be more familiar with my voice.

I reworked some of the stories to fit her circumstances. Baby books are not written with critically ill babies in mind and I felt she should have some stories that fit her and her world.

I retold P.D. Eastman's Are You My Mother? with Natalie in mind. I am typing it here so I can remember it for her later. Humor me.

A mommy sat by her baby’s crib. The baby slept. “I must pump some milk for my baby girl to eat!” she said. So away she went.
Inside the crib, the baby woke. “Where is my mother?” she said. She did not see her anywhere.
She moved her arms and kicked her legs. The baby girl could not cry, but she could watch patiently.

“Now I will look around and find my mother,” she said.

“Are you my mother?” the baby girl asked a big machine. The oscillator just tissed and tissed. It did not say a thing.

“Are you my mother?” the baby girl asked a nurse.
“No, said the nurse, "but I will take very good care of you.”

“Are you my mother?” the baby girl asked a doctor.
“I am not your mother. I am a neonatologist. I will do what is best for you.”

“Are you my mother?” the baby girl asked a respiratory therapist. "How could I be your mother?" said the therapist. "I am a man!"

The baby girl stopped to think. The oscillator and the nurse were not her mother. The doctor and the respiratory therapist were not her mother.

“I have a mother,” said the baby girl. “I know I do. I will find her. I will. I will!”

Just then the baby girl saw a big thing. “YOU are my mother!” she said. The big thing just said “Stand back, X-RAY!”

“Oh no!” said the baby girl. “You are not my mother. You are a scary X-Ray machine. The machine took a picture of the baby’s miraculous lungs and then left the room.

Then something happened! The glass door slid open and the mother came back to the baby’s crib.

“I know who YOU are,” said the baby girl. “You are not an oscillator or a nurse or a neonatologist. You are not a respiratory therapist or an X-Ray machine.”
“You are a Mommy and you are MY mother.”

The mommy kissed the baby’s cute bald head and they were both very happy.

ps I gave up on editing this post. It is what it is.

Wednesday Again

Saturday night Natalie's O2 requirements climbed overnight. She went from consistently being in the low 30's back to the 50's. Since Saturday she has been in the mid 40's to 60's. It was a big disappointment but I am reminding myself of how much higher those numbers can be. Sunday her CO2 was crazy. We got the morning update and I immediately dressed and went to the hospital. Her ET tube was 11 days old and I knew it needed changing. I was not leaving the hospital until it was done. Her tube was changed, it was "gunky" and the CO2 came right back down. Unfortunately this was the first time there was difficulty reintubating her. Just one more reason for us to anxiously await the day she can have a trach.

There is no explanation for why her Os were so good when her stomach was so bad. One doctor had a theory that while her body was mounting its response to the NEC her adrenal glands released high levels of natural steroids and the steroids helped her lungs as steroids will do. It's a theory but not one that can be proven and it also does no mean she should go back on steroids. Steroids leave her vulnerable to infection and can actually slow lung growth.

Her perfusion has improved over the past few days. I was feeling frustrated by her getting lasix, having poor perfusion and then needing a bolus of fluid to improve things. What exactly is the point of chasing fluid out only to replace it a few hours later? Eventually things were reevaluated and her perfusion was checked BEFORE she received lasix. She missed a few doses and did not need the boluses. Yeah for logic!

Her drains are letting out some yellowish fluid. It does not look like pus or anything infection related. The surgeons are happy with how her belly is soft and the redness has cleared up.

Well, you know I have been anxiously awaiting the MAP being turned down. This has been postponed every day for nearly 2 weeks. Natalie is just not going to give a perfect day for weaning.

Yesterday she had another cut down to place a new central line. During all the X-rays to check line placement they felt her lungs looked very expanded and agreed to try for a one point reduction in MAP. She seems to have tolerated it well.

Today will be a chest X-ray and belly X-ray. Hopefully all looks great and we can get some reassuring news.

Sorry this is so choppy. Mostly she is stable and we are waiting to see what her belly does. The surgeon is mostly concerned with her clinical signs and feels the next big step will be a bowel movement. That can take up to another week or more, especially with all the sedation she is on. I swear when she does dirty a diaper we may go out for dinner to celebrate.

As for her respiratory status, that too is wait and see. They are holding nitric oxide weanings for the time being. Hopefully she can manage a few more weans in MAP. Her CO2s have been all over the place but nothing crazy.

In other news, Natalie has lost her hair and now has a big, beautiful and lumpy head. The lumps are unavoidable because of being in bed all the time but I have promised her the reshaping helmets even if we have to take out a loan. I will not make her go through life with a wonky head. At any rate, the bald look is pretty darn cute and I love to rub her head.

Friday Night Update

Last night was a whirlwind. Once the decision was made to operate Natalie's little room had to be turned into an OR. There is barely enough space for her crib, oscillator and iNO tank. Luckily her room is adjoined to another room by a glass partition that was opened up after much discussion and maneuvering of equipment. The OR staff had to be called in from home. I give them a lot of credit-they are only allowed 30 minutes to make it to the hospital. Everyone was very kind and supportive. As a side note, one of the doctors is married to someone who was in the OR when Natalie was born. I am still being introduced to people who shared in her birth and were amazed by how well she did.

We spent the night in one of the family rooms. I have to say that this NICU experience has given me an appreciation for the smallest things. Last night is the first night I slept under the same roof as Natalie since I was discharged. What a comforting feeling to know she was just down the hall and I could check her with my own eyes at any time. These little things are so big when you can not take them for granted.

This morning the surgeon seemed pleased. The redness on her belly had dissipated and her vitals were stable. Her white count had gone down a little and there were no more bands. The platelet count was comparable to yesterday's numbers. Her blood gases were some of the best she's had. They had gone down on one of the vent settings (the power, which correlates to CO2 levels) but it looks like they may have overshot the mark again. Her evening CO2 was up significantly from the afternoon. I am now trying to not read into this but it is so hard to not get wrapped up in each little detail.

We are now in a place of waiting. The surgeon says it will be a long process. So far her drainage has stopped. That is a good sign. She has a tube in her stomach for suction and the fluid amount and quality is improving. Her O2 requirements remain good. Today she was at a whopping 30% most of the day. How I wish we could sit back and delight in her respiratory progress without this new fear. Her doctor said today that if it were not for her belly issues she would have gone down on her MAP. SO frustrating to lose that opportunity.

It seems the plan is to constantly monitor her clinical signs and take daily abdominal X-rays. The surgeon may replace the drainage tubes with smaller ones next week but it all depends on how she is doing.

She has needed two boluses of fluid to improve her perfusion but her blood pressure remains stable. She is also on a great deal of pain meds to keep her comfortable. Luckily she has a urinary catheter so she does not have to be manipulated for diaper changes.

Everyone is still very surprised that Natalie developed NEC at such an advanced gestational age. It happens, but it is not common.

Interestingly enough, our NICU seems to be encountering an outbreak of NEC cases. They went from a long stretch without any and have now had 6 in a short period of time. They are working with the infectious disease department to try to find a common link. So far there has not been a common factor among the babies. I asked and Natalie is the 4th or 5th case among the 6. While they are trying to determine what is going on all the NEC babies are on "contact precautions" and the nurses have to gown up and wear gloves when entering her room. She will not be released from this until she has a clear stool culture. That should be interesting as we have no idea when she might stool again.

I had felt pretty optimistic today. People kept coming up to me to say they were sorry but really I felt confident for no reason I could pinpoint. Now I am just feeling tired. The conversations and decisions last night are still haunting my mind. I so wish I could turn off my head and have about 20 hours of dreamless sleep. It is scary to have one more thing to watch, one more thing to be afraid of. I am very grateful to Dr. Lee for his quick response last night and for all the people who came together to make her surgery possible. I hate to think of what today would have been had they waited. I am also grateful that she tolerated the surgery so well and that she has remained stable. Many prayers that she remains that way and her belly can do all the healing it needs.

NEC Update

I will try to share as much information as quickly as I can but we are headed back to the hospital to spend the night so forgive me if I am not clear.

Natalie had one of her best days respiratory wise. Her O2 requirements were as low as 28% (room air is 21%). Her chest X-Ray is one of the best I have seen. I told Steve I wanted to take a picture of it and hang it on the fridge I was so impressed.

So, on the day she seemed to make much headway with her lungs, things fell apart from the diaphragm down.

When I arrived at the hospital she had areas of redness on her abdomen. This indicated an inflammatory process was certainly going on. Her CRP that had been all the way down to 15 recently shot up to 196. Her body was clearly reacting to something. On the upside her white cell and platelet counts remained stable and normally it severe NEC those numbers tend to plummet. Her blood pressure also remained stable, another positive.

I was told her 12 o'clock belly film looked no worse and that her other clinical signs were good with the exception of the redness on her abdomen. Their best guess was she would sort it out on her own within a day or two.

Enter Dr. Lee, the pediatric surgeon who stopped by in the early evening. He was not happy with her films and was very concerned about the redness. I called Steve to meet me at the hospital and we were presented our options.

1. Full exploratory surgery to look for areas of bowel that have died. This is what they would normally do but all doctors involved felt it would be too much for Natalie to tolerate the recovery from the surgery. It involves an incision all the way across the abdomen and is a major ordeal.

2. Surgically place drains to allow bacteria to exit her peritoneal space. This reduces the bacterial load and makes it easier for the antibiotics and her immune system to control the infection.

3. Do nothing and see what happens.

We opted for the surgical drains. It was a much lower risk from a surgical stand point, but we are taking a risk that large portions of her bowel need to be removed and we will not know.

She had the surgery right in her room and tolerated it very well. The surgeon removed some cloudy fluid that indicates there is an infection present. The good news is that there was no evidence of blood or stool, so her bowel has not perforated.

She is on major antibiotics to kill all of her intestinal flora. They are culturing the drainage fluid from the surgery so possibly they will be able to better target the antibiotics.

I will do my best to keep everyone updated. The next 24-48 hours are very crucial in letting us know if the surgery will be of help to her.


Thank you in advance for your good thoughts and prayers.

Thursday morning

Yesterday started out with fantastic news. Natalie's O2 requirement was all the way down to 34%. When I got off the phone we both just stared at each other repeating "34???" Natalie has not had such low O2 settings since she was about 3 weeks old.

When we arrived at the hospital in the afternoon her nurse met us outside the isolation room. She noticed Natalie's heart rate was higher than normal and her temp was up. She had just come off her antibiotic the night before. A CBC (blood count) and CRP was ordered. Her white count was actually the lowest it has ever been but the CRP was back up.

Annie came in to see Natalie and they had a great visit. We brought the art work big sister had made to hang on the wall. She had a lot more questions this time about the medical equipment but overall just thought her sister was "so little and cute."

I had been tense all day despite the great O2 numbers. There was no reason to be worried but I couldn't shake it. Late evening the phone rang and it was U of R. We were both taken aback-we had just let and things were stable.

Natalie had a bloody stool. Her belly was looking distended. An X-ray was ordered and she had classic finding of NEC (necrotizing enterocolitis). You can read more about it here http://kidshealth.org/parent/medical/digestive/nec.html

It is unusual for a baby Natalie's age to develop NEC. It was a complication I had really written off at this point so the call was a major shock. I think it may be related to her recent changes in food and calorie concentration.

She will continue to have belly X-rays every 4-8 hours to watch the progress of the NEC and look for possible bowel obstructions. They have started 3 new antibiotics to kill off her intestinal flora. Her repeat CBC early this morning is showing a response to infection. Her white count doubled and she went from no bands (immature neutrophils) to 20 bands over the course of a few hours. Her body is clearly mounting a response to something.

We are understandably very upset. It is so disheartening to have several good days be taken back by a new complication.

I will do my best to keep more frequent updates over the next few days as we see how she responds to the NEC treatments.

I think I can, I think I can...

Today we purchased a digital recorder so we can tape ourselves reading and Natalie can listen at her leisure. I had a lot of fun tonight picking out her book selection and of course "The Little Engine That Could" is at the top of the list.

I think I have mentioned before that nutrition is key to treating BPD. Babies with BPD expend a lot of energy breathing and in general have growth issues. Growing new lung tissue is the only way to improve her respiratory status so it is crucial she gets the nutrition needed for growth. This is further complicated by the fact that BPD babies tend to be fluid sensitive, meaning the more fluid going into her body the more that tends to end up in her lungs worsening the pulmonary edema. She is fluid restricted to help control this and on powerful diuretics to reduce the fluid in her lungs. This then creates issues with electrolyte balance, so she receives many supplements to replace what is peed out. Natalie wears diapers a few sizes too big because appropriately sized diapers could never hold her urine output. We like to say that Natalie loves her lasix.

Anyway, to maximize her nutrition while limiting her fluid intake they have to be creative. Her usual mix was 2 parts 30 calorie liquid formula to 1 part breast milk plus 4 packs of human milk fortifier. This was a whopping 28 calories an ounce (normal breast milk is estimated to be around 21 cal/ounce). What a contrast to feeding Anneliese who had nothing but my milk until she was 6 months old.

Late last week they revisited her nutrition and changed her to breast milk with some high octane powder mixed in to bump calories. She will be getting much more of my milk as it is no longer diluted with the liquid formula. Now Natters receives the usual benefits of human milk in addition to something about the osmolarity of human milk vs formula in regards to her fluid balance, but I will spare you the explanation. It is so nice to have a concrete thing I can do to provide for her and gives renewed purpose to my hours pumping.

The new mixture brings her to 30 cal/ounce. Unless they decide to add vegetable oil (no joke) this is the maximum amount of calories they will try to achieve. The only thing left to do is go up in volume.

After the feeding change Natter's girth increased. She has always tolerated her feeds well so we were a bit alarmed. She was still having BM's and the abdominal X-ray looked normal so the feedings continued as planned. During my visit Saturday it was obvious that she was uncomfortable. She would bring up her legs, turn dark red and desat down into the 50's. Oh, how I miss the days when an uncomfortable baby meant loud crying and sleepless nights, not incredibly poor oxygenation. The sweet baby. There was nothing we could do to help.

Sunday she pooped like it was her job and filled at least 5 diapers in addition to soiling her bedding. We were VERY proud parents and all parties involved were relieved. Since then she has looked more and more comfortable every day. Thank God we can still say she is tolerating her feeds well. That's the one thing we can always count on even during most bad days.

So much talk about feeding all to put off talking about today's news.

Natalie continues to be responding well to the iNO. As a matter of fact, when she had such a dramatic response it raised a few eyebrows, including my own. I was afraid that it indicated a return of pulmonary hypertension. Babies with severe BPD are at a high risk for developing PH. At the last care meeting I asked how often she would be screened (by echocardiogram) and they said every 4-6 weeks.

It is very fortuitous that they agreed to the iNO when they did and that her response to it elicited an echocardiogram. She was not due to be rechecked for at least 2 more weeks. Her heart is in fact showing changes that indicate PH. The echo was yesterday and the unofficial word was that she was in the clear. Now I know to not celebrate until the official word is in. The good news is that it was caught and she is receiving appropriate treatment. The changes to her heart should not cause permanent damage. We could easily have gone another few weeks NOT treating the PH and been facing life threatening heart issues. Still, PH is life threatening and a complication we were very much hoping to avoid.

The last two days have been stable and we are VERY grateful. She tolerated the first wean of iNO (from 20ppm to 16ppm) beautifully. In fact the echo was done during the wean so they could watch for changes in her heart and the wean did not make the PH visibly worse. I hesitate to put it in print and jinx ourselves, but since last night she has been in the 50's and low 60's for O2. She has been handling her cares (diapers, position change etc) without needing major increases in oxygen support. All good.

The have yet to reduce her MAP and boy am I struggling with that. I am hoping that tomorrow she continues to be stable and they take the chance. I want that number down, and while I am prepared for patience I really don't see what they are waiting for.

Her CRP (marker of inflammation) is down to 15, which is wonderful. They want to see it below 10 and she is well on her way. Her antibiotic and antifungal meds are done this week. They may remove her PSC line, which reduces a potential route for new infection AND reduces her fluid intake by not needing constant line flushes. She will have a follow-up kidney ultrasound to look for evidence of fungal infection and another urine culture at the end of the week.

In general she seems to be resting much more comfortably. Yesterday little noises were upsetting to her but today she seemed to enjoy conversation. Her nurse was very amused that Natalie was eavesdropping on talk in the hallway and became agitated when the door was closed. She opened it back up and Natalie happily returned to looking around and listening.

As you know Annie has only met Natalie once, and that visit had to be advocated by the child life specialist. Now that we know we are in for an extended stay I became very bothered by the idea that the two would not meet again until Annie turns 3 in September. Our time with Natalie is not guaranteed and I do not want Anneliese deprived of knowing her sister because of a hospital policy. I made my case and we have been granted an exception. As long as she is healthy Annie can now make brief visits every other week. My hope is that if all goes well visits can be increased to weekly but I am not pushing my luck. Oh, and if you happen to know anyone at the hospital we are not supposed to advertise that she is under 3 since they don't want to set a precedent. I am trusting the readers of this blog to not blow us in to other NICU parents or Strong staff members.

When I tucked her in tonight I told Annie she would see Natalie tomorrow and she perked up immediately. She then composed a list of all her toys she wants to bring in to show baby sister. Such a sweetie. I am thrilled at the prospect of being in the same room with both my daughters again.

We are "holding up." I lack better words for it. We are functioning the way you might expect people under extreme stress to function. Today NY had 90 degree weather. We came back from a full day out and about to find the air conditioning on full blast and the back door unlocked (very wise in our neighborhood). The door blew open so we spent who knows how many hours today trying to chill the backyard. We were then home for a half hour before we realized we left all the groceries in the car.....in the heat. I consider it an accomplishment that we even made it to to the grocery store. We have been surviving on a great deal of restaurant food because by the end of the day neither of us has the brainpower left to plan and cook dinner. Well, assuming one of us was clever enough to even purchase food to cook.

It is certainly the biggest challenge we have faced as a couple but we are figuring it out as we go. I take a lot of inspiration from Ms. Natalie Evelyn. She has been a relentless warrior-working hard for every single breath she has taken yet fighting on. If such a little person can display so much fortitude certainly her parents can follow her lead.

(I would like to take a second and thank you for overlooking typos, misspellings, repetitions and anything that may just sound wonky. I am normally diligent about editing but my head is pretty foggy these days.)

Another Monday Morning

I know it has been a long time since I have posted. I keep waiting to feel like we are on some sort of solid ground so I know what update to write. As that point seems a ways off I will just let you know where we are now.

At the care team meeting her doctors agreed to another trial of nitric oxide despite her not having a current diagnosis of pulmonary hypertension. I have spent the past month or so approaching every new attending with the research I found on using nitric to treat BPD. When the new attending came on last weekend it was only hours before I asked to sit down with him. He smiled because he already knew what was coming. Well, finally there was agreement and a plan was set for the upcoming week when she was further along in her antibiotic course.

That was last Wednesday. Thursday I arrived at the hospital and Natalie was not doing well. There was the normal work-up of a chest X-ray and blood gases. Her high CO2 was easily remedied with an ET change. In under a week she had managed to block another tube with secretions. Her oxygenation however was not improving. For the first time since being put on the oscillator her MAP (mean airway pressure) had to be increased. I can not tell you how awful that felt. Her lungs requiring more pressure to open means they have become more fibrous and less compliant. The higher MAP also works against her lungs forming healthy tissue.

Steve joined me at the hospital and we spent a long afternoon discussing what this progression of her lung disease meant. I know we are not the only parents to face difficult medical decisions for children but it is a very small consolation.

We were determined to give the nitric a chance and resolved to ask the doctors to move ahead with administering it now rather than waiting a week. We were actually on the same page with the doctors. When we returned from our walk the acting attending was there to say that she felt they had done all the usual things to improve her oxygenation and everything failed. It was time to give the iNO a chance.

My sweet niece was with us last week to help with Annie (thank you again Kate!). We went home to have dinner with her and Anneliese. It is funny how on the inside my heart can be absolutely wrenched but on the outside I still need to perform normal functions. I still marvel whenever I manage a normal conversation or do something ordinary like grocery shopping. My mind is in this constant crisis mode but you can not present yourself that way to the world. People would stop coming near you.

Waiting to see if she would respond to the iNO were some of the longest hours of my life. If it did not work we were reaching the end of the treatment options. In fact most hospitals would not have even allowed the trial of iNO and would have said we were already at the end.

Steve stayed home with Annie and Kate so I made the return trip to the hospital alone. I decided against calling because I did not want to hear over the phone. I gathered up my courage, walked into her room and saw her O2 requirement was 48% and her saturation was 93%. For the past 24 hours she had been struggling to saturate in the 80's at 90-100% O2. I looked at the number, pointed and looked at the nurse. She nodded and smiled. The iNO was started at 5:30PM and her O2 settings went down about 20% an hour until reaching the mid 40's.

I called Steve, told him the news then went to stand by my baby girl. She held my finger and I alternated between staring at her O2 monitor on my left, her perfect face and then her pulse ox display. What an amazing feeling. I never wanted to leave. It seemed if I stayed right there things would remain this good and only get better.

With great progress comes great anxiety. We felt as celebratory as we did nauseated and could barely sleep. Her O2 requirement actually made its way all the way down to 40% in the morning. sigh.

Now if this were a movie it would be clear sailing from this point on. I wish I could say that is the case.

The iNO has made a significant difference in her oxygenation, but it is not the magic bullet we were hoping for. She did not last long in the 40's and her new baseline seems to be in the 50-60's range. She still has times at higher requirements and they have not yet been able to lower her MAP. This is crucial for her recovery-we need to find a balance of lower pressures and lower O2 exposure for her lungs to develop healthy tissue.

This has been a particularly difficult time for us as we have had to confront the reality of Natalie's lung disease. As a mother this week has felt like being continually kicked in the chest. I want very much to just hold my sweet baby and comfort her. Instead we continue our patient vigil next to her bed hoping that somehow things will turn around and her lungs will make progress in the right direction.

Last night we had a quick visit and Natalie was wide awake looking around. We talked, changed her diaper, took her temp and enjoyed seeing her bright eyes. Before we left I ready her Everywhere Babies ( a personal favorite) and Goodnight Moon. She relaxed and fell asleep. A normal bed time were it not for the oscillating vent, the tanks of iNO, the nurse checking vital signs and our inability to hold her for even a moment. This is not how I planned to mother her, it is not how I want to mother her, but we will enjoy every bit that we can amidst the constant fear and medical atmosphere of the NICU. Natalie is our treasure and we are so lucky she is ours.