Tuesday, August 3, 2010

A Hundred Days of NICU...hoping for no end in sight.

I have been trying to clarify things in my head so that I know how to update everyone on Natalie. I will do my best to share but need to simplify some of the medical decisions or risk a two hour post.

Thursday the neonatologist did meet with the pulmonologist. She also received an initial report from the doctor at CHOP who is reviewing Natalie's case. The three key issues she is facing are slight pulmonary hypoplasia (from the low amniotic fluid), bronchopulmonary dysplasia and now pulmonary hypertension due to the BPD.

Friday we had a very late meeting with the outgoing neonatologist and the incoming one. At this point Natalie had several days of increased oxygen (up to the 80's) for no known reason. Unlike earlier in the week there were no mechanical explanations for her to be requiring more O2.

We were given several options. The first option was to do nothing and allow Natalie to stay on the oscillating vent. There is a chance that over time her vent settings could be gradually reduced to the point that a switch to the conventional vent would be more successful. The downside is this prolongs her time being heavily sedated and she is basically attached to the bed. Developmentally we have no way of knowing how this is impacting her. In addition to all that, as long as her O2 requirements are high her lungs are being damaged by exposure to high concentrations of O2 and the chances of them improving are greatly reduced.

The second option was to optimize her chances of a successful wean to the conventional by using steroids and some experimental medications. The main risk of the steroids is immunosuppression and her developing another infection (this is what happened last time on steroids). The risks of the experimental drugs are primarily low blood pressure ( to the extent that frequently the meds must be stopped before they reach therapeutic levels), but because these meds are not approved for use in babies the real risks are unknown.

The doctor from CHOP sent a list of meds he recommends. The main objective is of the drugs is to act as a vasodilator and hopefully improve her pulmonary hypertension. One of those drugs is Flolan, an IV med used to treat severe pulmonary hypertension. Our doctor is investigating the possibility of nebulizing the Flolan so it could be directly delivered to her lungs and hopefully avoid some of the systemic side effects. Flolan is a "big gun" and we do not want to use it if we can get away without it.

The idea presented was to use these meds to give Natalie the best chance of succeeding on the conventional vent, but they would also suggest we make a commitment to not return her to the oscillator if they could not get her to reasonable vent settings. We would then let the disease run its course and offer her comfort care.

That was an extremely difficult plan to take in. At what point do you wake up and say "Yes, today I take the chance and see if my child will live or die?" We have been facing life and death decisions for so long now, and we have known for some time that Natalie is critically ill. That in no way makes these conversations any easier. She is loved and as valued as any child of ours would be and we still intend on giving her the best chance at a good outcome.

As always, there are options somewhere in the middle and that is what we are trying to sort out now.

We asked for time to think things over while they investigated the drug options.

Meanwhile, on late Saturday to early Sunday Natalie's O2 needs improved significantly. She was once again in the 40's and sometimes amazing us with high 30's. We met with the doctor yesterday and tried to form a plan. At this point we are holding off on the Flolan but they are still investigating the best way to administer it. As long as her O2 requirements remain reasonable (below 60%) we are buying some time and letting her stay on the oscillator in the near future. In place of Flolan she will be started on sildenafil, or as you may better know it, Viagra. It too acts as a vasodilator and may improve her lung function. Animal studies also suggest it may help her grow new healthy alveoli and reduce some of the negative BPD changes. It takes several days to weeks to reach maximum effectiveness, while Flolan is a much faster acting drug (within hours of starting). However, it comes with a much lower set of side effects and can be given orally. It is also a med she can take long term and even come home on. No word on if it will help her throw footballs through tire swings as the Viagra commercials suggest, but time will tell.

Many of these medications are drugs that I had researched in the past and asked the doctors to consider. At the time I was told they rarely used them and when they did it was when their backs were up against a wall. Well, here we are, our backs against that wall and hoping for the very best outcome for Natalie.

We have yet another meeting tomorrow to discuss meds and plans. It gets very complicated and is not helped by the fact that the NICU doctors themselves do not know what direction we should go. There are not hundreds of Natalies all over the US who have been studied and understood. She is writing her own book and no one knows what she really needs at this point. Steve and I also have to reach the same conclusions regarding her care so that we are moving forward on the same page.

We are trying to weigh her long term outcome, ultimate quality of life and her immediate quality of life. We are researching medications that do not have proven track records in the NICU and doing our best to make decisions that seem impossible.

I remind myself that now I have endured more life and death discussions over Nat than I can recall, although they have all left marks on my heart. We have sat in that hospital holding hands and bracing for the worst. Each time she has amazed us. She fights, she holds on, she is trying to get to the other side of this. I have been told multiple times that when babies have had too much and are ready to go that they find ways of letting their parents know. I do not have that sense from her at all. I constantly evaluate the situation she is in, because I know it is a hard, uncomfortable and sometimes painful existence that she has right now. I am not oblivious to what we are asking of her. My litmus test is thinking "Would I do this to survive? Would I be willing to fight through knowing how wonderful life can be?" As long as I can still answer "yes" than we are moving forward. I can only hope that the choices we make are the choices Natalie would choose for herself if possible. I recently told Steve that if Natalie pulls through we owe her big time and need to be the best parents possible. For all that she has endured, for all that this has taught me I feel more charged than ever with the responsibility of parenting our girls.

Right now my mind is on a constant loop of Natalie's vital signs, her vent settings and the future plans. It is a maddening place to be and has taken a toll on us emotionally. I ask that in the immediate future we are given a break from discussing the finer details of what we are deciding regarding her medical care. It is too much emotionally to sort through all the information time and again. When there is a plan in place of course we will let everyone know as soon as possible.

For parents in a similar position, reading now or in the future, I am happy to answer any questions about the treatment options we have investigated. Please feel free to contact me and I will do what I can to share what we have learned. Through this journey I have had so much help and encouragement from other parents and I hope to provide the same assistance when possible. In addition, if you are reading this and have experience using Flolan, Viagra, inhaled iloprest or tracleer in the NICU setting I would love to hear from you.

OK, so on to lighter news. Natters did several days of pedialyte and proved her intestines were ready. On Saturday she was finally allowed breast milk again. I was thrilled to be there when the order was written so I could actually go pump fresh milk. Don't ask why I was so excited. Partly because fresh is better, partly because any little thing I can do for Natalie is a wonderful thing right now.

They have been increasing her 1cc every 12 hours and she is currently at 8cc. She has had several stools (one dirty diaper I had the privilege of changing today) so on the tummy front we are pleased.

In respiratory news they have weaned her power from 4.6 to 4.0. The power correlates to her "shake" and ventilating CO2. This was done gradually over the past few days and according to her blood gases she has tolerated it well. The neonatologist was encouraged by the ability to wean the power although she has a long way to go before the settings are at a point where she can be moved to the conventional vent.

Today I was there while they were retaping her ET tube and the lights were on full power. Usually the room is very dim as Natty prefers it that way. This was the first glimpse of her in bright light that I have had in some time and I noticed she is starting to grow hair again. My sweet bald baby won't be bald for long. I am anxious to see what color it comes in. So far it seems light, so maybe another blondie? Somehow I always imagined her as a brunette, but I think that is because all the famous Natalies I know have dark hair..Merchant, Portman, Woods. Well, maybe I can only think of 3 famous Natalies but at any rate the name always seemed like a dark haired girl to me. I will be thrilled with whatever color she grows and am just curious. Nice to be curious about something that is not of vital importance.

Here are some recent pictures. It never fails to amaze me how healthy she looks on the outside. I feel like I could unhook her, pick her up and walk out the door. She is right around 9lbs now and is about to outgrow her bed. She can't move up to the real crib until she is off the oscillator, though.

She really likes this sock and frequently holds onto it.


  1. I often think of your baby girl, she is such a fighter. I hope the DR find a way they want to treet her soon.

    Stay strong.

  2. what an incredible little girl, she will keep fighting this! You are an incredibly strong mummy and your knowledge on her condition is incredible - she is lucky to have you as you are to have her! x

  3. Natalie is an incredible girl, and is teaching you and everyone who reads your blog about the meaning of life and what a fighter she is. I replied to your post on the trach board, but I wanted to comment here, I have quite a bit of experience with bpd, and pulm hypertension, Viagra was our drug of choice for a very long time, feel free to email me sunriseinchaos@hotmail.com with any questions you might have.

    Prayers for you and your sweet baby girl,

  4. My prayers are with you and your sweet little girl. She is beautiful! Just wait and see all the wonderful things God is going to do! HUGS

  5. Prayers continued! Its so scary when you have so many things to consider. I will also prya fopr you and Steve,so that you can make and face these decisions. You guys are doing great so far and Im sure that wont change. Natalie is a lucky girl. If she is still on steroids..dont be surprised if she grows A LOT of hair! :)

  6. Natalie is such a beautiful baby. You constantly amaze with your research & decision making skills in this stressful time. As always, our prayers flow abundantly for Natalie, her doctors, nurses & of course, you guys.

    Much love--

  7. JT,

    I was encouraged by the news that she is allowed breast milk again. I know you are excited about that. It was also encouraging to see her shakes are down. And how wonderful her O2 suprised everyone by reaching the 30s in the last few days.

    Much love and hugs to you, Steve, Annie, and Natalie from Bethany, Chris, Christopher, and Katie.

  8. Sending you all of our best wishes, hopes and prayers... love you guys so, so much!

    Dana & the boys

  9. Prayers sent your way. We spent 94 days in the NICU last year....seemed like forever, but are reaping the rewards of prayers answered...a 18 month old that is tiny but all around healthy. Praying for all of you during this trying time and may Natalie amaze you the way Abby has amazed us! Hang in there!!!!!

  10. Hey, Jenn!

    Counting days with you. Cannot believe we just passed the 100-day mark. It seems like ages ago that we were PPROM buddies and that our kids have been NICU babies for an awfully long time.

    I'm sure that the pictures don't even begin to do Natalie justice - our NICU lighting isn't the greatest for pictures - and even still she truly is breathtaking! What a beautiful little girl you have on your hands! And what a sweet little fighter.

    My heart aches for the ride that you have been on. I wish there was a simple solution and I had it for you but you really do sound like you have done a lot of research and Natalie can only reap the rewards of having a mom so informed and on board and parents working together for her best interest.

    Always, always, always thinking of and praying for you all!