Making plans

Nora loves hanging out with her big sisters!

I feel like I need to write a disclaimer-I don't really know what I'm talking about. If you're reading something and think, "Ummm, that's not correct, she's crazy" you're probably right. It's dangerous letting a sleep-deprived lunatic try to relay information, but I'll try my best:) I try to keep things short as possible, but then sometimes leave out important information. But then sometimes I ramble on (like now:) and don't make any sense either...feel free to ask questions or correct me. 

Thank you so much to all of you that have been praying for Nora and our family. We are so grateful!  

When we met with the vascular anomalies team last week, we were hoping to walk away with a clear plan. Instead, we left more discouraged than anything. A couple of the doctors were really not comfortable with trying the diet. Their reservations made us question things, even though we had felt very comfortable and optimistic about it going it to the meeting. We still didn't feel right about trying the Sirolimus, so we asked the doctors if they would be willing to talk to the Dr. in Seattle, hoping that would put their minds at ease. They said they would, but last we heard, they were still trying to get in contact with Seattle. 

Yesterday, we went to a conference for families of kids with PHACE syndrome. There was a photographer, Rick Guidotti, who spoke about his organization, Positive Exposure. One of our Drs wanted us to hear him and have him take Nora's picture. It was nice meeting him and hearing about his organization. (Here is a link if you want to check him out-he used to be a photographer for some high-profile clients, but now runs not-for-profit organization that wants to "transform public perceptions of people living with genetic, physical and behavioral differences – from albinism to autism" <http://positiveexposure.org/> ) While we were there, a mom introduced herself and said she was from Seattle. I told her we had been talking with Dr. Perkins in Seattle, and she said her daughter sees him too! She had great things to say about him, which was very reassuring to hear. I love how God arranges these 'random' meetings at just the right time:) After talking with her, I walked back to Evan and found him talking with one of Nora's doctors. She was saying they had been in contact with Dr. Perkins. They are still waiting to talk to one more doctor, but she said she thinks we should start making plans to go to Seattle. I was not expecting her to be so on board with it! 

That was the confirmation we needed. So, as of now, we are going to move ahead with trying the diet. We feel a total peace from God about the decision. So...now we need to start figuring things out. My head is kind of spinning, thinking about all things we need to do. First I need to talk with Seattle and see if they are still up for trying it! If so, we are hoping to start it soon. 

For those of you that are praying for Nora, please pray for wisdom as we proceed, and that things would come together nicely.  We are asking that God will continue to provide and open doors if this is His will, and if it's not, that He would make it clear. 

Nora's tongue seemed to be a little better for a couple days, but it is currently as swollen as ever and still bleeding off and on. Please continue to pray for healing for that. Please also continue to pray that she stays free from infection/sickness. The rest of us are feeling a little under the weather, and it's no small task keeping her adoring big sisters off of her! 

Besides her tongue, Nora has been doing well. Her little smile never fails to melt my heart! Lina says Nora is "shining" when she smiles:) Nora is moving faster, sitting up longer, and getting stronger every day-her therapists said they are amazed at how well she is doing. We are so grateful for Nora's progress. Psalm 105 says, "Give thanks to the Lord, call on his name! Make known his accomplishments among the nations and tell about his miraculous deeds!...Seek the Lord and the strength he gives. Seek His presence continually" I think what God has done with Nora is pretty miraculous, and I don't know what I would do without His strength!
Thank you again for praying for Nora and being a part of this journey with us!

Nora maxing out her leash:)

6 months

It's only taken me six months to get this up-hopefully it won't be that long before the next update:)
I intend to eventually start at the beginning, but I am going to start at the present because it has taken me 3 days just to write this:)
It is really helpful for me to read the blogs of others that are going through similar situations. I have been in contact with a couple other families who are further along in their journey, and it has been so encouraging. If someone dealing with these issues happens to be reading this, we would love to hear from you!
I'm assuming most people that will read this already know what has been going on, but just in case, a quick back story...
Sweet little Nora was born 11/19/13 with a rare lymphatic malformation. She has cysts inside the mass on her face, in her tongue, on her lungs and around her airway. Because of all that fun stuff she has a trach and a G-tube. We didn't know if she would survive delivery, but God is good, and (obviously;) she did! After 6 weeks in the NICU she was able to come home. It has been an adjustment, to say the least, but I think we are finally getting settled into our new normal:)
Nora has been doing pretty good lately. She was in and out of the hospital the month of March and beginning of April, but has been home since Easter. Currently, we are dealing with her tongue being very swollen and bleeding frequently. She started an oral steroid a week ago (which usually takes care of her tongue issues) but we haven't noticed any change. We started an antibiotic today (in addition to the steroid) to see if that helps. It doesn't seem to hurt her, thankfully, but it's not nice to see my sweet little baby covered in blood! But she smiles through it all:)
Developmentally she is doing great. She is right where she is supposed to be on most things. She is starting to move now, which will be a challenge! She has about 4 feet of tubing keeping her tethered to an IV pole, which doesn't give her much wiggle room. Hopefully she will start tolerating the HME for longer periods of time so she can have a little more freedom.  (The HME (also called the artificial nose:) is a little thing that goes on the end of the trach that traps the moisture and allows her to be off the humidification, but it is more difficult to breath while it's on)
We have been talking with many different doctors as we try to decide the best treatment plan for Nora. We sent her information to doctors all over the country, and are in the process of reviewing their recommendations.
It doesn't seem like the cysts have responded to the sclerotherapy treatments (I think she has had 5 rounds of sclerotherapy? That is where they drain the cysts and then inject them with medication).
There is a drug called Sirolimus (also called Rapamycin) that has been used to treat lymphatic malformations in the past few years. It is usually used for organ transplant patients. Patients with LM's saw a lot of positive results from Sirolimus, and it seemed like that was the only other thing to try, besides surgery (she will definitely need surgery, but from what I understand, it would be more effective if the cysts could be scarred down before removal). We were reluctant to try Sirolimus because of Nora's age. They haven't used it on someone so young, and she wouldn't be able to communicate if she was experiencing side effects.
Despite our reservations, we were getting all set to start the Sirolimus when a Dr from Seattle called. He had good things to say about Sirolimus. He also told us about research that has given them reason to believe that a ketogenic diet could benefit LMs. (A ketogenic diet is most commonly used for patients with seizures)
We are still asking questions and doing research on the diet, but it seems like this may be something we would like to try. It has never been tried on someone with a LM before, so they have no idea if it would actually work. But, in our minds, we feel like even if it doesn't do anything for her, it wouldn't hurt anything to try it. And if it doesn't help, then we can try the Sirolimus.
We have an appointment next week Tuesday to meet with the vascular anomalies team at Children's. We are hoping to come up with a game plan at that time. It doesn't seem like many of the doctors here are too excited about trying the diet. They are concerned about her tongue, and the fact that she is so trach-dependent. I think they feel that since Sirolimus has been proven to help, we should start with that and not waste time. It will be good to hear everyone's thoughts and opinions and see what options we have.
If we do decide to do the diet, that presents some challenges as the Dr is in Seattle. It seems like we would have to go out there several times, and we would need to find doctors and dietitians here that would be willing to work with him long-distance. But we'll cross that bridge if we come to it (Matthew 6:34 Do not worry about tomorrow, each day has enough worries of it's own!)
We really appreciate all of you that have been so faithfully praying for Nora and our family-we can definitely feel your prayers! For those of you that have been asking how to pray specifically, if you would, please pray for wisdom for the doctors and for us as we decide what would be best for Nora.  It is a little overwhelming. No one knows for sure what to do-it's everyone's best guess. But we know God will give us a peace about which choice we should make.  You could also pray that her tongue issues would resolve so we can start working on oral feeding. Lastly, please pray that she continues to stay free from infection!
We are so grateful for how well Nora is doing despite everything. She is a strong little girl! God has already brought her so far, and we are excited to see what He has in store for our little light:)