Saturday, December 31, 2011
Brenna's skin has continued to astonish us with how quickly it is "shedding" to reveal her less scaly pink skin underneath, and her eyes, nose and mouth look so much better too. She is eating so much now compared to even a few days ago (at this rate, she will surpass Connor's weight soon!)
The doctors were a bit worried with her kidney function yesterday, so they ran some tests and did an ultrasound on her belly this morning. The medications she is getting can affect the kidneys and her urine output was pretty high, but the tests all came back fine - yea!
So today, our doctor and nurse surprised us with the best gift ever: some cuddle time with our girl! I even got to try to nurse her...while she seemed interested, she conked out so quickly before she even got to try to eat. Maybe next time!
To keep her body temperature and her moisture up, they coated her with ointment and wrapped her in plastic, then wrapped the blanket around her. It worked quite well, and she was a pretty cozy little thing!
I didn't want to put her down. I thought my heart was going to explode out of my chest, and I got pretty emotional a few times.
But Daddy needed a turn too!
Not much can top that as a New Year's Eve celebration for us!
Friday, December 30, 2011
This is not my usual personality at all. I keep a very organized schedule and home, and I hate when plans change. I have expectations that I want to be met. I have “why me?” moments about bad hair days and ruining dinner because I didn’t know that al dente meant to actually cook the noodles first.
But never once have we asked “why me?” about Brenna. Not when she was born, not when she was diagnosed, not even when we thought we were going to have to say goodbye to her on Christmas Day. We know why, and God continues to verify this reason every single day. She gives me such a sense of purpose that I have never felt before.
When we were deciding what to name our blog, we wanted the name to encompass exactly how we felt about our situation…BLESSED is the first word that came to our heads.
Someone I know sent me a link to another mom’s blog after Brenna was born, where the woman had written about her child with special needs. She wrote:
“She is the perfect child, the child we wanted so badly. Even when society sees her as imperfect & pities us for they know no better - I pity them because they don't have a child like her. We are blessed!”
That is exactly how we are feeling about our Brenna :)
Thursday, December 29, 2011
Look past that cute new nametag and pink blanket...
Brenna was sporting a bow! :)
Nurse Mindy had fashioned an adorable bow for our girl using a piece of fabric from a newborn hat and some yarn. Now she is stylin'!
The doctors had taken out her feeding tube and stopped feeding her milk because your body helps fight off infection better if it's not trying to digest food as well.
Brenna's blood is still testing positive for the E. Coli, which does have us a little worried because it could be her central line (through her belly button) that is continuing to cause it, since the central line is very prone to infection. But there is really no other good way to give her medicine, draw blood or anything else without her central line open, so it's a tricky situation. We're continuing to pray that she'll beat this!
Evan and I got to spend the whole morning there yesterday thanks to some amazing friends, the Reavys, who not only came over to play with Connor but cleaned our entire house for us. What a blessing!
Evan and I got to give Brenna a "bath" for the first time - dabbing saline-soaked gauze over her gently - and applied her ointment. We also fed her several times as she began to get used to the bottle again. She really has a pretty good suck, and her lips are less taut as the skin begins to break up around her face.We are so encouraged by the fact that Brenna seems to like being held and handled. I've never really seen her fuss when her ointment is being applied, and she always calms down when someone picks her up. She even does really well with being sat up and burped!
Evan held her for a few minutes while our nurse Mindy changed out her padding that she lies on, and she was calm and checking us out the whole time. It was precious.
Can't wait to spend more time with our little miracle today...she is now 10 days old, I can hardly believe it!
Wednesday, December 28, 2011
Harlequin Ichthyosis is caused by a mutation in the gene ABCA12. Everyone has this gene and receives one from their mother and one from their father. Evan and I met with a genetic counselor last week to discuss this gene and what it means for us in the future.
Here's how I understand it: In about 93 percent of cases of Harlequin babies, the baby receives two recessive (mutated) ABCA12 genes from the mother and father. There is a very, very extremely small chance that it is just a random mutation that occured.
Most likely, both my family and Evan's family have had this mutated ABCA12 gene in our families for decades and passed it through many generations. Then, Evan and I got married and brought our mutated genes together :)
Brenna's DNA is currently being studied by the only lab in the country that will analyze this particular gene. It will take about eight weeks, because the genetic counselor said it's "a pretty big gene."
What they will do is analyze the entire code of both of her ABCA12 genes, looking for a specific "error." Once they figure out the error, they can then go straight to my blood and Evan's blood to search for this same error in our genes. In the most likely scenario, they will find the same errors in all of our genes, meaning that Brenna received one recessive gene from me and one recessive gene from Evan, causing her to be born with Harlequin Ichthyosis.
So, if this is the case as we are assuming now, every one of our babies has a 1 in 4 chance of being created with this disease.
The chances of both Evan and I having this recessive mutated gene? Literally one in a million. When we learned that, I looked at Evan and said, "I always knew we were special together."
Tuesday, December 27, 2011
Brenna and I had some much-needed girl time tonight. I spent about two hours with her, and we chatted and I sang her some songs. "You Are My Sunshine" was a definite favorite.
At one point, I told her: "Girl...you have so many people praying for you and thinking about you, you wouldn't even believe it. You definitely have your own fan club, your own cheering section. Everyone is on Team Brenna!"
Brenna didn't get to wear my beautiful baptismal gown. Her godparents weren't able to be there, nor were Evan's parents. And there was despair over Brenna's declining health.
But it was the most special baptism for our baby girl.
On Christmas Day, at 1:30 a.m., Brenna Helen Marie was baptized and given to the Lord by Evan's childhood pastor, Pastor Larry. To avoid removing her oxygen, he annointed her shoulder with sterile water as he made the sign of the cross, while Evan and I both were able to place our hands on her other shoulder.
Baptizing Brenna gave us such a peace that early morning as we prayed for her condition to improve, and that peace hasn't left our hearts.
Our wonderful nurse took photos for us...
There is a lot of glare from the glass, but you can still make out Evan's and my hands...
Monday, December 26, 2011
Today is a new day, and another chance to spend more time with our angel, and for that, we are so grateful. We had to remind ourselves last night that God has given Brenna to us - to care for, to love, to share with others and to cherish - and it is all part of his plan when she will be returning to him in heaven, whether it's tomorrow, a few months or in 30 years. And what an amazing gift she has been in just seven short days!
Sunday, December 25, 2011
I felt so helpless as I sat there pleading with God throughout the night, again and again, "Please don't take my baby girl on Christmas."
The main concern was her breathing, and they had started to give her oxygen. If her breathing became bad enough, the only other option would be to go to a respirator. They have told us from the beginning that this is practically an impossible option - it is extremely difficult to do this for tiny babies anyway, and the respirator must be placed in a very specific position down her mouth and not moved. With her skin the way it is, attaching the respirator is, like I said, pretty much impossible, not to mention would likely be extremely uncomfortable for Brenna.
It was the worst feeling in my entire life as Evan and I sat there with the doctors discussing the likelihood of having to say goodbye to our precious girl. We were so torn between wanting to try everything in our power to save her life, and facing the reality that choosing to insert a respirator would most likely only inflict pain on her, which we absolutely do not want. When the doctor and nurse told us that if we decided against the respirator, they would make her "as comfortable as possible while her breathing deteriorated," we could do nothing but sob.
We are so thankful that Evan's childhood pastor who married us answered our call at 1 a.m. and came to the hospital to baptize Brenna Helen Marie. It soothed our souls to have her baptized.
They ended up then doing more tests at 1:30 and again at 5 a.m., and they showed slight improvements in her blood, and for the time being, she was as stable as she could be. By that point, we were past the point of exhaustion and decided to try to get a couple of hour's sleep at home.
This morning, we talked to the nurse and Brenna's lung saturation is up to 95%, which is really good, and though she is still on oxygen, it is at lower levels. PRAISE GOD.
We are by no means past this infection, but our little girl is showing us that she is a fighter. We continue to ask for your prayers as, God willing, Brenna continues to fight through this.
Saturday, December 24, 2011
Because Brenna's temp was a little high today, they had the humidity down in her incubator, which equaled the perfect time to get some beautiful photos of her. I took a million because I want to record every single detail about her, but I won't bore you with all of them :) Here are some of my favorites...
I told her she has a little friar hairline going on :) very fashionable...
Her skin looks better every day thanks to the wonderful care they are giving her. The ridges in her skin seemed so much better today, and some of the thick skin she was born with is already starting to peel off. Once the medicine starts to work, we are hopeful it will progress even faster.
We are following the "blueprint" of the care of a little boy who was born a couple of years ago in North Carolina with Harlequin Icthyosis. The boy, who spent about four months in the NICU, is now two or three years old and doing well - we read his case study today and it gave me a lot of hope!
My ob/gyn, Dr. Brown, called this morning and said she had just been to the hospital to see Brenna and said she looked "amazing." It warmed my heart :)
We called to check on her this morning, and the nurse said that her body temperature was up, but they didn't seem concerned over the phone, so that is something we want to talk to the doctor about. The doctors make their rounds around 10:00, and the nurses "handle" Brenna every four hours (8:00, noon, 4:00 and so on) - changing out her pads under her, cleaning her, feeding her, etc. - so late morning is a really good time to be at the hospital, so we can talk to the doctor if we want to, and then hopefully participate in her feeding at noon.
Yesterday, I spent time with Brenna for the first time by myself - it has definitely helped my strength to have family around when visiting with her, especially Evan, but I wanted some alone time too, even if it meant crying the whole time. I read to her from her first book that my best friend Kristin got her (and cried through the whole thing; it was a beautiful story!) and just sat and talked with her.
Brenna was lying on her back sleeping the whole time I was there and cried out quite a few times. Like any newborn, she startles easily while on her back, and of course swaddling isn't an option, so she likes to lay on her side much more than her back. But they have to make sure she isn't lying in one position very much of course.
Anyway, every time she would start to cry, I would talk to her and sing to her, and she would quiet immediately. I like to think my voice was soothing her :) I'm not sure how much she is hearing right now - there's no reason to think her eardrums aren't completely fine, but the skin around her ears is SO thick. It is starting to come apart now, but when she was born, it looked completely closed. She seems to know our voices though, so I am hopeful she is hearing us talk to her!
After I had spent a few hours there, it was getting close to 4:00 and Evan was leaving work, so he came by and we got to help feed her again! She is making so much progress with her sucking, and she loves her binkie too :) She ate almost 10 mL from an actual little bottle for me, and the rest went in the feeding tube. They are increasing her food again now to 20 mL, which is so great - she needs those calories to help her skin "shed" and produce new skin!
Hope you all have a wonderful Christmas Eve and Christmas Day, celebrating the birth of Jesus Christ! Our precious angel was the best present we could have asked for this Christmas, and I can't thank you enough for your wonderful words of encouragement, your prayers, and your heart-warming messages of support - you are all a huge reason why I am able to get through these difficult days!
Friday, December 23, 2011
We've been talking all week about how Mommy and Daddy have been going to visit Baby Brenna, and we thought it would not only be positive for him to "meet" her but also to see exactly where we are going when we talk about the hospital.
As we expected, his attention span was about 2 minutes long before he wanted to go play in the waiting room, but he was so sweet when we walked into the room. Unfortunately her incubator was extremely "foggy" from the moisture when we were there, so he couldn't see in very well, but he kept asking us to "open it." :)
Brenna was born with Harlequin Ichthyosis, which is a very rare and extremely serious skin disease. Basically, her skin lacks a certain protein which helps it form correctly, and instead it was formed as thick, white-ish scales with ridges all over. It is so thick that it is very tight and keeps her from moving very much. The tightness in her skin caused her eyelids to be pulled outwards, so that they are almost inside out, her ears are pulled tight against her head, her nose is turned up and her lips are pulled outwards as well. While her physical appearance is definitely startling, we think she is one beautiful girl.
Because her skin lacks this protein, it cannot retain moisture like normal skin, nor can it regulate her body temperature as our skin does, so she must be kept in a very warm and moist environment in the NICU at St. John's Hospital. They are closely monitoring her temperature and humidity levels in her incubator and have to have everything sealed unless they absolutely must open one of her "doors" to feed her, apply ointment, etc.
Most likely, it was caused by both my husband Evan and I carrying the recessive gene for this condition - which is literally a 1 in a million chance for us both to have it.
Right now, Brenna's biggest risk is infection, because she has such a high susceptibility, and it will be a very big challenge to both avoid infection and treat it if it does occur. We have begun aggressive treatment with a medicine that will help her skin "turn over" more quickly. We are so fortunate to have a wonderful pediatric dermatologist right here in Springfield (which is a rarity), and based on the very few cases there are like Brenna's, this course of action will help her shed the very thick scales and be left with a more reddish, flaky skin if it is successful. There will be a lot for her to overcome to get to that point, and it will take months at minimum.
Through it all, however, our priority is pain management and we want to make sure Brenna is as comfortable as possible and not in much pain. There are so many what ifs that we are just ready to take this day by day to see how she does, and to cherish each second we get to spend with her.
We found this video online about a teenage girl who is living with Harlequin Ichthyosis in the U.S., and we found it so helpful in understanding what is happening with Brenna's skin and to understand what a vital role our skin plays to our body, which is why this condition is so serious and not just an aesthetic issue.
(scroll down a bit past the ad)
Above all, Evan and I feel so proud and honored that God chose us to be the parents of this special girl. We have so much love for her already and know that whatever is to come, she has already made such an important impact on our family.