A little back story: For years, I struggled with very irregular menstrual cycles. Even as a teenager, I was becoming aware of my family’s history with infertility, and started to understand that I would have a difficult time getting pregnant. I was open with my future husband about this, which is why we didn’t wait a full year before initiating infertility testing. In 2008, after visiting a reproductive endocrinologist, I was diagnosed with polycystic ovarian syndrome, or PCOS. At this time, we also discovered that my husband, John, is a carrier of a very common cystic fibrosis mutation, Delta F508 (DF508) - if you have never been through infertility testing, they check you for everything, including common genetic conditions and mutations that can cause problems for a fetus or an infant/child. My tests came back clean - at the time, we didn’t realize that most clinics will only test for about 36 common cystic fibrosis mutations. Therefore, we assumed I was in the clear; the most that could happen was we could have a child that would be a carrier of the disease, and it would only become an issue if our child would try to have a child with another carrier.
Wrong. So, so wrong.
Fast forward to the day of her discharge, and on her orders (and in the letter dictated and sent to our primary pediatrician), we were again told that she would need to undergo further testing because of the positive hit for CF. After a visit with our primary pediatrician, we set up an appointment at Hershey Medical Center for a sweat test, which would be the deciding factor. The test would involve a technician rubbing a special chemical on Judith’s arms, then attaching an electrode to the spot and sending low doses of electricity through the electrode that would activate the chemical, causing her to sweat. A pre-weighed piece of gauze was placed on each arm and wrapped to collect her sweat. Poor Judith hated the test, and who can blame her? She was not a happy camper! We also had an appointment to meet with a pulmonologist at the CF clinic after her test, and we would go over the results together. We received some information about the disease, then found out that, because of her size, she didn’t sweat enough for them to get an accurate result. We had to return and repeat the test once she gained some weight and grew. I was bummed that we had to go back, since that would mean another appointment among the already high amount of appointments, but I understood why we needed to do it. The pulmonologist didn’t seem too concerned given the history we told him, so I thought this was just a formality, and the second visit would be the last.
It was April 20, 2011. Judith was about 8 lbs., a magic number for us because that meant she should be able to sweat enough to give us an accurate result. We went through the test again, and once completed, walked over to the offices to meet with the pulmonologist. The nurses weighed and measured her, then told us that the doctor would be in shortly. We sat in the room for almost a half hour before the doctor finally came in and gave us the news: Judith did indeed sweat enough for this test, and the numbers were high enough to make a diagnosis of cystic fibrosis. I was in shock; John wasn’t with me to hear the news (my mother came with me to help), and I had no idea how he would handle it. Everything was a blur: a team of people came into the room to talk to us, we were given multiple prescriptions, a spacer, and a manual percussion cup, and instructed on how to do breathing treatments with an inhaler and how to administer chest physiotherapy (CPT) to help move the sticky mucous out of her lungs. We also received orders to set up a repeat test for a second opinion since her numbers were on the lowest end for definitely having the disease - for a baby 6 months or younger, any number of 60 or greater means the infant has the disease, 30-59 means it’s borderline, and 29 and lower means no disease at all. Judith’s numbers were 60 and 61.
We returned for the second sweat test, and the results were conclusive: she definitely has the disease. This time her numbers came back at 64 & 65, moving her farther into the category of certainty. Judith had some blood drawn for genetic sequencing; we knew she carried a copy of DF508 that she got from John, but we had no clue what mutation I passed on to her. A few weeks later, we received the results from the test, and were no further than we were before. The second mutation, the one I passed on, is still unknown; it’s possible that it’s a more recently discovered mutation that the lab panel doesn’t include yet, but it’s also possible that it hasn’t been discovered. It could be years before we find out what the mutation is, or it could show up when we retest her after the lab expands the panel.
I never thought I’d end up being a dragon parent, one who will do anything to help their child survive and have the best quality of life possible. I feel like a lucky dragon parent, though: DF508 has the potential to cause severe disease in CF patients, thus shortening their life expectancy more. To date, Judith appears to have a more mild form of the disease, but things can change at any time. So far, she is pancreatic sufficient, meaning her pancreas functions normally and her body can absorb fats as well as you and I; however, she has been showing signs lately of a change, and it’s starting to look like she’ll join the more than 90% of CF patients who are pancreatic insufficient and need to take enzyme supplements before meals to help the body absorb fats. We’ve also been lucky with avoiding respiratory infections. Thanks to her preemie status, she received a full course of Synagis injections last year, and due to the CF is receiving the full course again this year. Aside from a couple colds and a sinus infection, our precautions appear to be working.
If you would like to read more about our journey, starting with our days in the NICU, you can read our blog 2 Bostons, 1 Preemie, &65 Roses.
Around the holidays our preemie group started to get a little glum so I asked everyone to share their happiest NICU memory. Even though it’s a hard place to be we all had good times, too!
We'd been there for a month or so when our bay had emptied out and the nurses were hanging out. I'd never seen them not zooming and it gave us time for lazy conversations about normal things like wedding table place settings and how one was in a band with her almost-DH. Cute! Their pamphlet had said "ask your nurses if you would ever like a hand or footprint and they will be happy to make them for you." I thought, well, it's been a while - I wonder if they'd help me do it?
The next day I brought in my baby books and paper and our primary brought out lots of ink pads and told me it was going to get messy - DS was a riot and covered in ink by the end. He didn't want to lay his hand flat (do they ever??) and so they came out looking like racoon prints! She was like 'I'm sorry they aren't perfect!' I told her they were - she'd given me the best gift of all - laughter. I'd never laughed with him or had a time when I felt so free
Despite all the "downs" we actually had a lot of fun in the NICU. Two of my fondest memories are Easter and Mothers Day.
The twins first Easter, they were able to wear clothes and I got the smallest size I could find, but they were still swimming in their outfits. But the true fun came when EVERY. SINGLE. nurse in the NICU that day set up a "photoshop" for us right their at their bedsides. It was unbelievable. They drapped sheets over the monitors and took off any wires they could. We laughed so hard because DD was less then amused and DS was making all these funny faces. The nurses found a basket to put them in and everything.
My first Mother's day, while very bittersweet, was AMAZING. One nurse has a tradition of making "footprints in seashells" each year for the moms and or dads. I had no clue until later that night when they handed me this little box. Truely amazing... and the NICU was super busy that day yet they still found time to think of us mom's, a moment I'll never forget.
I'll play! I have a couple:
- I distinctly remember the moment when we started talking about "when" our son was coming home, rather than "if." The nurse who told us it was okay to talk about our eventual homecoming was named Trish. I even have pictures of her that day.
- The first time my husband changed my son's diaper, he got Z's hand stuck in it. That was hysterical. And, the first time my husband changed Zach's clothes, it took him about 15 minutes. I was laughing so hard I was crying.
- After we'd been there awhile (sometime after the terror of the first days and before the excitement of the last days), a nurse from one of the other hospitals in the hospital system brought us a hat for Zach. It was crimson and grey - the colors of Ohio State. The joke was that, since we were from Michigan, home of University of Michigan (Ohio State's big rival), our son was "Born to be a Buckeye." This nurse from the other hospital had heard our story and had grabbed the hat to give to "The Michigan Family." It made me so happy that someone was thinking of us like that. I was so touched. Later, my sister got Z an Ohio State onesie that actually said "Born to be a Buckeye," so that completed the outfit.
- The day or so before discharge, one of the night nurses got a camera and got all of the staff together to take a bunch of pictures with Z. They got him all dressed in his Born to be a Buckeye stuff and did pics where the staff was all pretend crying to see him go. She mounted them on paperboard in a scrap booky way. It was so sweet, especially since we never met very many of the night nurses.
Thanks for posting this question! Makes more memories come back... Good ones for a change. ;-)
Hands down, for me it was the first day I walked in and the nurses had dressed the boys in the clothes I left. They looked soooo cute! And all the nurses make a big deal of telling me how cute they were.
When Henry was 3 days old we got to give him his first bath. They took off his CPAP and all his wires. The nurse told me to be quick because she wasn't sure how he'd tolerate it. He fussed at first but then as I sat him up to do his hair he got real quiet and just STARED at me- right in the eye! He was too cute and went cross eyed with all the effort. I knew then that he knew I was his momma and it was fun to see his whole face at once. The added bonus was that he did great off CPAP so they changed him to nasal cannula that night. We did every bath after that first one. They were always a lot of fun!
Here's a pic from that night:
When I got to hold LO for the very first time and the nurses took our first family picture...Only the baby was looking at the camera..we were both looking at him :)
This is a silly one:
Mine was one time when DH was changing DD's diaper. She was a few days old (and in an open crib), but her poop was still pretty dark and gross.
Just as he took it off, she let out an explosive poop that went EVERYWHERE! Luckily, it missed DH, but it splattered on lots of medical equipment and the floor. I was laughing so hard, I could barely tell the nurse outside what had just happened.
We actually had some good times in the NICU - one of our closest friends is a NICU nurse and she was a godsend more times then I can count from the day DD was born until our rooming in "sleepover" - She works nights so she would dress up DD and put her in funny positions and places and take pictures and send them to me - I would be up pumping and get a photo of DD doing something hilarious! it would make my night at 4 am! I loved the first time DH got to hold her - or the time she pooped and it shot across her isolette and hit the plastic!!! they had to give her a whole new isolette! lol She was fiesty from day one and even at 24-26 weeks she was a comedian - she would pull everything and move everything in her isolette - especially the stickers with the day to change stuff - we would come in and find her with a sticker on her bum that said change thursday!
I had some happy times in NICU...
1) When DH changed DS dirty diaper for the first time. He kept saying are you sure this isn't tatooed on. LOL
2) The first time I was able to hold him. It was so amazing.
3) Also the 1st time he was able to eat
4) Then the day we were able to take him home it was so special and amazing.
I have a couple:
-The day I was able to hold them BOTH at the same time. I was over the moon that day!
-When we walked in one morning to see them both out of their isolettes and in open cribs. What a fun surprise!
1. The first hold.
2. The fact that our primary and secondary nurses got to know us beyond just DH and I being Owen's parents. Our primary had a magazine addiction and would always leave them at his bedside for me to read when she was done.
3. When our primary would work the night shift I come in after lunch and stay until midnight. It was always so quiet after dinner so it would give me time to have an actual conversation with her, whether about Owen or what happened on tv the night before.
4. Towards the end of Owen's stay he was the only baby in his bay so we got our own nurse. I loved coming in in the morning and seeing all of his clothes arranged nicely and the furniture in his room moved all around.
5. From day one Owen LOVES to be held in an upright position while your standing. When he was stable enough the nurses would let us unhook him and go for a walk around the halls of the NICU. The first time he looked out a window was priceless. It was a bright sunny day and he tried to do everything in his power to close his eyes and look away from the bright light.
We always try to stay positive and in the last few weeks we've had many happy times: both boys being moved into big boy cribs, Ryan getting off oxygen, Aiden finally getting extubated. I go on and on. But these few moments really stood out:
1. When the boys were born they were so unstable that I wasn't able to hold them at all. All I remember was saying that I can't wait to hold my babies and hear them cry. One night, when Ryan was just a few weeks old, the nurse was giving him his bath and he began crying. She stopped and let us listen. She even let my husband record his cry on his Iphone. I will cherish that moment forever, and to this day I cherish his cry.
2. Both boys have managed to poop on me an my husband during diaper changes. For some reason, they only pee on me though.
3. I love when the nurses have regular conversations with us, not only about our boys. There is one nurse who is always singing and is cheerful and joking around with the other nurses. Makes me feel like we are in a 'normal' setting.
I'm sure there are more moments that I am forgetting.
Great idea, Urbanflowerpot! (Your story made me laugh because we had a similar experience trying to get DS's prints for a wall hanging.)
We had a happy experience one night after about a month. We had a nurse and respiratory therapist we'd never had before.
My husband and I have a bad habit of randomly quoting our favorite movies and shows, which are often fairly obscure. We were doing a little back and forth quoting, when the baby's nurse jumped in with a quote from the same movie. It turned out we liked a lot of the same obscure stuff, and we spent most of the evening and other times she was our nurse talking about our favorite movies. (It really took our minds off our troubles.)
The RT, had a really uncommon, eastern-european first and last name. But she was planning her wedding for the fall. She was marrying a man with a very common last name, that happens to also be my maiden name. She was telling us how her family had started teasing her that she was going to be too plain-named after the wedding and calling her by a very common first name as well. The first and last name were my grandmother's name. We took it as a sign that my grandma (deceased for more than a decade) was watching out for our son.
One of my absolute favorite days in the NICU was the day Michaela went off her oxygen and DH and I were able to bring her into a parent room for an hour. It was the first time since she was born that just the three of us could hang out -- no nurses, no doctors, just us.
I also loved the first night her primary said we could put her in clothes! It was so unexpected, so I hadn't brought any of her clothes with me, but we got to go through the ISCU stash of outfits and pick out a cute one. Then the nurse took a family photo for us. Will never forget that night!
I also laughed alot every time Michaela would poop or spit-up on me or DH. It was such a "normal" baby thing during such a stressful time when nothing in life seemed normal anymore.
Our happiest NICU day was the day we were all reunited as a family... July 1, 2011, after 11 days of being apart, the twins were erunited in side-by-side isolettes, in the same NICU room.
After birth, both were in the NICU, but on two different sides of the walls (each room has 4 "walls" with babies on both sides...) Will was on wall 1, and Erin was on wall 4, making us split up to see them. 5 days later, Erin was moved to the intermediary care unit, but Will had to stay in the NICU as he still had a PICC line in. When they removed the PICC line, 5 days later, we were all reunited! Our primary even reserved two window spots so that the twins were side-by-side, and we had a private window space just for them.
Best NICU day ever!
Ours were kind of spread out.
While I was bummed that I missed Santa, we had an awesome surprise waiting for us before Christmas: Santa visited the NICU, J got an adorable stuffed bear wearing a shirt that said "You are Loved," and she had her first picture with Santa! I was so happy that she was off humidity by that point, because they were able to pop her top and drop the sides of the isolette to get a good picture.
One of the nurses is a hobby photographer, and she always takes pictures of the babies for the holidays. She'll bring in props and will make a scrapbook page to display on the supply cart/white board by each baby's bed. I was always excited to see J's new page as a holiday approached!
Best night ever was when we started quoting Princess Bride at like 10pm with me, hubby, and two of the nurses. I was laughing so hard I was crying. Then I realized the pump reminded me of The Machine from the movie, and started laughing again. It was great to just joke around and form those friendships.
A few stand out to me as I read over everyone else's replies:
1. Soon after he moved to CPAP (he was on the ventilator for just over the first month) I got to spend a morning helping give him a bath. I had done baths a few times before that, but I was always so tense, and since he was vented it was a very quick wipe down. This time, we took our time (it was still just a sponge bath), took pictures, and laughed at his funny expressions. The nurse took off his CPAP to change it out, and it was the first time I got pictures of his entire face.
2. The day he moved to the nasal cannual. He was just over 2 months old, and eariler in the day had a completely scary desat when he needed to be bagged and his oxygenization went down to single digits. It sucked. (I was there). Got a call a few hours later from the nurse who said they switched him to the cannula. What the what?!?!? They suspected (correctly) that he was just fighting the CPAP and that he was clamping down and stoping breathing. Stubborn little guy. Well, a few hours later when we went back in it was like there was a party in the NICU. All the nurses were thrilled to take him out of the isolette and pose with him. And one of them took pictures of the whole transistion from CPAP to cannula which I will always treasure.
3. Probably a no-brainer, but the day we brought him home...the nurse let us disconnect his wires and it was the first time (besides a few baths) that we held him wire free. Knowing we were leaving what had become our second home for the past 4.5 months was a mixture of pure happiness and sadness to leave all the nurses and doctors and staff that we grew to love over those months.
Meet my g-tube baby, Gracie!! Gracie was a former 27weeker, born on August 3rd and because of the g-tube she is thriving, gaining & doing great. In the NICU she was always referred to as a “pukey baby”, because every time they gave her formula she would throw it back up. They thought a milk protein allergy, and put her on strict broken down formula. It wasn’t until an October ultrasound finally found a Hiatal Hernia in her belly. The hernia was “very large”, according to the surgeon. Who said she didn’t expect it to be as big as it was, when she went in to repair it. While having that repaired, a g-tube was placed in her belly at the same time. We had many discussions around the g-tube prior to the surgery, we weren’t absolutely sure that she needed it. But after a few weeks of waiting to help her gain weight for the surgery, we soon realized that the g-tube was the best choice for her.
Gracie’s surgery was on November 7th, and she weighed 4lbs. 1oz. By the time we finally left the NICU on December 5th, she weighed 6lbs. 2oz. Way to go, Gracie!!
I’m not embarrassed that my daughter eats differently than other babies. In fact, I’m proud of her and how far she has come. As with many preemie babies, there is always the fear they aren’t going to make it; not only has Gracie made it, she’s doing it with flying colors. She’s a brave, strong little baby.. destined for only the best.
I’m not sure how long she’s going to need the g-tube. We have a really good G.I. Specialist, that we see every two weeks. He’s not very concerned about taking the g-tube out. But when asked by my husband, he says maybe 8 months to a year. He wants her to continue to get good nutrition and be healthy. Whatever it takes, I say. “Whatever it takes.”
The only downsides of the g-tube I would say is that it leaks A LOT!! Unfortunately, I don’t think Gracie is really ‘wearing’ the right g-tube size. The smaller one causes skin friction, but the larger one causes a lot of leakage, which means the acid of the stomach gets on her skin & makes it ulcerated. We put some special cream on it a few times a day, to help the ulcerations. It’s been a fine balance to make sure she’s getting the correct nutrition, and to have her not be in pain with it. But all in all, it’s helping her grow.. and that really is what it’s all about. She is currently 10lbs 8.5oz!!
Thanks for reading about Gracie.. I’m sure you’ll be hearing from us again!!
Hilyer was born weighing 1lb 7oz. He was (and is) my Itty Bitty. After birth his weight dropped to 1lb 4oz, but he seemed to rebound quickly. He was able to start on small feeds of breast milk (BM) within 5 days of birth. Initially, he was on 27 calorie (fortified) BM but he wasn't gaining weight as quickly as his brother. Due to his IUGR and immature lungs, it was explained to my husband and I that his little body was "running a marathon day and night". Even though he was doing well with the BM, he was burning all the calories just lying in his isolette. Then the neos bumped him up to 30 calorie (fortified) BM. After reaching the 4 pound mark, he finally seemed to be on track. Gaining every day.
When we were given the green light to start bottle feeds, I thought "YES! He will be home soon." He took his first bottle a couple of weeks after the boys due date. I just knew that he'd be home in no time. First bottle was in early/mid June. He wasn't discharged until July 31st. That's a long time to work on feeding. We worked with PT, OT and Speech on his bottle feeds. Everyone agreed that he could drink from the bottle. His suck, swallow, breath (SSB) was functioning, but it was just too hard for him and his weak lungs. Everyone kept saying "Once he hits 5 pounds, he'll turn the corner." Five pounds came and went...."It won't be long now. He's growing new lung tissue every day." Six pounds came and went. After weeks of therapy, one of the speech therapists (our favorite!) suggested something -- pull his NG tube see what happens. This was a Thursday. His NG tube was pulled and we went to an on-demand feeding scheduled. The neos agreed to minimum amount of BM that had to be taken per 24 hours for the next 48 hours. I roomed in Friday and Saturday night. If Hilyer was awake, we tried the bottle. On Friday, he exceeded his daily goal. Woo Hoo! Then on Saturday, we barely made it but met the goal. We were discharged on Sunday morning. Finally, we were all home and done with the hospital.
We were discharged with oxygen. Hilyer was diagnosed with BPD after not being able to lose the oxygen support within 28 days of birth. When we left the hospital, we had follow appointments made for every specialist possible.
You always hear stories about babies that "thrive at home". I guess it depends on what your definition of "thriving" is. He was happy. He slept very well. But he didn't care to eat. We had our first weight check on Tuesday afternoon with our pediatrician. He had gained a measly ounce in nearly 3 days. Our pedi wasn't too concerned, just chalked it up to adjusting to being at home and being overstimulated. We went back on Friday -- no weight gain. Our pedi still wasn't too concerned, but stressed that we offer the bottle every other hour over the weekend and be back on Monday. What a frustrating weekend! Back to the pedi on Monday morning. Up an ounce. If you're counting that's 2 ounces in a week. Not exactly ideal.
I love our pedi. She didn't "wait to see" what would happen. She got our follow up appointment with Hilyer's pulmonologist moved up and they could see us the next Monday. Part of our pulmonary plan includes a nutritionist and she was quick to mention a feeding tube. I scoffed at her. He could eat. He would eat when he was hungry. We modified his diet a little to see if it would help. We watched his weight for the months of September and October. After seeing very little progress, my husband and I started discussing the possibility of a g-tube. Finally at our November pulmonary appointment, Hilyer was officially diagnosed as "Failure to Thrive". What a blow! He was doing so well at home. He was happy. He was getting stronger, but still not gaining weight. For him, weight gain equals new, healthy lung tissue. New lung tissue equals FINALLY ditching the oxygen.
After doing my research, my husband and I agreed to the surgery. In addition to having the g-tube placed, we also had a nissen fundoplication done. Hilyer's pulmonologist thought he may be silently refluxing and possibly aspirating.
Hilyer had his surgery on November 22nd. Before surgery he weighed just under 9 pounds. Since his surgery, we have been feeding him 22 ounces a day. Initially, we were doing BM and Similac Sensitive. After a couple of torturous weeks, we finally saw a GI. He suggested switching Hilyer to Elecare due to a suspected dairy protein issue. Since the change, it has been smooth sailing. Hilyer receives about 24 ounces of Elecare a day. He is fed every 3 hours during the day and then continuously over the pump at night. Just last week, he weighed in at just under 13 pounds. Yep -- 4 pounds in just over 2 months!!!
It's amazing how different he is now. He is full of energy. He has a double chin and a Buddha-belly. He's meeting milestones much more quickly. He talks all.the.time! Without the gtube, I do not think Hilyer would have broken the 10 pound mark yet.
For us, the gtube is necessary. How long will he have it? Who knows. I hope we're able to ditch it sooner rather than later. Right now, our main goal is weight gain. All of our specialists assure me that we can get him to eat when we're not dealing lung issues. They've been spot on so far, so I just have to trust that they are right about this.
Please stop by the Feeding Tube Awareness page and read the success stories. They are truly inspiring!
Addison is primarily G-tube fed.
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