Genius Baby!

Gwena had her 18 month checkup last week and they asked me if she could say 6-10 words. Ha! Try 32! Here they are:

mama
dada
uh oh
up
down
hi
bye
eyes
ears
toes
belly (bellybutton)
boom
dog
bear
bird
baby
book
ball
apple
milk
back (as in "put it back")

pee pee

poo poo

elbow
eyebrow

peeka (peekaboo)
Oui-Oui
Doudou
Mickey
blankie
cookie
keys

Of course there are a ton of words she can understand but not say, and a few she will repeat after I say them but never says on her own. So yes, that's right, I have a genius baby. ;P

The End of our Clinical Trial

Well, we returned to Stanford and finally had Gwena's second pulmonary function test, or PFT. She is now done with her clinical trial, and has a real prescription for the study drug, hypertonic saline! Here are her PFT numbers for last year and this year:

	last year	this year
FVC, ml	91%	76%
FEV 0.5, ml	80%	81%
FEV 0.5/FVC	88%	106%
FEF 25, ml/sec	76%	97%
FEF 50, ml/sec	59%	103%
FEF 75, ml/sec	56%	73%
FEF 85, ml/sec	57%	11%
FEF 25-75, ml/sec	57%	96%

They told me for the ones that went down, they think she was closing her glottis and the result wasn't accurate. Most of the numbers went up! Yay!

Last year's report said, "Early obstructive changes at the small airway level and with evidence for air trapping."

This year's report says, "Fairly normal flows and volumes without evidence for obstruction."

YES!!!

We don't officially know yet whether we had the real hypertonic saline (7%) or the control drug, isotonic saline (0.9%), but I will be very surprised if they say she had the control.

Hypertonic saline is a pretty new treatment for CF (the study on it was published in 2006) and this is the first time it's been studied in kids under age 6. I think it's an amazing thing and I'm so glad Gwena was able to start on it at such an early age!

Baby's First Lung Infection

The past couple of weeks have been pretty tough. About 5 weeks ago, Gwena developed a cough. She had a fever for about a day, then the cough seemed like it was getting better on its own, and then it got worse again. I called the CF clinic and they put her on amoxicillin for two weeks. The cough improved but didn't entirely go away.

On March 1st I spent the day packing for what was supposed to be our last trip up to Stanford for the ISIS clinical trial. That day Gwena had a runny nose, but no other symptoms. We drove up that night and the next day, her cough started to get worse. Thursday morning we starved and sleep-deprived her for her infant pulmonary function test, took her to Stanford, and found out that because her cough was so bad (the worst it's ever been, I'd say) and they heard wheezing in her lungs, they couldn't do the test.

They prescribed her a steroid (Prelone) and we started her on it right away. She finally got to sleep and eat and we decided we might as well head for home. As much as I had tried to avoid catching what she had, by this time I was also suffering from a stuffy nose. We drove about half way home, then stayed the night in a motel. The next morning we drove the rest of the way, straight to her CF clinic. They prescribed her a new antibiotic, Bactrim, and did a throat swab culture.

Three days later, on Monday, Jon left on a business trip to New York, and Gwena and I went to visit my folks. The steroids my poor baby was on turned her into a crazy little terror, but we still had fun and she got to spend some good time with her grandma. The clinic called me to let me know Gwena had cultured Moraxella catarrhalis, which should be sensitive to the Bactrim.

We're all home now, Gwena is off the steroids, and her cough has improved considerably, though it's still lingering. We have a new appointment for the pulmonary function test, on March 31st. Hopefully the M. cat will be gone by then!

EEG Results!

This update is late, but Gwena's EEG result was in on Tuesday, much faster than they said it would be! They said that it was perfectly normal!! We are so relieved to hear that, even though it means we may never know what happened last week. Her doctor said maybe it wasn't really a seizure, maybe it was just "well, I don't know." Thanks doc, that's very informative!

I've had an awful sinus infection this week, and Gwena has had a cough and had a fever on Wednesday. We're both doing a lot better now, but we had a tough week. We've been resting a lot and watching Fraggle Rock on Netflix streaming. Gwena now knows which button to press to start the next episode! She loves all the songs and for me the show brings back happy childhood memories. An episode is ending right now, and Gwena is dancing to the end credits song. So cute!

A Scary Thing Happened

On Tuesday Gwena woke up in the middle of the night and wouldn't go back to sleep. She rolled around, she talked to herself, for what must have been at least an hour or two before she finally went to sleep again. When she woke up in the morning, she was fussy. She rolled around on the bed next to me, seemingly still half asleep, fussing and fussing, like she sometimes does when she has gas.

All of a sudden I saw her body shudder. It was a cold morning, so I figured she was shivering with cold. I drew her to me and tried to warm her with my body and the blankets, and as I held her that shudder happened again. It was like her body made one big twitch. She started crying more and it happened again, and kept happening, every few seconds. I began shouting for Jon and throwing things at the bathroom door.

Jon got out of the shower and I told him what was happening. He took Gwena and I remember the terrible look on his face when he felt her body shudder. We threw clothes on, put Gwena in her car seat and left to take her to the emergency room. I sat beside her in the car and covered her with some blankets. She was staring out the window vacantly, but when I said her name she turned and looked at me. She didn't seem to be twitching anymore, and as we started to drive, she closed her eyes and fell asleep.

I called her pediatrician's office and spoke with a nurse there, who said Gwena was not in immediate danger and we could bring her to their office rather than the emergency room. We got the earliest appointment we could, for a few hours later, and went home to wait. Gwena woke up at home and I nursed her and fed her breakfast. She sat on the couch and I put her favorite video on, and she just stared at it, tired and out of it.

The pediatrician said it could have been a seizure, and ordered an EEG and referred us to a neurologist. She said it was a good sign that Gwena didn't lose consciousness and that she was alert and responsive while it was happening. She said that sometimes a child will just have one unexplained seizure and then never have one again. We hope that's all it is.

The rest of that day she seemed extra tired and fussy, but by the evening she was back to her normal self. Wednesday we went to CF clinic, where everything looked great and her weight was 20 lbs 15 oz. They increased her zantac and want more blood work to check her iron levels. Dr Y didn't seem concerned about the possible seizure when I told her, but then again she is a pulmonologist, not a neurologist.

Yesterday we took Gwena to CHOC for her EEG. We were told to not let her go to bed until 10 the night before, then wake her up at 6, and not let her nap until the test. She napped for 10 minutes during her saline treatment before I could finally wake her up again. She was alternating between out of it and fussy when we got there, then started screaming as the tech began attaching things all over her head. It was hard to see her so scared and upset.

When he was done he wrapped her head in gauze. Then I got to lie on the bed with her and she fell right asleep. They flashed a light at her for a couple of minutes, then turned it off. The test went on for a while and I almost fell asleep too! Gwena wasn't the only one sleep-deprived from getting up at 6am (an hour I prefer to ignore the existence of!)

At the end of the test we had to wake her up, which it turned out nothing but a cold washcloth could do. Poor baby! She didn't open her eyes, but began to cry and scream, then he removed the cloth and she conked out again.

The tech said it was a very successful test and we should expect results in 7-10 business days. Is it just me or is that a really long wait??

Words, Words, Words

Gwena has been learning new words all the time now. I'm so proud of her, but it's also been frustrating, because sometimes she'll learn to understand or say a new word, and then will refuse to cooperate when I try to show someone else. Or else she'll completely stop responding to the word the way she was. Like with "nose" for instance. It was the first body part word she understood; we asked where her nose was, and she grabbed it every time! But then she learned "eyes" and got very confused... now she'll point to her eyes when you ask for her nose, or point to her nose and say "eyes".

I need to get some videos of her talking and otherwise being really cute, but the memory on my camera (aka my phone) is currently full, so I'll have to delete some stuff and/or move the picture and videos to my computer first.

These are the words she can currently say or at least try to say, in no particular order:

mama

dada

uh oh

up

hello "eeyo"

bye bye "bah bah"

eyes "eyesh"

ears "eesh"

toes "doe"

dog "dah"

cat "kya"

boom "bao" (when she falls down)

bellybutton "buh"

book "bah"

She also understands a lot of words that she doesn't try to say. I don't think I can list them all but here are a few:

kiss

clap

wave

sit

dance

twirl

arms up

milkies (aka mama's milk)

mouth

teeth

baby

I'm sure there are more... maybe I'll add more later if I think of any.

It is so fun to hear Gwena talking and see her understanding and responding to things I say!

And Many More

Yesterday was Gwena's birthday! I can't believe my baby is 1 year old now. Once I get more pictures, I'll make another post about her baby luau. For now I just want to say how very happy I am that her first year has gone so well. She is happy and healthy, is on (the very bottom of) the growth chart, has never had a lung infection, and is still nursing despite a rough start to breastfeeding in the beginning. Her pancreatic insufficiency is severe, and I am fully convinced that without the enzymes she takes whenever she eats, she would not have lived to see this first birthday.

I am so thankful for her good health, and for Gwena herself and all the joy she has brought me. When we sang to her yesterday and at the end of the song everyone sang, "and many more!" it caught me so off guard I almost cried. Those words have never meant so much to me before, but I know that Gwena will have many more birthdays, and each time we will celebrate the blessing of another year of life, and wish her many more!

July Clinic Visit

This is my second belated update post!

Last Wednesday we went to the CF clinic. Gwena's lungs are doing great! The nurse told me I'm doing a great job. :)

Her weight wasn't too good though... she weighed only 15 lbs 5 oz. Two weeks before, at the pediatrician's, she had been 15 lbs 8 oz. I know it's two different scales, but we had also weighed her on our home scale, and she seems to have lost a few ounces in those two weeks, instead of gaining! Her growth curve had a little dip in it.

So the plan is to feed her more and richer foods. Right now she's nursing 5 times a day. In addition to that, they want us to give her the foods we're eating blended up, give her more rice and pasta and crackers, cook for her using butter, oil, or cream, or add those to her foods directly. We are to weigh her every week on our scale and email that figure to the dietitian. They will see us back at the clinic after 4 weeks to see how she's doing.

It's kind of disheartening to find out she hasn't been gaining well, that we have to fight harder to get her to grow. Getting extra calories into her hasn't been too hard though, and I can already tell from her stools that we've outstripped the amount of fat that her enzymes can handle, so we might need to increase her dose as well.

I can't believe she's 10 months old already! It's crazy to think that without enzymes and treatments, she might not have lived even this long. She's standing in her playpen right now waving at me, and she just looks so old!

Our New Pediatrician

We went to see Gwena's new pediatrician on Thursday for her 9 month checkup. We had been driving down to Aliso Viejo to see her old one, and I finally got around to switching doctors to one that's closer to us. How does that make sense, I'm too lazy to switch doctors so I've been driving 45 minutes for checkups? Anyway, she weighed 15 lbs 8 oz, which was great news! The new ped was very happy with her growth and development.

Dr: She's doing great! She's petite, but she's growing well.

Me: The CF clinic said they want all of their patients to be 50th percentile in weight.

Dr: Yeah, but this is the real world!

Yep, I love him already.

Weight Worries

Ever since she hit 6 months, Gwena's weight gain has slowed down dramatically. She's always been small, but at least before she was growing well. Now, not so much. At 9 months old she has just hit 15 lbs. When people ask her age, they usually say something like, "How old is she, like 5 months?" She is wearing size 2 diapers and size 6 months clothes.

We've changed enzyme brands, finding that Pancrecarb works better for her than Creon. Because Pancrecarb isn't being manufactured anymore until they get FDA approval, we tried Zenpep, and she didn't do as well on it. Now we're back on Pancrecarb until the pharmacy runs out.

Lately we've been trying really hard to get her to gain more weight. I've been nursing her more frequently, 6 times a day if I can manage it. After she nurses, I give her solids, which I've now begun to add olive oil to to add extra calories. She's very active and loves to crawl, stand, and bounce, and I guess it's going to take a lot of food to overcome all that calorie burning.

When we go to CF clinic, I feel like I'm going in to take a midterm or something. It feels like it's my personal achievement or failure if she has or hasn't gained enough weight. When the dietitian tells us she needs to be gaining more, it feels like I'm being scolded by a teacher for slacking. Right now, my deadline is July 14th, the date of our next clinic visit. I have until then to try to get Gwena's weight up, so I don't get an F.

I wish I didn't feel so guilty when she doesn't gain enough weight. I know I work hard every day to feed her and give her her enzymes and other medicines, but it still feels like I'm the one to blame even though I know it's really CF's fault. I don't want to blame myself for the effects of this disease. I'll just keep fighting CF as hard as I can every day. I'll do all I can, and then I'll do even more. Hopefully on July 14th, when I lay her on the scale, we will get an A+!

My Linguistic Hypocrisy

Gwena has been babbling for a few weeks now. She started with "ma ma" and later moved on to "la la" "ba ba" "da da" and my favorite "blah blah".* Now, I am well aware that she isn't actually talking yet. "Mama" was not her first word. This is what logic and my knowledge of linguistics and language development tell me. But I won't deny it melts my heart every time she says it!

"Mama" means the same thing in many different languages. It's the first thing most babies say, not because they're talking about their moms, but because it's simply one of the easiest sounds for babies to make. Of course, the moms then interpret it as referring to themselves! Who else would the baby be talking about?

I always thought this fact was funny... I thought other moms were silly when they'd say their 6 month old had said his first word, "mama".* Yet now when Gwena says it, I find myself responding!

"Mamamamamama!"

"Aw, I'm right here baby."

"Dadadadadada!"

"That's right, daddy's coming home from work soon."

I know she doesn't attach meaning to those sounds yet, I know she isn't talking! Maybe I'm just so starved for human interaction from staying home all day that I'm willing to pretend she's talking. Maybe it's an instinctual maternal reaction. Maybe that's how she'll eventually learn to associate the meaning with the sound. In any case, I feel like I'm betraying all my linguistical education.

Jon laughs at me every time I answer her. "She's not talking about you, you know."

But I can't help it, her babbling is just so cute and it's fun to have "conversations" with her. So I'm not going to try to stop, I'm embracing the motherly silliness!

*Oh yes, I put those periods outside the quotation marks on purpose. Long live the Campaign for Typographical Freedom! (If you don't know what that is, please read The Great Eskimo Vocabulary Hoax by Geoffrey K. Pullum!)

What is Cystic Fibrosis?

When Gwena was about 2 weeks old we finally got the phone call with her genetic results - she did have CF. We went to her first CF clinic visit that week and started her on her medicines and treatments. At that visit, she was almost 3 weeks old and had lost 5 oz, she was down to 4 lbs 8 oz. After getting on her enzymes, she gained 2 oz a day for about the first week.

But I'm getting ahead of myself. First off, for those who don't know... what is cystic fibrosis?

CF is a genetic disease that causes the body to produce a thick, sticky mucus that causes problems in various parts of the body, most notably in the lungs and digestive system. The mucus builds up in the lungs and leads to life-threatening infections, and it obstructs the pancreas, stopping the enzymes that are needed to digest food. CF is a relatively rare disease, with 30,000 people with CF in the US and about 70,000 worldwide. As recently as the 1950s, children with CF were unlikely to live long enough to attend elementary school. Now, the median life expectancy for those living with CF is 37 years, and for those born today it is likely to be even higher.

Gwena has cystic fibrosis, and there is no cure! There are, however, many amazing drugs and treatments in use now or in development that will help her fight this disease and extend her life. She takes enzymes every time she eats, to allow her to break down and digest her food. She has breathing treatments twice a day to thin and loosen the mucus in her lungs. Without these treatments, I know she might not even be here today. I will always be so grateful for these medicines, and thankful to God for the extra time Gwena has been granted. I pray that a cure will be found for Gwena and for all those with CF, and that my girl will live a long and healthy life!

Babbling Baby

This is my favorite video of Gwena so far. She shows off some of her expert babbling! And she does her "Danger, Will Robinson!" arms. And tries to eat a teddy bear's brains. I'm so proud!

Playing and Babbling from Kelly Omahen on Vimeo.

Flashback: Birth Story

The story continues from my previous post!

Jon and I had been preparing to have a natural, unmedicated, low-intervention birth. We read books, took a Bradley Method class, and practiced relaxation techniques. Well, things didn't quite go as we had hoped.

At one of our ultrasound appointments near the end of my pregnancy, it looked like the baby's growth had slowed down. We started doing non-stress tests and amniotic fluid checks, and every time we came in the amniotic fluid was lower, and lower, and the baby was definitely measuring small. On September 17th the fluid level was below 5, and they recommended an induction the next day, at 37 weeks. We talked it over, consulted with our childbirth instructor, and asked for another fluid check, which this time was below 3. I desperately did not want an induction, knowing that there would be greatly increased risks of interventions, fetal distress, and c-section, but we agreed that there was now a medical need for it.

I was admitted to the hospital for the night, and my OB placed a foley bulb to ripen my cervix overnight. At 7am they started Pitocin, and at 9:30 my OB took out the foley bulb and broke my water. The nurse gave me low doses of Pitocin, gradually increasing it and then turning it off to see if my body would take over. In the afternoon, it worked! I didn't need any more Pitocin, my body was able to take it from there. After some decelerations in the baby's heart rate, they put in a tube to pour fluid back in.

I started to have very painful back labor. Earlier in the day I had been able to walk around, but now the discomfort from everything I was hooked up to combined with the back pain made me prefer to stay put. It was hard to use the relaxation positions we had learned, because the monitors would slip and the nurse would have to come in and fiddle with them to find the baby's heartbeat again. Jon was an amazing coach and helped me through each contraction.

My OB went home and was replaced with the on-call OB. She came in around 9pm and checked me, and I was at 6cm. I was elated to have progressed so far, but the new OB told me I hadn't made much progress, and maybe I should get an epidural because it might speed things along. Yeah right!! Thanks for the vote of confidence, and for ignoring my birth plan! I told her no and she left, saying she'd be back to check on me. Shortly thereafter I went into transition, and before the OB even came back I was pushing.

I was told not to push until the doctors arrived, but we ignored that and I pushed anyway. The baby's heart rate was having decelerations again, then they lost it on the fetal monitor so they put in a fetal scalp electrode. I heard someone ask the OB, "Are you going to try vaginally??" She answered "Yes" but that question had told me how close they were to doing an emergency cesarean on me, so I pushed as hard as I could and in just a few contractions, our baby was born. She was placed on me for only a moment while they had Jon cut the cord, then she was taken across the room to be checked out by the team of neonatologists that had attended the birth. They took some of the cord blood to do a genetic test to determine whether she had CF. The OB stitched up the two tears I had acquired from all of that overly-hard pushing.


Our little Gwena weighed just 4 lbs 13 oz, and was born at 12:59 am, September 19th (Talk Like a Pirate Day!). Her Apgars were 8 and 9. They gave her back to me and let me warm her with skin-to-skin contact. They wanted to take her to the NICU for observation, but we asked that if nothing was wrong that she remain with us. As hard as they looked to find something wrong with her, they just couldn't! During the next two days we would spend at the hospital, they checked her blood sugar, her weight, jaundice levels... and everything was fine.


The week after she was born, Jon finally found a new job! Everything seemed to be going well. Our only clue that something might be wrong was her frequent poopy diapers. It wasn't until more than 2 weeks after the birth that her genetic results came back, showing that she did have CF.

We ended up with many interventions during Gwena's birth that we had hoped to avoid. However, I am proud to say I made it through without an epidural or any other pain meds. I believe if I had gotten an epidural, that since it would have slowed my labor and taken away my control of pushing, it is very likely I would have ended up with that c-section. I am also convinced that with drugs in her system at birth, Gwena would not have done so well and would have been taken away to the NICU.


Even though we didn't have the birth we had hoped for, and there are a couple of things I would do differently if I had it to do over, I am satisfied that for the most part we made informed decisions and did what we believed was best for Gwena's safety and for mine!

Flashback: Pregnancy

To tell the story of Gwena's CF I have to start with the pregnancy. Jon and I had gotten married in July of 2008, and we were living in Hawaii, where we had until recently been going to school. We found out I was pregnant just before we moved from our tiny 300 sq ft apartment into our "huge" 500 sq ft apartment (on the 4th floor of the building with no elevator). It was close to the hospital, just a few minutes walk, so we walked to all of our prenatal appointments. My due date was October 9th.


At 19 weeks we had our 2nd trimester ultrasound. We found out our baby was a girl (there went Gilgamesh as a possible name!) and she had a strong heartbeat, good movement, well-developing brain, etc etc. When the tech came to her bowels he spent a lot of time looking at them and not saying anything. He marked the pictures he took with a question mark. This didn't exactly put us at ease.

The doctor came in and looked, and explained to us that they were seeing "echogenic bowel" on our baby's ultrasound. This means the bowels were showing up brighter than they should, which meant they were harder than they should be. Several things could be causing this, so they would do some tests to try to find out what it was. One of the possible causes was cystic fibrosis.

A lot of waiting followed. We had to wait for my insurance to approve the carrier test, then we had to wait for the carrier test results. They called me one day while Jon was out and told me the result was positive, I was a carrier. (Or as my sister said, the result was positive in a negative way.) I cried my eyes out that day, even though Jon hadn't been tested yet.

Sometime in the midst of all of this, Jon and I both lost our jobs. Hawaii isn't the cheapest place to live, especially when you have no job, so when Jon's search for a new job proved unfruitful, we began to plan an emergency move back to California. We already had plane tickets for July to visit our families, so we decided not to come back. This added no small amount of stress to our lives.

We spent most of our first anniversary packing boxes. The next morning Jon shipped our things while I was at the doctor's having a 3 hour glucose test. The next day we got the carpet cleaned, I got a Rogam shot, and then in a fit of insanity we went to see the midnight premier of Harry Potter 6. The next morning we handed over the keys to our landlord and left. Somewhere between the apartment and the airport, they called to tell us Jon's test result - he too was a carrier of CF.


We both carry the same mutation, the most common one, Delta F508. There is a 25% chance that any child we conceive will get both mutated genes and have CF. We declined an amniocentesis, deciding to wait until our baby was born and have her blood tested then. We spent the rest of the summer moving back and forth between our two families' houses, looking for a job for Jon, and going to many doctors' appointments. The baby's bowels got less and less bright, until it was almost unnoticeable. We hoped and prayed that she wouldn't have CF, knowing it was very likely that she did.

The story of Gwena's birth will follow soon!