Our Great Strides walk in Yorba Linda was today, and it was awesome! Our team, Go Gwena Go!, raised more than $5,135 for cystic fibrosis research. Gwena walked about half a mile and had a great time.

Thank you to everyone who came out to walk with us, and all those who raised money or made a donation to cystic fibrosis research! It really means a lot to us.

More pictures to follow!

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!

No Guilt Here

I think I may be the only CF mom who doesn't feel guilty that my child has CF. (Please note: I am not trying to deny or belittle the emotions of those who do feel that guilt, I just want to share how I do feel about it and why.)

Now, as I've written about before, I do sometimes feel guilty about other things, like her slow weight gain. But the fact that she has CF? I have never felt an ounce of guilt over that fact. But she got the gene from me, right? I passed that mutated gene on to her, giving her CF? Well, that's not the way I see it.

What have I given my daughter? One half of her DNA, 8 months in the womb, 10 months of nurturing and meds and treatments. It's not a stretch or a boast to say that she owes her life to me, as everyone owes their life to their mother, and once over again, as many children with CF owe their lives to those who care for them.

Yes, one of those genes in the DNA I gave her causes CF. But for Gwena, what other options were there? The only other options were never to be conceived, or to die before she was born. I don't think those are better options than living, even living with CF. For me the other possibility was to have a different baby, one without CF. But there is no "Gwena without CF", the odds of conceiving a baby with the exact same DNA as Gwena, minus the CF mutation, are mind-blowingly small. If I had a baby without CF, it wouldn't be Gwena. Gwena is unique and amazing and you know what? She has CF. And I am so glad that she's my daughter.

The other reason I don't feel guilty about Gwena's CF is that while, like all CF parents, I know I didn't choose to pass that gene on to her, I also believe that Someone did. I believe that God designed my daughter and chose every gene in her DNA, including the CF genes. That is sometimes a hard truth to accept, but at the same time I am comforted to know that this was always His will for Gwena's life and my own, and He will work in it for His glory!

Psalm 139:13-16 (NIV)

" ¹³ For you created my inmost being;
you knit me together in my mother's womb.

¹⁴ I praise you because I am fearfully and wonderfully made;
your works are wonderful,
I know that full well.

¹⁵ My frame was not hidden from you
when I was made in the secret place.
When I was woven together in the depths of the earth,

¹⁶ your eyes saw my unformed body.
All the days ordained for me
were written in your book
before one of them came to be."

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!

Organization: Week 1

This is the first of two belated updates I should have written earlier this week!

My first week of attempted organization/cleanliness went pretty well. We got everything cleaned up on Friday (the 9th) before Jon's brother got here to hang out for the weekend. This was nice as it gave me a basically orderly apartment to work with. As of Friday the 16th, one week later, I am proud to say that the apartment actually looked the same, if not better, than the Friday before!

I was able to keep things basically tidy from day to day, and keep up with the dishes. I always want to put this off, but I hope if I force myself to do it every day long enough, it will become a habit. In addition, over the course of the week I managed to do laundry, vacuum, dust, clean the counters, sweep, and mop. The toilet and the stove each got a superficial cleaning, not as thorough as I had wanted to give them. Jobs I had on my list that I didn't get to at all included cleaning the shower, mirrors, and windows.

So it wasn't a perfect run this first week. But it was definitely an improvement over my normal housekeeping performance, so I'm calling it a success!

I also made some headway in the less obvious behind-the-scenes work of organizing things. There is one big thing I've been re-examining because of the book my grandma lent me - where things go. I'm now trying to have a definite place where each thing goes, and to have that place be in the most convenient location.

I'll use Gwena's meds as an example. I used to keep her enzymes in the living room, because that's where I usually feed her. It seems convenient, but there were always empty enzyme capsules all over the coffee table. Now I've realized, even though I feed her in the living room, I get her food ready in the kitchen. So now I've started keeping the enzymes in the kitchen, and pouring them onto the food before I move to the living room. Now the coffee table is much cleaner!

I still have a long way to go before I can call myself an "organized housewife", so we'll see how long I can keep this up. In other exciting news, my grandma says she has another book she wants to lend me! Why am I excited to read about housework??

This Day In History

One year ago today, Jon and I went to the midnight showing of Harry Potter 6, then went home and got a few hours of sleep on the floor of our empty apartment.

One year ago today we woke up, packed the last of our things, did our last minute cleaning, and turned our keys over to our landlord.

One year ago today we got the call telling us that Jon's CF carrier test was positive, that any child we had would have a 25% chance of having cystic fibrosis.

One year ago today, my eyes already red from crying, we boarded the plane for our red-eye flight and said goodbye to Hawaii.

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+!

CF Sucks

That last post was how I think about CF when I'm in my happy and hopeful mode. But I won't lie, as much as I try to keep up a good attitude, I can't do it all the time. CF sucks! As much as I hope Gwena will stay healthy for a long time, I know that she may not. Since I joined the online CF community earlier this year, two girls with CF that I knew of have died, young women in their twenties.

So far for Gwena, CF means enzymes, albuterol, saline, checkups, coughing, Haemophilus influenzae infections... but I have read about all that CF can mean and all that might be in her future. Pseudomonas aeruginosa, Burkholderia cepacia, home IVs, hospital stays, G-tubes, PICCs, Ports, bronchoscopies, sinus surgeries, hemoptysis, oxygen, lung transplants..... so many things and I hope she never has to face any of them.

Yes, I do try to keep a positive attitude, but it's hard to be positive all the time. There is so much to fear that sometimes it is overwhelming, paralyzing. Most of the time I don't dwell on these things because I don't know what the future may bring, and we may never have to worry about any of this. I will do my best to never let Gwena see the times the fears and worries do overcome me, and to focus on being thankful for how healthy she is now, rather than dwelling on the possibilities of her future.

But sometimes, it's hard.

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!

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!