I am on Kalydeco!

This weekend I got the FedEx delivery of my dreams - my first shipment of Kalydeco!

I thought it would be fun to log my initial experiences on the drug, so here we go :)

Baseline: CF-wise, my baseline usually consists of a cough from 10:00 AM until around lunchtime. And my nose is usually at least partially stuffed up. On top of that, I have good days and bad days, which I think are triggered by allergies.  On the bad days, I have night sweats and wheezing in the middle of the night, and then I wake up feeling tight and wheezy. And I feel tired and gunky all day - clearing my throat, coughing, and congested. Sometimes I also feel achey, and sometimes I have a sinus head ache.

Day 1 - This particular morning I woke up feeling good after a good night, so it was a sign that I was going to have a good day. The FedEx man showed up at 8:30 AM, and I immediately took my first pill with a scoop of peanut butter (you need to take fat with the medication). Then I headed to the gym.

I did start coughing up some gunk about an hour after my first pill, but that is normal for me at that time of the day. The rest of the day I felt good. I took my second pill with dinner, and again I had a little bit of a cough an hour after I took the pill.

Day 2 - I ended up having night sweats and a bit of wheezing in the middle of the night, and I woke up feeling tight and wheezy. I had my normal mid-morning cough, and I was tired enough to need a nap in the afternoon, (but Grace also woke me up in the middle of the night, so that was at least part of it). My nose was half stuffed-up most of the day. My stomach had little pains off and on.

Day 3 - More night sweats and a tight feelings in my chest. I immediately did an albuterol treatment when I woke up, and I notice it didn't help with the tight feeling right away, like it usually would. But, when I did my hypertonic saline 90 minutes later, (and an hour after taking Kalydeco), I noticed that the hypertonic saline was less irritating than it usually is. I still had my mid-morning cough.

I would say, overall, I haven't yet noticed Kalydeco having an affect, but it's hasn't even been 72 hours, so no big surprise. I am so excited to see how this goes!

Big Kalydeco Day!

Today was another big step forward in getting Kalydeco in my hands! My CF mutations are dF508/R117H-5T. Today the FDA had an advisory committee meeting to discuss the results of the clinical trial of Kalydeco in patients with the R117H mutation. The committee voted to approve it, 13-2! Now I have to wait and see if the FDA will decide to go along with the committee's recommendation, which it usually does. The FDA has until the end of the year to decide.

I was almost in tears listening to the testimonies of people with R117H who have been on the drug. They all said they can't imagine going back to the way they were before Kalydeco. They all said they can now plan their retirements. They all said it was a miracle. I can not believe I might be able to feel that difference soon - to not cough anymore, to not be so tired and achy by the end of the day, to be able to fully breath out of my nose and really smell things again! And to have the confidence that I will be here to help raise my grandchildren :) Crossing everything!

It's Been Forever

I know I dropped off the face of the earth almost two years ago. Life got insane once I had two kids! But, lately I've been thinking about blogging again. When I started this blog about six years ago, it was because I couldn't find much information about pregnancy in women with CF. Now, it seems like that has all changed. There are hundreds of women with CF having babies, which is so awesome! But, I've realized that I am still one of a few in the CF world - almost 40 years old (I turn 38 in a few weeks), with two kids! I think I still have information to share, and I think I may finally have the time to blog again. We'll see how this goes....

So, I think I need to share an update since my last post! My sweet Grace Helen was born on 2/9/13 at 40 weeks 4 days. I went into labor on my own, but I failed to progress and was administered pitocin, which caused such strong contractions that her heart rate was dropping with each one. When her heart rate didn't recover, I was sent for an emergency c-section. That was very scary, but she was born perfect - even had an Apgar of 10!

Here is a picture of Grace from the day she was born:

And this is her now:

Hopefully more info to come! I am excited to get back into this!

Almost 40 Weeks!

I figured I needed to do at least one more post before I had this baby! I'm due in two days, although things are looking very similar  to when I was pregnant with Lily: I started making progress between 36 and 37 weeks, but I've been stuck at 1cm dilated, 25% effaced since 37 weeks. Last week, my OB said, "It looks like you are going to go past your due date again." And we are already thinking I may have to be induced again, which scares me after my labor and delivery experience with Lily.

My OB said I will have three non-stress tests this week, and, if I make it to 41 weeks, I'll either be induced or do daily non-stress testing until I either go into labor on my own or make it to 42 weeks, at which time I will definitely be induced. This is a hard decision for me because I don't want a repeat of last time, but my OB also told me the risks of problems associated with post-term infants starts going up after 40 weeks (which is why it is now standard for OBs to induce at 41 weeks). I'm making Greg come to tomorrow's appointment because I want us to make this decision together.

In the meantime, I am trying every natural remedy that might possibly work. Evening primrose oil, red raspberry leaf tea, acupuncture, sex, squats, riding the elliptical...I even ate chicken parmesan the other night!

The pregnancy has been going well. My lung function only dropped 2% (FEV1 118% at CF clinic three weeks ago), and I've gained over 40 lbs. I didn't WANT to gain that much weight, but it's a good thing in the CF world. I also haven't had any illnesses (:::knock on wood:::). The biggest thing has been that I have been so tired the entire pregnancy - I don't sleep well and I can't survive the day without a nap. And my back has been killing me!

I am feeling very excited to meet this girl!

Long Overdue Update

It's been ages since I've blogged! I'm now almost 28 weeks pregnant and doing well. My only complaints are that I am having trouble sleeping and am tired all of the time. I still take a nap every afternoon, which means I don't get much done around the house, but it's become the new normal around here. Being pregnant while chasing after a toddler hasn't been too bad. Some days I feel like I am just dragging myself around, but it's totally manageable. I am so thankful that Lily still naps.

I've also been having some hip pain. I also had it with Lily, so I decided to see a chiropractor this time, and it has really helped! I don't need 800,000 pillows or an extra mattress topper this time, so that is nice.

I am gaining too much weight (although, in the CF world, that is not really a complaint) - I'm already over 25 lbs. I gained over 40lbs with Lily, and I was hoping to gain less this time, but I guess my body just likes to gain extra weight during pregnancy. I will admit that I am not eating perfectly, but my diet isn't horrible. I am just starving all the time! Oh well, I guess I will just go back on Weight Watchers after this one is born.

CF-wise, I am doing great, for which I am so thankful. However, I am very nervous about how things are going to go once the baby arrives. February through April seem to be my sick times of the year, so it should be "interesting" to see how I do with a new baby and a toddler bringing germs home. I know, whatever happens, we will get through it, but it may suck, especially if I end up needing IVs.

I am planning to look into Kalydeco once this baby arrives, but I am not very optimistic. I am extremely confident that it will work for my mutation, based on the in-vitro data, the fact that Vertex is currently doing clinical trials on my mutation, and the results one woman (who has my mutation) experienced. But, my doctor hasn't seem to keen on the idea. Before I got pregnant, he didn't seem to really want to write the script because it's so expensive, but he said he would because my insurance would cover it and he "didn't want to the be roadblock". However, now my insurance is requiring pre-authorization, so I think my doctor will have to fight to get me on it, and I'm not sure he will be willing to do that. And, even if he is, I'm not sure insurance will cover it.

And, I can't believe I forgot to mention that it is a GIRL!!!! I am so excited to have two girls. I grew up in a family of two girls, and I just love having a girl, so I am really excited to have another. Now we just need to decide on a name!

12 Week NT Scan

Today I am 12 weeks and I had the NT scan. Everything looked good! The baby was very chill - just laying on its side, not wanting to be bothered. I still can't believe everything is going well!

One thing I forgot to mention about the second time around is how I am so distracted by Lily that I almost forget that I have a baby in my belly :)

I'm still very tired and feeling nauseous, but I'm hoping it will get better soon. My hips started aching last week, so I decided to visit a chiropractor. I had my first visit on Monday and I really liked the chiro.  I can't say yet if it helped (my hips felt better when I left, but they ached last night). I'm going to see her again tomorrow so we can talk about my treatment plan.

The Second Time Around

So, can I just say that the second time around has been hard? The first trimester exhaustion has been kicking my butt! I'm not sure if it's actually worse, or if it just feels that way because I have am now a SAHM to a toddler, which means I can't sleep in, sit at a desk all day, or leave work to rest. Lily doesn't go to bed until 9:00, so I rarely get to bed before 10:00. And then I've had insomnia, and I keep waking up to pee. And the sun usually wakes me up at 6:30. On the plus side, I do get to take a nap while Lily does every afternoon, so I'm usually EXHAUSTED until 2:00, but then I feel better until I go to bed (although I still feel very lazy).

I also can't work out like I want to. It's too hot to go for a run (bad for the baby), and I'm too tired to workout while Lily is napping. So we make it to they gym about two days a week. The other days we either have errands or doctor appointments. And, once in a while, I like to do something fun with Lily. I'm also feeling guilty about not being able to do as many fun activities as we usually do.

To top things off, Greg has been traveling for work like crazy.  That just sucks and I hope it gets better by the time the baby gets here.

I've been counting down the weeks until my energy may come back! I'm hoping I'll be feeling better by 14 weeks (although my fingers are crossed for 12 weeks). I'm 11 weeks today, so hopefully there are only three more weeks to go!