Clinic 04/10/13

E had a pretty good clinic visit today!  She's been feeling great for the last month and a half or so.  No more coughing or congestion.  Yay!

She weighed 18 lbs 10 oz and is still 27 in long. 

Her weight is good but the dietician wants us to try to get a few extra calories into her every day.  I really do try to give her an extra bottle most days but she very rarely takes it.  So we'll work on that.

Also, Dr. R mentioned that he wanted us to come in for a throat culture in between clinic visits.  Since she is being seen every two months right now and it will change to every three months after she turns a year old, he doesn't want to risk her getting a bug that we don't know about until it has already made a nice little home in her lungs!  (Those weren't his words but that's pretty much how I understood it!)  From what he told me, this is not something that all clinics do.  But I'm realizing more and more how proactive he is and I think it's great that he wants to know as soon as possible if she cultures pseudomonas so that we can treat it and keep it from causing too many problems.

SmartVest Airway Clearance System

For months I've been looking forward to the day we could get an airway clearance vest for E.  Doing manual chest PT has become quite the challenge lately since she doesn't sit still for long and if we try to do percussion on her chest she just swats our hands away or tries to chew on the percussor (or "bopper" as we've come to call it at our house)!

So at our last clinic visit we asked if she was big enough for a vest and they told us she was!  We went through a long few weeks of waiting for insurance to approve everything and finally got it ordered and shipped last week.

If you have no idea what I'm talking about, this page will help explain how it works.

Basically, she wears a vest that is attached to an air compressor that shakes her chest to loosen the thick mucus in her lungs.

CF Can Be A Lonely Disease

When we were first learning about cystic fibrosis after E was diagnosed, a lot of people asked us if there were support groups where other parents of kids with CF could get together and talk, share stories and resources, etc.  It might be nice for E to have a friend one day who could relate to what she is going through.  Someone who has to do the same treatments and take the same types of medicine and someone who understands how she feels.

However, there are risks involved when two people with CF are in the same room.  There is a risk of one person with CF spreading destructive germs to another person with CF that can lead to severe or worsening lung disease.  Bacteria such as pseudomonas aeruginosa (pseudomonas) and Burkholderia cepacia (B. cepacia) are resistant to many antibiotics which makes them difficult to treat.

The Cystic Fibrosis Foundation released these new changes to their infection prevention and control policy today:

We are writing to let you know that the Cystic Fibrosis Foundation recently implemented a new infection prevention and control policy for all Foundation events, meetings and offices to protect the health of people with CF. We took this step based on increasing medical evidence that the risk of people with CF spreading destructive germs to one another is greater than was previously thought. Our new policy reflects the advice of leading medical experts on this subject.

The health and well-being of people with CF is our topmost concern — it is at the heart of all we do. We have long had infection control guidelines in place to promote the safety of people with CF. With this new policy, we are acting proactively and immediately to reduce the risk of cross-infection among people with CF. 

The key elements of the Foundation’s new policy are:

• At any CF Foundation-sponsored indoor event or meeting, including gatherings like committee meetings, only one person with CF may be present and he or she will be designated in close consultation with event chairs and key event volunteers.
• At Foundation-sponsored outdoor events or gatherings, people with CF need to maintain a distance of at least 6 feet from each other.
• Under no circumstances shall individuals who have ever had a confirmed positive sputum culture for Burkholderia cepacia (B. cepacia) complex attend any CF Foundation events, meetings or offices. 

Just another reminder of how cautious we have to be about germs, not just at these events, but everywhere we go.  And another reminder about how socially isolating this rare disease can be.  

I feel badly for adults with CF that cannot attend some of the fundraising or advocacy events that they used to.  And I hate that we can't get together with other CF families and let our kids play together.  But I'm grateful for all of the people I have "met" over the past few months either through facebook, blogs, or online forums.  I'm glad we have a way to connect and ask questions and to support each other when we need it.  And I hope as E grows up she will have friends who take the time to really understand what she is going through so that she will have the support she needs as well.

Let's Try Something Else

Three weeks ago, E caught a cold or virus that gave her a stuffy nose and a cough.  Since it didn't go away after a week, the protocol is to start an antibiotic to treat her for an upper respiratory infection.  She took Keflex for 2 weeks and the cough is still lingering around.  It's not worse, but it's not gone.  After talking to her CF Clinic nurse today, we decided to start another oral antibiotic - Cipro.  She'll take this for two weeks and if the cough is still not gone, we'll go in for a bronchoscopy. 

So...

Why do we have to put her on all of these antibiotics if it's just a junky cough and some congestion?

Her sisters have the exact same thing and we're not treating them for anything.

Here's why...
Since we all passed around this respiratory virus, we all had coughs and congestion.  The good news for us is that we are able to get rid of the congestion on our own eventually.

E doesn't really get to just "wait and see" if it goes away.  The mucus in her lungs is so thick that it is the perfect place for bacteria to hide out and cause problems.  That's why if a cough lingers around like this, it is usually a sign of a CF flare up in her lungs.  Every time she has a CF flare up, there is the possibility of causing permanent damage/changes to her lungs.

So, if antibiotics and extra airway clearance (Chest PT) are what she needs, then that's what we'll do.
Let's hope this does the trick!

Clinic 02/13/13

CF Clinic....I look forward to it and dread it at the same time.  I like going because she gets checked out by so many different people who are making sure she is growing well and staying healthy.  But seeing all of those people takes a long time!  And it's not so easy to keep a baby entertained when you're sitting in a small room for two hours during her nap time!

But we survived!

I had a lot of questions to ask about giving enzymes before she eats.  E normally eats 3 meals a day (baby food and formula) and then has an extra bottle at some point.  There have been times lately when I wanted to give her a bite of a cracker or something else that I happened to be eating but I hesitated because she hadn't had any enzymes.  I asked about how that would affect her and if it was okay to do that.  They told me that having one bite of something isn't likely to cause any problems, but if she wants to keep eating she needs to take the enzymes or else it could cause her stomach to hurt.  So basically, I don't have to panic if she eats a bite of something without taking enzymes first!  Good to know!

She gained almost 2 lbs since her last visit and weighed about 17 lbs 14 oz today.  

Great weight gain!

Now for the not-so-fun part:

Right after I wrote my last post, E started to get sick.  She caught a cold and had a cough that lasted for about a week so I called and got her started on an antibiotic.  Today was day 9 on the antibiotic and we haven't seen any improvement. 

It's possible that she just has a cold that is lingering around for 2 weeks, but if she's coughing for that long it could be a sign of something else going on in her lungs.  Dr. R decided to keep her on this antibiotic for another week and if there's still no improvement we'll start Cipro (a different antibiotic) and inhaled Tobi.  

Hopefully it won't get to that point, but if so, at least we have a plan.  And that plan could always change once we get the results of her throat culture in a few days.  So I guess we'll just wait and see what happens.

No News is Good News!

I haven't been posting lately because luckily, there is nothing to post!  E has been feeling great!  Somehow she has managed to stay healthy so far through this nasty flu season (knock on wood).

She is eating lots of new foods and gaining weight really well.  The one thing that is so different about feeding a CF baby is having to do the enzymes before she eats.  I remember giving my other girls a bite of whatever I was eating or throwing a few Puffs out to keep them quiet until I could get their food ready.  But with E, I can't just give her a random snack whenever I feel like it.  If she doesn't get the enzymes first, the food just goes right through her.  So I think that has slowed us down a little when it comes to trying new things.  I will get better at it, just takes some adjusting!

We go to Clinic on the 13th so I'll update again then!

Coughs, Colds and Christmas

Last week my two oldest girls started coughing, throwing up, and running fevers.  They both ended up with ear infections and nasty sounding coughs.  I did my best to keep E away from them so that she wouldn't get sick.  I even sent her to spend a few days with her grandparents but she caught it anyway.  Luckily it was just a cold, not the stomach ache and fever.  We started another round of Cephalexin (Keflex) that she will be on for the next 3 weeks and we've increased her CPT.

Even with all of the sickness in our house we had a great Christmas!

Clinic 12/12/12

E has been feeling great since we left the hospital.  This past month she has received her flu shot and her first dose of Synagis.  We're doing everything we can to keep her from getting sick these next few months!

Today was her 6 month clinic appointment.  We talked to them about the possibility of malabsorption over the last few days and decided to increase her enzyme dose.  She will now take 20,000 lipase units of Zenpep (or two 10k capsules) before every meal.  They also increased her dose of Axid to 1 1/2 mL, 2x/day.  This should help her stay on track with her weight gain.

Right now she weighs a little over 16 lbs which is great!

The dietician gave us a list of higher calorie baby foods that we can give her and also suggested adding rice cereal to her fruits and vegetables for extra calories.

We did the Pea Pod and a blood draw for her research study and both of those went really well.  This was a much better lab experience than the last one!

Now her appointments will be spread out to every 2 months so we don't go back again until February.

Home Sweet Home

We're home!!

Dr. R said that we could leave on Sunday instead of Monday if it would be easier for us so of course we said yes.  She is feeling great, no more coughing, and I think we all would like to sleep in our own beds again.  We made a Clinic appointment for mid December so I'm hoping we don't have any issues before then.

The doctor that discharged us today gave us very strict instructions about how to keep her healthy this winter.  She told us to wash our hands ALL THE TIME!  Keep her away from sick people (obviously).  And if someone in the house is sick they should probably go somewhere else for a few days, or send E somewhere else for a few days.  Not sure how that one is going to work out but we'll cross that bridge when we get there.  She also talked about all the holiday parties we will have coming up and said our best bet is to skip them altogether.  I'm a little confused because a few months ago they were telling us not to put her in a bubble, but this is sounding a little bubblish to me.  I guess a hospitalization at 5 months old will change things, though.

Anyway, we're home and so happy to go back to our normal routine.  Thank you to all of our friends and family who have helped us out over the last two weeks.  I don't know how we would have made it through all of this without you!

Don't Get Your Hopes Up

Why do people even say that?  Don't they know that the very next thing you will do is get your hopes up?  It's like saying "don't look down!"  Of course I'm going to look down!

On Monday they tested her Tobra trough level to make sure she didn't have too much of the medicine in her system.  High levels of Tobramycin can be toxic so it is important to monitor it closely.  Her level on Monday was 1.2 which is within the safe zone (under 2.0), but higher than Dr. R expected.  So he said we would test again on Wednesday.  He said that if the level is higher than 1.2 we would probably stop treatment and let her go home...but "don't get your hopes up!"

Now, obviously I don't want my baby to have toxic levels of antibiotic in her system but was there a small part of me that was hoping for a 1.3 so that we could finally get out of this place and go home?  Of course!  But when they checked it today her level was down to 0.8.  So.......we're still here if anyone needs us!