"Everyone needs a place to call their own"

Thursday, June 30, 2011

Dry Cough.....New Doctor

I had taken Ayla to her pediatrician last week and he heard a crackling in her lungs.  For someone who doesn't have a "cough" whenever I hear a cough (wet or dry) my mind starts going.  Some people just cough with CF and coughing is good to bring up some of those thick secretions that can get gunked up in the lungs but Ayla doesn't and so if she does it makes me think....

He wanted to take the "wait and see" approach to see if that would get cleared up through coughing.  But as much as we love her Dr he is not a Pulmonologist and when it comes to the lungs they are the experts.  So I took her back to him on Tuesday to see if he still heard that noise and he did.  I left there and got on the phone with Nemours Children's Clinic.

I had been pondering bringing her back to Nemour's instead of Shand's for a few weeks now.  I drive 4 hours to go to Shand's and I have been seeing a student Dr and really don't like that.  Having resident's in the room is all fine and dandy but I would like the person with the credentials to be making judgements about my daughter's condition.  And the benefit of having a specialist locally is very important for reasons above.

So yesterday I took her to Nemour's and saw a new Dr. who had come from AZ.  She has worked in 3 CF centers and even has some patients flying from AZ to book appointments with her (impressive!)!!  She spent a lot of time with us and Ayla really took to her.  I really got the sense that she "gets" CF.
We talked about her cough and any changes that have been made over the last month.  I told her that she was taken off HPTS when we were at Shand's last.  She was having a bad reaction and coughing to the point of vomiting.

She said that she may need to get back on that.  Hypertonic Saline has 2 main benefits:
1. Causing coughing
2. It works to correct the Salt chloride level in the channel (people with CF need extra salt)

But...she said she shouldn't be coughing to the point of vomiting.  Albuterol should work to protect her lungs if given correctly prior to doing the HPTS.  And albuterol given through her spacer (device to administer albuterol) isn't doing a good enough job.  She recommended Nebulized Albuterol.  So between that and the HPTS and Pulmozyme we are up to 3 nebulized meds per dsay, 2 of which are twice a day and the other once a day.

If you know anything about nebulizer's you know that you can't mix meds in the little cups that attach to the hosing, and you have to keep them separate and they must be disinfected after each use.   So for three different meds given multiple times a day and vest therapy to do for an hour a day we are going to be busy folks.  

But whatever we need to do to keep our girl healthy-is considered a pleasure.

The Dr. also ordered a chest x-ray and ct of the lungs if the xray doesn't show what we need to see.  We had that done this morning.  I am no doctor but I thought I could see some scarring.  We will see if I am right when the Dr. calls.  She also did a throat culture-we won't have those results till next week.

I feel like we have a plan in motion and really feel good about the change in Docs.  Now if we can figure out what this noise is and how to treat it we will be good to go!

By the way my child is a TROOPER!  She was not happy about having the culture done yesterday and covered her mouth.  The RT talked her hand away and she agreed to do if she could do it herself.  He put her hand on the swab and he did most of the maneuvering but she really thought she was cool and in control herself.  She didn't shed a tear.  Then today getting the x-ray I stood behind the glass (b/c of the baby bump) and this nice x-ray tech gave her a stool and she stood tall and took orders like a pro.  I was able to see the images so that was cool.  I have to admit she may be getting to big for mommy's liking!

Will update with more information as I get it.

Tuesday, June 28, 2011

Frequently Asked Questions about CF & how it affects our lives...

Often times I get asked different questions about Ayla's CF and how it affects our every day life...and most recently how it will affect our baby due Nov 28th.

Some of the questions I can answer, some I cannot...here are a few I have been asked lately and my best effort at answering them.

Q: Does Ayla have to take pancreatic enzymes forever?
A: Yes, Ayla will have to take these pills every day for the rest of her life, unless a miracle happens.  She takes 3 pills at each meal and takes up to 20 each day!
Q: What does her day look like living with CF?
A: Pretty Normal, the differences are fatty meals for all meals and encouraging high-cal snacking.  She does her Airway Clearance Therapy twice a day for 30 minutes and does an inhaled nebulizer treatment before bed. Unless she is sick then the number of treatments go up.
Q: How will CF affect our unborn child?
A: There is a 25% chance this baby will have CF, a 50% chance this child will be ONLY a carrier (like Larry and myself)
Q: Does any of the routine blood work that my Dr. orders determine if the baby has CF?
A: No, the only way to tell if the baby has CF is to do an Amniocentesis
Q: Am I going to have an Amnio to determine if she has CF?
A: No, I am not going to have this test.  I always thought for sure I would.  But with this pregnancy I have felt differently.  Of course my Dr will be taking extra good care of me and be watchful.  I am going to have her tested when she is born.
Q: Does anyone else have CF in our families?
A: No, as far as we know from testing my mom, sister, Larry and I are carriers.   None of our other family members have been tested.
Q: How did the Dr know Ayla had CF?
A: Bowel Obstructions are a common sign of CF and Ayla had one in utero.  We did not know for sure until she was tested through newborn screening.
Q: What is CF?
A: Click here to read about CF

There are a lot of questions about the future that I can't answer.  We can only do our best to keep Ayla and everyone else in our family healthy and pray for the best.  For now I am just trying to enjoy my pregnancy and the normalcy of it all...

Monday, June 27, 2011

Had To!

My sister Allison (2010) 16 wks

Me 18 weeks!
No we are not pregnant at the same time but I raided her maternity clothes and realized we had both taken this picture around the same time!  Thought it would be funny to compare them~

Isn't my sister GORGEOUS!!???

18 Weeks!

18 Weeks!

This picture Ayla took of me (LOL)

I am trying to be better about posting these pics, I know I will love having them once I am not pregnant anymore!

Changes....hmm....well my nausea has subsided a bit.....I am currently on day 4 with no vomiting spells so that's great!  I have had more energy and more motivation to get out and do things!

Name update: This weekend we may have found THE name for baby girl Tucker....it has meaning, it starts with an A and goes great with Ayla!  I will reveal it soon once we have firmly decided.

Other than that our next check-up in July 8th and I will be 19 weeks and  4 days!  Halfway there!!

I am going to go do Ayla's treatments and get ready for the rest of the day!

Saturday, June 25, 2011

Things are so different so far......

Before I graduated from High School I had a name picked out for my baby girl.  Ayla was so easy to name and I never knew until a few years ago the profound meaning it would have in our lives.  
"Oak Tree"

With this pregnancy I really felt like I was having a boy and had a name and everything all picked out.  Even though deep down I really longed for a sister for Ayla...and I am so grateful she has one on the way!  
But.......I have no idea about a name for this girl........every name I hear-I think they are cute but they don't seem fitting for our second child.

There is a name I like and it has meaning to me but it's taken.  My husband also loves it and us agreeing on names is kindoph been tough.  I call him old fashioned and he calls me crazy!  I like boy names for girls and unique names that you don't hear everyday and he looks at me like i'm crazy..hahah...BUT....I don't want to take someone else's name and that's how it feels.  

This child's name has to have meaning though, b/c of the super special meaning Ayla's name has to us.  Just hearing a name off tv isn't going to cut it...And for some reason my personality won't let me plan anything else (room, colors, etc.) until I have figured this out....

So Suggestions please......

Saturday, June 11, 2011

It's a.........

We are very excited and a little surprised about our news!!  We had everyone telling us this was a boy and we thought so ourselves.  But I have to admit that deep down I wanted Ayla to have a sister at some point.  There is something to be said about a "sisters" bond.  

With that I know that my husband Larry like all men really long to have a son.  Although I'm not sure he's willing to risk having a 3rd wedding to pay for, we could always try again...

We are stumped as far as naming this girl....we had the boy all picked out but I can't decide on a girl name...I had a few in mind just in case...

All of her measurements were perfect and these normal appointments are really beginning to grow on me.  We are praying for this little girl's health and believing she will be CF free!

It's so great to call her a "her" instead of Baby all the time....