Clinic 9/26/12

Between regular pediatrician visits and the CF Clinic appointments I feel like we're taking her to the doctor every week!  But I would rather be going in for check ups than to be going in because she's sick so I won't complain!

Everything looked good as far as her weight gain goes.  She weighed 13 lbs 12 oz today.  She's taking 6 oz bottles now and we're still adding an extra scoop of formula to increase the calories.  The dietician told us we can start giving her rice cereal now too if we want.

We're almost finished with the antibiotic and she seems to be feeling pretty well lately.  We talked about getting her a flu shot in November since she'll be over 6 months old and we also talked about getting Synagis shots to help keep her from getting RSV.  Dr. R said that while it is important for her to get the Synagis shots, it is even more important for her to be protected from the flu.  I didn't realize how bad it would be if she got the flu but apparently it isn't pretty.  I had already planned on being pretty cautious this winter and making sure she isn't around anyone who is sick but this made me even more nervous!  I would love to keep her in a bubble and never leave the house but I know we can't do that.  So I guess we'll just make sure we all wash our hands 100 times a day and hopefully that will be enough to keep us healthy!

Now for the sad part...

She has to get annual lab work and a chest x-ray so they told us we were going to do that at this appointment.  We went down to the lab and laid her on the table next to SEVEN empty tubes that they were supposed to fill with blood. SEVEN!  Two different nurses and several half-successful attempts later we decided to call it quits and finish the lab work another time.  She was screaming the entire time and it felt like we were torturing her.  Ryan and I couldn't handle it anymore.  They got 5 out of the 7 so we have to try again next month...this momma is not looking forward to it!

4 Months Old

Baby girl turned 4 months old this week.  She is changing so much and really starting to recognize people now.  Every time someone comes up and talks to her she gets so excited and has the biggest smile on her face!

She's figuring out what to do with her hands.  She'll look at them, put them in her mouth, look at them again, turn them back and forth...it's fun to watch her discover new things.  

She soothes herself and puts herself to sleep by sucking her thumb.  It's something she's done since the day she was born.  I tried and tried to get her to take a pacifier but she always went back to her thumb!

Whatever works...    

We spend all of our time together either at work, dropping her big sisters off at school, picking her big sisters up from school, going to the grocery store, running other errands, and she never complains.  She's always happy!

Her check up today was great!

Height: 24 3/4 in.

Weight: 13 lbs 3 oz

First Round of Antibiotics

Well her list of meds has officially become too much for me to keep track of in my head!  Time to get organized!

On 8/31 E started coughing in the mornings and a little throughout the day.  She also had a pretty stuffy nose so I waited it out for 7 days to see if it went away and when it didn't I called the Clinic to talk to the nurse about what we should do.  It wasn't only the cough I was worried about but I could tell she just wasn't feeling well.  She was arching her back and crying quite a bit which for E is not normal.  She never cries!  The nurse decided to increase her dose of antacid and to go ahead and start her on an antibiotic that she will take for 3 weeks.  The antibiotic will knock out any bacteria that is lingering around in her lungs while she's sick.

So now her daily medicines are:

Cephalexin (antibiotic) 3ml 3x/day

Nizatidine (antacid) 1ml 2x/day

AquADEK (vitamins) 1ml 1x/day

1 Zenpep 10k capsule with each bottle

Chest PT 20 min 2x/day

While she wasn't feeling well I carried her around in the sling quite a bit.  I didn't realize it before this but she hasn't been much of a snuggler.  Usually she just lays right down and goes to sleep or plays happily in her bouncer.  But while she was sick she was happiest when she was laying up against me.  I loved having her fall asleep in my arms!

She's been on the antibiotic for a week now and she seems to be feeling better. I think the antacid adjustment helped too.  She's back to her happy self again and getting lots of work done with mommy!

Clinic 8/29/12

We met with E's CF team again this month.  She is doing great and growing like she should be, weighing 12 1/2 lbs now.  No changes on her enzymes or meds...still taking Axid twice a day and 1 Zenpep 10k capsule before meals.  Her last 3 throat swabs have grown staphylococcus aureus, which is one of the most common bacteria found in the lungs of CF patients.  It's not something that needs to be treated, though.  They do the throat swabs to check for pseudomonas aeruginosa because that is the one that causes the most problems.  Luckily we don't have to worry about that one yet!

We signed all of the consent forms for E to participate in a clinical trial called BONUS: Baby Observational & Nutritional Study.  It is a research study conducted by the Cystic Fibrosis Foundation where they look at factors that may contribute to poor growth in babies with CF.  Everything will be done at our regular clinic visits and it is pretty much the same stuff we normally do there anyway.  But instead of weighing her once, for example, they weigh her twice: once for her clinic records and then once for her research records.  Same with her height, pulse ox, throat swab, etc.  They also put her in this pea pod that gives a more accurate measurement of her body composition.  She was so cute in there just looking around and smiling at us through the glass!

When they first mentioned this research opportunity to us, Ryan and I were both very much in favor of doing whatever we could do to help.  I hope that the results of this study will help someone else's baby one day just like the results of other research studies have helped us keep E healthy so far.

More Sweat Tests

After E was diagnosed with CF, Dr. R told us that siblings should always get tested as well.  We put it off for awhile because 1) they seem pretty healthy to us and 2) we've had our hands full lately just getting used to having 3 kids!  I finally found a day that would work where I could take them to the testing center at Children's and my mom could come help me out.

I made sure not to tell my oldest about it too early.  She tends to think too much and I knew if I told her about it she would ask too many questions and freak herself out!  So that morning when she asked me her usual "Mommy, what are we doing today?" I just said "Well, you know how E has Cystic Fibrosis?  We're going to get a test done to see if you have it too.  Then we're going to have lunch!"  

Sounded like a good plan to her!

The girls did so great.  They sat there like big girls and did everything the nurse asked them to do.  She used a machine to stimulate their sweat glands and then wrapped a piece of gauze on their arms that they had to keep on for 30 minutes.  

Two days later the pediatrician's office called to let us know that everything was fine and their results were normal!

Clinic 7/25/12

Today we went to E's 2nd Clinic visit.  At each visit we meet with her CF Team which is made up of two different nurses, a dietician, Dr. R (the Pediatric Pulmonologist), and a few others who are there to teach us about airway clearance and to go over the research study we are participating in.

Needless to say, these are long visits!  But it's great that we have so many people working together to make sure she stays healthy.

For the past week and a half, we have noticed some signs of malabsorption.  E has gone from having one dirty diaper a day to having 7 or 8 of them.  We answered several questions to see if we could figure out the root of the problem:

Were the enzymes expired?  No.

Had they been left in the heat?  No.

Are we using generic enzymes?  No.

Since it wasn't any of those, they decided to put her on an antacid to see if that helps.  Apparently the pancreas not only produces enzymes to help digest food, but it also produces bicarbonate which is a base that helps neutralize acid that travels from the stomach to the small intestine.  If her body is not producing enough bicarbonate, the stomach acid can break up the enzymes before they get to where they need to go.  So now she is taking Axid twice a day to see if that's what the problem is.

Even with all of that going on, she is still gaining weight.  She weighed 11 lbs today!  The dietician said she wants E to be gaining 23 grams/day and right now she's gaining an average of 15 grams/day so if we increase her calorie intake a little more she should be right where she is supposed to be.

The major thing we learned about today was chest physiotherapy, or CPT.  CPT is a method used to loosen the thick, sticky mucus in her lungs so it will be easier to cough out.  We learned how to use this little rubber mallet to do "percussion" on her chest, back and sides.  Basically we use our baby as a little drum for about 30 minutes twice a day!

So far she doesn't mind it but I'm sure we'll have our work cut out for us when she starts moving around more!

2 Months Old

Usually I talk about how time flies and about how my babies are growing up too fast!  And you would think I'd say that I can't believe she's already 2 months old...but that's not the case.  I feel like she's been here forever!  How is she ONLY 2 months old?  I guess it's because so much has happened in the last two months.  So many doctors visits and weight checks and medicine changes and hours of reading each night through the giant pile of CF information I was given. 

It's a good thing she's such an easy baby!  I never would have had time to read with the other two girls at this age!  It feels so strange having a baby that doesn't cry all the time.  I actually get to ENJOY having a newborn right now!  That's something I never thought would happen.  She's been sleeping 8 hours a night for weeks.  She only cries when she's hungry.  She smiles and "talks" to us all the time.  She's the happiest little baby I've ever known.

At her 2 month appointment with the pediatrician, she weighed 10 lbs 12 oz and was 22 1/2 in long. 

Color Me Rad

Several weeks ago, my sister told me about a 5k that some of her friends were planning on running called Color Me Rad.  Everyone wears white and then while you're running you get sprayed with color so you come out at the end looking like a bag of skittles!  She said they found out that the race helps raise money for the Cystic Fibrosis Foundation so we all decided to do it too!  I barely even mentioned it to anyone (I've been a little busy lately) but the word spread and we ended up having several other people sign up to run with us!  I can't even explain how much it means to me that all of these people came out to support a cause that means so much to us.  We have the best friends and family!

Pancreatic Enzymes

We had our first visit to the Cystic Fibrosis Clinic on the 6th of June.  They gave us a ton of information about CF and the types of treatments that E will eventually need. 

We also learned that she will need to take pancreatic enzyme replacements from now on.

Many people with CF have trouble gaining weight because the enzymes that are produced in their pancreas cannot make it to the small intestine where food is digested.  So we are giving her an enzyme replacement that she will take every time she eats.  Once she learns how to swallow a pill she can take it that way, but until then we have to break apart the capsule and pour the medicine out onto a spoonful of apple sauce.  

We are also giving her a daily multivitamin and adding salt to her bottles since people with CF lose a lot of salt when they sweat.

It's not a quick process, but we're getting the hang of it and as long as she gets to eat, she's happy!

Now that she has the enzymes she's gaining weight a lot better and when she turned 4 weeks old she was back up to weighing 9 lbs 8 oz. (her birth weight).

What is CF?

Google will absolutely terrify you, so I wouldn't suggest doing that!  The best source of information is the Cystic Fibrosis Foundation:

What is cystic fibrosis?

Cystic fibrosis is a life-threatening genetic disease that causes mucus to build up and clog some of the organs in the body, particularly the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria (or germs) to get stuck in the airways, which causes inflammation (or swelling) and infections that lead to lung damage.

Mucus also can block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food, which provides important nutrients to help people grow and stay healthy. People with cystic fibrosis often need to replace these enzymes with medicine they take with their meals and snacks, which helps them digest food and get proper nutrition.

How do people get cystic fibrosis?

Cystic fibrosis is a genetic disease. This means that people inherit it from their parents through genes (or DNA), which also determine a lot of other characteristics including height, hair color and eye color. Genes, found in the nucleus of all the body's cells, control cell function by serving as the blueprint for the production of proteins.

To have cystic fibrosis, a person must inherit two copies of the defective CF gene—one copy from each parent. If both parents are carriers of the CF gene (i.e., they each have one copy of the defective gene, but do not have the disease themselves), their child will have a 25% chance of inheriting both defective copies and having cystic fibrosis, a 50% chance of inheriting one defective copy and being a carrier, and a 25% chance of not having CF or carrying the gene.

How does CF affect the lungs?

Normally, the healthy CF gene makes a protein—known as CFTR (Cystic Fibrosis conductance Transmembrane Regulator)—that is found in the cells that line various organs, like the lungs and the pancreas. This protein controls the movement of electrically charged particles, like chloride and sodium (components of salt) in and out of these cells.

When the protein is defective, as in cystic fibrosis, the salt balance in the body is disturbed. Because there is too little salt and water on the outside of the cells, the thin layer of mucus that helps keep the lungs free of germs becomes very thick and difficult to move. And because it is so hard to cough out, this mucus clogs the airways and leads to infections that damage the lungs.

Is there a cure for cystic fibrosis?

Currently, there is no cure for cystic fibrosis. However, specialized medical care, aggressive drug treatments and therapies, along with proper CF nutrition, can lengthen and improve the quality of life for those with CF.

The best way for people with cystic fibrosis to fight their disease is to work with their medical caregivers at a CF Foundation-accredited care center. The care center partners with people who have CF to help keep them in the best health possible.Statistics

• About 1,000 new cases of cystic fibrosis are diagnosed each year.
• More than 70% of patients are diagnosed by age two.
• More than 45% of the CF patient population is age 18 or older.
• The predicted median age of survival for a person with CF is in the late 30s.

If there is one thing I've learned so far, it's that every single CF case is different.  There is no way to predict what will happen with E because there is no way to know when she will get sick, how she will respond to antibiotics or treatments, how well she will gain weight, etc.  We just don't know.  All we can do is our best and hope that will be enough to keep her healthy for as long as possible.