Thursday, September 30, 2010

Celebrity Waiters

We were asked to be a spokes family for an upcoming CF Fundraiser called Celebrity Waiters. More than 30 local sports and media celebrities are scheduled to appear to shake hands, sign autographs, and enjoy lunch with some of their biggest fans. Headlining the event this year are Bengals defensive coach Mike Zimmer, Bengals Linebacker Michael Johnson, and basketball hall of famer Oscar "the big O" Robertson.

We will be speaking together with another family affected by CF. They too have a son with CF, but their son is 26 years old! I'm not sure what we will be talking about just yet. We wanted to show all of the donors how their donations have changed the world for people affected by CF over the past many years. We are meeting next week with the other family to figure out how to say what we want to say to make the biggest impact.

Click HERE to see the official website for the event. We would love to see some of you there. If you can't attend but are interested in making a donation there is also a button for that on the page. You will also see that Drew's picture is on the promotional materials for the event. Hope to see some of you there!

Tuesday, September 28, 2010

Nothing Important

Its been a busy couple of days! This past weekend my parents and sister were in town. We hauled out to Lebanon on Saturday for Applefest. YUM! The weather was perfect and there was a ton of great stuff to see and do and eat. Martin & my dad stayed home with the babies. Later that afternoon we geared up for our big night out sans kids. My sister and Martin and I went to Go Bananas Comedy Club to see Mike Birbiglia. He's a favo comedian and he certainly didn't disappoint. We were drinking beers and laughing away. A good time was had by all. On Sunday I was able to attend a baby shower for a dear friend. She has an incredible family and awesome friends and I had a great time (despite being every so slightly hungover from the night before).

My folks left on Sunday and Martin's sister & family from Oakland, CA arrived. Ruby told me that she wished Ella was her sister. They got along famously, and Otis just bopped around minding his own business, looking for something to play with here and there. We had plans to go to the park on Monday but the rain forced us indoors. We made the best of it by grabbing lunch at Chik-Fil-A and enjoyed their indoor playset. The 5 of us and the 4 of the Davies made for a pretty big lunch crowd but it was good times. We relaxed for the afternoon and then Martin's sister made us dinner. It was definitely a nice afternoon!

The Davies packed up and headed out this afternoon after a quick trip to the park. The girls were definitely disappointed to have to leave one another but hopefully we can find some time to get together again soon.

Wednesday was picture day at Ella's preschool. I can't wait to see how these turn out! It will actually be her first photo that was taken by someone other than me! She really seems to be enjoying school. I haven't seen a ton of artwork or anything but thats because there is an art show at the end of October that they are holding everything for.

Drew and Lily are doing well. Drew said "dada" the other day but won't say it again. Both kids are on the move, rolling everywhere to get whatever they want. And I thought things were tricky with two stationary kids! Drew can't shake the cold he got a couple of weeks ago. Hes got another bronchoscopy this coming Tuesday and if things don't look good they will stick him back in the hospital for IV antibiotics. This stinkin' malacia is causing more harm than it should. He shouldn't have to get put on antibiotics every time he gets a cold, but because of his inability to clear his lungs the way he needs to, things start to grow down there. The surgery made a huge improvement, but there's still the floppy trachea. Hopefully that will get stronger every day and before we know it we will be in the clear. Fingers crossed.

That's all I've got for now. I hope to post some more later. Schedules over the past week or so haven't permitted much free time, but I'm working on getting back into a routine.

Friday, September 24, 2010

A Long Overdue Thank You!

I never wrote thank you notes. I never made phone calls. I never personally got to show my thanks to everyone who did so very much for us over the past six months.

I've never been one to promptly send a thank you note, but they usually come. Not this time, and I'm sorry. The first few weeks that the twins were with us is a blur to me. I've had people ask if I received their gift and I honestly don't know the answer. I have been with people who have said "Oh is that the outfit I got them?". Don't know. I didn't open most of the gifts that came in the mail or arrived otherwise on our doorstep. And if I did I honestly don't remember where they came from. I don't know who made me what for dinner and I frankly don't remember eating it either.

What I do remember is the outpouring of love & generosity that we were shown. I will never forget how that made me feel. We felt loved. We felt supported. We felt cared for. I want to express how much that meant to us. We always knew we had great friends, but we never knew how great. Food came for THREE MONTHS! Three whole months of not having to worry about cooking dinner. The weight that took off of our shoulders was unimaginable. We have closets full of clothes, still, for both babies. The gratitude simply cannot be expressed in words. The love and happiness that filled our hearts when we received your donations to the CFF brought us to tears. I cannot possibly begin to tell you how much this has all meant to us.

And now that life has settled down a bit (knock on wood!), I assure you that I will do a better job going forward of sending out thank you notes to share our appreciation. It has taken us a few months to get it together (and many of you reading this might think 'She's got it together?') but we're getting there. Things are getting better and hopefully will continue to do so. Know that all of your gifts and thoughts and prayers mean more to us than we can express in a blog post or thank you know. Know that we appreciate it all. Thank you! Thank you! Thank you!

Thursday, September 23, 2010

Poop vs Gas

Its hard for a 2yr old to understand. She hears some gas and goes around smelling to see who pooped. She once insisted that daddy needed a diaper change.

I think its fair to say we're officially potty training. Wearing big girl panties and all. And we haven't peed in them once in the past 2 days! We still diaper for naps and bed, but the days are going better. Except that we spend WAY to much time on the potty for gas, or "little poopies" as Ella likes to call them. She'll stop dead in her tracks, and yell at the top of her lungs "POOPIES ARE COMING!!!" and off we run. We aren't wearing any pants these days, just to make things a little easier because from the time she says shes gotta go until the time she actually goes is a very very short time. But we make it. Whenever the poopies are coming we rush in there, plop down on the potty, pull out a magazine (usually This Old House or Cooks Illustrated) and we hear some gas. "Oh, its just little poopies". Are you done honey? (we aren't allowed to watch her and must either close our eyes or leave the bathroom). "Nope, more poopies are coming". She may or may not pee, she washes her hands quite thoroughly, and promptly asks for her M&M (I'm not above bribery). She knows when she has to go, she just doesn't yet know whats gonna come out when she gets there. I think [read:hope] we may be nearing the end of diapering. (knock on wood!)

Another funny Ella moment I had today was at lunch. She asked for peanut butter crackers. Easy! Peanut butter on saltines. Simple as it sounds. So I make her 3 peanut butter cracker sandwiches and put them on her plate. She gives them a good look before picking one up for a bite. "Hmm, these don't taste quite right". Wha?! "I think something's missing". Its peanut butter on saltines. I assure you nothing is missing. These were made exactly the same way I always make them. "No mommy, something is missing. They just aren't quite right." And with that she pushed them away and had a peach for lunch. I love her.

Wednesday, September 22, 2010

I do not know best

I read a blog that I shouldn't read. I haven't in a while, I had give it up. But I checked in today and I wish I hadn't. Its a mom, just like me, but she's loved and lost. She lost her baby to CF and her anguish and pain are unbearable to read about. That's why I had stopped reading. I don't know why I read today.

Drew had a CF appointment yesterday and they were thrilled with the changes in this breathing. They even took him off of one of the inhaled medications and we're going to give that a try. Hes got a cold now though. A GD FREAKING COLD!! GRRR!!! I know I can't prevent it but I want to so so bad. He sounds terrible again, like he sounded before the surgery. Coughing a lot, sounds like hes always got a frog in his throat. I wish I could make it go away.

The thing about going to the CF docs is that they always tell you that you know more than they do. NO I DON'T! I know my son better than you do, but I don't know what to do for him. I don't understand this disease he has. I don't know the difference between a bronchodilator and a corticosteroid and whatever else these different medicines do or benefits they provide. They always say "Well we can try this" or "What do you think about doing that" when what I want them to say is "This is what we need to do". I don't know if I'll ever hear that because no one knows what exactly needs to happen because everyone is affected differently. That's what I hate. I hate that and I hate how much this affects our whole family. Yesterday, I took Drew to the appointment alone because the girls had colds. So I had to wake up Lily early to feed her before I left, and then Drew's nap schedule was all screwed up because of the appointment so when I tried to put him to bed when we got home he woke up Lily, who also has a cold and is miserable. In the evenings when Ella wants to play she is often put off because we have to take care of Drew. We do our best to make sure that one of us gives her some attention because she needs that, but shes noticing how much time we spend with Drew, both at home and at the doctor. It just sucks.

I shouldn't have read the blog today. I'm tired and stressed out and worried again because he has another cold and hes already on antibiotics for this last issue so the last thing he needs is more mucus blocking things up. He has another bronch scheduled for Oct. 5 to check things out and make sure the antibiotics cleared everything up, and if there's still mucus he will go back into the hospital for IV antibiotics. The one thing that the CF docs are all certain about and agree upon is that at this age, eradication of any and all bacteria to the extent possible is best thing to do. I'm hoping for a clear bronch. Thank God its not this week because I know I wouldn't get the news I want.

Monday, September 20, 2010

They've Been Shot

Today was the 6mo. checkup. Everyone looks good with weights in the 60th percentiles and heights in the 40-50th percentiles. Lily's head is still apparently small (30%) but I like it just the same.

Drew wasn't allowed to get his vaccines today because its to close to the time he had his surgery. He was however allowed to get his flu shot. Ella also got a flu shot while we were there, and poor Lily had to get the whole shebang. The twins need 2 flu shots this year so when we go back for round 2 we should be able to get Drew up to speed on all of his vaccines.

Tomorrow we have a CF appointment. We were planning to take the whole gang along since the appointment is at 7:30am, but Ella and Lily have a cold (which Drew thankfully hasn't caught yet), but we didn't think it would be appropriate to take two kids with colds to a doctor where there will be other kids in Drew's situation and if they were to catch a cold, well, we all know what happens.

I'm so tired. I think we're finally beginning to see what life with 3 kids is like. Someone always needs to be changed, be fed, be bathed, be played with, be medicated or be someplace. We definitely had it nice for the past 6mo. with family in town helping us out, though it is nice now that we're all getting to know each other and figure out the best way to run this show.

The babies are getting so big so fast, sitting up and starting to amuse one another, and both highly amused by an ever moving Ella. Ella is doing great too. This is our last week with the air cast before we're home free. Shes walking around great, still with a bit of a limp, but it doesn't hold her back. She loves preschool and I love it for her. Shes sweeter than sugar and just gets prettier everyday.

Two big events are approaching. Our anniversary is coming up in October. I got a sitter for 8pm so we can put everyone to bed before heading out. Not doing anything fancy, not a big anniversary, 4 years. Four years being married, 3 kids, 2 houses and a partridge in a pear tree. The other is the dirty thirty. That's right, I'll be celebrating the first anniversary of my 29th birthday in just a few short weeks. If we had no kids and pockets full of moolah I'd probably be heading to Vegas or to wine country with my best girlfriends. But alas that is not the case. I will probably drink a little to much (thats about a bottle of wine these days) and mope around for a bit. I'm not sad about turning 30 because of all of the wonderful things I've had and done and created in the past 30 years. I'm pretty okay with it, I guess as much as one is really ever okay with the big 3-0.

I've got 3 sleeping kids which never lasts for long, so I'm going to take advantage of that time and make myself a cup of coffee, kick up my feet, and think about how I should be cleaning up from lunch.

Thursday, September 16, 2010

Please Vote!

If you read the blog with any frequency, you probably noticed a new button I added just under the main picture of Drew. Its a button from Pepsi to vote for the Cystic Fibrosis Foundation to win a grant to be used toward a research study for babies. This is so important; more important than you can probably imagine. Let me tell you a little bit about the study and why its important to vote.

Here's the researchers description of the study they are hoping to be able to perform if we win the grant from Pepsi.
"A growing body of evidence supports the importance of intervention in CF lung disease during infancy, in order to potentially delay or prevent irreversible lung disease. Yet, aside from antimicrobial therapies, the CF community has no clinical trial evidence base with which to guide pulmonary therapies in infants and toddlers. Hypertonic Saline (HS) is the most attractive chronic maintenance therapy to investigate in infants because it addresses defective mucociliary clearance, an early step in the cascade of events leading to CF lung disease that is expected to be abnormal prior to the onset of airway infection and inflammation."

I'm going to take things back a step and really try to explain CF to you, mostly using info I've taken off of various websites (thank you Johns Hopkins CF Center website!). Bear with me, it might take a while to get to my point. Here goes!

The cystic fibrosis transmembrane conductance regulator (CFTR) gene codes for a protein also known as CFTR. The CFTR protein resides in the surface of cells which line the lungs, digestive system and sweat glands. The symptoms of CF are caused by the inability of the CFTR to release chloride leading to the production of thick, sticky mucus in specific organs.
In normal cells, the CFTR protein acts as a channel that transports chloride into and out of cells, and regulates the passage of salts.

The CFTR protein is composed of 1,480 amino acids—the building blocks of all proteins—and is inserted into the cell surface or membrane. The protein is made up of a single chain of amino acids that are grouped in five functional regions called domains. Since each domain has a special function when it comes to transporting chloride through the cell membrane, mutations in different domains will cause a range of mild to severe CF symptoms. Mutations in CFTR often affect the three-dimensional structure of the protein and prevent CFTR from reaching the membrane.
The location of the CFTR protein, which is found in several organs, determines where the symptoms of CF occur. The organs that are typically involved in CF are the skin, pancreas and lungs.

The Sweat Gland
Sweat is produced in glands under the skin, transported to the skin surface through ducts, where some of the salt and water are reabsorbed. In normal skin functioning, as sweat moves along the duct most of the sodium and chloride -- the components of salt -- are reabsorbed.

The salt reabsorption process is markedly abnormal in people with CF. Chloride transport is virtually eliminated because CFTR, which is the main way for chloride to enter the apical, or top, surface of cells is defective. Chloride must travel through the CFTR, located on the surface of the duct cells, at the same rate as sodium to maintain a balance of electrical charges. This results in very little sodium and chloride reabsorption, leading to a high salt content in CF sweat -- so high that the sweat chloride concentration is the most reliable single test for CF.

The Lung
The lung’s airways are covered with a thin, moist film called airway surface liquid, or ASL, a salt-containing liquid and a mucus gel layer. The ASL traps bacteria and foreign particles, while cilia on the surface of airway cells constantly move the particles out of the lungs and toward the mouth. This process, called mucociliary clearance, or MCC, is an important defense mechanism that protects the lungs from infection. ASL also contains antiproteases, antioxidants, antibodies and other substances that work together to neutralize or destroy invading organisms without damaging the lungs. In CF airways, decreased chloride transport is coupled with excess sodium transport out of the surface liquid. Since water follows the flow of sodium, the ASL loses volume and the mucus gel layer becomes dehydrated.

The Pancreas
Pancreatic gland cells secrete digestive enzymes that join a bicarbonate -rich fluid secreted by cells lining the pancreatic ducts. This combination forms an alkaline, or non-acidic, mixture which maximizes enzyme function. Enzymes are proteins that speed up chemical reactions; without them, our bodies would grind to a halt. In CF patients, pancreatic gland cell secretion may be normal, while bicarbonate fluid secretion decreases. The two transport pathways at the apical cell membrane involved in bicarbonate secretion, and the way the CFTR interacts with these exchangers, are under investigation.

Although CF was originally described as cystic fibrosis of the pancreas, pulmonary problems are the leading cause of disability and early death. The mechanism of lung injury is twofold:

• Chronic bacterial infections occur because thick secretions are not well removed from the lung. Alterations in the lung airway surface liquid (ASL) affect the body’s natural antibacterial defenses.

• Inflammation causes continuing injury to the lung that leads to scarring and loss of function. This process is exaggerated because CF cells produce inflammatory chemicals called mediators, even in the absence of infection.

The primary site of lung injury is within small airways called bronchioles. Think of the anatomy of the human airway as an inverted tree -- the trachea is the hollow trunk and the bronchioles the smallest stems before the leaves. The outer wall of the ‘trunk’ is comprised of connective tissue and muscle that gives shape and structure, but is flexible to allow coughing. The inside layer is covered by epithelial cells containing CFTR and other ion channels, cells with cilia and cells that secrete components of the ASL. In CF, the ASL is abnormally thick and viscous because it contains thick mucus, bacteria, neutrophils - white blood cells attempting to kill the bacteria - and cell debris like DNA remnants from white blood cells. The blockages in the smaller airways caused by thickened secretions can be made worse by swelling of the lining of the airways from constant inflammation.

The thick, dehydrated mucus that obstructs bronchioles sets off a vicious cycle of infection and inflammation. Interestingly, the immune response to lung infection in individuals with CF is not impaired and in fact may be too aggressive, leading to lung damage. In healthy lungs, introduction of bacteria results in a rapid influx of neutrophils, or white blood cells, and eradication of the bacteria. In CF patients, however, the production of certain compounds allows the bacteria to escape the normal immune response.

If you look at the images below, you can see in the first the normal looking airways of a person without CF. Imagine that their airways are like breathing through the straw you get with a triple thick milkshake from McD's- big and wide and open. When you get to the bottom picture of infected and inflamed airways like Drew has, you can imagine the airways being more like breathing through a coffee stir straw. Big difference.

Normal Airways

CF Airways with Bacterial Infection

CF Airways with Inflammation

GI Problems

Over 90 percent of people with CF have an impaired pancreas leading to an inablilty to produce enzymes, the chemicals that digest food. Some people with CF have at least one CFTR mutation that allows some production of functional CFTR protein: Their pancreases will be able to produce enzymes. Normally, food proteins are digested by stomach acid and enter the duodenum, a specialized portion of the small intestine that allows pancreatic and gall bladder secretions to mix with the food.

Three Types of Secretions

• Bicarbonate creates an alkaline solution to neutralize the stomach acid.

• Pancreatic digestive enzymes aid in the beakdown and absorption of fats, sugars and proteins from the gut. These work best in an alkaline environment and may become inactive in an acidic environment.

• Bile, produced in the liver and concentrated in the gall bladder, helps with fat digestion and eliminates waste products.

Malabsorption of essential nutrients is characterized in CF by poor weight gain, growth retardation, delayed puberty, and muscle wasting. Patients with CF need to be frequently monitored because nutritional needs change with disease progression and normal growth. To maintain a desirable body weight and prevent or correct nutritional deficiencies, CF patients might have to consume twice as many calories as recommended for healthy children and adults. Increasing the intake of calories can also aid in adequate fat, vitamin, and mineral absorption.

Nutritional therapy helps prevent complications, such as glucose intolerance, intestinal obstruction, cirrhosis, and pancreatic disease. Vitamin and mineral supplements such as the fat-soluble vitamins A, D, E, and K are often used. Since these vitamins use dietary fat is necessary for absorption of these vitamins, high-fat food consumption is often encouraged. Fat is also the most concentrated source of calories and improves the palatability of foods, and with caloric needs so high in CF patients, this becomes essential. Protein deficiencies are most likely to occur during periods of growth and infection.

Hopefully this provided a good explanation of CF. Here's why I need you to vote. The inhaled saline study that the Cystic Fibrosis Foundation is in the running to win a grant for could make a dramatic difference in the life of many infants and children living with CF. Adults with CF already use hypertonic saline (concentrated salt water). By using this therapy, some of the salt lost by CF patients in replaced and allows for the ASL to move more normally throughout the lungs, reducing the risk for infection leading to lung disease. They will not prescribe Hypertonic Saline to babies because there has not been any research done to show its benefits.

The Cystic Fibrosis Foundation is NOT FEDERALLY FUNDED and relies solely on individual and corporate donations to fund its research. I'm not asking for any money, i'm just asking for a moment of your time. We are in the running to win a $250,000 grant that will be used to fund this critical research and without the grant money the study cannot take place. Your votes can help us to win. You can vote every day. It only takes a minute. You can text 102534 to Pepsi at #73774 or you can vote online by following this link - . You can vote everyday now through September 30th. It will only take you a moment but it will allow the many babies with this disease the opportunity to live a longer, healthier life. Please vote!

Tuesday, September 14, 2010

The Two +1

Happpy Half Birthday Babies. Loves you!

Monday, September 13, 2010

Two 6mo. olds

Tomorrow the babies will be 6 months old. I seriously did not think I would live to see the day. Seriously. These have, by far, been the hardest 6 months of my life. Even aside from all of the health issues, if I had 2 healthy twins I think I'd feel about the same way.

I remember taking someone to the hospital for something (Lily for her staph infection at 1 week old, Ella for a broken leg, Drew for some fit of breathing trouble, you know, one of those times) and one of the medical personnel telling me that she had 18mo old twins, a boy and a girl, like I did. I remember asking her if she liked them yet.

The past 6 months have been full of many emotions. Exhaustion, if thats an emotion, is at the top of that list. Confusion is up there too, trying to remember who ate what and when they ate it. I've been forced to become organized lest someone go without dinner. So so very much love. I didn't think I had anymore love to give once I had Ella, but apparently love grows. Stress. Happiness. Sadness. Worry. Elation. Pride.

As each day passes and I long to keep them frozen in time, I like them more and more. I can't say that its fun exactly, but I wouldn't trade it for the world.

Thursday, September 9, 2010


That's right, we're out of the hospital and back at home! It was a big surprise to us too as we were expecting a two week run of IV antibiotics. Instead, we got a 4-5 week course of oral antibiotics. Let me explain.

When Drew had his surgery on Tuesday, they did a culture of the mucus they found in his lungs and it was positive for staph. They decided that trying to eradicate all of the bacteria was the best thing we could do for it, essentially give him a fresh start. They decided that on Friday morning he would get a PICC line (a special IV) that would be used to administer the antibiotics for the 2 week course. On Thursday morning, the doctors came around for rounds and they said that overnight they were able to test the bacteria's sensitivity to a number of antibiotics and that it was not resistant to anything, therefore we could treat this orally and it would be just as affective. They want to save the really strong IV antibiotics for when they really need them. And just like that, they signed the discharge paperwork and sent us on our way! (okay well really it took a couple of hours like it always does, but you get the point).

The antibiotics need to be administered every 8 hours around the clock, pretty strictly. In about 3 weeks we will go back to the hospital for an outpatient bronchoscopy so that they can get another mucus sample and make sure that the bacteria is gone, along with the majority of the mucus. They will also take a look to see how the trachea looks a few weeks post surgery. It had opened up significantly immediately after the surgery, but they are hoping that over time as his cartilage strengthens it will grow to an even better shape.

You seriously would not even know that this kid had surgery just 4 days ago. He's happy as can be and sounds like a new person. I was looking at some pictures that I had taken just the day before the surgery and poor Drew looked so sick. His face was all red and puffy and his eyes tired from the incessant coughing day and night. Since the surgery he sounds better, looks better and is acting better. He is like a new person! I'm so very happy at what a huge success this procedure was. It was a scary one. They're all scary, but this one dealt with two major parts of the body, his trachea and his heart. I'm just so very happy that it all went the way it did and more importantly the outcome. Just so elated!

Here's what else we have going on. Ella got her cast off about a week ago and is starting to get back to walking around like she use to. She kind of looks like an old man, limping around, but missing her walker. Every day it gets better and we realize how nice it was to have her contained by a non-weight bearing cast for a few weeks :) She started preschool last week as well and just loved it. She has such an outgoing and friendly personality and she just loved meeting and playing with the other kids, and talks all about the teachers and everything she learned. I'm happy that we decided to send her this year. Its good for her to get out of the house and have some
stimulation and interaction with other kids her age two mornings a week, and its nice for me to have 2 hours of time with just the twins. While I'm still a bit of a nervous wreck about the germs shes inevitably going to bring home, I'm glad that we stuck with our decision and sent her.

Lily is a rolling fool. You can't take your eyes off of her for 2 seconds. She rolled all the way across the porch this afternoon back to the doorway inside. Her demeanor has improved dramatically, and she is no longer the screaming demon for most hours of the day. Shes got a laugh that could make you smile on your very worst day and her eyes truly twinkle when she grins her huge Lily grin. I think shes gonna be a mama's girl. I'm okay with that :)

We got a new computer. The jerk who "fixed" our sound broke our network card so our old computer was rendered useless. This is my first time with the new machine. So far I'm pleased.

My dad has been here helping us out for the past week while Drew was in the hospital. He leaves tomorrow and its back to just me and the three. Its definitely nice when we have an extra set of hands in the house, just to play with a kid, feed someone a bottle, fold a load of laundry, or any of the other things that go on here in a day.

I'm looking forward to the fall and hopefully finding our grove...finally. I hope that this is the end of the nonsense with Drew for a while. It sure seems like this last week may have changed his life for the better. God willing, with all of the new treatments and therapies out there for CF, he can lead a healthy and happy life just like his sisters.

Wednesday, September 8, 2010

The best part about being in the hospital

The cheesecake. Its not even that good, but its definitely the best part about being in there.

Drew's doctors consider the surgery a huge success!! They were able to lift the artery off of his trachea and allow it to open it a normal size. Its no longer compressed flat. While its still a little floppy from being compressed for so long, its definitely a big round opening now, like its supposed to be. We could instantly tell a difference in the way he sounded and hope that this solves many of his problems. He still has the CF, unfortunately theres no cure for that (yet), but hopefully with a normal sized and shaped trachea he will be able to clear his airways affectively and breathe a little easier.

While they were doing this procedure, they also did another bronchoscopy to look into his airways with a camera to see if the shape changed (it did, dramatically, for the better...incase you didn't understand the previous paragraph :) ). Unfortunately they also found a lot of mucus. A lot of thick, sticky, CF mucus that had been trapped down there. And there's bacteria in it. Its not terrible, none of the really bad CF infections were found, but there was some staph and they want to treat it. They want this to be a total cleansing and clearing new start for Drew, so they have decided to put him on a 2 week IV antibiotic treatment to try to eradicate all of the bacteria and mucus and give him a chance to start new. Tomorrow morning he will get a PICC line (Peripherally Inserted Central Catheter) and thats how they will administer the antibiotic (its basically a fancy IV). We will talk to the doctors in the morning about whether or not we can take him home and have Home Care nurses come so that we can administer this from home rather than having to spend another 2 weeks in the joint. Its looking like we should be able to but will have to take him back toward the end of the 2 weeks so that they can do another bronchoscopy and take out the PICC line. But bringing him home for at least a week would definitely be the preferable option, even if it means being house bound until the antibiotics are done. I guess we'll see.

I'm so happy that we did the surgery. I was nervous and less than optimistic about how effective it would be, but I can see and hear a difference and the hope is that he will just continue to improve. There is still a lot of inflammation and soreness in his airways from all of the coughing and labored breathing for the past several months, but things finally seem to be getting better, and with the antibiotics hopefully there will be even more improvement.

Our computer is back in the shop so I'm just using shared ones to post an update here and there. I'll try to give more updates complete with pictures and descriptions as I get them. Thanks so much for all of the thoughts and prayers - they have and will continue to help Drew and us get through all of this!

Tuesday, September 7, 2010

Hes Out Of Surgery!

More to come, but a bronchoscopy during the surgery showed a dramatic improvement in the shape of his trachea! He's still waking up so we aren't with him yet, but the doctor called this a huge success. Now we just wait to see how he behaves. Ill post more over the next day or two. Thanks for all of the prayers, I think they helped!

Monday, September 6, 2010

The Big Day is Here

I'm not sure why i'm still up and writing this, seeing as though we need to be out of the house by 5:45 tomorrow morning. Prior to our departure, we have 2 babies to feed, 30 minutes of breathing treatments to perform, 30 minutes of CPT to get done, breakfast to eat and coffee to drink. I think we'll be waking up sometime in the 4am hour of the day. I, frankly, don't even like the 7:00 hour. By about 8am I'm okay, but I'm really not a morning person.

I'm sure tomorrow will go fine. Its never easy giving your baby to a stranger and kissing him "see you later". The hand off is scheduled for 7:30. They said that it will probably be about an hour before the actual surgery starts as they have to knock him out, get 2 IV's run (a regular one and one in an artery to monitor blood pressure very closely since they are working so closely with his heart), place an epidural so that his entire mid-section is numb, and put him on a ventilator. Once all of that is done they will do the procedure which they estimate will take 1-2 hours. Then, depending on where they take him, either to recovery before he goes to the floor, or to ICU if they want to monitor him a little more closely because of the breathing issues that have been going on, we should be able to see him within an hour of completion.

I'll try to post a quick note on here at some point tomorrow but its going to be a crazy day. I'll definitely update Facebook when hes out. Please send all happy thoughts and prayers and all other good wishes our way tomorrow that this is a success. Its about time something starts going Drew's way!

Friday, September 3, 2010

Its kind of a big deal

This morning we met Dr. Manning, the director of the Division of of Cardiothoracic Surgery at Cincinnati Children's hospital, regarding Drew and what he has going on. Here's a brief recap of how we ended up here.

Drew has had noisy breathing and a cough for the past several months now. Through a bronchoscopy (camera down his throat) they were able to take a look at his trachea (windpipe) and saw that something wasn't quite right. There were two major issues. The first is the compression they see right where the aorta crosses the trachea. The second is sort of "broken" or misformed cartilage causing the trachea to look funny. I'll touch more on the second issue a little later, but right now I'll try to explain the first. Keep in mind that I'm not a doctor, so if you're reading this and you are a doctor don't criticize.

In your chest behind your breast bone, there is more than just your heart. Your trachea is back there, some arteries, your thymus gland, your esophagus and that’s all I can remember. Anyway, it’s crowded between your breast bone and your spine is my point. Your trachea and my trachea are hard as bone. Babies’ tracheas are soft as butter. Many kids suffer from something called Malacia, which is just some floppiness of the trachea. While they are still little, their already soft cartilage is even softer than normal so when they breathe, rather than the trachea expanding and contracting normally, it doesn’t keep its form and causes rubs against itself a little bit, causing a noisy sound, like you would hear if you had a frog in your throat. Drew has a little bit of this floppiness going on, but additionally, one of the arteries that is back behind the breast bone seems to be pressing on the trachea a little bit. It’s not terribly uncommon, and because of how soft the trachea is at this age still, anything that’s pressing on the trachea will cause some level of compression.

The aorta, one of the main arteries to the heart, crosses over the trachea in all of us. In babies, because of the “crowding” behind the breast bone, sometimes where this crossing takes place there is some level of compression. This is what’s going on with Drew, and here’s why it needs to be fixed. Let me first say that this is not a CF issue. This is an anatomical abnormality that is complicated by the CF, but is not a CF issue. The compression that is being caused by this artery does not allow for proper airway clearance. Drew just coughs and coughs and coughs because this compression is causing sort of a tickle in his throat, and then all of the coughing is creating irritation which is causing more coughing, but when he coughs the trachea just essentially closes for a second and he struggles to breathe. And on and on the cycle goes. So what needs to happen is to move that artery off of the trachea to allow it to remain open the way it’s supposed to be, and the way they do this is with a procedure called an aorta pexi.

On Tuesday, September 7 Drew will have this procedure, the aorta pexi, done. They will make an incision on his left side (the aorta is on the left side of the heart) kind of under his arm and spread his ribs cage to get in there. They will cut out a part of his thalamus so that they can get to his heart. This shouldn’t be a problem because studies have shown that the thalamus’ job is mostly done prior to birth, that it really has no effectiveness once you are born. That said, he will not lose his whole thymus so any unknown purpose of the thymus will be preserved. They will cut a flap in the sack around his heart (his pericardium) and then sew that to the inside of his breast bone. By doing this, they will lift the value off of the trachea and open it up. If you are curious about the order of things in your chest, it goes (front to back) your breast bone, thymus, your heart (arteries and all), your trachea, your esophagus, your spine.

What we know for sure is that by removing part of the thymus and moving the artery closer to the breast bone it will allow a little more space for the trachea and will relieve the pressure of the aorta on the trachea. What we don’t know for sure is how this will help and we won’t know until after the surgery. The reason that we just don’t know is because of problem #2, the broken cartilage. This is the issue that no one has ever seen before and we really don’t know how it’s affecting him, and won’t know until we get problem #1 fixed.

If you take your hands and make a C with each hand and put them together to make a circle, that’s what the cartilage that supports your trachea looks like. Its one nice, big, round piece of cartilage. Now looking at that circle you made with your hands, take one hand and slide the fingers down and in under the other fingers. You make an off shape circle that sort of closes in on itself. This is what’s happening with Drew’s trachea below where the artery is compressing. Its never been seen before and its unclear as to what kind of problem it is. It may not be anything anyones ever seen before, but if its not causing a problem, who cares what it looks like. We will see how this upcoming procedure affects him. With any luck, it will help to open up his trachea so that he can breathe and cough and clear his airways just like you or I, and then our problem will be solved. After this procedure we will just wait. We will wait and see what difference this makes. We will wait months to see if that broken cartilage fixes itself, if he sort of grows out of it, of if its going to be the real problem that needs to be fixed. I hope and pray to God that it doesn’t because the thinking is that he would need tracheal reconstruction which is a major major operation, not done pretty much anywhere else in the country other than Cincinnati, but its not something that we want to have to have done.

So we’ll see what happens on Tuesday. It won’t make things worse. It’s a big deal but its what has to happen so we’re going with it. Keep us all in your prayers for a successful procedure Tuesday and future free of trips to the OR.

Thursday, September 2, 2010

A Few Updates

We have an appointment to meet with a cardiothoracic surgeon tomorrow. This is the doc who would do the surgery to move the artery off of his trachea. After much discussion among many great docs, the feeling is that doing the surgery is the best option, and even though it may not solve the problem, doing nothing is more detrimental to his health. We have to try because the best case scenario is that this will help.

Naturally, the appointment got scheduled at exactly the same time Ella was scheduled to get her cast off. We were able to get a later appointment for Ella so that everyone's needs are met. I think we will probably be scheduling a surgery before we leave there tomorrow.

Lily finally sleeps past 5am. The first day she did it we had to wake her up to go to Drew's doc appointment. The past 2 days she got up at 7:30. That would be awesome if Ella hadn't start waking up at 6am.

In other baby-related news, making my own babyfood has thus far proven to be reasonably easy. The babies only eat about 1 cube a day right now so I don't have to have a ton on hand. I've made squash, sweet potato, green beans, carrots and peaches. They've only had sweet potato and squash but seem to take them alright. After I go grocery shopping I'm going to try making some more fruits. Its not hard to do and its really not terribly time consuming.

Ill try to post an update on Drew after tomorrow's appointment. Cross your fingers that Ella's leg is healed and we don't need another cast!