Doin' It For Drew - Hawaiian Style

The GREAT STRIDES Walk in Honolulu was this weekend. This is the last walk that we have family walking in this year. Martin's sister, Maureen, and her husband, Bob, live in Hawaii and walked to support Drew and sixty five roses. I think next year we should plan to attend all of these walks to support everyone who is supporting Drew. I wouldn't mind trips to Connecticut, South Carolina, Chicago, San Francisco and Honolulu! Here is a picture of Mo & Bob at the end of their walk.

We couldn't be more proud of and excited by the support that we received this year. We found out in the middle of March that Drew was going to be living with sixty five roses, and in just about 6 weeks we managed to raise over $11,000. It really touches our hearts that we have so many friends and family members who care enough about us and our family to donate to this cause. We are confident that this support will go a long way in funding research to find a cure for this disease. If you are still interested in making a donation, there is a link on the right hand side of this page that you can do so through.

Thank you thank you thank you again for all of your support, and we hope that we can count on you again next year!

On our own!

We survived a week on our own! We had our ups and downs, but we are all still alive and sane...a little tired...but altogether not in bad shape. This was our first week without any parents in town to help us out. The laundry got a little backed up, and dishes certainly weren't being washed with the regularity that Meme had been providing, but the house is still standing. Here are some of the milestones of this week.

The whole family made it to Drew's appointment with the surgeons on Monday. That's right, all 5 of us. Ella was exceptionally (and shockingly) well behaved. Lily slept. Drew did what he needed to do. It was a successful trip in my book. Drew's surgery is scheduled for June 10. We've got our fingers crossed that he doesn't catch a cold or anything before then because they will postpone it.

Tuesday was a good day. We pretty much just hung out around the house. The kids all napped at the same time in the afternoon which allowed me some time to do laundry...and more laundry and more laundry and more laundry. You can't possibly imagine how much laundry two newborn babies produce. Between spitting up on themselves, burpclothes, and our shoulders, we probably go through an average of 3-4 outfits a day (per person), about 30 burp clothes, and a number of swaddle blankets. Luckily laundry detergent was on sale at Kroger this week, so I stocked up.

Wednesday brought some entertainment...in the form of traumatizing fear. Saw a snake today. Almost had a heart attack. To preface this, I will tell you that when I was about 10 I saw a little tiny snake out back at my parents house and didn't go out back for the 15yrs following. I am terrified of snakes. We skip the snake house at the zoo. We change the channel if a snake comes on. I'm terrified. And I always have my guard up fearing that one will show up someplace I don't expect and eat me alive...or something ridiculous like that.

So on this lovely Wednesday morning, Ella really wanted to go out in her pool. We got her into a swim diaper and went outside to spray her down with sunscreen, came back inside to put on her swimsuit and headed toward the door. Thank the LORD Ella didn't run out ahead of me because I think I would have just had to sacrafice her. When I opened the door and looked down to step out, there it was - an oval head, beady little eyes, and a slimy scales body, approximately 6 feet long and about 2-3 inches around. Initial reaction - AHHHHHHHHHHH!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!! MARTIN!!!!! Luckily Martin was working from home this week and was in the basement and heard my blood curdling screams. He admitted that he had thought I was overreacting...until he saw the beast! I was shaking, holding all 3 kids. Ella was crying because I wouldn't let her go outside. And Martin was trying to figure out what to do. He said we could either call someone or he could kill it. KILL IT! KILL IT! KILL IT!! No question. Kill the damn thing! So he went outside with a shovel and after a few fatal blows, managed to solve our problem. This thing was huge. Way bigger than a snake in your backyard should ever be.

He went back to work, and I was not about to head outside. So we went swimming in the bathtub. Martin came back up for lunch and told me that he had looked up online to see what kind of snake it was, and was fairly certain that [my thinking: snakes regenerate a new head after killed? Snakes live in herds? Snakes smell fear and are going to overtake our house???] it was harmless. He said that if it had had a triangular head and jaws that we should have been afraid. If i saw a snake with jaws I would be a goner. He said our snake wasn't big enough to eat anything bigger than a chipmunk, mouse, or other small rodent. (In my head I'm thinking our babies aren't that big!! What if the snake ate one of our babies?!) I told Martin that we needed to call someone for our snake problem and he suggested a psychiatrist. Hopefully this is the first and last snake that we will see.

Thursday we had another doctor appointment. I took the twins and Ella napped at home while Martin worked. Its always a challenge to take both babies out together alone. Its only been done a handful of times but it takes an hour to pack a bag with everything you could possibly imagine you might need and you inevitably forget something. All things told, the appointment went smoothly and we made it home in one piece. The afternoon was a bit of a challenge with everyone waking up at the same time and Ella not being in the best of moods. Martin helped to feed Drew while I nursed Lily and sang songs to Ella.

Then I did something bold, something i had never done before. We needed to drop some labs off at the hospital for Drew, and I decided that I was going to venture out with all three kids by myself. First time! Everyone was fed and happy and I figured that the twins wouldn't even have to get out of their carseats. We made it to the hospital, made it into the hospital, did what we had to do, made it back into the car, and then all hell broke loose. Traffic was horrendous and it took us almost 45 minutes to make the 20 minute trip. Everyone was hungry again, or just wanted to get out of their seats, and screaming at the top of their lungs. It was a long 45 min, but I survived and so did everyone else. Going out with all 3 in tow won't be a regular occurrence for some time, but I know now that I can do it...if I need to.

This weekend will hopefully be reasonably relaxing (KNOCK ON WOOD!). Some friends are coming over for dinner (with dinner) tonight, and Martin is off on Monday. His parents are returning on Monday and his mom will be here until the end of June. It was nice to have a week to ourselves but it will certainly be helpful to have an extra set of hands around again.

We're very much looking forward to getting past Drew's surgery. I will try to post updates on here or facebook throughout his stay in the hospital, as much as time allows. Say a little prayer that all goes smoothly! Happy Memorial Day!

9lbs 4oz

Thats the latest weight! The folks at the sixty five roses clinic are so impressed with his weight gain and that makes us so happy. I hope he continues down this path!

We met with the surgeon this morning and he is ready to schedule the surgery for the ostomy reversal. We should hear from his office by Wednesday to put a date on the calendar. We've heard that it takes a few weeks to "book an OR" so I guess we'll see. We're hoping for sooner rather than later so that we can just get this over with and move on with our summer, but I guess we'll just have to see.

I went to a CF Information Conference at Children's this weekend. I wasn't able to make it for the whole day, but I was there for a good part of it and was glad that I went. There were different speakers talking about things like new airway clearance techniques and different drugs and research in the pipeline. One of the best parts of the day was the panel discussion. There were 5 people who had CF (ages, 15, 17, 18, 24, and 40!) as well as two parents who had children with CF. Their illnesses were to varying degrees, as CF affects just about everyone differently. The people in attendance were just asking questions and there was an open discussion and it was good to hear what they had to say. It gives you hope, seeing some of these kids (and adults) looking and living healthy normal lives. Several people had been through a number of surgeries for different complications due to CF. All of the folks discussed the dedication that they had put into their CPT and medication regimes. The parents on the panel who had children with CF discussed some of the challenges that they faced as their kids dealt with having CF.

There was a young girl on the panel, I think 17 or 18 years old. She was about to graduate from high school, however was not going to be able to go onto college because she was waiting for a lung transplant. She called herself "one of the sick ones". It was sad and it was scary. Her brother also had CF, but she said he didn't really have it. He didn't need to religiously take his meds and do all of his treatments to stay healthy. He was relatively unaffected by the same disease that had her in the hospital about once a month for her whole life, and now she was waiting and hoping for new lungs so that she could continue her battle. She was emotional about it, as was the entire conference room of people.

The hardest part of this disease for me so far is fear of the unknown. I'm scared that he will be sick and in the hospital a lot. I'm scared that Lily and Ella will resent him or resent us for the care we will need to give him. I'm scared that people will be afraid of him because they don't know or understand the disease. I'm scared that people will pity him. I'm scared that they won't find a cure soon enough.

I (we) are trying to keep a positive attitude. All things considered, hes doing great right now and we just have to take it one day at a time. We're working on treating him just like we treat the other two, a normal kid who has CF. CF is not who he is, its just something he has. Hopefully we will continue to have to strength and support of our friends and family to fight this and give Drew the best care possible.

The 40yr old man on the panel was married with two children of his own. At the end of the conference he said that the best advice that he could give, both living with CF and now having children of his own (they don't have it), is to be their parent, not their doctor. Throughout their lives, this disease will have them seeing enough doctors. Although you have to help them with their care and their meds, be their parents and let the doctor be the doctor. So thats what we're going to do.

Here's hoping that this next surgery is quick and easy and we can just start living our new normal crazy lives with twins and a two year old!

Philadelphia GREAT STRIDES Team

The Philadelphia GREAT STRIDES Walk was this weekend at the Philadelphia Zoo. My sister, mom and cousin, and a few friends raised money and walked for sixty five roses. Here are a few pictures. Looks like a beautiful day to be at the zoo!

Thank you Philadelphia walkers!

Yup, he tastes salty

One thing that I'd heard and read about patients with sixty five roses is that they taste salty. "How would one know that" you may ask? Great question! I licked him. When he was in the hospital I licked his head and he didn't taste salty (at least not any saltier than Lily, who I licked for comparison). But the other day I was giving him a bottle and I kissed his head and then happened to lick my lips and he sure does taste salty!

CF is usually diagnosed by measuring the amount of salt in a persons sweat. CF affects exocrine glands. Sweat glands are one kind of exocrine gland. The altered CF gene changes how the body handles salt. More salt is lost in sweat. The amount of sweat is normal, but the salt in it is high.

Normal mucus in thin and slippery. In the airways, it removes dust and germs. It also lubricates the ducts and passageways of other organs, kind of like oil in a car. In CF, the changes in salt transport cause changes in water transport. The mucus loses some of its water and is thick and sticky. This thick mucus blocks tubes and organ passageways.

Sweat tests are done to diagnose CF and they can only diagnose it; they cannot predict the severity. There is no connection between sweat salt level and the severity of the disease in an individual. So just because Drew has days where he tastes salty and days where he does not doesn't tell us anything other than what we already know - that hes got CF.

We give Drew sodium in his bottles to replenish and compensate for what his body loses. Right now, its liquid that we just add to his already not-so-tasty bottles (its no wonder the kid hates feeding time!) but eventually we will just have to pour table salt on all of his food and he will also be encouraged to drink things like Gatorade which contain a lot of salt (would have been nice to know that Gatorade was loaded with salt BEFORE the feet swelling started during pregnancy!)

So for anyone curious, yes, he tastes salty, but I just noticed it for the first time a this week. And please just take my word for it - don't lick my kid.

A Graphic Description of the Ostomy - PROCEED WITH CAUTION

Because I have received so many questions about the ostomy that I complain so much about, I thought I would try to explain more about what it is, what it looks like, and how it works, equpiment and all. I'm more than happy to answer any and all questions you might have. Trust me, no question is too gross or to personal :)

WARNING: THERE ARE SOME GRAPHIC PICTURES BELOW. PROCEED AT YOUR OWN DISCRETION.

So below is a diagram of a persons digestive system. Basically, food passes through a persons stomach, all through the small intestine then all through the large intestine before passing through the colon and eventually being pooped out the anus (i'm just gonna go ahead and call it the butt). Its important that food follows this path for optimal nutrient absorption. The colon basically removes water from our waste so that we don't all have diarrhea all the time.

Drew's large intestine was blocked with meconium (very very thick hard poop that the body can't pass on its own) . This caused the entire large intestine to be very distended. To correct this, they cut into his belly and right above where his colon is (see diagram above) they cut a little slit and pulled out all of the meconium. Here is a picture I found online of what a bowel of a baby full of meconium looks like (WARNING - ITS GROSS!!!!!)

You can see just how stretched out and thin this baby's bowel is from being clogged with this poop. So anyway, they went into his belly and pulled out his intestine and cut a little slit in it to pull out all of this thick hard poop he couldn't pass. Then they sewed him back up and waited for a few days for the bowel to heal because of how stretched thin and worn out it was (distended). Then, a few days later, an x-ray showed that there was air in his belly and it apparently was because the bowel was just worn so ragged trying to deal with the meconium that it couldn't handle the air and pressure once it was all clear and trying to work properly that it blew a hole in it. Here's where the ostomy comes in.

So they rush Drew back into surgery, and snip his large intestine right into two pieces - if you go back up to the diagram at the top, its right about where the word Colon in - they just cut it right into two pieces and poked both ends right out his belly. Here is a picture of what it looks like, only he has two sticking out right next to eachother - each end of the intestine (another gross picture - this isn't his, just some random picture i found online, but his looks just like this X2):

Poop comes out this hole, so you can't just leave it unattended hanging out of his belly. When he eats, his food goes through his stomach, all through the small intestine, and then starts through the large intestine until it gets to where it was cut in half, and then it all comes out this hole (called a stoma). If you hooked both ends back together, then poop would come out his butt, but because the path was cut it comes out this hole and collects in a bag we attach to it. Here's what the bags look like:

So to avoid having poop just sitting on his belly (which is both gross and dangerous), you need to attach this bag to his belly, but just have to cut a hole just the right size for the stomas to fit through so the poop doesn't get on his belly. You can kind of see the little hole that is cut in the bag on the left. We have to have two holes, one for each stoma. Even though poop only comes out the one hole, we still need to keep the other one wet, so it stays in the bag. Cutting these little holes, probably about as big as a finger is around, is a pain in the ass because you have to get them just right. Then there is skincare prep that serves two purposes - one is to protect his skin if the poop does leak and get on it (and we all know that happens!), and the other is to help the bag adhere. So we use this caulk and this other paste stuff and try to get this bag stuck as close and tight around these stomas as possible. I have to admit that its getting better. We're only changing the bag once a day, but its still a royal pain in the ass.

Why does the bag leak so much you may ask? Well, there are several reasons. First, remember that Drew's belly is about 6 inches wide. In this bag that is probably 5 inches wide, we need to cut two perfectly spaced and shaped little holes. We do good, but its hard. Second, the vehicle by which this thing sticks to his belly is quite literally a sticker. When he moves, and babies do nothing but move, it peels up. When it starts to peel up and air gets under it it causes leaks. So we've done just about everything short of stitching it to his belly, and we can now get about 18-24 hours out of a bag. It just is what it is. We've talked to skincare specialists at the NICU. We've talked to wound care experts, people who deal with this kind of thing everyday, and the basic summary is that it just is what it is. The two stomas is kind of part of the problem. A lot of times when doctors do this kind of surgery they knot off the one end of the intestine and leave it inside, so there is just one end of the intestine sticking out (the end that the poop comes out of ).

I found a picture of a baby with one of these stuck to him (see below). Drew's pretty much looks like this too. Just a bag, usually full of liquid poo (remember its not going through his colon so its very very liquid) hanging from his belly. We try to tuck it up in his diaper, but gas also comes out this hole so it fills up with air and if its secured under his diaper it often causes it to leak or burst when it gets to full of air if its all folded up. We also have to use a giant syringe to suction out and measure the poop several times a day. If the bag gets to full it leaks (surprise, surprise). Plus the folks at the sixty five roses clinic need to know how much is coming out to determine whether or not the enzymes are providing the appropriate amount of fat absorption. Once the ostomy is gone we will just have to pay attention to wet and dirty diapers.

Hopefully this answers a lot of questions you might have, and hopefully I haven't grossed you out to much. This is why we can't really take him anywhere until the bag is gone. I know that there are people that live their entire lives with ostomy bags, and I feel for them. But for us and the success that we've had (or haven't) its just way to big a pain in the ass to clean poop off of a 9 week old baby in the back of a car and spend half an our getting the thing caulked back up and a new bag put on. So we'll just keep him home for a few more weeks. The appointment with the surgeon is on Monday and we're hoping we find out when we can have this thing fixed. They said its a simple surgery - open him up, sew the two ends back together, close him up, call it a day. I hope it goes that way :)