My 1st Birthday!

Well I celebrated my first birthday on March 10! It was a bittersweet day because we had a funeral for my great grandma Madeline on the same day. I will miss you great grandma. I thought it was really cool when all the people at the funeral luncheon sang me happy birthday! I was all smiles...big cheese. I didn't like my cupcake...I threw most of it on the floor. It was a dairy free chocolate cupcake and I even gagged on it. Mom is going to try a new recipe for my little celebration with the whole family on Sunday. This time she is going to try vanilla with "butter" cream frosting. The chocolate cupcakes were from a box. I don't really like sweet foods anyhow. I love my liquid meats and my favorite veggie is peas. I am like an old man when it comes to taste. I love savory, salty foods...I need the extra sodium though so that is good. I could use the extra calories from the sweets though!

Mom and Dad didn't have their camera while we were out on my birthday so we don't have little video to share, but there were a couple of pictures from my grandmas camera. Mom and Dad told me they will get a video of me on Sunday.

The first year of my life has been one crazy ride, but I am doing very well now. I am getting my top two teeth and standing up all over the furniture. I think I will be walking soon. I am still a very good boy when I get put down for naps and bed time. I love to stand in my crib and play with my IV pump. I have to be careful though because I tend to wrap myself up in my tubing...kind of scares Mom and Dad. I also have a CD alarm clock that blinks 12:00 and I love to look at it. Just like Tom Hanks had a volleyball named Wilson I have an alarm clock named Sony.

Well I have to get back to my nap...I had to get three shots (Synagis (2) and Prevnar) yesterday and my teeth are killing me!

Life is good~

Ben

Grandpa gums

I can't believe I will be one year old next Tuesday! It has been one heck of a year and I am happy to celebrate on a healthy path. I am down to two days of TPN a week. Hopefully by my next appointment with Dr. Brown (March 18th) I will be able to go off of it. I am still being hydrated with sodium chloride bags the other five days I am not on TPN. I am getting 25 calories per ounce of formula so we went up a calorie per ounce...slowly increasing. I love water so I drink about 6 ounces of water a day to help keep me hydrated and keep my kidney levels down. The more I drink the sooner I will be off of this darn IV hydration. Mom puts two diapers on me at night and a vinyl training pant. I still pee through that sometimes! Too many fluids if you ask me.

Today Mom and I did the TOBI dance! Today was our last day of 28 days on so now we have a break for 28 days before I am back on it. Yee-haw! That saves us about 45 minutes a day. I now only have to do three different breathing treatments a day compared to five. I like that.

I am getting my top two teeth. The other day my gums were bleeding and a piece was hanging off where those ouchy teeth were cutting through.

I love to eat big people food too. I have had chicken, hot dogs, McDonald's hash browns, ground beef, sweet potato fries, hummus, eggs, and sausage. I know some of those foods are not very good for you, but they are high in calories for me. I still eat my veggies and fruit to stay healthy. No dairy products or foods with milk proteins such as hey and casein. That eliminates a lot of fattening junk. There are many ways to substitute and improvise though.

I saw Dr. Nasr yesterday. I weighed in at 19 pounds 8 ounces! I am hoping I will be 20 pounds by my first birthday. They were very happy with my progress. Now that I am going to be one I will be on an every three month appointment schedule with her. My next appointment is scheduled for June.

I can pull myself up and I am getting the itch to walk. I have a walker and I like to try and use that to assist me in walking around the living room.

I think I am going to have my big boy car seats put in the car this weekend. I get to face forward soon too. I am excited for that.

I am off for a walk in my stroller! ~Ben

Crawling Man

Well I haven't posted in a while and a lot has happened, so I will try to give you all a quick re-cap of the last month. I am about 18 1/2 pounds and 28 1/2 inches. I am getting big and can't believe I will be 11 months tomorrow. I had a few trips to the ER last weekend. On Friday the 30th I had an appointment with Dr. Nasr and that went well. Later that night when mom was getting ready to change my broviac dressing she noticed I had a small tear in the outer opening of my broviac. She called the on-call peds GI doc and they said it would be best to take me in to have it checked out because of the risk of infection. We knew the line was obviously weak because of the outer tear so we did not want it to open all the way. We got the the ER at about 8pm on Friday and then we had to be there until 4am. The peds surgeon on call was in a major surgery so we had to wait for him. It took them about 10 minutes to "repair" my line. Sunday night came along and Mom was getting ready to head out to some friends for the super bowl when Dad yelled for her help. I was crawling around on the floor and my line had come apart and I had blood all over. It would spew even faster out of my line because I was crying. Mom quickly washed her hands and tied a knot in my line. This stopped the blood flow. We quickly packed up to make another trip back to the ER. This time Mom talked to the peds GI on call and told them she and Dad wanted the darn thing out. Mom and Dad couldn't help but think...what if my line had come apart while I was sleeping and I bled to death. The risks are outweighing the benefits at this point in the game. Well because it was Sunday they would not take it out because it requires surgery to remove (not considered urgent) and Dr. Brown feels I still need it (more on that later). They did another "repair" and this time it seemed better. I am a mobile man and this makes it all the more riskier since I spend a lot of time on my belly. Also I am hooked up to my IV machine for 16 hours so Mom has to follow me around holding onto the machine and bag of fluid. She said this is silly and something needs to change.

My creatinine levels in my blood were high, so that can mean I am dehydrated. This is why Dr. Brown feels I still need fluids. We are not sure why all of a sudden I have there elevated levels. Because of this Mom draws my blood on Monday along with taking a sample of my urine to the lab. This allows them to do comparative ratios and keep an eye on my levels. The levels that they look at could affect my kidneys if they are high so that is why they don't want to take a risks and want me pumped full of IV fluids. I drink my formula, but the doctors said I must need extra right now because of my conditions. I see Dr. Brown this Wednesday, so hopefully Mom can discuss things further and we can come to a compromise on my care.

I saw an allergist on Monday Feb 2nd. I have had a few reactions to milk products to include butter and yogurt. I vomited on the yogurt and broke out in rashes around my face from both. Right now I have to stay away from products containing milk...that rules out a lot of things. I can't even have Goldfish crackers! I am still too young to get a reliable allergy test. The doctor said we can get blood now to run a few tests, but there are high chances for false negatives. We can do the needle tests when I am bigger. There is also the possibility that I could grow out of it which would be great. Dr. McMorris was who I saw and I really liked him. Mom had asked him if I could have more severe allergies to food because of my short gut since we don't have any history of food allergies in the family. He said it is probably more likely due to my stomach acids being altered from the Zantac and Zegrid I am on. There are always the goods and bads to all medical intervention. I have been on Zantac basically since birth. They put in in my TPN from the time I was born. I am still on it to help make my stomach neutral so the pancreatic enzymes can work better.

I also met with a Nephrologist (kidney doctor) on Friday Feb 6th. They agreed at this point that I needed more fluids based on my lad work. Mom was hoping they might have had something else to add to the reasons why or more of an explanation, but she didn't get what she wanted.

After my five visits to U of M this past week it was nice to take a break from that Maize and Blue place for a weekend. I have a peds well checkup tomorrow to get my Synagis vaccine and then off to Dr. Browns for full day at U of M again. I have yet to not spend over 4 hours there for one appointment. Pack your lunch and put on your crazy hat for that place.

Well life is good overall. I can't wait to see this white stuff melt and the green grow. Mom says the spring is a beautiful time of year and she is ready to put me in a jogging a stroller so we can both get out of hibernation.

Ben

Liquid Meat

Well my surgery that I last posted about was cancelled. I was very happy about that. When Dr. Brown talked it over with Dr. Tittlebaum they decided to wait until I grow more before they put my broviac in further. The line is out a bit, but they felt it was in far enough for now. My echo will be on hold for now as well. They diluted my TPN solution, so the sugar concentration wouldn't scar my veins. I am still getting the same amount of calories from it, but more volume due to the dilution. Hopefully I will be off of TPN soon and that way we could schedule the surgery for removal! I am not growing as rapidly as I was, but I am still gaining. We had to switch to my Dad's insurance and the home nurse we had is not in our new network. Mom is now drawing my blood every Monday and she takes it to the lab at Beaumont. Thankfully she can draw my blood from my line and doesn't need to poke me! One benefit we get from the broviac. I started to eat pureed meats in hopes that would help me get more calories in. Mom mixes olive oil in with it. I really like the meats and so do my cats. They come and cry while Mom feeds me. I get a kick out of it. Mom and Dad tell me my stool smells of garbage now...very rotten garbage. I also got to try freeze dried apple bits and I like those. The only thing is with my short gut...I don't have much travel time for the apples to digest, so they come out whole. I also am trying a new enzyme, Creon 5, because my bum has a horrible rash we thought we would try and see if it makes any difference. Those little enzymes make my skin burn and bleed. Back to trying 50 different creams...from scripts to over the counter. My vitamin D levels were low again so we increased my ergocalciferol from .125ml to .25ml. My vitamin A and E levels were on the low end of the normal range so we have increased my AquaADEK vitamins from 1ml to 2ml's. I am also decreasing my Zantac and adding in omeprazole. These antacids help keep the acid in my stomach low so the enzymes can do their job. I finally off of TOBI for 28 days...that adds about one hour of free time to my days. I like that! We got a call today from Dr. Nasr and my throat culture from last week is showing that I still have PA...maybe the form of bacteria I have is resistant to the TOBI. I have a bit of a cough again too. The culture is not done growing, so Mom will call Dr. Nasr towards the end of the week and we will come up with a plan of action then. Please pray that my ugly PA bugs go away!

Ahhhhh....da da,

Ben

Bundle of new "firsts"

Well I have experienced quite a bit these last few weeks. I got a bunch of gifts from that goofy guy I saw at the mall. I guess he isn't so scary after all. Mom and Dad made me dress up in a few outfits for Christmas like I was in a photo shoot.

I had my first power outage from Sunday morning until Tuesday afternoon. Dad had to go buy a generator because we had to keep all of my medications cold in my med fridge. I also had to be able to do my breathing treatments so we needed electricity to power my compressor. We have to sterilize my nebulizers after each use so we used these Medela micro steam bags in the microwave and had one lamp hooked up for light at night. I didn't know much of a difference, but Mom and Dad said this is how people used to live (minus our bit of power from the generator). Thankfully we had our gas fireplace to keep us warm. I loved seeing all of the candles and flashlights. We even made shadows puppets on the wall.

I was up late for my first New Years Eve to watch the ball drop. I was eating my ba ba while I watched TV and then Mom planted a big smooch on my cheek at midnight. I was whisked right back to bed after the festivities.

Well it is now 2009 and I have a lot more "firsts" look forward to. I will be turning one this year. I can't wait to actually be outside this spring and summer.

I also have some appointments this next week at U of M that I am not looking forward to. I have surgery scheduled on Jan 6th to have my broviac inserted back in to the original spot. It has come out over time. I probably haven't helped it since I like to grab at it any chance I get. Plus I have grown and with my chest cavity expanding that may have pushed it out a bit too. When I am in the recovery room from that surgery they will be performing an echo cardiogram on me. I had a visit with my cardiologist on Dec 22nd and they could still hear the heart murmur that I had when I was born. I have a small Ventricular Septal Defect (VSD) which is basically a hole in my heart. The echo will let the doctors know how big the hole actually is. The pictures will determine if I need surgery or if it will heal over time. I also have my monthly checkup with Dr. Nasr on Jan 7th. I think I have been behaving so she should be happy.

Wishing you a healthy, happy new year!

Ben

Merry Christmas

I wish we could've sent a card and letter to everyone. Unfortunately, we ran out of time and cards.....we thought 160 would be enough. Merry Christmas!!

Christmas 2008
Dear Friends & Family,

We wanted to thank everyone for the incredible support you have given us throughout the past year. Thank you for always asking about Ben. Thank you for the food, hospital visits, e-mails, cards, phone calls, donations, gifts and everything else.

We cannot begin to express what a roller coaster this year was. It was filled with many highs and an unfortunate amount of lows. We’ve questioned many things along the way….our doctors, our strength, our faith. One thing that was never in doubt was the amazing network of family and friends that we have. The outpouring of love has been overwhelming.

Ben is the strongest person we have ever met. He has endured more in his first 9 months than we have dealt with in our lifetime. Throughout it all, he does it with a smile. His attitude is amazing despite the constant annoyances we put him through. He has taught us patience, compassion, empathy and several other things that were lacking. Isn’t it amazing that we’ve learned so much from someone who can’t talk? We are better people because of Ben. He is our hero.
As you can see in the photo Ben is doing great. He is steadily gaining weight and is over 16 lbs. He can stand with support and is cutting his first two teeth.

A dying man, Jim Valvano, gave an amazing speech about 15 years ago. I always loved the portion below. It was something I aspired to do but never really seemed to reach until Ben’s arrival. Ben has given us something special.

“To me, there are three things we all should do every day. We should do this every day of our lives. Number one is laugh. You should laugh every day. Number two is think. You should spend some time in thought. And Number three is, you should have your emotions moved to tears, could be happiness or joy. But think about it. If you laugh, you think, and you cry, that's a full day. That's a heck of a day. You do that seven days a week, you're going to have something special.”

We only wish we could fully express to all of you how much your support and donations mean to us. As Ben fights everyday, it’s people like you that will help make the fight that much shorter. Every day brings us closer to a cure.

You can donate to the Cystic Fibrosis Foundation to make CF stand for Cure Found. Your donations are tax deductible. Ninety cents of every dollar goes directly to research. You can sign up to walk and/or make your donation through the link on Ben’s Blog (website address below). Any amount is greatly appreciated, nothing is too small.

We wish you and your loved ones a Merry Christmas and a Healthy New Year!
With Love, Chris and Kati

Peas with sugar on top

I have been a busy boy. I am now a whopping 16lbs 10oz! I finally hit the 5th percentile for my weight! I am 27 1/2 inches in length. That puts me in the 25th percentile for height. I love eating my sweet potatoes, peas, and carrots. I get butter and brown sugar on top. Who wouldn't like that? I ate my veggies by themselves for the first few times and I still really liked them. Dad says for all of the times I have been "starved" I would probably eat hay.

I am cutting my first two teeth. One is through and the other is cutting through my gums right now. It does not feel very good. I can also stand up with support. I can sit on my own, but I don't really have an interest in crawling at this time.

Mom got a yucky call from the doctors last week. I had a cough so they did a throat culture and I have cultured pseudomonas aeruginosa (PA) (again) and klebsiella oxytoca. It is very important we eradicate the PA because if I were to colonize it that can affect my lung function and cause damage. I have to go back on the inhaled TOBI. I am on it for 28 days and off for 28 days for at least the next year. It takes about 25 minutes, twice a day for this treatment. Mom and Dad have to do a lot of entertaining while I get my treatment.

I am vitamin D deficient. That is common for most people with CF and can be common for people with short gut. Dr. Brown started me on a vitamin D supplement.

I am still on TPN 4 days a week. I have an appointment on December 23rd with Dr. Brown. Hopefully they will be very impressed with my weight gain and they will make the decision to take me off of TPN soon. I no longer get NG feeds at night. No more tube in my nose! Mom gets up with me during the week nights to feed me a few times. Dad helps on the weekends so Mom can get a bit more sleep.

I got to visit with Santa...I am not quite sure about that silly man.

I am a very happy guy. I go to bed so well...I have never fought it. I get excited to get in my crib and snuggle up with my bear blanket. I even play and chat in the morning and let Mom and Dad sleep in a bit.

Happy Holidays,

Ben