Lainey's Cystic Fibrosis Journey

Just a Coincidence?

On Tuesday evening I was watching my favorite smart alec doctor, House, and working on my student's grades when the phone rang. I though it was 1 of three people: mom, my sister Dayna, or my friend Jenn. To my surprise, it wasn't one of them. There was a woman on the other line telling me about reading my blog. Reading my blog.....is my phone number posted on my blog? No, keep listening...............

Brittney, the woman on the other end of the phone, explained that she had spent a couple hours reading my blog and viewing all of the pictures. Although she was hesitant about calling, she gave it a go. As you read on you will realize that God works in mysterious ways..........His work is never "Just a coincidence!"

Go to google, type in 'infants with cystic fibrosis in gainesville, ga'. Choose the 'images' button and our photo appears in the middle of the screen. Choose the 'blogs' button and we are the first blog listed. This is how the God's plan began to touch our lives.

Brittney has a 3 year old son, Landon, who seems to be in good health. On February 18, 2009 her daughter, Lanie, was born. YES, her name is Lanie! Sounds just like my Lainey, but spelled differently. A couple weeks after Lanie was born, Brittney and her husband received a call about the concerning results of the newborn screening. She took Lanie to have several sweat test, only to receive the confirmation that she indeed does have Cystic Fibrosis. As any mother, Brittney was concerned, overwhelmed, grief stricken, scared, and looking for answers. It is at this time when her mother-in-law did a google search for 'infants with cystic fibrosis in gainesville, ga'. After finding our blog on Lainey's cystic fibrosis, Brittney's mother-in-law insisted she check it out.

Brittney spent several hours checking out our blog and pictures, when she realized something for the first time. She knows my husband, Sid. Brittney and Sid worked together at the nursing home and he had trained her! She had also seen him at the hospital a week prior. She came to our house for Logan's baby shower! She knew she could call us!

Our phone conversation went on for about two hours. We had so much to share, so many similarities. Both of our precious girls are named Lainey or Lanie! Our boys also have "L" names! She lives in Lula which is just a hop, skip, and a jump from Gainesville! We both are committed to finding a cure for CF! I felt the presence of God within our conversation. He brought us together for the much needed support we both desperately need.

Lanie, Brittney's daughter, has had issues with foul smelling and loose stools. Lanie is also constantly looking for more food! After having her stool tested, she was told that there was no fat found! This is awesome for Brittney and Lanie! She knows that Lanie has Delta F508, but the other mutation is unknown. I shared as much information with her that I could think of.........I just had so many questions for her! Although we live very close to each other, we see different pediatricians. Her pediatrician sent Lanie to the Scottish Rite Cystic Fibrosis Center, while our Lainey goes to the Egleston Cystic Fibrosis Center. We spoke about trying to get her Lanie changed to the Egelston location because it is closer, but we aren't sure if Dr. Caplan would be able to keep Lainey and Lanie straight!

Brittney and I plan on working together to raise money for the Great Strides Walk in Athens, Georgia on October 17th. I feel so much better now that I have someone to help me! Any ideas on a team name for our Lainey and Lanie?

God is so good! HE has so many ways, some subtle and other grandiose, to show his love, care, guidance, support, and commitment. I am truly blessed to be HIS child and to have benefited from his sacrifices. Thank you God for bringing Brittney's family and our family together. Through HIM and my blog, I am thankful our story has provided comfort to others as well as created new friendships. HIS plan will bring great things!

DeAnn

Gigi, the Birthday Grandma!

Today, 57 years ago, my mom was born. My mom is truly a miracle. At 1 year old she weighed 10 pounds and couldn't sit up on her own. The doctors were sure that if she lived past her 1st birthday, she would not be able to walk or talk! She was in and out of the hospital her entire first year of life. She had difficulty keeping food down. The doctors told my grandparents that my mom would most likely not make it and they should consider having another child to "soften the blow." My mother came home from the hospital close to the same time her brother was born. She and her brother were over a year apart in age, yet they were the same size. Many people thought they were twins!

Over her childhood, my mother was sick. At one point the doctors told my grandparents that she had Cystic Fibrosis. They began numerous CF treatments, only to discover several years later that she did not have CF.

When my mom was a senior in high school the doctors finally found a hole in one ventricle of her heart. It was determined that her ventricular septal defect (VSD) was unrepairable. Her defect is associated with Eisenmenger's Syndrome. She continued on despite her diagnosis. She graduated from the University of Georgia with a bachelor of science degree in education with a focus on speech pathology. She later went on to get her master's degree in special education.

Due to her health, my mom and dad decided to adopt. After being on the waiting list nearly 7 years, my parents received the call they longed for....a baby was available for adoption. My parents adopted me in July 1980, when I was only 2 months old. Through private adoption, 4 years later, they adopted my sister at a mere 13 days old.

Through the years my mom has been in and out of the hospital numerous times. She has always taken medicine and began using oxygen in 2000. For as long as I can remember, she has had a blueish-purple hue to her face and hands as well as a very distinctive, persistent cough. With the progression of her disease, she has developed secondary pulmonary hypertension. She is also in the early stages of congestive heart failure. Her doctors have had her on breathing treatments, experimental medications, and even Viagra! She was on the list to receive a heart and double lung transplant at Barnes-Jewish Hospital in St. Louis, Missouri, but has since been taken off the list due to her age.

Gigi ~ aka: Diane, Didy, Mom, grandma, wife, daughter, sister (to 4 younger brothers), aunt, teacher, and best friend ~ has WOWed her doctors and has been a true blessing to everyone she meets. She has lived 57 years; some years she has been healthier than others. She taught elementary and middle school for a total of 25 years. She has been a wife to my dad for almost 36 years. She has been an awesome mom for over 28 years. She has been the BEST Gigi for 5 years. She has beat many odds, yet very seldom complains. Every birthday is a huge accomplishment. We are thankful for every moment we get to spend with her.

Lainey is so lucky to have a Gigi that can teach her what it takes to "beat" the odds. To take what the doctors say and prove them wrong! To keep going when the going gets tough. We pray that Lainey will have the same outlook on life as her health is effected by the challenges of Cystic Fibrosis. Lainey has a lot to live up to, but Gigi has been preparing her since birth! Lainey and Gigi are two of a kind....the kind that you could never live without!

Mom, I know pizza and a Dairy Queen ice cream cake may not be the ideal birthday dinner, but we hope you had a great day! Thank you for everything you have taught me. Thank you for all of your love and support. Thank you for your friendship. We hope to have many more birthdays with you. We love you!

With Love,

DeAnn, Sid, Logan, Liam, and Lainey

Liam and Logan helping Gigi blow out her candle!

Happy 57th Birthday, Gigi!

We LOVE you!

Lainey's List

Lainey's List

Life with CF.........

1. Lainey was my third pregnancy in 4 years! I had ALL day sickness for about 4 months and that is when Zofran became my best friend! I gained a lot more weight with her than I did with my other two pregnancies. I had placenta previa and a large bulging blood vessel in my right leg which could be quite painful at times. Other than feeling exhausted (who wouldn't be with a 3 year old boy, 1 year old boy, teaching 21 first graders, and being pregnant), I had an easy 2nd half of my pregnancy. I opted out of all blood tests since my two boys are healthy, plus nothing would have changed my mind about keeping my baby girl! My awesome OBGYN let me see her sweet face on the 3 - D ultrasound machine at each appointment!

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2. Lainey Noelle Smithson was born Tuesday, May 20, 2008 via a scheduled c-section. She weighed 7 lbs. 3 oz. and was 19 1/2 inches long. She was absolutely perfect!

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3. Lainey was great at nursing. She ate and slept....we were loving the nurses waiting on us! The doctors mentioned being concerned that she had not had her first bowel movement...I was excited because I remembered how much I hate the first ones with my two sons! The nurses tried forcing her to drink formula and performed rectal stimulation trying to get her to pass her first stools. She did not seem to be in pain and was not bothered by the attempts from the nurses.

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4. When Lainey was 2 days old, the doctor ordered an x-ray of her bowels. At this point I was still unconcerned because I always had issues using the restroom as a child. She was still acting okay until late afternoon. She began spitting up yellow stomach bile (the nurse told me it was just spit up) and her stomach was distended. It was apparent that she was beginning to feel uncomfortable. The doctor believed that Lainey had a bowel obstruction, possibly atresias (we never saw the blockage in the 3 - D ultrasounds, although her stomach was noticeably larger toward the end of my pregnancy...but my boy's stomachs were large also). They whisked her away to the NICU. She stayed in the NICU in Gainesville for 2 days until the Angel II ambulance transported her to Egleston Children's Hospital.

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5. Lainey had surgery on Sunday, May 25, 2008 on what we believed to be a bowel obstruction, possible Hirschprung's, with the chance of atresias. Although we were nervous, we were sure they would be able to fix her and we would be leaving several days later. The surgery took longer than we anticipated. The doctor informed us that Lainey had meconium ileus and it look like a classic case found in children with Cystic Fibrosis. We were devastated. They had not said anything about Cystic Fibrosis. The meconium was so thick that the doctors were unable to push any through the intestines. They placed a t-tube in her bowels rather than an illestomy, in hopes that it would be enough to drain the meconium.

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6. Lainey was a trooper through her surgery. The evening after her surgery she needed a blood transfusion, but was doing well otherwise. She finally passed her first stool at 9 days old, although it was less than 1/4 tsp! On June 5th (Lainey was 16 days old) I spent the night with her at the hospital. The next morning the doctor came in the room to inform me of her having Cystic Fibrosis; Delta F508 and R560T mutations. I will never forget the feeling of finding out such devastating news after getting such little sleep and not having any family with me at the time.

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7. Lainey was introduced to her enzymes and applesauce for the first time when she was 20 days old. Although having difficulty gaining weight kept her in the hospital longer than we wanted, she was released on June 18th after being in the hospital for 29 days.

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8. Lainey takes one Ultrase MT18 (equivalent to 4 Ultrase) with each meal or snack. She takes 1 ml of Vitamax Vitamins per day, 2 ~ .3 ml of Fer-In-Sol (iron) per day, and 2 ~ Prevacid 15 mg capsules per day for acid reflux. She is also on a 30 calorie Alimentum and Polycose diet (regular formula or breast milk is 20 calories) with 1/4 tsp of salt in each bottle. She drinks approximately 20 oz. of formula per day and eats a bowl of 30 calorie rice cereal with 2 jars of food. We add 1 TBS of vegetable oil per jar of baby food.

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9. Lainey gets 2 ~ 30 minute CPT (chest physiotherapy) sessions per day....and she loves it! :)

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10. Lainey has taken Bactrim 2 times for a green runny nose, but that is the only antibiotic she has taken outside of her hospital stay. She currently receives Synagis shots once a month to help prevent RSV. These shots are given by a home health care nurse who comes to our house...or Gigi's depending on where Lainey is hanging out!

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11. Lainey goes to the Cystic Fibrosis Center at Egleston Children's Hospital in Atlanta once a month and goes for regular checkups at her pediatrician's office here in Gainesville.

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12. Lainey is babbling up a storm, sitting up, scooting, and into EVERYTHING in her reach! She is always smiling, laughing, and overall a very happy 9 month old baby. She has taught a lot of people in our small town A LOT about Cystic Fibrosis and will hopefully continue to spread awareness through her story.

Thanks for keeping up with our sweet Lainey Bug's story!

Here are some pictures of our Lainey Bug from our trip to Target and playing at home!

Our Flower Bug

Bunny Bug!

Happy Bug!

Smiley Bug!

Silly Bug!

Babbling Bug!

Have a good week!

The Smithsons
Sid, DeAnn, Logan, Liam, and Lainey

8 months ago today....

Lainey ~ 2 days old ~ May 22, 2008

8 months ago today was the day doctors told us that our perfect baby girl would need to have x-rays of her stomach. 8 months ago today the nurse force fed our baby in all attempts to produce her first stool. 8 months ago today our baby girl began to spit up yellow bile and show signs of a distended stomach. 8 months ago today I was asked to call my husband so the doctors could talk to both of us about our baby girl. 8 months ago today we were told that our 2 day old baby girl had a bowel obstruction. 8 months ago we were told our sweet baby girl would be taken to the NICU. 8 months ago today our baby girl was ripped out of our arms and whisked away. 8 months ago today our baby girl was severely dehydrated which resulted in fluids being pushed through an IV in her head. 8 months ago today a tube was placed down our baby girl's throat and into her stomach. 8 months ago today we were instructed to wash our hands and arms for five minutes before visiting our baby girl. 8 months ago today we were told that our 2 day old baby would most likely need surgery. 8 months ago today we sent visitors away without seeing our new bundle of joy. 8 months ago today we cried like we had never cried before. 8 months ago today we had to call our family and friends to let them know the status of our precious baby girl. 8 months ago today we prayed more than we had ever prayed before. 8 months ago today our perfect family was changed forever....

Lainey in NICU ~ 2 days old ~ May 22, 2008

Today, 8 months later, we are still changed and forever changing. Lainey has been a blessing to our family. I never imagined how one perfect little baby can make such a change, an awareness, an impact, a difference in and on so many people. Her smile could fool anyone into the falsities of Cystic Fibrosis. Lainey truly makes CF look good! God gave us this perfect child to be our teacher, our leader, our strength, our believer, our endeavor, our light.......and for that we are forever grateful. Thank you to everyone who has helped us over the past 8 months. Thank you to everyone who has prayed for us over the past 8 months. Thank you to everyone who has followed our story for the past 8 months.....and hopefully will continue following us and praying for us for many years to come. We love you!

The Smithsons

Sid, DeAnn, Logan, Liam, & LAINEY

Lainey ~ 8 months old ~ January 2009

Blessings

Who was that overweight man by the Christmas tree?

Now that I am finished stuffing my face with Thanksgiving yummies, I am finally free to update everyone on our most thankful Thanksgiving. Although dad and grandma Lil were sick with the stomach bug (Logan and I began spreading it several weeks ago) and it was cold and rainy, we had a wonderful time camping at Stone Mountain! We arrived and set up our campsite just in time for dinner Tuesday evening. After eating a wonderful meal made by grandma Lil, we were actually able to go to bed by 10:30!! The next morning came quickly along with the news that both dad and grandma Lil were up all night with the stomach bug! With that, our camper became "hang-out" away from sick people!

On Thursday everyone was feeling well enough to eat a full Thanksgiving feast. Dayna, Brad, and Lilly Anne joined Sid, Logan, Liam, Lainey, mom, dad, grandma Lil, grandpa Bob, Uncle Donn, Aunt Jenny, Emily, Erin, Evan, Elizabeth, and I for Thanksgiving dinner. We were like the original Indians and pilgrims ~ eating outside at a long wooden table with a feast of food fixed using numerous methods of preparation! Note * campers have ovens and stoves, but not big or durable enough to handle a Thanksgiving feast. Luckily our campsites were equipped with big grills ~ ideal for our cooking minus the fact we had to do the cooking outside!

After a day full of compiling lists of items I wanted to purchase, I was up and shopping by 7:00 on Friday! Fortunately not many people that live close to Stone Mountain park were interested in shopping! I was able to get everything I wanted and did not even have to wait in line at the register....and I was at Wal-Mart and Target! I saved a lot of money and almost completed my Christmas shopping within a few hours....now for my least favorite thing ~ wrapping!

We were thrilled to have Sid's grandparents, Dick and Juanita, come to visit us in Stone Mountain. On their way to Florida for the winter from their home in Michigan, Dick and Juanita came to see our family and to meet Lainey for the first time! It is always great to see them although it is not as often as we would like! :(

It began raining mid afternoon on Friday and continued through our packing, our trip home, and the remainder of the day on Saturday. We had a wonderful time with our family and camping at Stone Mountain, but it is always great to be back home.

The Smithson Elves

After much convincing from the kids and I, Sid got the decorations out of the attic and began putting up the tree on Saturday evening! I am not sure who is more excited about Christmas this year, Sid and I or the kids! Logan and Liam are really into making Christmas lists this year. Their understanding of Santa and the true meaning of Christmas with the birth of Jesus is more apparent than ever. The innocence of a child at Christmas is priceless.

Buggy Claus!

I have added the video below as a reminder of the things our family is thankful for. This year has been full of emotions ~ happiness, sadness, anger, helplessness, jealousy, fear, awe, wonder, numbness, loneliness, amazement, hopefulness, and so many more emotions that continue to be with our family daily. The support of our family, friends, and people we don't even know, has been the greatest of all blessings. We are eternally thankful for everyone who has and continues to keep our family in their thoughts and prayers. It is our mission to share our story with as many people as possible in order to spread awareness of Cystic Fibrosis. We hope that Lainey will be able to tell her children and grandchildren that she had CF, but with the help of others CF now stands for Cure Found. Please consider making a donation to the Cystic Fibrosis Foundation ~ 90 cents of each dollar donated is used directly for research purposes. Donations in Lainey's honor can be made at: http://www.cff.org/GiftReg/DeAnnSmithson

** Before watching the video, scroll to the bottom of the web page to pause the music. The pause button is on the top left of the music playlist.**

Sincerely,

The Smithsons

Doctor Visits!!!!!....and new pictures!

More illness at our house! Logan was out of school on Friday and Liam should have been....but who wants to take care of a 2 year old that doesn't slow down when he is sick....even when given medication that causes drowsiness?! After convincing the kids into going through the doors of the doctors office (they remembered the blood test for Cystic Fibrosis and didn't even want to go in), we soon found out that Logan had Strep Throat and Liam had Bronchitis! Two co pays and two prescriptions later, we were headed home for a weekend of recuperation.

It is amazing how hard it is to keep the baby away from the boys and even more difficult to keep the boys away from the baby!! Every few minutes Logan wanted to know if it was okay to kiss the baby, since he was feeling better! After a Saturday of not feeling well myself, being home with 3 kids while Sid was working, nursing two sick boys back to health, cleaning the house, cleaning out closets, doing laundry, and watching Georgia "blackout" during the game...I was wishing I hadn't taken the boys to the doctor(or watched the game)!! They were more than feeling better with just one dose of antibiotics and I was worn out!! Whoever had the wonderful idea of making Saturday "Go outside and Play" day on the cartoon station, did not have the moms' of sick children in mind! Although I was feeling better on Sunday, our day was just as crazy...but I managed to take a few pictures of Lainey!

"I hope those boys don't give me what they have!"

On Monday we went to the pediatrician for Lainey's 4 month check-up. She did not have Strep Throat, but her throat was red!! We are praying that red is all she has in her throat...no germs! She weighed 12 pounds 13 ounces...that is a gain of 10.5 ounces in 13 days!! YEA!! Lainey was not very happy to have Dr. Onal checking her out...especially when he was pushing on her belly. She showed him by dirtying her diaper before he was able to check that area! Although we have seen her reach milestones quicker than Logan and even faster than Liam could have ever imagined, she refused to show Dr. Onal! She didn't want to hold her head up, roll over, be on her stomach, "talk," or grab on to his fingers! Mom and I were bummed out that she was not showing off (which is a Smithson trait), but we know what she can do and that is what counts!! After much reading and research, Dr. Onal decided it was safe...or at least that the benefits outweigh the risks...for Lainey to receive the Rotovirus orally. She did not get the first dosage at 2 months due to the precautions...DO NOT take if you have had a blood transfusion (Lainey had one at 6 days old), if you have had bowel obstructions (Lainey had for her first 5 days of life and then some), if you have had bowel surgery (Lainey had at 5 days old), and if you have digestion issues (Lainey's everyday issue). With all of these precautions, it is easily understood why we had an issue giving her the oral vaccine! Dr. Onal did a lot of reading and research, received a thumbs up from Dr. Caplan (the CF doctor), and I said I was comfortable with it, therefore she received her first Rotovirus dosage out of 3. So far, so good. No issues related to the vaccine that we have noticed. She has been running a fever, but we are contributing that to the other 4 vaccines she received! Overall, her visit was very good. Dr. Onal was happy with her progress and her unchanged status of health related to the Cystic Fibrosis. Two hours later, we were all happy...because we were finally getting out of the office!

Lainey liking the view from her Bumbo!

Today at 3:30 I will be having a MRI brain scan. The endocrinologist wants to check for any tumors in the pituitary gland. In addition to the MRI, she also had blood drawn for 15 different tests (one being pregnancy, so I know the answer is "not" an issue, but I am worried about the other 14). I should know results from the MRI and the blood work by the end of the week, or the beginning of next week. For my family's sake, I hope nothing is wrong with me! I think I could handle it personally, but if someone had to take over my responsibilities, I don't know what I would do! On that note, I think I will start a manual to surviving at the Smithson household! :)

Please keep all of us in your prayers! We need good health for everyone and bad germs out of our house! Thank you for everything ~ we count on your thoughts and prayer more than you will ever know!

Much Love,

The Smithsons

"Sitting up is way cooler than always lying down!"



Lainey and Logan watching cartoons!

Mommy's Love Bug!

Have you ever seen a "Naked Bug?" Well, now you have! If you look closely...I mean get real close...you can see her scars from the surgery and t-tube. The scar from the surgery is about an inch above her belly button, about 2 inches long, and parallel to the top of her diaper. The t-tube scar is next to her thumb nail! The doctors did an awesome job, didn't they?!

Relief!

This week has been wild! Saturday Lainey went tailgating for the first time at the Georgia game. She stayed in Gigi and Papa's motor home due to the heat, but didn't miss a thing. She watched everyone and everything going on around her! We had a blast.

On Monday we received a letter and a card stating Lainey's approval for Medicaid through the Katie Beckett/Deeming Wavier. Lainey's Medicaid goes back to the day she was born, meaning every one of her bills is being covered 100%. This is such a relief since she accumulated a number of bills being in the hospital for 29 days. In addition, when receiving Medicaid, you automatically qualify for WIC. After 2 hours of sitting in the local Health Department with some of the children that attend Fair Street School where I work, we received numerous vouchers that are good until November. Each month we will be able to get 8 cans of Alimentum formula. This is approximately $250.00 worth of formula! Since I am a post-partum mom, I receive a box of cereal, 4 bottles of juice, one dozen eggs, 3 gallons of milk, and 2 pounds of cheese each month until Lainey is 6 months old. She will continue to get WIC vouchers until she is 5 as long as we continue to receive Medicaid. This is this answer to many of my prayers!

Along with all of the other wild things going on this week, we have changed Lainey's enzymes again. Now she is taking 2 Ultrase enzymes before each meal. I am not so sure they are any better than what she was on before, but we are willing to try anything to make our princess's life better! It seems as though she is having more bowel movements than before. With each bowel movement, her little bottom gets more raw and bleeds. We have been through more tubes of "butt cream" than we can count! After running out of samples of Ultrase given to us by Lainey's CF doctor, we picked some up at the pharmacy.....and this is where additional WILD things occurred! Somehow the pharmacist misunderstood the doctor when he called in the prescription. The prescription I picked up was Ultrase, but not the dosage she had been taking. Ultrase MT 12 was filled for Lainey. Not paying much attention, I picked up the enzymes from the pharmacy and packed them in Lainey's luggage for her trip with Gigi and Papa. After using the new enzymes for a day, my mom made me aware of the numerous differences in the new prescription when compared to the samples given to us by the doctor. After researching the different types of Ultrase enzymes via the internet, Sid and I realized a major difference. We advised mom to cease the new prescription and continue using the samples. On Thursday I confirmed our concerns with Dr. Caplan. Although he called in a specific prescription, the pharmacist filled it with a similar, yet stronger dosage than prescribed. We were giving Lainey 2 capsules of the new prescription, which gave her 15,000 more units of the active ingredient than needed for someone her size! No wonder mom hadn't changed any poopy diapers! Her doctor said that she was absorbing fat like we want, but the new prescription was also causing her to absorb her own waste! NOT COOL! We are thankful that mom caught this when she did or we could have one sick little girl on our hands. Currently we are still working with the pharmacy to "fix" this mix-up. This has definitely taught me to check all of her medications very carefully before administering them.

This weekend will continue our WILD week as we have been cleaning and preparing our house for Dayna's baby shower on Sunday. Knowing that I am beat when I get home and that I have the kids all day by myself on the weekends, my mother-in-law hired a maid to clean my house! This was an awesome surprise and my house is happy to have had a deep cleaning! In addition to the cleaning, Sid and his mother worked hard to make our yard look beautiful. I find it humorous to know that now I would rather have my house cleaned than go shopping for new clothes!!!

Thursday the boys underwent their Cystic Fibrosis tests. WOW! It took 4 adults to hold Logan down long enough to get 3 vials of blood. Liam was upset for a few seconds and then all was cool! I'm not sure he feels pain! :) It is recommended that siblings of children with Cystic Fibrosis are also tested. Hopefully we will have good news ~ neither boy has Cystic Fibrosis. The test will not only let us know if either boy has Cystic Fibrosis, but will alert us if they are carriers of the Cystic Fibrosis gene mutations effecting Lainey. We should have their results within the next couple of weeks.

Today I received my Cystic Fibrosis test results for last Thursday's test. The results confirmed my contribution of the delta F508 gene. This is the gene all Cystic Fibrosis patients carry. The mutation of the gene can also be another delta F508 or one of the other 1500 + mutations. Since Lainey has one delta F508 and one R560T mutation, we now know that Sid is the carrier of the "odd" mutation. I think we should have known that Sid would have been the one with the "odd" mutation!! The purpose of my being tested was solely for determining which of us carried which gene. In addition to finding out this information, I also learned that my prolactin level is high. My doctor is sending me to an endocrinologist for further testing. I am hoping this helps in finding the cause behind losing my hair, lactating, and several other odd symptoms I have had for a while.

On Wednesday Lainey's has an appointment at the Cystic Fibrosis Center. We are praying that she weighs at least 13 pounds and that her lungs are clear of bacteria. This would be a gain of 2 1/2 pounds. She is really growing in length, but is still skinny! She is finally wearing 0 to 3 month clothes ~ and she will be 4 months old on Saturday the 20th!

Sunday, September 21st is Lainey's Baptism. We are inviting all friends and family who wish to attend to join us at 10:55 am in the Sanctuary of Gainesville First United Methodist Church.

Thank you for all of your prayers! Please continue to pray for us as we receive news on the boys' Cystic Fibrosis test results and in Lainey's appointment at the Cystic Fibrosis Center.

DeAnn

Gaining Weight!!

Today Lainey went to her pediatrician for her 2 month check up. She is doing well in all areas, especially growing! She has gained almost 1 pound in 2 weeks! This is awesome for her since people with Cystic Fibrosis have difficulty gaining weight. She is now weighing 10 lbs. 5 1/2 oz. and is 23 1/4 inches long! She is right on target for her development as well. She is lifting her head and trying to turn over! She has been smiling for a couple of weeks and last week she began cooing! Thank you for all of your prayers. They have helped her as well as our family in so many ways. We will be going back to the Cystic Fibrosis Center on August 18 for another check up. Please pray that Lainey has grown by atleast another pound and that she is free of any infections!

Sincerely,

DeAnn, Sid, Logan, Liam, and Lainey

Lainey's 2nd CF appointment

Hello. Yesterday Lainey (8 weeks old) had several doctor appointments and I am happy to report that she is doing awesome! Our first appointment was with the surgeon. After looking a her incision (from surgery when she was 5 days old) and the site where the t-tube entered her colon, Lainey was given a thumbs up! No more visiting the surgeon unless she were to need future surgeries! Our second appointment was with her team at the Cystic Fibrosis Center. Her team consists of a nurse, respiratory therapist, social worker, dietitian, and doctor. Lainey weighed in at 9 lbs. 6.7 oz! This was a gain of almost one and one half pounds in 3 weeks!! She also grew another 2 inches to make her 23 inches long! Her weight has been a huge concern of ours, thus putting most of our effort into fattening her up! The more weight she gains her first three years of life, determines how well she will do as she progresses in her disease. The team would still like to see her gain 3 pounds per month, but they were satisfied with what she gave them this time! To assist in gaining weight, her enzyme intake has increased to one and one half capsule per feeding. Since the capsules assist in food absorption, they are hoping this will help her to gain even more weight. Anemia is also a concern with Cystic Fibrosis. Since she has been whiter than me (that is hard to believe) indicating low iron levels, her iron intake has also increased. Aside from learning how to perform chest physical therapy for preventative measures, her lungs sounded great! For now, and hopefully forever, her Cystic Fibrosis is only effecting her digestive system. Although she will be on enzymes for the rest of her life, we are hoping to prevent any and all respiratory infections that might reduce her lung function. With the new school year quickly approaching, please pray for my parents as they will be caring for Lainey. Since even the smallest cold could send Lainey to the hospital for a couple of weeks, the doctor strongly suggests keeping her out of daycare for at least one year! Taking care of Lainey will be a big job for my mother since she is in poor health. It will also keep my parents from traveling; something they have enjoyed since dad retired last August. We are fortunate to have them care for Lainey. She is guaranteed to be spoiled rotten at the end of each day! Thank you to everyone for your thoughts, prayers, words of encouragement, and gifts. To learn more about Cystic Fibrosis and how to help find a cure, check out the Cystic Fibrosis Foundation web site at www.cff.org .
Sincerely,
DeAnn, Sid, Logan, Liam, and Lainey

Vacation

Hello! It has been a while since I have updated everyone on Lainey and her progress. We had a wonderful vacation at Flagler Beach just north of Daytona. The five of us spent 9 days in our camper just yards from the beach. We are so lucky to have an awesome family with whom we spent our vacation. My parents, grandparents, three uncles and their families, my sister and brother-in-law, and his family were all together for vacation. The boys absolutely had a blast on the beach! Before we arrived, they had already been at the beach for a week with my parents. Tough on my parents, nice for Sid, Lainey, and I getting to know each other. It was a much needed break from the hospital, doctors visits, and stress of the past month! We came back last Saturday, but our 8 hour trip turned into a two day 12 hour trip! Traveling with 3 kids under four years old, 2 in diapers, 1 that likes to check out every bathroom, and all three that like to eat ~ it was interesting to say the least! Sid began nursing school this week and boy what a transition for all of us! He is in school 4 days a week, then works 12 hour shifts in ICU at NGMC the other 3 days in the week! Our time with him is limited, therefore we cherish every minute...and will until he is finished with school in 2 years!
Lainey gained half a pound while we were on vacation (this is what happens when you hang out with my mom...she likes to feed anyone that will eat!) The doctor has decided that she gains weight better when she is taking only formula, therefore the breast feeding has ceased. I wasn't opposed to that, but the formula is $26 a can and the can lasts for 4 days!! YIKES! We are fortunate to have a very knowledgeable case manager at the Cystic Fibrosis Center. She has helped us to apply for several grants, waivers, and government assistance programs in ways that income is not the deciding factor in obtaining assistance, but is based on the disability and it's longevity. Since Cystic Fibrosis is a lifelong disease that requires many hospitalizations, medications, breathing treatments, possible organ transplants, and leads to premature death (median age of survival is 37 years old), we are confident that we will qualify for much needed assistance in paying hospital bills, doctor bills, co-pays, deductibles, and medications.
Next week will bring new information with the two doctor visits we have scheduled. We are first meeting with her surgeon for a checkup, then we will go to the Cystic Fibrosis Center for her monthly checkup and physical. We are praying that she will be at least 9 lbs. when we go for her visit on Wednesday. The doctors want her to gain 3 lbs. per month, but we will be happy if this month brings us at least 1 pound! We will let you know what the doctors think of her progress after her visit next week. It is amazing how healthy she looks, yet knowing what she is dealing with physically is heart breaking. Please continue to pray for her good health and strength for our family. Thank you for all of the wonderful meals, cards, gifts, and comforting words. We are eternally grateful.
Sincerely,
DeAnn, Sid, Logan, Liam, and Lainey Smithson

Lainey's 1st CF appointment

Today Lainey, Sid, and I went to the Cystic Fibrosis Center at Egleston for Lainey's first CF appointment. Everyone at the center is so very nice and welcoming. We met a few of the people that are par of Lainey's CF Care Team ~ the respiratory therapist, dietician/nutritionist, nurse, case manager, and her primary doctor. Sid and I learned how to perform clapping exercises on Lainey's chest and back in order to break up any mucus in her lungs, used now for preventative measures only. She was weighed in at 8 lbs. 1 1/2 oz. This was a gain of 1 1/2 oz. in 6 days. Not too bad, but we still have to catch up for lost time, therefore we have to pack in more calories! She is now 21 inches long...tall and skinny...she can't possibly be related to us!!! :) The dietitian wants to see her gain at least 3 pounds per month in order to catch up to her height/ weight ratio. She is currently in the 10th percentile for her weight and 50th for her height. It is important that she gains weight, possibly is even chunky for the first 3 years of her life. The more she grows, the better functioning of her lungs. How well she thrives until her 3rd birthday will directly effect her health for the rest of her life. In addition to the extra calories we will be adding to her breast milk bottles, she will also receive 1/8th of a teaspoon of salt. People with CF tend to lose salt through their sweat, therefore it must be replenished through their food. Dr. Caplan, Lainey's CF doctor said that she looked well and checked out to be very healthy! He performed a throat culture to check for any infections that may be brewing. They like to catch anything before it sets up in her lungs. He also collected a stool sample to check for fats. If there are too many fats in her stool, then she will need to go up on the amount of enzymes she takes at each feeding. The enzymes help in fat and vitamin absorption. Lainey also qualifies for free enzymes and vitamins with enzymes until she is 3 years old! This helps a tremendous amount...about $2,000 we will not have to pay out of pocket for these two medications!! Some of the best news of all is that Dr. Caplan said it was okay to take her to the beach on vacation!!! We are so excited that we will all be able to get away for some family time at the beach for a week! Thanks again for continuing to pray for our family. Now we need prayers for weight gain and no harmful bacteria lingering in Lainey's little chest!
Lots of Love,
DeAnn, Sid, Logan, Liam, and Lainey

4 weeks old

Hello! Well....Lainey is still in the hospital at Egleston. &Today she turned 4 weeks old and has been in the NICU at Egleston for 24 days. We are not exactly sure when she will be coming home. A few factors have been keeping her there, such as the t-tube (in her intestines), feedings, weight gain, enzymes, and stools. Today they took her t-tube out, so we are grateful to have one less thing on our list that is keeping her there! Since people with Cystic Fibrosis have a difficult time gaining weight, the doctors are fortifying the breast milk she is drinking. On Friday she had 30+ stools indicating a problem with either her milk, her enzymes, or both. After several stool samples the doctors have decided to go from one capsule of enzymes at each feeding to one half at each feeding. They have also changed her formula fortifier to a more sensitive formula that they are adding to the breast milk. Although she is eating well, she is losing most of it due to the high number of loose stools she is having each day. Hopefully this change in the enzymes and fortifier will do the trick. Once her stools and weight gain are normal, she will get to go home. We have given up on asking when they anticipate her going home ~ they will no longer tell us when, rather they say it is up to Lainey! Please continue to pray! We need prayer for regular stools, finding the correct formula fortifier to create regular stools, determining the correct amount of enzymes at each feeding, and WEIGHT GAIN!!! For all of you trying to lose weight, just send it to her!!! Thanks again for everything you have done. We are hoping to have our princess home soon.
Love,
DeAnn, Sid, Logan, Liam, and Lainey Smithson

Enzymes

Here is the newest update on Lainey. Today Lainey is 3 weeks and 1 day old. She is up to 7 lbs. 12 oz. (she weighed 7 lbs. 3 oz. at birth, and 6 lbs. 12 oz. when we arrived at Egleston). Today the doctors took out the PICC line in her arm, ending her lipids (fats) IV and clear fluids. They also quit the irrigation into her bowels and clamped off the t-tube. They haven't removed the t-tube in case she has any more issues going to the bathroom in the next couple of days. She is up to eating a little over 2 oz. of breast milk with powdered formula added to increase her calorie count. People with Cystic Fibrosis usually have great difficulty gaining weight, therefore the doctors want to add as many calories as possible to her diet. In speaking with the Cystic Fibrosis doctor, we have learned a lot about what the future will and may bring for Lainey and our family. She will have an appointment each month with the Cystic Fibrosis Center. Her doctor is very curious and eager to learn more about her stands of Cystic Fibrosis. People with CF are born with the delta F508 strand and another mutation of the 7th chromosome. Approximately 66% of people born with CF have 2 strands of delta F508. This is one of the worst cases of CF, causing digestive and respiratory problems and eventually causing death from numerous infections in the lungs. For the other 34% of people with Cystic Fibrosis, they have delta F508 and one of the other 1500 + mutations of the 7th chromosome. Lainey has delta F508 and R560T. Her doctor at the CF center looked through the Georgia data base on known CF patients and she is the only patient that has the combination of delta F508 and R560T!! She is also gaining weight...another plus for people with CF, whom usually suffer from "failure to thrive". She is definitely testing the doctor's knowledge and curiosity, as she is unlike any case he has ever had! She also hasn't had any respiratory problems! After performing several tests on her stool, the doctor found from one test that she has too many fats in her stool. These fats are usually absorbed by the body in people that do not have CF. Since she has too many fats, the doctor has decided to put her on enzymes. Starting yesterday and for the rest of her life, Lainey will need to take enzymes before each meal or snack in order to properly digest her food. This has been difficult since newborns can't swallow capsules! Before each feeding I have to break open the enzyme capsule, put the enzyme beads in applesauce, and finger feed the applesauce and enzymes to her!! This takes forever, but I am sure she will catch on soon! Originally we thought she might go home this Friday, but the doctors are saying that it may be Monday or Tuesday of next week. We are just ready to bring our "Lainey Bug" home!! Hopefully she will soon get to meet all of the people who have been helping to bring her home through all of their words of encouragement, thoughts, prayers, and support. We also appreciate all of the people that have called and taken care of my parents (Dwight and Diane Schwartz) with meals and in helping with our boys! Thank you all!
Sincerely,
DeAnn, Sid, Logan, Liam, and Lainey Smithson

Bad News

Hello! I am sorry that I haven't posted an update in a while. My parents have been an awesome help! They spent the $4.75 a gallon on diesel and took the kids and I to Stone Mountain for the week in their motor home. Stone Mountain camp grounds is about 10 minutes away from Egleston ~ much better than the hour plus ride from Gainesville! Being there gave my boys the opportunity to play and have a "mini vacation,"while I was able to spend more time with Lainey. On Monday they began feeding her 5ccs of milk. They also moved her to her own room in the NICU. Having your own room is the last place to move before going home! They have increased her feedings each day, every 3 hours. She has been very sleepy and not waking to feed, thus much encouragement has been needed! The fear of her having a feeding tube has made us pressure her to EAT! Last night was the first night she ate voluntarily, we think because they lowered the amount of IV nutrition she is receiving (and the pressure from us!) We did learn yesterday morning that Lainey does have Cystic Fibrosis. We don't know much about the illness, but we will soon have an appointment with the Cystic Fibrosis Center here at Egleston. The doctor did explain that most babies present the illness with respiratory issues, but Lainey's happened to present itself with digestive problems. Although this is what sent us here (digestive problems due to Cystic Fibrosis), the doctors do not think she will have to take enzymes to breakdown her food ~ at least for the time being. As long as she continues to eat, poop, and gain weight (something that is difficult for CF babies), then she will be on the right track for going home. If Lainey continues to do well, the doctors are hoping to release her on Friday. This has been very hard for us, but we have decided that God must have a plan for us and our new baby girl. Please continue to keep us in your thoughts and prayers. We definitely need them and we have truly felt them in our most desperate times. Thank you all so much for everything.
Much Love,
DeAnn, Sid, Logan, Liam, and Lainey Smithson

Lainey's transfer to Egleston

Hello All. Thank you so much for all of your thoughts and prayers. We are just having to take things one day at a time. Today, Sunday May 25th, Lainey had surgery at Egleston. She is 5 days old and has yet to have her first bowel movement. They believed that there was a spot in which her colon was not connected, therefore she wasn't able to go to the bathroom. After opening her, they realized that everything is connected. This is great because they did not have to cut or sew her colon. The issue that continues to be a problem is that the meconium (the first stool a baby has made from amniotic fluid and stomach bile) is unusually thick. The doctors were unable to manipulate the bowel in a way that would allow the meconium to pass through the colon. They put a tube into her colon to allow them to loosen the meconium with special liquids and for them to be released. This is a step they take before an ilestomy (similar to a colostomy, but it can be reversed). They are hoping that this will loosen things up enough so that she can begin having bowel movements. This will take approximately 4 days to occur. After passing her first few bowel movements, feeding will be presented. She may have to be tube fed if she does not tolerate breast milk or formula. They are calling her condition, Meconium ileus. With what the doctor saw, she thinks Lainey has a classic case of Cystic Fibrosis. A geneticists will test Lainey for Cystic Fibrosis some time this week. We will most likely be at Egleston for 3 weeks, but it could be longer if she has difficulty with feedings. It could also take longer if they find it to be true that she has Cystic Fibrosis. I know this is a lot and maybe more than you would like to take in, therefore you can understand how difficult this has been on our family. Our dreams (and my checklist) of bringing her home from the hospital on Saturday changed into following her in the Angel II transport to Egleston. All we can say is thank you for everything. I know that your thoughts and prayers have helped her and will continue to help her and our family through this difficult time. Thank you again for everything.

DeAnn and family

Meconium ileus: Obstruction of the intestine (ileus) due to overly thick meconium, the dark sticky stuff that is normally present in the intestine at birth and, after trypsin and other enzymes from the pancreas have acted on it, is normally passed in the feces after birth. Meconium ileus results from a deficiency of trypsin and other digestive enzymes from the pancreas, as in cystic fibrosis (fibrocystic disease of the pancreas, mucoviscidosis).

Lainey's Cystic Fibrosis Story

Lainey Noelle Smithson was born on Tuesday, May 20, 2008. She weighed 7 lbs. 3 oz. and was 19 1/4 inches long. My c-section was routine and all went well. Lainey's APGAR scores were high and all appeared to be perfect! We had two days full of hugs, kisses, snuggling, visitors, and pictures!
On May 22nd Lainey's pediatrican became concerned because Lainey had not passed her first stool. X-rays were ordered only to find a blockage in her colon. The doctors quickly rushed her to the NICU. Once in the NICU Lainey was poked, proded, and x-rayed. The doctors determined her blockage may be due to unconnected bowels. Lacking the ability to perform pediatric surgeries, the doctors at Northeast Georgia Medical contacted Egleston.
On May 24th Lainey was taken by the Angel II ambulance to Egleston.Sid and I spoke with the doctor upon our arrival at Egleston only to learn that Lainey would have to have surgery the next morning. We were scared but confident that Lainey's surgery sounded as though it would be routine for the doctors. We were unable to stay with Lainey at Egleston due to the level of care she was receiving in the NICU and the unavaiablity of beds for parents. This was the longest night of our lives!
On Sunday, May 25th, Lainey underwent surgery at the young age of 5 days old. The surgery took approximately 3 and a half hours, but it felt like a life time. Lainey's doctor greeted us in the waiting room to report on Lainey's surgery. The doctor showed us several pictures of Lainey's colon and explained that everything was infact intact. She called Lainey's obstruction Meconium Ileus. The doctor placed a t-tube into Lainey's colon to assist in breaking up the meconium. She also informed us that Meconium Ileus is a classic sign of Cystic Fibrosis. We all fell apart at this news. Since I was adopted, I was unaware of traits I may carry. Sid was sure no one in his family was ever diagnosed with CF. In addition, we already had two perfectly healthy boys!After hours of research, we quickly found that both parents had to be carriers of the Cystic Fibosis mutation gene in order to have a child with Cystic Fibrosis. We decided not to dwell on what she MAY have until we were given proof through blood work. Our goal was to get her well.
On Friday, June 6th, one of Lainey's doctors informed me that she did have Cystic Fibrosis as found through her blood work. She has the gene mutations delta F508 and R560T. I was in shock! I had spent the night with Lainey for the first time since she was wisked away to the NICU, I was tired and totally unprepared for the news of Lainey's diagnosis. Sid and I decided to be proactive and we went to the Cystic Fibrosis Center at Egleston to get some information. While looking around for information we ran into Dr. Caplan. He spoke with us for about an hour and gave us materials to teach about Cystic Fibrosis.Over the course of 29 days in the hospital, we learned more about Cystic Fibrosis than we could have ever imagined. We also learned that Dr. Caplan was unable to find anyone in the Georgia Cystic Fibrosis data base with the same two gene mutations as Lainey. The severity of Lainey's CF was unknown, even through comparison. Her Cystic Fibrosis causes pancreatic insufficiency with malabsorption.
Lainey visits Dr. Caplan at the CF Center every month for a check up and see her peditrican for routine check ups. A home healthcare nurse visits our home once a month for Synagis shots to help prevent RSV. As of now, Lainey does not have any issues with her lungs. We pray that she continues to stay healthy and does not require breathing treatments! Lainey has turned our world upside down, but we would not have it any other way! She is our princess! We are fortunate to have my parents keep her while I teach school and Sid is in school and working in ICU at the hospital. Logan and Liam love being her big brothers! Every day is an adventure! We hope that CF soon stands for Cure Found.Thank you for following our Blog! Check back regularly for details.