Frequently Asked Questions

I get a lot of questions about Beckett, so I thought I would write a post answering frequently asked questions. I hope it helps clear some things up!  Unfortunately, we still have several un-answered questions. 

1.  What is his diagnosis?
That is a tricky one.  They are calling it atypical cystic fibrosis, at this point.  Some doctors believe that there is something else going on as well, while others are now pretty confident that this is it.  Most of them have settled into this diagnosis as being our primary answer.  It is his main diagnosis at this point, and it is likely the cause of his health problems-- his failure to thrive and extreme digestive issues.  For more about his diagnosis and the round-about way of getting here--to this diagnosis, you can read this Letter to Beckett that I wrote explaining it all to him. The technical diagnosis has changed from the time I wrote the letter, but I think it is still a great summary of what we are dealing with here. In May, we will take him to the geneticist at Texas Children's for a more complete mitochondrial disease work up as well as one other possibility.

2.  What is Cystic Fibrosis (CF)? --here is how I explained it to Beckett--

Cystic Fibrosis is a disease that affects both the lungs and the digestive system. For you, it seems to drastically affect your digestion with less lung involvement. Usually in CF, the mutated gene tells the body to make a defective form of a protein called CFTR. The CFTR is in your lungs, digestive system, sweat glands, and genitourinary system. So this defective CFTR function keeps your body from regulating the way chloride and water move through the systems. Instead of having a normal, thin layer of fluid and mucus, people with CF build up a sticky, thick layer of mucus.

The sticky and thick mucus traps germs in your body. So your body doesn’t fight off germs the way most people’s do. This makes it easier for you to get frequent infections (as you have displayed.) This causes mommy to be extra-cautious in where we go and what we do. We want you to stay healthy. Some research shows that lung problems can develop later in people with residual CFTR (like you). 

3.  And what does it mean to have atypical cystic fibrosis?
Hmmm...That is another toughy. Beckett's genetic testing does not back up a CF diagnosis in the United States thus we have atypical CF.  Actually, over 8% of CF patients do not have positive genetic testing, but they have been diagnosed based on other testing---typcially the sweat test. Beckett has the colon biopsy which says CF, while the genetics do not say CF.  I don't think we actually know what this really means for Beckett.  I don't think the doctors do either.  A lot of this is new research, and we just have to wait and see basically. His local doctors are currently treating him as a CF patient as they feel he presents just like CF and needs to be treated as such.

4.  When did you or doctors first think there was a problem?
When Beckett was 5 days old, we took him to the pediatrician for a quick check up on his jaundice.  He had not gained any weight since we left the hospital, and he was spitting up a lot.  I think this was the very first tiny red flag although the doctor did not alarm us.  He just proceeded cautiously.  We returned a week later for his two week check up...still several ounces under birth weight, and now, he was no longer spitting up-- he was projectile vomiting. a. lot.  So Beckett had his first upper GI to check for pyloric stenosis.  We also discussed feeding and set a date to return for a weight check.  The first of countless "weight checks." During the week between the weight checks, this caring doctor called to check on him at home during the evenings.  We started medicine, and he was quickly in contact with the GI specialists at Texas Children's.  We returned for his weight check, and the sweet baby, despite eating, had lost weight. Not gained. Not maintained. Lost.  A few days later, back again. More weight lost. We are so blessed to have had such a careful and cautious doctor working with us and closely monitoring Beckett.  He quickly got Beckett in for an appointment at Texas Children's and thus began our journey.  The GI saw Beckett, and she admitted him to the hospital on the spot.  If you want to start from the beginning, click here.

5.  What does he eat?  Why?
This can be a sensitive topic, believe it or not.  Beckett eats pureed food, crunchy food like cereals, and Elecare, a prescription formula (not available at the grocery store).  There are few reasons as to why this remains his diet.

One- he got a late start to eating.  He is a bit behind the curve on the feeding progression bc he did not begin to eat until he was 15 months old.  Up until that point he was on formula only because he could not digest anything more. 

Two- he struggles to digest food that has much more texture or complexity to it.  He basically does not digest it and it can make him feel ill.  He has a hard time breaking it down.  We let him try it every now in then to see if it has changed and perhaps his system has matured, but he is still struggling with the digestion end of this. We are working to figure out how to help him digest more table food so he can at least have the opportunity to try it. 

Three- he is still on formula because it is nutritionally complete for him, and it gives him the protein and nutrients that his simple diet lacks. We are hoping to get him off of formula in the near future as we find ways to blendorize more whole foods to balance out his diet. His formula is a prescription amino-acid based formula so that it can be digested easily.

6.  Will he grow out of it?
Again, a difficult question.  I think no one can predict the future.  No one really knows what tomorrow will hold. We don't have guarentees.  So, I can't say that no, he will not grow out of it.  I do believe in prayer, and I do believe that he could be healed.  That being said, the doctors now seem to think it is unlikely.  After all, one doesn't outgrow a disease like cystic fibrosis.  However, I completely think we can keep his symptoms under control and that they could subside, and he can live a very full and healthy life. I think it is very likely that he could be less affected by his symptoms as he gets older.  His body just might require a little extra management than most.

7. Why Cincinnati Children's?
We took Beckett to Cincinnati in December of 2011 because when you have a very specialized problem, you need a very specialized specialist!  Cincinnati Children's has a bowel management program and the only colo-rectal center for children in the country.  It is the place to go if you have extreme bowel probelms.  This was the game changer for Beckett.  This is what brought us to this diagnosis, and this is what got him on the treatment that he needed.  We will always be thankful for what this hospital did for Beckett.

 7.  But he looks so happy, and he is growing now, so isn't he okay?
He is a beautiful child - if I do say so myself :) - with a contagious smile and funny personality.  And while he is still small, he can now pass as "petite" as opposed to "too skinny."  And this is because he is now being treated for his problem.  We have worked very hard to get him to this point. We now know that his body requires digestive enzymes due to the atypical CF.  We know that he needs a surplus of liquids and calories.  We know that he needs intervention to have a bowel movement.  We are able to treat all of these needs, so he looks and seems very healthy.  It means that his chronic illness is under control.  But it is still there, and his body works very very hard because of it.  So, yes, he is okay.  But no, unfortunately, it doesn't mean that it is all over or behind him.  It means he is maintained and we are so thankful for that! He has come so far, and we feel very blessed.  We have come so far since his first birthday and the emotions we felt surrounding it.  Click here to read our feelings when sweet baby turned 1.

Thanks for reading! If you managed to get through this whole post, I really appreciate your willingness to learn more about what is going on with Beckett. Thanks for your prayers.

The Lord Will Provide

 

As many of you know, at the end of last summer, our family loaded up and relocated-- again. The movers came, and they worked together like a well-oiled machine as they packed up our belongings- our last 18 months- into boxes. Towers of boxes. Box "sandcastles" in our kitchen and box mazes up stairs. A little boy's playhouse, really. We watched as they quickly wrapped, taped, and stacked. Some boxes that hadn't been opened since the day we moved into the house, just 18 months prior. Items unused in the whirlwind that was the last year and a half.

It is tempting to think that we must have just despised city life. Just 18 short months. But in fact, I don't think either one of us could ever hate that city. That city offered a lot to our family in a time when we needed a city. Amazing, really. It was exactly where we needed to be. And God knew. Those who know us know that we are not huge fans of the hustle and bustle and busyness of the city, but we were blessed by being there. All in God's perfect timing. We were in the place He wanted us to be. And we are confident of that.

But back to the box towers. It was surreal, really-- to see all those boxes, stacked up, and ready to go. It felt like we had just gotten there, but then again, it felt like we had been there forever. After all, so much had happened in that short year and a half. Lives changed.

And then, the men started stacking the boxes onto dollies and rolling it out of the house and onto the truck. Again, a little boy's dream. As you can imagine, this whole process kept these boys very entertained. As my boys felt excitement (and Beckett felt confusion), I felt overwhelmed. Not by one single emotion necessarily. Just overwhelmed. How is it possible to just leave a place where so many memories were made? First claps and waves. First steps. First amazing words spoken. First words read aloud by a little voice. First numbers added. All here. And the little voice in my head says, "Remember Kaylan, this place is not your home..."

True, true. This is not our home. Because we know that our ultimate home and destination is in heaven with our Lord and Savior. But as I walked through this now empty house, and looked at the empty walls, I could see nothing but God's perfect provision- especially during this time of weakness. It overwhelmed me to tears. Tears that wouldn't stop. Tears of amazement and awe of what God had done for our family.

Spoken perfectly by Matt Redman in his song, Never Once,

“Kneeling on this battle ground
  Seeing just how much You’ve done

 Knowing every victory
 Was Your power in us.”

The perfect description of how that moment felt to me.  In that house, we had fought- gone to battle…more than I can even put into words here.  We cried, we laughed, we grew, we grieved.  That house held a lot for us.  And for me, it was the place that God taught me (in a very clear and obvious way) that He is faithful and He provides.  Almost like a slap on the face…a very loving slap. The kind of slap that says, “Be still. And know that I am God.” (Psalm 46:10)  He cares for us in a deeper way than we can ever understand.  He loves us.  He is there fighting battles with us and providing for us the whole time.  We are not alone.

You see, He put us right there.  In that house.  In the big city.

- 20 minutes from a wonderful Children’s hospital where Beckett visited 8 different specialties more times than I can count.

           - Close to one of my sisters at a time when I needed to be close to her.

- Gave my husband a job that provided wonderful insurance. Insurance that paid for the most expensive of genetic testing. Insurance that paid for home nursing. Insurance that paid for an entire trip to Cincinnati—not just the medical bills, but the flights and hotel stay.  Amazing.

- Provided us with income to pay for food, clothes, a home, and medical bills.

- Moved a long-time friend from the same small hometown to the city at the same time—just 5 minutes away from us. A friend who loved, cared, and helped above and beyond.  A friend who babysat when she knew I couldn’t return the favor.

- Gave my son a school where he felt loved and cared for.

- Built a new playground in our neighborhood that we could walk to and burn off some energy.  A wonderful distraction.

- Put us in a home that kept us safe and comfortable with lots of space and a bathroom for each child when we really needed separate bathrooms. (Even the little provisions are real and evident.)  A home where we grew in huge ways. 

The list could go on and on and on.

A home where every where I looked, I saw that The Lord Will Provide.

It was hard for me to leave that home.  I really do know that this is not my eternal home and all of that stuff in boxes means nothing.  But the lessons I learned in that home are unforgettable. I saw God’s provision for my family in a very clear way.  There were changes in my life that will forever remind me that God is the great provider.  And when I think back to that home, it is as if I am walking through it all over again, looking at the empty walls and seeing God’s provision.

The Very One who numbered all the stars knew that for that 18 months, the big city was exactly where we needed to be.  He is faithful.  The Lord Will Provide. (Gen 22:14)

2 Corinthians 12:9

My grace is sufficient for you, for my power is made perfect in weakness.

 

 

Feeding Tube Awareness Week

This week is Feeding Tube Awareness Week! There are children and adults everywhere that are dependent on a feeding tube as their only source of nutrition, others use it to supplement nutrition if they are struggling with growth for any number of reasons.  Regardless, it greatly helps these individuals with their quality of life. 

 

I am going to be really honest.  The first time Beckett was admitted to the hospital for Failure to Thrive (click there to read all you ever wanted to know about failure to thrive) at 4 weeks old, I was overwhelmed just sitting in the little parent lounge on the 12th floor...the place with the refrigerators, microwave, aquarium.  I thought, wow, how am I here? And then I saw a young infant girl being carried around by her mama with a little tube taped to her face and going up her nose.  And I thought to myself, "oh, that sweet baby must be really sick to need that tube up her nose.  I wonder what could be wrong." And I don't even think I knew the proper name for it.

 

 

Less than a week later, I watched as it took 3 nurses and several tries to get that tube up my sweet son's nose.  After an x ray, we learned it wasn't in the right place.  So they had to do it all over again.  That tube is called a Naso-Gastric (NG) tube.  It gets its name because it runs from the nose down to the stomach. It is not placed surgically, and parents are even taught how to replace it.

 

This little tube got my baby back up to his birth weight and gave him the energy that he needed to learn to suck from a bottle correctly. Once he started drinking all the calories he need, we were able to stop using the feeding tube.

 

Now here he is at 4 months old. With the feeding tube.  He was not growing, and the doctor put him on a continuous feeding at this point.  You can read more about our experience at this time here.  He did not tolerate the pump well, and it led to a lot of crying and upset.  Once again, we managed to get him off of it and taking it all his nutrition orally.

 

Also, I just have to mention, this is a great lesson on how NOT to tape an NG tube. I plan on writing up a post on how to tape an NG tube since we have now become pros at it.

Now a year later, at about 16 months, we gave the NG tube another go.  Again, his weight was a concern and now his length had stopped increasing.  He went from the 65th%ile to the 0 rapidly. This time the feeding tube did help and was more successful.  Though he still did not become huge on it. Click here to read about this go around with the NG tube and being pushed out of our comfort zone.  After 3 months of the feeding tube, he had a bad case of bronchiolitis.  We pulled the tube so that he could breathe...or should I say that he pulled the tube. :) The doctors thought we could give him a chance to grow on his own. And he did well without it.

 

 

 

 

Now here we are, another year later, another feeding tube awareness week, and the tube is back again.  So I decided after the past 3 years of having a tube during feeding tube awareness week, it was time to post about it!  Somehow, it is always at this time of year.  I am not sure if it has something to do with the winter or what... :) But here we are tube in nose. And it is helping him! (But more on that later)

 

 

 

I just wanted to take a moment to share out journey on and off an NG tube with you all this week.  I think it is important for others to know what this is and how it helps people who truly need it.  It should be a last resort and not the first step in helping a child with failure to thrive.  Feeding tubes are life savers.  They are absolutely amazing, and they have helped are little guy through a lot of rocky times.  For more on this, check out: feedingtubeawareness.com

 

 

And here are the top 10 things that I learned back in 2011.  One thing that I have learned since then is that they aren't as scary as I once thought they were.  It is simply a tube going into his tummy to deliver nutrition and hydration.  I was very scared at first, as I suppose is the case with anything new and unknown.  The fear of it all has gone away.  It is easier now than it was 2 years ago.  We are all more comfortable with it, and that makes it much easier.

 

**from 2011-- This feeding tube thing is harder to learn then you might think. Here is what I have learned so far:

1. Continuous night feeds aren't as convenient as that might sound. Yes, I am sure many of you may think how nice to have a machine feeding your baby so you can sleep. However, it has been anything but that. I am awake more now. And you think of things such as the fact that you can't feed him formula that has been sitting in the bag on the pump stand, unrefrigerated for 8 hours. I mean, you would never feed your baby prepared formula in a bottle that has sat out for 8 hours. So, when Kyle and I realized this would mean waking every 2 hours to add fresh formula to the bag, my engineer husband quickly devised a way to keep the formula cold for 8 hours involving an insulated lunch box and lots of cool packs.

2. A nursing degree would be very helpful in feeding a tube feed child. Any time before I put anything into his tube, I must check the position of the tube with a stethoscope. I put a syringe in the end of his tube, the stethoscope on his belly, and actually blow 3 mL of air into his already gassy belly to listen for air in his tummy. If I hear the air, I know it is in the right place. This would all be much easier if I knew where his tummy was. (I have now learned, but still struggle with it every night). I also have to draw formula back through the tube to assure its placement. I have also learned to vent air from his tummy using the tube. The tube also needs "flushing" to stay fresh. This upkeep and "nursing part" has become the most stressful part of the feeding tube for me.

3. A huge sudden increase in formula in a baby's diet will make him VERY cranky. Imagine if you were used to a 2000 calorie per day diet, and someone suddenly increased it to 1 4000 calorie per day diet. You wouldn't feel so great either.

4. Timing is key. I am still trying to figure out the best time to start the pump. I don't want it to be too close to his last bottle of the day to where he will be over full- cause that makes him mad. But I also don't want to wait so long that he is too hungry to wait for something that only pumps one oz per hour into his belly.

5. It is hard to leave him alone for even a minute without watching him for fear of him pulling out his tube. He enjoys watching his mobile in his crib, but now I just wonder about the tube the whole time...

6. No book on healthy sleep habits, scheduling, baby whispering or even the happiest babies on the block will help me with this.

7. It is exhausting. Counting ounces and calories and timing it all just right. Making sure he doesn't burn too many calories while eating. Making sure he doesn't burn too many calories being awake or upset. I am exhausted, and I don't deal with many of the medical complications that many other moms of tubies deal with. Many of these children require around the clock care. I admire the strength of these parents doing what feels like the hardest job on earth. But still, I am exhausted.

8. It is worth the hard work. This week we are thrilled that BECKETT GAINED NEARLY 7 OZ! Babies at this age are expected to gain one once per day, so about 2 lbs per month. Up until now, Beckett has gained 2 lbs in 4 months. So we are very excited about this gain! Also, I have noticed a decrease in the amount of spit up today. Yay! It is odd that this tube-- that honestly wears me out--could change his quality of life. That is a good thing.



Trying Something Different

So I know I haven't posted many updates lately. To be honest, we have just been in a bit of a holding pattern--doing more of the same.  We have been working very hard to manage his digestive system as unpleasant as it may be at times.  Lots of ex-lax (LOTS!) and lots of enemas....which all lead to a lot of cramping and upset.  These are high volume enemas...not a typical child enema.  It has been a frustrating pattern, and I suppose it discourages me from posting.

At our last appointment with the GI, we decided it was time to try something new.  What we are doing does not at all feel like a solution to me.  There must be a better way to manage this.  So we are back to the feeding tube...again.

This time we are using the feeding tube in a unique way.  Instead of using it for nutrition, we are using it as a way to run fluids through his little digestive tract, and hopefully, keep things moving.  So every night, we are hooking him up to a pump and running fluid through the tube in order to clean him up and stimulate his colon to move.  He will still need the Senna, but hopefully, it will be a much more reasonable dose.

The doctor had him admitted to Children's on Monday morning, and we got to go home yesterday evening. I put the tube in his nose, and he tolerated the feed, so thankfully, it was a short stay. 

My heart broke a little when we pulled into the hospital driveway, and he immediately said, "no, no" and started crying.  He knew exactly where he was. Again, when we walked into his room, he began to cry and said, "bye bye. let's go." Bless his little heart. He is so familiar with a hospital at such a young age. Remember when I posted about this world that is becoming all too familiar(--nearly 2 years ago!)? I guess it has for him, too.  It made me think of all the sweet children who spend as many days in a hospital as they do out of a hospital.  My heart really goes out to them. We are truly blessed that Beckett has done so incredibly well, and Brant, age 5, has never once had to spend the night in a hospital. 

True entertainment for a little boy--Watching the big trucks go by on the freeway and playing with his own big truck!

Thank you for praying for us. We are very thankful for all the encouraging messages and phone calls.  We are praying this "top- down" approach works for Beckett.  When I explained the reason of the tube to Brant (and our hope to get him off enemas), he replied, "that's good. So I can spend more time with him in the evening.  And he won't have to spend all night in the bathroom." Exactly.  This is for quality of life.  Here's hoping it works.  It was definitely time to try something different.  I will let you know how it goes.

Despite getting "messed with" every 2 hours (or less), he managed to get a little sleep.