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Archive for August 2010

Thank You Mumsy and Aunt Brooklyn!

Tuesday, August 31, 2010

The house is quiet again - no more games with Aunt Brooklyn or kisses from Mumsy - as they have both returned home.  Now just games and kisses from plain old Mom and Dad will have to do.   Here's a picture of Mumsy and her boys taken right before she left to go home.  Mumsy is a ball of energy and we love when she visits!


Unfortunately, Aunt Brooklyn escaped without a picture of she and her nephews, so we've included a recent picture of her.  Both Bennett and Oliver *love* playing with Aunt Brook.  She has an incredible knack for charming children and a laugh that is contagious!

We already miss Aunt Brook and Mumsy but have plans to see them again soon!

Thank you both for helping us make this past weekend wonderful!  We know - because we do it every day - that caring for our boys, particularly Bennett, can be hard work.  Thank you for all you did!

Now, can you come back next weekend and do it again!?!

Momma is back home!

Monday, August 30, 2010

Breck is now home to all three of her boys...something that almost didn't happen as expected.  Yesterday evening, we found out one of Breck's flights home was delayed an hour, risking her ability to make her connecting flight.  But thankfully, the flight carrier delayed the plane so she was able to arrive home right on time!


Having been gone only two days, Breck was surprised how much Bennett seemed to have grown.  He seemed, to her, both taller and heavier than she remembered when she left.  Maybe it's because Bennett turns 11 months old today...or maybe it's because it always takes a few days away to really notice small changes.  But the truth is, as Bennett is getting big and is looking more and more toddler-like, rather than just a blob of baby.  

Mumsy and Aunt Brooklyn (or as Oliver calls her, "Aunt Book-en") have done a wonderful job keeping life normal for the boys.  And Brian has even gotten a break himself as these women are a power-team together! 

But as the power-team returns to their respective homes and Brian gets back to school, life is returning to normal.  And normal is so so good!

Breck's Thoughts: A Weekend Away

Sunday, August 29, 2010

I left Friday for a girls weekend.  One of my closest friends from college was having her wedding and almost all of our close friends were going to be there to celebrate!

Months ago, I hesitated to make plans to go as I was unsure how Bennett would be doing at the time (we have a "zero tolerance" sort of rule in our home these days regarding Bennett:  Bennett's health is our primary concern and ultimately nothing is too important to cancel if it conflicts to getting/keeping Bennett well).

But after we saw that Bennett had begun thriving and after my mother and sister offered to care for the kids (along with Brian) while I was away, it was decided I would go.

I has been an absolute blast!  It's fun to reminisce with friends and laugh about the naivity of being 18.  Its enjoyable to see how friends have changed over the last 12 years of their lives (yes, that means we are in our 30's!).  And its fun to have friends remind me of how much I've changed.

Spending time with old friends is like getting to revisit yourself years ago.  I love doing that.  It reminds me who I am.  I'm not just Breck, Brian's wife...or the mother of two...or the mother of a CF child.  I'm also the girl who showed to college wearing a bow, the one who loved to snap photographs during dinner to the annoyance of friends, the one who (as friends reminded me) always wanted to ask deep deep questions, and the one who was determined to rebel in whatever innocent way possible.

But over the weekend, I have enjoyed remembering both who I was and reminding myself who I am.  I have both had an absolutely wonderful time and longed to get kisses from my sweet boys.  I have both appreciated taking along only my purse and felt a bit naked without all of my diaper bags.  I have both wanted to get dressed up in my cocktail dress for a wedding dinner and simultaneously wanted to take off my shoes, climb into Oliver's bed or stand near Bennett's crib and put my little ones to sleep.

What a gift I have to be able to be somewhere I love...and to love somewhere I will at some point be!

That some point later this evening I will return home.  But not before a good couple of hours shopping, a pedicure with one of my best friends and a lunch full of girl talk with other adults!!

Playing At The Doctor

Friday, August 27, 2010

A few days ago, we went in to get Oliver the Flu Mist (they now give many kids and adults the flu vaccine through their nose) and to get bloodwork done for Bennett.  The boys were awesome playing in the examination room for a short wait.  Oliver played with a $1 measuring tape Breck keeps in her purse for moments such as these.  Bennett played with whatever he could get his hands on.


We will be returning in another few weeks for Bennett when the Pediatrician has their batch of Flu shots ready.  Bennett will never be able to get the Flu Mist and must always get the flu shot since the Flu Mist has the live virus in it - therefore potentially compromising Bennett's health.  It's not a big deal for kids who are healthy but is not good for kids with respiratory issues such as CF.


Thankfully, Bennett's bloodwork has come back all clean.  His C.Diff. is gone and we're in the clear for a while more!  Hooray for good health and a happy, active baby!

Hairdo

Thursday, August 26, 2010

Our big boy is hard to capture these days.  But we do have a few to share.  This one is of a recent visit with Mumsy.  Bennett loves being kissed!


Bennett is pulling up with such ease these days!  He has even let go a few times and watched himself balance for a short bit.  After enjoying his own bath, he likes to stand up and hold the side of the tub and watch Big Brother play in the bath too!


We thought these few minutes of his staying still would give us enough time to focus in on this:


Bennett's curly hair that is ever so slightly growing into a mullet.


Business in the front but all party in the back!


We're not yet sure if we're going to cut it or just let it grow some more.   For now, we think it's pretty cute just the way it is.

Refrigerated Poo. And Don't worry, we're family.

Tuesday, August 24, 2010

Can you find it?

Top shelf.  In the "Refrigerated Specimen Bag" so it won't be mistaken.


There it is - next to Bennett's medication is Bennett poo in our fridge.  Fortunately, it didn't stay in there long.   We took it from his morning diaper and had it at the doc's office within two hours.  It's still creepy to reach for the milk and find this though. :)

Bennett had some bloodwork done this morning and we brought in a stool sample to double check if his C.Diff. is gone.  We look forward to hearing the results in the next few days.  We're pretty confident that things should be fine.

When we went in for Bennett's bloodwork, the sweet ladies at the lab upstairs of our Pediatrician's office asked us if Bennett had ever had bloodwork before.  Breck chuckled, "yes, all the time."  Then the lady realized that she recognized us from a few weeks ago.  She said, "oh that's right.  And your son did really well when your friend was holding him."  We were surprised she remembered the time when our friend Emily (a nurse) came to help hold Bennett during his bloodwork.  But there was something comforting in being remembered.

It's nice to become acquaintance-friends with the upstairs bloodwork ladies.  We already adore the front desk lady, Deborah, at the Pediatric office.  And we know all of Bennett's Peds nurses on first name basis.  And yesterday, we saw one of Bennett's old NICU nurses at a local children's museum.  Somehow, it feels sorta like we're apart of a family.  And that's ok.  It makes it less scary somehow.

Of course, some "family members" tend to join by coercion, rather than by pure will.  A nurse at our GI doctor's office used to respond to us despondently and with little interest in caring for our needs.  However, Breck is quite persistent when she fights for Bennett and was recently unrelenting in her daily phone calls to this nurse until the nurse herself became invested in what we needed from her.  Later, after the issue was resolved and the nurse began to warm up to us (aka started calling us back), Breck felt comfortable apologizing as to why she was calling so much.  She said, "I don't mean to bother you but I am certainly aware that 'the squeaky wheel gets the oil.'"  The nurse laughed and said it was "no big deal."  She replied, "that's ok.  I have a few mommas like that - a few I keep on my radar screen..."  And then she said, "...and that's not a bad thing."  Breck felt good to hear that the nurse finally put Breck on her "radar screen" as we're sure she finally decided: if you can't beat 'em, join 'em.  Welcome to our team, GI Nurse.  We're family now!

Our Quiet, Our New Schedule....and a word of thanks.

Monday, August 23, 2010

We have been quiet in our tiny blogosphere.  Bennett is doing so well right now (we are still asking ourselves - what just happened?!) that we have been very preoccupied with *life* and feeling less and less overcome by CF.  It's amazing and we are so thankful for good health and peace! 

We continue to take pictures of Bennett to document what he's doing these days.  But 90% of them are blurry as he's on the go constantly!  In fact, he is a very fast crawler!  And he is pulling up so fast and easily that we wouldn't be surprised if he was walking on or before his first birthday!

Brian starts back to school today.  This means our world gets turned upside down a bit.  It will be nice in many ways.  We love the routine that comes with school and we welcome a break from these 100 degree temperatures.  However, Brian's PhD education is grueling and can be very non-flexible.  This can be hard as the fall also brings an early sundown and cold/flu season. 

So, we are embracing a new schedule and new expectations starting today.  Despite that we enter into the school year fall schedule with some anxiety, we have had to remind ourselves that what happened last year won't happen likely happen again...and this year is bound to be better:  No more additions to our family.  No more CF diagnoses.  No more critical care of our infant. (we hope!)  No more big overwhelming changes to our life.  Heck, if life could be normal this fall, it's going to be a breeze!!

We have felt many many prayers in the last few weeks.  As we have hit many lows over the last year, we have also experienced many highs.  And these highs have, ironically, seemed to always followed the lows.  Sure, one could argue that anything would feel like a "high" after experiencing a "low."  But we're more apt to say that we can feel God's presence more clearly during times of despair, than during any other time.  Many people have shared with us - via the blog, email, notes and in person - how they have prayed for Bennett and for us.  And we want to share just how much we have felt these prayers.  

It is evident God is near to us.  He has been like a ribbon that has flowed through every ebb and flow of Bennett's short life so far.  Thank you to everyone who has interceded on our behalf.  It has mattered.  We feel it most these days through Bennett's laughter. And it's awesome!

Bennett Laughing and His New Tricks

Friday, August 20, 2010

Well, this started out as spontaneous "dolphin talk" between the boys (which sometimes also includes Breck, who, as you can see, sometimes also participates) but it turned into just wild silliness by all, which prompted our bringing out the camera. While we had the camera on him, we thought we'd catch some of his new tricks. :)

Guess how much this Vest costs???

Thursday, August 19, 2010

We are *loving* Bennett's vest! It's absolutely amazing! And he is tolerating it so well!



Check out how Bennett's little fingers are vibrating in the picture as he holds his tubing while wearing The Vest. :)



Vibrating toes!


Despite our best effort to minimize the noisy, flashy plastic toys in our home, we were more than happy to buy all of the brightly lit musical toys as possible to keep Bennett happy during his Vest time.


Daddy sits by to keep Bennett company.


The machine counts down the time left (see the right side where it says, "4 MIN").


The machine also counts how many hours it has run.  We've done 5.4 hours on this baby!

This thing IS amazing.  But this amazing little thing comes with a price. 

Let me tell you what things you could buy with the same price as The Vest by Hill-Rom:
* 74,620 (or 533 boxes of) Huggies Snug and Dry Size 4 Diapers 
* 20 Refrigerators
* 16 LCD Flatscreen TV's
* 8 Months Rent in a 1 bedroom New York City Apartment
* 1 Year of In-State Tuition at the University of Nebraska
* 1 Semester of Tuition at Harvard University
* 2 Harley Motorcycles
* 1 2009 Used Honda Civic or 2010 New Kia Rio5

Yes.  Believe it or not, but Bennett's Vest costs $16,000!!!

Thank goodness for health insurance!!!

It's an amazing contraption, though.  And one of the reasons why it is so expensive is because it has a limited lifetime warranty (the limited part refers to it only applying to Bennett - the warranty can't be passed on).  At any point, if we need new tubing or a new vest (Bennett will need larger vests as he gets older), all we have to do is call the company and they will send it to us!  Pretty nice!

Oliver likes to watch the fan on the back of the machine.  He loves how it goes around and around while putting out air.

He likes to tell us about it too!

This Vest is like a part of our family.  We all gather around during Vest time (Oliver loves watching it and we end up trying to sing songs, play with toys or dance - whatever it takes to keep Bennett happy).  It's expensive but it's worth keeping our baby well!

Breck's Thoughts: Carnival Conversations

Tuesday, August 17, 2010

A few weeks ago, a woman stopped me at a city-wide carnival in our neighborhood.  I noticed her glancing my way but wasn't sure, at first, what she was referring to when she said,  "I just noticed your feeding tube."

She had evidently noticed Bennett had a big clear cord from his belly to the handle of the stroller.  Caught off guard, I wondered how she knew that's what it was (since few people really recognize what was going on).

It turned out her daughter uses one too. (She has Cerebral Palsy.)  We (the woman, her husband and I) ended up having a great discussion about g-tubes and feeding pumps, how we're so thankful for them and how often our kids use them.  And we chuckled about the uniqueness of our conversation.  Only one special needs mother to another can really appreciate all that goes into feeding tubes and pumps.

It was a strange situation to have in the middle of our city-wide neighborhood carnival: dozens of people were swarming around us visiting the booths, music played loudly in the background while this woman and I discussed our children's feeding tubes.  But I was so happy.

It was only moments before that I was self-consciously thinking about how weird it was that I was strolling my kid along at this event while he's hooked up to a pump forcing fluid into his tummy.  Will this ever normal??," I thought.  Just before speaking with this mother, I was keenly aware of just how different I felt.  It was like I was among all these people but yet sort of felt alone.  And now, here I was, just a short time later, laughing about g-tubes with a family, in some way, just like me.

This mother and I compared feeding tube tips and she shared how she admired how I had set up the somewhat complicated situation - a feeding pump hanging from the stroller.  And I was so proud.  (I am a pretty good pump-situation-maker, if I do say so myself.)

But most importantly, I realized, I'm not as different as I thought I was.
And that feels good.

Update: Happy Baby!

Sunday, August 15, 2010

Bennett is doing phenomenal these days.  He is like a normal baby and we are pleased as peaches!

Bennett's pain is almost a distant memory these days.  We never seen him curl in pain or scream like he is being stabbed like we used to see.  We are keeping him on the lower formula concentration and this has taken away all symptoms of intolerance and tummy aches.  In fact, we haven't had the slightest clue on when Bennett is making a dirty diaper so we haven't seen any more rectal prolapse issues.  There is always a chance he is still having problems with it, but we don't see him showing any warning signs that this is happening.

But to top it off, Bennett's vomiting issues have all but stopped as well!  We're on day number 4 of no spontaneously throwing up.  We still have no idea what really caused his vomiting issues but we did notice that after a few days of his new medication his vomiting disappeared!  It has been awesome to have Bennett wear one outfit for most of the day and to have him wake up with dry bed sheets!

Last week, the doctor put Bennett on a new medication.  He now takes 3ml of metronidazole four times per day.  He will continue this for another week in order to treat the Clostridium Difficile bacteria found in Bennett's stool.  Clostridium Difficile (also known as C. Diff.) is common among those with CF.  However, it can be fatal if not treated.  This antibiotic is probably also treating whatever was causing Bennett's vomiting, if it wasn't the C. Diff. itself.

The GI doctor has received the results of Bennett's bloodwork last week.  We hope to hear on Monday if everything is good.  We received Bennett's throat culture results.  His culture was clear, other than some evidence of Staph (which is common and fairly harmless).  We are happy to know his lungs look good.  He'll have another throat culture in another 3 months - and will do so for the rest of his life.

We are so thankful that Bennett is doing so well!  He is cracking us up with his fun new tricks.  He loves to shake his head "no" when we ask him to do anything.  And he loves to run-crawl whenever we tease that we are going to tickle him.

In many ways, his feeling good, in the absence of pain and vomiting, is one of the first times we have actually thought to ourselves, Bennett is acting like a normal baby.  And despite a feeding tube, vest therapy and multiple doctors appointments, we are starting to feel like life with Bennett is, in fact, normal.

We are praising God for every bit of normalcy in our lives!  We will have to put a video soon of our sweet boy laughing.  There is nothing so beautiful than hearing the squeals of a happy baby!

Pictures: Baby On A Leash

Saturday, August 14, 2010

We are all getting used to keeping Bennett on his feeding tube for an hour at a time. As long as he doesn't step on his tubing, he doesn't usually notice that he's hooked up.  (Although, sometimes, he does like to pull on his tube or even chew on it.)  We just have to watch him and make sure his backpack is within range.  















Breck's Thoughts: Terrified

Thursday, August 12, 2010

I almost didn't post yesterday's blogpost.

I was hesitant.  My blog posts are a reflection of my feelings, thoughts, concerns and fears.  So, it's easy to look back over my blog posts and see patterns of how I was feeling at a certain moment in this journey.

I've been reflecting on my posts lately and wondered, "Am I writing too much about Bennett's difficulties and our struggles through them?"  Sure, this is what we are dealing with.  And this is what I am thinking about.  But I wondered, "Am I too focused on our struggles and not as focused on our hopes?"

When facing a struggle, some collapse.   Others ignore it and pray it goes away.  Then others seem to try to go right into it...to stare it down and confront it wholeheartedly. 

This is what I hope I do.

I'm a "realist."  I'd rather expect the worse outcome and be pleasantly surprised with a better outcome.

I'm a "problem-solver."  Give me an issue and I'll stay focused on it until I overcome it.

I'm a "tell-me-like-it-is" kind of girl.  I don't like surprises and want to know as much as I can about anything.

But yesterday, during my reflection of my recent blog posts, I realized that there is something more going on behind my problem-solving, realist, tell-me-like-it-is desire to confront Bennett's issues.

There is another reason why I tend to want to embrace my child's illness and long-term issues.

As I have dug deeper within my heart and peeled back the layers of noise I place in between life and my feelings, I realized that the reason why I tend to write alot about about Bennett's illness, particularly what makes his illness difficult.  This is because...


I.am.terrified.


I.am.beyond.terrified.


I.am.beyond.terrified.that.I'm.going.to.lose.him.


I had no idea these feelings were swimming within me.  But recent reflections of my writings - how I write about the difficulty of caring for Bennett, about the risks Bennett's CF causes him, about the lists of issues Bennett currently faces, indicate that I may be writing for comfort.

I want comfort.

And it is comforting to remember that my child is sick...because if I remember he's sick, then if he is taken from me abruptly, I think I believe it won't feel so painful.

I think that I have been unknowingly working on the premise that...

Healthy children don't get taken away too soon.
Only sick children do.

So, if Bennett is taken from us, then I shouldn't be quite as surprised/devastated for he was sick.

However, the truth is, sometimes healthy children do get taken too soon.  And many sick children heal and never see a death they once faced.

But underneath layers of thoughts, blog posts, coping mechanisms, daily routines, I have believed otherwise.

I'm keenly aware that CF takes lives.  And I'm fully aware that Cystic Fibrosis will likely take my own son's life at some point.

But I realize now that I have falsely believed that, somehow, if only I could keep the awareness of his potential early mortality in the forefront of my mind, I could prolong the pain should it happen.

However, it is not that easy.

For death is always painful, no matter the circumstances - whether or not you're expecting it.  And there is nothing I can do to add one second to his life.  When it is his time, it is his time.  As it will be with my own death, God chooses to give life and to take it away.  There is nothing I can do about it. 

Sure, rationally, I know that I am not in control.  But that doesn't take away that I want to try to be.

I am absolutely terrified to face the loss of my son.  And every moment that I fall in love with him more, I realize that that fear only grows...

...even when I don't realize I'm even thinking about it.

Bennett's CF causes me to face issues that, if I really thought about it, I feel about others as well.  I am terrified to face the death of many people in my life, including my soulmate and husband.  But I can pretend that that won't happen for many years, until we are in the old folk's home.  However, Bennett's CF reminds me that life is short and we are like here for only a short time.

You have made my days a mere handbreadth, the span of my years is as nothing before you.  Each man's life is but a breath.  Psalm 39:4-5

It is false to believe that my awareness of Bennett's illness will help me better cope with a early death or even somehow prolong it.  But now that I understand this, I can replace those false beliefs with true beliefs.  I must remember that God will comfort me, in time, when I need it.

For now, I must trust that God is good and will make something good out of Bennett's CF...whether he is sick or not.

For I know the plans I have for you," declares the LORD, "plans to prosper you and not to harm you, plans to give you hope and a future.  Jeremiah 29:11

Breck's Thoughts: Treatment Compliance and The Vest

Wednesday, August 11, 2010

:::EDIT: I almost didn't post this.  See why tomorrow.:::

Last week at Bennett's CF Clinic, Bennett's CF Doctor asked us how we were doing with his treatments. I thought about lying.  But I decided that lying wouldn't help anyone.  The doctor isn't my mother or my teacher.  She is there to help us.  So, I told her, "we're not doing so well.  we're not getting in his CPT but once a day and his nebulizer less than that."

I explained that it isn't that we are lazy or unwilling.  It's just that there aren't enough hours in the day due to Bennett's feeding pump, vomiting issues, naps, doctor appointments, splitting enzymes, other daily medications, keeping up with a toddler, running a house, etc.  We are doing our best to do what we are doing as it is!

The doctor, who sat down to talk to me about this issue, said, "Well, you know that having a child with CF is not like having a healthy child.  This is a very long road."

I responded, "yes, I know...and it is our working goal to do his treatments as ordered daily.  But I'm being honest, at this point, it's just not happening.  I'm struggling to balance it all."

I went on to list all of the things we are focusing on *just* with Bennett right now, not to mention that we're trying to keep our life as normal as possible for our other child and our own sanity.

The doctor tried to encourage me, "Well, it will get easier.  Bennett will get older and you will get better at handling all of this."

But I knew that what she was saying wasn't necessarily true.

I read alot.  I talk to other CF moms.  Maybe I will become more efficient with my time.  Maybe Bennett will be able to walk on his own and swallow his own enzymes at some point.  But overall, CF doesn't get easier.  Typically, CF only gets harder.

"Dr. Dambro," I replied, "you know that isn't necessarily true.  If Bennett's throat culture from today comes back showing he is culturing psuedomonas or MRSA, our life just got more complicated in an instant.  Bennett will be placed on new time-consuming medications and runs the risk of more hospitalizations.  And even if Bennett's labs come back clean from today...there will be a day when they come back showing he's culturing something and our life will have just gotten harder."

Bennett's doctor knew I was right.  Her face began to melt.

She responded, "Well, you know, most parents who have a baby with CF are typically only dealing with giving enzymes and doing CPT at this point.  Most parents have a sort of 'break' before their child's CF care becomes really time intensive.  But for you, it started from the day he was born.  So, yeah, you didn't get that break most parents get."

Possibly we did miss out on any sort of "honeymoon" period that some (but not all) CF families get.  Bennett was born very very sick, rushed to the NICU immediately, taken into surgery at less than 24 hours old.  His multiple surgeries, ileostomy, eating exclusively through a feeding pump, etc. are not necessarily typical for many babies with CF.   Bennett is of the 1% of Cystic Fibrosis babies born with a perforated colon caused by Meconium Ileus.  

So, it's no wonder when I heard her validate that our life with Bennett is no cake-walk, it felt good.  It felt validating to hear our doctor admit that our situation isn't necessarily "typical" of CF families in our stage of the illness.  We don't like hearing that Bennett's GI complications seem to be showing more severe at this point...but it felt good to be validated that our struggle is, in fact, a struggle...and not one that is supposed to be this way.

In the midst of hearing so many wonderful stories of CF children who thrive and families who almost forget their children have CF (there are many many stories like this), we have felt that this has not been our story.  We are barely keeping our heads above water.  We have felt CF has taken much more of our son and much more of our life these 10 months than we had expected and even had heard from doctors/other CFers that it would.

Bennett's CF has been complicated so far.  Hopefully things will even out.  But until they do (and realistically they may not) we have to learn how to embrace the life God has given Bennett and us to live.
 
...And this is the story of how we got the Vest.  It was during this conversation when the doctor agreed to give us an opportunity to make life easier. 

So much of the time, we are at the mercy of CF.  But choosing to get the Vest was like finally gaining some control over CF.  The Vest allows us to make life more manageable, at a time when we need to manage more with less.

I loved hearing Bennett's doctor respond non-chalantly to my request for The Vest with "You can have it now.  I don't care."  It was fabulous to feel like she was on board - anything to help us care for him better - anything to help Bennett's life become more normal.  

Now that we've had the Vest for almost a week, we are loving it!!  Bennett is handling it well and we are getting his treatments done every day.  Although, Bennett does sometimes try to get up and crawl with the vest on (it doesn't work), he is mainly happy to sit during the 20 minute treatment. 

Life is full of difficulties.  And maybe Life With CF forces one to face those difficulties more head-on.  But it's not the struggle that defines someone, it's how they handle it that matters.

We are working really hard to handle Bennett's situation well - trying not to merely cope but to succeed.  It's not easy, that is for sure.  Playing "Wack-a-Mole" with CF is exhausting. 

But we are thankful for the people in our lives who provide us with encouragement, the technology at our fingertips that provides us with information and the opportunities we have been given which provide us with a way to manage life.  It is these things that help us accept our difficulties and thrive. 

Thank you God for all your blessings, even small blessings such as The Vest!

Describing the CF effect

Tuesday, August 10, 2010

We have scoured the internet over the last nine months looking for helpful websites and colorful illustrations to describe Bennett's condition.  We haven't found very many good pictures but recently we found a few we like alot so we thought we'd share.  (We will continue to update this post as we find new charts.)

How CF Affects The Body

(Found at http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_signs.html)

How The CF Gene Works
This picture explains how Bennett's genetic mutation works. The CF gene is a recessive gene (meaning you can have one mutated gene but if you don't have two, you can't have CF).

(Found at http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_signs.html)

More About The CF Gene
http://www.genetics-health.com/articles/disease-risk-profiling/cystic-fibrosis.php

Why The CF Gene Doesn't Work Properly

http://learn.genetics.utah.edu/content/disorders/whataregd/cf/

Healthy Lungs Versus CF Lungs
http://shinerama.naitsa.ca/cf.htm


More About How CF Affects The Body


A Video about CF
http://video.about.com/cysticfibrosis/Cystic-Fibrosis.htm

Some Reasons Why Bennett May Have More CF Complications Than Other Kids With CF

Monday, August 9, 2010

We are still learning alot about Cystic Fibrosis and Bennett's CF in general.  They weren't kidding when they first told us that CF is very hard to predict.  Every person's cystic fibrosis disease affects them differently - due to genetic, enviromental and physical reasons. 

However, studies do tell us about trends, which helps us anticipate risks that one's CF exposes them to.  For example, Bennett's earliest clinical symptom of CF, his Meconium Ileus, has been researched thoroughly and places Bennett at risk for a number of factors.  This may be why Bennett seems to have more CF Complications than other children with CF.

Bennett's History of Meconium Ileus puts him at risk for Liver Disease
Findings suggest that patients with intestinal obstruction (such as meconium ileus or meconium peritonisis) are at greater risk for the development of liver cirrhosis.1  The frequency of meconium ileus or its equivalent is significantly higher in patients with cystic fibrosis and liver disease than in patients without liver disease.2  (Therefore, Bennett is on the medication Ursidol to prevent liver disease from forming.)

Bennett's History of Meconium Ileus puts him at risk for Surgical Complications and Intestinal Obstruction
Scar tissue can build up one day causing another obstruction in Bennett's bowel.  Therefore, Bennett is at risk slightly for a higher incidence of meconium ileus equivalent and a significantly higher rate of surgical complications. The risk of surgical complications is highest in those presenting with complicated meconium ileus and those undergoing resection as Bennett did.3

Bennett's History of Meconium Ileus (particularly because it required surgery) may put him at risk greater issues with malnutrition.
One study showed clear association of Meconium Ileus with malnutrition in CF.  The study observed poor growth among Meconium Ileus patients was not because of poor dietary intakes, but was related to surgical treatment for Meconium Ileus and poor essential fatty acid status. 4 5

Bennett's History of Meconium Ileus may put him at risk for a more severe CF symptoms.
Several studies have suggested that Meconium Ileus is a risk factor for lower pulmonary function. 6
Meconium ileus may be an early indication of a more severe phenotype of cystic fibrosis. This was suggested by the significantly lower pulmonary function found in children with a history of meconium ileus compared to age- and sex-matched children who did not have meconium ileus.7  Meconium Ileus children have worse lung function and more obstructive lung disease than those without Meconium Ileus. Such abnormalities are accompanied by reduced lung volume. Meconium Ileus is a distinct CF phenotype with more severe pulmonary dysfunction.8

However, Studies Conflict On Whether Meconium Ileus Puts Bennett At Risk For An Early Death 
"Patients with CF and Meconium Ileus presented a poor nutritional status at diagnosis and a lower survival rate compared to the general CF population."9 
"We conclude that babies presenting with Meconium Ileus have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period."10
"Long-term outcome is similar in CF patients who present with Meconium Ileus and those who do not."11
"These findings suggest that patients with cystic fibrosis and those without Meconium Ileus do not have major intrinsic differences and that the previously poor outlook in patients with Meconium Ileus has improved greatly."13
"These results suggest that adequate initial nutritional and medical management of Meconium Ileus allows further similar nutritional status and PFTs compared with other early-diagnosed symptomatic CF patients. In this study, Meconium Ileus did not represent an additional risk factor for the patient's life."12 


Bibliography
* Analysis of risk factors for the development of liver disease associated with cystic fibrosis., 2, 7

* Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a matched cohort study., 12

* Clinical and genetic comparisons of patients with Cystic Fibrosis, with or without meconium ileus., 13
* Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?, 10


* Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis., 5, 9

* Long-term outcome after neonatal meconium obstruction., 3, 11

* Long-term pulmonary outcome after meconium ileus in cystic fibrosis., 6

* Longitudinal pulmonary status of cystic fibrosis children with meconium ileus., 8



* Meconium ileus and its equivalent as a risk factor for the development of cirrhosis: an autopsy study in cystic fibrosis., 1, 2

* Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening., 4


Update: The Vest and Other Things

Sunday, August 8, 2010

We haven't yet heard back on Bennett's bloodwork and stool tests but hope to do so on Monday. 

Bennett continues to thrive.  We are still dealing with vomiting but we're hoping we will be able to resolve the vomiing issue when we have more information and the results of his tests.  There is a chance that the concentration of his formula is causing this vomiting.  But to put him on 18 calorie formula for good seems quite distressing to us.  There are just too many calories that he needs to consume in a day for us to have him on 18 calories per ounce (he'd have to be hooked up to the feeding pump practically all day!).  But we will talk with his doctor about it next week.  For now, the vomiting isn't *every* feed and sometimes not even every day.  So, we can handle it.

One other issue that we have been noticing is Bennett's lack of affect.  He is a happy baby but most of the day, he really is more of a "content" baby.  He doesn't necessarily smile unless smiled at.  He doesn't necessarily engage unless he's being engaged....and even then, sometimes he doesn't even respond.  It's not an autism-like issue.  It's very obvious that he's aware,  focused and wanting to connect with others.  But he just doesn't seem to smile or laugh as much as one would expect from a chubby little baby like him.  The doctor said he believes that Bennett probably just doesn't feel good most of the day.  The doctor reminded us that Bennett could be feeling nausea, pain or just a "yucky" feeling much of the time and we wouldn't necessarily know.  It's likely attributed to his GI issues but directly related to his Cystic Fibrosis.  Knowing this really helps us remember that while Bennnett looks very healthy and does have the most endearing smile, his body is really sick and he likely carries around his sickness in ways we don't even fully understand.

As far as the Vest goes, Bennett is rocking!  We were officially trained yesterday on how to use the Vest and find that it is very easy!   Bennett seems to be handling it wonderfully, which is such a relief!  We had been warned that young children or children with GI issues (children like Bennett) don't tolerate it well.  But this does not seem to be the case with Bennett.  We are most excited because this allows us to do his therapy well and do it right.  At this point, we still have to hold him and really engage him during the entire 20 minutes twice per day (which is exhausting).  But, it is so much easier than having to clap his back for 20 minutes by hand with the percussor.  Bennett will continue to get his Vest therapy for 20 minutes 2Xs per day for the rest of his life.  We hope this will keep him out of the hospital and keep him well!  We are so thankful for technology that will keep our boy well!

Pictures: 10 Months Old

Saturday, August 7, 2010

Bennett turned 10 months old on July 30!  He is growing up so fast!  Between 9 and 10 months old, Bennett learned how to wave, crawl, stand on his own and say his first word! 







Waving hello.



Getting ready for his morning nap.







This is a picture of Bennett and Mommy playing the I'm-going-to-give-you-your-pacifier-oops-nope-I'm-going-to-take-it-away Game.   It makes Bennett laugh *every* time.

Video: Bennett's First Time On The Vest

Friday, August 6, 2010

The First Time On The Vest

It started out pretty good.  Bennett handled the vest on the lowest settings for a full 8 minutes!  That's pretty good for a baby who looked like Violet on Willy Wonka (remember the little girl who blows up into a giant blueberry?).

The way the vest works is there is a plastic inner tube (called a "bladder") that fills up with air.  The outside vest (blue) is washable and made of light fabric.  Once you push "on" one time, the bladder fills up with air.  Once you push "on" a second time, the vest begins to oscillate.


Oliver was an amazing cheerleader.  He just laughed at Bennett while Bennett shook back and forth.


At one point, Bennett's enthusiasm began to wane.  He probably started feeling nauseated.  We stopped but after a short pause, he was able to keep going.


Oliver was completely mesmerized by the generator (the boombox-looking thing).  Bennett was able to concentrate on something else for a while.  However, Bennett eventually needed to sit in Mama's arms while he experienced his chest earthquake.

It was great to hear Bennett cough a few times after he was done.  Even at a low setting, the vest seemed to work well.  Ultimately, we hope it will move the mucus enough that eventually he will be able to cough up (and out) that sticky mucus sitting in his lungs.  The goal of Vest therapy is to shake the mucus up, which is usually a breeding ground for bacteria.  If bacteria has no place/mucus to make a home, it can't stay in Bennett's lungs.