Long Time No See

It has been a while since I've written, because I haven't felt like writing about what has been going on.  Here is a brief update about the last few months to catch you up on things. 

Last I wrote was in August before Lex's hospital stay.  He was a complete champ through everything, of course.  But a long hospital stay is very physically and emotionally draining for me and I just didn't have it in me to update the blog.  I apologize to those who care about Lex and were waiting for updates.  His sinus surgery went well and his bronchoscopy looked good and the culture didn't grow anything!  Which was kind of shocking and amazing!  Elmo came for his birthday and the hospital staff also made it pretty special, as did the friends and family who came to visit.

Since then I have been pretty preoccupied with other issues going on with Lex.  Even though it is not CF related, I decided I will talk about it here because I know that people who read this blog care about Lex and want to know what is going on with him.  And because it is just another part of who he is that we can understand better now.  Lex is being diagnosed with Autism.  He will not have his official diagnosis until his next appointment, Dec. 16, but the psychologist has told us what the diagnosis will be.  Lex has always had some quirks and strange ways of communicating and things that make him the wonderful, amazing kid that he is.  Now we have a name for and explanation of why some of his behaviors occur, it's because Lex has Autism.  Specifically "High Functioning Autism Without Cognitive Delays and Without Language Delays" if I remember it all correctly.  Quite a handle.  They don't use the name Asperger's Syndrome anymore,  but that is basically what it is.  So I have been dealing with that both mentally, emotionally, and also physically as I fill out questionnaire after questionnaire, take him to appointments, make phone call after phone call, etc.  I'm getting to be in a pretty good place with it, but it's been a journey.

So this brings me to today's appointment and the reason I was re-inspired to start writing again.  Lex had a super awesome appointment at CF clinic today.  It started out great right from the beginning in the little room where they weigh and measure him etc.  He has always been deathly afraid of being measured.  Whether laying on the baby table measure thingy (which he outgrew several appointments ago) or standing against the wall measure thingy, being measured has always been something that caused a complete melt down for Lex (we now know this is due to his Autism).  Therefore, getting an accurate height, which is vital to getting an accurate BMI, has always been a challenge.  Today he stood straight and still, holding my hands and looking at with fear in his eyes and a brave face as he let the nurse get his measurement not just once, but the three times they like to do to ensure accuracy.  I was so proud of him I almost cried!  He gained 2 pounds in the last 4 weeks, putting his BMI at 47%ile.  This is the closest he has been to the 50% goal since he was 10 months old!  He had a minor freak out for the finger thingy, but did ok, and his oxygen was 100%!!

The new fellow we saw today was named Dr. Smiley!  I kid you not, DR. SMILEY!!  I actually asked him if he was serious and told him that was the best name for any pediatric doctor there could be.  And he lived up to it.  He was adorable, friendly, amazing with Lex, and thorough.  I'm just sad that he is a military doctor so will most likely leave us when he finishes his fellowship.  We discussed stopping one of his medications, Flovent (a daily inhaler that is used for asthma and is not proven to help cf patients that don't have asthma).  This is something I have been wanting to do for almost a year and for one reason or another the timing hasn't been right for the doctors to agree to it.  Today they all wholeheartedly agreed that he doesn't show any signs of asthma, and has had no incidents of wheezing, trouble breathing since he had paraflu, so discontinuing the Flovent is the right thing to do.  We have to watch for any new symptoms to occur to be sure, but we are all pretty confident this is one medication Lex does not need.  So 1 medication of the list is a good thing in my book!

He also got a pop in visit from one of his favorite nurses, Amy.  She was on the pulmonary unit during his admission in August and left him this sweet present on the door of his room after he had had a particularly rough day.

 

He was due for his annual labs, so we had to go get lots of blood drawn, which is never fun, but he is such a trooper and gets over it all pretty quickly.

All in all, his health looks great right now and we don't have to go back to clinic for 8 weeks.  That is the longest he has ever gone between appointments!  So I am taking this one as a major win.  I've been needing one of those lately.

Sinus Surgery

I don't really feel like doing a big ol' blog entry, but want to keep those of you who care about Lex informed, so this will be a quick, to the point, post.

We went to clinic today and went over all his test results from his CTs and PFT.  His sinuses are completely full, it was pretty crazy to see the pictures.  He is going to need to have surgery and they don't want to wait long.  Because of the fact that my parents are leaving the country on Sept 4, combined with the schedule of our ENT, Lex is going to have to be admitted next Thu., 2 days before his birthday, and we are going to have to cancel/postpone his party.  He will be in the hospital a total of 2 weeks, so we will also be there for Garrett's first birthday.  Pretty sad about all of this, but Lex's health is obviously the priority.  He will be admitted on the 21st and given a PICC line, on antibiotics for one week before the surgery, which will happen on the 28th, and then we will hopefully be heading home after another week of antibiotics, on Sept. 4.

So, that's the deal.  Now I have to go contact everyone about his party.

annual tests

Lex had his annual pulmonary function test and ct's of his sinuses and chest today.  Last year he did not do well with the Chloral Hydrate they used to make him sleep, so I thought they were going to put him under general anesthesia.  I was happy to find out this was not so.  They gave him Propofol and Versed through an IV because they needed him to be able to breathe on his own without a breathing tube.  It was definitely a fight to get the IV in, and he was pretty angry for a little while , but he calmed down fairly quickly and did ok with it.  Much better than the fight he put up last year with the Chloral Hydrate!

                                                         Pre sedation selfie

They did his pft's first.  This is how they measure his lung capacity.  Because he can't yet do the "blow as hard as you can" type of pft, it is much more complicated.  I'll try to explain the best I can.  He is put into a sealed plastic chamber and his lungs are filled by a machine and then the air is forced out of his lungs.  The chamber measures the amount of air coming out, thus determining his lung capacity.

                                                This is Lex in the pft chamber

Next we went to get his ct's.  We had to wait a little while for them to be ready for us, so when we got into the room, he started to wake up.  The sedation team gave him a little more meds and I rocked him and sang to him for a few minutes as he fought it, but he went out again pretty quickly.  During the CT they had to "breathe for him" a little bit, which was scary for me, but they said they did it purposefully to get him to, essentially, hold his breath as the pictures were taken.

                                         
When this was done, they did a few small procedures like a throat culture and body mass as he was waking up.  He woke up pretty pissed off and really wanting his IV and all the other monitors off.  He calmed down as they were able to unplug him from everything.  He was super cute and funny as he was waking up fully.  He ate some fruit loops, and they let us go.

Before we left, Dr. Thompson  pulled me aside to let me know he got a look at the ct's and his sinuses were terrible.  He said he was going to make sure we saw the ent at our next clinic vist, and that we should prepare for the fact that they will probably do surgery in the very near future.  He said if ent decides to do the sinus procedure, then they will also do a brochoscopy.  He said since it has been about a year and a half since he has been admitted, he is due for a "tune up", so we should plan on a full 2 week admission for a course of IV antiobiotics and the full shebang.

He just called me after having a chance to look everything over.  He said his lung function is basically the same as last year, so this is good news.  His lungs also haven't changed much since last year, which is a big relief.  I was worried about this after the Pseudomonas.  He said there looks like a a few small mucus plugs and a little thickening of the lining, but nothing significant.  So, then we discussed the sinus issue.  His exact words were "serious sinus disease".  He said that Lex's sinuses are completely filled with scar tissue and mucus.  There is no open space for air to get through at all.  He said it is most likely causing him pain, and he probably can't smell, taste, or breathe through his nose.  He thinks this may be a reason he doesn't eat well.

In the mean time, he is prescribing an appetite stimulant in the hopes Lex will eat a little better and gain some weight.  We go to clinic again on Aug. 14 and will get to ask the ent more questions and find out for sure if she will want to do surgery, and exactly when.  Dr. Thompson said to expect it to take about 4-6 weeks to schedule, so I guess we'd be looking at sometime around the middle to end of Sept.  I will definitely keep everyone posted as I learn more.

Blah

We had a really long and exhausting clinic visit yesterday.  Kinda don't really feel like writing about it, but will give the basics.  Monkey was a champ through the whole thing.  Wore his mask, didn't freak out when they were taking his vitals/weight etc. 

His weight is down again (which I expected because he hasn't been eating well).  Dr. McCoy wanted him to get a chest xray, so we decided to go before we saw her so that she could look at the xrays before examining him.  He did pretty well with the xrays, but it took a bit of coaxing.  And if you know Monkey, you know getting him to be still long enough to get a good picture was kinda difficult. 

The xrays showed a spot in his lower left lung.  Dr. McCoy said it could be a mucus plug or an infection, or something else, but given the fact he hadn't had an appetite, she didn't want to wait for the culture results and thought we should just start treating it is if it is our friend Pseudomonas rearing his ugly head again.  So poor baby is back on the Tobi and Cipro for the next month. 

Because of his weight loss, we spent about 45 minutes with his dietician trying to come up with a plan to get him to eat and gain some weight without letting him take advantage of the situation.

Dr. McCoy also wanted to talk about his nose, which is runny and gunky about 75% of the time.  A lot of kids with CF have sinus issues that require surgery (multiple surgeries in many cases).  She agrees with Dr. Thompson (our favorite doc from our old newborn clinic) that Monkey is going to fall in this group.  So she had the ENT come in. 

After examining him and talking with me, the ENT decided he needs to be on a daily nasal spray which will hopefully prolong the need for surgery until he is older.  So one more medication added to the list, and one more step added to the treatment routine.  They will also do a CT of his sinuses when they do his chest CT and Pulmonary Function Test, which should be happening very soon.

So finally, at the end of this super long visit, the nurse came in to get his throat culture.  She was a nurse we had never seen before and when she came into the room she said "so this is Emperor Lex, Dr. Thompson's favorite patient".  (I guess I might as well start using his real name here)  She went into a story about how when Lex came up in their meeting about this week's clinic patients, Dr. T. went on and on about him and how much he misses him.  So that was very sweet to hear and a good way to end this long (over 3 hours) visit!

Great Strides and Great News

The Columbus Great Strides walk was yesterday and Team Lex Express had a great turn out.  The weather was nice, just a bit windy, and we really enjoyed a lovely afternoon surrounded by family and friends.  Plus we raised over $5000.  It is hard to explain how full it makes my heart to see Monkey running around, playing, surrounded by all the people who came out to show how much they love and support him.  To everyone who came yesterday, thank you from the bottom of my heart!

                                                              Team Lex Express 2014

 

 Monkey in his special green shirt that identifies him as a cfer.  People with cf can pass dangerous bacteria to each other and are supposed to stay away from each other.

Today I got the most wonderful news since I found out Bug (Monkey's baby brother) didn't have cf.  After waiting anxiously for almost a week, I finally heard from the cf clinic about Monkey's last culture.  It grew absolutely NO Pseudomonas!!  I was so ready to fight for more aggressive treatment that I almost didn't believe what I was hearing.  I asked her 3 times: "none?", "all 3 strands are gone?", "even the mucoid strand didn't grow?"  I think I was floating on a cloud for a while after that phone call.  I wanted to tell everyone I saw, even strangers!

This little battle was just the first of many lung damaging monsters we will have to fight.  It was really scary for me and made everything far too real.  I just wish I could freeze time until that cure comes.  I know it's just around the corner, I just wish it would hurry up and get here.

Waitng

Monkey had "big boy" clinic a few days ago.  He did a pretty good job!  My dad came with me.  It was the first time he has been to a clinic visit since Monkey was teeny tiny and we were learning about what cf is and how it will affect our family.

The first hurdle was getting Monkey to wear the mask.  We all (and I mean just about everyone in the waiting room) put one on to help coax him to wear it.  It took a little bit of bribery, but he put it on and left it on much easier than I expected him to.

He even stood on the scale and let them measure him without a fight, things that always resulted in tears during newborn clinic.  He did still cry when they took his blood pressure and pulse ox, but he got over it pretty quickly.  He sat nicely and played ipad with Poppy most of the time we were in the exam room.  But he wasn't dancing around the room with a big smile like he usually was when we went to the newborn clinic and he knew everyone.

The throat culture was horrible.  It took 4 of us to hold him still so the doctor could get it.  He was so angry afterwards that he screamed and threw things around the room.  He felt betrayed by my dad and me because we had helped hold him still, and he couldn't be calmed down for several minutes.  He didn't want anything from us (even his beloved tootsie rolls).  The doctor came in and tried to give him suckers, and finally calmed him down by offering him a Donald Duck sticker. 

So now we wait for the results of  the culture.  I was hoping to get the call today, but will now have to wait all weekend.  I'm very anxious about the results.  I feel like the doctor was too quick to say he will probably just always have it, and I know there is more we can do to fight it, and it can be beaten!  Hopefully the culture will come back clean, and I won't have to worry about it, but I am prepared to fight to treat it more aggressively if I have to.  I wish there was a way to know for sure what is best for Monkey, but I just have to go with my Mommy-gut.  Waiting sucks!

Busy month

It has been a month since my last post.  Life has been very busy during that month!  My fundraiser was a big success.  We raised over $4000 for the CF Foundation.  I was so happy to see a lot of old friends as well as new friends and family come by to show their support.  It was really nice to reconnect with some old friends I had not seen in a while.  It was a lot of work, but definitely worth the effort, so I will be making it a yearly event.

Monkey just finished his month of Tobi, which more than doubled the time in took to do his treatments each day.  It was all we could do to get him fed, get his treatment done and give him his bath and get him ready for bed each evening.  I think we only got him to bed on time about twice during the last month.  Then, we had to wake him up early each morning so we would have time to get everything done in the morning before we had to leave for school/work.  He was exhausted, we were exhausted, and it felt like his whole little world was treatments.

I am so glad that is over with (for now), but am a nervous wreck about whether or  not we got all the Pseudomonas.  He goes back to clinic for another culture on Tuesday, and I think I will have this sick feeling until the culture results are in.  If this culture still grows Pseudomonas, they will likely admit him for a round of IV antibiotics for about 2 weeks. 

I am also nervous because it will be our first time at the Tue/Thu clinic, with new doctors, and I will likely have to go by myself!  Monkey will have to wear a mask while at clinic, something we have never successfully gotten him to do.

So there are a lot of unknowns right now, and unknowns and change make me very nervous.  Trying to keep my energy focused on positive things, like the Great Strides walk next weekend.  To join or donate to Team Lex Expess, visit my Great Strides homepage here. 

We are forever grateful to all the people who support us and show Monkey how many people care about him!

                                             Pictures from the Lex Express fundraiser
                                        

Pseudomonas

I missed a call this morning around 9:00, didn't hear the phone ring in my back pocket because that is the time my students are arriving, playing, and I am talking with parents as they drop off.  When we took the kids to P.E., I ducked out to listen to my voicemail.  It was Dr. Thompson, Monkey's favorite Pulmonologist from the CF Clinic.  He said he wanted to talk to me about Monkey's throat culture results, that it wasn't urgent and I shouldn't worry, and that he was in Clinic until 4:00, so would not be able to get back to me for awhile.  I shouldn't worry?  They do a throat culture every time we go to clinic (except the time he puked on me), and never call unless he is growing something.  On top of that, the doctor has NEVER called himself, it is always a nurse!  So of course I knew something was really not good, and I had all day to freak out and worry about it.

When he finally called back around 4:30 he told me Monkey is growing Pseudomonas.  I'm still fairly new to all this CF stuff, and since Monkey has been mostly healthy so far, I don't know about all the bugs our kiddos get, but I know enough to know Pseudomonas is the one you don't want your kid to have.  He went on to say that he has 3 different strands, they are all rare (of course, because everything about my Monkey is rare), and one is mucoidal.  The fact that it is mucoidal means it is likely to colonize.

Ok, deep breath Jodi, keep listening and stay focused.  Take in all the information.  Keep breathing!

He goes on to explain how they will treat it (2 weeks of oral antibiotics and a month of Tobi, an inhaled antibiotic).  If it colonizes, he will be on and off the Tobi every month to keep it at bay.  He tries to be reassuring.  Tells me some kids with Pseudomonas do just fine, while others who don't have it do poorly. It doesn't define anything, blah blah blah.

Monkey will have to start going to a different clinic, so he doesn't pass this horrible bug to other sweet babies with healthier lungs.  Monkey, up until now, was still in the newborn clinic because he was not growing anything dangerous.  Because kids with CF can easily catch these bugs from each other, they put them in clinic based on the bugs they have so they aren't sharing space with kids who can pass them a new bug they didn't already have.  He will now be going to the Tue./Thu. clinic with all the kids who have this ugly bug.  And he won't be seeing his beloved Dr. Thompson anymore.  That about broke my heart to hear!   

When I got home, I sent a message to a friend of mine who has a twelve year old son with CF.  They have  been through about everything, and she is very knowledgeable. I turn to her when facing something new with Monkey because I know she will tell me like it really is, from the perspective of a mommy, not a doctor, which is what I really need at these moments.  She is also amazing at explaining the medical stuff in a way that makes sense to me, so I really understand what is happening in Monkey's little body.

She said colonized mucoidal Pseudomonas is like a pilot light waiting to flare up.  It stays dormant, then when you get another bug, such as staph, it flares up.  Every time it flares up it causes more damage to the lung.  She says where she lives, every time a kid gets Pseudomonas, they are admitted for IV antibiotics.  Her son had mucoidal Pseudomonas before and they were able to eradicate it.     

So, with this information, I am now prepared to be my child's advocate and call tomorrow to find out how much is growing, and why they don't think it should be treated with IV antibiotics.  In the meantime, I will prepare to do whatever is best to keep my baby's lungs as healthy as possible until that cure comes along.       

Planning

I haven't written in awhile because I haven't had much to write about, which is pretty good in our world.  That means Monkey has been mostly healthy, and life has been fairly normal. 

I have recently started to really dig into the planning of my fundraiser.  I have the location, date, and time set.  If you are going to be in Columbus April 12, you should plan to attend our event at 4:00 at Alumni Club Sportsbar.  I have also started to solicit items for auction and am so excited by the responses I have gotten so far.

I decided to email PR/ Community relations people for our local sports teams yesterday.  I looked up each team and found the person who I thought would handle the decision to make a donation.  I'm so excited by the response I got from the Columbus Crew, our Major League Soccer team.   First thing this morning I received an email from him stating that he had a cousin who had CF, and he was thrilled to send us some items for auction.  I guess karma, or the universe, or whoever/whatever force is out there, guided me to choose the right person to send that email to.  I can't wait to see what he sends. 

I have also received some generous offers from family and friends and will be hitting up some local businesses this weekend.  Since this is my first time doing this, I was a little nervous, but everything seems to be falling into place. 

Hope

Today Monkey had his clinic visit.  The appointment went well.  His weight is in the yellow zone still, but nothing to be concerned about.  They think his constant runny nose may actually be caused by allergies, so we're going to try some allergy medicine and  see if that helps. 

For some reason he has developed a serious fear of the room where they take his weight, height, and vitals.  As soon as we got near that room, he dropped to the floor in an absolute panic.  He screamed and cried the entire time we were in there.  The cutest thing happened though.  As we were trying to get him to stand on the scale and he was totally freaking out, Dr. Thompson happened to walk by.  Monkey LOVES Dr. Thompson.  He saw the doctor in the hallway, got a huge smile, yelled "HI!", and ran out and hugged him.  Dr. Thompson stopped and talked to him for a few minutes, came into the room and said "show me how you can stand on the big boy scale".  Monkey hopped right up on the scale.  We've been seeing the nurse practitioner the past few visits and Monkey has really missed his favorite doctor.  I hope after what happened today the team will realize how much better Monkey does with Dr. Thompson and we'll get to see him again for our next appointment.

I asked to talk to someone from the research team this visit and Hubby and I were so excited by what she had to say.  The first thing I asked was: Is "fixing" one of his mutations enough?  And, as I thought, she said it is.  Because that essentially makes his body work like a carrier (such as Hubby and me).  We each have 1 mutated gene, but our bodies work fine, it takes 2 to show symptoms of the disease.  So fixing one is all that is needed to "fix" someone with CF.  That being said, the super exciting thing is that, out of the almost 2000 different CF gene mutations, the 2 that Monkey has are BOTH being highly researched.  She basically said that she's pretty certain by the time he is old enough to swallow a pill, there will be a pill to essentially cure him. This means, rather than 3-4 different medications a day on top of 1-2 hours of chest therapy every day, Monkey will simply have to take one pill a day, for the rest of his LONG life (with the same life expectancy of any healthy person)!!!!

She said right now they are not testing these new medications in children as young as Monkey, but eventually they'll have to.  She will be sure to let us know if there are any trials that he can be a part of.

So, for anyone who ever wondered, THIS is what I have been fighting for since he was diagnosed.  THIS is why I work so hard to raise awareness and money for research.  It is happening RIGHT NOW!  This horrible disease is being cured, and I am helping it happen, and my baby is going to benefit from these medical advances and have a long, healthy life!  I cry every time I think about it!!

 

This is the happy face I make thinking about this news! (It's actually kinda scary looking, but it's a happy face, really!)

 

 

Energizer and Adorableness

Wednesday night my friend Annia and I went to the Columbus CF Foundation's annual meeting.  They gave a chapter report, a national report, and a medical report, then gave some awards.  The medical report was by far the most interesting part for me.  I learned a lot about recent advances and came away with some questions for Monkey's doctors and a renewed sense of hope that this disease will be cured soon.  The average life expectancy jumped from 36 to 41, which Dr, McCoy said is the biggest single year increase she has seen in her career. I was also surprised to see my name listed in the program as someone who raised over $1000 for the Great Strides walk last year (I raised close to $5000).  Go me!!

The meeting ran really long and had some boring moments for me, so was probably extra boring for Annia.  We had Baby Brother with us, so that was an added challenge.  Annia is awesome to have come with me to this meeting, since my husband had to stay home with Monkey (people with CF are not supposed to attend indoor CF Foundation events because they can make each other sick).  Annia has been great support to me at other times also, such as going with me to Monkey's Pulmonary Function Test appointment and even going in to his CT with him because I was pregnant and couldn't go in the room.  It's amazing to have the support of friends like her.  If you know her, tell her how awesome she is next time you see her.

I left the meeting more motivated then ever to raise awareness and money for CF, which was perfect, because our next stop was to the Alumni Club bar, where we were going to talk to one of the owners (a friend of Annia's) about having a fundraiser for our Great Strides team.

I was overwhelmed by his openness to the idea and willingness to do whatever I envision to make it successful.  He also agreed to do a pin up campaign in the bar during the weeks leading up to the event.  This is where customers donate a few bucks, then get to write their name on a pin up to hang in the bar.  I think a bar is a brilliant place for a pin up campaign, because drunk people will give a lot of money!! ;)  So, I have some planning to do, and will be having another meeting with him soon to nail down a date and the details of the event.  Hoping to get some bands to play and items donated for a silent auction.  Donations taken at the door.  Maybe a special signature drink that a portion of the cost can be donated to the cause.  Will hopefully have our team t shirts finished and ready to sell, or at least a design to show and take pre-orders.  I'm open to suggestions and/or help from anyone who wants to get involved, leave a comment here or contact me through facebook, or email, or in person if you know me in real life.

I'm just feeling really energized, excited, and positive about this right now, which is a good way to feel and a good time to make things happen.

And since I have no picture to go with this post, I'll just leave you with some adorableness.

 

Support and Fundraising

Shortly after Monkey was diagnosed, I found out about a fund raising event for the Cincinnati chapter of the CF Foundation that was coming up.  It was a Halloween 5K called Run Like Hell.  It was at night, through a cemetery, and people came in costume.  I was looking forward to beginning my association with the CF Foundation, and this sounded like a fun event, so I started a team in Monkey's name and set a fund raising goal of $500.

I remember my sister telling me I should set my goal higher, that people would surprise me.  I reluctantly raised my goal to $1000.  I posted a link to my fund raising page on facebook, and watched in amazement over the next few days as the donations began rolling in.  I was surprised not only by who was donating, but by the amount people were donating.  Of course I got donations from family, close friends, even business associates of my dad's.  This didn't surprise me.  But I got donations from high school friends I hadn't spoken to in 15 years, people I babysat for when I was a teenager, even friends of family members and other friends that I had never met.  And these donations ranged from several hundred dollars, to 5 dollars.  The donations of 5 dollars were the most touching to me.  It said to me that these people had little to spare, but wanted to spend their hard earned money to show me their support.  I was moved to tears so many times in those weeks as I reached and raised my goal over and over again!

I ended up raising over $5000 for that event.  I raised so much so quickly, in fact, that the CF Foundation employee in charge of Run Like Hell called me personally and wanted to meet me and Monkey.  She couldn't believe how quickly I was raising money and wanted to talk about the foundation and what they could do for us.  This started a great relationship with the team at the Cincinnati chapter CFF that lasted the entire time we lived in the area.  But I guess details of that relationship (which I really miss since we moved to Columbus) is a topic for another post.

I decided I wanted to come up with a theme costume for our team.  I wanted something that we could dress Monkey as the center of, and have other team members dress as supporting cast.  I came up with the idea to dress Monkey as Yoda, and the rest of our team would be Jedi.  We had a small team for this first event, just my husband and I, my parents, and our good friend Zack who has supported us in many ways as we have raised funds for different CFF events.  My dad's cousin also joined us for the event.  I knew I really had the support of my parents when they agreed to dress as Jedi and be seen in public.

 

our team

                                                             My tiny Yoda

The event was so much fun!  It was fantastic people watching and seeing all the creative costumes people came up with.  Monkey was 3 months old, and was ooh-ed and aww-ed over all evening.  Our tiny Yoda and the rest of the team were photographed many times, and we enjoyed mingling with the crowd as we walked the 5K at the very back of the pack (the police actually took down the street barricades and told us to walk on the sidewalk towards the end because we took so long to finish).  It felt amazing to know we had the support of so many people, and that we were helping raise money that would go towards research to someday cure CF.  This event sparked a passion in me to do everything I can to raise money and awareness, and this passion has become a huge part of my life and who I am.  I know in my heart that these efforts will be rewarded with the chance to see my little boy grow up to become an incredible man.

CF yo-yo

The winter is the absolute worst!  Just as soon as we get Monkey healthy and get his weight back up, he gets sick again.  I feel like he spends more time sick than healthy in the winter. 

We very quickly went from super happy "yay he gained weight and got rid of the cough", to "crap, he's sick again."  In literally, like, 24 hours!  His clinic visit was Wednesday, everyone was thrilled with how great he was doing.  Thursday night he had a fever of 102.  We stayed home Friday, Saturday the fever was gone, but he still was not quite himself, meaning he actually sat still for more than 30 seconds at a time.  Sunday he seemed pretty much back to his usual self, bouncing off the walls and just a little extra whiny and quick to fits and tears when things weren't going his way.  So back to school we went today.

Around lunch his teachers told me he was very calm and quiet today.  Not a good sign!  If you know my Monkey, you know for him to be calm and quiet, he must not be feeling well.  Tonight he barely ate dinner and by bedtime, was running a low grade fever.  His nose is running like a broken faucet, it's turning from clear to a lovely shade of neon green, and his eyes are red and glassy.  At this point we can just keep hoping it won't go to his lungs, but it seems unlikely that hope will be fulfilled.

So, I will go to bed tonight, praying he wakes up in the morning fever-free so I can go to work.  Wish us luck as we continue to bounce up and down on the CF yo-yo.

Now

I've been trying to decide what direction to take this blog now that I have given the basic info.  Do I keep going chronologically and write about all the things that stand out from the first 2 years, or do I skip to how things are going now and start blogging real time events.  I decided I will do both.  Now that everyone has the background of our story, I will begin writing about events as they happen, and also sprinkle in some entries about important things that happened the first 2 years of Monkey's life regarding his CF.

Today Monkey had a clinic visit.  We had been to clinic 3 weeks ago, and that visit had not gone well.  He had had a cough for several weeks at that point and even with extra treatments and antibiotics it seemed to be getting worse.  He had also gotten taller, but lost weight, which put him in the red zone.  These were both very concerning to our team.  They did a viral culture (a horribly unpleasant nasal swab) to find out what was causing the awful cough.  We also got orders from his dietician to basically let him eat whatever he wanted, whenever he wanted to get his weight back up. I was afraid we were on the path to another hospital admission, which will be very difficult this year seeing that we have a 4 month old baby that is solely breastfeeding.

His culture came back positive for RSV, which sounds bad, but is actually a good thing for us, because it means a.) we know what is going on, b.)it is not bacterial.  After about another week, his cough was much better, and then gone altogether.  We continued to try to get his weight up by letting him eat what he wanted instead of insisting he eat what we put in front of him or go hungry.  And before anyone freaks out on me, by whatever he wants, I do not mean he ate cookies and candy for three weeks.  We really try to limit his sugar intake and he has never had much of a sweet tooth.  What that means is, if he didn't want to eat what we made for dinner, he could have cheerios, or yogurt, or a banana, or whatever else he requested.  It also meant that when we were out at a restaurant with my inlaws over Christmas, we let him eat 3 buttery yeast rolls and then barely touch the meal we ordered him. 

Well, all this apparently worked because he gained a whole kilo (2.2 lbs) in those three weeks between clinic visits!  His team was ecstatic with his progress, as were my husband and I.  Since allowing him to eat what he wants still means he is eating nutritious things (for the most part), it was decided that we should stay the course for now.  So this means I will continue to pack his lunchbox everyday with plenty of options for him to choose from  if he does not like what is being served for lunch or snack at school.  It also means we will continue to feed him whenever he says he's hungry, and give him (pretty much) whatever he says he wants.  It may cause some dirty looks from people who do not understand his special needs,  but we can learn to ignore them.

So, our clinic visit today went very well except for one small issue.  When the nurse practitioner took out the swab to get his throat culture (done at every visit to test for bacterial growth), he must have remembered the super unpleasant nasal swab they did last time, and he totally freaked and could not be calmed down.  He got himself so worked up that when the nurse stuck the swab in his mouth, he puked ALL OVER (and, of course, he was sitting on my lap).  The nurse said that had never happened to her before (leave it to my Monkey to be the first) and we all felt horrible for him.  So we decided to skip the culture this time, and I got to leave in a stylish new outfit!

Next clinic visit: 5 weeks!

Treatment

Our first visit to the CF Clinic was an educational visit.  My parents came with us, and we learned about Cystic Fibrosis, and what it would mean  for Monkey and our family.  We found out that Monkey's 2 mutations are fairly uncommon, and the combination of the two was very rare, therefore they did not have a lot to go on as far as what to expect.  We  were very encouraged to discover that Monkey is pancreas sufficient, which means that his pancreas functions normally, unlike most people with CF.  He would not have to take the tens of enzyme pills a day in order to absorb the nutrients in the food he eats that most people with CF have to take.  This was a huge relief.  They do not know for sure that his pancreas will continue to function throughout his life, but they believe that it will.

So, keeping Monkey healthy was going to be mainly about keeping his lungs healthy.  We learned about the different medications he will take and about airway clearance.  They taught us how to do Chest Physiotherapy (CPT).  This is a way of giving percussions, or pounding, on specific spots around his chest, back, and sides to loosen the mucus in his lungs in order for him to be able to cough it out.  This is done with a small rubber percussor.  There are 12 different locations on his body that need to be percussed for 2 minutes each.  This is done 2-4 times a day.  I used to joke that I had to beat my baby everyday per doctor's orders.  In all reality, giving CPT was incredibly difficult and emotionally draining.  Monkey was 4 weeks old when we started giving him these treatments.  He would scream and cry the entire time.  He was so tiny and vulnerable, and it broke my heart to have to continue to pound on him as he screamed.  Every bit of me wanted to pick him up and comfort him , but I knew that to keep him healthy, I had to finish the treatment.

 These are Monkey's percussors.  The small pink one was his first, and when he got a little bigger, we got the cream one.

                                   This is Monkey having his CPT treatment when he was about 4  months old.

 Eventually, when he was 10 months old,  Monkey was big enough to fit into an airway clearance vest, which is attached to a machine and shakes vigorously in order to break up the mucus in his lungs.  This was much easier for everyone than doing the CPT by hand.  He was able to sit upright, and we could read books or play while he was getting his treatment, he was even able to move around a little bit.  This is how he gets his airway clearance treatments still.  When he turned 2, his settings on his machine were changed from 24 minutes to 30 minutes.  It was also set to shake a bit harder.  As he gets older, his settings will continue to change and shake harder to break up as much of that nasty mucus as possible.

      This is Monkey having a vest treatment at 10 months old.  This is one of the first few times he wore the vest.

In addition to the airway clearance, Monkey also takes 2 medications through a nebulizor.  Albuterol is taken before every airway clearance, and is a bronchiodialator, which helps to open things up so he can cough out the mucus easier.  This is a medication common to people with asthma and other respiratory illnesses.  He also takes Pulmozyme once a day.  This is a mucus thinner and is a medication specific to CF.  It helps to keep his mucus from getting overly thick and sticky.    He also takes Flovent through an inhaler twice a day.  This is another medication common for asthma, it is a corticosteroid.

In all, his medications and treatments take a little over an hour a day when he is healthy, and up to 2 and a half hours a day when he has a cold, cough, or any other respiratory illness.  People often tell me they don't know how I do it.  My response is always, I don't know either, but if it were your kid, you'd do it to.