Friday, January 17, 2014


Preface- This post is very, very long, and as much as it pains me, I have to ask you to forgive the capitalization errors. My keyboard is broken, so every letter 'b' you see had to be copied and pasted in, and seeing as it took me over two hours to do that, I can't pain myself to go back right now and capitalize some of them.  


Instead of sitting down and writing this post, I laid down on the couch and took a short nap after getting Jackson into bed.  However the nagging little voice inside my head wouldn't let me sleep.  I've written maybe two blogs in the past year.  I've spent a ridiculous amount of time wondering how much sharing I should do.  How important is sharing our journey with other families, versus how important is it that Jackson isn't able to give me permission to share such intimate details of his life? As I sit down to bite the proverbial bullet, I have no idea what I'm about to say.  Only that I've been quiet for too long, and it's time to get back to work.

And aside from that, we've spent what time we can having, what is to us, a normal life.  And a not so normal life.  but the emails started.  And the postcards started.  And then in the past few days, I have seen many of our fellow CF families launch their Great Strides fundraising campaigns for this year's walk.  (Every year, we have a team for the Great Strides walk , benefiting the CFF to raise money to fight Cystic Fibrosis)  And I'm unable to be passive any more.   

Last year, we didn't do a big fundraising event like we usually do. As a matter of fact, I did little beyond the walk and online fundraising.  As the years have gone by, our team has dwindled rather than grown.  Our donations have tapered off rather than increased, and fewer people have attended our events.  That is not to say we haven't experienced incredible generosity from or friends and family.  It is always absolutely humbling to watch how people give of themselves, in essence, to save my child and his entire CF family.  but the support we had in the first year of his diagnosis has trailed off, and my efforts to put together a successful fundraiser have likewise been met with dwindling enthusiasm.  I hate to say it out loud at all, because I have had the support of some fantastic people to make it happen, but I feel like I've failed, and last year I let it keep me from trying again.  I'm not sure what I'm going to do this year, but I'm about to tell you why I have to pull myself up by my bootstraps and try again. 

I don't really want to talk about it.  I don't like seeing it all listed in print.  It makes me sad and scared, and my mom reads this, among other people who love me, And it hurts her and them to see me sad and scared.  but truth is paramount, and the truth is…the last year has been HARD.  Hard is relative, I understand.  I've watched other mothers lose their children to this, and other diseases.  My hard is not the only hard.  but nevertheless, I am uncomfortable in the moments when I can't smile and say 'it's ok'.  

This year in Cystic Fibrosis:  (PSSST….THIS IS THE REALLY LONG PART)
Last September, Jackson got a sinus infection, one month after getting out of the hospital for the standard ten day stay. He did a ten day course of antibiotics, and the infection came back.  He did another course of antibiotics, and a course of antibiotics with steroids, and then a longer course of antibiotics.  The infection would seem at bay as long as he was on them, but within three days of completing a course of meds, his symptoms would flare up again.  We dodged another hospital stay in December, and tried one last, long term course of daily antibiotics to give his body the chance to eradicate the symptoms on its own.   As spring rolled around, he was still unable to function without the antibiotics, and we were also facing the fact that Jackson wasn't on track developmentally.  The summer brought every kind of developmental test we could fit in to figure out what exactly was going on.  Developmental pediatrician, hearing and vision tests, referrals to physical, occupational, and speech therapy, and the referral to an Ear Nose and Throat specialist for the snot that ate Manhattan.   As far as the developmental aspect goes, we don't know what caused Jackson to experience delays.  Statistically, children with pre-existing conditions that cause them to spend extended amounts of time in hospitals or undergoing extensive treatments for those conditions are more likely to experience these sorts of delays.  Ultimately, the cause was not as important as helping him be the best HE could be, and live his own best life.  He began physical and occupational therapy, and we paid our first visit to the ENT.  The CT scan of Jackson's head made it clear that it was time to take action against the demons playing inside his sinus cavities.  You might wonder why 'they' didn't take decisive action sooner?  1- because sinus surgery on a 3 year old isn't antibody's idea of a party.  2- a scan of the sinuses of a person with CF will almost always look 'abnormal'.  We needed some more definitive evidence, before taking definitive action.  His CT shows that the swelling of the tissues in his sinus cavities had not only completely closed them off, but had begun to push his eye socket out of place.  One week before surgery, Jackson developed a seriously nasty form of bacteria called C Diff that threatened to keep him from surgery, and worse, stripped him down to about 32 pounds. We treated aggressively with probiotics and a different kind of antibiotic that would specifically target the bad bacteria, and were able to go forward with the surgery necessary to relieve his sinuses. 

ON October 18, Jackson had surgery to clean out diseased tissue in his sinuses and remove his adenoids.  His recovery was gloriously quick, and the three weeks that followed were fantastic.  The best of our year. He ATE.  I mean, he really ate.  Entire helpings of nachos, multiple slices of pizza, whatever he could get in his mouth.  It was like he could taste food for the first time in his life.  The second week of November, he got a cold.  It held on a little longer than a normal cold, but because he had just spent seven solid months on antibiotics and had battled  C Diff as a result,  giving him a course of antibiotics then was not the right call.  Around this time he also began to throw up his tube feedings.  Not every time, so we tried slowing them down.  We tried giving him zofran to keep him from vomiting.  His weight sunk down to thirty pounds, and his bmi dropped from the 60th percentile down to the 3rd.   Every vertebrae stuck out.  His hip bones were sharp.  The back ridge of his pelvis began to protrude, and his elastic waist, skinny jeans wouldn't stay up. Not only could I see each rib, but I could see the outline of where each rib connected to his sternum.  His collar bones stuck out, and his eyes were sunken in his face.  I glanced a picture of what he looked like a year before and I sobbed.  We consulted with his doctor and nutritionist and got approval to try giving him 24 hour a day tube feeds in an attempt to put some weight back on him.  If he didn't do well, he would be spending Thanksgiving in the hospital.    Over the weekend, 24 hour feedings and zofran managed to stack about 4 pounds back on him, and we were cleared to be home for the holidays.   Jacks and I made a quick surprise trip up to Nebraska to see my folks.  And he started throwing up again.  In spite of the joy of being together as a family, my Mom's eyes would follow him around the room with sorrow. It was impossible not to see the worry on her face.  And the day before we left to come back home, in the middle of a busy, noisy family gathering, my rambunctious child crawled into my lap and fell asleep, too weak and tired to play with the other children.   He was spiking fevers around 103. I looked up the Children's hospital nearby to make sure they had a CF center and made a contingency plan for scenarios I didn't really want to consider.  The next day we left early.  Jackson slept 8 1/2 out of the ten hours we drove home.  The following day was a Monday, and I knew we would be checking in to the hospital.  

We had a bag packed as we went in to visit the CF center.  As we waited to make sure a room was available, Jackson played and hid under the table, and didn't seem to feel as bad as we thought.  I prepared myself for the standard ten day 'tune up' that helps CFers bounce back when they're just a little too beaten down to do it on their own.  Two hours later, as we began to unpack our little suitcase in our room, he suddenly wasn't playful at all.  He cried and crawled into his hospital bed, and he didn't get out of it for four days.  His high fevers continued to spike.  He continued to vomit. His white blood cell count skyrocketed. His blood pressure was abnormal.  A small spot of pneumonia in his lungs bloomed into a full-scale war inside his chest.  My baby wasn't in for a tune up, my baby was in for a fight.   During his PICC placement, he was on 6 liters of oxygen. (that's a lot) He was in acute respiratory distress. There were x rays, blood  draws, blood cultures, sputum cultures, vital checks every 4 hours, nurses with worried faces, and the fear that we were headed for the PICU if things didn't look up.  As the labs rolled in, they confirmed that everything he was fighting was viral.  Around day five his fevers stopped, and his whole body broke out in a rash.  He began to eat a little, and slowly began to tolerate feeds.  by late day 7, we had hope again.  We managed to make it home on day ten.  

In the month since we have been home, our main goal has been to fatten our little dude up. His tube feeds have supplemented him with 15000-1900 calories a day.  And yet he's roughly the same weight he was when he was discharged from the hospital.  We have watched his appetite turn to nothing. He has suddenly begun to have a total meltdown any time it's time to take his enzymes (which is at every meal and snack).  He has also thrown up a couple of tube feedings, and spent over two days refusing to eat ANYTHING.  We have an appointment on Monday, at which we will do some more digging and try to uncover what the hell is going on this time. 

This has been one year in Cystic Fibrosis for us.  A disease that progresses with age. I can't do nothing just because feel like my last efforts weren't good enough.  I'm not fighting just because I don't want this for Jackson.  I don't want this for your child.  I don't want this for my friends.  I don't want this for ANYONE.  We are coming SO CLOSE to treatments that can give people with CF a 'Normal' lifespan.  We are so close. Sitting in the hospital, watching my child struggle to breathe, watching the flesh shrink  into bone, and we are SO CLOSE, but we are not there.   We have to get there.  And I need you to help me do that.

This is our Great Strides Team. Click through and join us. Help us.  Ask your friends and your co-workers, your bar buddies and your golf pals, have a little contest at work. Shoot me ideas.  You don't even have to be AT the walk to be on our team.  but I am asking you to join us.