Back to the OR :(

Leadup – Where do I start. Sort of crazy last few days. Guess it all started with a uncharacteristic cough on Sunday night which naturally worried Mom and Dad since I have CF and we never want me to get sick. Then on Tuesday, Mom went to Dallas for training on some new software so it was just me and Dad and we were hoping to watch the Capitals continue their hot streak in hockey and get some rest. Instead, I just couldn’t get comfortable and kept waking up Dad (more like not letting him go to sleep) so he contacted Children’s National. Upon explaining the symptoms to the on-call Pulmonologist, the suggestion was simple – “Bring Jack in and let’s take a look at him.”

Wish Children’s would get some more manly-colored gowns

TUES: Upon Immediate Arrival – So Dad loads me up in the car and we head to Children’s at 12 midnight. The good news about heading to the ER at midnight is there is no traffic getting there and usually isn’t much of a line so we were seen within 15 minutes and headed straight back to an ER room. We then waited for hours until a room was available on the Pulmonary wing of the hospital (9:00 AM). This whole time Dad never told Mom we were heading at the hospital. He dodged FaceTime calls and everything to avoid worrying Mom who was many miles away. Mom is not very happy about this. Expect some changes around the Peden household as it relates to information sharing.

The symptoms which Dad felt were necessary to bring me in were actually warranted – slight fever, hard and distended abdomen, lack of appetite and no stooling. Dad’s heart was sinking. We had just left Children’s about a month ago for what was a small blockage and Mom was out of town. The ER nurse hooked me up to and IV sent me off to X-Ray.

CF Sucks! Let’s Find a Cure!

Above, is where I am getting ready for my first X-Ray this stay. Dad said it reminds him of Peter Quinn in a recent episode from the hit show Homeland. Dad wondered what it would be like to send this photo to mom who was still 1340 miles away. He thought better of it and opted to simply text sweet messages about how perfect I am.

Trying to find some relief

WEDS: Off to Pulmonary – After four X-Rays, an IV, and a NG tube…we headed up to Pulmonary where were finally examined by the specialists. Naturally, they were concerned as to why I was back so soon and wanted to get to the bottom of it and Mom began to become concerned as to why Dad was ignoring FaceTime calls so then Dad answered and holy s***, you would have thought Mom slammed her fingers in a car door. She immediately left the breakfast table and began sobbing on the video – exactly what Dad hoped to avoid. So Mom sat through half of training, changed her flight and headed home early…arriving to the hospital at about 10:30 PM. From a medical perspective they kept me hooked up to the IV for continued fluids and an intermittent sump (suck) on my NG tube to try and suppress the distention in my bowels. They also pumped 800 mL of GoLytely through my NG tube in hopes to soften up any stool which was causing a blockage.

I don’t want to go to Radiation

THURS: Enema #1 – Oh sweet, more enemas. Not really. Since the sump wasn’t doing too much and my distention or comfort level hadn’t improved much at all, this was the next procedure to try. For anyone who hasn’t had an enema, let me just say…they are not fun and if you don’t believe me, I highly encourage you to listen to a fellow CF’er, who takes a humorous approach to an enema he had as a teenage boy. You can listen to it here – Sickboy Podcast.

Contrast Enema – Large Bowels / Colon is the Bright White Area

In this photo, the contrast successfully made its way from my colon and large bowels, but stopped right along the area where it joins up with the small bowel.

Playing the waiting game

While this enema gave us some hope, it’s still concerning. The team of surgeons felt we should try Mucomyst down my NG tube and would try another enema in the morning. My abdomen continued to grow even with suction on my NG tube.

FRI: Enema #2 – At about 3:00 AM Friday, I began to vomit up the Mucomyst that was inserted into my NG tube which wasn’t good as that was a sign that this block likely wasn’t going away like last time. It also meant the enema was the only chance to avoid surgery. At 9:00 AM Friday,  Dr. Sami rushed me down to the radiology department to get me in right away. The doctors opted to include Mucomyst in my enema this time around hoping to penetrate the blockage and loosen it up. Humans would be shocked at how much fluid one could shoot up one’s colon into their bowels. At any rate, this didn’t work and the contrast actually didn’t make it as far as the day previously so Radiology, Surgery, Pulmonary, and everyone else weighed in on what to do next.

Surgery – Without much hesitation, everyone felt surgery was necessary so it was back under the knife since day two of birth. At 2:15, they put me under, while Mom, Dad, Rachel, and Pop waited upstairs. After about two hours, the surgeons came out to give Mom and Dad the news.

Surgery decided to stick me 500 times after I pulled out my IV just prior to surgery. Here’s one of my arms. My legs and feet look the same way.

While surgery is never good, this surgery was as favorable as surgery could be, given the options that were on the table. Cutting along my previous incision, surgeons began to pull out my small bowels. They quickly noticed  that my small bowels had adhesions, likely from the previous surgery that was completed to correct my Meconium Ileus (MI). Anytime the bowels are cut or touched, adhesions are not uncommon. Abdominal adhesions are difficult to predict and do not show in imagery so this wasn’t something that had been discussed with Mom and Dad. All Mom and Dad were told is that the exploratory surgery would lead to any number of things – removing part of my small bowels, an ostomy bag, etc. Surgeons worked quickly to remove the adhesions and straighten out the small bowels where they seemed to have “corkscrewed” right at the Ileum. Given its location along the bowels, the Ileum is the most difficult area to get to and work on which is another reason surgery was practically unavoidable at this point. After my bowels were pulled out, they sucked out all remaining fecal matter through my NG tube and felt there were no signs of Meconium in my small bowels so they did not need to cut along my bowels again. Because the small bowels did not need to be cut open, this was great news. In a way, it means that my mechanical workings of my bowels appear to be operating properly, but the adhesions were the likely cause of the problem. As such  this was not a direct correlation to CF, but more-so of my previous surgery (which was due to CF).

In the PACU Recovery Room

Sat: On the Mend – My team here at Children’s reassured the other half of Team Jack that there was nothing they could have done to prevent this and that they have been doing a great job thus far. Abdominal adhesions are just a thing that is more common in individuals who’ve had some sort of abdominal procedure (i.e. Appendectomy).

This stint at Children’s should serve as a friendly reminder to Team Jack and others that we have to be extra careful this holiday season and do our best to keep me away from people that are sick or might be sick. We all must do a good job at cleaning everything thoroughly to reduce my exposure to germs. Also, anyone picking me up or holding me…shall be very careful not to pick me up by my arms/underarms/etc as that will pull on the incision and other areas of the abdomen which are incredibly tender and sore.

Thanks everyone for the well wishes and to all of those checking in on me. I’m determined to not let this slow down my ability to full out walk. I was making good progress just prior to this incident so while I’ll be sore, this isn’t going to hold me back. I’ve already begun pulling myself up on the crib rails. Hoping to get out of here before Christmas (maybe as little as one week).

National CF Month and 3 Months Old

I turned three months old this week and have been out of the the NICU now for 49 days (counting today). My friends at Children’s NICU would tell you I’m 92 days old today. That’s nearly one day for every year my one great grandmother that I saw a couple weeks ago is old.

It was a big sports weekend and my parents and I watched them all (derby, fight, playoffs) but the highlight was definitely the Capitals Game 1 win against the NY Rangers Thursday night. Ovi had a nice goal Saturday, but it wasn’t enough to overcome the early NYR lead.

Jumping for Joy

On Friday night, I hung out with my grandmother while Mom and Dad went out to dinner with another couple from Arlington who has a child with Cystic Fibrosis (CF). Mom and Dad told me that they have a super cute daughter who is doing really well (thriving). Their daughter has a different mutation than I but should actually benefit form the Kalydeco drug I referenced in my last post when eligible to begin using it (have to be older). During dinner, both sets of parents explained the treatment plans in place for their children and “compared notes.” Mom and Dad left dinner with a wealth of questions they plan to email their team of doctors to ensure I’m getting the best treatment options available. Their daughter is on Levalbuterol and Pulmozyme (AKA Dornase Alfa). They also tuned Mom and Dad onto a new nebulizer which was recently approved by the FDA, ERapid Nebulizer, which is designed to cut down the nebulizer time. So if you’re a reader or stumbled across this blog and have any other thoughts on CF Treatments, please feel free to reach out or leave me a note in the comments section.

“Only a CF parent would know!”

While the conversation between parents came easy and natural, all four chuckled when discussing what its like having Creon granules randomly stick to their feet after countless nights of preparing Creon-applesauce mixtures in the middle of the night while they’re dead tired. Perhaps when I’m done here, I’ll encourage Dad to look for a list for “things only a CF parent would understand” and so he can keep up the laughs.

Reading a book

Above is a picture of me reading a book sent to me from Dad’s pre-school teacher. Here’s me and Dad saying Thanks Ms. Ward.

Lastly, I’d be silly not to mention what May is. Yes, May is the famous for Memorial Day Weekend AKA “MDW” AKA the start to summer, but the month of May for me will always be known as the National Cystic Fibrosis Awareness Month.

Here’s a link to TMZ’s Great Strides page where she’s walking to raise awareness in Reston, VA. You can also click on the icon below to head directly there.

Click here to see TMZ’s Page in Support of Me

As this is National CF Awareness Month, many of Mom and Dad’s friends and family ask what can they do to help or when are Mom and Dad going to do something/host an event. Mom and Dad know how awesome all of their friends and family are and would like to tell you that they are working on something. It’s just been a whirlwind of being first-time parents, coupled with the fact that I sent them through the ringer those first 42 days in NICU, but they are working on doing something in the future and will let you all know as details emerge. That being said, feel free to offer up any suggestions/tips in the comments section below or reach out to Dad directly for information on non-profits, establishing a foundation, good ideas for an event, etc. Mom an Dad are all ears at this point, but express desire to conduct something annually, that’s everlasting, and raises money for a cure. Thank you all so much!

Pancreatic Elastase and Sweat Test Results

Pancreatic Elastase 

My pancreatic elastase results came back Wednesday and as suspected, I have a severe pancreatic enzyme deficiency (less than 100 mcg/g). My results yielded less than 15 mcg/g. A normal range would be >200mcg/g, while a moderate insufficiency would be between 100-200 mcg/g. This didn’t really come as a shock to myself or my parents since the CF Dietician already began introducing the enzyme, Creon. This test was the original “gold standard” for determining Cystic Fibrosis, prior to the sweat test.

Sitting in Dad’s Lap

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Sweat Test 

The sweat test was conducted on Thursday and the results of the new “gold standard” are in. Harvesting sweat from both my arms, doctors cited a score of 30/95 in my right arm and 40/98 in my left arm. The low numbers you see is the volume of sweat obtained while the “95” and “98” are the chloride levels. Below is a breakdown of the chloride level thresholds.

For infants up to and including 6 months of age, a chloride level of:

• Equal to or less than 29 mmol/L = CF is very unlikely
• 30 – 59 mmol/L = intermediate means that CF is possible
• Greater than or equal to 60 mmol/L = CF is likely to be diagnosed

This test indicates that I have more chloride (salt) in my sweat than a normal, healthy individual. This is what leads to pulmonary issues amongst individuals with Cystic Fibrosis.

Sweat Test Results Reaction

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Conclusion

The results of these two tests plus factoring in several other symptoms and complications such as Meconium Ileus (MI) lead doctors at Children’s National Medical Center to diagnose me with Cystic Fibrosis (CF). I am now one of the 30,000 Americans living with CF.

Everything is going to be alright.

Tuesday, March 10, 2015

Sort of an interesting 48 hours since I last posted.

On Monday, the Children’s Pulmonary Team stopped by to talk mom’s and my ear off for a long time. While still no confirmed diagnosis of Cystic Fibrosis, more and more signs are pointing in that direction so they are just preparing us for what’s likely in store. Children’s has already seen nine children with CF this year alone, four of which had Meconium Ileus prior to a confirmed CF diagnosis. The team mentioned how they were going to attempt the sweat test again on Tuesday, but they never stopped by so who knows when that will happen. Tomorrow I’ll for certain have a hearing test (standard test given to all babies prior to leaving the hospital).

Creon (Enzyme)

At noon on Monday I reached the full feeding level (80 ml) for someone of my height and weight (4.25 kg) and they began introducing an enzyme, Creon. The CF Nutritionist didn’t seem 100% sure if I should be taken it yet or not because I’m not showing obvious signs of malnutrition. That being said, the risks are minimal to non-existent so we are proceeding with caution. The biggest risk is that the capsules have to be split in half and the tiny granules are mixed into applesauce. By mixing it with the applesauce, the applesauce serves as the coating to help the medicine make it further down the digestive track. The exposed granules can also lead to irritated skin on exposed surfaces such as my lips or as the food travels out if you catch my drift. 😦

While no “signs” of malnourishment, I have lost some grams in total weight so hopefully that does not continue. Once I’m consistently gaining weight and continuing to poop, they’ll likely discharge me. Last night I may have been overfeeding as I vomited after all feeds up to 9:00 AM this morning so we’ll monitor that as well. 😦 As of 10:15 PM tonight, I have not vomited or spit up the slightest so hopefully that’s working itself out as well. 🙂

And today, they finally removed the PICC line from my right arm. This is awesome as I can begin wearing clothes more normally and not have the collars all stretched under my right armpit. Only four monitoring cords remain. 🙂

Lastly, my Great Aunt Dianne stopped by unannounced to say hello to me and we had a good afternoon full of laughs. 🙂

Oh and around 10:00 PM, Mom came back up here to see me, thanks in part to her long and previously documented battle with FOMO. 🙂

February Recap

While February is the shortest month of the year, spending all of it in the hospital has felt like an eternity for me and my parents. I literally turned Mom and Dad’s lives upside down this month. I smashed my Aunt Coco’s NICU length of stay by double and I’m still going. Now that I own the family’s record for NICU stay in number of days, it would be nice to get out here anytime now. Let’s hope that March allows that. Maybe that kooky psychic will be right and I’ll be at home watching March Madness, NHL/NBA Playoffs and the start of MLB with Dad.

No NG Tube = Freedom

The surgeon’s opened up March with a bit of hope.  They are pleased with how far the barium/water fluid travel to my dilated bowels on Friday’s contrast enema and the number of stools (7). Today, they’ll turn the sump off on my annoying NG tube and remove it if I can stand to take the mucomyst orally. For those of you who don’t know…mucomyst smells like rotten eggs sitting in sweaty sneakers in a men’s locker room during the summer – absolutely horrible!

Stuntin Like My Daddy

Tomorrow I’ll have a seventh barium enema and that will determine if a) the NG tube should stay out, b) if I can begin drinking milk again, and c) if they think I’ll need to have an ostomy. The team of surgeons, led by Dr. Sandler, mentioned that I may need an ostomy at some point. The idea behind the ostomy is that it will allow for the bowels to flow more freely on their own and not cause a bottleneck near the narrowing passage from where it goes from dilated to normal (imagine a funnel full of rocks trying to work there way out).

I’m watching you

So there you have it. My first month on Earth has been spent in a hospital. Hopefully I receive a good bill of health and my Mom and Dad feel they can head to Raleigh for Jerel’s wedding in two weeks. Jerel shares a birthday with Dad and they played football, basketball, and baseball together in high school. Dad’s looking forward to being in the wedding and wishing his childhood friend many of years of happiness with his beautiful bride.

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Lastly, for those of you who like craft beer, good food, and silent auctions, you should book your tickets for the Brewer’s Ball at at the National Building Museum on March 7 from 7:00 PM to Midnight which raises money for the Cystic Fibrosis Foundation. The event usually sells out, but tickets are still available and it’s for a good cause.

Happy Valentine’s Day

Not sure what all the hoopla is about with this holiday. Hopefully Mom is understanding of the fact that Dad hasn’t had too much time to make the Hallmark holiday too special for her this year. If I know Dad, he’ll have pulled a little something together for her and if not…aren’t I good enough? I mean…I wasn’t even expected to be here for another 8 days.

My First Valentine

Dad also thought it would be a good idea to do something special for the nurses and staff here for providing such good care to me over the nearly two weeks so he ordered a nice Edible Arrangements bouquet for the staff. Hopefully they enjoy it, but not too much that they don’t come in to check on me.

Ladies are Safe, I’m Locked Up Tonight

Because my abdomen is actually a little firmer than first expected, the surgeons agreed with the nurses and opted to place a nasogastric tube through my nostrils so that I the bile and gaseous buildup will subside some so that I’m more comfortable and can continue eating. For now, all my nutrients are coming through the PICC and not Mom’s milk. They may also try an enema (where fluid is pumped inside of me in an attempt to get the poops flowing). I tried to tell them that my abdomen is firm from the workout program my Dad put me on. Just trying to get that six pack ready for Memorial Day Weekend.

They put this tube in my nostril

Also, my Great Aunt Dianne sent me some cool set of boxing gloves. While they’re cute…they’re not practical :). They do not fit my gorilla hands. Guess I can thank my Dad for blessing me with these huge hands. Aunt Kate took Dad’s idea a step further of modifying an outfit (cutting off right sleeve) by adding buttons down the length of the right side and shortening the sleeve so that I can wear an outfit without having to have it hang off my right shoulder while also protecting the PICC line. I’ll post a picture of these tomorrow, but don’t feel like removing this swaddle at the moment.

Mom’s Valentine

My Tuesday Evening Update (02/10) – Mmm Mmm Mmmilk

Today was another awesome day. Aunt Rachel came up to visit with me and mom again and I had my first real taste of mother’s milk. While healthy, week old babies my size, typically put down about 60 mL every three hours, the plan for me is to limit me to 5 mL according to Nurse Nicole.

Milk Does a Body Good

Also, my nurse thought it would be okay for the oxygen tubes to be removed again, so Dad thought it would be fun to write on my tape as though it were eye black worn by football players on the gridiron.

John 3:16

John 3:16 seemed to be what jumped out in Dad’s mind. Dad admittedly had to look up what the verse said. I wasn’t a huge fan of Dad’s antics as you can see from the picture he took of me seen above.

For God so loved the world, that he gave his only begotten Son, that whosoever believeth in him should not perish, but have everlasting life.

You Talking to Me?
(No Tubes)

On a bit of a sad note, this will be the first night without Mom or Dad sleeping at the hospital with me. Aunt Rachel will be coming up to stay with me so Mom and Dad can finally spend a night together since January 31. I hope they enjoy it because I expect Dad back up here tomorrow night.

Jack’s Status: February 08, 2015 – I Pooped

At about (2 hours ago) 11:30 AM while dad was rocking me in the chair, I was able to muster up my first bit of poo. Mom asked the nurses if this was a good sign or not. I just rolled my eyes because I could of told her that it was good sign as I feel much better now.

Dad rocking me until I poop

Hopefully I can poop a little more so they can introduce some of mom’s milk she’s been working so hard to produce.

I’ll post a more comprehensive update as I approach my first full week on Earth (8:02 PM). Stay tuned!

Cal Ripken Jr or Jack?

So my dad is confused? He doesn’t know why a photo of him and Cal Ripken, Jr. has garnered more Facebook likes (94) than a my very own WordPress site which outlines my journey (88) that he also posted. Could dad’s FB friends really like Cal more than me? Dad tells me I’m the most important thing in his life right now.

Don’t get me wrong, dad definitely feels it is a great photo of him and his childhood idol — he even considered using it for Christmas cards (see card he did in Paint below) and possibly for my birth announcement if Mom let him. No one in this world grew up liking Cal more than my dad. It’s almost eerie in a way. I was born at an even 8.0 lbs and in delivery room #8 at VA Hospital at 8:02 PM. Cal wore #8 his entire career.

Dad’s twisted sense of humor to spread holiday cheer

So if my dad’s FB friends want, feel free to hop on over to his FB page and like the post for me – okay? Jack > Cal

DISCLAIMER: In 100% honesty, this post is not for my father to actually get more FB likes. He just wanted to point out the coincidences of the number 8 and what was running through his mind in the lead up to me being born. Just follow me – Jack.

February 3-4, 2015: Postoperative

Following surgery on Tuesday evening, I was transported back up to the third floor, but with a endotracheal tube (breathing tube) shoved down my throat to add to the laundry list of other items (gastric suction tube for stomach bile, Intravenous (IV) tube for nutrients, thermometer in armpit, heart rate/respiratory monitors, etc). To date, I think I’ve been stuck somewhere close to 400 times when including heel pricks. Needless to say, this shit is getting old, but it is suppose to make me feel better so I’ll roll with the punches.

Post Op 3

I had a blood transfusion immediately following surgery. It would turn out I would need another the next day, but that didn’t concern the awesome staff here too much. Glass half full types might say, “well at least I know what blood type I am”. After I bust out this joint, I hope I never need to know, but in case you’re interested, I’m A+ for blood type. Children’s has blood donations from 8:00 AM – 5:00 PM daily and dad and family members plan to donate. For more information on blood donations at Children’s, visit here.

A Positive