Happy Birthday, Porter!

Porter turned two years old today.  Most parents will say how fast the time goes; "I can't believe he's already two!"  Although we have a touch of that, the more predominant feeling is "Wait, he's only two!?"  Our family has overcome some pretty crazy challenges in Porter's lifetime which makes it feel like he's been with us longer than two years.

This year we have a lot to celebrate and be thankful for.  Porter is now 6 months post-transplant!  He is down to 4 medications per day and, just this week, came through his first illness without hospitalization!  We all got to stay home and be sick together...it was great! Ok, so maybe not GREAT but such a relief to know that he is allowed to get sick now.  Before transplant if Porter had a stomach bug like he did last week we would have been visiting our friends in the UVMMC PICU within a couple of hours of onset.  This time around we gave our team in Boston a call and they told us to try to keep the Pedialyte flowing through his g-tube and just wait it out.  His body can handle it.  How awesome is that!?

Porter is growing and developing like a normal two year old.  His favorite part of Thanksgiving dinner this year was the turkey.  The pumpkin cheese cake was a close second.  His verbal communication skills are coming along.  He loves drawing and play dough and he can't get enough of tractors and trucks.  Oh, and  you should see him dance...pure joy.

Happy November!

I really mean it. Happy November! This week will mark 5 months post transplant for Porter. And, with the exception of his up and down white blood cell count (neutrophils), he is doing better than ever. He is continuing to eat, and with his birthday on the horizon next month, we are glad we aren't planning a low protein celebration.

Sunday feels. Shhh. Don't wake them.

We had a nice Halloween. Katie was Ariel and Porter was a tiger. The tiger stayed at home with Dad to handout treats and Katie and mom trick-or-treated around the neighborhood.

Is she Raggedy Ann, or Ariel?

Porter loves tractors and lawnmowers. He will sit on the lawnmower for hours if I let him. Just pretending to "drive, drive". He will also watch me mow the yard from start to finish. I hope he's just as enthusiastic when I make him mow when he's 12. #tractor #mowthelawn #PorterforPresident

A post shared by Mr Johnstone (@techiesciguy) on Nov 4, 2017 at 12:10pm PDT

We borrowed a @tinkercrate kit from a friend. Katie is seriously hooked! She connected the electronics herself and ran out of paint making awesome art on the Spin Art Machine. Looks like someone is getting a subscription for the holidays. Does tinker crate have stuff for grown ups? #tinker #makestuff #electronics #art #project

A post shared by Mr Johnstone (@techiesciguy) on Oct 27, 2017 at 2:04pm PDT

Neutropenia

Author:  Sarah

Two weeks ago BCH called to let us know that Porter's Absolute Neutrophil Count (ANC) was low.  Neutrophils are a type of white blood cell that help us fight infections, especially those caused by bacteria.  If your neutrophil count is too low you are considered "neutropenic" and even the normal bacteria we all have in our bodies can cause an infection.  The team told us that if Porter were to get a fever we would need to take him to the Emergency Department at UVMMC right away.  I was hoping that maybe the low ANC was a false lab result but they explained that one of the medications Porter has been taking since transplant can have the side effect of suppressing bone marrow.  Yikes!  So they asked us to get repeat labs the next week (last week) to keep close tabs on those neutrophils.

The repeat labs taken last Wednesday (10/11) showed a continued drop in ANC.  The team advised us to discontinue the drug valganciclovir (an anti-viral used to prevent cytomegalovirus) and also the cyproheptidine (an antihistimine which we were using to stimulate his appetite).  We also began a regimine of G-CSF (granulocyte colony stimulating factor) which is a medicine that stimulates the bone marrow to produce more white blood cells.  The bummer is that the type of G-CSF treatment Porter is getting is injected.  The double bummer is that we have to do the injecting 😢.  The good news is despite Porter's dislike of the injection it really doesn't seem to hurt him all that much.  He cries more from anticipation (the boy screams when he sees the alcohol swab) than he does from the shot itself.

We started the daily G-CSF injection on Thursday 10/12.  That evening (it seems like this stuff always happens at night) Porter developed a fever.  We called our friends at UVMMC and brought him in to the E.D.  They were ready for us and took us straight back to a private room where they accessed his port, drew labs including blood cultures, and started IV antibiotics.  We are so spoiled with our quick E.D. admissions.  If we ever have to be there for something "normal" and have to wait to go through triage, I won't know what I will do with myself! Porter was admitted to the regular pediatric floor (no ICU!) and we had to sit tight for 48 hours to wait for the blood cultures to do their thing.  That is standard procedure for people who are neutropenic with fever.

Porter was in isolation during the admission to protect him from other people's germs.  Unfortunately, it was probably the smallest room on the floor but the double doors made it quiet and it had a private bathroom.  Porter never showed any signs of illness and the only fever was Thursday night at home.  That meant that he likely developed the fever in response to the G-CSF injection but we'll never know for sure.  His ANC did drop to 0 (as in no neutrophils) at one point but after the requisite 48 hours and once his neutrophil count started trending upward the BCH team was comfortable letting us go home.

Porter's "we're going home" face.

Labs taken this morning show that Porter's neutrophil count is now up over 4,000.  Well within normal range.  Our BCH team wants us to hold off on the G-CSF treatments for now and get labs again Friday.  We will also head to Boston this weekend for labs and a transplant clinic appointment on Monday morning.

September 2017 Update

Well, I would say it is time for a Johnstone update! We last left off in mid August and Sarah and I were headed back to work as the school year was getting underway. In August we were traveling to Boston every other week for clinic visits for Porter and visiting UVM for his blood work on the off weeks. At Porter's last visit to Boston in August, they lengthened our clinic visits to 4-6 weeks! We had one clinic visit this month, and we will return on November 6. Porter also does not need blood labs every week! All of these updates should tell you how well Porter is doing.

Noodles are the best!

Every day is a bit different, but Porter is now eating a significant portion of food by mouth. We still supplement his daily intake with some liquid formula and pureed foods. But, we are starting to back off a bit on the purees as he eats more and more on his own.

The physicians also reduced Porter's medications a bit. He was taking nine medications since discharge from the surgery in June. He now only takes six medications including one that stimulates his appetite a bit. We think it is working and will continue until he shows us he can detect and satisfy his own hunger on a consistent basis.

Porter's morning regimen post transplant.

Porter's liver function tests are normal and he is maintaining a good body weight. Our only issue lately has been keeping his kidneys happy. Even with a reduction in medications, his blood tests show that his kidneys are still working hard to process all the meds. The secret to reducing stress on his kidneys is hydration. While Porter is eating a lot more, he is more reluctant to drink. So, we are trying our hardest to provide more than a liter of fluid via his G-tube every day. That is a bit tricky! A liter is a lot of fluid for a little person!

During our visit to Boston last week they said that they wouldn't need any blood lab work for a month. We realized that two weeks is the longest Porter has ever gone without blood work, let alone a month! Another sign that Porter's transplant has made his life more stable. We are also grateful that with the exception of his little infection back in July, we haven't been to the emergency department in months! With at least one ED admission per month essentially since birth, it's more than a bit surreal.

Also at our last clinic visit, Porter's blood test for Epstein Barr Virus (EBV) was elevated. We knew EBV was in his system; his liver donor tested positive for EBV, as do many adults. Porter's EBV has risen since our August clinic visit, so we were told to lower his immunosuppression medication (Prograf) in hopes of giving his immune system a small boost so it can effectively lower the EBV level.

We had a nice conclusion to our summer and the leaves are starting to change around here, telling us Fall is well underway. Our next challenge will be cold and flu season this winter. Stocking up on hand sanitizer!

 

A Tribute to My Father

Author:  Sarah

If you’ve ever tried to write a eulogy for someone, then you know it’s pretty challenging.  How do you express a lifetime of love in just a few paragraphs?  I assure you that what I have to say is inadequate; but I hope to convey a reasonable reflection of my father.  

Dad was kind and generous.  He was a good neighbor.  I have memories of him using his snowblower to clear nearly every driveway on our street.  Always willing to lend a helping hand.

Roger J. Metivier

October 19, 1949 - August 12, 2017

He loved his family and he LOVED ice cream.  That’s right.  Dad’s passion for ice cream is eulogy worthy and I have a story to prove it.  My husband Chris is more of a cake guy.  He grew up in a family that celebrated birthdays with cake but not necessarily ice cream.  So, for our daughter’s third birthday Chris decided that we would serve cake but no ice cream.  Please let the record reflect that I did caution him.  Well, when it came time for dessert naturally Dad asked “where’s the ice cream?”  When we responded “there’s no ice cream, Dad,  just cake” he couldn’t believe it.  I’m sure that he’d spent all day anticipating a couple of scoops of his favorite frozen treat and we had clearly disappointed him.  He was so put out that on the drive home from our house he made Mom stop for an ice cream cone.

I learned many life lessons from my father but the one that I think about nearly every day is something he said to me several years ago.  I must have been home from college on break.  I was complaining about something and I honestly don’t remember what it was which only helps to illustrate this point.  Dad finally shrugged and said to me “worse things have happened to better people.”

Now, that seems sort of harsh but Dad wasn’t exactly a tactful person and I know that he did not intend to offend me.  He simply wanted me to gain a little perspective.  No matter what your challenges are there is always someone out there who has it worse off than you.  I’ve learned that when you embrace that idea it’s easier to appreciate the good things that might otherwise be overlooked.

For instance, I am so thankful that I had a chance to be with Dad last Saturday.  I brought my children for an afternoon visit and we spent a couple of good hours together.  Later, when he was in the hospital, Mom, Eric, and I were all there with him.  We were together when he passed.  We were there for him and for each other.    It couldn’t have been better than that.

Earlier this year my son, Porter, needed a liver transplant.  The cool thing about the liver is that surgeons can take a portion of a healthy liver from a live donor and place it into the recipient.  Dad was devastated when he found out that he was not a viable candidate to donate a portion of his liver to Porter.  He was too old and definitely not healthy enough but he really wanted to give Porter that gift.  So, when the hospital called and asked if we wanted Dad to be a tissue donor we said yes.  It is with great pride that I wear this butterfly pin from the musculoskeletal transplant foundation.  This pin is a symbol of Dad’s gift.  The long bones of his arms and legs will be used to improve lives.  Dad would encourage all of you to consider organ and tissue donation as well.

I believe that Dad is at peace now.  He is whole again.  Healthy and strong.  I sincerely hope that wherever he is now he does not need to miss us because missing loved ones is painful.  I especially hope that he does not miss us because if he misses us than he DEFINITELY misses his ice cream. And that would be tragic!

So, before the summer is over take the time to stop for an ice cream cone.  Enjoy it with someone you love and think of my Dad.  He’d like that.

Feeding Porter

Author:  Sarah

Ever since Porter's first bout with hyperammonemia he has been a sub-awesome eater.  I suppose that can mostly be attributed to the fact that we began a strict micromanaging of Porter's food consumption immediately after his OTC diagnosis.  Not only did he have to drink a nasty-tasting metabolic formula but the nitrogen scavenger medication had the unfortunate side effect of decreasing his appetite.  Add to that the rigorous feeding schedule which stressed us all out and who can blame him for avoiding food?

One of the things that has always annoyed me about the way we had to feed Porter was how unnatural it is.  My reality check (when I got really frustrated and tired) was that his survival was pretty "unnatural" and the whole crazy process of feeding him was how we kept him safe and alive.

In the spring after his diagnosis Porter's formula consumption started to drop off.  At first it was just an ounce or two here and there but eventually he was eating far less than he should which lead to concerns about dehydration, failure to thrive, and the dreaded catabolism.  Porter had a few rapid-re-admissions between April and May of 2016.  After that we had a relatively quiet summer thanks to the placement of a gastrostomy tube (G-tube).

A naso-gastric tube was placed when Porter was admitted to UVM prior to the placement of the G-tube

When our medical team suggested that Porter would benefit from a feeding tube Chris and I both balked at the idea.  My first thought was that feeding tubes are for people with severe disabilities; and while not being able to metabolize protein properly is a pretty HUGE disability it didn't seem to qualify in my mind.  I've since learned to love Porter's G-tube because it has allowed him to grow and thrive.  With the G-tube there was no more waking him in the middle of the night to eat or take medications, we could simply put it through his tube.  He packed on the pounds over the summer.  Suddenly folks were using words like "solid", "chunky", and my favorite phrase "he's all there" to describe him.  Our metabolic team wanted Porter to have extra reserves because "fat babies with OTC are healthy babies with OTC."   The G-tube allowed us to get there.

Do you eat while you sleep? If yes, then you probably have a G-tube.

Now, don't get me wrong.  G-tubes aren't all sunshine and rainbows.  Just ask Chris; he has at least one tubie nightmare per week.  One day Porter pulled his G-button completely out! A handful of times we either forgot to fully clamp the tube or it accidentally opened during his sleep or in the car seat and drained his entire full stomach. And, don't get us started on the automatic food pump. We've learned more about the physics of fluid dynamics than we ever thought we would. But, ultimately, the tube saved Porter's life so we try not to complain too much.

The consequence of the G-tube that we tried quite unsuccessfully to avoid was the development of an oral aversion.  It's sort of a blur now how it all happened.  The original plan after Porter's G-tube was placed was for us to offer him a bottle at each scheduled feeding and only use the G-tube if he didn't finish what we offered.  I honestly don't remember the transition.  All of a sudden Porter just wouldn't take a bottle any more.  We tried sippy cups and straw cups but he wasn't interested.  And why would he be?  Given the choice between tasting the yucky formula/doing the work of eating vs. just having that stuff pumped into your stomach so you can go about your day playing and exploring?  I think we'd all choose the latter.

Thanks to the guidance of our occupational therapist we were able to avoid a total oral aversion.  Porter would put non-food items in his mouth all the time and would occasionally (usually only for the OT) put a food item in his mouth or taste juice from a spoon.  Porter's muscle tone is good despite not having much experience with eating because of the exercises she prescribed.  All of that work and then actually feeding Porter through his tube took a lot of time.  Most of the fall and all the way up until transplant we were spending almost 8 hours a day feeding Porter.  THAT is a full time job.

We've made major gains in the eating department over the last week and a half.  We finally realized that the pre-transplant feeding schedule we were using for Porter was unnecessary.  It is safe for him to go without food for more than 6 hours now and we've been dying to eliminate the 10 pm feeding.  So one night we just didn't do it.  Lights out for us at 10:00 instead of 12:00 and it was fabulous!  We've reduced Porter's formula volume throughout the day by about half and are supplementing those calories and nutrients with real food.  By reducing the volume of formula we stimulated his appetite and now Porter is interested in eating.  He's been doing great so far even taking food off of Katie's plate when she's not looking!  The tricky part is helping him figure out how to move the food to the back of his mouth and swallow.  We've found that offering him frequent drinks helps wash things down.  He's still not eating enough food on his own so once he's decided he's had enough we throw the remaining food in the blender and make a puree that goes through the tube. More on Porter's "food smoothies" in a future post!

Fork skills

Scrambled Eggs!

I think I'll just finish this yogurt Katie left on the table.

Teaching Porter how to eat

Helping Porter develop his appetite and skills for eating is a slow process. Our new activity this week is watching other kids eat. We literally walk up to kids we see eating in public so Porter can see real peers eating, but at home YouTube fits the bill! . Luckily, there are countless parents out there who post videos of their kids eating. . Mostly, he just laughs at the videos and doesn't really eat. But, I do think it helps his brain with the concept of food. One YouTube mother said it took a year to get her son weaned off the G tube. If we can do that, I will be thrilled. . #livertransplant #organtransplant #ureacycledisorder #gtube #tubies #tubielife

A post shared by Mr Johnstone (@techiesciguy) on Jul 30, 2017 at 4:09pm PDT

GHP PHD

When most Addison County people find out our son's name is Porter, they immediately ask "Did you name him after the hospital?"

You see, he was born at Porter Medical Center, in Middlebury.

No, we didn't name him after the hospital, thank you very much! Porter wasn't even born IN the hospital (see blog #1: "The Background").

Porter is actually my mother's maiden name. The exact path to choosing this as a first name for our child escapes me at the moment, but it is indeed a family name. Because of his name, when I think about Porter I often think about my Grandma and Grandpa Porter. My grandfather passed away earlier this month, July 7, 2017. It is fitting, then, to reflect now upon my son, his name, and my Grandpa Porter.

 Gilbert H. Porter

I only saw my grandfather a few times before our move back east from California when I was 12, and then sporadically afterward. It's interesting how alike we are! I share my grandfather's passion for do-it-yourself projects, and like him, cherish my meditative time in the workshop. My grandpa's basement workshop in it's prime was a thing of wonder! Saws, sanders, vices of all sizes and uses, and every tool one could ever need for a lifetime. Hanging from the workshop ceiling, several RC aircraft separated into wing and fuselage sections. Grandpa shared his passion for hands on projects and aeronautics with me when I was very young, scheduling in time on his visits to build and fly simple balsa wood models. I too, have grown into an avid RC pilot, but only in the last several years.  Sadly, I never had the opportunity to fly alongside Grandpa.

We share a passion for science, and specifically biology. Grandpa has a PhD and worked in the field of agriculture science and contributed a lot to contemporary knowledge and practices in bovine nutrition. 

I miss my Grandpa a lot but I am thankful he had a chance to meet the newest Porter in the family. I am beyond proud to have my son carry the Porter name. And I look forward, vehemently, to the days when I will share stories of Great Grandpa and I can pass on the joy of model airplanes!

A break from Boston

Since we were discharged from Boston on Thursday, the transplant team told us to take this Monday off, and return to Boston on July 24. But, still in need of some blood labs, we visited our good friends at the Infusion Bay at UVM Medical Center. Porter immediately recognized the children's clinic and ran down the hallway to the Infusion Bay! Katie reunited with her favorite hospital volunteers, and child life specialist Jenny, and got right to work on some new craft pieces while Porter worked with the nurses.

We also stopped by PICU so Porter could show off his new "shark bite" scar. Always nice to be in PICU under happy circumstances!

We hope we get to see more and more of our favorite UVM doctors and nurses as our Boston team slowly becomes comfortable with another team taking on the majority of his analysis and care.

Porter looking for a high five from his old friend at UVM Children's Hospital. Our first visit back to UVM clinic since his transplant in Boston last month. We love our UVM crew and enjoyed visiting everyone today! #livertransplant #organtransplant #ureacycledisorder @uvmmedcenter

A post shared by Mr Johnstone (@techiesciguy) on Jul 17, 2017 at 7:56am PDT

Update July 12

We know our blog followers are looking for a Porter update after our admission for the infection last week Friday. We are working on a couple other posts, and forgot to update.  So here it is!

Porter's infection was identified quickly. Luckily he was started on antibiotics on Friday even before the infection was confirmed. So, his cultures have been clear for two days now. He is now on oral antibiotics and the plan is for discharge tomorrow!

Porter's drain at the graft site was left in over the weekend so the fluid could be cultured, but it is also the suspected source of the infection. So, with bacteria on the decline as of  yesterday, his drain was removed!

Hopefully this time tomorrow we will be arriving at home.

Showing off that sweet transplant scar. Look, no drain!

Hiccup

Author: Sarah

Porter and I got to take a ride in the UVM Health Network's newest critical care transport truck yesterday.  Porter woke up Friday morning very fussy and vomiting.  We have a pretty low threshohold for behavior changes with Porter (for obvious reasons) so after touching base with the folks at BCH and UVM we decided to head to the UVMMC emergency department for evaluation and IV fluids.

The emergency department is not the place you want to hang out with your child but we were warmly received by our UVM team.  Porter and Katie had several visitors from PICU including "Uncle" Nurse Pat who took Katie to her favorite place in the hospital: the gift shop.  They came back with a new pair of purple sunglasses for Porter and a bracelet making kit for her.  A perfect distraction.

Our medical teams decided right away that Porter should be transferred to BCH.  Our friend Nurse Clare hooked me up with ear plugs and an eye mask to help me sleep better while in Boston--THANK YOU!  Doctors Amelia an Iris came down to check on us and helped to coordinate Porter's care.  We are so grateful for our UVMMC family!

Ready for a ride in the big rig with the Boys in Green.

Thanks to the transport team for a quick and smooth ride.  They handed Porter and me off to the Emergency Department staff at BCH around 3:30.  Chris had dropped Katie off with my Mom and was about two hours behind us.  It's a good thing he arrived when he did and with food in hand...my stomach grumbles were about to become embarrassingly loud!

Spilled drain juice on Mom. Lovely ED replacement apparel!

After some more tests the Transplant team is pretty confident that Porter has developed an infection inside his abdominal cavity;  most probably from some extra fluid in there or perhaps from the drain itself.  The plan is IV antibiotics and to get the drain out soon so that it does not remain an infection risk.  The great news is that Porter's liver labs all look good no signs of rejection so far.  Hopefully we caught this infection early enough that we can clear it quickly and be home by the end of the week.

Boston Day Trip July 3 - Checkup

Family hike today at Quechee Gorge Trail on our way home from Boston. Porter's checkup in Boston went very well. His blood work is right on target, liver tests are normal. We will make another round trip next week, and hopefully the docs will advise to extend checkups to every other week.

A post shared by Mr Johnstone (@techiesciguy) on Jul 3, 2017 at 5:32pm PDT

BFF

Touching questionable mushrooms

White Admiral Butterfly

Thank You to ACSD and Middlebury Communities

When we started this transplant journey earlier this year from our first conversations with our UVM medical team to our consultation at Boston Children's, it was clear that a major part of our commitment to providing Porter with an opportunity for the transplant would be the cost. Not the financial cost of the surgery or medications, but the expenses of multiple trips from Vermont to Boston and back, including food and lodging for our family.

We had such a positive experience with the initial transplant and hospital stay, and we owe it to our friends and family who generously donated money, hotel accommodations, meals, laundry, and special care and support for Katie.  To our neighbors who mowed the lawn, brought in the mail, and made sure our cat survived.  Thank you.

We wish to send a very special thank you to our friends and colleagues in the Addison Central School District community and Middlebury area businesses. Last Friday we received an incredibly generous financial donation which encompasses donations from our closest friends and colleagues at work, ACSD members who just know who we are, and even Middlebury community members we have never met before!

We also want to thank our friends at Addison Home Health and Hospice for organizing additional donations to our family.

Porter's post-transplant care will require many more trips to Boston for follow up examinations, lab work, and (hopefully not too many) hospital stays. Your donation will be used to help pay for our travel expenses including lodging, food, and fuel. The overwhelming support from our community allows us to focus on our family and helping Porter stay healthy without worrying over the financial burden of his care.

Words cannot express how fortunate we feel to be part of the ACSD and Middlebury communities. Thank you.

Porter's new liver is working wonders!