Life is Good. Summer 2019.

I intended to write a blog post for our 2nd Transplantaversary; but we've been so busy with really wonderful and normal stuff that I'm just getting to it now.  On June 8th we celebrated the second anniversary of Porter's liver transplant.  I remember writing in a previous post, marking Porter's first birthday, that it felt like so much longer than one year had passed.  The lack of sleep and constant state of stress made one year feel like two or three.  Well, I'm happy to say that the past two years have flown by for us.  Porter is doing better than we had imagined.  He still has regular blood tests to monitor his immunosuppression and the integrity of his liver graft but even those have become less frequent the further out from transplant we get.

Porter is eating well now.  We still use his G-tube for medications and to ensure adequate caloric intake and hydration but it is just a safety net now rather than the life line it was before.  Believe it or not, our biggest struggle with Porter right now is potty training.  Two years ago I never would have imagined that my predominant worry about my medically complex and fragile son would be that he might not be potty trained in time for preschool to start in the fall.  It helps to remember that when I am changing yet another soiled diaper.

After school let out in June we took our first ever family vacation to Cape Cod.  We rented a house with our good friends and had a blast exploring the sand and tide pools.  Katie collected every shell, bone, and crab part she could find and then proceeded to classify and catalog them in what was our arts and crafts drawers.  The arts and crafts materials are now all mixed together in one big bin but the dead sea creatures (whole and partial) are very organized.  We also enjoyed the Cape Cod Rail Trail (Porter is riding a pedal bike...no training wheels!?!) and lots of delicious ice cream.

We had a pretty low key Fourth of July week and then set out on our second adventure: camping in southern Vermont.  Chris and I were campers before kids and we are determined to be campers with kids.  We hiked in about a mile from the trail head to our cabin and had a peaceful, beautiful few days hiking, building fairy houses, climbing trees, "hunting" for bears, eating s'mores, and not worrying that we were three hours from the nearest Level 1 Trauma Center.  It was AWESOME.

In the fall Katie will enter first grade and Porter will begin preschool.  Until then we plan to spend the long summer days together biking, swimming, exploring, reading, and eating lots more ice cream.

Science Classroom Presentation

Porter and I visited the Frederick H. Tuttle Middle School 8th grade science classroom to teach them about genetic disorders. The class had just concluded their unit on the human body and genetics and they were able to answer many questions about DNA, genes, proteins and human physiology. Many of the students are thinking about medical careers and so throughout the presentation I paused and asked them to put their "doctor's hat" on and think about what it would be like to have a newborn with puzzling symptoms and lab results. What would they do next? And, once the students had understood Porter's Urea Cycle Disorder, what could be the course of treatment? What would he need in order to go home healthy? And, stay healthy? The students also asked great questions like Why didn't he show symptoms before birth? And, Can't geneticists simply change his gene?

This is all on the cusp of Rare Disease Day 2018 on February 28. A nice opportunity to focus on genetics.

Teaching @FHTuttleMS about genetic disorders and #ureacycledisorders! #RareDiseaseDay #genes @GlobalGenes @UVMMedCenter @rarediseaseday @RareDiseases @RebeccaBellMD pic.twitter.com/T4VkMO1iAD

— Chris Johnstone (@TechieSciGuy) February 21, 2019

It was also great to share Porter's transplant story and how it has affected his life. Several of the students in each class had friends or family that had received some sort of organ or tissue transplant. We swapped stories about transplant, recovery and the various injuries, conditions or diseases that have been remedied with organ or tissue donations.

The students loved meeting Porter and gave him copious high fives. I had the hand sanitizer ready!

Finally, this opportunity was particularly special for me. The teacher, Lauren Bartlett, was my science teacher when I was in 7th grade. And, as a fellow science teacher myself now, it was surreal and special to share the classroom with her and share in the education of her current students!

Here is my presentation slide deck:

All the forgetting

Dear Porter,

Just a few days ago, we celebrated your third birthday. Many friends and family joined us to have a nice meal, ice cream sundae's, and gifts. Your mom and I thanked everyone for the love and support these people have given you and our family since you were born. We also took the opportunity to recognize the wonderful gift that your organ donor gave you. We all raised our glasses to toast Jamie and the opportunities she has given you and several others to live a more healthy life.

At that moment, an interesting notion came to me. It occurred to me that because you received your transplant at nearly exactly 18 months old, your third birthday marks the time in which you have had your new liver for also exactly 18 months and, half your life. First of all, I can hardly believe it's been 18 months since transplant, and in a juxtaposed way, it's also hard to believe you're only three years old! Most importantly, as the months and years progress forward from now, the fraction of your life in which you endured the extreme symptoms and consequences of your Urea Cycle Disorder will continue to get smaller and smaller. That is a dream come true for us! You will not have any memories of the struggles you and our family faced during these first three years, but as the two people with the most detailed memories of this time of your life, your mother and I are both ecstatic about that dwindling fraction, and also somewhat fearful that we will, over time, begin to forget the time when our family struggled together and grew in ways we never thought possible.

Your mother has a much better memory than I do. So, for me, the forgetting has a significant head start. I noticed a few months ago that I couldn't remember when you received your G-tube. And, just the other day, while looking through the photo album, I realized many more things that I had begun to forget.

I have started to forget the precise schedule for your metabolic formula and Buphenyl medication the nitrogen scavenger that you couldn't go more than four hours without. I do remember the smell and taste of the medication, however!

I have started to forget exactly how and when we decided to transition you off the formula pump. I do still remember holding the open formula syringe, barely able to see it, and slowly, messily pouring the formula into your G-tube only to realized in the minutes after that the tube has become disconnected and I've actually been pouring formula on you, the chair, and the floor the whole time. I guess I remember more than I thought!

I've started to forget the feeling of being aroused suddenly and violently from my sleep by the sound of you crying and vomiting.

I have started to forget the rushed trips to the hospital in Burlington in the middle of the night and the agonizing wait for the blood lab work and the disappointed but not surprised feeling when the result came back, high ammonia.

I have started to forget the faces and names of the emergency department doctors and nurses we came to know so well. I do fondly remember, the warm welcome we received from PICU, every time.

I guess what I'm trying to say is that sometimes forgetting causes mixed emotions. Someday, the detailed memories of managing your metabolic disorder will have nearly completely faded. And, losing memories can be sad. But, we couldn't be more pleased with the upside of all this forgetting. You are healthier today than you have ever been! We are proud of you and we can't wait to see what great things come next. Happy Birthday.

Dad

Best Boston Trip So Far

By Sarah

I dread our trips to Boston for Porter's appointments.  I can't stand riding in the car for that long.  I'm terrible at sleeping anyplace other than my own bed.  And, I get anxious about meeting with the liver team because, for the past year, I've had to defend the fact that Porter is a tall, lean little boy. 

I am not ungrateful.  I give thanks every day for the generosity of Porter's donor and her family, and for the skillful care with which the transplant surgeons changed Porter's life.  I appreciate the attention to detail, and concern of our transplant team.   I respect them and I know that they are among the best in the world.  That is what makes it difficult to openly disagree with them. 

Porter was baby-obese before his transplant.  Our metabolic team had us fatten him up on purpose because the extra reserves made him safer.  Believe it or not there is actually a saying "fat babies with OTC are healthy babies with OTC."  So, he was 98th percentile for weight when he received his new liver.  He was the only kid on the transplant floor who looked like a linebacker!

During the year after Porter's transplant he grew in height but did not gain weight.  He didn't lose weight either, but his growth chart didn't look the way they wanted it to so we had to have more frequent visits and we had to delay what we really wanted to do which was restrict calories to stimulate his appetite and get him eating and off the the G-tube.  Even through the end of this summer (14 months after transplant) we were supplementing all of his meals with pureed foods and formula.  I know it doesn't seem like a big deal, and there are plenty of families who have bigger challenges than us, but we were frustrated because we really felt that if they'd just give us some time we could get him to eat.

After our appointment in August we had three months and the team's blessing to experiment with the timing of feeds and to decrease the amount of formula we were giving on a daily basis.  All we had to do was report his weight to them every two weeks.  They really didn't want to see a dip in his growth chart so we had to be on top of it.  Finally, we had some breathing room!  It took a couple of weeks and Porter's weight did dip a little.  I may not have been completely accurate when reporting his weight that first time.  However, Porter quickly developed an appetite.  For the past couple of weeks we've rarely supplemented his meals with pureed foods.  He has been getting 12 oz of formula (4 oz, 3 times/day) and he even gained a bit! We were so hopeful and confident that we purposefully did not pack our blender for this trip.

Our drive down was beautiful.  The fall foliage was at its peak most of the way through Vermont.  The sun was shining and the sky was a brilliant blue; especially when viewed through my rose colored glasses.  Seriously, get some shades with a little pink in them and you'll enjoy the colors even more. 

In Woodstock we took a quick detour to one of our favorite nature spots Eshqua Bog (featured earlier in our blog) where we hiked around the leaf covered trails and along the board walk.  It was a great place to stretch our legs.  From there we headed to West Lebanon, NH where we found a kid-friendly Italian restaurant and we all enjoyed our first meal out as a family. 

We arrived at the Bergeron home in Melrose, MA just before dinner time and spent the evening playing, visiting, and enjoying the hospitality of family we don't see enough of.  Baby Liam is growing so fast.  We all wish we could see them more.

The night was typical with Katie sleeping no problem, Chris sleeping pretty well, and me not really sleeping at all.  Porter was the anomaly.  He slept hard at first and then was awake around 1:30.  He was sort of restless, moving around a lot and trying to get comfortable.  He also had a bit of a stuffy nose; no doubt the same virus Katie developed the day before.  Finally, around 4:00 am he sat up and said "I'm all done sleeping.  I need some lunch."  (Ha!)

So, I got up and squeezed a fruit and granola pouch into some water and gave him that through his g-tube.  He was asleep 15 minutes later.  Duh.

After a quick goodbye to Lindsay and Liam we made our way to BCH.  Where we did a quick blood draw and then met with our team.  They were impressed with Porter's progress and happy with his labs.  Here is the best news:  They said they'll see us in FIVE MONTHS!!!

We wasted no time hitting the road and stopped in Concord for Common Man fast food (don't judge, he's actually eating!) and were home in time to eat dinner, take baths, and get the little people to bed by 7:00.  How's that for a parenting mic drop!?

Transplating Hope

Last night, the documentary "Transplanting Hope" aired on PBS. Sarah and I stumbled upon it while surfing the channels after Katie and Porter were in bed. The show documented all sides of modern organ transplantation. We were immediately hooked.

PBS - NOVA: Transplanting Hope (film teaser)

We could clearly relate to families in the film, and the patients, each with life-threatening conditions, in need of transplant. The most riveting part for us, was the story of a family faced with the loss of a loved one and the choice to donate organs. The most poignant segment of the film was the series of highly orchestrated, high stakes, medically-magical events that take place when harvesting and transporting the donated organs to their respective recipients.

The night of Porter's transplant, obviously, our mind was on Porter. I do remember briefly thinking about the donor and the situation of the donor family and how Porter's new organ was coming to us following of the death of another person, but those thoughts were fleeting. The entire day of Porter's transplant is the clearest memory I have of all the days of transplant and recovery for him. This film helped me see and understand what the donor family was going through that exact same day.

The video is currently available to view on the PBS website (as of Sept 27), with no further air dates listed. We recommend you watch this hour long film. It may be a little outdated by the time Porter is ready to watch, but we definitely plan to watch it with him.

PBS - NOVA: Transplanting Hope (full episode)

No News is Good News

By Sarah

Here we are, the middle of July, and life is good in the Johnstone household.   With two active kids, and no sleepless nights in the hospital since last fall, we've found it difficult to keep up with our blog.  Porter is doing really well.  He finally had his infusaport removed on June 8th (Transplantaversary!).  In our last post I mentioned Porter's new food allergies and getting him to eat on his own as our biggest challenges.  We haven't made much progress with eating yet but the good news is he is healthy and growing.

Trying to catch bumble bees.

We recently had an allergy appointment at BCH where Porter's skin test for milk allergy was negative.  We are hopeful that he has overcome his milk allergy but won't know for sure until we can get him back to the BCH allergist for a milk challenge.  (It's not what you think!!)  They will give Porter some milk, NOT a whole gallon, and wait to see if he has a reaction.  If he does they will treat it; if not we'll get the green light to stop for ice cream on our way home.  😊

Unfortunately, we also learned at that appointment that Porter is, and I quote, "definitely allergic to cats."  We've struggled with Porter's eczema since the very beginning.  The doctor said that cat dander is probably a major factor in Porter's eczema symptoms and that a positive skin test often precedes more serious symptoms. Porter has a higher risk of developing asthma if we keep our kitty.  😞  So, we are searching for a loving home for our 12 year old cat.  In the mean time our vacuum cleaner is earning its keep.

In just a couple of weeks we will meet with Porter's metabolic doctor from UVMMC.  It's so strange to think that we haven't seen her in 9 months!  Prior to transplant we communicated with Dr. Burke several times each week.  Her number was at the top of the list in my phone favorites. I finally took her out after Porter accidentally Face Timed her by himself in October (oops!)  Now we will see her annually to check in and monitor Porter's citrulline needs. 

Porter will have to go back to BCH soon (hopefully before school starts up again) to have a liver biopsy.  The transplant team likes to get biopsies at 1, 3, 5, and 10 years post transplant to see how the liver is holding up on a cellular level.  They told us that even when kids have really good liver function tests they have occasionally found scarring in the grafts.  Scarring can lead to cirrhosis so they want to stay ahead of it.  We'll go down to Boston for a Monday morning clinic appointment and ultrasound and then have the biopsy done on Tuesday.  The procedure is pretty straight forward but Porter will be under general anesthesia.

We are thankful to Lindsay, Steve (and Liam!) for continuing to host us on our visits to Boston.  We're so lucky to have family there who are willing to put up with the tornado of us coming and going.

Hanging out with cousin Liam.

Katie learned to ride a bike this summer.  From balance bike to pedal bike (she don't need no stinkin' training wheels!) in record time.  He didn't say so, but I think Chris was a little disappointed that she didn't need him to run along beside her.  So independent.  She'll start swim lessons next week but is already pretty good at swimming underwater. 

Can you say "college scholarship?"

Crusin'

Riding his "motorcycle."

Found a pretty garden on the BU campus.

Porter loves machines.

BFF's

April 2018

Author:  Sarah

I can't believe it's been 4 months since our last post.  This winter was busy for us in a "normal family" kind of way.  Mostly, we stayed in our bubble at home which allowed us all to stay healthy (yay!)  We played in the snow a lot and both kids got to try skiing.   Katie hit the magic carpet at the Middlebury Snow Bowl a few times (she loved it) and Porter kicked around in our yard.

  

Yesterday was our most recent blood draw visit and there's some exciting news to report on that front. Porter has had a central line since he was four months old.  He had a Broviac catheter for 10 months and that was replaced by an infusaport which he has had now for 14 months.  During our pre-transplant evaluation the team in Boston told us that they generally remove central lines by 6 months post-transplant because they increase the risk of infection. The team decided to delay removing Porter's infusaport until his ANC stabilized because of the need for frequent blood draws.  We knew that we needed to ensure that Porter would tolerate peripheral blood draws before the port could come out.  So, our UVMMC nurses and child life specialists made it happen.  Porter is a peripheral blood draw rock star now and his infusaport is scheduled to be removed in mid-May.

The transplant team has been telling us for months that they only need labs once each month (more frequently if they make changes to his medications).  But, up until the middle of March the hematology folks had been requesting labs every week.  Finally, this past stretch between lab tests was 3 weeks--the longest Porter has ever gone between blood draws.  Unfortunately, Porter's liver function tests were up (likely because he has a cold right now) so we have to go in again next Monday.  Hopefully, next week's labs will look good and we'll be able to hold off for another 3 week stretch until liver clinic in May.

Nutrition is our biggest challenge now with Porter.  He still does not have much interest in eating so nearly all of his food goes through his G-tube.  We have had some success in stimulating his appetite by slightly reducing his calories via G-tube but even with the increase in appetite, and therefore eating, he tends to lose weight.  The doctors don't want to see any dips in his growth chart so we have to go back to total tube nutrition.  It's hard not to get frustrated.

We've learned recently that pediatric liver transplant recipients who take Tacrolimus as their immunosupressant commonly develop new food allergies.  It is possible that the allergy develops because the donor was allergic (they don't collect that information so we don't know if Porter's donor had food allergies).  More likely, the new food allergies develop because of the immune properties of the new liver and the way that tacrolimus effects the immune system.  Guess how we found out?  Porter suddenly developed allergies to milk and egg.  Yup.  All those times when I wished that Porter would just have a food allergy instead of a UCD came back to bite me.  Hard.  So, he was already allergic to peanuts and tree nuts.  Now milk and egg are out.  We keep our benadryl and epi-pens close now.  Who knows when another allergy might develop?  Splendid.

Even with Porter's food allergies life is still better for us post transplant.  We sleep better.  He's developing normally.  We can breathe.  I won't complain (not out loud, anyway).  We've got it pretty good. 

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.