Author: geekino

At the beginning of the year, Brian was called as a counselor in the Stake Young Men’s presidency. If you’re not familiar with this aspect of the Mormon church, this means that he was asked to volunteer as one of three leaders over the program for boys age 12-18 in 7 congregations in our area. The program combines a very spiritual aspect with fun activities and the Boys Scout program to hopefully help these young men develop a good solid foundation for the rest of their lives.

When he first accepted this assignment, we were told that they were planning a pioneer trek for their summer Youth Conference.

Basically, along with the Young Women’s presidency, they’d be planning to take 130 teenagers and adults to the middle of Wyoming where they’d dress in pioneer clothes and spend three days pulling handcarts through the hot, barren prarie and visit some historical sites. The hope was that they’d gain a better appreciation for the sacrifices made to give them the life they have today and a stronger relationship with each other and with the Lord.

To understand why one might think this kind of venture a good idea, you probably need a little bit of Mormon history. See, when the church was new and small, but quickly growing, early members were persecuted and literally forced from their homes over and over again until finally they decided to head West. They travelled by wagon train to Utah and started a new life there.  There, they hoped to find a place where they’d be free to worship together in peace.

Meanwhile, though, converts were joining the church at a rapid rate in Europe and were eager to join with the other “Saints” in America. However, many couldn’t afford the journey. And so, church leaders started to organize handcart companies. Converts travelled by ship and then by railroad as far as they could, and then they pulled handcarts loaded with their possessions from Nebraska to Utah.

The trip was challenging, but most made it safely through. However, two companies were late in arriving and ended up trapped in blizzards in Wyoming without enough rations. All suffered and many died.

This year’s trek visited the sites where these companies camped and were eventually rescued.

Starting to see the lesson they were hoping to teach our teenagers about?

Like the handcart companies, our youth leaders seemed to be a bit late in beginning the planning for their trek. To make up for it, in the months leading up to it, Brian’s had many a long meeting as they worked to prepare. He was most all afternoon every Sunday and most Wednesday evenings, too.

Of course, with Patrick to care for, it wasn’t an option for me to go on trek (though many couples went). But I wanted to be a part of the experience. So, I dove in learning the history by watching movies and reading pioneer stories. I tried to tag along to Rawlins, WY so that Brian could go to a training. I even found us a nice bed and breakfast to stay in. But Patrick got sick and we spent that week in the hospital instead.

And so, since I couldn’t go anywhere near trek physically, I took the second best thing and joined in the preparation work. Brian came home one Sunday and asked if I’d help make a logo for their bandanas. And so, I spent a Sunday designing sample logos for them to choose from.

And then, he offered to help put together a booklet of pioneer stories, schedule, and songs. That project was a beast! I can’t tell you how many nights I stayed up past midnight (since I couldn’t do it while Patrick was awake) laying out pages, typing pioneer stories, editing songs, and even assembling the thing. 144 copies of a 40 page booklet is a significant feat!

Still, it let me be spiritually a part of something that I couldn’t attend physically.

It was kind of hard for me to not be able to go. When we went to buy Brian a new sleeping bag for the trip, I cried for the entire day. As much as I love life with my little boy, meeting his needs often means staying behind and sometimes it gets very lonely. And it often means sacrificing things I love.

At long last, the day came for them to leave. We got up early on a Thursday morning.. before the sun… to drive Brian to the church. Patrick was confused about what we were doing. But he was excited about the charter busses. I had to drive back past them 4 times so he could see them before we left. Then we went out for breakfast, just because.

I’m glad I had the Garden Tour to distract me. Still, my mind kept going back to my husband up in Wyoming and the pioneer stories I’d been reading for months. And thankfully, three days went by quickly and before we knew it, we picked up a very dirty daddy and said a fond farewell to the charter busses one last time.

Then, we made a mad scramble on one final project. We collected pictures from the people who’d been at trek and assembled a slide show to be shown in a meeting on Sunday night. This was really a great way for me to hear all about what Brian had done all week and what he’d learned.

But, since I’m not really in a place to tell those stories, I’ll tell what I learned instead.

As radically different as our lives are, I have learned to feel a kind of kinship with the pioneer women.

In one of the stories I typed, I read:

“When little Daniel (age 18 months) died, Aug 12th, I rode the cart holding his wee body until we camped and he could be buried. Soon afterwards, I birthed a baby boy; alas, he lasted but a few short days.”

I haven’t lost a child. But since Patrick entered my life, I’ve met many mothers who have. And I have felt my little boy stop breathing in my arms. I understand now in a way I never could before just what it might have been like to hold your baby boy in your arms, knowing it would be the last time. My heart aches for those women who lost children and husbands on the trail. And I am so much the more grateful that because they carried on, I am here today.

Which brings me to another thought and another quote.

“The Latter-day Saints need to remember that those of us who live now are being called upon to work out our salvation in a special time of intense and immense challenges. The last portion of the dispensation of the fullness of times is a period during which great tribulation and temptation will occur. . . Therefore, though we have rightly applauded our ancestors for their spiritual achievements and we don’t and must not discount them now; those of us who prevail today will have done no small thing. The special spirits who have been reserved to live in this the dispensation of the fullness of times will one day be praised for their stamina by those who pulled the handcarts.” – Elder Neal A. Maxwell

This quote bothered me when I first read it. I think I’ve been a bit innundated by talk about the “last days” and the “chosen generation”.

But, as I’ve thought about the handcart pioneers and their trials while living on in my own trials, I have realized that we really do face a unique and difficult set of trials.

Pioneers had no NICU’s. Babies who were premature or had a birth defect like gastroschisis didn’t survive. Heck, up till 20 years ago, Short Gut patients didn’t survive. Transplant wasn’t even a consideration.

And while I would never, EVER want to experience the agony of frostbite and starvation and exhaustion and crossing frozen streams and wondering if help would ever come… and I can’t even comprehend burying a child in the snow along the trail because the ground was too frozen… I can see now how a pioneer woman might see our family’s battle against prolonged illness and desperate struggle for survival as it’s own rugged trail.

One tradition of the pioneer trek is the “Women’s Pull”. In the closing meeting of the conference, they invited the youth to stand and share their thoughts. At least 80% of them mentioned this as the most memorable moment.

As they approach one of the steepest hill of the trail, they take the men away from the company. They talked to the boys about how many women made this journey alone, either entirely, or after losing a husband. They talked to them about respecting and appreciated women. They told the girls about how there may be times when they find themselves facing challenges alone. And then, the girls pulled the carts up the hills while the boys watch. There is rarely a dry eye when it’s done.

Ironically, while person after person told this story, my mom brought Patrick to me at the church and I took him in the halls… alone.. to entertain him till the meeting ended.

And I couldn’t help but think about my few days without my husband and how much harder things are when he’s away.. and the many times I’ve faced crises with Patrick when no one else could be there to help. And how lonely and sorry for myself I’d felt. And I realized that while I am facing my own set of challenges, I am fortunate that I am rarely asked to face them alone for more than a few hours or maybe a few days.

And, like the pioneer women, I am never really left alone. Because my Heavenly Father is there.

One last quote and then I’ll end my philosophical history lesson. From a favorite pioneer hymn:

“Why should we mourn or think our lot is hard?

‘Tis not so; All is right.

Why should we think to earn a great reward if we now shun the fight?

Gird up your loins; fresh courage take. Our God will never us forsake;

And soon we’ll have this tale to tell-

All is well! All is well!”

I think I’ll end with a few pictures of my own Pioneer.

 

 

 

Wow! What an eventful couple of weeks! It’s going to take me forever to capture it all in writing. I think I’ll start by telling you about our day at the Mountain Valley Garden Tour.

I already mentioned how excited I was after the reception we attended for garden owners and volunteers. I am still in awe at the kindness of these people whom we had never before met! They opened their hearts and their homes to us and I will be forever grateful.

The tour ran Friday and Saturday. Since Brian was away on a pioneer trek (I’ll tell you about that in another post), I decided I could probably only handle attending one day with Patrick.

So that I wouldn’t be doing it alone, my mom and my good friend Tifanie came along. We left here bright and early and arrived at the first garden moments before it was scheduled to open. We were thrilled to find that it belonged to the one garden owner that we hadn’t been able to meet at the reception.

The tour organizers gave Patrick a gift basket of toys and music and other things to entertain him on that day’s drive. In that basket was a little garden shovel and rake. And the rake was his favorite of all the gifts. After greeting our hosts, he set to work in the garden right away. However, instead of raking the dirt, he busily combed the flowers with his rake. And he did it everywhere we went.

 

 

It was fun to meet and visit with the people who were there. They all loved meeting Patrick and he charmed them as only he knows how.

You could always spot one of the visitors because they were decked out in their adorable gardening clothes.

At 11:00 they had planned a meet and greet at one of the gardens. Loretta and Shan, the owners of that garden, were very kind and enthusiastic to have us there. They let Patrick feed their fish. (They have a trout pond almost as big as my entire front yard! Patrick loved feeding them. At least, until one jumped and touched his foot. Then he wouldn’t sit down by them anymore.) They let him pet their dogs. Shan even let Patrick rake his hair. And before sending us on our way, Shan who is an entertainer by profession, made Patrick the most amazing spiderman balloon.

 

 

 

By the time we left there, Patrick was pretty exhausted. We were going to head home, even though we’d only been to a few gardens. However, we noticed that one garden’s description mentioned it had antique cars and, well, that sounded like Patrick’s dream garden.

So, we grabbed some lunch and started the 30 minute drive from Park City to Heber to see the cars.

Patrick fell asleep immediately.

And when we got to Heber, he was still asleep.

So, Tifanie and I decided that, since we’d come all that way, we’d at least go in and see the garden and thank the owners and volunteers. The kind man who owned the garden and the amazing garage full of antique cars offered to let me bring Patrick back another time and we were on our way back out.

And that’s when Patrick woke up.

So, since we had come all that way, we got him out and let him look. He was in heaven! The garage had 6 antique cars in it and Patrick just ran from car to car touching the wheels and making faces in the bumpers and trying the door handles. I’m sure that they had to polish all of the cars again after we left. But they were so kind to let him explore!

It was a perfect day and all of the gardens we saw were so beautiful!

We made one last stop to thank the volunteers running the silent auction at the grocery store in town. And then we drove back home.

We still haven’t heard final results from the fundraiser, but we know that there were 83 attendees on just Friday.

This event came along at just the right time. It’s been a long time since our little fundraising account has seen any activity and COTA was beginning to ask me if we were done with fundraising. While I know that many of us are tired and feel we’ve done the biggest and best we can, our thermometershows we still have $50,000 to go. And in a month where we had unplanned home repairs and travel for checkups in Seattle and hospital stay expenses and tax woes, it was nice to see that people really do still care and want to help. It left me wondering if maybe it’s time for another restaurant day or facebook or e-mail challenge again. What do you think?

I can’t express enough thanks to the people who put this garden tour together. A few months ago, they had never even heard of our little family. And yet, they just put their arms around us. If any of you involved are reading this, thank you for your love and support. Thank you for helping us in our journey. And thank you for a much needed dose of hope.

What the tour organizers didn’t know was that they happened to schedule this event on Patrick’s second Miracle Day. July 15 is the 2 year anniversary of the day that Patrick’s heart stopped and then was started again. I couldn’t have planned a better celebration.

 

It seems that every time I turn around these days there is another drug shortage that threatens Patrick’s health. Today, I spent over an hour on the phone trying to figure out the best oral form of L-carnitine to give Patrick, who is showing carnitine deficiencies but can’t receive them in his TPN because of shortages. Sadder still is the fact that I did all that work for a nutrient that he most likely can’t absorb in oral form anyway.

This was the final push I needed. I’ve been working on a draft of a letter to my state senator inviting him to help sponsor legislation that would require drug companies to actually tell the FDA when they expect that they won’t be able to produce enough of a drug so that they can try to encourage others to boost production.

I will mail this letter to the Senate today:

Dear Senator Lee,

My name is Emily Hoopes and I am mother to Patrick, a 2 year old who is waiting for an intestinal transplant. Patrick was born with a rare birth defect that led to the loss of his entire small intestine, a condition commonly known as Short Bowel Syndrome. Without a small intestine, Patrick cannot absorb nutrients from the food he eats. He relies entirely on intravenous nutrition, supplements, and drugs in order to grow and stay healthy while he waits for transplant. For the past two years, Patrick has beaten the odds time and again, defying doctor’s expectations. He is a happy, healthy, and active toddler, as long as he has this intravenous help to maintain nutrition and hydration.

That is why I am writing. Recently, an alarming number of drug shortages have occurred across the country, many of which have had a direct impact in Patrick’s quality of care. I am writing to ask your help in addressing the patient harm that is caused by drug shortages. Specifically, I am asking you to cosponsor S. 296, a bill that would help address the issues leading to drug shortages.

We are facing a drug shortage crisis in this country. Currently the American Society of Health-System Pharmacists (ASHP) DrugShortageResourceCenter lists 150 drugs in short supply. These include life-saving drugs, drugs to relieve pain, drugs to treat serious infections, medications used to provide parenteral nutrition (intravenous nutrition like my son uses), chemotherapy medications, and more.

Where these drugs are unavailable, doctors have been forced to use alternatives that have safety concerns, higher risk of error, and increased healthcare costs. When an alternative is unavailable, patients have no choice but to go without and hope for the best.

Since the beginning of the year, my little boy has already been directly affected by these shortages several times. In just the past few months, he has gone without several essential drugs, including vitamins, calcium, carnitine, amino acids, erythromycin (an antibiotic and motility medication), and ethanol (used to prevent infection.) There are also national shortages of other nutrients such as trace elements, vitamins, and electrolytes. Because Patrick cannot absorb nutrients by oral intake, when intravenous forms aren’t available, he simply goes without. For him, this means malnutrition and risk of serious complications. If this weren’t enough cause for concern, shortages of antibiotics and ethanol put Patrick at greater risk for a life-threatening systemic infection. Put more simply, a shortage of even one of these drugs can mean the difference between life and death for my son. With them, he has a good chance of surviving until he can receive a transplant. Without them, his prognosis is poor.

My son is not the only one whom these drug shortages have hurt. I am the founder of the Short Gut Syndrome Families’ Support Group, a national support group for children and families affected by Short Bowel Syndrome. Several of our members have felt the impact of these shortages and their children have been put at risk as a result. Just within our local chapter and hospital, rates of infection have increased, as have the number of nutritional deficiencies. As parents, we are scared for our children.

S. 296 would help improve communication between the U.S. Food and Drug Administration (FDA) and drug manufacturers. The legislation enables the FDA to work with drug manufacturers to establish contingency plans for manufacturing interruptions such as raw material shortages, adjustments to production capabilities, and product discontinuations.

Although I typically am of the opinion that less government regulation yields better results, the growing number of drug shortages is evidence that more needs to be done. S. 296 gives the FDA the resources needed to improve this problem.

Thank you for reading my story. And thank you in advance for your consideration to cosponsor S.296.  Please do not hesitate to contact me if you have any questions or would like more information. I look forward to working with you as we work to resolve this import public health and patient safety issue.

Sincerely,

Emily Hoopes

If you’d like to know more about the drug crisis, just google “drug shortages”.

In case you haven’t noticed, when my life gets hectic and I’m feeling overwhelmed, I stop blogging. Either I don’t have time, or I’m afraid that my emotions will get the better of me and I’ll post something I shouldn’t have.

Well, I’ll confess that my long hiatus between blog posts was one of those cases where I wasn’t writing because I felt like I was barely treading water. Between medical choices and a hospital stay and a holiday and youth conference and remodeling and mold and taxes… I just didn’t want to talk about it.

Brian and I just kept saying one one bad night that had us up past 1 after barely sleeping the previous two nights that surely this was all a sign of good things to come.

Well, tonight I found the good things. I am so filled with joy that my heart is brimming!

Tonight, Patrick and I attended a garden reception with the planning committee and garden owners responsible for the Mountain Valley Garden Tour that will be happening tomorrow and Saturday. I am so completely overwhelmed by the absolute love and kindness shown to us by people who are complete strangers to us!

All week long I’ve been listening to radio spots and reading news articles and watching TV interviews about the Garden Tour and every time I heard about it, it started sounding better and better.

If you’ve missed my shameless advertising, they’ve planned an event similar to the Parade of Homes… only it’s the chance to tour private gardens throughout Park City, Midway and Heber. That’s what I found out before. But through the advertising, I’ve learned that there will also be gardening workshops. And, there will also be live bands. And there will be a silent auction. And there Jazz Bear will be there. And.. well… it’s going to be simply amazing! Two days of garden bliss!

At least, that’s what I got a taste of tonight. First of all, I was worried about driving all the way to Heber – an hour through the canyon – that late alone with Patrick. But I got on the road and realized that it wasn’t much longer than I drive when we are in rush hour.. Only it wasn’t rush hour. It was beautiful mountain scenery! It was fresh air! There were cows!

We arrived just a little early, so I decided we could stop at Dairy Keen – a local hot spot burger joint with a toy train that goes around and around the ceiling. I stopped so Patrick could see the train. It was worth it.. he was in heaven.. But while I went to pick up my burger, he dumped his cup of water in his lap.. And by the time I got him changed into dry clothes – instead of being a fashionable 10 minutes late, we were half an hour late.

Thankfully, they understood.

And Patrick more than made up for it. He was a perfect angel tonight! Social as can be. He went and personally greeted every single person there. He even played with one girl’s celery for a good 10 minutes straight.

Ronald McDonald made a guest appearance tonight. We weren’t sure how this would go over.. But once I pointed out his big red shoes, and then Ronald let him stand on them to reach his big red zipper.. Patrick was sold! They were friends from there on out.

Thank goodness someone else there had a camera, though, because Patrick was so quick that taking any pictures of my own was impossible.

Those will have to come later.

Anyway, we took pictures with Ronald (a.k.a. “Ro-Ro”) and all of the guests and then Patrick wandered and looked at the gardens and mingled some more. I even got a second to sit down and eat a very yummy, though messy, eclair.

The event organizers had a gift bag for Patrick all full of fun things like CD’s and stickers and toys and his own gardening shovel and spade (which he will LOVE! because they’re real metal). They said it was to keep him entertained in the car for all the extra travel he’d do this weekend.

And then we said our goodbyes. Patrick would have stayed another hour. But I knew he’d be too tired tomorrow. So I took him to the car and put on his pajamas. We drove home listening to soft music. He fell asleep and I got a peaceful one hour drive down the canyon as the sun set over the mountains and lake.

I can’t tell that these words aren’t really capturing it. But trust me.. this evening was amazing! A true gift!

I don’t think I’ll ever get used to this kind of attention. I never feel like I know the right things to say and do. Thank goodness Patrick is so good at it that it almost makes up for me.

The thing is, though, that there are no words or actions that can really capture what it is to have people just reach out and help you and love you like this! There are no words to capture the absolute treasure it is to have been given a summer evening with my son.. of giving my son the chance to just be a little boy… even if it was all done because he’s not your average little boy.

If this evening is a foreshadowing of the next two days to come, then this weekend is going to be wonderful. I wish more of the people I love could share it!

I guess I could throw in one more plug. It isn’t too late to buy tickets. They’ll be for sale for $15 tomorrow and Saturday at any of the gardens. Just go here http://www.cotaforpatrickh.com/node/315 and follow the link to their website and there’s a list of locations.

Patrick and I will be back there Saturday morning at 11 a.m. at the Paulus garden in Park City. With trout. And the Jazz bear. Seriously, how can you resist that?

It had really been too long, don’t you think, since we holidayed at the hotel on the hill?

Last Sunday, Patrick wasn’t quite himself. He was AWFUL in church! And then I took him to his nursery class only to be pulled out half an hour later by the concerning news that he was vomiting.

And oh boy was he! He was covered! So I wisked him home, changed his clothes, drained his belly, and put him to bed. He slept 3 hours. Straight.

At 4, we got him up to go to Sunday dinner with my family. He went to play with his cousins, but then came back quickly and asked for Brian to hold him. And just layed down in his lap.

NOT normal.

Then he got shivery and started to get goosebumps. We knew we had to go. These were the early signs of rigors again and it was almost certain that he’s have a fever of 103 or above within an hour.

We said quick goodbyes, hopped in the car and drove straight to the hospital. Halfway there, his fever shot through the roof. He was trembling and every few minutes would shake so badly that it was almost a convulsion. He was terrified. So was I.

We called ahead and told the GI on call that we were coming. Thank goodness it was Dr. Pohl, a GI whom we trust and who has taken care of Patrick at his worst. He knew we needed to be careful.

So, he had us go straight to the ER. They took his vitals and he was admitted immediately under the septic shock protocol – level red. His temp was over 104, his heart was racing and his breathing was shallow.

You know you’re level red when the doctor comes into the room.. immediately. Tailed by a nurse practitioner. They did a quick evaluation and ordered several STAT labs, including blood cultures. The nurses were running. Patrick was miserable. No one had given him anything for the fever in the rush to get him treated. However, fluids were running and they started antibiotics next.

With extra fluids, his heart rate started to come down a bit and his blood pressure looked better. We asked permission to start his TPN. (He’d been on his break) and got it. They finally got some tylenol and started to feel better. We watched Elmo in Grouchland 3 times.

However, the first antibiotic they gave him caused problems. For some reason, the pump gave it at twice the prescribed rate.

And then his neck started to swell right where the central line catheter entered his jugular vein.

So they stopped the TPN and the next antibiotic and we got to take a trip to X-ray to make sure that the line was still viable.

The good news is that it was. The bad news was that Patrick was developing hives. Probably a reaction to the med going in too fast.

So they restarted the antibiotic, but didn’t let us restart the TPN.

Mistake. The next drug was vancomycin. With the histimine already in his system, Patrick had his first every “red man’s syndrome” reaction. He turned beet red and started to itch.. everywhere.

Finally, we got some benadryl and all the redness and swelling and hives went away.

And then they admitted us to a room.

Because we’d run to the hospital, we only had a diaper bag with us. None of the usual supplies. So we had to jury rig a drainage system for Patrick from the parts the hospital could find. It worked, but it distracted the nurse and it was past midnight before she got the maintenance fluids (like TPN) running. Scary, but Patrick did ok. With tylenol, benadryl, and antibiotics on board, he was back to himself.

They watched him closely through the night and then for most of the next day they asked us to keep him on monitors.

This was a bit tricky because he doesn’t like to hold still enough for them to stay attached, let alone read. But we did our best to entertain him on the bed.

We made the choice this stay to let him have a big boy bed for the very first time. This was nice in that when he fussed during the night I could just crawl into bed next to him and cuddle him back to sleep.

It was challenging, though, in that I snore. And our second night in the hospital, he let me know it. Things started out ok. After watching 4th of July fireworks and the life flight helicopter from his hospital room window, he went to sleep fairly easily.

However, a code on the floor woke him at midnight. He jumped up and his g-tube got caught on the bed rail and got tugged. It stayed in place… but was very sore and we had an adventure trying to check to make sure it was still ok.

He didn’t sleep again till 6 a.m. He’d almost sleep, but I was so tired I’d fall asleep trying to help him sleep and the next thing I knew a little hand would be patting my face and Patrick would imitate my snoring. I was keeping him up.

Thank goodness for kind nurses who take toddlers for walks at 5 a.m. to their mommies can sleep. And for other kind nurses who will tiptoe around all through the day shift while little boys make up for staying awake all night.

We stayed 72 hours in the hospital, waiting for the blood cultures to grow to explain the fever. They never did. Either it was viral (which seems unlikely, given the quick recovery)… or the bacteria never got into his line.

Meanwhile, as the above pictures demonstrate, we learned how to keep a 2 year old entertained in the hospital.

This is good news, though it does make me nervous as we only gave 3 days of antibiotics and are now home without any further treatment.

So far, so good.

Just a little reminder to me that I’m not exaggerating when I call Patrick medically fragile. Things can go from OK to life-threatening at the drop of a hat.

Hoping we don’t have another scare like that for a long, long time!

After a nice little day vacationing in Seattle, the next day was a little more business.

Well, eventually it came to be business. We started out with more fun. I met another mom through our Facebook support group. Her youngest daughter also has Short Gut and is a patient of the intestinal rehab/transplant clinic at Seattle Children’s. Her older daughter is Patrick’s age. So, since she needed to come in for an appointment with homecare that same day, we decided to bring families along and meet.

We went to Pike’s market together, since it wasn’t too far from where they’d ferry in. Ok, so the environment was maybe a bit hectic for two toddlers and a baby, but as grownups, we really enjoyed the chance to just BE! It’s always so comforting to be with another family who is used to IV lines and g-tubes. It’s nice to eat with someone who doesn’t even bat an eye at the fact that your child prefers playing with a straw and sipping bottled water to eating the treats that everyone else has.

It was just.. nice. And far too short. I’ll have to plan better next time we’re out there so the kids can play a bit more.

After sharing some piroshkies, we all headed back to our cars and drove to the hospital.

Our appointment this time was with Dr. Dick, a transplant surgeon whom we had never met before. We were supposed to see Dr. Horslen, but he had a patient who needed an emergency appoinment and we decided to trade our appointment since our questions were really more in the surgical field, anyway.

However, as seems to often happen with the surgeons, Dr. Dick was called away just as we arrived and so we waited for him. And waited, and waited.

Waiting in a small exam room with a toddler is hard work!

Finally, the usual steady stream of professionals started to come in. We finally got to meet Patrick’s new transplant coordinator in person. (We talk on the phone all the time, but hadn’t yet met.)

His dietician, as always, was thrilled with his progress! Few children with Short Bowel Syndrome, especially children who rely entirely on TPN for nutrition, are in the 50th percentile for weight. Because I’ve been worried about the delicate balance of liver health and lipids (IV fats), I asked her to review his fatty acid profile. I’ve been worried that by only giving lipids 2 days a week that Patrick would start to develop deficiencies that might affect his brain development. However, after looking at his lab results, she told us that his profile looked like that of a patient receiving Omegaven. This is kind of the gold-level standard for treating TPN-associated liver disease.. and so it’s very good news indeed.

Finally, Dr. Dick had reviewed the radiology we brought (we brought copies of the images I’ve shared on this blog previously) and came into the room to talk to us about it.

We kind of introduced Patrick and he looked and his current health and overall progress and was quite pleased.

And then we asked the million dollar question – the one we’ve been asking every doctor for the past year. Is it still ok for Patrick’s intestine to be horribly distended and for us to be draining a liter or even two of bile and replacing it for him every day.

Dr. Dick looked a bit like a deer in the headlights when we asked this question.

We discussed things a bit. We told him that we’re pleased with how stable Patrick is and don’t want to rock the boat, but that we also don’t want to compromise his remaining intestine being used for transplant.

Then he decided to go get a second opinion. He went to find Dr. Javid, a general surgeon who works on a lot of the intestinal cases. And we waited, and waited, and worried the longer they were gone. Dr. Javid is the surgeon who placed a line in Seattle a few Christmases ago when Patrick aspirated during surgery because of stasis. He is familiar with his motility problems.

Finally they came back together.

They explained that they had reviewed the films, along with Dr. Horslen, and had some recommendations.

The mentioned a procedure called a plication. Basically, it would mean putting a row of staples down the center of Patrick’s duodenum… narrowing it to a more normal size and allowing the other half to shrink away.

In theory, it sounds like a solution that might work, but it comes with a LOT of risks and we are nervous about the idea. Honestly, I don’t know what route to take right now.

On the one hand, reducing the size of that organ would probably slow down the hypersecretion and help Patrick to not need liters and liters of IV fluid to stay hydrated. It certainly would be nice to not have to worry about whether or not drainage tubes are working so he doesn’t wake up vomiting in the middle of the night.

However, the duodenum is what is distended. And the duodenum is not a part of the small bowel transplant package. It’s it’s own organ. It does it’s own things. And, while it absorbs very little, it plays a major role in digestion and absorption that can’t be replaced if it was gone.

We asked the transplants surgeon if they were comfortable with the risk of us making changes to this crucial organ. He replied that it may already be too unhealthy to be used for transplant. YOUCH! That is a really tough pill to swallow.. especially as things got to this point precisely because we were trying to preserve the duodenum.

And then, of course, there are the risks that come with major abdominal surgery. Simply getting through the scar tissue in his abdomen would take at least an hours. And there are the risks to follow the procedure: staples in the intestine leaking and causing ulcers or infection, loss of bowel motility or absorption (that fish oil is being absorbed SOMEWHERE), trauma to or loss of any portion of the remaining intestine.

All in all, it’s a pretty risky surgery that may or may not fix the problem long term. We could do this and find that the distension and lack of motility are caused by something completely different and end up right back here in another year.

Not an easy choice to make for a boy who 95% of the time seems completely happy and healthy despite the fact that his is critically ill.

I’m going to jump ahead a bit to after our return home when we discussed these options with Dr. Jackson, Patrick’s main GI. He suggested trying a STEP procedure instead so that at least we wouldn’t lose any bowel surface area. Not sure if that would decrease secretions, but it might help with the stasis and the motility… which would both help reduce bacterial overgrowth and translocation problems. He also said, though, “But I don’t want to rock that boat”.. meaning he doesn’t really want to mess with Patrick’s miraculous and unlikely good health.

All of the doctors agreed that they needed a conference. Seattle’s GI and white-haired transplant and general surgeons.. as well as Patrick’s GI and surgeon here at home. They need to talk about all the options and the possible outcomes and the risks and come to a concensus on their recommendation.

And then we’ll have to choose what to do next.

We also discussed going ahead with an endoscopy after all.. just in case the barium enema lied. (Did I tell you we did a barium enema in preparation for the bowel dilation procedure only to find no evidence of a narrowing that they could dilate.. and so we dropped the procedure?) And maybe doing some more specific tests to try to treat and manage bacterial overgrowth.

But for now, we’re just letting things be and waiting for the doctors to talk.

We left the clinic 3 hours after we arrived. Patrick was exhausted and fell asleep before we’d driven a mile.

Thankfully, we had a good evening distraction. We drove up to Everett to go to dinner with Lindy and Kelly, our wonderful friends who so often take us in. Lindy was due to have her baby well, any minute it seemed.. and so we kept the visit brief so she could rest. Just dinner and a trip to the train station.

 

That night, Patrick decided that he was done sleeping in the hotel room crib. He cried every time I tried to put him down and it was well past midnight before any of us slept. The next morning, he was furious to find I’d deceived him and refused any and all offers of naptime.

So, after wasting half the day in that effort, we packed and moved out of our suite. Instead we drove and drove trying to get a nap, then finally gave up when it was time to meet one of Brian’s business colleagues for lunch.

They gave Patrick a cup of water and a straw that he could blow bubbles in it with, so he didn’t mind being awake.

Our friend recommended a stop at Alki beach on our way out of town. Patrick loves walking in the surf, so we decided that since he wasn’t sleeping, that would be a good place to kill the rest of the afternoon. Of course, that’s when he napped.

So, we just drove past Alki beach and drove around West Seattle until time to prepare TPN and head to the airport.

It was a strange trip – a wonderful vacation, though very sleep deprived, and with many more questions raised by the transplant team than solutions given.

But it gave me a goal. One of these days, I’d love to go to Seattle and rent a beach house on Alki beach and just take a vacation. No clinic visits. Just the Seattle skyline and the Sound. Maybe when Patrick’s all grown up.

You know you’re a parent to a child with Short Bowel Syndrome when your vacations revolve around doctor’s appointments. At the end of June, we took Patrick for his semiannual transplant checkup in Seattle. As the friend we usually stay with was both very pregnant and in the middle of moving, we opted to stay in a hotel near the hospital this trip and make a little vacation out of the trip.

Amazingly, this plan of ours actually worked.

We decided on a late afternoon flight this trip, which offered us the rare opportunity to go through security without Patrick’s TPN running. That meant he could go through the metal detectors without needing to be patted down (which is good, since we forgot our stroller). This made the security process seem SO much quicker!

We arrived at the hotel in the evening and checked it to find that the hotel had decided to upgrade our room after I called to verify that my room had both a fridge and a crib for Patrick. Even though we were paying their lowest possible price, they upgraded us to a suite. Imagine our surprise when we walked into THIS room!

Patrick’s favorite feature were the large windows overlooking the street. He spent most of his time in the room watching the cars.

That first night was a late night. After a late run looking for ice cream (and settling for frozen yogurt), we got back to the room to realize I’d forgotten to pack alcohol wipes as well. So Brian had to make a run to Walmar for stroller and alcohol wipes, while I stayed behind to try to get Patrick to settle down to sleep in the room.

The next morning started out really early. Patrick was up with the sun at 6 a.m. But despite being sleepy, we got to get out and have a lot of fun.

First, we went to the zoo. Patrick was a bit confused that there was a different zoo than the one we know at home. But it was great to let him see some different animals! Of course there were the favorites, the elephants and monkeys and tigers and giraffes. But he was also excited to see some different animals, too. Bears and penguins are both animals he knows from books but has never seen in person. One thing was constant, though.. He found a curious orangutan to play with.

We heard one of the zoo docents explaining that this orangutan has an obsession with looking in people’s ears and Patrick was more than happy to oblige.

We ended our visit by going to the farm section of the zoo. We thought this would be a big hit, as it had a petting zoo. However, the petting portion was closed and Patrick was starting to feel the effects of his late night and early morning.

So, we went and got some lunch. (Yummy gourmet burgers). Then, we headed back to the hotel and all crashed for a nice long nap. During our nap, his TPN shipment was delivered to the room and I ended up spending half of the afternoon discovering that one of the boxes hadn’t made it from the front desk to our room.

After getting that all squared away, we headed out for more fun. We drove into downtown Seattle to wander and get some dinner. We started near the Space Needle and then walked a ways to the Palace Kitchen, a Zagat rated restaurant. They looked at us a little funny for walking in with a toddler. But Patrick did GREAT there! And it was by far his favorite meal stop of the trip.

I decided to splurge, since Seattle is famous for seafood, and order some Sockeye (salmon). Patrick was asking for a taste, so I gave him some and he LOVED it! I could barely keep up with him! This kid has good taste, what can I say?

After dinner, we took the monorail to get back to our car. Patrick was so excited when we told him we were going to ride the train. (“Koo koo!”, as he called it.) And once we were in motion, the smile didn’t leave his face!

By far, this was our most successful vacation day in Seattle yet.

Next post: Our checkup at Seattle Children’s.

I guess I succeeded in wearing Patrick out yesterday. He’s sleeping in, so I’m going to sneak in a post and some pictures.

Once a year, our early intervention provider holds a Family Fun Day at Wheeler Farm. They invite families from all of their locations, spanning several cities. And so far, every year, we’ve been out of town when this rolled around. Yesterday, we got our first chance to attend.

Festivities officially began at 10 a.m., which meant we rushed out the door early to finish our errands and get there on time. We checked in and went straight for what we knew would be the longest line later – face painting.

Amazing what these artists can do with a sponge and a couple of brush strokes. This masterpiece took less than a minute on a kid who kept wiggling away because.. well, what were we doing to his face? But once he saw himself in the mirror, he grinned from ear to ear.

The next stop was the wagon ride. I knew that Patrick’s love of tractors and wagons guaranteed this would be a fun adventure. We arrived a bit early for that and got to go out on the first ride of the day. Patrick sat right up front where he could see the tractor pulling us really well.

They started the engine, and he grinned… We started to move and a smile of pure joy spread across his face. THIS was the best thing we could have done all day!

After that little ride, we stayed in the farm to visit the animals. We went first to the goats. Patrick loves how friendly they are and I’ve been promising him goats at a petting zoo for weeks. He walked right up to a little one and gave it a kiss. Then we visited and petted them all.

We saw other animals, too, as we walked back towards the carnival. There was a cow napping against a fence and letting kids pet her, so I took Patrick over to have a try. Just as I pulled out my camera, though, he spotted a goose and started saying “Duck, Duck, Duck” and took off.

I was a little worried, as geese tend to bite, but this one was friendly enough. She was sure Patrick was there to feed her and kept begging. After they explored each other a bit, I led Patrick away.

But the goose didn’t believe that we didn’t have food and followed us honking for the next several minutes.

Back at the carnival, we grabbed a quick drink of water, then went to play some of the little games they had set up. We sat in on a music group singing some of Patrick’s favorite songs. Then headed over to blow bubbles. He played games with a parachute, bowled for pop bottles, sat in a little soccer goal (just because another boy had)… and then, as we had other places to be and were getting thirsty and tired, headed out on our way.

It was a thoroughly fun morning.

I’m going to take a minute for a serious note. Here in Utah, a winter with great snowpack combined with a cold, late, snowy spring has waters running higher than usual in all our rivers. One lake rose 8 feet just last week. There’s a little river that runs through Wheeler Farm that was quite scary to look yesterday. It was overflowing it’s borders and flowing so swiftly it could have easily carried someone away. Over and over again we heard the caution “Keep your kids away from the river.” At one point, the family in front of us was frantically searching for a toddler who’d wandered off and I couldn’t help but worry that the worst had happened. (Thankfully, he’d just gone for an extra turn in the bounce house.)

There have been several drownings of small children in the state this month. And because of them, I’ve been getting a lot of questions about organ donation. So forgive me for taking a serious moment to address these questions.

First of all, yes, drowning victims are often organ donors. In the case of a child, it is the parent’s choice whether or not to donate organs. I will never forget watching a family I met during Patrick’s PICU stay making the decision to turn off life support and donate her organs.

Their consolation was knowing that, in a way, she’d live on.. and that in losing her life, she’d helped to save others.

One organ donor can save 8 lives.

Yes, these children are the right age to be donors for Patrick. No, that doesn’t make us feel any better about their deaths. We’d prefer for him to stay healthy enough to put off needing his transplant a while longer. And we’d prefer to these kids to stay with their families, too. The death of a child is never anything but tragic.

Yes, distance does make a difference in organ matching, so a match listed and living in the same state is given first preference. But there is a lot more taken into consideration. Matches are based on blood type, size, distance, and degree of illness. So, those living closest get first priority, but if a high priority match isnt’ found in the state, then the offer will go to the rest of the country… sickest patients first.

Remember that Patrick’s transplant won’t happen in Utah. So proximity doesn’t come into play in our case. Both Patrick and donor organs will have to travel to Washington. We live about as far from our transplant center as is allowed.

Finally, many of you have asked if all these tragic drownings mean that the odds of Patrick’s transplant happening improve. Well, in a way, yes. They mean more transplants are happening. And that means that the list is getting shorter. However, that doesn’t change the fact that Patrick is a very difficult match for a very rare organ transplant.

And ultimately, it doesn’t change the fact that this is all in God’s hands. Only He decides when a child should come home to him or when their life will be miraculously extended. He guides grieving parents and doctors and organ allocation people. He orchestrates miracles. And He heals hearts when a miraculous physical recovery is not in His plans.

Sometimes seeing Patrick sick and suffering makes us impatient for the transplant to come. But most of the time, we are content to wait on the Lord’s timing. We have been very blessed with a miraculously healthy boy, despite his severe illness and despite all the work it takes from me to keep him that way.

Hopefully that answers some of your questions.

And now, seriousness aside, here is one more seriously cute picture. This is what Patrick looked like by the time we were headed home yesterday.

three blog posts all composed in my mind. This week, I have fallen into bed exhausted so many times that just taking the time to write this quick update seems like a huge sacrifice. But I really do need to get an update out.

So here are a few discoveries we’ve made in the past week.

Discovery 1: We won’t be dilating Patrick’s bowel after all.

…… in fact, we won’t be doing anything about the dilation and narrowing. Why? Well, Patrick’s surgeon asked that we have a barium enema done to get a better picture of the narrowing before putting Patrick through an endoscopy. In the images I posted, the stomach got in the way of the image. So, they hoped that by putting the contrast in from the other end, they’d be able to get a better picture.

And they at least got a different picture. There was no evidence of a narrowing. Just a skinny colon and a fat duodenum. The best guess is that the small intestine is just so big that sometimes the weight of it pinches things off so they don’t move. So, good news that there may not be a stricture. We’re glad he won’t need a risky procedure. And it’s a relief to not have to worry about going from a few dirty diapers a day to 10 or 20 or more.

However, it’s kind of disappointing to have no other recourse. Surgery is far, far to risky and uncomfortable to be worth it. And medications are risky, too. So, for now, we’ll just hold the course. We’ll take the imaging to Seattle Children’s when we go next week and get a second opinion on the problem. However, as Dr. Horslen in Seattle has been talking to Dr. Jackson all along about this, I don’t know that anything will change.

Quite simply, this is a problem that transplant would hopefully fix. And we have to decide if trying for a temporary solution is worth what it would cost Patrick.

Discovery 2: Patrick loves the garden hose. His current favorite job is watering the flowers. We had a miraculous Saturday a couple of weeks ago where I got to plant all my gardens. Now, I’m hand watering them at least once a day. And Patrick often comes along to help. He doesn’t think I do it right and will take the hose and push me away saying “nya nya nya”. Then, he’ll soak the garden and himself.

Discovery 3: Labs don’t always give clear answers. I got lab results yesterday about Patrick’s liver. They confuse me. The good news is that his liver enzymes are back to normal for the first time since last fall. Careful management of his lipids has gotten us there. However, his clotting factors, a symptom of liver problems, are getting worse. This means he’ll bleed a lot more easily. And, well, I don’t know quite what to make of that. We’ll get a second opinion on that, too.

Discovery 4: Even toddlers can have car lust. Ok, well, we all knew that Patrick’s a car fanatic. But yesterday I combined two favorite things… cars, and the hose. We washed the cars yesterday. While Patrick’s tubes were off, I pulled them up on the lawn, then filled a bucket, and handed Patrick a rag. He LOVED it. He walked around the cars washing from time to time, admiring most of the time. The hose was a pretty fun element, especially for putting on the cars. But the cars parked together on the lawn was irresistable.

Discovery 5: It takes 3 days for Patrick to clear barium contrast from his system. It’s very thick stuff. That was a challenging couple of days. We’ll leave it at that. Can’t help but wonder what that says about his motility. We’ll talk about that with the docs, too.

There’s a lot more I’d like to share.. but I think I’m going to leave these discoveries for now and hopefully get another blog or two soon with the other many discoveries I’d like to share.

“My grace is sufficient for thee: for my strength is made perfect in weakness.”  – 2 Corinthians 12:9

 

Patrick reading in his chair. He spends several hours a day here.

Today, I took Patrick to the rehabilitation clinic for a round of Botox shots. No, I’m not a crazy mother trying to improve upon the already near perfect cuteness of her child. This is actually a pretty well accepted medical treatment for dystonia, the muscle spasms that sometimes make walking difficult for Patrick.

The premise is simple. Because of the damage done to Patrick’s brain when his heart stopped, sometimes the signal to his right leg gets confused and instead of moving normally when he walks, the muscles overly contract and his foot turns inward, keeping his heel from lowering as he walks. The faster he tries to go or the more tired he is, the worse the problem is, and the more often he falls.

Yes, I’d hoped to have this done under sedation along with his bowel dilation procedure. But planning for the latter is taking forever and I didn’t want to delay the Botox any further. So, we opted to do the shots without any sedation in clinic.

So, today his doctor injected small doses of Botox into the muscles that were the most tense in his leg. The idea is that these strong muscles will be weakened, allowing the other, weaker muscles to be developed. The Botox will take a week to take effect, and then we’ll start some intensive physical therapy to work with the weaker muscles. The effects will last for about 3 months before they begin to wear off. We hope that at the end of that time, that the weaker muscles in his leg will be strong enough to fight against the dystonia. If not, then we can repeat the therapy.

It was an interesting process. First, his doctor stretched his leg, feeling for tightness there. Wherever a muscle was tight, she marked it with a pen. Then, she told her assistant what dosage of Botox she wanted, and they called down to get it from the pharmacy. It took about 20 minutes for it to be prepared, and then they came back to do the treatment.

I brought along Patrick’s doll, Tubes, to help explain to him what would happen. I asked Doctor Gooch to “give” the Tubes Botox shots so that Patrick would know what to expect. She was really cute pretending to give shots. She was so tickled with the idea that she instructed her assistant to go out and order two dolls just for that purpose in clinic.

After the explanation, we had him lay on his belly so she could reach the back of his leg. He was worried, but trusting, as I knelt face to face with him to offer comfort. They used a special machine that attached to the needle of the syringe to read the electronic signals in Patrick’s leg. I assume that this system helped them to confirm that the Botox was given in a muscle.

Meet “Tubes” (pronounced “Boo), a doll that I added a g-tube and broviac line to to give Patrick someone to relate to, especially when talking about medical things.

I was SO proud of how well Patrick did with this. He worried when we asked him to lay on his belly, and then started to cry when he recognized the feeling of alcohol wipes preparing the skin. They used a “cold spray” to numb his leg, and then put in the needle. He got two shots in the back of his leg: one in the hamstring, one in the calf.. then one more in the muscle in front of his shin. Although he cried the whole time, he tried his best to hold still. And as soon as the needles were put away and we said he was done, he calmed down, accepted hugs, dried his tears, and even signed “thank you” to the doctor when they gave him a matchbox car as a prize.

And then, because that kind of good behavior deserves a reward, we called Brian and he left work a little early to meet us at the zoo.

Boy, was Patrick in a fun zoo mood today! He loved the new replica dinosaurs all over the zoo. He ran around happily visiting all of his favorite animals and places. And, as icing on the cake, we let him ride the carousel for the first time. You should have seen his face when the carousel started to move! He was so excited that we were on our third rotation before he noticed he was sitting on an elephant.

Patrick’s first carousel ride. I don’t think he quit smiling until I told him he had to get off the elephant.

I’m proud to say that at the end of the day, I think he remembered the fun trip to the zoo much more than shots in his leg.

We’ll just hope that the next month of physical therapy will go as smoothly.