TPN – Patrick Hoopes

The line placement was a success

We are home and in our own beds after a very VERY crazy day. Before you have to read too far, I’ll tell you that the surgeon was able to get a central line into the same vein.

Now back to the day…Patrick wanted out of the room after being cooped up yesterday, but we were a little too late in our attempt and the nurse needed us to hang around to make sure medications were given on time this morning. He spent the entire 2 hours the medications were running doing some running himself around the room, despite my protests that he was pulling his line with the very short tubes.

When he finally got to leave the room, he took off literally running… fast enough that I had to run to keep up with the IV pole.

We got to the playroom and he starting flitting from thing to thing.. And then I realized that the damp spot I’d seen on his chest was more than just damp… it was dripping wet. So I looked and, sure enough, the good lumen on the central line had broken.

So, we turned and headed back to the room, picking up Patrick’s favorite child life specialist on the way back. You should have seen the nurse’s face when I walked onto the pod and said, “His central line is broken. Please call the team and the IV team right away.”

We cleaned him up, talked to the doctors, and wrapped up the line to keep it clean. Then Jo from child life helped Patrick place an IV into Tubes’ (Patrick’s medical me doll) hand. As we finished, IV team arrived and we went and got a real IV for Patrick. Explaining the procedure actually seemed to help a little bit with his fear.

Then the GI team came for rounds, got brought up to speed, and redressed the broken line to keep it sterile. We opted not to repair it as it was due to be replaced anyway. Then surgery came by and had me sign a consent.

By then, Patrick HAD to move. So we took advantage of the short break from tubes that having to wait for a bag of fluid that was safe to run through a peripheral IV (TPN has too much sugar and other goodies in it that wreak havoc on veins.). We hopped on his physical therapy bike and started doing rounds of the hospital. My goodness is that boy fast! And even with only one good hand to steer with, he was flying through the halls.

Did I mention that Patrick’s OT said that she was going to recommend that Patrick always get to borrow a bike when he’s inpatient to burn off energy and help him calm down?

Amazingly, riding the bike wore Patrick out. He was so tired he didn’t want to pedal anymore. We came back to the room, rubbed his lavender calming cream on his feet, and he fell right to sleep.

While he was napping, my cell phone rang. It was Dr. Jackson (Patrick’s GI) calling to say that he and Dr. Rollins (the surgeon) had looked at the radiology and decided it was too risky to change out Patrick’s central line. And as I started to explain that that ship had sailed, Dr. Rollins arrived in the room.

He’d just been brought up to speed and knew that the line needed changed. But he told me that he honestly didn’t know where a new line could go. We talked about how much of Patrick’s access had been lost and how he wasn’t sure he could get a catheter to pass over a wire to change the line out over a wire. Then we talked about those scary other places central lines can go like in the groin or liver. It was the first time those options seemed real and I was quite scared when he left.

Thankfully, Patrick napped a little and Brian came up and we grabbed some lunch and talked and I didn’t spend too much time alone with those thoughts.

And then Patrick woke up from his nap and Jo from child life came back with a box of things to teach Patrick about surgery. And surgical transport came to get Patrick, then got called away on an emergency, and came back an hour and a half later.

Patrick finally made it to the OR around 3:30 p.m. The surgeon was cautious, but hoped it wouldn’t take more than 3 hours. We went and set up shop in the waiting room.

And because I didn’t expect a fast outcome, we were surprised to see him back after a little over 2 hours instead

We were amazed when he told us that he’d been able to change out the line over a wire. We were even more amazed when he told us that there was so much scar tissue in the superior vena cava (the vein that enters the heart where lines are usually placed) that the wire wouldn’t go through it. And surprised yet again when he explained that somehow his wire had landed instead in another major vein called the azygos vein. He said that sometimes they do put lines in that vein, but usually have to go in surgically through the chest.

I can’t help to think that that represents a bit of a miracle.

And when we got back to the room we flushed the line. I almost wanted to cry at how smoothly it flushed. I knew Patrick’s line was bad, but feeling the contrast makes me amazed that it lasted for 15+ months.

And then, because they offered and because we were tired and homesick, we accepted a quick discharge.

We will still need to go back in a couple of weeks for a venogram to see exactly where Patrick’s veins run now. There’s this amazing process called collateralization where the body, when it starts to have thrombosis or scarring in a vein, starts to make new blood vessels instead that connect to the heart in all kinds of crazy ways. A venogram will show us exactly how those things connect.

We did one in Nebraska, but it was somewhat inconclusive. So we are going to give Primary Children’s a go.

But for now, it seemed like we’d had enough hospital and enough sedation and it was time to let everyone rest. Patrick ran around home very happy tonight. And very funny. He was making up all kinds of silly jokes that made me laugh. He even ate 1/4 of an applesauce cookie.

And on that note, I’m going to quit staying up while the rest of the household is asleep.

Thank you all for your prayers and thoughts and love.

Watch out for waterlemons

A week ago Saturday, we decided to go out for breakfast at Paradise Bakery. Because of nut and egg allergies, we brought his breakfast along, but when he asked to play with my fruit cup (practicing his fork skills) I agreed.

He picked up a piece of pineapple, dropped it, and gave a very big scowl. I looked to see what was wrong and discovered a long, thin cut in his finger. I thought maybe one of the finger pricks from the previous weeks’ hospital stay hadn’t healed and was infected.

So, when we got home, I put some neosporin on it, and a bandaid. That night, when I changed the bandaid, Brian pointed out that the rest of that fingertip and the one next to it looked like they were burned. We asked Patrick how he got hurt. He said the “waterlemon” poked him.

We kept doing bandaids and neosporin. And then a couple of days later, I bumped Patrick’s other hand and got the same reaction. He jumped, pulled back, scowled, then started to cry. I looked and, lo and behold, the other hand was dry and cracking too. All of his fingers looked calloused, dry, flaking and cracking.

I panicked a little bit. TPN dependence means risks of nutritional deficiencies. Zinc deficiency causes horrible flaking skin ulcers. Fatty acid deficiency can cause dry skin, but only once it’s severe enough that you also start wondering about brain development.

I called his dietitian and made her check his labs. They were ok, except a note about low Omega-6 fatty acids. The doctor had just said he thought it was ok. She suggested I rub Patrick in safflower oil.

This created quite a quandary for me. Last time I tried safflower oil in Patrick’s diet he had an allergic reaction because all culinary oils are processed on the same equipment so safflower oil can have peanut oil and almond oil in it.

So I decided to start using his regular eczema cream and keep asking.

I got a good picture of the problem and sent it to, well, everyone. His nurse suggested an allergy to the bandaids or neosporin. His GI and I had a long talk where we discussed ways to get him more fatty acids from increasing the lipids in his TPN to feeding him microlipids through the g-tube, to rubbing him in it. (Which I put more effort into. We stopped at Sprouts market that night and read every label until I found a lotion that had safflower oil, but nothing else he might be allergic to.) Finally, the University of Nebraska called back and said that Patrick’s labs had looked good in February and could not have possibly tanked that quickly and to stop worrying about deficiency.

So then I turned to Dr. Google. I try not to research symptoms on the internet. However, Google Images is a wonderful took for looking up skin conditions. I looked up pictures of zinc and fatty acid deficiency and they didn’t match. Then I looked up pictures of cracking fingertips and I found it…

Fingertip eczema. Caused by… a systemic allergic reaction due to prolonged exposure to an allergen.

I ditched the bandaids. Switched to hydrocortisone cream and aquaphor… kept rubbing Patrick in the safflower lotion for good measure because of the other deficiency anyway. And I stopped cheating Patrick on his benadryl pretreatment.

Because Patrick is allergic to vancomycin, the antibiotic they started him on 3 weeks ago, he is supposed to get a very high dose of benadryl before every dose of antibiotic. But that high dose does a number on his gut, not to mention his concentration and his mood. So when we came home from the hospital, I started backing off the dose.

So we upped the dose.. and then we got hospitalized and they took him back up to the full prescription strength. And they made him stay on vanco a few extra days in case that was the cause of the fevers, and then finally let it be discontinued.

Patrick’s fingers almost immediately started to get better. They are still dry and we are still doing a good lotion regimen a few times a day. Patrick voted down a few brands, but is really in love with Aquaphor (“Apa-poh”) and several times a day will ask permission to just dip his fingers in it. This is helping a lot.

Poor little kid.. as if there weren’t already enough other things to worry about this month. And I still can’t convince him to lay hands on another “waterlemon.”

Adoption memories

We had Patrick’s g-tube study done. (Great results! Nothing wrong. Just a slightly upward angle that makes positioning the tube tricky.) As part of the history, they asked when the gastrostomy (g-tube hole) was created and I realized last night that I could have answered “exactly two years ago.”

Why do I remember that? Well, because exactly two years ago yesterday, the court officially named us as Patrick’s legal guardians. It was the best birthday present I’ve ever gotten.

A friend of mine has been doing something special this month on her blog. Because it’s national adoption awareness month, she’s been posting daily adoption related posts. She invited me to be a guest blogger and, by coincidence, will be running my post today… a very significant 2 year adoption anniversary for us.

So, I thought I’d share with you what I wrote for her. Here goes:

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Ours is not a typical adoption story, because Patrick is not a typical little boy. His life was meant to be something different, something miraculous, and so it required that it start in a very different and miraculous way.

But my part of the story starts the way a lot of others do. We wanted to have children. When that didn’t happen easily, we involved doctors. For years, we went through the ups and downs of charting and temperature taking, tests and medications. Finally, after several years and a minor surgery, our doctor sat us down for “the talk.” He explained that there were several causes of my infertility. The cards were, essentially, stacked against us. He still felt it very possible that we could have children, but only with major medical intervention. We had some big choices to make.

We talked about it and we prayed about it. And then, that Sunday, as we sat in church, we received a clear answer that it was time for us to stop medical treatments. Our child would come to us through adoption.

With a path finally before us, we moved forward quickly. I’ve never felt so driven to do anything before in my life. In under a month, we completed the application process, training classes, and were mostly done with our home study.

During our home visit, we had a conversation with our case worker that would play a major part in bringing Patrick into our family. She’d looked at our “preferences checklist” and noted that we seemed more open than most to adopting a child with special needs. We explained that we felt that adoption was a faith process. We believe that Heavenly Father puts families together. We knew we’d never turn away a child born to us with medical problems. So, if God was in charge of adoptions, too, then why would we limit His options? We knew Heavenly Father would help us find our child and that, if the child really belonged in our family, race and health wouldn’t stand in the way.

We decided to adopt in June. Our application was approved in September and we hunkered down for a nice long wait. We figured two years, at the least, was the average we’d heard. And still, by the end of October it felt like far too long. My heart ached for a child it knew was missing.

Then, on a very snowy morning the first week of November, my phone rang. It was my case worker. She started out by saying, “There was a little boy born on Halloween in Michigan.” My heart skipped a beat. I grabbed a pen and a piece of paper and started scribbling notes. She told me he was Korean. And then, she went on to tell me that he’d had a birth defect. His intestines had developed on the outside of his abdomen. The doctors were saying he had a life expectancy of 1 to 2 years. They needed to find an adoptive home quickly because doctors wanted to discharge him from the hospital. All she could tell me about his family that his birth mother wanted him to be able to go to the temple to be sealed to a family.

She said she’d send an e-mail with more information and a picture. She encouraged me to talk to Brian and decide if we’d like to be among those families considered to adopt this little boy, and then to call her and let her know.

As soon as I gathered myself, I called Brian. But he wasn’t at his desk. Meanwhile, two e-mails arrived. One was a short paragraph from the baby’s caseworker in Michigan explaining his medical needs and the unconventional and hurried search for parents. In the other were two photographs of a sweet little Korean boy with great big eyes and an IV in his head.

Since Brian wasn’t at his desk, I called the insurance company to find out if this we even had coverage to pay for this kind of medical problem.

That’s how Brian first found out about the offer. While I was on hold with the insurance company, he called back on my cell phone, so he heard me finish the conversation about “preexisting conditions” and “adoption”.

I gave Brian the information and, after a quick moment of thought, he said he’d come right home.

We had a prayer together, then went to the temple – the perfect setting to make decisions about life and death and eternity.

I knew that families are eternal. I knew that mortality is not the end of life. And yet, I was filled with grief. It was as if I’d just been told I was carrying a child with a terminal illness, but he wasn’t even mine yet. And I was scared. I didn’t know if I was ready to leave the life I knew then.. abandon it all, and become mom to a child who would need so much help, and who had such an uncertain future.

Still, when Brian turned to me and said, “I think we should pursue this,” my heart leapt with joy.

So, we called our caseworker and gave her a list of questions we had. And then we went to visit our parents. We felt we should tell them about the offer, because we knew that whatever happened, we were never going to be the same. And we both wanted father’s blessings. We showed them the little boy with the angel eyes and explained that we didn’t know if he was ours.. But from that moment, all of our families were praying for a little boy whom the e-mail called “Patrick.”

That was Wednesday. Thursday, I sent a copy of our profile. Friday afternoon, as I on my lunch break with Brian, our case worker called my cell phone. The birth family had seen our profile and had chosen us to adopt their baby.

Now, we had a choice to make. Because we’d been selected, we could finally start filling in the gaps in the medical information we were getting. And boy, where there gaps! We called the baby’s caseworker, who referred us to the hospital social worker. Finally, we decided we needed to talk to doctors, and we needed to do it face to face.

I called my mom and told her to take my credit card and buy airplane tickets. Then, I went back to work, explained what had happened, and asked for a leave of absence. After that, we went to the adoption agency where we signed pre-placement paperwork required for us see the baby in the hospital.

Friday night, we tried to get ready. We booked a long-term stay hotel room. We faxed legal documents to Michigan. We make a shopping list of nursery items. And we tried to pack.

I packed my bags that night not knowing what exactly I was packing for. We still didn’t know enough to say if we could take care of this baby. We didn’t know if or when he’d be discharged. We didn’t know how long it would take before we’d be given permission to leave the state again.

And yet, Saturday morning as I sat on a plane to Detroit, 10 rows ahead of my husband, I felt a quiet, happy calm. If nothing else, I knew it would be ok.

We met Patrick, his family, and his doctor Saturday night. It wasn’t what we expected. Due to unforeseen problems, things were tense at the hospital when we arrived. We felt like we knew nothing at all about his condition when we heard the doctor’s account. His case was much more severe than we’d understood, but the immediate prognosis was better.

At last, they led us to his room. My first impression was of how small he was. He was SO tiny! Just a little ball with wires and tubes attached. Without them, you’d have never guessed there was anything wrong.

They let me hold him while we talked. He felt so small and fragile.

I thought that the moment I met my baby, or the moment I held him, that I’d know he was mine. But that isn’t what happened for me. There were too many questions, still and I’d have to wait for that confirmation.

Sunday, we arranged to spend the day with Patrick. The nurses were so kind to let us change his diapers and help with other aspects of his care. I sat for hours singing him lullabies and watching monitors and letting him sleep.

When we arrived, the nurses warned us that he had a reputation as a very irritable little boy. There was even a sign on his door warning not to wake him. He was famous for screaming hysterically if his sleep was interrupted. But that’s not the baby I met. He was just a sweet, tiny little boy who wanted to be held.

I remember singing to him: “I am a child of God, and he has sent me here. Has given me an earthly home with parents kind and dear.” And my voice choked on the words because I knew that right at that moment, Patrick didn’t have that. I couldn’t imagine how any little boy could go through all he’d need to go through alone.

That night, as we looked at pictures from the day, I came across one that showed just his face with a white background. I knew, when I saw that picture, that I loved him.. and I wanted to keep him.

Monday morning, we held a “family conference.” It was a business day so we finally had been able to confirm that there were doctors to take care of him at our hospital at home. Our insurance confirmed that he’d be covered. Brian needed to hop on a plane to go back to work. (He was running a conference that week.) So, knowing we had the resources to provide for his physical needs, we asked Patrick if he’d like to be a part of our family. I swear, he looked up at Brian and smiled.

The case worker rushed to the hospital and by 1, we’d signed paperwork, and I was on my way to the airport with my husband. I was staying behind to start a whole new life.

The next few weeks in Michigan are among the sweetest of my life. With nothing else to do but hold my new baby and learn to care for him, I virtually lived in the NICU. My mom came for a week and shared with me in Patrick’s first feeding, first bath, and first time wearing real clothes. This time was also some of the hardest I’d experienced as I received a trial by fire as a mom of a child with major health problems. Patrick had his second surgery the day Brian flew back to be with us.

Two weeks after we signed papers, on my birthday, the birth parents appeared in court, and we were named as Patrick’s legal guardians. A week later, we had permission to bring him home. At 4 a.m. Thanksgiving day, Patrick and I arrived at Primary Children’s Hospital by air ambulance. He’d spend the next few weeks there as the doctors here got to know him and made arrangements for us to take care of him at home.

Because of his medical needs, the courts granted an early finalization of his adoption and we were able to take Patrick to the temple to be sealed as a forever family in February when he was just 4 months old.

Patrick just turned 2. He is an active, happy toddler who loves cars and music and Elmo. He is a living miracle! Patrick’s birth defect came with a rare complication. As a result, at birth he was missing over 95% of his small intestine. Without intestine, he doesn’t get nutrition by eating. In fact, eating large amounts puts him at risk for dehydration and bowel obstruction. Instead, he is entirely dependent on a form of IV nutrition called TPN. He has a permanent IV tunneled through his chest, into a vein in his chest or neck that runs to his heart.

The TPN leads to complications like infection and liver disease. In his short 2 years of life he has already struggled with both. Patrick’s doctors warned us before we adopted him that we’d become such regulars in the E.R. that we’d be on a first name basis with the staff. We soon found that to be true not just for the E.R. staff, but also the IV team, the infectious disease team, the PICU team, most of the residents, several of the medical students, and the entire gastroenterology department.

At 9 months old, as a result of infection, Patrick’s heart stopped. The fact that he is alive now is nothing short of a miracle. No doctor who hears his story and then meets him can help but confess that he has beaten the odds in countless ways.

Patrick will eventually need an intestinal transplant. He is already running out of places to put new IV’s and each new infection makes him a little more fragile.

Since they don’t do intestinal transplants where we live, we have chosen to have Patrick listed at Seattle Children’s Hospital. Patrick has been on the waiting list since April of 2009. He is status 1A and will have his transplant is soon as a donor match is found.

People try to tell us sometimes what a tremendous thing we did in adopting Patrick. We don’t really feel it’s something we can take credit for. As we told our caseworker when this all started, Heavenly Father puts families together. He knew Patrick needed us. And what’s more, He knew we needed Patrick.

Raising Patrick has taught us more about life than any other experience. We have learned to rely entirely on the Lord. We have learned to live each moment to it’s fullest. We have learned to lean on one another when things are hard and we to trust in hands of friends and strangers when we felt too weak to stand on our own. And we have learned to love like we didn’t know it was possible to love.

Little Heroes

You’ve gotta see this story!

http://www.abc4.com/content/news/top_stories/story/Donor-transplant-brings-two-Utah-families-together/_A0j2PcCfEOkr4N_XWaMmw.cspx.

As you know, Patrick attends a weekly play and music group for children with a variety of health problems. One of my heroes from this group is a little girl named London, or as those who know her call her, Lulu.

Lulu had a liver transplant a year ago. When she first came to our play group, her mom immediately recognized Patrick’s TPN, having been on it herself when she was at her sickest. Lulu is one of the most vibrant, outgoing little girls that I’ve ever met. She and Patrick seemed to have an instant draw to each other.

One other thing they have in common is that Lulu’s family, like ours, is trying to raise awareness of the need for organ donors. Both of us have been active in working with Yes Utah, our local organ donation awareness organization, and in sharing our stories to help encourage people to sign up as donors.

Last night, Lulu’s story was featured on the evening news. A transcript of the story has been published here. (Just in case you didn’t click on the link at the top of this page.)

Please read it, and share it. And if you aren’t already registered, please consider registering as an organ donor.. and make sure your family knows of your desire to be an organ donor.

On another note, we never got a call yesterday inviting us to come up to X-ray, so that test will probably happen sometime next week. I’ll admit, I didn’t mind much. Patrick and I were both burned out from the previous two days and it was a rare treat to get to spend the day at home. Chris, his developmental specialist came to play. Patrick adores this man and LOVES their visits. Then his cousins stopped by for a short play time. And then, after dinner, we curled up on the beanbag movie and watched Cars.

I suppose that that’s only a partially true statement. Patrick was all over the room, but he returned regularly to cuddle with me or to tickle or to blow zrbbts on my tummy. This is the first time Patrick’s seen the movie Cars and, as I had predicted, he loved it. He’d return often to just sit with me and watch a scene or two. He got especially excited whenever Mater appeared on the screen.

And then, after Patrick was in bed, Brian finally made it home from Chicago. There is so much comfort just in having him here. And it was so nice to have extra hands when Patrick pulled his usual trick of picking his central line dressing off during the night so we had to change it first thing in the morning.

Today I’m proudly sporting a new Giordano’s t-shirt that Brian brought back for me. In case you don’t know, Giordano’s is the best Chicago-style pizza chain on the planet. And if I can’t have the food (Howie’s trying not to rub in the fact that he had Chicago-style pizza not just once, but twice this week), at least I can wear the schwag.

When the cat’s away, the mice will….

…Well, the idea was to play. This is the 2nd and last night that Brian will be away on business and I thought that, other than a Wednesday full of appointments, I had a pretty laid back schedule. So far, this past few days have been anything but laid back.

Wednesday morning, right after kissing Brian goodbye, I went to set out Patrick’s morning fluids. They weren’t cold. This was especially concerning as I’d been setting the fridge to colder and colder settings all week and there was snow on the ground outside.

So, as Brian was finishing up his last work before heading the airport, I was scrambling to shop for a new fridge. Thankfully, Patrick’s morning therapy appointment was cancelled which gave me just enough time to hit a few stores before his nap.

After his nap, we went to his monthly appointment with his GI at the hospital. All the news is good. Patrick is growing at a good rate right now… he’s still 50th percentile for weight… just above the target weight for his height. His liver is healthy. He’s about as stable as can be.

The only change we discussed was a change to his antibiotic regimen. Patrick takes oral antibiotics to help keep bacteria from accumulating in his stomach and gut. Only lately, it seems that he’s had more and more problems that lead to stomach upset, vomiting, and diarrhea. So we switched around the medicines a little bit in the hopes that a new plan would make a difference, and then headed out.

We stopped at Sears on the way home to pick up the fridge that I’d picked out. You should have seen the look on the man’s face when I told him I wanted to put it in my car with my baby. Patrick wasn’t too excited about the idea either.

Still, we made it home and got the fridge up to his room and furniture and Patrick ready for bed.

We’d almost made it.. and then, as we were saying goodnight to Brian by video chat, Patrick stepped on the tube that was draining from his stomach and I heard a “pop”… and looked down to see that his button on the floor, the balloon that was supposed to hold it in still inflated.

Thankfully, my neighbor answered when I called and came quickly. It wasn’t easy, but we managed to get the button back in and Patrick comforted and into bed.

Thankfully, today was easier. Patrick and I managed to stay home the whole day with time to help my sister with a paper for school. But I got to talk to the GI clinic several times.

I started it. I called them to talk about how very difficult and painful placing buttons has been recently for Patrick. So first the nurse called back to find out more. Then Patrick’s dietitician called to talk to me about his TPN orders.

Apparently, as Patrick has had more and more stomach upset, and we’ve had to give more and more replacement fluids, his labs have been showing increased dehydration. They decided to try to make some changes to help him be more comfortable. They’re adding another half a liter of fluid to his TPN, plus some electrolytes so that we won’t have to give as much to catch up for what he loses.

Then, this evening, Patrick’s doctor called to talk. We reviewed the plan for hydration, and then he asked about the g-tube.

In the end of the conversation, we decided it was best to check to make sure that there isn’t a space between Patrick’s abdominal wall and his stomach making it hard to get a g-tube in. They’ll do that by taking out the tube, and then putting it back in filled with some contrast. This will let them see if there are any spaces or leaks to be concerned about.

We talked about doing this tomorrow, and if not tomorrow, then sometime next week. The good news in the plan is that they’ve offered to give him a little bit of sedative to help him calm down and not remember the discomfort.

That’s a lot of changes in just a couple of days. Thankfully, I’ve had all the right help come at all the right times. Thank goodness for good friends, visiting teachers and family who’ve been there to help. And I’m sorry for those who may have called and gotten a frazzled forgetful me who couldn’t even think through all the details of this, let alone speak them.

Most of all, thank goodness for a good-natured, patient, loving little boy who has been incredibly cooperative and given hugs at all the right moments.

Brian may rethink his next business trip.

In the midst of miracles

This morning as I was laying in bed trying to convince my tired body to get out of bed to greet a happy 7 a.m. Patrick, I was contemplating on just how far my munchkin has come. And all of a sudden I realized that I am living in the midst of miracles right now.

I am a VERY tired mother right now. By about 5 p.m. every day I just want to call it quits and go to bed because the work of taking care of a TPN-dependent, developmentally delayed toddler is exhausting! Patrick is a very active little boy right now. He’s on the brink of walking. He’s finally mastered the skills of carrying things from one room to another, of opening drawers and doors and emptying the contents found therein, and of putting things in places where I sometimes never find them again.

He’s bordering on two with the fully independent attitude that comes with that age – while simultaneously he’s finally getting the strength in his body to explore his world in ways he never has before. My good-natured patient boy has discovered tantrums. And he’s not afraid to use them to tell me when he doesn’t approve of me stopping some unintentionally self-destructive activity.

And, if the exploration weren’t dangerous enough, try attaching IV tubing to this strong-willed child! Even with a 10-foot extension, he manages to get himself wrapped in and around furniture. He knows no limits! If I leave a baby gate open for even a few minutes, you’ll find him at the top of the stairs grinning, waiting to run from me the second I come to catch him before he reaches the end of his line and gets yanked back down.

And this morning as I lay procrastinating getting out of bed I realized just what a miracle my total exhaustion is! This time last year, we’d just come home from the hospital. Patrick was thin and frail, not even able to roll over. Our current battles in physical therapy show that there should have been much more major consequences of his illness and arrest last summer. No one who hears his history ever expects to find normal looking boy smiling up at them.

But right now Patrick has been blessed with exactly what I’ve always wished for him… The strength of both body and spirit to not know boundaries. He may still be limited, but he doesn’t feel limited.

I take it for granted sometimes because they’ve been so common in our lives. But Patrick, Brian and I live in the midst of miracles each and every day. And we do so with full expectation that more miracles lie ahead.

“For behold, I am God; and I am a God of miracles; and I will show unto the world that I am the same yesterday, today and forever” 2 Nephi 27

Swimming!

That’s right! Patrick go to go swimming for the first time last week! Brian’s company had it’s summer party at Cherry Hill waterpark.

This is a super big deal! You must understand that we were told that Patrick couldn’t even have a bath in a bathtub as long as he had a central line. But – after experience with baths and trading ideas with other parents, we decided that we could probably pull this off.

So – I packed an enormous duffel bag full of emergency medical supplies, just in case his central line dressing got wet.

We waited till his 1 hour TPN break so he wouldn’t have need his backpack. We dressed him in his brand new swimming suit.. and then we got started on the waterproofing. First, came an aquaguard, a waterproof piece of plastic with adhesive that you can cover the central line with. We tucked the ends of his line up inside of that. Then, we wrapped his torso in Glad Press n’ Seal wrap to catch any gaps in the aquaguard from him moving around. Then we put his little swim shirt on and headed down to the water.

When we sat down in the kiddie pool, he was pretty uncertain.. Daddy splashed water on his head and I thought that for sure he was going to decide he was done. Then we decided that since it was a wading pool, maybe a walk would help. Brian took one hand and I took the other and away we went. This was the clincher. He was fine being in the water once he realized how much extra balance it gave him.

Once he was comfortable in the water, he was brave enough for other games. He sat in our laps and kicked to splash. He went with dad down the water slide. His favorite, though, was for Brian to take him by his hands and swing him way high in the air, and then splash him down in the water. So long as he didn’t get too wet above the waist, he was happy. (We can’t complain too much about that. It helped protect his line.)

After about half an hour, we had to go back to get his TPN reconnected. But he was pretty tired and done by that point. We dried him off. (Our waterproofing worked and his dressing had stayed dry.) And got him dressed and he had a great time making friends at the barbecue that came with the rest of the party.

Who needs sleep?

There’s a song by the Barenaked Ladies called “Who needs sleep?” Here’s a line from that song: “With all life has to offer, there’s so much to be enjoyed. But the pleasures of insomia are ones I can’t avoid.”

If you’ve been waiting for an update on the concert, I need to apologize. See, Patrick’s been having a hard time sleeping this week. It seems every few nights something goes wrong and wakes him up. First it was diaper rash. (When his prescription strength creams fail him, the result is massive skin breakdown that makes me want to cry just looking at it.) Then, I accidentally turned off his TPN pump and had to monitor glucose and hydration in the middle of the night. And my little happy-go-lucky optimist responds to these discomforts by trying to cheerfully play through them. So instead of being up crying, he’s up jumping and playing until I pinpoint the cause of discomfort and get him settled.

So – my good intentions of writing earlier in the week were thwarted by extreme exhaustion. And then a series of coincidences landed us in the hospital for about 36 hours.. not helping sleep, but helping to remind me not to procrastinate.

Here’s a rundown of the other events of the week.

Wednesday, Patrick had an appointment with his GI, Dr. Jackson. Patrick’s central line was a bit slow to heal this time around and was a bit weepy even 2 weeks after placement. So I asked the doctor to look just to make sure there was no infection there. Since we were looking for infection, he checked his temperature and it was 99.3. So – Patrick and I hung around for an extra couple hours in the hospital. Dr. Jackson came in and we took off his central line dressing so he could examine it up close and take a culture of any fluid that was there. It looked healthy, just healing, so I went ahead and put the dressing back on. Then we went down to the lab and had blood cultures drawn. Those cultures were all negative.

That night, I got that getting sick tickle in my throat and started to run low-grade fevers.. kind of like when you get a flu shot. Never sick, but not quite right. Since the cultures were clean, I said “Ok, he has a virus, too” and didn’t think more of it.

Friday, Brian came home early from work and since we’d all missed a lot of sleep, we all laid down for a nap. I got Patrick up to put on his afternoon TPN around 4. Only when I tried to draw ethanol out of his line, I just got air. Tried again, got air again. Finally, 3 syringes full of air later, I looked and found a hole in Patrick’s central line.

So away to the E.R. we went. They’ve implemented a new policy that sent us to the Rapid Treatment Unit (RTU) for the repair which, by the way, is WAY preferrable to the E.R. Many fewer bugs and much quicker, more attentive care. The RTU is set up to give basic medical care that takes 24 hours or less.

Well, part of any admission is to check a temperature and Patrick’s read about 100. They rechecked it rectally and it came up 99.8, so we could justify not automatically being admitted. I explained the viral symptoms, but they decided to check cultures anyway. Then they repaired the line and sent us on our way.

The next day, Patrick woke up feeling great! No fevers. So since it was memorial day weekend, we packed up and headed out to Tabiona – a small town in Eastern Utah – for a family reunion. He loved the car ride.. playing in the back seat, singing with the radio, napping, and even trying to figure out how to whistle. Had a great day with cousins, aunts and uncles.

That evening, we got home to find two messages on our answering machine. The blood cultures they’d drawn were showing a staph infection.

Now, in case you haven’t noticed this, I’ve spent a lot of time learning from infectious disease over the past year. And one thing they’ve taught me is that 1 in every 20 positive cultures is a “contaminant”.. that is, something that grew in the culture that didn’t come from the blood sample taken. And staph, although it lives on all of our skin and can get into central lines, usually isn’t one you pick up at home. It’s most often contracted in the ICU. Every positive “staph” culture Patrick has ever had has been a contaminant.

So – I called the doctor and made my case that Patrick wasn’t sick and that this was likely a contaminant. We decided to recheck the cultures on Sunday.

Well, Monday morning rolled around. For once, we were planning to be home for Brian’s day off and had a big to-do list.. And at 8 a.m. the phone rang. Sunday’s culture was positive for staph, too. Patrick’s still healthy, but we’d better go in.

So, just to be safe, that’s what we did. We got there at 9:30. Because it was a holiday, things took longer than usual.. but by early afternoon they’d drawn a new set of blood cultures and by 4:00 p.m. had started some antibiotics. Meanwhile, Patrick’s nurses got to run to try to take care of all of his basic daily needs.. a slow process when doctors have to write for them and pharmacy has to fill them before it can happen.

A quick soapbox moment. One of the most frustrating things about going into the hospital is how difficult it is to maintain the same quality of care and quality of life as at home. There are so many more steps, so many more people, and so many more lawsuit-prevention policies that it is exponentially more difficult to accomplish the same things that I do at home in the midst of daily life. In a short 36 hour stay, I think the nurses had to call the pharmacy at least 10 times about administration questions, late medications, and my ever-hated argument about whether or not they’ll let Patrick have his home TPN. (I usually lose this battle and they hang something with sugar, water and electrolytes but none of the good vitamins, minerals, and fats that he’s used to.) They started him out on a super high dose of antibiotics. (I won’t let that happen again. I’ve seen it done 3 times now with the same result and I’ll speak up next time.) And they accidentally ran his TPN at a 5% of it’s prescribed rate for the night. ( Thankfully, this only resulted in a grumpy, sleepless night as Patrick got hungrier and thirstier. They caught it in the morning and there was no other harm done.) I can’t really fault the nurses here. They work their tails off trying to get everything right within Patrick’s first 24 hours. The fact of the matter is that he’s a complex kid who has a lot of special care. For me it’s routine.. but in the hospital, it’s the exception. In fact, there are some things that require special permission every time because it doesn’t match hospital policy. Still, it’s frustrating to me to have to work so much harder to maintain the status quo. I much prefer to just do it myself at home. Ok. Getting off my soapbox now.

Yesterday morning, Dr. Jackson came on service. I ran into him at the nurse’s desk looking up info to find out why Patrick was in the hospital. We talked about the 4 sets of blood cultures that had been drawn. By then, the cultures drawn in the hospital Monday were still negative for infection. Looking back, it was looking more and more likely that we’d had two contaminants in a row. So Dr. Jackson said the words that we love him for saying so often: “I think you can do this at home. Would you like to go home?”

He helped sort out a few more questions and then set the wheels in motion for us to go home. Because they’d started Patrick on an extremely high dose of antibiotic, we had to stay till 4 to have them check his blood one more time to make sure that he’d been able to get it back out of his system. Brian got off work and up to the hospital by 5:30 p.m. and we made it home shortly after that.

Patrick will be on antibiotics for the next 2 days at least and then they’ll check cultures again to make sure that he doesn’t have a real infection. And then hopefully things can go back to our at-home normal again for a while.

Whatever happens, we’re resolved to made better use of this time at home. Procrastination isn’t really an option when you can’t tell where you’ll be hour to hour. I would hate to get the transplant call and leave my house in the condition it’s in right now.

And – I’ll be getting that blog entry about the concert up hopefully before the end of the day tomorrow.

Oh – the best news of all? With us healthy and at home, Patrick slept a blissful 11 hours last night! Which meant mom and dad got some sleep for once, too.

Unpredictable

I like planning and I like things going as planned. I’ve never been very spontaneous. Patrick is teaching me things.

We thought we’d be heading home a couple nights ago. Patrick’s weekend was completely uneventful. He was healthy and happy and doing really well. Everything was in order – he was on the schedule for his line to be placed on Tuesday and then once it was in, we were going home. We’ve done that surgery outpatient before, so why not go home the same day, right?

Monday night my nurse couldn’t find a record of him being on the surgery schedule, though.. even though I was sure they were planning on it because his surgeon had talked to me about doing it. All night long I had nightmares about him not getting his line.

But before the sun was up Tuesday morning, I was signing a consent for surgery. That afternoon, out of the blue, surgical transport came to get Patrick. I had a pow wow with his surgeon in the hall on the way down then went through the usual presurgery consent and exchange of history, kissed him goodbye, and went to the surgery waiting room. Pretty routine.

This time, though, things were just slow! This procedure usually takes 30-45 minutes. And an hour and 15 I was asking for updates from the O.R. But then, minutes later, his surgeon came to tell me the line was in and everything had gone well.

Great, I thought, and sat down to wait again. Typically 15 minutes or so after the surgeon visits me I get to go back to recovery. 15 minutes passed and then the anesthesiologist came to talk to me. He explained that Patrick had somehow been extubated during the procedure, but that he thought he was ok.

2 hours after I got to the waiting room, they finally called me to recovery. Patrick seemed to be just fine.. mellow and with tears dried in his hair, but ok. We brought him up to the room and he cuddled up with me in the rocking chair.

Soon it was time to give his afternoon medicines. Because of his allergic reaction to the caspofungin, they give him Benadryl first now and that on top of the anesthesia helped him fall very fast asleep.

Brian came and packed up the room. The doctors came and gave me discharge instructions and prescriptions. We were just waiting out the medication and waiting for his TPN to be delivered.

Then I noticed that Patrick felt rather warm. So, reluctantly, I paged the nurse and asked her to check his temperature. The result – 103.3.

Just then, the TPN arrived.. the last piece we’d needed to go home. And we were staying. They drew a bunch of labwork to look for infection, just to be safe.

Patrick’s lungs seemed a bit coarser than normal and he had a cough. The doctors theorize that the fever was a result of something that happened with the anesthesia or his airway while he was in the O.R. Throughout the night, he finally got some good coughs and his fever went down.

By morning, he seemed to be just fine. They stopped the antibiotics they’d added as a preventative measure.

So – here we are still. I’ve learned never to really count on the first discharge date we plan for… but we were so close this time!!

We’re ok, though. We’ve got a fair amount of cabin fever. And I’m definitely missing some of the comforts of home like soft toilet paper and going barefoot. But we’ll make it through. At least he’s happy and healthy and allowed to leave his room to go to the playroom where he can spend hours running around the room holding my hand with one of his, and pushing a push toy or chair with the other while i try to keep up with the IV pole.. keeping his feet out of the tubing.

We’re hoping to go home maybe tomorrow morning, now. <Knock on wood>. To do that his cultures have to stay negative and he has to not give us any more surprises.

An excellent Seattle trip

Patrick had his quarterly appointment at Seattle Children’s on Thursday. And it was a very good trip in every way.

Because Patrick’s morning med schedule is so complicated right now, I opted to fly out on Wednesday afternoon. Our flight left at about 2 p.m. As usual, it took some effort to get through security and I probably looked insane hauling Patrick, his duffel sized diaper bag, two suitcases, a carseat, and of course, him in his stroller around the airport. But we made the flight without incident. In fact, we landed early and had time to visit and exchange blogs with a very nice woman from the same flight while we waited for our ride.

We stayed with my friend Lindy, her husband Kelly, and her little girl, Lauren. Lauren is 4 months younger than Patrick. They have always gotten along really well and it was fun to let the two of them play. Most of the play consisted of stealing each other’s toys and pacifiers.. but they did spend some time dancing to YouTube videos and there was more than one hug exchanged.

Patrick and Lindy

Thursday were the appointments. It was kind of strange to actually be seen in clinic. This is the first time since Patrick’s evaluation a year ago that we’ve done this visit in clinic instead of inpatient.

They did the usual set of vitals: weight, length, blood pressure. As we finished, another little boy about Patrick’s size came in to be weighed. He had a Broviac line and TPN in a backpack, too. I think this is the first time that I’ve ever met another kid on home TPN. It was kind of strange for me to see.

Our first visit was with Patrick’s dietician. She walked in and her first words were, “This weight looks spectacular! I had to come see if it could be correct!” She remembered meeting a tiny, frail, jaundiced baby last year. To be met by a happy, chunky, energetic (almost to a fault) toddler was a surprise.

She looked at Patrick’s TPN, his labs, and his growth charts. We talked about his current diet and in the end, she said that she was nothing but pleased with what she was seeing. She even said that it’s time to back off a bit on his feeds so that we don’t make him overweight.

It’s been recommended recently by some doctors to try continuous feeds again so I asked her her opinion of it. She told me that it’s pretty common for kids with anatomy similar to Patrick’s to stop continuous feeds after this long. She said that focusing on oral feeding so that Patrick would have an easier time learning to eat after his transplant was her preferred goal.

She also explained that some kids who’ve had problems with hypoglycemia when tiny can outgrow the problem and tolerate breaks from TPN. She watched Patrick attempt a few head dives off the bench we were sitting on and said that she thought it might be good for him to have some untethered time. This is something I’ll discuss more in depth with Patrick’s GI and dietician here. We’ve always been a bit nervous, considering his history.

Looking at books in the waiting room

Next, Patrick’s transplant nurse came in and took copies of his labs and other medical history that I’d brought with me. Then Dr. Reyes, the transplant surgeon joined us.

Again, he was excited to see how much Patrick has grown. He asked me how well he was eating and pooping since his ostomy was taken down. I explained to him all the questions that had been raised last month about whether or not Patrick had an obstruction that needed to be fixed. Then I told him that some of the doctors wondered if he needed another surgery to try to correct the problem.

Dr. Reyes’ reaction was quite direct. He said “No. We’ll get him a transplant. That will fix the problem.” He didn’t think it was a good idea to mess with things when Patrick is otherwise stable and healthy… especially if that reduces the remaining pieces of intestine.

I asked how Patrick’s reaching 10 kilos in weight would affect his candidacy for a transplant. Dr. Reyes said that that was a really big deal for him. This size changes the rules a bit for what he needs in a donor. Before, we’d been told the donor needed to be the same size as him, preferably smaller. Now that he’s bigger, they can reduce the size of a larger donor, too. His donor could be up to 6 or even 8 years old. The result is that his chances of finding a match go up.

So I had to ask if they could estimate a wait time. The answer, for all who are wondering, is still no. Dr. Reyes was careful to explain to me that Patrick’s B positive blood type is a mixed blessing. It means that there will be fewer matches. However, it also means that there are fewer waiting children with his blood type, which means his priority is higher, even while he’s healthy. Dr. Reyes just kept saying “We’ll get this transplant done.”

Next we talked about liver health. Patrick’s biopsy in September showed some early scarring of his liver. However, doctors responded quickly with a low-lipid diet and for the past several months his bilirubin and liver enzymes and other measurable signs show that his liver is relatively healthy. The clarity of his eyes and skin are also proof of this fact.

I told Dr. Reyes that we’ve been worried that Patrick’s spleen reacts so severely to infection. He admitted that the scarring in the liver was probably contributing to problems with the spleen. Recurring infections don’t help either. However, he said that a large spleen wasn’t as much of a worry if the liver isn’t also large.

Transplants are scary in a patient with a failing liver because as the liver fails, the body stops clotting as well. Dr. Reyes said he’s not worried about that at all with Patrick. He feels safe doing the surgery. Then he said that if you fix the problems with the intestines, the liver can heal, and the spleen will get better. And he told me again, “We’ll get him transplanted.”

I asked one last question. Should we be keeping our bags packed? The answer was a resounding “Yes”. I really need to wrap my mind around that and get things in order so we’ll be ready to go quickly. The regular trips to Seattle and to the hospital here keep me practiced in packing and packing quickly – but still, it would be good to feel in some way prepared.

The mood of Patrick’s appointments was almost celebratory. His good health, his weight gain, and just the fact that we made it to a clinic visit without being admitted were all worthy of celebration.

We’ll go back again in July.

Roughousing with Lindy

The rest of the trip was pretty laid back. Lindy, who was kind enough to drive us half an hour to the appointments and then wait two hours for them to be done, took us back to her house. Patrick and Lauren crashed early. I was amazed that Patrick put himself to sleep there on just the second night.

And then, after a pretty amazing feat of getting three babies (Lindy was babysitting a 4 month old that day) into the car and off to the airport on time to catch our flight home… including all of Patrick’s medical care.. was impressive. Not the smoothest, but we accomplished it.

We got home Friday afternoon exhausted. Patrick and I both went to bed early. We all slept in. And today has been spent mostly in recovering from a pretty intense week.

I can’t really complain, though. It may be exhausting to chase after a one-year-old who crawls around the house emptying drawers and making monster noises… especially when I am the only thing standing between him and many broken lines. But I wouldn’t want to trade having him happy and wiggly and full of life – and best yet, at home – for anything in the world.