Short Gut Syndrome – Page 4

Transplant day 5

First of all, let me say that this was a much better day. Last night, right after I finished posting, the team came in and said that they felt it was time to mix up the pain medicine routine. Immediately, he became more himself. Also, right afterwards, respiratory therapy stopped by to start doing a treatment they call CPT. Unlike the mask that terrified Patrick, CPT is just like a great big massage. It put Patrick right to sleep.

It also told us that it was time for mommy to sleep in Patrick’s room. So, with Patrick asleep from his breathing treatment, I snuggled right up next to him and crashed. With pain under control, his oxygen saturation popped up to almost normal (though with oxygen running). Patrick and I slept snuggled all night. Since he is too sore to roll over and attach, he slept holding onto my ear. (That is sweeter than it sounds.) And by morning, he’d found his voice again. We gave him a bath and put on a new hospital gown.

Physical therapy came by early in the morning and we decided that with him feeling better, it was time to try walking again. They fitted him with a child sized walker and away we went. Patrick was scared at first and just kept crying for mommy to save him. I just got down on my knees and cheered him on and pointed to the next landmark until we got him to where Daddy was waiting to hold him in the chair.

The rest of the morning was quiet. They tried a nebulizer. He hated it. They discontinued those orders (hooray.) I did laundry and made a few more phone calls to Patrick’s doctors and therapists to cancel his appointments and let them know where he’d gone.

And before i knew it, it was almost noon. Time for Brian to leave to catch his flight home. So we walked Patrick back to bed with still some tears, but a stronger body and more courage. Then kissed daddy goodbye and settled in for a nap.

Patrick was in a great mood after nap. I’d decided we needed to do something sitting up in bed to help his lungs clear this afternoon.. So I reached into the big box of trick-or-treat prizes and found a set of paints. Patrick was SO excited! We gathered up a too-large hospital gown and pulled up his table and away he went to work.

The nurse gave him a cup of water to rinse his brush in and he immediately tried to drink it. Patrick is desperate for a drink of water. He was furious when I told him no. (Thanks to steroids and not feeling well, little tantrums are big explosions right now.).. But eventually accepted the little pink mouth moistener that his medical team had approved and his mouth looks and feels so much better.

Patrick’s transplant doctors stopped by to check on him this afternoon. Their jaws about hit the floor as they saw him today compared to yesterday, up in bed and playing. And that’s what he did for most of the rest of the day. We painted. We blew bubbles. We played with a harmonica. Child life sent a medical student to come make putty out of borax and glue. After changing his ostomy bag and taking a short nap, we got Patrick up one more time and walked him to the chair. This time he was pretty quick and made it with no tears. We sat in the chair and called grandma, then watched the new episode of Daniel Tiger, then walked him back to bed where he’s been playing with the cars he got for his birthday happily ever since.

We still had our hard moments. Beginning to understand having his ostomy has Patrick worried. It broke my heart when he apologized to the nurse that she’d had to clean him up when the bag leaked. And the tears over wanting a drink of water are heartbreaking. But these are big, big things that would upset anyone, big or small. And they won’t last forever.

Again, the kindness of family, friends and strangers has astounded us. Gifts and cards arrived at just the right times today. It seemed that in all of the hard moments, something else would show up. Thank you, thank you for your generosity.

Those first steps were scary and hard, but he’s getting better.

He’s figured out this posture is the best way to stay out of the way while the nurses get his lines settled

Patrick was so excited to be able to sit up and do something creative after so many days. I could barely keep up.

Patrick’s Getting a Transplant – Day 1 (and a half)

Last night, as Brian and I were turning off the lights to go to sleep, my cell phone rang in my hand. I looked at the caller ID and my heart skipped a beat. It was the transplant team. It was 10:30 p.m. It took me 2 rings to get the courage to answer.

Patrick’s transplant coordinator asked how his health had been, and then she told me that she was calling because they had received an offer for donor organs for Patrick.

I thought I’d be excited when this call came. I was not. I wanted to shout “NO” and hang up the phone. Patrick has been having a very good year. His health has been good. He is loving kindergarten and for the first time has had friends his age. I’d just helped to a Halloween party in his class. It was a hit and he’d had a great time. We’ve gotten in to one of the best mental health programs in the state and were making good progress with his attention and behavior. We’d been trying a medication for his ADHD and it was a hard adjustment, but it seemed to be helping. He’s been learning to read. And I just planned his birthday cake and finished wrapping his presents.

Did I want to change any of that!? NO!

But at the same time, we know a very hard truth. Patrick has been defying odds as he lives with a terminal illness. There is a reason they let us do a wish trip. Patrick is running out of access. His intestines have been redilating and sooner or later would need surgery again. Patrick has been living on borrowed time.

We have said for 5 years that transplant would come at the right time for Patrick. Why that time happened to be during one of the happiest seasons of his life, I don’t know. BUT it did. And we couldn’t pass up the opportunity. Who knows when it would come again.

Getting ready to go was a chaotic mess. We had bags packed for us, but needed to pack Patrick’s things. We needed to call insurance and the doctors and somehow get to Primary Children’s, then to Life Flight and then to the Nebraska Medical Center. We needed to gather family to say goodbye and get blessings. And we were supposed to do it in 6 hours.

We didn’t do it in 6 hours. I was too confused. The hospital and Life Flight couldn’t agree.. And it took extra time. And it was ok.

Patrick's doctor- with us since we got to Utah, almost as much family as physician — Patrick’s doctor- with us since we got to Utah, almost as much family as physician

We got to the hospital a little after midnight. There, we were met by Patrick’s amazing GI who had come in and stayed up just for us. He wrote orders and then saw us off.

We got to the airport and were met by two amazing nurses and a pilot. Patrick had to ride lying on a gurney, but they managed to make it fun enough that, even though we’d woken him 2 hours after he went to bed (on a day he hadn’t napped), he laughed and played and was ok.

We arrive at the hospital around 6 a.m. and were shown into the PICU. There was some bustle of admission, but things were pretty quiet. Before long, Patrick had snuggled up and fallen asleep. And so did I.

Eventually, they came for some labs. We let him open his birthday present. Then, Child Life came by and asked if he needed a Halloween costume. As I’d left his awesome purple minion costume at home, we borrowed a Buzz Lightyear. It barely fit, but he was happy. And it came just on time, as the Nurse Practioner told us just then to let him up to move around. We made it out just on time to go trick or treating. Each department of the hospital put together mostly non-food goodies. Patrick happily went to each of them saying “Happy Halloween” and being showered with gifts. He came away with quite the haul. We even made the news.

http://launch.newsinc.com/share.html?trackingGroup=69017&siteSection=ndn&videoId=28082677

Trick or treating in a borrowed costume (with accessories)

About 2/3 around the room of trick or treats, we got a call that they wanted Patrick back in the room for a procedure. We made a hurried run back to the room where they explained that they wanted him to go to interventional radiology to try to place another line. That sounded like a request to do the impossible. It turns out it was hard, but possible. Patrick currently has 6 lumens.

Going down early for this meant that Patrick would need to be intubated and sedated earlier than we expected. It wasn’t worth the risk to wake him up again. So I used the little time that we had before sending him down to try to explain to him what would happen. I could tell he didn’t get it. I could tell he was scared.

But it was time to go. So we did all we could to tell him we loved him and help him feel brave, and then we walked him to the procedure room and kissed him goodbye.

He spent the afternoon intubated and asleep. He woke a little once and made a furious fight to take the breathing tube out of his throat. It took several people to keep him safe until they were able to get some more meds to settle him down. Hard, hard moment.

But the rest of the day was peaceful. With him asleep, they were able to get the other catheters and lines in that they will need to be able to monitor and take care of him during and after the surgery.

Finally, around 6 p.m. they came to take him to the O.R. We sat a bit on pins and needles till them, because until the organs arrived here and were inspected, there is always the chance of the transplant not going through.

It’s 9 p.m. now. The last update said that they were finishing putting in the liver and were just about to start putting in the other organs.

It has been a very emotional day. We have shed tears of fear and of hope and of grief and of joy. We have celebrated a birthday and Halloween and then said goodbye to our son for an indefinite amount of time. We have doubted ourselves, and we’ve been given flashes of reminders of faith. We have been touched again and again by the encouraging words of our family and friends and even of strangers.

Once again, we find our lives entirely overturned. I honestly don’t know how we are going to do this. But I am trusting it is going to be ok.

I’d like your help with something, if you don’t mind. Patrick’s birthday celebration got cut short when they took him early to place that line. I’d love for him to wake up to a room full of birthday wishes. Would you consider mailing a birthday card or sending an e-card? Mail to:

Patrick Hoopes
Patient Mail
Room # 5349
P.O. Box 6159
Omaha, NE 68106-0159

or you can send an e-card that will be printed and delivered to him http://www.nebraskamed.com/patients/well-wishers

Mrs. Hoopes Goes to Washington

**Disclaimer: I know you all want to read about our much bigger news. However, I had this post all but written before I got busy with Halloween and birthday planning last week. So, I figured I might as well finish it up and hit post. I promise I am following tonight with other news, too.

Our family did something that, for us, was completely upside down last week. I went on a trip. And I left Patrick home with his Dad. I recognize that this is not unusual in the world we live in. However, it is incredibly unusual for the primary caregiver of a home infusion patient to travel without them is very unusual.

Let’s start by saying that we survived. In fact, I think it was good for all of us.

A few months ago, I was invited to attend a research panel on the subject of lost Central Line Access. This came through a doctor who I had e-mailed a few times because of my support group and later because of the troubles we have had in placing a line in Patrick.

At first, the idea seemed a bit crazy. But the more I thought about it, the more important it sounded. Here a group of experts was gathering to help try to figure out how to research and solve the exact problem that is putting my son’s life in jeopardy. Why wouldn’t I take the chance to go meet them and contribute to the discussion.

Well, why, except that we’d have to pay my way and have Brian take time off to fill in with me while I was away. As we discussed it, we decided it was a really good idea for me to try to go. (Brian also thought the break would be good for me.)

So, last Sunday I snuck out of church early and headed to the airport. I got picked for expedited screening at the airport and made it to my gate in under 30 minutes. Then I looked around and thought “Well, how what am I supposed to do with myself?” Remember that usually when I fly, we spend something around half an hour in security alone, and then we have to repack bags, change a diaper, gate check our stroller, and make arrangements for early boarding. Oh, entertain a very active child while keeping his IV tubing safe.

That Sunday was one of the longest and quietest in my memory. I read books, watched movies, played video games, sent e-mails, checked on Facebook, worked on a birthday video and still had time to spare. I finally made it to my room, called home, and then went to bed a little after midnight.

Morning seemed to come too early, but without anyone else to get ready, I was still dressed and ready with time to kill.

The panel itself was amazing. I tried taking notes, decided writing was too slow, switched to a computer and filled 12 pages. I learned so much about what might cause patients to lose central line access. I am itching for this panel to bring forth fruit in the form of better understanding of how to keep patients from losing central line access quickly the way that Patrick did. I can’t go into details about the research plan that was decided upon.. but I can say that it is a huge but simple step that could lead to so much better understanding, and with better understanding comes better care.

It was also an amazing experience to be in a room filled with these brilliant medical minds. I got to sit with and get to know better many of the people responsible for designing and manufacturing central lines. I came home with a stack of business cards and am already working on hopefully becoming a little bit more of a patient advocate in this area.

And then, when the conference ended, I took a cab into Washington D.C. I spent the evening and morning playing tourist. (Only getting myself lost a couple of times and never irreversibly) before time to come home.

On the flight home, I sat next to a talkative man who had been all over the world. We compared travels and experiences and opinions. When we landed, another man asked me what my career was that allowed me to be so well traveled. When I told him I was a stay at home mom of a special needs child his jaw about hit the floor. For some reason, that gave me a bit of an ego boost. I think I sometimes feel like I disappear a bit in this important but unlauded role.

Being away from Patrick and Brian was an eye opening experience. I think it was for them as well. I think we all like our roles. (Though, I’ll admit, it was hard to come back home and dive right in.) I think we can go several more years before sending Mom away on another big trip. But it was really fun to stretch my wings and remember what it’s like to be just me for a while.

Summer vacation

Tomorrow marks 3 weeks since Patrick’s last day of school. For most people, summer vacation is in full swing. For us, we’re finally starting to get our bearings. It’s been an unusual start to summer.

Things seemed smooth enough. I spent that first weekend after school ended gathering myself to start a tradition Patrick and I are calling “Mommy School.” Between therapies, Patrick has a LOT of “homework”. He’s supposed to spend 10 minutes every day doing eye exercises. He’s supposed to be practicing writing and cutting and and gluing and using a keyboard. We’re supposed to be strengthening his core, working on activities that use both sides of his body evenly, and encouraging him to cross midline. He’s supposed to be practicing telling me stories in various verb tenses, using articles, and correcting his use of pronouns. Plus working on vocabulary building, sorting skills, categorization skills, social skills, attention skills and on and on. All of this to help keep him progressing on his current trajectory which, discouraging as I may find it, is approximately two years behind most of his peers.

Except, it seems, when it comes to the alphabet. Patrick has the alphabet down pat and tested in the top 25% of his preschool class.

Our first day of Mommy School. Learning the shapes of letters by building them from playdough.

A practical life mommy school lesson on how to sweep.

And so, this summer we introduced “Mommy School.” We’re doing an abbreviated letter of the day curriculum, since letters are what Patrick loves. Every week is assigned two letters and every day a word that starts with that letter. Then I go out and scrounge up books, worksheets, crafts, activities and field trips on that theme.

Teddy Bears — B is for Bears, and a teddy bear picnic.

The first day of Mommy School went GREAT! Until, that is, Patrick developed a fever. The next day of summer break was spent cuddling a sick, feverish kid in a chair. He had a cold and we were grateful to be able to stay home sick like other children. By Thursday, Patrick had mustered the energy to play on his own again. But, by then, I was sick.

Patrick sees 3 therapists on a regular basis in the summer. It's hard work, but fun too. Patrick's reward for a good hour of work in physical therapy is the chance to climb the rock wall. — Patrick sees 3 therapists on a regular basis in the summer. It’s hard work, but fun too. Patrick’s reward for a good hour of work in physical therapy is the chance to climb the rock wall.

In occupational therapy, Patrick gets to take sensory breaks in the gym. Diving in the ball pool is a favorite exercise.

Our second week of summer break, Patrick and I were finally starting to be healthy again. But Brian wasn’t. And it didn’t matter if we were healthy or not because on Saturday of that week, my little sister was getting married in my backyard. So, I declared “C is for Camping” and we pitched a tent in the living room. Amazingly, this both occupied Patrick and kept the room clean while he binged on movies and we scrambled to finish last minute details on the house and yard.

I meant to pitch a tent outdoors, but it rained all that day, so we brought it inside. It worked so well we kept it a second day, too.

We also sneaked in a couple of outings.. a family night at Red Butte Garden and a field trip with friends to the Natural History Museum. (I picked up a free month-long museum pass at the local library just so we wouldn’t have excuses to just stay home and work.) Besides, in order to get Patrick to let us work, we let him watch his birthday movies on endless loop, which left us all craving some family adventures.

Patrick loved the children's garden best, especially hopping on stepping stones across the fish pond. — Patrick loved the children’s garden best, especially hopping on stepping stones across the fish pond.

The best part of our trip to the Natural History Museum was not the dinosaur bones. It was the chance to be there with other friends who know exactly what living with Short Gut is like.

The wedding went smoother than expected, though playing hostess and trying to keep Patrick from completely melting down about how his house was filled with children who were not sharing his toys with him kept me busy enough that I didn’t manage to take a single picture.

I was too busy to take pictures, but there were lots of cameras there. I snagged this one off my mom's Facebook page. — I was too busy to take pictures, but there were lots of cameras there. I snagged this one off my mom’s Facebook page.

My sister was a beautiful bride and the happy couple truly looked happy and in love. Patrick eventually settled down with Grandma who let him fetch endless cups of water from the refreshment table and pour them all over himself and her and all went well enough.

Except that it turns out that maybe the bug that had Brian sick isn’t the one that had Patrick sick as I ran a fever all night.

So, Sunday we took a sick day and we stayed and home and did absolutely nothing except that Brian cooked us a delicious roast. The day was delightful and refreshing. Perfect after two frantic weeks.

Yesterday, I put together a week’s worth of Mommy School worksheets, made a chore chart, got Patrick’s TPN labs drawn and kind of regrouped. Then, in the late afternoon, I changed Patrick’s central line dressing and discovered that, much to my dismay, what had once been a tiny little hole in the clotted side of the line had grown big enough that I was worried it would pull off. But it was after hours and I wanted the head of the IV team to be involved in choices about the repair.

So today, we spent our kind of restart to summer day exactly as summer days seem to go for us.

Over breakfast, I made a bunch of phone calls to people at the hospital trying to find the best way to get the line repaired. Eventually, I sat down and had a little cry because I wasn’t getting in touch with the people I wanted and I knew we’d have to go to the E.R.

Then, Howie gave me a pep talk and a hug and a kiss. Then he talked to Patrick and helped him to gather the courage to get his line repaired. (He was terrified that it was going to hurt when they cut the broken part off of his line.) We headed up to the E.R. where our timing really was perfect. We didn’t wait at all for them to start working. Patrick’s line is a different brand than our hospital stocks because it was placed in Nebraska, so that complicated things and the repair took about an hour and looks a bit sloppy. But it will do for a clotted line.

And then, when all was done, we headed off to the Children’s Museum for a couple of hours. We managed to get there at a nice slow time on a nice slow day. Patrick actually got to enjoy exploring the museum and would have happily stayed there all day. His favorite spots were the Bob the Builder exhibit, the gas station, and the pretend E.R.

I didn't know they'd built on a pretend E.R. with the life flight display. Patrick found a little girl willing to play the patient and had a great time — I didn’t know they’d built on a pretend E.R. with the life flight display. Patrick found a little girl willing to play the patient and had a great time

Eventually, we had to go because I was parked in a 2 hour parking space and, honestly, we both needed a rest. So we stopped in at daddy’s office to pick up the leftovers from a lunch he’d cooked for them today. And then we came home and convinced Patrick to nap by 4.

Which brings us to right now. Brian just walked in the door to a quiet, clean house and me blogging and said, “Now that’s a sight I don’t see very often.” Here’s to maybe a little bit more of this? Perhaps a few prayers for this line to hold out through the summer and grant us a little bit of peace ..and maybe even health?

How the Hoopes Family does bedrest

In addition to the 3 hours of totally immobilized bedrest, Patrick’s doctor ordered that he stick to light activity for the next couple of days just to be sure that none of the little holes they put into major veins started bleeding. Patrick does not do bedrest.

So instead, this is what we did to keep him as still as possible.

First, we went out to breakfast. Then, we got in our car and we drove, taking the longest scenic route Google maps would provide, to Lincoln, Nebraska, the state capital. We stopped and walked around the capital building.

And then we visited the Lincoln Children’s Zoo, which is, in essence a big petting zoo.

He experienced a hedgehog's quills. — He experienced a hedgehog’s quills.

Patrick got to ride a pony named Tinkerbell.

We also fed goats, petted a llama, watched the penguins feeding, rode the zoo train, shared snacks with a peacock, and of course, fed ice cream cones filled with feed to camels.

This is the wrong way to feed a camel an ice cream cone. Brian is pouring feed down the little pipe holder they gave him. The camel didn't mind much. — Put an ice cream cone on a pipe, hold it out, and the camel grabs it.

Then, we drove back taking the fastest route we could find so Patrick wouldn’t fall asleep in the car. And when we got back to the Ronald McDonald House, we snuggled down and napped until dinner time.

When we woke up, Patrick took advantage of the first break from tubes he’d been allowed in a week and played on the house playground. He didn’t mind that it was almost 100 degrees and over 80% humidity.

And then we went for a stroll on the riverwalk.

Not a bad day of recovery by any account. The next day we spent flying home. Booking last minute meant having to book Patrick’s first layover. A shame because he squeezed in his nap on the short flight and was exhausted and overstimulated through the second. But we finally made it home safe and sound Sunday evening.

Getting a line in and back on the transplant list

Sorry to have kept you waiting for updates. As you’ll see from this post, it’s been a crazy few days.

First of all, if you haven’t heard word any other way, the doctors in Nebraska were able to get a new central line into the Superior Vena Cava and Patrick is back on the transplant list. I mean to post updates but didn’t expect them to order bed rest and minimal activity for the next days after the procedure and, well, accomplishing that is kind of a full time job where Patrick is concerned.

We checked in to pre-op at 5:30 a.m. which is 4:30 a.m. Mountain Time and we were all incredibly sleepy. Pre-op was the usual flurry of activity as we met anesthesiologists and got fluids ordered and labs drawn. (They had an amazing phlebotemist who managed to draw blood without Patrick even crying and on her first attempt.) We met the OR nurse whom Patrick loved so much he said she was “Kinda Jo”, in other words, put him at ease like his favorite child life specialist. He collected teddy bears and various and sundry medical equipment to use on the bears and charmed everyone in sight.

Then we got the meet the doctor and go over the plan.

The procedure it took to get us there absolutely terrifies me. I order to get around the blockages, they put a guidewire in through Patrick’s femoral vein and ran it up to his heart where they repositioned the veins in order to reach the right ones and get around the clotting and scarring. At least, that’s what I understood. He followed along with x-ray and ultrasound the entire way to make sure things went where they should.

What I really gathered from the description of the procedure is that it was insanely dangerous. As the interventional radiologist was going over the risks with us, I just kind of had to shut off the part of my brain that could process what those risks might mean and remind myself that without it, he had no long-term chance of survival. Instead, I honed in on the fact that he sounded confident in what he was doing and that, well, it just felt right. I couldn’t help but think that THIS was the reason we’d been impressed to transfer Patrick’s care to the University of Nebraska. Because they had doctors who had developed this technique to save access and save lives.

Knowing how difficult Patrick’s access can be, we all kind of hunkered down expecting them to need hours. So, when we looked up at the status board after just a couple of hours and see that they were closing. Then, sure enough, there was the doctor telling us that everything had gone well and that he had a new line in place.

As we understand it, this is a rare direct superior vena cava line, entering the vein directly after going through soft tissue. (Patrick’s GI is questioning this, so I’m getting a copy of the procedure notes so I can go back over what was done and we can understand it.)

He went ahead and put in another double lumen line for us. Because they stock a different brand, the line they had was rather large, a 9 french, but that could kind of be a good thing. Should be less prone to clotting problems.

He also us that this line is to, under no circumstances, be taken out without his approval. If Patrick gets and infection, if the line breaks, if it clots, whatever… the team at the University of Nebraska needs to get involved. Maybe they will teach the team here how to keep the site open. Or, maybe they will fly us back out again. Whatever happens, they do not want to have to attempt to repeat this procedure. We were told that, next to transplant, this is one of the riskier and more specialized things that they do at their hospital.

Whew!

And by the end of the day, Patrick was back active on the transplant list.

The rest of the day was kind of crazy. Because of the risks of bleeding from that femoral access, Patrick was ordered to 3 hours of total bed rest with that leg completely immobilized. Knowing our child, we quickly agreed to request sedation for that.

At first, the sedation made things a little easier as we scrambled to attached fluids to the new line to keep it open. We also called to ask that his old line be removed while he was sedated (they’d left it in thinking it was still needed, but decided we didn’t need that to be done at home.)… and then scrambling to round up information about the new, different brand of line and repair kits, etc.

Patrick started to wake up just a little and was, well, cuddly. That’s normal for him, but normally I’m also allowed to hold him. This time, he had to stay in bed. So he settled for holding onto my head. For half an hour, he held my head as tightly as he possibly could. (Meanwhile, the doctor came back to pull the old line.)

Patrick making his teddy bear play tablet while on forced, sedated bed rest

About an hour in, the nurse decided to stop giving him sedation and just let what was in his system and me distract him. So, we played games on the tablet and we checked the blood pressure of the teddy bear and tried out a Nintendo DS. Meanwhile, Brian went back to the Ronald McDonald House to bring back TPN and to try to meet our delivery of medical supplies.

2 hours in, the doctor said that Patrick could sit up in bed and make sure his leg was ok with the extra movement. The post-anesthesia nurse was kind of tired of him tying up the space in PACU I think (they could only keep giving him sedation there) and so she discharged us to their extended care unit.

The extended care nurse acted as though she hadn’t received report from the PACU nurse, though. When we got there, she tried to insist that Patrick needed to stay lying still in bed for another hour because that’s what the orders in the computer said. And so, well, yes, I went a little angry mama bear on her and told her that I would not have agreed to come to her unit had those been the instructions.

And then we spent the next half an hour trying to keep Patrick still sitting up in bed, doing crafts, sipping water, and not chewing on or removing the COMPLETELY inappropriate non-pediatric pulse oximeter on his finger.

And then, I don’t know who talked to her, but the nurse came back in and cheerfully told us we could get Patrick dressed and he could be discharged. Like the nurse who I’d gone crazy on didn’t even exist.

Anyway, we were grateful for the dismissal as we had managed to squeeze in an appointment with one of the transplant surgeons at 1 p.m. and 1 p.m. is when Patrick was supposed to be cleared after 3 hours’ bed rest.

So, we hurried over to the Intestinal Rehab Clinic and checked in for our visit and a little while later, Dr. Langnas joined us. We explained to him our concerns about whether or not waiting for a combined liver/intestine transplant might be increasing Patrick’s wait time. He listened to our concerns and then explained the benefits that he sees for Patrick in the liver listing. Then he promised us a future date where, if Patrick has not received a transplant, he will take our concerns back to the transplant review board for reconsideration.

The rest of the day we spent trying to help keep Patrick down. We went back to the Ronald McDonald house again for a little back and let Patrick play in the playroom. Then, in the interest of some forced holding still, took a drive instead. Patrick heard us mention the temple as a possible destination and voted for that. So, with our bruised and bandaged and druggy little boy, we went and visited the Mormon Trail Center and Winter Quarters Temple grounds. Then came back on time for a yummy lasagna dinner courtesy some generous Ronny House graduates, then went to bed early and exhausted.

Playing pioneer at the Mormon Trail Center

After tucking Patrick into bed, it hit me just how serious what he’d been through that day and week really was. Another case of him surviving against the odds, an unquestionable miracle. And a VERY close call. I shed a few tears of fear and relief that night, said a prayer of gratitude, and went to bed.

Mission Impossible

I probably have this idea in my head because we watched a Mission Impossible movie a couple of nights ago. But I swear that today, as I ran from one major problem to another and felt myself being powered by pure adrenaline, that my life is no less demanding than a Mission Impossible mission. Maybe a little more dull and much more sedentary.

Here’s why today had me thinking that. I stayed up till a little after 11 last night finishing off Patrick’s care notebooks, a 30 page medical history and emergency plan for Patrick’s school staff. I was woken 4 times during the night, twice by IV pumps alarming. Twice by Patrick’s mylar balloon drifting into the ceiling fan in the kitchen.

At 7:30, Patrick woke up and I tried really hard to explain that we had a busy morning and needed to get ready quickly. After a summer of lazy, he did his best but we were definitely out of practice.

We finally made it downstairs and I scrambled to put finishing touches on school supplies. That doesn’t mean pencils and papers for Patrick. It means putting together a medical supply emergency kits, diaper changing kits (with instructive labels on each bottle of cream), care notebooks, first aid response cards (miniature and laminated to fit in Patrick’s backpack), and allergy safe labels on boxes and bags of snacks.

Patrick’s school open house started at 9:30 and we got there a little after 10. I felt really bad for coming so late.

BUT we had a chance to meet some other parents and kids and explore the classroom a bit. As we were nearing the end of the open house, I had a chance to meet the speech therapist and special education teacher who’ll be working with Patrick this year. I kind of tried to make a mad scramble in my tired brain to remember the relevant information I wanted to discuss with them about his goals. I think we covered the main points and I was impressed that they seemed to be on the same page as me. Then I went over with the teacher and classroom aides a refresher course on his medical care and diaper care and what ADHD and sensory processing disorder mean for him. And, of course, how and who to reach in an emergency.

I left the classroom half an hour after the open house was due to end. We walked Patrick’s medical supplies down to the school nurses’ office, along with a copy of the emergency plan, and briefly went over their questions.

Then I pulled out my phone and noticed that I had missed phone calls. Lots and lots of phone calls.

On the drive to the school, I’d called Patrick’s dietitian to tell her that we can’t get blood to draw off of his new line right now and ask if she really needed any labwork done today. (A nurse visit popped up in the schedule yesterday.)

That call prompted her to call Patrick’s GI, Dr. Jackson, who’d spent the morning bringing himself up to speed on Patrick’s new line and being put on hold for transplant. And he was quite concerned.

Oh, and I’d missed the call back from the transplant nurse in Nebraska.

So, when I got in the car, I called back Dr. Jackson. He apparently spent the morning going over operative notes and talking to the radiologists and other surgeons. And he’d learned something about Patrick’s new line that was alarming.

We had misunderstood what we’d been told about the placement of the line. The azygus vein is not a central vein. That means, it doesn’t directly connect to the heart. The tip of the new line is in a dilated part of that vein. But, after the tip there are some collateral (spiderlike veins that grow around a clot to reroute bloodflow like the little streams that form around the sides of a river if it is partially blocked.) And it is those that are connecting to the main veins and to the heart.

And since those veins are small and could infiltrate just like a peripheral IV vein, (or swell and close off that access, too) Dr. Jackson wanted Patrick off of his TPN ASAP.

Because of Patrick’s low lipid protocol, there is a lot of sugar in his TPN. It’s a very high osmolarity formula that kind of rips up small veins. So tonight we got a shipment of a lower osmolarity, lower sugar formula to run until we get a resolution.

Dr. Jackson also contacted the nurses and doctors in Nebraska on our behalf.

Anyway, I spent the drive home talking to him, then brought Brian up to speed, grabbed a quick lunch then called back the Nebraska Medical Center. They asked me to fedex them a CD of all of the imaging done of Patrick’s vein in the recent past and e-mail them all the radiology reports I had.

By then, Patrick was pretty tired and pretty tired of me on the phone, so I tried to rock him to sleep for his nap. But the phone rang. A homecare nurse seeing if I needed my TPN pump reprogrammed. Then it rang again. The homecare pharmacist setting up a shipment of the new formula fluid.

By this point, I texted my sister. I could tell I was in over my head and needed more time than I had.

She drove over while I got Patrick down for a nap, then stayed with him while he slept. Meanwhile, I scanned all my radiology reports. Then I drove to the hospital to pick up the CD of radiology images that Dr. Jackson had requested on my behalf, stop in medical records for accompanying reports, and then down to the pharmacy for a prescription for ranitidine to replace the IV form Patrick usually gets in his TPN. Of course, on the drive up talking to the homecare pharmacy to order tubing and other supplies to go with the IV fluid. And, in the waiting room, e-mailing the nurse in Nebraska to decide that they wanted their own venogram done anyway and that they could schedule Patrick’s procedure without me sending a CD after all.

By now it was 3 p.m. and I was feeling a bit like my mind was doing stunts Tom Cruise could only dream of. I was exhausted with trying to change gears and think of entirely new life-critical details. Fortunately, 3 p.m. is 4 p.m. in Nebraska and close of business for the intestinal transplant office.

So, when I got home, I just had to make dinner and clean up a little bit until evening.

Dr. Jackson called this evening and we had a good conversation about where Patrick is and where things are going. The best phrase of the conversation was when he told me that he thought that the doctors in Nebraska are just smart and daring enough to be able to “Macgyver something” to keep Patrick listed if possible.

But we also had a good talk about where else central lines can go and how to reduce and treat clotting in veins and genetic predispositions and a whole bunch of other crazy things, kind of like Dr. Jackson and I like to do. Is it strange that he and I kind of enjoy talking over medical problems together?

The encouraging thing to me is that, although he called our situation “sobering”, I could hear in his voice that he has a lot of hope still.

And that he’s pushing to get things done, and quickly. I think he said he’d e-mailed the surgeon twice and had sent a copy of all of the radiology reports that he was able to send by e-mail to his nurse.

This evening, we received the delivery of new fluid and got it started. (The sad thing about this change is that, with fewer calories, Patrick can’t afford a tubes-free time every day. The good thing is, the bags are split into two per day so they weigh less and he’ll be able to wear his pack.).. After connecting his fluids for the night, we picked out clothes for school, put on PJ’s, and tucked Patrick into bed.

I’m hoping for a little more calm tomorrow. I’m happy Patrick doesn’t miss the first day back at preschool. And that, amazingly, we pulled that all together in the midst of all of the rest of this madness.

And maybe I can get phone calls done while he’s at school. Maybe even a plan for how to get out to Omaha. Maybe.

Or maybe, if they’re not ready for me yet, I’ll just go sit on the lawn at the park next door and read a book and delight in the knowledge that at 10:15 every Monday-Thursday, Patrick gets to go visit the sensory room. And all his favorite aides from his old class get to come into his new class for morning circle time. And some of his familiar friends are still there.

I think I had enough adrenaline for one day today. Heck, I’ve had enough this month to get me by for a year.

Looks like we need another miracle after all

Patrick insisted I take this picture of him eating an applesauce cookie. So good to see him with an appetite again.

I truly feel like getting Patrick a line through his interior jugular vein into his azygus vein was a miracle. An absolute answer to prayer.

So I was more than a little confused with the phone call I received from Patrick’s transplant nurse coordinator yesterday. In the morning when we talked, she was quite pleased with the ingenuity of Patrick’s line placement and thrilled to hear he was doing well. We discussed infection prevention strategies and his position on the transplant list.

Then she called me back in the afternoon. She’d updated one of the transplant surgeons. And, as it turns out, in order to perform an intestinal transplant surgery, you have to have a central line in the superior vena cava. It can’t be in the azygus vein. The logistics don’t work.

They asked us to fly Patrick there next week to let their specialists see if they can solve the problem. Until it is resolved, Patrick has been put on hold on the transplant list.

At this point, other than knowing that the insurance company has approved the trip, that is all we know. We have about three dozen questions that we don’t know the answers to.

Despite the news, Patrick is doing great. Infection free and bacterial overgrowth at a minimum, he is feeling great. He ate at all three meals and had several snacks today, too. I swear he was eating once an hour. He’s napping well. He’s playing happily. He is very confused about why mommy is in a bad mood.

Mommy is doing her best not to be overcome by her tendency towards catastrophic thinking.

At least I have plenty of distractions. Hospital stay followed by a week of travel followed by another hospital stay is not the way to stay on top of your housework. And I’m nowhere near ready for Patrick to go back to school, even, or perhaps especially, if we might miss the first day next week.

We are hoping to get some answers soon.

Thank you for your prayers. I know they’re helping. If you don’t mind, we could really still use them for a little while.

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.

Losing the line we were told never to lose

Or perhaps I should title this post “A date with the devil.” We are being forced to get to know the devil we don’t know.

Believe it or not, we finished a course of antibiotics and we went on vacation. Brian went on a business trip to the Ukraine. Patrick and I went to visit our good friends, the Laylands, in Seattle.

But writing about those things will end up waiting for another day. Because I’m writing tonight from a hospital room. Again. And there are things on my mind that, for me, have been the subject of nightmares for many months now.

Patrick has lost his central line. Not entirely yet. But it will have to be replaced.

It all started Saturday night right after I put Patrick to bed after our first day home from Washington and went to clean up before Brian returned from Ukraine. Patrick had been in bed about half an hour when I heard a plaintive “Mommy”, and then that unmistakable, heart stopping sound of him throwing up in bed.

I ran up the stairs but was too late. The poor little guy threw up half a liter of nasty green stuff. He must have felt awful all day. It took an hour to settle him enough to sleep.

Saturday morning, a homecare nurse had come to draw some labwork and follow up blood cultures. All seemed well at that point.. But i was very glad for those cultures when Sunday morning, Patrick was having low grade fevers. We went to church just to be able to take the Sacrament and see how long we could make it. We had a backup plan for our assignments at church.

And it wasn’t long before it was obvious Patrick didn’t feel well enough to sit quietly. So I took him to the foyer to let him move and I got on the phone with his doctor to see if we could come get more bloodwork done. And I pulled out a thermometer, and there was the number we had waited all morning for. 100.4 degrees farenheit. And official fever.

So, we made a bit of a scene leaving church, packed bags, and came to the hospital.

It took several hours to restart Patrick’s antibiotics. 8 for the one I thought he needed most, which just about made me crazy waiting for as he got feeling sicker and sicker. But as soon as they were given (along with a Priesthood blessing), Patrick started to feel a little better. We even slept through that first night.

All seemed to be going smoothly by the end of the 2nd day. I was a rockstar advocate mommy and got Patrick therapy, a picture schedule, integrative medicine & aromatherapy, and even talked to the palliative care team about signing him up for their services. I even managed to get his GI, surgeon, and a radiologist to have a care conference to discuss replacing Patrick’s positional line. They came back in favor of a change and suggested a plan of action to decide if and how to proceed.

And then, at 4 p.m., we came back to the room to draw cultures and start his antibiotics and his line wouldn’t draw. Or flush.

We ordered TPA and we worked it, and we worked it… and, well, after 4 hours of effort, he was exhausted, I was heartsick, and the line was still not working. And the plan from the care conference was upgraded from a hypothetical, optional plan to a necessity.

I did not sleep as well last night. (And not just because the nurse was slow about keeping the pumps from alarming and banged the door.)

Today we put the plan into action. Patrick went to radiology for a sedated ultrasound of the veins in his upper body. The order was for light sedation.

We learned today that Patrick is very capable of fighting light sedation. Made him silly and sleepy, but he was still perfectly able to fight the test. Moderate sedation didn’t fix things either. In fact, with sedation all of his inhibition control was gone and none of my usual distraction tricks were even an option. The ultrasound was wiggly, to say the least.. but the technician was patient and kept trying to get the information the doctors wanted.

Listening to her review her images with the radiologist, though, made my heartsick. As usual, Patrick’s right side vessels are fairly scarred. (Though perhaps a little better looking than the last I’d known.) But, they worked enough to give a better answer about the left side than before. Instead of just saying “Inconclusive”, what they said was that the left subclavian vein (the one in his shoulder) has lots of little collateral vessels around it too and shows evidence of being swollen at least, if not scarred, and also probably has a clot in it.

I asked the radiologist if that would explain the tricky behavior of this line for the past year and he said absolutely, that the vessel may have been pressing on the line. He also said that changing out the line over a wire was a risky move, given the clot in the line. But that I’d need to discuss that plan with our surgeons.

Other vessels were open, but most of them looked to have some kind of narrowing.

The rest of the afternoon was a little crazy. I got permission to change out Patrick’s button while he was still sedated and that went quickly and smoothly and he doesn’t seem to understand it even happened. That’s what we hoped for.

And that meant that Brian came up for the ultrasound and stayed the afternoon.

But it was a rough afternoon. Patrick didn’t sleep under sedation and he didn’t sleep after, either. He fought REALLY hard all afternoon to stay awake. We could tell when it started to wear off because he finally stopped fighting.

But because of low heart rates, he still had to stay in bed till 5:30. We got out for a short walk, but when we came back for TPN at 6, and I asked the nurse if all was done so we could leave, she told me no. She didn’t want to use the automatic settings to taper Patrick’s TPN levels up (to protect his blood sugar levels)… and so, we couldn’t leave the room.

The idea of making Patrick stay any longer in the room with the same old toys and bed and short IV tubing kind of broke my heart. So I had a very short little cry about it. Then I went and helped myself to several fitted sheets and used the extra safety pins around the room to build us a fort.

That tided us over for the rest of the evening.

Tomorrow will not be an easier day. As of tonight, they plan to take Patrick to surgery tomorrow. The surgeon will probably attempt first to put a line back into the same vein. That means that he’ll put a wire into the catheter, remove the old line leaving the wire, and then use the wire to guide a new line in.

That is the best case scenario.

The worst case scenario is that that won’t work. In that case, they want to repeat a venogram.. which is a contrast study of the Patrick’s veins. And they will use that to pick a new vein to put a line into.

It is entirely possible that the surgeon will come in in the morning and decide that he wants to have that done before Patrick goes to the OR. And then we’ll just do that tomorrow and the surgery the next day.

And the worst of worse case scenario is that the wire over doesn’t work, none of the other vessels are still wide enough to get a central line into, and then we’ll start discussing desperate measure that I don’t really want to get into in a blog post unless they happen.

Needless to say, this is scary. I am still terrified of the idea of Patrick no longer having veins to give nutrition through. I do not want to imagine that happening.

We are not to that point yet. There are veins in other places in the body that can be used. But there is still a lot of pressure to make one of these sites work because in order to have a transplant, Patrick has a have a central line in his upper body.

I’m scared and I’m sad.. and I’m really, really lonely for some reason this stay. But I’m calm. We have been praying and fasting about this line for a while now. I can’t help but think that this is an answer to those prayers.

So, if you’re the praying type, please join us in praying for the surgeon and his team and the radiologists tomorrow. We’d love to save this access site. We’d love to get another year and a half or more out of another. And I have this faint little hope that maybe the next line won’t scare me every time someone else flushes it, (and sometimes when I do, too.)