trials and triumphs – Page 3

Transplant day 4

Today was a hard day for Patrick. It seemed to start out ok. His nurse overnight did a great job keeping him comfortable. At 6 he was well rested and talkative, though a little sad. His nose was itchy and when he rubbed it, the cannula in his nose rubbed just enough to make it bleed. His oxygen saturation was low, so we had to suction his nose, too, which is pretty awful for him. But, with Daddy cuddling, all was good for a few hours. Until they wanted him up to walk. That hurt.

When he got to the chair (about 2 feet away), he was sore and tired, but in good enough spirits that he dared work himself down into his favorite snuggly sleep position. That’s when the trouble started. Snuggling down made his oxygen saturation dip even lower. Soon, his nurse came back to suction his nose, really thoroughly this time. He was hysterical. And, it didn’t help.

So they decided to call for a chest x-ray and his nurse had him walk back to his bed for it. He was already sore from fighting the suction and walking was agony. But when he was done walking, he still had to sit up straight on the bed and have an x-ray taken. And I had to step out of the room while they did it.

Unfortunately, that x-ray showed that Patrick has gunk in his lungs and fluid buildup around his lungs. (They run a ton of extra fluid in the first couple of days after a transplant to make sure the veins stay open and happy so the graft will take… All that fluid has been just sitting in Patrick’s tissues and he was rather plump and sore this morning.)

They’d already started him on a medication to help him get rid of the fluid. They decided to order some medicine to help him cough. Patrick is refusing to cough. So they gave something IV and then brought a mask to administer a breathing treatment with.

That was the end. Patrick didn’t want a mask on his face. (I’m sure he’s seen enough of them in the OR.) He toughed it out the first few 10 second tries I did for him… But then he decided to fight… And when I tried to help hold him because he was fighting… Well, he lost it. Kicking, screaming, squirming, fighting with all his strength. This is amazing considering how little strength he has. But it surely left him worn out.

The breathing treatment didn’t work. They had to increase his oxygen several times to get things stable. Patrick was spent. He just sat there, not moving, not talking. I finally told the doctor that this wasn’t like him. We were going backward if he’d lost his words again.

They listened. They let him rest. They added some pain medications. They ordered a different kind of breathing treatment that just gently massages his sides to break up the junk in his lungs. And he has done better. He’s seeming calm. He’s slept a bit. His vital signs look a little bit better. And I’ve learned my lesson about letting them push him. Patrick is not one to be pushed. Challenged, yes.. But not compelled. I won’t let tomorrow go this way.

The hardest thing about the day is how just plain sad Patrick was. Because it was Monday, we had a parade of people in and out all day introducing themselves: social work, ostomy team. nurse coordinators, child life, and on, and on. And all the while Patrick just sat there looking like he wanted to cry but with a firm determination he wasn’t going to cry in front of anyone. And anytime I tried to talk to him about it, either his oxygen saturation would drop or someone new would come in.

He’s figured out he has an ostomy. (Where the intestine is brought outside the abdomen in one spot and drains into a bag.) He needs this so they can monitor for rejection for a year. I’m sure he’s confused. But anytime I’d try to help explain, there was one more person. Always trying to cheer him up. Some days, you don’t want to be cheered up. Some days you need to cry.

I think the treatment plan is better now. I’ll spend the night in the room tonight to keep an eye on things and to help him feel comforted. Tomorrow is another day. (And hopefully a good one, as tomorrow Brian goes home to get what Patrick and I need to stay here for a while and I’m gonna be on my own.)

We did have some good things in the day. Brian made it to Walmart which means we have food and socks (only I packed socks in the hurry to leave) and slippers for Patrick and some other needs. We got a lot of answers to questions we’ve had from the people who came by and called. I got time to get some of the e-mails and phone calls taken care of to tie up loose ends at home.

And in a very special bright spot, Patrick received 35 e-cards today. How amazing it was to read words of encouragement and support from friends and family and also from people we have never before met. I’ll admit, that is what got me through the moment today when my heart was breaking.

Thank you. Thank you!

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

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?

The end of another school year

Patrick woke up crying this morning at 6. Not just a little “I’m sleepy” cry. Out and out sobbing. When I asked him what was wrong, he replied “Push!!” “Push what?” I asked. “Push TAMMY!” He answered.

And I understood exactly what he meant.

“Push Tammy” is code for “I fought with my classmates at school again.” This time it was a dream. But it hits on a really real fear and a very big problem that I’ve been so baffled and busy with that it’s kept me away from this blog for quite some time.

This has been a hard school year for Patrick. My sweet, friendly, optimistic little boy has felt stressed and discouraged and just plain rejected. It was bound to happen one day that his medical issues and his developmental delays and his just-plain-awkwardness would catch up to him and cuteness wouldn’t carry him anymore. At least it started in his last year of preschool with a teacher who knows him and is OH SO patient and willing to adapt and help.

Patrick’s frustrations have manifested themselves in a lot of hitting, pushing, and kicking. Usually it’s subtle and small. Sometimes it’s angry. Some would say we’re lucky that he’s perfecting the art of the tantrum this late in life. The problem is that at age 5, it’s a whole lot more frowned upon. And it certainly is costing him friendships.

So, if you’re wondering what I’ve been doing since I wrote about the IEP meeting last fall.. I’ve been volunteering in Patrick’s classroom, advocating for a behavior plan and better support for him at school, trying to find a good psychologist and then trying to implement what she’s trying to teach me to do.

I’ve also been being humbled a lot as I realize how far I need to go still in developing patience, self-control, long-suffering, flexibility.. and on and on…

Oh, and watching a lot of Mister Rogers and Daniel Tiger’s Neighborhood. Because they teach lessons both Patrick and I need to learn.

Now – why am I telling you this? Well, because I am really hoping that I can spend the summer blogging about how Patrick and I are having wonderfully fun adventures together as we explore the alphabet. Patrick’s got just a few days left in preschool.

So if I tell you now about this struggle, maybe I’ll stop worrying about how to tell you about this struggle and can get on with writing about our lives, imperfections and all.

The rest of today didn’t exactly pan out much better. Patrick’s nap got delayed because of our kitchen remodel (subject for another post soon).. and then I had to wake him early for speech therapy. And he was so tired that he fell asleep in the car for the first time in a year. And the he wouldn’t talk to his speech therapist and threw the flash cards she was using on the floor again. And when that was done, we took him to the zoo to see the new lions. And I’m not sure if he had a good time or not, because he was so sleepy he only cared about pushing the stroller (a tendency his psychologist calls a perseveration, which means something he gets stuck on obsessively). And when we would take it away, it just plain made him mad. And he tried tantrums, and we tried not to give in to tantrums… But when I reminded him he needed to calm down, he took a breath and counted to 4, then asked again in a nice voice. That is actually really good progress for him, and for me.

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.

The devil you know vs. the devil you don’t

Patrick and I had the most frustrating of mornings yesterday. Got up as usual and started out happy enough, but then it was time to connect his 8 a.m. antibiotic. I went to withdraw the ethanol lock out of his line and it wouldn’t come. Tried to flush. Wouldn’t budge.

So I grabbed a dose of Cathflo (the super declotting agent for central lines that I am learning I am VERY lucky to be allowed to administer by myself at home.) Worked it into the line, which took me almost half an hour. I left it in for half an hour.. drew back and the line drew back beautifully.

Tried to flush, and nothing. Let go, and the syringe filled with blood.

Not good. I tried several more times and just got the same result. The antibiotic was due. The TPN was running. The two can’t go through the same tube at the same time. And I was stuck.

So I called the hospital and asked for the GI on call, but they wouldn’t give them to me because it was almost 9 a.m. and the GI clinic opened at 9. So I called Patrick’s nurse, and she was stumped. She called the department at the hospital that specialized in central line repairs, and they were stumped.

Finally, Dr. Jackson’s nurse got word I was calling and called me back. And she was stumped.

It really seemed like the blood was flowing backwards in the line.. out instead of in. Which either meant a slipped placement.. or a line broken under the skin.

Both line killers.

So – while they did their research, I did the only thing I could think. I said a prayer. I put one more dose of TPA in the line. I forwarded our home number to my cell phone. Then, I packed Patrick up and we went to mommy-and-me exercise class. Makes sense, right? We needed some normal, and some distraction. And it was his last guaranteed chance to go to class before school.

Halfway through exercise class, as I was sweating and huffing and puffing… in the middle of the popcorn song where we jump all around the room pretending to be popcorn… my phone rang.

It was Dr. Jackson’s nurse telling me that they’d arranged an admission to the Rapid Treatment Unit. The plan was to evaluate the problem, do an x-ray if possible, have the IV team look at things. And then, she said, it didn’t look good.

I hung up and stayed till the end of exercise class. Why not?

Then we came home, I showered, and I packed a suitcase to see us through a one day hospital stay and a likely surgery.

We rushed up the hospital, lugged our way into the RTU, got vitals, met our nurse…

And then tested the line.

And the 2nd dose of TPA had fixed the problem. Best bet? That the clot was working like a stopper that allowed blood to flow in, but not to flush. Kind of the opposite of usual line function. And that it was blood trapped in the line that was flowing back after attempts to flush… Not the line bleeding as it appeared.

We called it a nurse visit. The doctor came to “not” consult with me, so we wouldn’t be billed for the service. He’s seen us struggle to keep this malfunctioning line going for the past 15 months.

I asked him if he’d give up and replace it yet. Knowing the risks of lost access for Patrick.

He shrugged and told me, “It’s the devil you know versus the devil you don’t.”

How often that is true with Short Gut. How often do we weigh the choice between the familiar but uncomfortable present situation and the risk of an uncertain outcome?

The choice about transplant is certainly that kind of a thing.

It takes a lot of faith and prayer and hope and stepping into the darkness to move along this journey.

And a lot of mornings derailed by emergency hospital admissions that leave dirty dishes and dirty laundry and a mountain of other unfinished tasks while we wrestle with uglier demons.

Brian and I have been doing a lot of pondering and praying to know what is the best choice about this troublesome line.

If you’re praying for us, that would be a good thing to pray for. For us to know whether to keep fighting with this line or take the risk of swapping it out in the hopes of fewer infections and clots and frustrating mornings.

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.”