There’s a saying in medicine: “When you hear hoofbeats, think horses, not zebras.” In other words, the most common answer is usually the correct one. This is good advice when googling your symptoms as well.
But what do you do when it is a zebra?
The last day of February each year is National Rare Disease Day. (Thus encompassing leap day — a rare date for a rare day.) It’s a day to bring together people with rare diseases.
A disease is considered rare when it affects fewer than 1 in 2,000 people. That can be pretty isolating. It means that you may be the only person anyone’s ever heard of with a condition. It may mean there’s not a doctor in your state or in your town familiar with your disease. It may mean treatments that work for others won’t work for you. (But that doesn’t mean you won’t regularly be offered miracle cures by strangers who know nothing about your disease.)
There are 300 million people worldwide with rare diseases. Alone we are rare. Together we are many.
Patrick is rare. Gastroschisis affects 1 in just under 2,000 babies each year. However, Patrick experienced a complication so rare that I can’t find statistics about it. His bowel died, leaving him with Short Gut Syndrome. Short Gut itself only affects 30 of every million people. Only just about 100 intestine transplants are performed each year. Fewer than that are multivisceral. By the numbers, he’s rare.
Patrick and his Beads of Courage: each bead represents a procedure, test, or milestone
I’ve been feeling his uniqueness in other ways this month. Especially at school.
Patrick attends a very unique school, one designed for children with special needs, particularly those with autism. Although Patrick checks all the boxes for an autism and ADHD diagnosis, his diagnosis is not as simple as that. Patrick’s intellectual, memory, and sensory issues come largely from a brain injury he sustained during a cardiac arrest caused by a central line infection when he was a baby. That means that, while many of the things that help autistics help him, there are differences in the help he needs. Also, although he has anxiety, his anxiety isn’t founded in ordinary fears, but in the complex medical trauma that he experienced as a young child.
There is a reason we don’t domesticate zebras. They are in many ways quite like horses. Zebras eat almost the same things as horses. (Almost, but not exactly.) They behave in many of the same ways. But zebras are not the same as horses. And the differences are more than just the stripes.
Zebras are smaller but are very strong. They are not strong in the same ways that horses are; their bodies are not made for riders or for carrying burdens. They don’t run as fast as horses. They have evolved to survive as prey. They are skittish and easily startled, quick and agile, known for a bite and a strong kick. Zebras can be sassy, stubborn, smart, and sensitive.
Zebra keepers know that they need to spend extra time building trust. These animals don’t trust easily. Handlers need to be consistent and gentle. They make sure to feed their zebra personally so that it sees where the food comes from and develops trust. Patient handlers are rewarded with an extremely faithful bond.
But you won’t get there if you treat your zebra like a horse – or even like its closer cousin the donkey.
And this is where my frustration lies this month. I’ve heard teachers say that January and February are when most of the teaching happens in a school year. I don’t know if that’s the reason, but every year in January and February, it seems, we end up talking to teachers about the same thing. Patrick falls behind in these months. And I find myself explaining that he can’t be asked to do the same things as the other students if he’s only offered the same supports they are. His issues with motor skills, memory, spatial reasoning, attention, vision, and math require more.
So we end up talking about IEP accommodations, whether there is sufficient staffing, whether he is too dependent, what is attention seeking or escape, whether staff understands his needs and accommodations, how to help them understand, whether he should have to ask for accommodation so receive them. And all of that leads to the question of whether or not he belongs in the classes he is in. Every few years, this one included, I get a bonus IEP team meeting out of it.
This year is better than some because I know that his teachers actually care and are genuinely trying to find ways to help. At least this year I’m not having to start by trying to convince them that his needs are real. But there is a reason people don’t ride zebras. And there is a reason we are always talking about where we can make compromises.
Patrick and Brian launching a blast car for his science fair project
Next year, Patrick moves into 9th grade. For him, that means moving into high school. And that has opened a whole new set of issues. Demands will be higher. Classes will be harder. It’s getting more and more painful to keep Patrick in grade-level classes without grade-level skills. Because classes are for credit, there are limits to modifications. And he likely will never have the executive skills and memory to handle it alone. The question is if we can find a way to give him enough help to handle it at all. And if doing so is worth it for what he’d get out of it.
So now we are talking about whether or not he should pursue a diploma. Whether he would benefit from some time in a functional skills class. What he will do after high school and what, if any classes, they offer that can help prepare him. And although I’ve known for a long time that Patrick wouldn’t likely get a traditional diploma, or go to college, or have many job options, my heart is having a hard time catching up with my head.
Patrick is a normal teenager in so many ways. He loves his friends. He’s girl crazy. He listens to his music too loud and wastes hours on YouTube. He’s obsessed with cars and can’t wait to own his own. At school with his handful of friends is the one place where I think he truly feels like he belongs and is accepted as himself. His advisory teacher is a keeper in both senses of the word.
Except that, even with a sense of belonging, Patrick doesn’t understand what’s going on a lot of the time and sometimes sits lost and without help for an entire class period. And the question is whether that’s actual belonging. (For Patrick, maybe?)
So here’s to Rare Disease Day, a day dedicated to belonging, even when you are rare.
They say that zebra’s stripes make it harder for a predator to pick an individual out. When many zebras stand together, their uniqueness combined protects the group.
That’s not actually true. Recent research shows the stripes protect against tsetse flies, who are so confused by the pattern that they abandon their attack and fly away. That’s probably more representative of how things work with rare disease zebras. We confuse everyone so much that they flee.
But I like the image of the herd of zebras standing together. So I’m going to stick with it.
“A child born to another woman calls me mommy. The magnitude of that tragedy and the depth of that privilege are not lost on me.”
Have you seen that meme? It captures my feelings towards birthmothers in general, and Patrick’s birthparents in particularly so perfectly. I feel it to the core of my being.
It also only captures a fraction of the unrepayable debt of gratitude that I owe as a mother.
Another child was lost young in an accident, and that family chose in the face of unimaginable loss to give a piece of the life of their child to mine through organ donation. That is tragedy so vast and privilege so profound I struggle to comprehend it.
The pandemic has pushed this truth to the forefront of our awareness. “Patrick’s a transplant recipient” is a phrase we say so often it sometimes loses meaning. It tells you he has health problems that require extra accommodations. It may even tell you his health is fragile or that he’s been through a lot of trauma.
But in our house, lately, we’ve had a lot of discussion about what transplant actually IS. See, Patrick received his transplant the day he turned 6. He was too young and had far too little language and understanding to know what was happening to him then. But there’s nothing like a pandemic to provide time for questions and answers.
Patrick has some fairly distinct memories of his recovery. The staples itched. The stitches were blue. But many of the memories are fading. (“Mom, what’s an ostomy?”) He’s twelve and a half, more than double his age at transplant. Early childhood memories fade and our minds protect us from trauma. He remembers feelings, but very few details.
This week, he found a memento of transplant: a pillow in the shape of a liver with the hospital logo emblazoned on it that was signed by many of the ICU staff. Patrick loved writing and names, so it was a way he connected and found peace.
I can’t put faces to most of the names anymore. But as we talked, I pointed out how many of them had written their specialty and that could tell us who they were. Nurses helped with pain and meds and dressings and keeping him comfortable. Respiratory therapy would come to make him take deep breaths and refill his lungs, since it hurt too much to breathe, let alone willingly cough. And child life made sure he had toys and volunteer visitors fun activities and the occasional magic moment.
Respiratory therapyUp walking on day 4 post transplant
One of the signatures was from the physical therapist. She’s one of the only faces I clearly remember. I told him how she came every day to help him learn to walk again and how, at first, that was very hard because it was so painful. (I also told him about how much he loved her because she would hide Blues Clues in the halls for him to find.)
That led to talking about his scars.
Patrick had a vertical incision that ran the length of his abdomen that was closed with staples and, as a result, he has a very impressive scar. We talked about what he remembered about how that looked and felt. Then he turned his attention to other scars. He has all kinds: from big to small, from straight to round and some that are curvy. Some are surgical, some from his birth defect, and others from accidental injuries. And he made me help him inspect his entire body and tell him how each one got there. Sadly, there really is a scar and a story for almost every appendage.
“Why” has been a big topic of conversation lately. We’ve had to answer a lot of “Why’s” about the COVID precautions we are taking. And a lot of why’s about other people who are not longer taking them. And in the middle of one of those “why” we still wear masks when others don’t, it occurred to me that Patrick knew “transplant” as part of his identity, but didn’t understand what the word meant.
I’ve been thinking that, since we moved and so much time has passed, many of our friends, colleagues, classmates and fellow congregants don’t know the why and how either. So, since we’re already opening the emotional pandora’s box by explaining these things to Patrick, I thought maybe I’d tell you what I’ve been telling him.
Let me take you on a tour of his scars.
(Just a heads up, Patrick is very private about his scars and doesn’t like to talk about what he’s been through.)
First, there’s the short little horizontal one that runs to the right of his belly button. This one’s from his gastroschisis. Gastroschisis is a birth defect where the abdominal wall doesn’t close and the intestines develop outside of the body. This was the start of his troubles. Patrick had complicated gastroschisis. The intestines twisted and died. He was born with a small hole in his belly. The surgeons did a quick exploratory surgery where they found that his entire small intestine and most of his large intestine were missing. They placed some drain tubes (since his intestine was too short and too narrow to reconnect) and closed the hole surgically. They also placed a PICC (peripherally inserted central venous catheter) to give him nutrition by IV as his digestive system was completely non-functional.
A few weeks later, those drains weren’t working. So they pulled the end of his duodenum to the skin’s surface in an ostomy (which means a hole in your body that accesses an organ) and placed a feeding tube in his belly. (That tube is still there, used for meds, not food.)
After his 2nd surgery, age 17 days
We were granted guardianship and flew him from Michigan to Utah by air ambulance. A short time later, he developed his first central line infection while still in the NICU. He has a scar on the back of his right heel where a busy nurse missed that the IV antibiotic being given there had caused the vein to burst. This caused a nasty IV infiltration wound. He also earned his first broviac line scar. (Broviac lines are also central lines, they run to the heart.. but they go in a tunnel through the chest. Patrick’s chest and neck have several pock-mark looking scars from broviac lines.)
The next 5 years were spent waiting for transplant. Beginning in July of his first year and continuing for the next year, he seemed to have constant sepsis. We’d clear one infection, and two weeks later be back in the E.R. with another. It was terrifying, especially when yeast started to grow inside his body. Yeast is sticky and difficult to kill. The antifungals used to treat it are harsh. Eventually, their toll was too much for his little body and his heart stopped.
This left scars you can’t see in the form of an anoxic brain injury, but also a new little one in his thigh from a femoral arterial line. It also left some emotional scars for all of us. We were fortunate that, because the amazing team at the hospital that day performed such excellent CPR, he was revived and has such minor damage. Still I know what it is to have my child stop breathing in my arms. And I know what it is to have arms ache for a baby that isn’t there. We live with the effects of that day constantly.
In the ICU after cardiac arrest
At 9 months old, Patrick’s first transplant team said they didn’t want him to have an ostomy anymore. So a surgeon here in Utah connected his duodenum (the segment between stomach and small intestine) to his large intestine. That incision was a small vertical one next to his belly button. It left his belly looking like a star and we lovingly called him our star-bellied sneech.
Playing in a hospital crib after reanastamosis
Over the next 5 years, he had lots of lines, another surgery, a couple of liver biopsies, and significant scarring inside his major blood vessels from his chronic need for total parenteral nutrition and the accompany central lines. His veins became so scarred that we though he might lose access to IV nutrition and starve, but the interventional radiology team in Nebraska managed to restore access through a very risky procedure than passed a needle tipped catheter up his femoral vein, through his heart and out his jugular.
He had IV pumps in a backpack that he took with him everywhere he went that provided him with nutrition in the form of TPN and lipids. Not having an intestine meant a chronic diarrhea and vomiting, too, so we had one more pump for IV hydration fluids, and the occasional extra pump running broad spectrum IV antibiotics. We carried a diaper bag full of saline and heparin syringes and emergency kits, along with an epi pen (he had several serious food allergies) everywhere we went. We were pros at swapping out broken IV tubing, performing sterile dressing changes, and clearing air in line issues in the dark without waking the child the tubing was attached to. I knew every creaky spot in the floor and could dance through the room without a sound, kind of like a master criminal weaving through security system lasers.
First day of Kingergarten, with his TPN pumps in the backpack
We kept our GI and the emergency room on speed dial. We knew every member of the resident class at Primary Children’s spanning several years. (They made a point to come visit us when they knew we were there.) And I spent more time talking to our infusion pharmacist and insurance case manager than most of my friends.
Meanwhile, Patrick grew up, went on many adventures, attended preschool, started kindergarten, and was granted a wish.
Patrick’s wish was to visit Give Kids the World Village and Disney World in Orlando
At last, Patrick received his transplant. He not only needed an intestine, but also a liver due to damage from the IV nutrition, and since the vasculature is all one package, that came with a new pancreas, too. Remembering how HUGE an intestine is, it might make more sense to know that they also removed his spleen to make space along withhis gall bladder because that way they don’t have to worry about gall stones later. They also did a gastric bypass to help food move into the new anatomy of the gut.
As I explained to Patrick this week, they pretty much just took everything out so it was empty inside, and then put in the new organs and sewed all the parts back together. And that is a LOT of parts when you consider all the blood vessels, nerve endings and splicing back together his lymphatic system.
A couple of hours after transplant
Transplant left the biggest scars: the long one down his tummy with the frankenstein-like staple marks and the one the size of a silver dollar where he had an ostomy for biopsy purposes.
That brings me back to the “why” questions. Because transplant and the journey to get there are why we do most of what we do.
So here’s a question we get a lot.Why are transplant patients more at risk that other people? Doesn’t a transplant cure them?
This answer was one they drilled into us. When you’re being considered for transplant, they sit down with your family and they go over all the risks and precautions that you’ll be living with after the procedure. You also commit to a lot of things to protect the graft like avoiding contact sports, not swimming in rivers and lakes, and being careful about diet.
And then of course, there are the meds. To keep the body from identifying the transplanted organs as a threat, transplant patients must take immune suppressants. Twice a day, every day. For the rest of your life. These have to stay at a constant level in the body.
At first, immune suppressants have to be at a pretty high dose. Eventually, as the body gets used to the new organs, that dose can be reduced. However, since Patrick received 3 organs (including the second largest organ in the human body) his minimum dose is still high compared to, for example, a kidney transplant patient.
Still, with a lower dose, Patrick has been able to live a pretty normal life. He could attend school, except when there was an outbreak of illness in the classroom. (His IEP provides for home learning under these conditions.) He could go to church and play adaptive sports and ride roller coasters. Granted, he and I spend pretty much from November to February sick every year because he catches everything he’s exposed to. Transplant patients get sicker and stay sicker longer, but eventually, he gets over most bugs.
A COVID-style road trip to Mr. Rushmore
But COVID… it’s a different type of virus. It sends the body’s immune system into hyperdrive. That’s what makes it so deadly. Can you imagine the effect of that in a transplant patient?
I wondered if perhaps it was my imagination inflating the risks at first. However, a few months back as they were just about to approve the pediatric Pfizer vaccine, I had the opportunity to join a webinar where researchers shared their current data about COVID, the COVID vaccines, and transplant patients. And some of their numbers were rather concerning.
First, they found that although the general population has a better than 99% chance of surviving the virus, among transplant patients, the death rate is 10%. Those odds are scary.
Even more concerningly, Johns Hopkins, in the absence of data from preliminary studies into vaccines, did a study where they measured the antibody response of transplant patients. As the researcher said in his presentation, they discovered that “we have a problem.” Only around 40% of transplant patients had any detectable antibodies following a first dose of any of the vaccines. After the second dose, only 56% did. They also found that there was a high rate of patients who resumed normal activities after they were “fully vaccinated” who went on to contract the virus.
You know when they say that most people who are vaccinated don’t catch the virus, don’t pass it on to others, and certainly don’t need hospitalization or die? Well, guess who that small percentage who do are? Yup, the people with compromised immune systems.
So EVERY communication I have with Patrick’s transplant team or GI doctor ends with a reminder. “Act like he’s not vaccinated.” “Keep following precautions.” “There’s still risk.” Just this week, Patrick’s GI ended an e-mail about lab results with “don’t let down your guard.”
6th grade was all online, supervised by Max
The thing is, everyone is ready to be done with the pandemic. WE are ready to be done with the pandemic. In fact, the more our community lets down their guard, the harder it becomes for us.
But cases are rising across the world, across the country, and definitely in the state where we live. Vaccination rates are low here and children, though not at risk of severe disease, are still carriers. Add to it that the Delta (now Delta+) variant is more contagious and more deadly. Despite our best wishes, statistics show that the virus is no longer in decline where we live.
Our alternate field trip to the bird rescue with an awesome duckling named Vinnie
We made a commitment years ago. We made it when we adopted him, again when we listed him for transplant, and again when we accepted the offered organs. We promised that we would follow the medicine, keep the protocols, and make the sacrifices to keep him alive.
Patrick’s life is a miracle. Many times over. And that miracle has been contributed to by so many people. His birth family, the NICU teams, his nurses, and pharmacists, the surgeons and infectious disease doctors and interventional radiologists and IV team who responded to emergency after emergency. The nurse who performed CPR and the code team who helped to revive him. The therapists who taught him to walk and talk and write. The family and friends and strangers who helped raise money for his transplant expenses. And most of all, the family who trusted their memory of their child to the future of mine.
In coming to understand transplant, Patrick’s come to know that he has an organ donor. The other night, he was asking me about “the other kid.” Health privacy means we don’t know a lot. But often, when I’m tempted to take some risk, Patrick’s donor and his family cross my mind. I can’t bring myself to take chances with such a sacred gift. Especially when there are so many waiting for donors who will not live to be matched.
We live with a miracle every day. But faith without works, as James wrote, is dead. So we are doing our best to do our part and be patient until we get the all clear from the miracle workers we’ve grown to trust to keep Patrick safe. Some of them are like family, so when they say wait, we listen.
Masking up for physical therapy at Shriner’s
We are hopefully trusting in the promise of vaccines. I was able to enroll Patrick in that same Johns Hopkins vaccine antibody study for kids and we’re watching to see signs of his body reponding to the shot. (With caution, as every doctor emphasizes to us that antibodies do not mean he can’t get sick.)
We are cautiously beginning to dip our toes in. We are visiting with fully vaccinated (and still reasonably cautious) family. I’ve invested in KN95 masks that fit Patrick and am letting him go to in-person physical therapy and occasional uncrowded places in off-peak hours, like the library.
We really want to begin to allow loved ones back into our world and are anxiously waiting for approval of vaccines for younger children.
We want to see you again. Right now, we’re limited contact to vaccinated people. So help us out. Get the shot. Wear your mask indoors and in crowds and around us. Avoid risky behaviors.
Be especially careful with your children. Not only can they spread COVID, but there’s an out of season cold and flu season happening as we come out of quarantine and their risk of catching another illness is higher right now. Please stay home if you have any signs of illness! (Even when we were really counting on you.)
This is an awkward time right now. For everyone. But especially for us. With the political climate and the need for extra caution, we sometimes have to turn down invitations or even walk away when risk is too high. If one of these awkward moments happens with you, please know that we don’t mean to cause offense. We’re not trying to make you feel guilty or sway your choices. (Though we really want you to be safe.) It’s just, with all other precautions taken away, we are needing to be a lot more careful.
We appreciate your patience, your love, your caution, your efforts to keep us included and show us we are remembered. We are eager to be with you again. And we are THRILLED that vaccination has brought some of your back into our lives in person. Patrick’s very best days are when we can say “So-and-so has had their shot. Let’s go do something together!” Hoping for much, much more of that soon.
As usual, summer has been very busy. Brian survived the Youth Conference pioneer trek (they did a one week recreation of the handcart pioneers that helped to settle Utah). Getting last details like their trek booklets and video slideshow kind of ate up all of my attention before and after. And Brian was swamped with getting everything ready both to prepare and clean up from trek, as well as getting work responsibilities squared away. Of course, this coincided with my amazing respite provider being sick for two weeks in a row and I barely held it together, honestly.
As a nice treat, though, my sister came and helped Patrick to bed one night so that I could drive up and visit my husband on the trail. That was a little bit of payoff, despite all of the craziness. Brian was called on to speak to the youth that night. He talked about the women’s pull, when the boys and men leave the carts and the women pull them up the steepest part of the trail in memory of the many pioneer women who crossed the plains without husband because they were divided by circumstance or death. As he talked, I saw a little bit of how it must be for him to watch me struggle right now with my current demands. How helpless he must feel watching me. I’ve been so focused on my own battle that I haven’t really seen things through his eyes that way before.
While Brian was away for youth conference, we celebrated Patrick’s “miracle day.” The 6 year anniversary of when Patrick, basically, died in my arms.. but didn’t leave us. I am still in awe of where we are now considering what happened then. I decided that I’d just scrap all responsibility that day and just focus on Patrick that day. It was swimming day at school, which means I got to go play in the pool. We left there and went out to Arby’s for lunch. (Arby’s has generously donated summer lunches to kids this year and so Patrick and I have been regulars this year.) While there, I asked Patrick what he’d like to do to celebrate and he chose the zoo. And, as we finished at the zoo, I decided to go follow a lead from the morning. The lifeguard at the school pool overheard me saying how hard it is for a transplant patient to find a pool clean enough to swim in. He gave me suggestions of a therapy pool that might be willing to sneak us in during a water aerobics class for a private swim session. In the end, that’s exactly what happened and Patrick and I enjoyed a full hour of having the shallow end all to ourselves. We came home exhausted, ate a quick dinner, and then went to bed early. A perfect way to spend the day.
Patrick’s last day of summer school was a week later and that’s why you haven’t heard much from me. I can’t quite put into words what it is to spend all day every day trying to supervise, teach, potty train, clean up after, and keep nurturing a little boy with this many needs day in and day out.
I’m trying to make the best of summer and shake the guilt of the mom who formerly had amazing mommy school themes planned for every day but now just makes it through the day. Patrick gets up at 6:34 every day. We go immediately to the bath. Patrick’s discovered laying down in the tub and so he has decided to learn to wash his own hair. This means that I can’t just put him in the tub and do things until I’m ready to get him out. He takes care of himself and gets out. Most often. Sometimes I catch him with dry hair and have to send him back to wash it.
We sometimes take lazy mornings where the only goal is making sure he makes it to the potty every time he needs to and that he eats a good breakfast. (He’s discovered cereal now with soy milk and that’s leading to better breakfasts.) Some days, we take some time for playing and learning. I let mommy school slide for a bit and it certainly isn’t organized and awesome, but Patrick started to miss his schoolwork about a week into this stretch of summer break and started to get out his writing books and practicing his letters every day, or grabbing his sight word readers and practicing with me.
Potty training is going well. Patrick has had several all underwear days, even using the bathroom away from home. But sometimes he forgets and sometimes he gets stage fright.
We accomplished Patrick’s first away from home potty success when I offered to buy him this car ONLY if he used the potty in Walmart. We went back 3 times until he finally did it.
He’ll get restless midday and so we take lunches at the park. We gave up on the location by our house because there were never kids his age there. We now bounce around between different parks, going most often to the one near our home with a shaded play structure. It’s nice to give him the chance to move and interact with other kids. And we’re pretty used to eating the lunches I pack now. Of course, Patrick’s gotten a bit fixated on corn dogs in vegan ranch dressing and picks that most often. But build your own pizza kits, hot dogs, pasta salad, chicken nuggets, and hummus are regular favorites.
A lot of our time is also spent practicing time outs, too. Patrick’s been angry again lately. I talked to his psychologist about it and she pointed out that he’s got a lot of new skills (language, potty, eating) and a lot of new independence now that he doesn’t need feeding tubes. And she thinks that he’s trying to figure out his boundaries again. So we worked out a behavior plan with 3 very careful worded warnings and then consequences when he’s out of control in time out.
I tried starting this behavior plan on the Pioneer Day weekend and it made for a very LONG weekend as Patrick fought back against the new rules and consequences. I don’t think we’ve got things quite right yet.
Just when we were making ground, he caught a cold. Amazingly, it only lasted a few days and went away without many problems. But we had to start all over again once he was better.
We’ve also been continuing to go to social skills group at the autism clinic and Patrick’s attention seems to be getting better the more we go. Plus, I get a pretty fun little show watching a bunch of autistic 4-7 year olds practice circle time and social skills through a two-sided mirror. I’m the old-pro parent there with a bunch of brand new, doe-eyed new parents who are terrified of the diagnosis. I just sit there knowingly, quietly watching. They see behaviors that are confusing and scary to them. I just see autism and know that with a little practice and help, that won’t be a big deal. I know there is a lot more parents can survive and learn to do than they realize, and much more potential in children, too. I also see how Patrick doesn’t act exactly like the other kids in the group and remember why I don’t often use that label to try to explain his needs. But the group helps, regardless, and provides some entertainment for me, too. Especially when the kids come play with their reflections.
With a little bit more time back, we’ve snagged some family time this July. We got our bikes in good riding condition and went to the Jordan River Parkway. I went once. Brian has been taking Patrick back. Brian and I even squeezed in a couple of dates. We went to a movie last night and realized that we aren’t ready for that much leisure time yet. It just felt wasteful to sit in a theater doing nothing and we couldn’t quite comprehend people having time to be regular movie goers. (Not that it’s wrong. Feeling like leisure activities are frivolous is a pretty common side effect of the kind of extreme trials we’ve faces this year.) We also took a morning and went out to breakfast and to the driving range. That didn’t feel quite as frivolous and it was fun to see all those skills we learned in golf lessons coming back. I’ve lost less than I expected. We went to the zoo a few times. Brian had a company party at Boondocks so we went drove go carts, played bumper boats and arcade games, and introduced Patrick to bowling. With a ramp and bumpers, he actually did pretty good at it. Especially in a total overstimulating environment and with a cold.
Jordan River Parkway
Dino impressions at the zoo
Racing arcade games
First time bowling
That’s the long and short version of most of July in a nutshell. I think I’m gonna wrap up this sort of travel log sort of blog post right now. There are some other big things that happened in the past couple of weeks, but I think they deserve a post of their own.
Happy April! Don’t you just love April? It is teeming with new life. The trees are in blossom. The tulips opened this weekend. There are little green shoots poking up out of the soil in all of the gardens around my yard. There are birds nesting in the eaves of my house. (No, that’s not necessarily a good thing.)
April and spring also bring us Easter. A celebration of Christ’s victory over death. And spring surrounds us with reminders of the Lord’s power to bring forth life. To turn what appears dead and gone into glorious beauty. It as if all of nature is shouting the promise of renewed life.
April is also national Donate Life month. This year, with our family’s transplant journey fresh in my mind, I can’t help but see lessons about Easter and Christ’s atonement in it. I thought perhaps I would share some of those thoughts with you.
Restoration
In the Book of Mormon, a prophet named Alma describes the resurrection in these terms.
The soul shall be restored to the body, and the body to the soul; yea, and every limb and joint shall be restored to its body; yea, even a hair of the head shall not be lost; but all things shall be restored to their proper and perfect frame. – Book of Mormon, Alma 40:23
I used to say that I looked forward to the resurrection because Patrick and I were going to have a week-long feast. With a perfect body, I want him to be able to taste every wonderful thing that he has missed experiencing in this life. Â I never imagined that to be even remotely possible in this life.
And yet, since transplant, Patrick is getting to do just that. He is finally able to begin to experience some of those things. It is incomplete. Allergies and diet restrictions and motor deficits from his brain injury still limit him. And we will still need to have our feast.
Transplant is not a perfect restoration. In fact, an x-ray or ultrasound of Patrick’s belly would reveal an anatomy that looks more like a jury-rigged mess. But it is the closest approximation that I know of in this life. Â Transplant takes what is broken or missing and puts things back to their “proper” frame.
And seeing what a transformation this human attempt at restoration can bring, I look forward with joyful anticipation to a day when not even a hair is missing, let alone major organs. When everything is made right. When little eyes can focus to read without effort. When words don’t get stuck in formation. When little legs can run without weakness. When everything is made whole and perfect again.
Resurrection
There is one part of transplant that I have a hard time understanding. When Patrick was 9 months old, his heart stopped. In essence, he died in my arms. For 2 weeks afterwards, we came back to our house every night not knowing if he would survive. I was destroyed. I had not understood until that time the literal physical ache of grief that accompanies the loss of a child.
Yet somehow, in the midst of that grief, another family found in the midst of that grief the compassion to give the gift of life to mine. Before transplant, Patrick was terminal. We didn’t talk about how very real that possibility was because we didn’t want it to get in the way of his living the life he had. But we knew. We had made plans and were preparing to one day have to let him go.
With transplant came something different. A hope of a full and long life. A gift that rose out of the grief of loss and death. And, in a very real way, Patrick’s donor also lives on in him.
Again, from the prophet Alma:
And he will take upon him death, that he may loose the bands of death which bind his people; and he will take upon him their infirmities, that his bowels may be filled with mercy, according to the flesh, that he may know according to the flesh how to succor his people according to their infirmities. – Book of Mormon, Alma 7:12
Our Savior voluntarily laid down his life. He suffered pain and sorrow so great he bled from every pore. He hung and he suffered and he died. For us. His mother and his friends wept as they watched him die. They laid him in a tomb and they went home mourning. They wondered how and if they would be able to go on. And all of it. For us.
And on the third day, the returned to find the tomb empty. Because Jesus had risen. For us. He overcame death. And because he rose, we will rise. And death is not forever. Loss and sorrow and separation need not last forever. Because of Him.
I see in transplant a whisper of this promise. It is possible to conquer death. And I know that Christ has conquered death and that my son, if he dies, will live again. And so will his donor.
Odeath, where is thy sting? O grave, where is thy victory? – 1 Corinthians 15:55
Weakness
If you are a medical nerd like me with an interest in transplant, I highly recommend that you sometime read the autobiography of Thomas Starzl, the inventor of transplant. It is called The Puzzle People and it is fascinating to read the journey, the determination, the trial and constant failure that led to this amazing medical breakthrough. It was an amazing confirmation to me that God inspires science and discovery and he leads human beings to be able to master the eternal laws that govern the world we live in.
I’ve learned that in science there are also important eternal lessons. And in transplant, there is an important lesson taught about weakness.
You see, in his early experiments, Dr. Starzl found that he had mastered the surgical technique of transplantation. And yet he struggled as recipients rejected the life-saving organs because they were foreign and seen by the immune system as a threat.
Transplantation did not move from the realm of science fiction into medical science until Dr. Starzl discovered how to use immunosuppression to weaken the body’s defenses enough to accept the transplanted organ. Transplant of larger, more complex organs wasn’t possible until the discovery of a drug called FK506, better known as Prograf, that could weaken the body’s natural immune response enough to protect the transplanted graft. The reason that intestinal transplant is so new and so rare is that the intestine is so large and so intertwined with the body’s immune system that it took such a high degree of immunosuppressive therapy.
In layman’s terms, in order for the body to accept a change as large as transplant, it first had to be made weak. Weak enough to be susceptible to infection and illness.
For the week following transplant, Patrick stayed in the ICU so that he could be given a drug that completely wiped out his immune system. It removed it so completely that they then prescribed him a year of antibiotics, antivirals, and isolation in order to try to protect him. All of his defenses were removed. Because that is the only way to prevent his body from immediately rejecting the gift he had been given.
The apostle Paul wrote about an unnamed affliction that plagued him for years. He frequently prayed and asked for this “thorn in his side” to be removed. And yet, it never was. After much time and certainly much struggle, he recorded the Lord’s response to his pleas.
And he said unto me, My grace is sufficient for thee: for my strength is made perfect in weakness. Most gladly therefore will I rather glory in my infirmities, that the power of Christ may rest upon me.
Therefore I take pleasure in infirmities, in reproaches, in necessities, in persecutions, in distresses for Christ’s sake: for when I am weak, then am I strong. – 2 Corinthians 12:9-10
Sometimes, the Lord gives us strength through weakness. Sometimes he leaves us with a thorn in our side, with prayers that seem unanswered, with trials that seem neverending. He does it because sometimes the only way for us to be prepared to receive His gifts.
But he was wounded for our transgressions, he was bruised for our iniquities: the chastisement of our peace was upon him; and with his stripes we are healed.
All we like sheep have gone astray; we have turned every one to his own way; and the Lord hath laid on him the iniquity of us all. – Isaiah 53:5-6
Christ atoned for the sins of the world, taking upon him every sin and sorrow and transgression. But what good is that gift if we, thinking our own defenses are strong enough, reject His grace. Sometimes, it takes weakness first for God to work the change in us that will make us strong. Not all healing is painless.
I am so grateful for Jesus Christ. For His resurrection. For His atonement. For His grace and for His love. I know He lives.
I see reminders of His gifts and His promise of life all around me. In the tulips and the tree blossoms. In tender shoots in garden beds. In the sparrows. And especially in my son.
We are doing well. Patrick’s responded well to the antibiotics he was started on last week. His liver numbers are normal again. We are still giving IV antibiotics. Therefore we are sleepy. But we are happy. And we are healthy. We had a great Easter full of bunnies and feasting and magnificent sermons. This life is not always easy, but it is good. We are blessed.
I mentioned a while back that we were taking Patrick to see a doctor who specialized in rehabilitation and development. It’s about time I write about it.
The appointment went a lot more positively than I’d expected. I went in half expecting for her to see the muscle tone in Patrick’s foot and decide it was time for dramatic action. Patrick’s physical therapist had suggested botox injections and casting, and I was afraid that that was the next step.
I was so nervous, in fact, that I had Brian come along with me. That wasn’t the sort of decision I wanted to make alone. And he was kind enough to take the whole day off, which was an extra special treat.
They make you feel out a history every time you go into this office, including a list of everything that’s changed in the last 6 months since you were seen. This can be quite the task to list everything and I often just write blanket statements like “typical to short gut” so I don’t have to fill in all the hospitalizations, diet changes, etc.
The good thing about this form, though, is that it gives the doctor an idea of where to start, so she came in and right away said, “So why do you think he’s having problems with his foot?” We stripped Patrick down so she could see his muscles as he walked and then we had him walk back and forth between us. We also talked about the developmental lag I’ve seen on his right side versus his left. She watched the way he hold his hands when relaxed. She stretched his muscles to see how they move.
In the end, we didn’t decide anything new… but she confirmed a lot of things I’ve suspected. Basically, it looks as though there was some degree of brain damage caused by Patrick’s cardiac arrest. This isn’t a surprise. They did CPR for 15 minutes, which means that for 15 minutes, he didn’t have a good supply of oxygen to his brain. In fact, everyone’s always amazed at just how well he IS doing in spite of that.
This doctor, even telling me that there seems to have been damage, still calls him a miracle and says that he is far, far healthier than his medical history would predict.
The official diagnosis she gave is “dystonia”… which basically means “irregular muscle tone.” In layman’s terms, it means that his brain sends the message to move the muscle, but the message gets garbled somewhere along the way. The message the foot get tells the muscles to flex harder than they should or in the wrong direction. The faster he tries to go, the more the signal gets confused and the more unpredictable the movements.
She said to just keep working with him. She said that these muscle tone issues could vary in how much they affect him as he grows and the muscles relax or get tighter. In the future, they might still recommend injections of botox into the muscles to weaken them so he can have better control of them. But that’s not for now.
For now, we’ll continue to do what we’ve been doing. We’ll keep a brace on his foot and we’ll do stretches every morning and night. We’ll keep encouraging him to walk wherever he goes and we’ll hope that, like the rest of his right side movements, he’ll get better with practice so that you don’t even notice a difference unless you know what you’re watching for.
It’s a relief to have my suspicions confirmed and plan of action approved. For the past year I’ve been telling therapists that I suspected something was wrong. It’s only been since we started working on walking with his newest PT that it’s been clear that something actually was wrong.
It’s discouraging, though, too to know that he’s going to have this battle ahead of him. He’s young enough that his brain is very “plastic”, very adaptable and it is possible for him to overcome most of the effects of this… But it’s going to require a lot more effort from him and from me to learn to do those basic things.
Still, he’s making amazing progress with his “boot”. He spent 15 minutes yesterday walking 6 feet between his therapist and myself. Then, he spent most of the evening walking across my mother’s living room. He lets go when he’s playing next to furniture. He refuses to have two hands held while he walks. He is getting faster and steadier and braver. He’ll be walking in no time.
This morning as I was laying in bed trying to convince my tired body to get out of bed to greet a happy 7 a.m. Patrick, I was contemplating on just how far my munchkin has come. And all of a sudden I realized that I am living in the midst of miracles right now.
I am a VERY tired mother right now. By about 5 p.m. every day I just want to call it quits and go to bed because the work of taking care of a TPN-dependent, developmentally delayed toddler is exhausting! Patrick is a very active little boy right now. He’s on the brink of walking. He’s finally mastered the skills of carrying things from one room to another, of opening drawers and doors and emptying the contents found therein, and of putting things in places where I sometimes never find them again.
He’s bordering on two with the fully independent attitude that comes with that age – while simultaneously he’s finally getting the strength in his body to explore his world in ways he never has before. My good-natured patient boy has discovered tantrums. And he’s not afraid to use them to tell me when he doesn’t approve of me stopping some unintentionally self-destructive activity.
And, if the exploration weren’t dangerous enough, try attaching IV tubing to this strong-willed child! Even with a 10-foot extension, he manages to get himself wrapped in and around furniture. He knows no limits! If I leave a baby gate open for even a few minutes, you’ll find him at the top of the stairs grinning, waiting to run from me the second I come to catch him before he reaches the end of his line and gets yanked back down.
And this morning as I lay procrastinating getting out of bed I realized just what a miracle my total exhaustion is! This time last year, we’d just come home from the hospital. Patrick was thin and frail, not even able to roll over. Our current battles in physical therapy show that there should have been much more major consequences of his illness and arrest last summer. No one who hears his history ever expects to find normal looking boy smiling up at them.
But right now Patrick has been blessed with exactly what I’ve always wished for him… The strength of both body and spirit to not know boundaries. He may still be limited, but he doesn’t feel limited.
I take it for granted sometimes because they’ve been so common in our lives. But Patrick, Brian and I live in the midst of miracles each and every day. And we do so with full expectation that more miracles lie ahead.
“For behold, I am God; and I am a God of miracles; and I will show unto the world that I am the same yesterday, today and forever” 2 Nephi 27
Not sure what we were thinking, but 3 days after returning from Yellowstone, we headed up to Seattle. It was time again for Patrick’s quarterly checkup and we decided to keep the vacation going by taking a few extra days and going as a family.
We arrived the day before our appointment and spent the afternoon and evening with our good friends. Lindy, her husband Kelly, and their daughter Lauren live north of Seattle and are kind enough to let us crash their home for these regular checkups. Lauren’s just a few months younger than Patrick and is one of his very favorite friends to play with.
Patrick’s appointment was Thursday afternoon. So, after making a stop for Mighty-O doughnuts (a special treat one of Patrick’s nurses introduced us to), we headed up to Seattle Children’s.
Every visit, they send us a detailed itinerary with individual appointments with his transplant coordinator, dietician, and doctor. But the reality is that they all come into the room at once to see Patrick. His dietician appeared first and couldn’t wait to show me his growth chart. While most kids’ chart shows a nice even curve that tapers off as they get older, Patrick’s has been taking a steep vertical climb lately. He’s in the 50th percentile for weight right now.. However, because he’s only around the 10th percentile for height, this means that he was at about 104% of his target weight. As the team gathered, they couldn’t help commenting on how nice it was to see chunky little legs.
Dr. Horslen, Patrick’s GI, seemed quite happy with how Patrick is doing overall. We talked what Patrick’s eating, how his stomach had been upset by travelling, and some of the strategies being used to prevent infections. Then, after examining Patrick, Dr. Horslen said that he didn’t see a reason for Patrick to need to be seen again as soon. Instead of 3 months, they suggested that we come back in 6. The team in Salt Lake has been doing a great job caring for Patrick and they are happy letting them continue to do so until the transplant comes.
Hearing an enthusiastic clean bill of health (well, except for the Short Gut and obvious problems that come with day to day living) was a special treat on that day. See, July 15th was more than just the day of Patrick’s appointment to me. It was the one-year anniversary of the worst day of my life.. the day of Patrick’s cardiac arrest.
When I look at Patrick now and compare it to what the doctors were telling us was the projected outcome of such a traumatic event I can’t help by say that I believe in miracles. One year before I was watching doctors work frantically and feeling uncertain of what the future could hold. Now, he was full of energy crawling around the doctor’s office, impossible to contain, showing off and flirting as only Patrick can.
After the appointment, we made a quick stop at the Ronald McDonald house to try to get a vision of where it is Patrick and I will be living during his recovery. Patrick LOVED sitting on Ronald’s lap, and especially, for some reason, honking Ronald’s nose. The house is different than I’d imagined, but kind of felt like it could be home for a while.
We decided that dinner that night needed to be something special. Brian suggested a picnic and Lindy and Kelly told us about a place called Carkeek Park in the city.
So, after a short detour chasing down a lost delivery of TPN, we headed into Seattle. We stopped at Pagliacci’s for pizza, then the Laylands guided us to a road where city suddenly turned to beautiful forest that then opened up to a beautiful view of the sound.
We ate dinner, then let the kids play on a little playground in the park. Lauren tried to teach Patrick to climb up the slides.. but in the end, he decided he was a much bigger fan of a giant teeter totter.
Then, we took a bridge with stairs that led us down the bluff and onto the beach.
This was the first time Patrick’s been to a beach and I wasn’t sure what he’d think of it. At first, he wasn’t so certain.. But soon he discovered the joy of walking and stomping in the sand.
Before long, he was cheering out loud! Then we sat for a while and watched Lauren, Brian and Kelly throw rocks in the water.
Finally, we decided it was past bedtime and we’d better head back. But to leave, we had to carry Patrick out. He wouldn’t take more than 3 steps without stopping to cheer.
Friday morning, Brian went in to his company’s offices in Seattle for a few hours. That left Lindy, the kids and I to play. We decided to go back to the beach because Patrick loved it so much. This time, we went to a beach about 10 minutes from the Lindy’s house.
Since I hadn’t planned on beach trips, I dressed Patrick in the only clothes I could come up with for the job. For shoes, he borrowed a bright purple pair of Crocs from Lauren. The result was quite the fashion statement.
It was a cold morning, though. Even beach savvy Lauren didn’t want to throw rocks in the water. I took Patrick down to watch the waves but decided it was a bad time to get wet. So I took his hands and went to lead him up the beach. He surprised me, though. He turned around and headed back to the water and stood where the waves would just lap onto his toes. Once he knew that was safe, he crept forward until the water came up to his ankles. Then he stood there until the cold water had him shivering all over. The only pictures I got there he looks miserable because he was already chilled to the bone. But at least now we can say Patrick’s stood in the surf.
I was grateful for an Ivar’s stand on the way back where we could get some clam chowder to warm us up. Patrick was just grateful for his carseat. He fell asleep immediately and both he and Lauren slept 3 hours.
We went to a japanese steakhouse for dinner. It was a first for everyone but Brian and myself. Most loved the show, but once Patrick saw fire come from the onion and oil volcano, he was pretty nervous about the rest. He was a fan of the chopsticks they gave him to play with, though.. That was our saving grace.
And then, like all good things, our vacation had to come to an end. We flew home Saturday. Patrick showed his true daredevil character on landing. Because of the heat in Salt Lake, the landing was a bit rougher than usual. The girl sitting next to me almost turned green. Then, as we touched down, I looked at Patrick. He was grinning from ear to ear and chuckling. The smile didn’t leave his face until we’d reached the gate. He LOVED the bumps. My little thrill seeker!
I don’t know anyone who loves life as much as Patrick does. Perhaps because so early on he had to fight to keep it. What a miracle it is to share his life with him.
When I started Patrick in heavy duty physical therapy 3 months ago, he was crawling like a wounded soldier.. on his belly with one leg dragging. The goal we set then was for him to be able to stand and walk. Last week, it’s like the little lightbulb flicked on and he’s brave enough to be putting some serious effort into learning to walk.
It started when he found a toddler walker (the medical kind) in the physical therapy office. His therapist pulled it out so he could play with it. Before we knew it, he’d made a successful lap around the hallway in the clinic. When we got home, all he wanted to do was stand and walk around the furniture.
The next day, I weighed down his little cart from Ikea with 10 lbs of flour and away he went.. Walking the cart across the room with me holding onto him by his harness to keep him from falling. Before long, I decided the space was too small, so we went outside. He pushed his little Ikea cart with me holding onto him for balance all the way from my house to the school parking lot behind us.. We sat down and rested after a couple of laps in the parking lot. I thought he’d quit when he saw our house, but no.. he kept going 3 houses the other direction before sitting down to rest, then getting up and trying to take the cart back home. It was at least half an hour of walking and about half a block.
The next night, he saw his cart standing there again, climbed up and pushed it across the room all on his own. The first several tries he leaned too far into it and had to basically run to keep up, but with some practice, he was soon able to control his speed a bit.
He still has a ways to go. His little cart tends to veer to the left and Brian pointed out that he seemed not to be taking as good of steps with his right leg. He walks on his toe and kind of turns his foot inward to take a step.
I pointed this out to his physical therapist yesterday. We talked about some of the possible causes. (Effects of the cardiac arrest, sensory issues, or the scar tissue). I remembered kind of out of the blue being told that the deep wound caused by an IV infiltrating the vein in his ankle when he was a month old could cause problems with walking. (This kind of out-of-the-blue-but-makes-sense memory I sometimes think is the Lord pointing us in the right direction.) In the end, the therapist explained that knowing the cause doesn’t change the treatment.
She did some evaluation exercises and found that he has limited range of motion in the foot, leg and hip. Now I have exercises to do with him at least twice a day to help stretch the muscles. We hope that this will improve things, but if not, she may order a brace for him to wear while he’s learning to walk to help correct the position.
Knowing this as he first starts walking is a HUGE blessing! We can make a minor adjustment now that will help him down the road. This is exactly why I have him in so much therapy right now.. So that we catch these little things that are results of his illness while they’re easy to correct.
This was a good reminder to get this week as I added occupational and music therapy this week and am trying to decide if I proceed with a feeding therapist as well. As if I weren’t already busy. The occupational therapy has made a difference, though, after just one visit. See, we think Patrick might be “sensory seeking”. In other words, he craves big physical input. The therapist has taught me some techniques to use to fulfill that craving, leaving his brain and body free to focus on other things.. Like walking or looking at books or learning new ways to play with toys.
It’s a little thing, but I think I’ve seen improvements. He sat next to me on the bench in church on Sunday and just looked at a book for a good 15 minutes. This is remarkably unwiggly for him.
And so, we keep plugging along.. doing all we can to keep Patrick learning and growing now with every moment he feels well enough to do so. I’d really like him to be walking before his transplant. I think that if he’s not walking when he goes in, that it will take him a long time to get well and strong enough to get back to where he is now. So if we have to walk to the school and back every day to accomplish that goal, that’s what we’ll do. He doesn’t mind the walk, especially now that school’s out so he can walk to the playground and slide on the slide.
WARNING: THIS POST INCLUDES DETAILS OF PATRICK’S CARDIAC ARREST AND SUBSEQUENT PROBLEMS IN THE ICU, INCLUDING PICTURES.
I’ve been asked to explain several times the events of this week. I’ve decided it’s probably easiest if I just take some time to sit down and write this in a blog. It will save frequent emotional repetitions for me… preserve some of the last week while it’s still fresh in my mind… and hopefully give some answers for those who are wondering how things got to where they are. These are things that are still hard for me to remember and talk about so please don’t be hurt if I don’t want to talk more about what I’m posting in this entry.
In my last post, I wrote about a yeast infection called candida that Patrick had in his central line and in his blood. He was hospitalized for 11 days in June for that infection and then went home on antifungal medications. On July 5th his fever returned and we came back to the hospital to learn that the infection had grown back again.
In order to treat the persistent infection, they decided to return to giving him a very dangerous medication called amphotericin (a.k.a. “ampho-terrible”). He was on a very high dose but seemed to be doing ok. He always got chills while it was being given and wanted to be held and held very still, but all other signs pointed towards it doing it’s job without too much problem.
On the 15th, we were making plans to go home. We would give 3 more weeks of treatment at home to make sure the infection didn’t come back. However, before we went home, we decided to try giving the medication the same way he’d get it there. This meant shutting off his TPN (IV nutrition) for a few hours while the medicine was being administered.
He’d been doing so well that I ran home to shower and take care of a few things right before they were supposed to start the ampho. I got back an hour into it and he just didn’t seem to be tolerating it well. His hands were as cold as ice, despite several blankets and heatpacks. He was pretty restless. We tried to feed him to comfort him, but that just make him sicker.
About 3:30 p.m. he got really agitated and started to squirm and pull at his ears and cry. Then he just went limp. I called his nurse in and she called in one of the charge nurses. They took him from me and started administering CPR.
They also hit a button on the wall that pages for a “Code Blue” on the PA system. In under a minute the room was filled with people. A PCT who had cared for Patrick before came immediately to my side, as did a social worker and they stayed with me until we were delivered into the hands of another social worker in the PICU.
One doctor took charge of calling out orders while another kept track of everything that had been done. There were at least 10 people bedside helping with CPR, medications, watching vitals and I don’t know what else.
After about 15 minutes (a.k.a. an eternity) they finally revived him and they took Patrick straight to the Pediatric ICU. I followed with the social workers and his nurses in another elevator.
On the way up I finally got a hold of Brian and told him to come up and where to find us.
Normally parents are taken to a waiting room, but they knew us and how involved we are with Patrick’s care so they let us stay and watch as they placed an arterial line to monitor his blood pressure and worked to find out what happened.
The best guess is that his weakened little body was hit with a few things at once. Being off his TPN caused his blood sugar to drop. At the same time, his weakened kidneys weren’t processing potassium in the right way. When those two things hit at the same time, it created a sort of perfect storm that caused him to seize and caused his heart to stop.The next day they worked madly to stabilize him. His organs had been starved for oxygen for long enough that they’d forgotten how to work. His blood sugar was all over the charts. His potassium levels were out of control. He was anemic and his platelet count just kept dropping. His nurse that day didn’t sit down for the entire 12 hour shift.
Over the next few days things started to stabilize but there were a lot of things out of whack still. His spleen, which had already been in bad shape because of his cholestasis (liver damage) and prolonged infection, decided to protect itself by sequestering all the platelets that went through it. As a result, although they were giving multiple transfusions, his platelet count was still low and he was bleeding easily from anywhere that could bleed. His kidneys also had kind of shut down and so all of the extra fluids being poured in to give transfusions and electolytes and medicines weren’t being cleared by his body. Instead they were soaking through the veins and collecting in the other tissues of his body.
To top it all off, he had some bacteria “leak” from his gut and get into his bloodstream causing a bacterial blood infection.
Saturday they tried to extubate him, but by Sunday morning it was pretty obvious that he was having trouble breathing. X-rays showed that the fluid in his tissues had made it’s way into his lungs. And so they had to put him back on the ventilator and started to give him medicines to make his body shed the extra fluid.
We watched and prayed and waited and tried to get ready to say goodbye until Wednesday when something amazing happened. Patrick peeked at me through heavy eyelids and seemed to know that I was there… He started reaching with his hands and so I gave him a rattle… and he shook it! The next day I was actually able to get him to laugh!
As the day went on, he woke up little by little and started to play peek-a-boo and smile and hold toys. His X-rays and labwork improved. As he woke up, they started to wean him slowly off the ventilator and off the sedatives.
His throat was very swollen and we weren’t sure if the airway would stay open without the tube in. But Friday evening they decided the only way to know would be to try. So they took him off the ventilator and put him on high flow oxygen. And he was the happiest kid you could imagine.
Without the breathing tube to bother him, they were able to turn off his sedatives and we became aware of what might be another problem. We noticed as Patrick started waking up that he wasn’t focusing his eyes well… staring off into space and not always following what’s going on in the room. He’s also been holding his head and arms very still. When these issues didn’t go away when the sedatives started wearing off, we became more concerned.
Neuro came to see him and said that they think there is reason to evaluate him. He will have an MRI on Monday as well as a neurodevelopmental workup. There is a possibility, especially considering how long he was down, that Patrick’s brain was damaged. Neuro will be able to help us see if that is true… and if it is, they’ll help us do all we can to help him do the best that he can.
Regardless of how weak his body is, though… the spirit inside of it is still Patrick’s. His eyes still sparkles and he loves to play and laugh with us. It has been a gift to be able to hold him and play with him again… to know he knows us and loves us still. It breaks our hearts to see that magnificent spirit confined by such a weak little body and we hope that those limitations will be brief.
As far as long term planning- Patrick will need to stay in the ICU until he is better able to breathe on his own. He is doing it right now, but he is working very hard at it. He’s on hold for transplant until he can go back to Seattle for another evaluation. At the very least, he will probably now need a liver transplant along with the small bowel transplant. We won’t know if he’s still healthy enough for transplant, though, until he is evaluated again. We’ll continue working with the doctors and therapists and nurses here to help Patrick regain as much as he can. And the rest we’ll leave in the Lord’s hands.
We’ve always known that there was a plan for Patrick’s life. We’re just humbled to get to be the parents in that plan. We trust Heavenly Father to take care of the rest.
Patrick Hoopes 
