Thinking zebras

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.

Happy Rare Disease Day.

Oh yeah.. and last week…

Blogging has been on my to do list for a couple of days. But when I sat down to write last night, I was so full of the thoughts and worries of that 48 hour period that I forgot there were other events last week that I’d meant to write about.

We had a couple of appointment last week. We finally got back in to see Patrick’s psychologist yesterday. Can I tell you how amazing she is? When Patrick’s insurance case manager called me to tell me about a new Autism clinic that she’d seen open at the University of Utah THE SAME DAY THAT SHE CALLED I was pretty speculative. Especially since we have never been big fans of the diagnosis of autism for Patrick. Spectrum diagnoses are tricky and, while time and learning have convinced me that Patrick does have struggles that fit into the definition of autism, his presentation is so atypical that I don’t feel like the diagnosis serves him well. Well, unless you are dealing with someone who really does understand autism spectrum disorders. Which the people at this clinic really do. And for all that I don’t willingly introduce Patrick as autistic, we have found the autism clinic to be a tremendous help for us. I’ve been anxious to get him back.

When Patrick met “Dr. Joo-la” and her “piggies” (guinea pigs) there was an instant connection. I could see that he clicked with her and listened to what she said to him.As icing on the cake, she also saw that with the responsibilities of being his caregiver, I wasn’t going to have a chance to go seek other help for myself. She told me in the first meeting that if we sometimes needed to spend sessions┬átalking about and taking care of me, too, that she considered that an important part of taking care of Patrick.

This last visit, I took her up on that. First of all, she spent a lot of the session reassuring me that Patrick really HAS made great progress. I’ve said before that it doesn’t seem like he is as plagued by constant sensory seeking as he was before. She pointed that out, too. Saying he seemed more focused, more grown up. Of course, his exploding language skills are an amazing step.

She reminded me not to be overwhelmed by after-school meltdowns. Pointed out that we had the same problems last fall, too. And she helped me brainstorm ways to make coming home from school perhaps a little better.

She also reminded me not to feel guilty about not being able to do all my heart says I should be providing for Patrick. She’s been following this blog, so I know that she was aware when she told me that she knew that a lot of days, we are just still surviving the day. She encouraged me to embrace summer school as respite time for me and NOT to try to spend it doing things for him or feeling like I need to save them from problem behaviors. I really need to call and see if it’s an option for me to swim in the mornings while he’s at school. If not, at the least there is a track at the high school on the same campus and I can walk.

And then she reminded me that I need help and tried to help me work up the courage to go and tell some of the people in my support system that I’m feeling lonely and overwhelmed and could use some company, if not some help. I don’t seem to be very good at that. (Does this count?)

Anyway – we talked about some other strategies for summer, for respite, for behavior, etc. We talked about bringing him back to their social skills group. (Which I’m very pleased to have found works well this summer.) We played with the piggies and Patrick tried to trade our bird Max for one. And then we made some return appointments.

That was the happy appointment of the week. The next day, I took Patrick to his allergist and I’m afraid it didn’t go as smoothly.

I learned two important lessons. 1) Don’t schedule appointments immediately following school. Patrick needs time to unwind first. 2) Don’t go to the allergist alone.

Because of his ADHD and sensory processing disorder, Patrick doesn’t do well in new environments and Patrick’s allergist just moved to a big, beautiful new facility. I’m very excited about this because he’s no longer sharing space with a regular healthcare clinic and there’s less risk of catching a virus there. But for Patrick, new spaces have to be explored thoroughly with doors banged, containers emptied, equipment disassembled. It’s a disaster. Also, because of a lifetime of doctor’s visits, Patrick doesn’t like it when I talk to a doctor about him and will do just about anything to get me to stop.

This day was particularly bad. I’d managed to get a tired Patrick to nap the day before, but insomnia kicked in that night and he was up till 12, getting just 6 hours of sleep.

The end result is that Patrick threw all of the flashcards, snacks, magazines, and tissues on the floor. Then ended up having to sit in a chair with all other furniture moved away from him. And then he screamed for most of the rest of the appointment.

I have to give props to Patrick’s allergist, Dr. Gleich. He still smiles, talks to Patrick like Donald Duck, tells me that I have my hands full but am doing a good job being patient, and just gets us through the appointment as productively as possible. He is a very good man.

So while I tried to keep as much calm as possible, Patrick’s allergist and I tried to talk through how transplant might change the strategy for his allergies. Obviously, the ability and need to eat are a significant step. And I wanted to talk about how to safely explore what he can have.. and just how safe it is to be dabbling in some of Patrick’s milder allergies while he is on immune suppressants that are making it so he doesn’t have many reactions.

I wish Patrick had been feeling better so we could have covered more ground. We reviewed Patrick’s last test results. The gist of them is that Patrick’s test results show him allergic to a lot of foods that he tolerates, at least to some degree. We still need to stay far away from cashews, pistachios, peanuts, and unbaked eggs with caution for other foods we’ve seen cause a reaction. He said to keep encouraging Patrick to eat eggs as an ingredient in baked foods as that mild exposure is believed to help kids outgrow allergies. He gave blessing to my efforts in allowing Patrick traces of milk, in extreme moderation. (Goldfish crackers, for example.) He actually was surprised that I was still being cautious about butter and regular cheese, but I pointed out that we see reactions to those foods.

He also took care to warn me of just how serious it is that Patrick’s spleen was removed, leaving him without a major defense against illness. He wanted me to be sure that, for any fever, I know I need to go straight to Primary Children’s. Some things don’t change.

And then, because we weren’t getting much further with Patrick screaming in the corner, we decided to not try additional testing that day. Instead, he asked me to bring Patrick back in July or August for repeat blood and scratch testing. In the meantime, we are supposed to explore and even push a little bit, with epi pen and benadryl nearby, and keep a log of what we discover about Patrick’s tolerances for certain foods.

I find that the further we get down this road the more obscure my question are. Neither Patrick’s allergist nor his transplant team really know how food allergies and immune suppression will affect each other. I don’t want to compromise Patrick’s new gut with a lot of foods he’s allergic to. (Food allergies can cause a sort of rashlike reaction and ulcers in the intestine). But I also don’t want to limit his nutrition and ability to wean off of tube feeds if that’s not necessary. I find myself wishing that I knew of an allergist somewhere who has an interest in transplant and immune suppression. I’m not sure such a person even exists.

Anyway – Patrick was asleep in the car 5 minutes after we left the appointment. Next time, I’ll try to allow time for a rest after school. Next time I’ll try not to go alone.

And maybe over the next couple of months we can figure out a schedule that lets Patrick outgrow naps, like he’s trying to do, without spending afternoons and evenings too tired and grumpy to function.

One other appointment this week, feeding therapy. Inspired by Patrick’s interest at a memorial day barbecue, I decided to work on hot dogs this week. I’m pleased to report success. So long as you cut the hot dog in half so he can fit it in his mouth. And watch him and remind him to take small bites. And maybe let him decide he’s done with the bun. Still, a victory in time for summer for a kid who doesn’t like his burgers grilled.

And speaking of burgers, I’m trying to figure out how to translate Patrick’s love of certain fast foods into a working menu at home. I’ve got him eating ham on english muffins a-la Burger King breakfast sandwich. And we’re working on thin sliced roast beef on hamburger buns as a tribute to Arby’s.

I do have one lingering worry. I’ve realized that if they do decide that Patrick can continue on to first grade next year, that means eating lunch at school. And right now, I mostly have taught him to eat warm foods. He does great with fast food, mac and vegan-cheese, pasta in red sauce, hot dogs, cooked veggies, soups, chicken nuggets and french fries. And this is exactly the sort of food that will be being served in the cafeteria. Except, well, that an elementary school cafeteria is not an allergy-safe place. I can’t expect them to watch for cross-contamination.

So I’ll be packing lunches. But I think one of the conversations we need to have in this week’s school planning meeting is whether or it’s an option to heat up food for Patrick in a staff microwave. (It’s already non-negotiable for me that he’ll need an adult to sit with him in the cafeteria.)

And Patrick’s feeding therapist and I did some brainstorming on cold foods that he might be able to eat if we work with him over the summer.

I’ve decided that we will for sure be frequenting the lunch park at the school next door again this summer. But this time, with the hope and goal of being able to figure out lunches that will work to send with Patrick to school next year. Last year, I was able to follow their menu and pack matching foods 80% of the time. But if those things can’t be warmed up at the school, then we may just have to work on being ok with eating the food you had packed for you, even when it isn’t the same as everyone else’s.

One other item of note from this last week. A family moved in across the street from us while we were in Nebraska. They have a little boy Patrick’s same age. We’ve talked about but not found a way to get them together to play. Until this week.. when this boy came and asked if Patrick could play.

This was a growing experience for this mom. I am trying VERY hard to stop being a helicopter parent now that Patrick doesn’t have IV’s to monitor. But it meant that both of us were a bit thrown by a same-age playmate. I’m not sure Patrick knew quite what to do with him. In many ways, he is like his peers. But in many ways, he still has a lot of growing to do. They drew on the sidewalk with chalk a bit, tried out all of Patrick’s ride-on toys. Then they went across the street and played in his yard, too.

I was doing my best to stay looking busy but also keep an eye on them. And to let Patrick build this relationship on his own without my coaching. Mostly they drove Patrick’s ride-on car up and down the street.

When I picked Patrick up for dinner, he was enjoying a snack of animal cracker. I was grateful they were safe, and I decided that next time I send him to play I need to make sure they know he has allergies.

It’s been a full week. I sometimes think my head might explode trying to hold all of this and have a normal life, too.