Can we get a punchcard with that?
Zoe was seen in the GI clinic on Tuesday. The lowdown: her liver is making proteins fine but she’s either not absorbing them or losing them because of inflammation in her intestines. This could be from a food allergy or her immunosuppressand or it could be because her lymphatic system is clogged from her previous heart surgeries. The lymphatic system absorbs proteins and when it’s clogged up from surgery it gets worse when there’s any infection in the body or something as simple as a cold (this sounds kind of familiar). Her protein level is not dangerously low so he wants to wait 4 weeks, repeat labs and then make a decision as to how we proceed. Eventually he wants to do a biopsy of her intestines. Can we get a punchcard going? Every fifth sedation is free? He would rather not do the biopsy until she’s being sedated for something else (less sedation = good) but if her protein level has dropped in the next month he will be forced to do it right away. The good news is that the biopsy will tell him everything he needs to know; it is a wealth of knowledge and we can get this protein issue under control. He kept reassuring me that her level was not dangerously low at which point I asked him when it starts affecting her brain development. No one can really tell (a phrase you REALLY want to hear from your kid’s doctor) causing me to chant, “serenity now” over and over in my head. For now I will continue to feed her pre-digested formula (tasty) since she may be able to absorb that and wait until her next labs.
Some results
I was given a few results yesterday as I left the hospital in regards to her iron level issues. She tested negative for both leukemia and PTLD (a transplant cancer). Her bone marrow biopsy indicated she has something called Transient Erythroblastopenia of Childhood. It is a remporary condition where her bone marrow stops making red blood cells. This website explains it much better than I can but we should expect her to start turning around in about a month. She will probably need another transfusion but will do it as an outpatient. The condition is not linked to her transplant or her medications.
We will see GI clinic on Tuesday in the hopes of getting some answers as to why her body is not making enough protein. In the meantime she is back to normal and although her iron level is still lower than normal (30.4 instead of 40) it is MUCH higher than before the transfusion (18). She’ll be watched closely by hemotology until this all resolves.
Home
We got home this afernoon. We have a few results (all good) but I’m too tired to post them tonight. I’ll put up something tomorrow.
Transfusion time
Yesterday morning Zoe’s doc called with the news that her iron level dropped some more and advised us to give her the transfusion. He suggested we do it as an inpatient (just overnight) so that GI and hemotology would come by and consult on her case; the idea being it would be less chaotic, easier to schedule and all around better. We got here yesterday at 11am and saw the hemotologist late in the afternoon. She advised us to have a bone marrow biopsy (a sedated procedure) done on Zoe before leaving the hospital so that will take place today at 11am. This will, hopefully rule out some undesirable reasons for her marrow not making red blood cells. It will also allow them to test for some viruses that can suppress the bone mrrow production.
That being said, the “convenience” of doing this all as an inpatient is pretty much lost on me. Things did not go well last night. They placed her IV at 8pm right after she’d finally fallen asleep. It was put in a pretty uncomfortable place because that’s all they could get so going back to sleep was extremely difficult for her. The blood did not get here until 3:15 in the morning for a variety of reasons, some of which could have been avoided and some which could not. It was a disaster but I learned a lot about what to do next time. Poor Zoe was uncomfortable (from the IV) and they have to do back to back vitals on her as they start the transfusion and then they have to come in and check her every 20 minutes for the remaining time. We’re tired and grateful we’re scheduled to go home today.
I’m again amazed at the individualized care Zoe receives. We’re so blessed to be living where we are and in a country where this care is available; in many countries kids like Zoe don’t ever have a chance.
I’ll post more later today.
Friday
We ended up going in to the cardiology clinic yesterday after the cardiologist called to check on us. Her belly was pretty large and he wanted to make sure there wasn’t fluid there and decided to check her iron levels just for fun. “Oh, and if you can arrange it, we’d love a stool sample too please”. All of the planets must have aligned because we were able to make it up there on time, with a stool sample and there wasn’t more than a 5 minutes wait at the lab. I swear I’m telling the truth.
Since Noah is still coughing he had to stick with us in the clinic room (I GIVE THANKS AGAIN FOR MY LAPTOP) and an amazing nurse (Jason) hung out with us when they did x-rays on Zoe (Noah’s not allowed in the room).
The verdict:
Zoe’s gut is distended because she is full of….hmm….how do I say this nicely…..poop and gas.
Her iron level continues to drop and we nearly had to stay for a blood transfusion but we are trying some injections first. She’s doing so well with anemia that her cardiologist had a little wiggle room. However, if she gets worse this weekend we will have to take her in for a transfusion.
Her stool sample showed lots of fat in it meaning we have a definitive answer…..malabsorption. Her cardiologist is consulting with GI and feels it is her immunosuppressant medication causing this. Before he switches her he’s going to further consult with GI this weekend. In the meantime, I have to get her to drink nasty, pre-digested formula four times a day or they will put the tube back in. I thought I was hallucinating when he said that. I asked him to give me a chance to mix chocolate sauce into the formula before we do that. She’s had it once today and said she liked it so maybe we can avoid that tube! This is not a road I want to go back down. For the meantime I’ll be calorie counting to make sure she’s still eating enough, getting formula in her that she can actually absorb the nutrients from and Paul will be giving her, every other day, injections to try and boost her iron level.
That’s the latest.
Lab results
Her labs are back and the increase in Magnesium and her immunosupprant drug did not increase her levels. Her white blood cells are coming back as are her red blood cells but her iron level continues to decline. Unless new symptoms appear or she gets worse her labs will be checked again on Monday. At that point, if her iron level has not turned around she will need a blood transfusion. I was able to speak with another family tonight who has had the same issue and it was reassuring to me, even though they don’t know why it happens, other patients have had this problem.
Take 2 jellybeans and call me in the morning
The last three weeks have been a bit of a rollercoaster. Or maybe a better description is the ride up where you balance just at the top before plummeting down. Three weeks ago Zoe woke up with a puffy face. Not a good sign for kids with heart problems but I was determined to wait it out and see if it was really worthy to call the team. Or maybe I was just lazy. The swelling decreased during the day so I decided to wait and see what happened. Over the course of the next week she woke up with varying degrees of puffiness and as the week went on her heart rate increased. So I finally called it in and they ordered labs. Her labs came back weird enough that the cardiologist called me. They looked at a measure of her overall nutrition as indicated by proteins in her body called the albumen level. They tested for this because last September when she puffed up like a balloon it was because her body had stopped absorbing proteins. The albumen level came back borderline, her magnesium level came back low even though they had just increased her dosage, her immunosuppressant came back low and her iron level had dropped 8 points in three weeks. This led them to conclude she must not be absorbing nutrients. So I played the game where I take a diet log to show them I’m not starving her (although I assume if someone was starving their child they would MAKE UP a diet log). In fact the girl gets TWICE the number of protein grams she needs a day. Then nutrition informed me that they don’t know why anyone tests an albumen level because a pre-albumen level is much more accurate (tests short term protein levels). Zoe’s pre-albumin level is completely normal.
The plan: Have GI look at her. Through a series of events no one sent in the referral so I didn’t even get to talk to a scheduler until this last Thursday. Her appointment is in three weeks unless cardiology steps in. In the meantime we’ve done more labs and her iron level has continued to drop (7 points in one week) and her bone marrow stopped producing a type of white blood cell you need to fight off infections. Sometimes this happens from the immunosuppressant but her dosage is so low they admitted it was odd. The way to treat this is to give her injections. Yes, injections. My trophy I hold over my head is that we’ve never been asked to give her shots. Paul decided to take this one on-yah-yah-yah. She did three days of that treatment along with liquid iron (taken with 2 jellybeans to mask the flavor) this last week and had more labs on Friday. Her white blood cells doubled (yah) but her marrow decided it didn’t need to produce anymore of those annoying red blood cells. Her iron level has dropped from 40 to 24 in about a month so she’s pretty pale but wears anemia well in that she’s nearly as energetic. And she caught a cold which made everyone a bit nervous with the whole white blood cell count thing. Overall she seems fine but someone REALLY needs to figure out what is going on with her.
Next up……get cardiology to bribe GI for a sooner appointment or call the GI nurse sobbing. Labs tomorrow night.
5 year old boys
I love 5 year old boys. I LOVE THEM. There’s never a dull moment. On Friday Noah was playing in his room while I threw some laundry in the dryer. All of a sudden he’s screaming hysterically and when I run upstairs he’s screaming, “I THINK I POKED MY EYE OUT…….WHAT AM I GOING TO DO……..I POKED OUT MY EYE………..WHAT AM I GOING TO DO!!!!!???? After about 10 minutes and seeing him shaking from the pain I decided we’d head up to the hospital early and hit the ER before Zoe’s labs. When t.v. doesn’t distract Noah then something is wrong. I couldn’t get near the thing and the small glances I caught showed swelling and that something was wrong with the shape. What did he poke his eye with……his finger. Yes, his finger. I still don’t know how it happened but he was very clear that it wasn’t the first time he’s poked himself with his finger but this time hurt much worse.
The ER was really great. Noah calmed down a little after arriving but was still in a lot of pain. His very kind medical student asked him to move his hand away so he could see it and Noah did it. This next part is hard to imagine. 90% of his eyelashes were underneath the lid. And Noah has the longest eyelashes you’ve ever seen. How? How does that happen from poking your eye with a finger? Sadly, those luscious lashes had been in there for almost 2 hours leaving his eye so swelled I don’t think he could have opened it if he tried. In a complete act of bravery Noah took his own fingers and pried his eye open for the doctor (they were going to give him an anti-anxiety drug to get the job done if he couldn’t). As soon as he got his eye open all the eyelashes popped out. They checked out his eye for damage and scratches. All clear. I think he was disappointed they didn’t give him an eye patch (come on docs…where’s your sense of humor?) The swelling was gone by the next day and there is one area of popped blood vessels in the corner (I’m assuming where the “poke” occurred).
It was interesting for all of us to change things up a bit. Noah seemed almost proud knowing all the equipment they used to take his vitals. Zoe acted like the whole thing was a complete bore until seeing a doctor she recognized at which point she put on “the scowl”. The nurses and docs were great in that they put on gowns and masks while in the room because they knew Zoe’s white blood cell count was really low (did I mention they almost wouldn’t let me take her in there?). If you’re in the Seattle area I highly recommend going to Children’s Hospital ER. They won’t even laugh at you when you bring in a kid for an eyelash injury.
