March Updates

 

We came home from MN on March 5th. It will be about two months until we find out if Raul is approved for the trial. If he is approved, we would likely head back to MN in the Fall. On the 11th he got an iron infusion. We had discussed whether to do iron or blood as he was a bit symptomatic (tired and tachycardic), but we want to hold off on blood as much as possible if transplant might be in his future. So he got iron, and will start to get iron more regularly to try to keep his levels up. While he doesn't like getting poked, Raul likes the infusion center because they always have good toys and crafts!

Getting his iron infusion

On the 18th Raul got his new power chair! His power chair has a seat to floor function so that he can scoot in and out himself and not have to be transferred. He does a great job driving it, and is now using it exclusively at school. It is very exciting for him to finally have this sort of independence!  We are looking into getting a wheelchair accessible van so that we can take it more places.

 Singing the weather song

 The night of the 19th we spent several hours in our local ER having Raul's hip looked at. He has osteopenia and abnormal hip joints, so is more likely to have serious hip injuries. His hip had popped while we were doing bandages, and he was complaining of pain, but luckily by the time it was x-rayed it was not dislocated and there was no fracture. The ER doctor said he may have partially popped it out and it popped back in. On the 31st he was seen by ortho. and had repeat x-rays. He was fine with them moving his legs around, but did scream when they touched the area. They said the popping could have also been tendon or scar tissue, and that at this point it may be more of a muscle issue since his hips are tight. We will follow up if he continues to have pain. 

Raul asked me to make a tent for his bed

Raul has not been able to take full baths regularly in a long time due to how traumatizing and painful it is for him, so we usually just use soaks on individual wounds. I tried a bath in September, and it was just as bad as it used to be. I tried again on March 21st, and it was actually a bit better! He still screamed and was unhappy, but it wasn't as bad as before. I'm not sure if I just got the timing right with his meds. or what, but I am happy it wasn't too bad! I put him in with his bandages on, and took them off as we went. He watched Mighty Machines, splashed a little, and popped some bubbles I blew.

Splashing in the tub

At the end of March Raul started to get a bit more tired and pale. He started to have fevers, and it was hard to tell if it was viral or infection, so we went to the ped. They ordered a blood culture to be added to his labs with his iron infusion, and started him on Bactrim. They thought he looked pretty good for all the fevers he had been having, and luckily his fevers improved quickly with the antibiotics. His blood culture was negative, but he was quite anemic on the day of his infusion (April 1st). He continues to become anemic more quickly, and is not always producing red blood cells properly. His next iron infusion is scheduled for April 27th.

Showing off his crafts he made during his iron infusion

Playing with foamy soap at the infusion center

MN Consults Day #3

We are finally back at the hotel after a very long day! Raul was back there for about 5 hours, and under anesthesia for over 4 hours. They gave him Versed in pre-op, then I suited up in the white jumpsuit and hair net and took him back to the OR, where they gave him laughing gas while they did his IV and put him to sleep. Then I joined my mom in the waiting room, where not too long later the GI team came out after doing his endoscopy. They said things looked pretty good, and let us know that they were going to go ahead with IR doing the esophogeal dilatation. A bit later IR came by to get consent.

Next the person from Dr. Tolar's team came out to let us know that she had finished the photos, skin biopsies, and a couple wound cultures. Unfortunately, they were unable to do the bone marrow biopsy due to the wounds on both sides of his hips. They prescribed Bactrim for him as we wait for the culture results to come back. She also told us that IR felt it safest to intubate him for the dilatation, and a bit later the updated us that they were starting the dilatation. When IR came out they let us know that he had one fairly tight stricture up high, and a less tight one at the top of his chest. Next it was time for me to get suited up again and go back to the OR to rebandage Raul. That went smoothly, and it was easy to do it with him still under anesthesia and not in pain! But I am glad that I don't regularly have to wear that get up while changing bandages, as it was hot!

Then they had me go back to the waiting room again while they extubated him and moved him to recovery. 25 minutes after the board had updated that he moved to recovery we asked if we could go back, and they said yes. It was quite a long time before he woke up, but he tends to be slow waking up, and today had been under for a long time. But when he woke up he immediately started talking where he had left off before he went to sleep, and wanted to sit right up (even though he was falling over). They didn't have any car balloons blown up, so he picked a pony balloon, as he is currently obsessed with horses/ponies (switched recently from camels). He is still being a bit goofy and sleepy now, but we are back at the hotel and he is playing on the iPad and with his trucks. He ate two yogurts when we got back, which I am sure felt good on his throat!

MN Consults Day #2

Today was our second day of consults. When we woke up it was snowy, which made Raul happy, as he says winter is his favorite season! It was a bit of a long day for Raul, and by the end he was telling us that he was feeling grumpy and that he didn't like meetings. Since he doesn't understand the reason for the meetings, or what is being discussed, I would imagine it is pretty boring even with the iPad! Luckily for us it is very interesting, and we are excited about the possibility of Raul qualifying for the trial!

Today we had a long meeting with the social worker to go over everything, discuss lodging possibilities for when/if we come back, different supports available to families, schooling, some insurance info., etc. Then we got to take a tour of the BMT unit, which we had been excited about! The unit is amazing, and certainly not like any hospital unit I have seen before! Raul was impressed by the giant TVs and the colorful panel of lights in the ceiling. Then we had an anesthesia consult., and Raul liked the toys in their waiting room. He got to talk to a couple other kids who were in the toy area with him. Last we met with the nutritionist, who went over his current regimen and what might change during transplant (possibly GJ tube, less formula, slower rate, and/or TPN if he didn't tolerate his usual amount/schedule).

Raul was exhausted, and finally fell asleep at the hotel late in the afternoon after fighting it for a while. We had to get him up so that he will still sleep tonight, and he was not very happy as he was deeply asleep. Tomorrow we have another busy day, but luckily Raul won't be aware of it! We have to get to the hospital at 9, and his procedures start at 10:30. He will have an endoscopy, be unbandaged for skin biopsies, a bone marrow biopsy, esophogeal dilatation, and then I will be called in to re-bandage him. 

MN Consults Day #1

Raul had his first three appointments today, and will have three more tomorrow, then his OR day Wednesday. We got to meet with Dr. Tolar, which is something we have been wanting to do for a long time! He was very nice, and went through some background info. on the history of the BMT for EB, the process for approval, the length of stay in MN for the BMT, and discussed the next BMT protocol that they are currently working on. If Raul is approved, he would be part of this next protocol, which would give us a slightly longer time frame until transplant, but not too long luckily! Dr. Tolar will also help manage Raul's current hematology issues. Today I signed all the forms with the nurse coordinator for if/when they look for a donor. We also saw GI, who is trying to figure out how to organize a scope and possibly esophogeal dilatation by Wednesday. They wanted to up-size his g-tube so that the stoma would be dilated for them to put the scope through, but they did not have the size they needed, so that part is sort of in limbo at the moment. They may be able to get one by tomorrow, and they were also going to check in with Dr. Tolar to see specifically what he wanted them to do.

Mall of America

We had a long night last night, as Raul was up a lot, but then we had a good day today! We did bandages, stopped at Target, and went to the Mall of America. Raul was very excited about meeting The Wonder Pets and Dora/Diego, as well as watching some of the rides. He even went on two rides: the blue train from Dora and a big rig. A very nice family who was leaving offered us their extra tickets for rides while we were waiting at the ticket booth. We also went into the Lego store where Raul got to play with Duplo at a play station, and pick out a new set (he picked a snowplow). He was definitely tired today, but he did awesome at the mall, which was pretty overwhelming!  He says he had a good day, and he is now busy playing with his snowplow.

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Winter Updates

Back in January Raul got a new mattress for his medical bed! We are the third family to use this bed, so the mattress had gotten pretty bad, and we were using a zippered cover to keep the old one enclosed while we waited. It is very nice to have a new mattress, and they were able to get him one that reduces pressure on his body, too. He was very happy about his new mattress, and demanded to sit on it for a while after it was delivered! 

In the last couple months we have been trying to figure out Raul's anemia, and how to get his infusions in before he gets too severely anemic, which has been happening more quickly lately. Raul had an iron infusion on 1/20, after having been symptomatic for a couple weeks (we got on the cancellation list, but couldn't get an infusion slot any earlier). That day they drew labs, and when those came back we decided that he was low enough that he needed blood as well, so he had a blood transfusion 1/22. At that time it also appeared he was not making as many new red blood cells, so the doctor ran a test for Parvovirus, which can slow/stop red blood cell production temporarily, but he did not have that. This will be something that we will watch over time.

 The weekend following that he started having viral symptoms, which we believe to have been RSV due to a positive test on my foster baby. He got a very bad cough from the virus, and would vomit when coughing or lying down. Thankfully after a week he was mostly better, but the virus had set off increased inflammation in his body, causing him extreme joint pain, and worsening some of his large wounds. Just this weekend, almost a month later, the joint pain finally seems to be significantly improved!

Shortly after recovering from the virus he started to show symptoms like he had the last time he was very anemic, so we ran labs, and thankfully that was not the issue (it had only been about 2.5 weeks since his iron and blood, so it would have been concerning if his anemia was already bad again). However, even on those labs it is evident that he is becoming more anemic more quickly than in the past. His doctor agreed that he likely needs the iron more often, so we will try to do every 4 weeks or so. We had planned to go to Cincinnati at the start of this past week for more grafting, but our flight was canceled due to snow, so we were unable. We also would have done his next iron infusion at that time, but now will have to wait until March 10th, unless we can get it done in MN.

We fly out Friday to MN for the initial consults and tests for their bone marrow transplant trial for severe EB. We will be seeing several doctors/specialists, as well as having skin biopsies, a bone marrow biopsy (to compare to his abnormal one from last summer), and possibly a GI scope and esophogeal dilation. Raul is excited about this trip, as he has big plans for everything he wants to see/do at the Mall of America! He is also hoping to meet at least one other child with EB, and somewhat understands that there is a doctor there that may be able to help his "owies" (though I have made sure to explain that this trip is just to talk to the doctors, we would have to go back for any treatment).

Happy Valentine's Day!

Raul got lots of valentines from his classes at school, and also made several Valentine's crafts! Today he got to pick out a special balloon at the grocery store, which he was very excited about! We got a surprise Valentine's treat from The Butterfly Fund, too, which was much appreciated!