Day -6

Today was Raul's first day of chemo, and he did great! He was actually particularly happy today! He started increased IV fluids last night, as the Cytoxin can be hard on the bladder. He has to pee a certain amount every two hours to make sure it is being flushed out of the bladder (this will go until noon tomorrow). He didn't pee enough the first two hours, so got half a dose of lasix, which seemed to solve the problem! He also received an IV medication during the day to help protect his bladder.

The other chemo med. he got today was Fludarabine. The Fludarabine will be given daily for 5 days, while the Cytoxin was just one dose. He also started receiving a preventative anti-fungal medication, and got a continual zofran drip (which was a huge help for his nausea from his antibiotics).

He had a great day, and was friendly and talkative with everyone who came in. He got to have PT today, and had fun playing on his floor mats. We did have to do a bandage change with a sponge bath, and will have to do that daily during the chemo. Raul did spike a fever late in the day today, so they drew blood cultures. Tonight the nausea seems to be hitting him a but more, but not too severely yet.

Day -7

My dad just visited for a few days, so we had fun going out on pass in the evenings. Sunday we had dinner at the RMH then went to a nearby accessible playground. Raul had a lot of fun, particularly on the swing. He was doing a lot of singing and kept saying, "this is the best day ever!". Every day since he has talked about it and said he wants to go back!

Yesterday during the day things were pretty quiet with just BMT rounds and a visit from PT. In the evening we walked to visit a fire station, which unfortunately was closed up tightly, but later as we were walking a fire truck drove by us with their lights going so Raul was satisfied! We got word while walking around that our insurance was denying the transplant, but of course it was then after hours at our insurance company so we couldn't reach anyone there. We also had dinner out at a restaurant before heading back. 

Today was a very long and stressful day trying to figure out the insurance denial. After many phone calls and various approaches, we got word that BCBS had now APPROVED the transplant! We still don't know exactly what, if anything, was wrong in the first place. It sounds like it may have just been a big error. So after getting that good news we went back to the RMH for dinner and play time for our last night out before starting chemo! We are officially on day -7 today for Raul's transplant! The days before transplant are negative numbers, transplant day is 0, and the days after that are positive numbers.

Inpatient Day 11

Yesterday Raul had OT in the morning, had rounds with the BMT team, and got to meet a bunch of therapy dogs in the lobby. There was one dog that could bowl with a foam bowling set, which Raul thought was pretty cool! In the late afternoon we got another pass to go back to the RMH. We had dinner, did laundry, and played in the room and playroom. We also got to meet Zuzia Macheta and her family. It was nice for Raul to be able to see another older child with EB, and he also thought it was pretty cool that she had the same stroller! 

Raul has been asking the nurses for some funny things when they ask if he needs anything, and yesterday starting saying he needed something "funny and amazing". The overnight nurse asked about what animals he liked, and he said camels, so when we woke up this morning they had printed some funny camel pictures for him!

Inpatient Days 9-10

Yesterday Raul had a repeat MRI of his legs done in the morning. They wanted to make sure there was not an active infection that could delay his transplant, and thankfully they came back late in the day to say the MRI showed no sign of oseomyelitis! However, he will be staying on the antibiotics through his transplant. Today they were able to change one of the antibiotics to something less potent, as his cultures came back as being sensitive to this more gentle antibiotic (and oddly, one was resistant to the powerful antibiotic!). 

We also saw the pain team, and are continuing to work on a good plan for Raul. Yesterday we changed Raul's bandages after his MRI while he was in the PACU and it was better than Tuesday, but he was still pretty awake and unhappy. 

Today we also got to get a pass to go back to the RMH for dinner and some time to play there. We had fun getting out for a break from the hospital! Raul has been getting used to the hospital, and is warming up the doctors and nurses. Tonight when we got back I was telling the nurse about how Raul had told the doctors they could work as a team to find him a toy, and she found him this super cool fire truck pillow! He was very excited!

Inpatient Day 8

We had so many people/teams visit us today that I can barely remember them all! ID, Dermatology, Pain Management, BMT, PT, OT, Speech, Care Partners, and a woman who brings by new books weekly. Most teams addressed the PACU fiaso from yesterday, and we are hoping tomorrow will be better when he goes for his MRI. The pain team is going to prescribe a new pain medication that we have not tried before for more acute pain. ID said his skin cultures came back with one bacteria, and luckily his current antibiotics cover that one (oddly it wasn't his most common one!). PT/OT/Speech talked and did assessments. Some will start services now, and some will wait until after transplant.

A volunteer from Care Partners came by with a huge gift bag to welcome us to the hospital. Raul was VERY excited to go through it! He also got three new books from the woman who brings by books, and a Love Your Melon hat from a table they set up in the lobby this morning. As you could probably guess, he considered this to be a good day with all the gifts! We also walked in the halls a bit, played Duplo on our play mat, played iPad, did a little school work, and had a failed nap attempt due to all the visitors I listed above!

Inpatient Day 7

We had a long day today. We did Raul's GFR test this morning, and thankfully I heard tonight that his results were good, which means his kidneys are functioning well. The test involved injecting a small amount of radioactive fluid through his central line, and then after 2 hours his blood was drawn every 40 minutes for several hours

After rounds I met with Dr. Wagner for Raul's exit conference and to sign all the consents. This involved going over his results from various tests, the transplant process, a few additional studies, any potential risks, and information specific to this BMT protocol. It was actually quite interesting, and it was nice to learn about the treatment from Dr. Wagner since he was one of the original doctors who started the trials for EB. Raul's echo, EKG, chest CT, and labs were all good to continue on with the transplant process. He does have past exposure to several viruses that they will need to watch for closely after transplant. 

Raul and his donor are full matches, which I was told is not too common with the EB patients with unrelated donors. They are 8/8 matches with their HLA typing and also have the same blood type. This should help reduce the chances of some complications after transplant, and having the same blood type will help his red blood cells recover more quickly. 

We also met briefly again with the pain team, and made a plan for which pain medicines to try/when to try them. This afternoon we headed back to the OR for a few more procedures. Unfortunately, the OR was running two hours behind again, but luckily Raul fell asleep while we waited. He first had a GI scope, which was surprisingly good! No erosions or major inflammation, just more mild inflammation in the esophogus and in both his stomach and esophagus if the camera touched the tissue it bled. They did note a high stricture, so IR came and did a balloon dilitation of that next.

After that a PA from the BMT clinic came to do a few tests for the trial. This involved several tiny skin biopsies on his thigh, a blister formation test, and photos of his whole body. The blister test tests how quickly someone makes a blister when it is induced with a special suction device. Most healthy people would take around an hour, a carrier may take around 30 minutes, and someone with EB might take up to 10 minutes (though usually much lower with severe EB). I asked how long Raul's test had taken, and he said the site began to blister almost immediately. 

Unfortunately, our PACU experience was not as positive this time. They allowed Raul to wake up fully before even calling me back to re-bandage him, so he inflicted some new wounds on himself itching and thrashing and worsened some current wounds (his entire body was unbandaged). They did finally give him more medication, but it didn't kick in until I was just about finished re-doing the bandages. Thankfully I do not think he really remembers that happening! Tonight he was back to wanting to watch cartoons, play on his iPad, and eating fruit pouches after a long day of being NPO!

Inpatient Day 6

Our day started off early with a 5:30 am blood transfusion due to a low hemoglobin level. Around 8 we had Raul's radiation consult down in the radiation department. We mostly talked, but at the end he went into the treatment room and had to get a bunch of measurements taken. He was really nervous about that part, but there was an awesome radiation tech that helped a lot! The radiation does not need to be done sedated, but there is no way he could stay still long enough alone in a room, so he will be sedated! 

When we came back to the unit he played with his cars/trucks, pretend snow, and water for a while. The BMT team came in for their rounds, and went over the plans. Later in the day the GI team came up to change his g-tube size to make it easier for their scope tomorrow. We went outside briefly, but Raul decided it was too sunny, so we went back to the room. In the evening orthopedic surgery came by again to see his knees and luckily they agreed with our Boston orthopedic doctors that they would not recommend biopsying the bone. He will have a repeat MRI on Thursday (they couldn't get it tomorrow with everything else). 

Raul also had his first ever "babysitter" today! We signed up to have a Care Partners volunteer come by and sit with Raul so that we could run back to the Ronald McDonald House for more of our stuff. He didn't complain at all when we left, and said he had fun, which was very reassuring as I will most likely need to use them occasionally while here by myself!

Inpatient Days 4-5

Saturday was not too eventful. We met with the BMT team, ID doctor, and GI team. GI will scope him when he is having his other procedures. They want to hold off on switching to a GJ tube for now since he is tolerating a continuous feed (almost to his goal rate), and they have seen more complications in the last year or so with GJ tubes. Raul had a full bandage change, as well as a change of his central line dressing. We are having trouble getting any dressings to stick over it and seal the site. We got to do some laps in the hall Saturday but were not able to go off the floor as Raul had a low grade fever Friday night. Raul had fun in the hallway pretending we were speeding and all the nurses were the police. He also got down on the floor in the room to play with pretend snow.

On Sunday, after Raul got up for the day at 4:30am, we were allowed to get a pass as Raul had been fever free for over 24 hours! At this point he will not be able to be discharged prior to transplant, so it was nice to get some fresh air together! Raul had to get his line dressing changed before we left as it was coming up again, but today's attempt looks promising. Due to his antibiotic schedule we can only have short breaks off the floor, but we had a lot of fun and he was much happier. We took a quick trip to the zoo to check out the giraffes, and Raul ended up riding a little car ride. He wasn't able to do it himself, and I wasn't allowed to stay with him, but the ride attendants were really nice and one of them pushed him around. Raul had a lot of fun, and was so excited that he had done it!

Over the next few days we will have several other consults, and it is looking like the tests/procedures will happen Tuesday at this point.

Inpatient Day 3

Raul had his tunneled catheter central line placed yesterday. Their hope was that they could place a PICC instead until his fevers are fully cleared up, but his arm wounds did not allow for that to happen. They had told us that if they got the PICC it would be a short procedure, and it would be longer if they did the full central line, so we knew when he was in there over an hour that the central line was placed! 

He was super unhappy during the day about being NPO. They had to stop his formula and any eating by mouth at 8am, and they stopped clear liquids at 1pm. Unfortunately, his procedure was delayed, and he didn't go back until after 5pm, so it was a long day! When he woke up he scarfed down a baby food pouch, a bit of drinkable yogurt, and some powerade! We also met with infectious disease and the pain team again, as well as the BMT team and visit from Dr. Tolar! Orthopedic surgery tried to come by at 10pm when he was sleeping and hadn't had his pre-medication and asked to see his legs, but I declined, so they will come back Monday! Thankfully he finally slept well last night!

Inpatient Days 1-2

We have had a change in plans, unfortunately. Raul was admitted to the BMT unit Wednesday due to a high fever. It was 103.3 when he woke up, and when we got to the unit it was 103.8 even though I had given him motrin earlier. He is not happy to be in the hospital, but felt pretty crummy the first day so spent most of the day napping. He was not able to get his central line Wednesday due to the fever, and they wanted him to wait 48 hours from his last fever, but today (Friday) he will be getting either a PICC of his Hickman line as peripheral IVs are just not holding up (he went through 3 in under 24 hours). He only had one fever yesterday, and it was after his bandage change, so it was most likely related to that.

We may switch to a GJ tube when he goes back to the OR next time to help with his nausea and gagging. While here we decreased his formula a lot, so the gagging has improved. Raul has also been drinking a lot by mouth, including powerade and strawberry milk (and even eating some yogurt again, though that has been making him spit up more). We have gotten to meet several other EB moms, which has been nice!

Work Up Day #2

Today was not quite as busy as yesterday, but we were still out from 8-3. We started our day in imaging for a chest CT scan and to start a GFR test. Amazingly, the CT scan was successful without sedation! He lay still, and they had lights/images projecting onto the inside of the scanner for him to watch. They also let him add a sticker to the inside. Then he got to pick a prize from the treasure chest, and of course he chose a car! The next part did not go so well. They did 3 pokes, by two different people, one with ultrasound, and still couldn't get an IV that could have blood drawn from it. The test involves drawing blood every 40 minutes for 4.5 hours, so we were unable to do it today and it has been rescheduled for next week when he will have a central line to use!

 Next we went back up to the Journey clinic and met with the nutritionist, which was quick as we had met with her back in March. After that we met with some BMT doctors to go over a few things and have a quick checkup. I asked about his Hemoglobin from yesterday, and amazingly it was 9.1! Then after that he also offered some more of the lab results and let us know that his EKG was okay and chest CT was good other than some inflammed lymph nodes (which is not a surprise/not concerning as he has large open wounds on his upper arms). His WBC was still high, but he was going to continue on antibiotics another two weeks anyway. We will find out tomorrow for sure if we are going to continue with oral or switch to IV antibiotics. Child life gave him a stuffed animal with a central line for him to learn about the central line since he was so bothered by the stuffed doll they showed him yesterday! 

After that meeting we had a bit of a break so we went on a little drive to get out of the hospital! We went down to Minehaha Park, but did not end up getting out as Raul was grumpy and not feeling well. Then we stopped at a grocery store for some lunch before heading back for the last appointments. Raul got an echo, and hated the gel, but did well. He picked some star shaped sunglasses as his prize after his echo. Our last meeting was central line training, which was pretty quick. We had a while to wait in the waiting room for parent education, but were the only ones there so it was nice and peaceful! Then we came back to the RMH and Raul had a nap before we went downstairs for dinner. He was exhausted today, and his stomach is not tolerating his antibiotics well at the moment, so he is already back to sleep for the night!

Tomorrow Raul will be going to the OR to have his central line placed and get skin biopsies.

Work Up Day #1

Today was Raul's first day of his work up week for transplant. It was a very long day; we were at the hospital from 8-3:30 for tests and appointments. He had vitals/calendar review with a medical assistant, met with a PA to go over things and for a few consents, got to tour the clinic and find toys with child life, a central line consult with radiology, a consult with integrated therapy, a meeting with Care Partners, a teaching with child life about our blood/the cells in our blood and what a central line looks like, a meeting with social work, a meeting with pharmacy, an EKG, and a blood draw.

He was a bit nervous, and very exhausted as he didn't sleep much last night, but still did well! He was great getting both the EKG and labs done, and he got a token to pick out a prize after his blood draw. He fell asleep for about 5 minutes during our last meeting, but woke right back up. In the middle we had a little break so got to run down for lunch and spend a little while in the library. They had a therapy dog in the library that you could read to, and Raul pet him a little. He also was able to pick a couple movies and books to borrow! When we got back to the RMH we met the house dog and then walked to a restaurant for dinner. Now we are about to do bandages and then hoping to get to bed early! Raul is already very much done with meetings, so the rest of the week might be interesting!

Thomas Land at Edaville Railroad

This afternoon we went to Thomas Land at Edaville Railroad! Raul had a good nap in the car on the way there, so then was excited to see everything. He got to meet Sir Topham Hatt, check out the trains in Tidmouth Sheds, look at all the rides (he didn't want to go on them, just watch), ride on Harold, play with the train tables, sit on Percy, wave to Thomas as he went by, and watch a show where the trains at Tidmouth Sheds talked. He also liked the Thomas music playing over the loud speakers in the park. His favorite things seemed to be the train tables and the show!

MN Update

On August 12th we got the news that the donor they contacted for Raul was willing to proceed, and the week of the 17th he had his physical exam! We are very grateful for the donor's willingness to proceed. Raul's donor is a 31 year old man in Europe. On August 28th we got word that the donor had passed their physical exam and had clearance to donate! They are scheduled for a collection date of 10/5, which means Raul's transplant would be 10/6!

Now that the donor has gotten clearance the hospital is working on setting up Raul's workup week. We do not have the schedule yet, but the hospital confirmed today that he will begin his outpatient appointments/tests on 9/14! That means that, if all continues to go as planned, we will leave for MN in 10 or 11 days!