Minnesota Update

I know a lot of people are curious about how things are going with the BMT process, so I wanted to give an update! 

Shortly after Raul was approved for the trial, the hospital requested that three possible donors undergo further lab work to determine who the best match would be. Two of the potential donors did this in late June, and one in early July. Then those results were submitted to Dr. Tolar to choose the best match. All were 8/8 matches, and two had similar secondary results, which is good as that means we have a backup should something not work out with the first. 

Today, after a few days of insurance confusion, a request for donor work up was made! It will take at least 1-2 weeks for us to get a response to that request. Minnesota has requested a donor clearance date of 9/9, meaning the donor would need their physical a week prior to that. If the donor clears their physical, that would mean we would likely travel the following week! If not, then they would have to approach the next donor and go through the workup process again. Once we go out there Raul will have his own workup week, and if all is okay he would begin the BMT process after that (likely 5 day of chemo, one of radiation, and then transplant). Of course this timeline could still be pushed back if there are any delays either on the donor side or with Raul's health, but hopefully around mid-September we will be going to MN!

Help Support Team EBRP and Raul!

Please consider supporting the EBRP (EB Research Partnership), an organization dedicated to funding research towards treatment, and ultimately a cure, for EB! My sister and mom will be taking part in a 5k, and my sister will also be taking part in the 10 mile race, at the Twin Cities Marathon in October, to raise money for the EBRP. This is extra special for us, as Raul will be receiving treatment in MN for several months. Please help us fund the cure we so desperately need!

Donate here:

 https://www.crowdrise.com/ebr-2015medtronictcm/fundraiser/ruhls 

Museum/Fire Station

We had a busy day today! This morning we went to a children's museum that was more half indoor playground/half museum. Raul really enjoyed it, and checked out many of the different areas. His favorite was the "heroes" room, of course! He dressed up in a fire suit, played with vehicles, and read books. At the museum he also did a few building activities, sat on the wooden train, and explored all the different structures. We got there right at the end of a reptile program, so he even got to see a tiny alligator up close! 

After lunch and a few errands we decided that we would stop at the fire station. We had perfect timing, as when we got there they had the ladder truck out with the hose spraying up top. It turned out that they had a new firefighter training, and they were teaching him how to use the ladder truck. We watched them for a while, and got to talk to several firefighters. They brought Raul a hat (and explained the different colors among their hats), and even showed us some of the tricks the ladder truck can do while driving. We also looked at the various other vehicles inside the station (firetrucks, ambulances, boats, a truck for driving in the forest, etc.). It was definitely a very interactive visit, which was great!

Beach Day

We are on Cape Cod for a few days, and today we went to the beach. Raul had a great time, and could have stayed out floating on the beach wheelchair all day! He also played in the sand with a few trucks, and made a sand castle. When we got back to the hotel we went down to the pool, but couldn't convince Raul to go in. He did sit for a minute on my mom's lap on the stairs, but mostly just wanted to watch us swim around and do tricks. At the end he got excited about playing with a pool noodle at the edge of the pool. Then tonight he played a lot with his Duplo at my grandmother's house. Yesterday's outing was to the library, as he loves the trains/train table at this library. He played trains nicely with a little girl for a while, and also scooted around the children's room looking for good books to borrow. 

May/June Update

The last couple months have been pretty busy medically. Raul's anemia has been hard to manage over the past few months, so he has had a lot of lab work, iron infusions, and blood transfusions. His bone marrow biopsy came back similar to the one from last summer, so that did not give us any answers for the anemia. It is most likely just progression of his severe EB. In the past two months he has had two iron infusions and two blood transfusions, and is still quite anemic. His hematologist also mentioned back in May that if he is ever having other imaging done, she would like to add on the scans to check for iron deposits, just to make sure he isn't having any issues with that. He also continues to have fevers that don't always have a clear source. 

Luckily he likes going to the infusion center, likes the nurses, and of course likes all their toys and activities! At his last blood transfusion the music therapy lady came in to see Raul. First she sang a song while Raul tapped on a drum and the baby and I shook a maraca, but then she and Raul did the cutest Twinkle Twinkle duet ever! She played guitar and they both sang. He will never do it for me if I ask, but I love listening to him sing while he is playing at home, and it was a treat to hear him sing with her when she asked!

At his last blood transfusion we were discussing that he was getting the blood to help his energy and make him strong, so he started saying he was going to be "strong enough to lift the heaviest rock into the biggest mining truck"! He says a lot of funny phrases these days. One of our favorites is one he uses when you ask a question that he either can't or doesn't want to answer. He will say, "I can't know, maybe ask me later". Then the other night he was still awake in bed when I went to fill his formula for overnight, so I asked him why he was awake, and he matter-of-factly stated, "I forgot to close my eyes".

Early in June he had a new type of grafting product placed on his leg wounds and his right side of his torso. The product was Matristem, and while we were hopeful, I did not see a difference in the wounds. We had the option of trying it a second time, but we have decided not to at this time as it requires anesthsia/OR time, casts, and painful in-office bandage changes as follow-up afterwards. Since he has been approved for the BMT now, we are hopeful that we will be heading out to MN in the next couple months, and that the BMT will help his severe wounds. 

Shortly after the grafting, Raul had another flare of extreme joint pain. This seems to happen when he has infections or viruses. His joints, especially his right knee, get excruciatingly painful. He has trouble sleeping as any time he moves he startles from the pain (can be up every half hour all night), and riding in the stroller or car is agonizing because of bumps. We have not found anything that really helps these episodes, we just have to wait them out. 

Raul is now out of school for the summer. He was going to attend the summer school program, but we have decided not to do that as we do not want him exposed to extra germs/viruses if he is going to be having the BMT soon. In June we had kindergarten visiting day at the school he will attend next year. It is our neighborhood school, and he will be in a regular classroom (still with an aide and IEP). He is excited that we can walk to school sometimes! For the visiting day we spent about an hour at the school. Raul went with all the other children to visit a classroom and do a picture frame craft, while I stayed in the gym for the parent meeting. It was nice to learn more about the school. 

 Raul likes doing schoolwork, so we have workbooks that we do at home when he isn't in school. We have been using Kindergarten workbooks, and he does great with them! Raul continues to do really well with his power chair, and is a great driver. In May we got a mini van with a wheelchair ramp so that we can take "big red" on more outings. We recently had our foster baby leave, and Raul is excited to have some baby-free time now. In May we did also visit one of my past foster babies who Raul likes to see. She has serious medical needs, and Raul is very sweet with her! 

Summer Haircut

When we got home today Raul rounded up all his fire trucks, police cars, and ambulances to make his own fire truck parade like the one we saw in Rockport. Then we took him to get a summer haircut, as this week is supposed to be really hot, and he gets super sweaty! He freaked out a bit when we got there, but was totally fine once the lady started cutting his hair. His hair has not been this short since he was two!

Happy Fourth of July!

Our week of vacation is coming to an end, and Raul is sad for it to be over! We had a great week filled with many trips to the playground, visiting the fire station, watching a house's foundation be demolished by an excavator, drives around town, a visit to the train station, and lots of playing at home! Unfortunately, Friday morning Raul woke up with a scratched eye,  so that was more of a low key day. It was still bothering him today, but definitely improved and he could open it okay. 

 Today Raul took a nice nap so that he could stay up tonight for the bonfire in town. We went to a neighbor's house for a cookout, but it was raining so we didn't spend much time out at the party. Raul and I did play a beanbag toss game that he liked. Then we headed to the fire truck parade. This is something that he has been waiting all year for! The noise bothered him a bit more this year, but overall he liked it. When we got home he used the candy he had gotten during the parade to play fire truck parade (the candies were various vehicles). We had brought a fire hat from home for him to wear, and then also decorated his wheelchair to be engine #1, just like the Rockport one. 

Then tonight we walked into town to see the giant bonfire, which is something we hadn't done before. The bonfire is put on by the fire department, and we had watched them making the pile during the week. For some reason it is always topped with an outhouse. The other good part about going to the bonfire was that all the firetrucks and ambulances from the parade were parked down there so we could check them out! 

At first the fire was having trouble catching on our side, but then it went crazy! After a little while they turn the hoses on, but they didn't seem to make much of a difference. We didn't get too close, but the heat coming off of it was intense even at a distance. We also got to see several fireworks displays over the harbor while watching the fire. When we left the fire did not appear to be slowing down any!