Just a quick update . We will be traveling back to Boston next Thursday. Matthew will be admitted to MGH. He will be given IV fluids overnight and is scheduled for both a J-tube and G-tube change. He will also be having a quick sigmoidoscopy done to examine his rectum. He has been having the same type of lower abdominal pressure that he was having before his colon was removed. He has also been passing bloody mucus like stool almost daily. This shouldn't be happening without his colon. We had a stool test done last month and got the results back last week. The test was a Calprotectin test. It looks for inflammation within the bowels. The normal range would be anything less than 50. Matthew's came back extremely high at greater than 2500. This would explain why he feels the pressure. Again, he isn't complaining about it and says it doesn't hurt. His pain tolerance is definitely that of a Super Hero.


www.facebook.com/mightymatthewcech

A "Mighty" Hero

Matthew just spend a very unexpected and long month in Boston. Which should have been a 2 day trip for some testing, ending up being 30 days. Matthew had emergency surgery to remove his colon and appendix. He had 8 sedated procedures and a few unexpected complications, but today he is home. This video is a glimpse into the obstacles that he faced and fought through. It's also to "Thank" all who have supported and helped our whole family through this pretty big bump in the road.





A "Mighty" Hero

We will be traveling back to Boston later this week for the first of three trips in the next month. Matthew will be having his motility studies done on Friday. We will also be seeing a genetic counselor and then have our blood drawn for the Whole Exome Sequencing testing. This is a very expensive test that took us a long time to get approved by both the hospital and Matthew's insurance . We are glad that it's finally getting done.It's been almost 3 years.  We are trying to also fit in an orthopedic clinic appointment while there. Matthew has been having increased foot pain in both feet now. He has been limping off an on daily.


The second trip will be on Matthew's Birthday in November. Matthew will be having a repeat gastric emptying test done. He is super happy that he will be eating radioactive oatmeal for the testing. He said its a great Birthday treat. Only Matthew would think that radioactive oatmeal sounds good. We will be staying through Saturday the 7th to see the Fenway Spartan Race. A team of "Mighty Matthews" will be participating in the race . It should be pretty cool to see .

Our third trip is also in November. Matthew has some follow-up appointments with Cardiology, Metabolism, Complex Care and possibly Nephrology.

I put together a new video and used it for Mito awareness week.

https://youtu.be/9X84sXDQ22E

Also, Matthew attended a family camp weekend a few weeks ago and performed in the talent show with his new freinds .

https://youtu.be/f7hXNN-p8BE

Only 2 more days. Lets see how many votes Matthew can get. :) Just click the link below and click on the blue heart to vote . Thanks everyone http://bit.ly/1QNRuNW

http://bit.ly/1QNRuNW

Matthew is one of only 12 Semi finalists in a Disney Kids Remember video contest. The video with the most votes at the end of the 2 week period wins. The prize is a family trip back to Disney World. This would be so cool if Matthew could win. All you need to do is click on the link and go to the semi finalist gallery and click on “Prince Mighty Matthew’s” video. He is wearing a blue shirt and hat. Click the “Vote “ button on the bottom left of the video. You can vote once a day. Please share with friends and family. There are only 7 days left to vote. Thanks so much

 

Spring has finally arrived here . The snow has melted and buds are on the trees. I would like it to be a little warmer, but its better than it was a few weeks ago. Matthew has been out a few times with the warmer weather. Spring is his favorite season.

We were in Boston last Tuesday through Friday for Matthew's GI appointment and his tube change surgery. His GI appointment went ok as far as his growth goes. He is now 10 1/2 yrs old, 46 inches tall and 40 pounds. :)  He had an x-ray of his abdomen which showed stool in his rectum. Although he has an ileostomy , he should pass small amounts of stool normally every few weeks or so. We have been a little concerned since he has not passed any stool in over 6 months. He had a fecal impaction removed surgically  at the beginning of October and has not pooped since. It looks like the stool is again collecting in his rectum. We will have to follow up with GI if he is not able to pass it using suppositories( they typically don't work on Matthew).

In true Matthew fashion there was a problem with the tube change. We spoke to the surgeon who said the tube change took longer than expected due to an issue with the tube. He said Matthew did well but the tube they were going to use tore while he was checking it. There is a metal piece that is used to flatten the tube when it gets inserted. The surgeon put the metal piece through the tube. Luckily he checked it beforehand, otherwise he could have put the metal piece through the intestine. Part of the delay was them trying to find another tube that was similar to the damaged one. They didn't have the one he was supposed to get so they were forced to insert a different brand. Matthew has used this brand before but it gave him problems with infections and granulation tissue growth. He's had the new tube for four days and he's already having issues with leakage from the stoma and skin irritation. Hopefully this will clear up but we will be contacting the surgeon's office to let them know what is going on.
We go back to Boston in a couple of weeks to see Endocrine and Physiatry (Rehab doctor). We'll be meeting with the Endocrine doctor to discuss some concerns we have with his glucose. We are hoping to allow him to eat some soft foods and clamp his G-tube. In the past when we've done this his sugars get really high. We want to have a plan in place if we decide to do this.
Matthew is going to be going to middle school in the fall and we will be meeting with the rehab doctor to see if there is some things we can do to help his endurance. We are looking at Matthew having a shortened school day. He will also continue to use his power chair to travel throughout the school like he does now. He will need to focus his energy on his academics since he will be switching classes, and will have more work to do. It's hard to believe that he is almost out of Elementary school.

Another busy month has gone by. We are still waiting for some signs of Spring here in the North Country. Things are finally starting to thaw and hopefully SOON it will warm up. Matthew has been feeling ok other than a lingering cold and stuffy nose. It seems to have stuck around for most of the  fall /winter.
Tomorrow, he is having casts made for his new AFO legs braces. He picked out a pretty cool pattern for them and has his fingers crossed that Jeff (his orthotist) can order it. We will  also be traveling back to Boston in April for more follow-up appointments. He will be seeing GI, Endocrine, Metabolism and a Rehab doctor.

Matthew sang and  danced the night away this past Saturday. His favorite band , Glengarry Bhoys  were in town. Sunday was spent resting and recharging. He says a day of rest is all worth it.

"I Lived"

"I Lived"

"You can come to me"

Happy Holidays to everyone. We had a busy but nice Christmas week with family and friends.










 Matthew has not been feeling the best so far this week. His sodium was low again just before Christmas and has continued to be low. We tried to have his labs done on Sunday since his eyes were looking pretty sunken in, 







 increased output and a belly ache, but they were not able to get any blood. We ran half pedialyte /half formula over night on Sunday and had his labd redrawn yesterday . His sodium was still low along with a low Chloride level. We spoke to his doctors and we came up with a plan to continue the pedialyte/formula again overnight and see how he does. So far this morning he is looking much better. He still has a belly ache, but he looks more hydrated. We will try to decrease the pedialyte and increase his formula back and see how he tolerates it. If he gets worse or looks more dehydrated, we will probably have to get some IV fluids to re-balance his system.
On a more positive note, Matthew had his ear appointment yesterday .His ears look great  and his hearing is perfect :) Today , he also has his first Orthodontist appointment. We know he will need braces but checking to see if he is ready for them.
Thanks to everyone who has sent Matthew Happy Mail and holiday greetings. Matthew loves reading the post cards, cards and letters from all over the world.

We are home from Boston. It was a long but pretty productive week mixed with some fun.

 
 
 
 
 
 
 
 

First the medical update and then the fun stuff.
Complex Care- appointment went well. Matthew was 38 1/2 pounds , and 45 3/4 inches tall. Matthew will be getting referred to physiatrist to assist with strengthening his muscles to possible help increase his endurance. Since our Mito doctor has left Boston, they have agreed to help us with school and insurance letters.
GI- This was a follow up to his fecal impaction from a few weeks ago. Overall the doctor is pleased with how Matthew is doing. He continues to very slowly gain weight and grow taller. He would like to see more growth but he is happy that Matthew's growth charts are following a nice curve and Matthew hasn't lost weight or plateaued like he has in the past. We talked about ways to prevent future impactions, two being enemas or physical therapy. We did find out that there are some new supplements similar to the Polycose that Matthew was on before. Polycose had been discontinued and we were given samples of 2 different brands to try and see if Matthew can tolerate them. This allows us, if Matthew tolerates it, to add more calories to his feeds without increasing the rate or volume.

Metabolism - Matthew's doctor also agreed to help with school and insurance letters. The main focus of the visit was on the testing Matthew had done for OPA1 which the doctor thought might be the genetic reason for Matthew's Mitochondrial Disease. The testing came back ok showing he didn't have this. The doctor ordered the Gene Exome testing for Matthew which tests over 22,000 genes. We had been part of a research study which our entire family had been tested but we never received results. The doctor hadn't received them either and since the test is now available commercially he wants it done. Matthew had the labs drawn and we are waiting to hear if the rest of us need to be tested.

Cardiology - Matthew had an Echo and EKG done and saw the doctor. The Echo and EKG were normal and we discussed some issues Matthew has been having with chest cramping and palpitations. She didn't think there was anything to worry about, but she is ordering a 24 hour monitor just to be on the safe side.
Orthopedics - The easiest appointment we have but one of the most frustrating because they are always way behind (this time 1 1/2 hours). Everything looks good, Matthew only has to wear his AFO's when he is going be active for a long time (gym, recess, walks). His current ones still fit but we have to get them adjusted since he has developed a bony growth "pump bumps" on both ankles.
Matthew has started a new medicine prescribed by his Nephrologist, sodium chloride, which is salt water. He has been having problems with low sodium and chloride recently. His doctor is hoping that this will correct these issues and also force his body to hold onto more fluid which would help with his chronically dehydrated state. If it works we might be able to decrease his potassium supplement and in turn improve his motility and his absorption of calories. 
Dentist- Matthew had his two incisors removed yesterday. His mouth is small and there is no room for his adult teeth to come in. He has had 9 out 12 baby teeth removed the past year and a half by his dentist.


Now for the fun stuff !
-We visited Hilliards House of candy when we first got to Boston. This is Matthew's favorite candy store and the only candy store that makes Rose Mints that Matthew can eat :)





- We were able to visit the Stone Zoo where Matthew was able to see a real Tree Kangaroo named Kakota. Tree Kangaroos have been Matthew's new favorite animal since he read about them last month in school .We practically had the zoo to ourselves since its almost winter and it was a school day when we were there.

- Matthew was able to eat a small sliver of his Icing Smiles cake that was delivered to the Yawkey Family Inn where we were staying. The baker, Stephanie was super nice and the cake was great. Matthew ended up having a big upset stomach after he ate it, but he said it was worth it just to taste real food. I was able to vent most of it  back out through his G-tube and he felt much better .



-We visited Sarah at MIT. She took Matthew up to see the Shutter Lab. Matthew loved it and had a great time. Its always a fun time when we see Sarah.
-The new Seacrest Studios opened up on Friday. We were caught up in Matthew's last appointment and missed seeing Ryan Seacrest and Usher, but Matthew was able to be a guest in the studio. He was on air and televised for about 45 minutes and was not shy at all. That kid is made for entertainment. He loved every minute of it.

Matthew had a wonderful Birthday weekend. Members from SUNY Plattsburgh Baseball team stopped over on Thursday night to wish him a Happy 10th Birthday. They stayed and visited for awhile and even had a mini dance off. We had a small family party on Saturday and Matthew took a few of his friends to see Big Hero 6 on Sunday. He loved the movie and now is interested in trying to build his very own robot. :)

We leave for Boston in the morning. Matthew has his routine appointments and testing through Friday. Friday afternoon we will be driving to Albany for the night and then off to Glens Falls Saturday morning. Matthew will be having his two incisors removed by his dentist. He needs some room for his adult teeth to come in.

 Its going to be a busy week medically but we are trying hard to fit some fun things in while we are there. Matthew will have an Icing Smiles Cake on Thursday. This will be a "Matthew Friendly" cake that he will be able to taste and maybe eat a small piece.  We are also going to the Stone Zoo to see a Tree Kangaroo. Matthew has been reading about Tree kangaroos and wanted one for his Birthday. We found out that they have one at The Stone Zoo which is only 9 miles from where we are staying. Since its not possible to actually own one, he will at least be able to see a real live one at the zoo. :) He is super excited.

It's been a roller-coaster kind of week. We left for Boston last Friday. Saturday, we had a wonderful time at the Binkeez/Peaches Neet Feet Superhero party and then dinner with family and friends. The MitoAction walk was on Sunday and it was a beautiful, chilly but sunny day. They had a great turnout and everyone had fun. We stayed in Boston through Tuesday for a few appointments and tests . Below is a summary of how the appointments went.

 

 

1) Dr. F(renal) had talked to us about adding sodium to Matthew’s regimen but wanted to wait until we saw the other providers before doing so.

2) Matthew had an Uroflow test and Kidney/Bladder ultrasound done and then saw Dr. E. He said both tests looked pretty good. His bladder doesn't squeeze very strong but, he said he is able to empty it completely . It just takes a little longer to do so. His only concern was Matthew was not peeing enough during the day. He wants us to make him try and go more even if he doesn't void

) The contrast enema was ordered because Matthew has been complaining of lower abdominal pressure and that he always feels like he has to “poop”. He will sit on the toilet sometimes as long as 2 hours. According to the MGH radiologist and Dr. BG there is a fist sized/baseball sized mass in Matthew’s colon near his rectum. They believe it is either a mucus plug or impacted stool . One of the GI Fellows at MGH tried to manually remove it but was unsuccessful. Dr. BG is scheduling a procedure with sedation to remove the mass.

4) Matthew had an allergic reaction to the contrast on our way home from Boston. He broke out in a rash and hives  . He has been on Benadryl since the reaction and has been seen by his PCP who wants us to continue the Benadryl for the next 3 – 5 days. He was covered in hives for almost three days.

Matthew missed school Mon-Thurs , but was able to go back today . He will be missing more school next week when we go back to Boston.

This weekend we are going to try and relax and not think too much about the busy week ahead. We will also be going to Potsdam tomorrow to visit Bradley at college for Family weekend.

Mighty Matthew

    Click the link below for Matthew's new video
             Mighty Matthew

I can not believe it's almost August already. The summer is flying by. Matthew loves his new adaptive bike. He has also been back in the pool and relearning how to swim a bit. He is able to swim underwater about 6 feet back to the ladder. He enjoyed camping even though he came back home at night. It was easier to do his meds etc at home. A good share of his summer days in between outings has been spent relaxing on the couch with his ipad and beanie babies.

Matthew had allergy testing done last week . He was tested for environmental things like trees, grass, dogs, cats etc and he did not show an allergy to any of them . He was also retested for eggs and nuts since he had an allergic reaction a few years ago from cracking the shells of some mixed nuts during the holidays. We were never sure what nut caused the reaction. Well, he still shows an allergy to eggs but he reacted to the nuts more. Within 5 minutes, the skin test marks were big hives and after 10 minutes , the hives started to blend into each other and he also started to get hives on other parts of his arm. They concluded that he reacted to all tree nuts, peanuts and  coconut.  He was very itchy and said the hives hurt when he touched them. They eventually started to go away and after two days , they were gone.
Tomorrow , Matthew is having a Gastric Emptying Scan done at our local hospital. His last scan was over 4 years ago . Matthew so desperately wants to be able to eat solid food even if just a few tiny bites. This test will give us a little more updated  information about how well if anything his stomach empties food. He is super excited to be allowed to eat oatmeal tomorrow for the test even knowing that it is probably going to cause stomach pain. We will not be able to vent his stomach at all until the test is complete. This usually is at least 4 hours long.
We will also be going to Boston on August 13. It will be a short stay for just a few appointments. He will have more appointments in September and again in December.