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.

 

It was a crazy busy month of June. Our oldest son Bradley, graduated from High School. He is off to Suny Potsdam in August with a full scholarship . He will not have to take out any loans or pay anything out of pocket. His education will be covered 100% including his dorm room and meals. We are very proud of all of his hard work . Steven will be going into 10th grade and Matthew in 5th. On the last day of School Matthew rode home in style. Our friends from the Essex County Sheriff's Department picked Matthew up in a Humvee . :) Matthew  though it was the coolest ride home. :)
Matthew is enjoying the first week of summer break. The weather here has been super hot and humid (I love it) , so Matthew has been relaxing in the pool and sitting by the fans. He was swimming a little bit last year , but can not swim yet this year. I tried a few times with him , but he can't keep his head up out of the water.
On Saturday Matthew will be receiving a new bright green "Mighty Matthew" adaptive bike from Preston's March for Energy. We will be meeting Preston and his family so they can present him with his bike. Matthew has tried for years to ride regular tricycles , but he never gets more than a 100 feet before he can no longer pedal it. This bike will have a pulley system that should allow him to use less energy and to ride with his friends. Our friends from Goss Dodge Chrysler will also be there to see Matthew receive his bike.
On a medical note, Matthew's labs have been off more than his usual the past month. His sodium which is always low , has been even lower. A few weeks ago it was 130.  He also was treated for a J tube and G tube infection. They were pretty nasty looking and building up quite a bit of bleeding granulation tissue. His J tube looks much better, but the G tube still has some healing to do.
A few weeks ago, Matthew was complaining of increased hunger. He was sneaking foods and drinks and then telling us, so we could syringe the food back out through his G tube. I was also called two days in a row at work while he was in school. He was caught twice eating food out of the garbage. As much as we wanted to be angry with him, its too hard when he cries and says he is so hungry. He tells us that his brain knows what the food will do to his stomach , but its his heart that tells him to take it. 
We contacted his GI doctor and with his approval, stopped giving him his Ciproheptadine. One of the side effects of this med is increased hunger.  This medication is also used to dull stomach pain . The hunger issue seems to be better now that he is off of it, but he now has stomach pains and pressure and is complaining of increased thirst. We spoke to his GI doctor once again and he started Matthew on Gabapentin. He started him on a low dose and will increase depending on how handles it. So far , Matthew's only side effects from it are headaches. Since its only been two days , we are hoping the headaches stop as he gets used to the med.