Flipagram - December 30, 2013

Matthew came home early from school this past Tuesday and Wednesday . He was feeling dizzy and having headaches. I brought him to his pediatrician on Wednesday afternoon. She was concerned that his resting HR lying down was around 106 and his standing HR was in the 140's. She wanted us to let his doctors in Boston know since this can be another sign of dehydration. His labs from Monday also showed a low K level and an elevated CO2 . On a good note , he stayed in school full days the past two days. We are holding off on giving him the extra water boluses through his J tube to see if it has anything to do with the dizzy spells. Matthew said that he still feels dizzy, but said its better than it was earlier in the week.

Steven is still not well. He went back to the doctors on Monday and is out of school for another month or until we get his medical issues figured out. He needs a GI and cardiology workup done. He will be going inpatient at our local hospital on Monday. He has lost over 16 pounds, feels dizzy and is not able to eats foods other than a bland diet of bagels, toast etc. We are hoping that we get some answers and that its an easy solution.  He is missing his friends and school.

A few pictures from Matthew's last inpatient stay in Boston

 Matthew's friend Aomar Naittalb from MGH :)

Matthew has not been doing the best in terms of his feeds. We have attempted to get more weight on him by increasing his feeds. He was getting his feeds and 58/hour and over a period of 5 weeks we slowly increased the rate to 61/hour. Unfortunately Matthew has lost a pound during this time. He is almost 9 years old and only 34 pounds. This is our 3rd time trying to get Matthew past this point and he has done the same thing each time.
Terry spoke to Matthew's GI doctor in Boston and he wants to put Matthew in the hospital to see if we can get some weight on him. He had us lower the rate back to 58/hour to see if Matthew can gain back the weight he lost. Matthew's doctor is going to try and think of some different ideas to help Matthew. A different formula, another supplement or possibly putting Matthew on TPN even for a short time. We are hoping to avoid a hospitalization but will do it if there are no other options.
Matthew is still without his Mito Cocktail as his insurance stopped paying for it. Terry is working on some things that may get it covered. We can't afford to pay for it ourselves as it costs $800.00 - $1000.00/month.

Matthew had his overnight sleep study done last week in Vermont. He didn't mind it at all and fell right to sleep. We should get the results this week.

Despite the current medical issues, Matthew is enjoying 4th grade. He likes seeing his friends and he loves his teachers. He also looks forward to weekend play dates with his friend Ava. They are super silly together. Last weekend, they spent the day together in Albany for a Starlight function at the Pottery Place.

                                  

We are in the midst of Mitochondrial Awareness week. We have been posting pictures of Matthew and our friends helping raise awareness for a disease that claims more children than pediatric cancer does. We have some really wonderful pictures of people showing their support of ridding us of this horrible disease.

We are not having the best week ourselves though. We are having a hard time with Matthew's insurance once again. We are still trying to find a way of getting his Mito Cocktail covered. The insurance was covering it and then they decided to change what they will cover. This has affected us but many other people as well. Another problem is getting his feeding supplies to us. Every so often the insurance requires an authorization to continue covering the feeding supplies (formula, bags, tubes, etc). It is always a nightmare because it takes so long to get and Terry spends a lot of time with the insurance and supply company to get this taken care of. We are always very close to running out of his formula and it gets very stressful. If he runs out of food we would have to bring him to the hospital.

 Matthew had his checkup with his pediatrician yesterday who has taken care of him for almost nine years. Just like every visit both the nurse and doctor went over his medicines and allergies. He is allergic to eggs and nuts and a couple of medicines. His pediatrician prescribes Matthew an Epipen for egg exposure.
Matthew has never been able to get the flu vaccine because of his egg allergy. There is a new egg free flu vaccine that doesn't use eggs in the manufacturing of it. Terry asked for the egg free flu vaccine for Matthew because of his egg allergy. He asked 6 or 7 times if the vaccine being brought in was egg free. He was told by the nurse giving the nasal FluMist that it was egg free. He wasn't given a fact sheet on the flu vaccine that Matthew received. I looked online and found out that the vaccine Matthew received is not for people with egg allergies and/or metabolic disorders (Matthew has Mito disease). Matthew had a reaction to the vaccine with muscle spasms, itchy legs and twitching around his mouth(luckily no swelling, hives or shortness of breath). Terry called the pediatrician and we did not get a phone call back from her. Terry called Matthew's metabolic doctor in Boston and was told Matthew needed Benadryl and would need to be brought to the ER if he got worse(he didn't).
Terry called the flu vaccine manufacturer and had to fill out an adverse reaction report because under no circumstances should Matthew have gotten that vaccine. He was told by a pharmacist for the manufacturer that the CDC doesn't recommend nasal FluMist for egg allergic people or people with metabolic disorders because there are NO clinical studies showing if it's safe or not in those kinds of people.
Terry talked to the nurse who gave Matthew the vaccine and was told she didn't know he was allergic to eggs (it's on his chart and Terry asked several times if the vaccine was egg free). The nurse claims Terry asked about an ingredient we have never heard of, Thimerasol a preservative. She said she was sorry for what she did and is glad Matthew is doing ok(he seems to be ok at the moment). Matthew now has a live vaccine inside of him that he should never have gotten. Imagine the fear of not knowing what the hell to expect  because someone screwed up.

Mito Superheros

Hero

We had a very busy weekend in Boston. We left Saturday morning and arrived at our ever so familiar Hotel in Boston. Matthew's Gramma, Papa, Uncle Johnny and "Hat" Sue were also staying the night there. "Hat" Sue gave Matthew a big bag filled with new clothes. Matthew tried on an outfit right away. He also was given two huge boxes filled with freeze pops, lollipops and a few water toys from his Aunt Kathy and Aunt Barbara.

Matthew's big surprise was later that evening. Our friends Nichole and Ava decided at the last minute to drive from Plattsburgh to Boston to surprise Matthew. He was speechless when he opened the hotel room door and saw Ava and Nichole standing there. :)

We had a beautiful day for the MitoAction on Sunday. They raised over 100 thousand dollars. Team Mighty Matthew raised almost 1300 dollars of it. A huge thanks to everyone who joined us or donated. Together, we can raise awareness and help find a cure.




Matthew had an orthopedics appointment on Monday morning. He will getting new Afo's with more padding and support build in. His current ones are only about 8 months old, but they have been hurting his feet.




His renal appointment went ok. Dr F is so very compassionate . He really has been trying to figure out Matthew's complex system, but has no answers for us. We have increased Matthew's formula rate from 58 ml/hr to 60 ml /hr in the past 4 weeks. In that time frame, Matthew had tripled his urine output and lost almost a pound. It makes no sense. Matthew's system is so very fluid sensitive that the slightest change in fluid intake causes his body to go wacky.




We still need to discuss things with Matthew's GI doctor, but the plan is to increase to 61 and see what happens.


Matthew is still not on part of his Mito Cocktail. This is the very expensive compounded one that is over 800 dollars a month. His insurance is currently not covering it.

Matthew will be having his sleep study done on the 26th in Vermont. Hopefully we can figure out why he is up so much during the night.

 

We are back from yet another trip to Boston . 

Matthew saw Urology, Nephrology, Nutritionist, Metabolism, a new GI doctor and an OT from the Augmentative Communication Clinic.

  Urology- Matthew only pees twice a day most days. He has a very large bladder but also does not produce much urine. We had a follow up uroflow done. He was able to empty most of his bladder. They scanned his bladder  when finished and about an ounce remained. The flow was also delayed , but that is common with Mito patients. 

Nephrology- We just had a follow-up with Dr. F who has been in charge of Matthew's GI care for past few months. He didn't want to change anything until we saw GI and see what they come up with first. He had some labs ordered and said that we could discuss things this week.

Augmentative Communication Clinic- They are recommending that Matthew use an iPad for all school work that involves more than writing simple questions and fill in the blanks. He had a full size laptop last year, but it was far too big for his small hands. 

Metabolism- A few of Matthew's meds were adjusted and they are also working on getting his Mito cocktail formulated differently. We have it compounded at out local pharmacy and has been always been covered . He took his last dose of it a week ago today. We are out of it because his insurance is no longer covering it. It would cost us over 700 dollars a month to get it filled. There are four different meds compounded into it and since he gets his meds into his J-tube, he needs liquid form. Terry will have to argue with the insurance this week and see what they say. He may have to file a complaint with the NYS Dept of Health about it.

Nutritionist- She added calcium and Vitamin D to Matthew's list of meds. He is not getting enough Calcium and since he already has osteopenia, she felt he needed the extra supplements. She will be working along with his doctors to see if there is anything that can be added to Matthew's current formula to give him extra calories. The polycose that we are adding currently has been discontinued . She said when supplies run out, we will have to find a replacement. It's not an easy task with Matthew's complicated system.

GI- We saw a new GI doctor. We really liked his personality and he took time to listen to Matthew's very long and detailed history. He also took time to listen to Matthew and joked around with him. He agreed that Matthew is not your typical case. Most mito patients need a lot of extra fluids. Matthew is just the opposite. His system does not thrive on extra fluids. He told us that he needed to discuss things with Matthew's other doctors and that he would try and come up with a plan this week.

  Sweet Eithene's 2 year angel anniversary was on the 8th. We were at BCH that day for an appointment. Matthew let a balloon go in the garden with a note attached. When he let it go, he said out loud' " get ready to catch this Eithene." As chances are, we met Eithene's mom Jessica and Aunt Jill when coming off from the elevator . They were delivering beautiful roses to some of Eithene's favorite spots in the hospital. Jessica gave Matthew one of the roses. He told me that it was special and that he was going to keep it forever. That evening Matthew and our good friend Sarah wrote Eithene's name out in seashells at the beach in Cape Cod

We did fit in some fun during the week as well. We brought our older two boys along and made a mini summer vacation in between all of the appointments and testing.

 We visited Hilliards House of Candy where Matthew bought his favorite rose mints and the older boys each had a huge ice cream cone.


 We spent an afternoon at our friend Sarah's families house in Cape Cod. Sarah took Matthew out on a kayak and they paddled to a beach not far from her house. I think its one of the few times that I have been able to sit and relax for a few hours while in Boston.

  We met our friends Tarra and Rich from Plattsburgh and ate dinner with them at the Hard Rock Café. Mmmm :) They were on vacation also. They live a few miles from us in Plattsburgh so it was weird meeting them for dinner 300 miles away from home.




We ended the week by going to Van Warped Tour. Matthew was selected to be a Dream Child by Living the Dream Foundation. This organization uses music to help children with life threatening illnesses. The Dream Child and family are given full access to the bands, are allowed on stage while the bands play and can meet the band members afterwards. The highlight of the day for Matthew was meeting Wallpaper who sing one of his favorite songs, The Best Day Everrr. He was hoping to see another band called Forever the Sickest Kids but we missed their show. However, he was able to meet the lead singer of the band and have his picture taken with him. Another band we saw called The Used brought Matthew in front of the crowd and had them chant MIGHTY MATTHEW three times. 

 

 

 

It's been a whole month since our last update. I finally have a free moment to catch everyone up on things.
Matthew's last day of third grade was yesterday. He is officially a fourth grader now :) I am also off for the summer as of this afternoon. :) Woo Hoo .Matthew is pretty happy to be home for summer break, although he did say he would miss some of his friends. He is not at all upset that he doesn't have to get up and get dressed every morning. He loves to lounge around in his undies :) 
This past month we have been busy trying to schedule his summer appointments in Boston. We are still in limbo with a new GI doctor . We waited over 6 weeks for his PACT team  to set Matthew up with a new GI doctor. After the last team meeting was cancelled due to his GI not being able to attend, we decided that we can no longer depend on him for Matthew's care. This follow-up meeting never was rescheduled , nor has Dr. R attempted to contact us by phone or email to check on Matthew in over two months. This is after this same Dr. told us that we were running out of time with Matthew if he doesn't immediately improve his nutritional status. His social worker from the PACT team finally told us that she can not find a single doctor at Children's who will take over Matthew's GI care from Dr. R . They claim that they don't want to step on  his toes. So basically, Matthew has had to wait( undernourished and hungry) with no GI care plan while we search for a doctor who will see him. Terry and I were beyond frustrated, so we contacted his Mito Dr. at Tufts to see if he had a GI recommendation there. He suggested a Dr  at Mass General and sent over some notes on Matthew. This doctor was able to fit us in the same week we will be in Boston in July for other appointments. We have our fingers and toes crossed that he will be able to help.

Matthew has a few things in mind that he would like to do this summer. He has a small list of his to do's:
1. See the Glengarry Bhoys play and dance to every song.
2. Go to Valcour island  with Nichole and Ava to explore and search for treasures.
3. See his friends in Boston and go to Hilliards Candy shop to get Rose Mints.
4. See Kacie and Terry at warped tour
5. Write some new songs. (He wrote his friend Lily the cutest love song and he is super proud of it) 
 
 
 

We are back from Boston. The big team meeting was cancelled late  Friday afternoon due to our GI doctor not being able to attend. He claimed that he didn't know about the meeting even though the entire team had confirmed this meeting weeks ago. We had a few other appointments to go to in Boston the same day , so we went just the same.
Matthew saw ortho for the bumps on the back of his heels. They sent Matthew for x-rays of his feet. They said that his bones looked good  but that he has bursitis on both heels. They said that his  AFO braces did not cause the bursitis , but that they needed some padding to protect his heels until they heal. The most common cause of bursitis is overuse of the area affected.  In Matthew's case he is probably harming his feet with his current activities, even though he is less active than typical children are. They are hoping that the bursitis goes away  in a few weeks with rest and not over doing things.
We had an informal appointment with his kidney doctor. He has been great with us and we really like him. He is going to talk things over with his nutritionist and see if we should increase Matthew's diuretic  or try and increase the formula volume slowly again. He also recommended that we make a follow-up appointment with Matthew's urologist to discuss the absence of "feeling the urge" to urinate. Matthew usually only urinates twice a day and only when we make him go. If we don't remind him to go, he just wont. Last week , he went over 24 hours without urinating and didn't even realize he had to go. His bladder is very large, and he produces such a small amount of urine (no more than  200 cc/24 hrs).  
Also, after meeting with two members of the PACT team, we decided that we will be getting a new GI doctor. Our current GI doctor is almost impossible to reach and hardly ever gets back to us or answers our emails. This is a big problem since most of Matthew's issues are GI related. They agreed that we needed someone who would be able to be involved more in Matthew's care and treatment plans. We will keep our old GI doctor only for motility issues.
 

After our appointments , we stopped by the Boston Marathon Memorial site where Matthew placed a small lollipop bouquet on one of the trees. He also left a small message of hope on one of the signs. We also were able to have a quick visit with our friend Sarah who is currently inpatient. :) We are bummed that we were not able to fit in a visit to see our other friend Chelsea who is also inpatient. Time got away from us, and it was too late to see her. We needed to get home. :( Chelsea, we promise to see you next time :)
New Pictures on :
www.caringbridge.org/visit/matthewcech 


Deputy Mighty Matthew's slide show;
http://www.youtube.com/watch?v=5c2SSEnJp7Q&feature=share&list=UUUx3MZfRTpCHv_6fRa9oiag

We now have a new Deputy in our house. Yesterday, Matthew was Deputized in the Town of Lewis. He went on his very first patrol and captured the "Lollipop Bandits" . His reward..... close to 1000 DumDum lollipops . He had a very adventurous afternoon and smiled from ear to ear. His favorite part of the "Capture" was spraying the Bandits with training "Pepper Spray" :). We can't thank the Essex County Sheriff's Office enough for making this day so special. It will definitely be remembered .

Matthew's formula rate is up to 59 ml/hr since Friday evening. His belly is quite distended and his urine has decreased even more than normal. Yesterday, his hands were puffy . This rate might be pushing Matthew past his fluid limit. We may have to back down a bit and see if he can adjust.

Matthew's follow-up team meeting in Boston is on April 29th. Terry and I are planning on attending. It will be a busy week for us . Boston on Sunday and Monday; Bradley has surgery on his foot on Wednesday ; Confirmation on Friday for Bradley; celebration party on Saturday; and back to Boston on Sunday and Monday for more appointments.