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.