A new plan

Dr. Cohen called me today. He had spoken with the anesthesia doctor about doing the MRI again. This anesthesia doctor he said is well trained in all the risks, dos and don'ts associated with Mito and anesthesia. Dr. Cohen said considering Jackson's current respitory status (aka THE COLD), anesthesia risks it would not be a good idea to do the MRI at this time. It is a good idea because if Jackson's respitory status decompensates at all it would disqualify Jackson from the drug trial. I don't want to do anything that would disqualify Jack, so he will not have the MRI this visit.
So the new plan is to get more baseline bloodwork in the morning. Then Jackson will get the first EPI-743 dose on Friday. There is  follow up bloodwork for a few hours after the first dose and an EKG. After all that we are free to go home. Mom and I would like to leave Fri. afternoon but I don't know if Jack will feel up to a long ride in the dreaded faraway after his two days of Mito adventures. When we leave to travel home is up to Jack. Thank you for keeping up with Jack and his new Mito adventure!

Ohio Trip Day 7, 8,9 10

Day 7 Friday

I heard from sedation that they were not comfortable sedating Jackson while Jackson has this cold. ****PUBLIC SERVICE MESSAGE #999,999,999*****

 Wash your hands after you go to the bathroom, cough, sneeze!!! Cover your cough or sneeze with your elbow or a tissue! Stay at home if at all posiible when you are sick. And above all people, KEEP YOUR GERMS TO YOURSELF!!!!!!

Sedation cancelled the MRI apt. Jackson had for Monday. This woman from sedation semed like it was no big deal. I was hot! I immediately contacted Hilary. She had not heard this news from sedation and made some phone calls. I heard later on in the day from Hilary, Dr. Cohen's research nurse. I found out that all was not lost. Sedation and General Anesthesia are two different things. Sedation does not intebate but General Anethsia does. If Jackson was to have a MRI it would be with General Anesthia. The only problem was General Anesthsia only does it Twice a month. Friday December 7 being the next available day. Plans were presented by Hilary to get the EPI 743 and go home without the MRI. I didn't like the idea of starting this big long 13 month process without knowing where we started from. Hilary said that they had enough info with the bloodwork and such. I was not satisfied with just bloodwork. Jackson has not had an MRI since he was about 13months old. If the future MRI shows good changes how can we know the changes were caused by EPI 743. There wouldn't be any proof that his brain didn't start off looking that way. For me, the MRI is very important. I had read that researchers had seen disease reversal on some MRIs. I am praying that this is true for Jack.  I felt strongly that the MRI was important and I was willing to wait.  Hilary contacted G.A. and waited to hear back from them on Monday. We would have to wait the weekend. UGH!!!

Day 8 Saturday and Day 9 Sunday
We stayed in mostly and Mom went out for food. I feel like we are hibernating! I am doing my best to get Jackson better and so he can have the MRI.

Day 10 Monday
Hilary did not hear back from G.A. at all. I began loosing hope. We decided to wait one more day to hear from them before we make our final bloodwork and EPI 743 delivery plans to come home.

Day 11 Tuesday
I finally heard from Hilary, Dr. Cohen's nurse today. Jackson will have an MRI on Friday with G.A. . I don't know the time yet. I am nervous about him having anesthsia. I am excited about seeing his brain after 5 yrs of Leigh's. I have lots of emotions now. Please pray that the G.A. goes great with Jackson and he gets over his cold. Jackson will get the EPI-743 on the folowing Monday and then we can come home!!!!,

Ohio Day 5 and 6

Day 5 Wednesday
We spent the day in the hotel room letting Jack rest ALL DAY. I gave him his breathing treatments, cpt therapies with the vest and suction. He took three naps and was happy each time he woke up. Mom left to get food for lunch and dinner.  Nothing exciting happened and I almost lost my mind being cooped up in the little hotel room. I stayed busy taking care of Jack. If it wasn't for getting out of the room and going one floor up to do laundry I think I would have. Being in this hotel room is soooo very different   than being at home. At home there is always something that needs to be done like dishes, laundry, bills, insurance paperwork, cleaning, cleaning and more cleaning and take care of Jack. The hotel room is straightened and laundry is done. There are not any dishes because we are eating take out. Jack is resting and I've seen every NCIS and Duck Dynasty on TV. If I have another day like this one, I will write more. LOL

Day 6 Thursday
We spent the morning getting ready to leave the hotel room for Jack's appointments today. It is always a  huge task getting Jack ready to go any where. His bag has to be packed with diapers, pads, wipes, formula, water, meds, syringes and extensions. His bag should have everything he needs to survive while away from home. We got to the hospital and it was like a maze getting around. It reminded me a lot of the way LeBonheur used to be before the new hospital except it was labeled better. the PT evaluation went well. We only had to do the first part that concerned rolling, trunk strength and head control. The second part was about balance and walking which Jack can't do. It really didn't last long at all. While we have been in Ohio Jack's O2 tank decided to leak. I went looking for his extra O rings that typically are the culprit when the tank leaks and couldn't find them. I asked the PT for help with the  leaking tank. She went to Respiratory dept. and got someone to come back to PT to help me. James, the man that came was pushing 100yrs old but was extremely knowledgeable and helpful. He fixed Jack's tank for us. While we were waiting on the O2 repair. The PT told us that Jack is the second child enrolled in the study at Akron. I think not so many have been enrolled because things for the study are just getting up and going in Akron. I think there are a lot of behind the scenes arrangement going on between hospital, Drug company and FDA. We also forgot to pack Jack's bath chair. It has been difficult giving him baths. I will not forget on our next trip!  Jack's next apt was way across the hospital in the Heart Center.  Jack got an EKG. It didn't take long at all. We found our way back through the massive maze that is Akron Children's Hospital to the garage. On our way back to the hotel we stopped at Target. I bought some supplies to organize Jack's medical records better. The medical record notebook I keep for Jackson came in very handy with our visit with Dr. Cohen on Monday. Organizing it will also keep me busy! We got dinner and went back to the hotel. I got Jack settled and he went to sleep. Mom and I ate dinner and now I am typing to all of you! Have a good night! Thank you all for your prayers!

ohio trip day 1, 2, 3 and 4

Day 1
It was a CRAZY day! Mom, Dad and I packed the car. John took Elizabeth, Wesley and Daisy Mae (the dog) to my sister-in-law's house. God Bless her! I love you, Lynn! Y'all know I married John just to have Lynn as a sister-in-law! LOL The good bye's to the kids were hard. Even though I have a college degree, I don't have a fancy career to go with it like I thought I would. I will probably never be employed outside the home because of the care Jackson requires. My children, home and mito are my jobs. The good bye's to the kids were, oh so very hard for me! Knowing that I would not see them for at least 2 weeks was just excruciating for me.  It was not hard, however to say bye to Daisy dog. I love her but she is a cat turd and garbage eating dog. LOL John also had to pick up Jack's prescriptions he would need while we were gone. Walgreens had to go round and round with insurance to get this done. Why can't anything just be simple?  So with the car packed to the roof with two weeks of supplies for Jack we set off towards Nashville with Mom driving. I sat in the back next to Jack so I could feed him, entertain him and meet all his traveling needs. Jack would cough and I would suction. I hoped that he wouldn't get sick before our trip but it happened regardless of my efforts to avoid illness. I  just prayed I wouldn't get car sick. I can get car sick quick!!! I am so used to driving myself everywhere that the least little change will make me nauseated for hours. I was so blessed not get car sick.

Day 2 Sunday
We drove from Louisville, KY to Akron, OH. It was uneventful until Jack decided he was really tired of his carseat. He threw the kind of  fit only Jack can throw when he is tired and wants his momma. Believe it or not that sweet little angel can throw a BIG fit when he wants something he is not getting (like being held/snuggled when he is sleepy).  I took him out of his car seat and he immediately calmed down and eventually fell asleep. I know...I know it was not a safe move taking him out of the seat. We didn't know what else to do. Nothing I did "fixed it" for Jack. He was not a happy boy and we didn't have the time to stop and let him snooze. We got to the hotel earlier than I excepted (thanks to John's lead foot) and moved in. We let Jack rest and ordered pizza for dinner. Jack and I collapsed early. I had a crazy dream Sunday night. I was shopping at an antique mall. I was with a whole group of people and we were all just having a blast looking at all the old junk. Somewhere along the way I bought an old, very large, very heavy upright piano. I then pushed it through the store for the rest of the shopping trip/dream. It doesn't take a degree in Psych to figure out what that dream meant. LOL

Day 3 Monday
John, Mom, Jack and I went to Akron Children's Hospital today. We saw the "The
Wonderful Wizard of mitochondrial disease", Dr. Bruce Cohen. After I saw the 15 page contract, I started second guessing our decision to participate in this drug trial. The idea that Jack might get the placebo and not take his Co-Q-10 made get me worked up. The way the study works is 1/3 of the participants will have placebo and 2/3 will get EPI 743 the first 6 months. The last 6 months of the trial everybody will get EPI-743. After I realized Jack really had a shot at getting EPI 743, I was less nervous. I was hopeful! There were other tests that Dr. Cohen did like a general neuro exam. Dr.Cohen also did a Newcastle score on Jackson. in order to qualify for the study the patient has to score between a 15-60. Jack scored a 34. Don't ask what's in it, I can't remember. The test questions of what I remember seemed very subjective in nature. I think it is just a way to try measure changes (good or bad) in the patient taking the EPI. I don't think the Newcastle score was as important as the genetic dx. We have more visits to Akron than I originally thought. The trial requires several visits for various tests.  Visit 1 lasts the longest and is the screening visit/baseline visit.  We will travel back to Akron at 4 weeks, 13 weeks, 26 weeks, 30 weeks, 39 weeks, 52 weeks. Each visit we will see Dr. Cohen, have  blood drawn and various tests. Jack had blood drawn at this first visit. Jack will have a PT evaluation on Thursday.  He will have his MRI on Monday. I hope before we leave I get to see the MRI or talk to Dr. Cohen about changes (if any) in Jackson's brain. We were going to try to get the MRI on Friday but Jackson has come down with a cold and we want to give him more time to get over it before sedation with MRI. If Jackson is congested when it is time for the MRI they will not sedate him. The sedation is necessary  for Jack even though he doesn't move. I think it would be very traumatic for Jack to be restrained in a strange place with a very loud machine and no momma in sight. I am very curious to know what Jack's brain looks like 5yrs after diagnosis. He has only had two MRI's. He had one in 07 after his initial onset. He then had another 6 months later. Every time I ask for records for this second MRI LeBonheur says they don't have a record of it. They also act as if I am making it up that Jack had a second MRI. Well, I have only one thing to say about that....here's a copy of the bill you sent me. I had another crazy dream. I dreamed I was in a perfectly good commercial airliner. Where we were flying I have no idea. Why I jumped out of the plane I have no idea either. I remember falling and pulling the cord to make the parachute of out of the pack on my back. When it came out it was pretty and I slowly drifted to earth. It doesn't take a degree in Psych to figure that one out either. LOL

I also got word late tonight that Jack's mito buddy, Eric Clement passed away. I met Eric, his mom Meghan and his Aunt Amy during my visits to Nashville. He was a sweet, bright, angel faced boy! He will be greatly missed by everyone that knew him. Jack's other mito buddy, Taylor is  still in the PICU at Vandy. She is fighting mito with all her might. These two situations just made me realize how important this drug trial really is. I am so grateful for this opportunity to try EPI 743. This opportunity to just have a chance at trying a drug that may offer so much for those with mito is truly an answer to prayer. Since the day I heard the words mitochondrial disease I have prayed for a cure, treatment, something for mito. I hope that my efforts today will eventually lead to cure in the future for mito. I saw a segment on the Today Show the week of Thanksgiving about Mrs. Barbara Bush and her daughter Robin that she lost to leukemia in the 1950's. She fought like a pit bull against cancer. She sought out the most modern treatments for her daughter. She said "look how far medicine has come with pediatric cancers".  She thought if Robin was dx now with the kind of leukemia she had she would live. I know they can't cure all cancer now but cancer medicine has made just remarkable advances since Mrs. Bush's daughter Robin was diagnosed. It was an inspirational story. My Super, Big, Huge, Amazing Dream is to one day have a hospital for mito like St. Jude is to pediatric cancer. I want a hospital with millions in the bank solely dedicated to mito research.

Day 4 Tuesday
Jack woke up with pink eye. I called Dr. Frizzell and got a script sent to a Walgreens near our hotel. I got Dr. Cohen to give Jack a script for antibiotics so the bacteria won't have a chance to get hold of Jack while the cold virus runs it's course. It was a day of rest for Jack. He needed it. He is still fighting his cold. I have had to suction a lot. I am getting out a lot of thick mucus. Jack can't mange his normal secretions so the suction is necessary so he doesn't choke on his on secretions. No tests, no appointments or travel today. We spent the day in the hotel room waiting to here if Jack go in the study "officially". About 1:00 we got the official word that Jackson is accepted into the drug trial. I am super excited! We also got part of the plan for the rest of the visit. Thursday-PT evaluation and Monday-MRI

Meat Order Pick Up

Jackson had a very big day today. He went to the pulmonologist. We just got refills on meds and a sinus xray to check for sinus infection. Jack has had lots of drainage lately. I just wanted to check on it before our trip. Then he ran errands with me and Elizabeth all day. His nurse is out sick. It was hard on him going with me on the errands. The errands I had to do were all drive through errands but he was in his carseat a while after sitting in his wheelchair for several hours. He was beat! We came home and he slept.

 

If you ordered a Ham, Turkey, or Boston Butt from Jackson, I am going to pick them up in the morning. You can pick up your Ham, Turkey or Boston Butt at the following locations #1 Wendy's Parking lot at the Stateline Road Exit off hwy55 at 10:00 #2 Bartlett (stage/barlett road) Kroger Parking Lot at 11:00-12:30 #3 E.A. Harrold Elementary (Wilkinsville/West Union) at 1:00pm #4 Millington Kroger (hwy 51) at 1:30-3:00. If you can't make these pick up times please private message me, text me (568-2788) to make arrangements to get your meat treats! Thanks so much for all of your support! Jack couldn't get to Ohio without you!

The B I G News!

The BIG news is.....Jack is going to be in the EPI 743 drug trial with Dr. Cohen at Akron Children's Hospital in Akron, Ohio!!!!
I haven't signed the papers yet but I have been emailing tirelessly to make connections and get medical records. It all started Friday when I listened to a teleconference with the doctors that are leading the EPI 743 research. The results they reported are amazing by Mito standards. The reports are of less vomitting, diarrhea, less seizure, less disease progression, improvement with muscle strength even disease reversal in some cases! This is BIG news in Mito world! It is really BIG! There's a catch though. In order to participate you have to be genetically dx with Leigh's and confirmed on MRI.  Patients can't have kidney, liver issues either. You can't be on a vent either. This is a double blind study which means some will get the placebo. The study is set up 6 months on placebo and 6 months with EPI 743. While you are in the study patients can't take the "Mito cocktail". No one will know what which patients have what till the end. Also, at the end patients get to stay on EPI 743 if you want. Is that exciting or what!!!

and this is how God is taking care of Jack...

Texas Children's Houston/Baylor University/Dr. Scaglia is in the start/set up process to have the drug trial. It would be several months before Jack would be able to participate. I was making sure he would have a spot in the drug trial by getting his records in first, I thought! When I called Dr.K's office to have records sent to Baylor for the study they told me they had already sent them to Dr. Cohen in Akron, Ohio! I had no idea!  They said they had tried to contact me. I think they must have had the wrong email.  They already have things ready to go at that location and Dr. Cohen had reviewed Jack's file and wanted to see Jack's MRI.  When I was making my own plans, He already had it worked out. God is in control and He is taking care of Jack!

Jack and the eye Dr.

I took Elizabeth to Dr. Frizzell this morning for her bad cough. He says it's bronchitis. I've been expecting her to cough up a lung soon. That makes 2 kiddos on antibiotics.

Jack is still fighting his bronchitis. I think he caught it from Elizabeth. He is getting mutiple vest treatments a day and lots of suctioning to clear the secretions he is coughing up. He is also sleeping a lot, and tiring out quickly after an activity. He had opthmology apt. today. We've had the apt. for at least 3 months because it takes that long to get in for an apt. I know Jack is sick and really didn't feel like going. He had not had his eyes checked in a year. I felt like we had to keep this apt. for his eye health. Dr. Hoehn says Jack has a stigmatism and needs glasses. She says we can try the glasses to see if Jack will tolerate having them on his face or not. She says right now things are a little blurry for him. I hope he will like them. I'll keep you posted on what he does with them.

An Ideal Day

It's Mito Week and I've been thinking. I don't think I've ever shared Jackson's ideal schedule with you. I say ideal because everybody knows that the day never turns out exactly how you plan it to be but you try anyway. Everybody also knows it takes alot to take care of Jack the right way but I don't anyone really knows the details. So in honor of Jackson and in order to make clear the day to day of taking care of a child with mito, I have decided to share as many details of his care as I can remember this morning.

Jackson's Ideal Daily  Schedule

7am Jack starts his day! Jackson is changed into a dry diaper first thing. Jackson is fed and gets his morning meds every morning at 7am. Jackson is fed by gtube every three hours throughout the day. Each feeding is 100ml of a formula called Nutren 1.0 w/fiber followed by  150ml of water. Jackson has a feeding tube (gtube) because his muscles in his esophagus are too weak due to Leigh's (mito) to coordinate a swallow. He will aspirate while trying to swallow. Aspiration is when food goes into the lungs. This could lead to pneumonia. The Leigh's has caused lesions (cell death) in the area of his brain that controls muscle movement. Jackson also takes his morning meds for energy, seizures, and GERD with his breakfast. His morning meds are Zonisamide 100mg, Clonazepam .5mg, folic acid 5mg, Keppra 6ml, Zyrtec 2.5ml, Pyridoxal 5-Phosphate (B6) 50mg, Co-Q 10 1.5ml, L-Carnitine 7.5ml, Ribflavin, omprezole 10ml. When he is sick he gets Mucinex 5ml to thin the mucus. 

8am Jackson does not have any self help skills at all. He can not lift his arms to help put clothes on or off or wiggle in hands in any way to help his self at all. He is completely dependent on adults for everything including hygeine. I wash face, take care of his dry lips with Beauty Control lipcare, brush his teeth, brush his hair, and dress him. At this time I will suction out his nose and mouth for secretions. He does not like his nose to be suctioned at all.

9am I give Jackson his Qvar inhaler and ProAir inhaler. He takes 10 breaths of two separate puffs from a spacer/chamber.  A spacer has a little mask at one end of a chamber that fits over Jack's mouth and nose so he has to breath the medicine that was puffed  the chamber. The other end of the chamber has a place to put the inhaler.  After the puffs, Jackson has Chest PT. He has a machine by Hill-Rom. It is called an Airway clearance system. He wears a vest similar to a life vest that inflates with air and vibrates. He has treatment lasting 25minutes. Its' purpose is to vibrate out the mucus in his lungs so that his lungs stay healthy. Jack has this treatment twice a day. When he is sick he has it more frequently.  

On Tuesdays and Thursdays at 9am Jackson has school. His schedule changes some on these days.  He is a homebound Kindergartener with Shelby County Schools. His teacher Sara comes to the home. She gets Jackson to smile and engaged by playing music, singing, and reading books to him. She also works with him with his switch. We are thinking of moving his time to the afternoon so we don't run into the sleepiness of side effects. 

9:30am Jackson gets 4ml of Reglan through his gtube.  Jackson's stomach doesn't empty as quickly as it should.  It empties slowly because of mito. The mitochondria in his G.I. system don't make enough energy to run at a normal pace and the damage in his brain slow the muscles too. Reglan is given to stimulate gastric emptying. 

10am Jackson is fed his "morning snack". It is 100ml of a formula called Nutren 1.0 w/fiber followed by 150ml of water. By this time Jack is worn out and the sleepy side effects of the seizure meds are kicking in. Sometimes he will want to get a nap. 

11am Jackson hopefully will nap. If he doesn't want a nap he will rest in his bed while watching his favorite movie Cars. We might also read a book, listen to music and dance or play with toys. 

12pm Hopefully, by noon, Jackson is getting a nap. Jackson's nurse arrives and helps with his care until 8pm.

12:30pm 4ml of Reglan given  even though he is sleeping. We have to be sneaky and not wake him up. 

1pm Jackson is fed 100ml of a formula called Nutren 1.0 w/fiber followed by 150ml of water. Again we have to be sneaky!

2pm Jackson is usually waking up by 2. He is usually very happy after he is fully awake. He feels rested and ready for the rest of his day. Jackson gets his AFO's on, is lifted and strapped into his stander.  AFO stands for Ankle, Foot Orthodics. They are plastic braces to support him in a standing position. A stander is a piece of durable medical equipment that is essential to Jackson's health. It allows him to be in a standing position. I'll have to post a pic because the equipment is hard to describe.  This is good for his organs especially his lungs. It is also good for his bones to bear weight.  He has built up enough strength to tolerate 45min in the stander. He likes to watch cartoon, read books, play with toys while he is in his stander. 

2:30pm On Wednesdays, Jackson has PT from the school. This session lasts about 30min. This usually will make him very tired. He will want to rest. 

3pm Jackson has another vest treatment for 25 minutes. We do his vest treatments while he is sitting up supported by pillows in his hospital bed. We can't do the treatments in his wheelchair because the vest inflates too large for him to fit. I or Jackson's nurse stays with him the entire time. He is suctioned of and on throughout the Chest PT therapy. 

3:30pm Jackson is given 4ml of Reglan again.

4pm Jackson is fed 100ml of a formula called Nutren 1.0 w/fiber followed by 150 water. 

5pm Jackson also starts his bowel care at this time. This is a subject that may gross some of you out or fascinate you either way it is a part of Jackson's daily care and that is what I'm talking about today. B.M's are something everyone takes for granted until you can't have one. They are extremely important! Jackson must have one everyday. I think it is important for his muscles to get used everyday. I am scared if he doesn't have one everyday his muscles may become to weak or "forget" what to do.  If you don't use it, you loose it. If Jackson is unable to have a B.M. we will have to go to the hospital. B.M.'s  can be a long process for him. Jackson never has a bowel movement on his own unless he is on antibiotics. When he is on antibiotics he has more frequent, unpredictable and large B.M.'s  They really hurt his tummy. I give him some probiotics to help. Having a B.M. has been a problem for Jackson since birth. Jackson is given rectally 2 pedia-lax liquid enemas to stimulate a bowel movement. Jack lays in his bed with at least 4 pads underneath him during his B.M. We give them after his feeding in hopes that his brain will get and send the message that something goes in the tummy means  something needs to go out. 

6pm Jackson gets a bath. He is washed head to toe. Sometimes he likes it sometimes he doesn't. We use a large shower chair that lays out and a shower wand to wash Jackson's body. This is the safest way to give him a bath. He is lifted in and out of the shower chair.

6:30 Jackson is given 4ml of Reglan again after he is dried off and dressed. His hair is combed and his gtube site is dressed. We use 2x2 surgical sponge to dress his site. It is secured with Medipore tape. Jackson is allergic to the cheap paper tape. The 2x2 surgical sponge absorbs any leakage that may irritate the site. His site is checked at each diaper change for leakage or wetness. If wetness is found, the dressing must be changed. Wetness will cause skin irritation. Jackson has been blessed to not have any skin issues associated with his tube. I know there are a lot of kids that have complications. 

7pm Jackson is fed is 100ml of a formula called Nutren 1.0 w/fiber followed  by  150ml of water. By this time of the day, Jackson is wore out and needs to rest in his bed. He usually watches his favorite movie Cars again!

8pm Jackson is resting or asleep.

9pm Jackson is resting or asleep. If he is awake when I get ready to get in my bed Jackson gets in my bed with me. I move the O2 sat machine and O2 tubes in my room. It makes it easier to take care of him if he drops his SATS and the alarm goes off. He whimpers when he wants to be turned in the night. 

10pm Jackson is fed is 100ml of a formula called Nutren 1.0 w/fiber followed by 150ml of water. 

Jack does not like to be left alone ever. If he even thinks he is alone he will have a fit. Someone must be in his sight at all times or if you go out of sight you must talk to him. Jackson's diaper is changed frequently through out the day because he can not control his bladder or bowels. It is changed at least 8 times a day for voids. At this time he is checked for skin breakdown that can occur with kids like Jack. We use A&D ointment (not the zinc oxide) to protect his skin from wetness. His position his changed often through out the day. His position must be changed every two hours. He should not be in one position (like sitting in his wheelchair) for more than two hours. His mouth and throat are suctioned to remove mucus when he coughs when he is sick. He does not like this at all. Try to imagine the suction wand at the dentist office in your throat. It is not pleasant. We try to make it quick. You also have to be careful when you do suction Jackson's mouth and throat. You can't suction right after he eats because he will vomit. If you don't do it right you can and will gag him. Guess what happens ....if you guessed vomit you guessed right. Then you have a clean up project. Jackson is blessed not to be a mito kid that vomits. I know a lot of mito kids that do. So, I try to be as quick and gentle as I can for his comfort. 
This is just an ideal day. This is just an example of how his day should go when it is not complicated by doctor appointments, diarrhea, leaking diapers, illness or pain. He is on oxygen every minute of the day for support if he drops his SATS (oxygen saturation levels). He drops his SATS sometimes when he has a seizures. He especially does this while sleeping. He also has to have his sheets changed weekly, medicine givers washed daily, suction machine cleaned daily, enteral feeding extension changed weekly, enteral feeding syringe changed weekly, oxygen tubing and suction tubing changed biweekly. I am sure I have left something out. So now you know what I do all day.  Some people that meet us say, "How do you do it?" I think the more important question is "How can I not take care of him?" You don't have a choice when God gives you a gift like Jackson. 

The results are in!

I am excited to say that Chick-Fil-a is donating $136.62 to the UMDF! Thank you all soooo very much for supporting the UMDF!

Also, the results on our second tab collection are in too! I am happy to announce that we recycled 53lbs. of pop tops and 94lbs. of cans for total of $77.00.

Chase community giving campaign is donating $10,000 to the UMDF. Thanks to everyone that voted.

It has been an exciting Mito week! Thank you, thank you,.thank you!

Ready or not

Ready or Not...It's Mito Week!

We will kick off this week with a Mito Spirit Night at Chick-Fil-A tonight Monday September 17, 2012 from 5-8 at the Millington location. I hope you all can come and say hi. Jack will be there at 5 but will probably go home early.

I will be selling green light bulbs at Chick-Fil-A tonight! Turn your front porch green for Mito! When
your neighbors ask "Why is your front porch light green?" You can say "Well, there's this little boy Jackson and he has Mitochondrial Disease." Neighbor says "Mito What?" Then you can tell about Jack and Mito or direct them the UMDF website for more info.  It would be great if take a picture of your front porch green and post it to facebook or send it to me mitojack@gmail.com. Awareness is a piece to  puzzle of finding a cure!

MITO SPIRIT NIGHT!!!!

To ALL my Friends:

Please come to Mito Spirit Night!!!!

 Chick-fil-A

8492 Hwy 51

Millington, Tn

Chick-fil-A will donate a portion of their profits to the UMDF! Bring the flyer with you!

LoVe,

Jack Jack

 

So why haven't you posted?

This summer I didn't post anything on this blog. At first I didn't blog because I was so busy with the kids. Then decided to concentrate on just having fun with the kids and let the blog go for a while. We made a list at the begining of summer of all the things they wanted to do. We accomplished most of the list.

Jacks health:
Jack has been doing GREAT for a child with Leigh's. We have not had a hospital visit since May of 2011. I give God all the glory for Jack's life and health. Like I have said before we keep him at home as much as possible. Everyone that comes into our home washes their hands before they have contact with Jack. We keep him away from as many germs as possible whenever and where ever we are. Elizabeth and Wesley change clothes when they come home from school. We protect his rest as much as possible. I work all of his appointments around his typical nap schedule. Caring for Jack is a full time job. It is not something I take lightly. Everytime Jack goes anywhere whether it's a long trip out of town or just to the doctor, it is with great thought and planning. I never leave the house without an extra day's worth of formula and supplies packed in Jackson's backpack. I do that because thoughts like how would I feed him if we were caught out away from home and an earthquake happened (fill in your favorite diaster here) and I couldn't get home right away. When we leave I have to be prepared.

He had a mild case of bronchitis in July but with lots of vest treatments, inhalers, and antibiotics he recovered without a trip to the hospital. This summer Jackson enjoyed sleeping in and getting that extra time in Momma's bed that summer time brings. Sleeping and just resting in my bed is Jackson's favorite spot in the whole world! He can be very upset but when I put him in "his spot" and snuggle he calms down and gets very excited. This low cost treatment goes a long way!

School:
Elizabeth started 6th grade at Tipton Rosemark Academy. She is really enjoying her new school. She decided not to continue her dance this year with Millington School of Dance. :(  She decided to get involved in the activities for 6th graders at TRA. She is playing on the 5th/6th volleyball team, cheering for the 5th/6th grade football team, running for student council. I am so glad that she is trying new activities and making new friends.

Wesley started 4th grade this year at E.E.Jeter. He is playing competitive baseball for the West Tennessee Bears for fall ball. He has been playing in lots of different positions. He just likes to play! He has been taking private pitching lessons too. I am looking forward to watching him play in a game soon.

Jackson started Kindergarten this year. He is homebound which means his teacher comes to the house. It is the best thing we did for Jackson. He is more alert for his lessons and is sick less too. His immune system does not have the energy to fight the onslought of germs that going to school brings. The getting ready for school and travel to school also just wears him out. We worked last year teaching him to use a switch to turn pages in a book on a computer. He can use the switch! He liked clicking his switch!  We are excited to say that we have an apt. at the The TAD Center in Oxford, MS. to get a free evaluation for Augmentative, Alternative Communication (AAC). AAC is using technology (computers, switches, etc) to establish communication for people who can not speak. Think of Steven Hawking if you need an example. Jack is just beginning this journey. We are expecting great things from Jack and AAC. I am looking forward to working with him to find out his simple wants and needs. We will be working slowly but diligently with Jack. 

UMDF:
Mom, Jack and I went to Nashville this past weekend to the Nashville support group meeting. They were having a recap of the yearly symposium. I haven't been to see that sweet group of mito people in a long while. It was nice to get to visit with them. Dr. Tyler Reimshil spoke about the scientific/medical side of mito. Sonya Murray spoke about the nutrition and pain management segments that she attended as a parent. I enjoyed to recap and plan to sit down when I have time and listen to the symposium segments posted on their website.

ahhh...summa time!

We have had sooo very much going on at the Culley house lately. Elizabeth graduated from 5th grade, Wesley turned 9 and Mom retired from 29 1/2 years of teaching.
Jack, overall for a 5 year old with Leigh's, is doing well. It has been a year since our last hospital stay. I am super excited about this accomplishment. It has been with great sacrifice and my sanity but I have kept Jack home alot this past fall, winter and spring. This past fall, winter, spring was the healthiest ever for Jack. He was on antibiotics a few times and for a awhile but he did not have to go to the hospital. Jack is continuing to grow and change so very much. He weighs 47.6lbs. and is 43 1/2 in. long. He wears a size 6 and likes to wear some of Wesley's size 7 handme downs. At our last Dr. Morgan visit we had to increase some of his seizure meds because of his growth. We were seeing an increase in the strength, frequency and duration his myoclonic jerks. It's a balancing act. We went to see Dr. Joshi. He is our Memphis cardiologist. Jack had not seen him since he was about 15months old. I needed him to be updated on Jack's cardio status and get on Jack's team. He is willing to work with Dr. Moulik in Houston and was confused as to why I went to Houston to seek care especially since Dr. Wheless was in Memphis.  I told him I thought Dr. Wheless was a very smart man and good doctor but he does not work well with others. Jack's mito care takes a village and I am the mayor. I can't have any one in the village not willing to play nice with others. I also told him Dr. Koenig sees Leigh's patients every week/month in clinic. Some Memphis doctors see maybe 1 or 2 their entire career. I told him I want Jack to receive the most up to date mito care as possible and (for Jack) that was in Houston not Memphis. Dr. K's office actively helps recruit patients for research and she is also involved with the UMDF. He understood and didn't ask any more questions. I wonder why...

Elizabeth is exicited and nervous to be starting middle school. She is zoned to go to Woodstock middle.We do not think it would be an appropriate learning environment for Elizabeth. We asked for a transfer to Arlington Middle. We did not get our transfer to Arlington Middle. It is one of the top middle schools in the County system. I was very angry and disappointed. John and I are considering all our options for August. We will have to make decisions soon about what to do about middle school for Elizabeth. Homeschool advice anyone?

Mom retired this year from teaching. We are super excited about this news!!!! People have asked me "What will your mother do with all her time now?" I reply "Whatever she wants!"  She is planning on being with Elizabeth, Wyatt, Wesley, Ava, Jackson, Mason, Harrison and Charlotte as much as they can stand.  Congrats Mom!

Thank you!!!!

Can I be honest with yall? I was extremely worried this year that the race would not be successful. I was worried because of severl reasons. First, the economy. People are out of work or their hours have been cut back and gas is almost $4 a gallon. (OMG!!!) Money is tight all the way around. Second, I was worried because I thought people have heard about mito, Jack's struggles and it's old news.  I found out Saturday that I was wrong to worry and think those things. You guys ROCK!!! Yall really came together for Jack and I am truly grateful! The last total I got was $14, 600. Since this race started wehave been able to give the UMDF $60,000. Can you believe it!!! It gives me so much hope that a cure/treatment will be available one day!!! Sometimes, as Jack's mom, I feel so powerless aginst mitochondrial disease. There is nothing I can do to make Jack better. There is not any over the counter, prescription, alternative medicine I can buy to make him better. I am powerless to make his seizures stop. I am powerless to stop Leigh's. If there was a cure I would have found it by now. I have had endless internet sessions hunting for some obscure, rare mitochondrial disease cure. Holding this race  makes me feel powerful for one day. Race day is a day that I feel that a treatment is a possibility. A BIG Thank to everyone that donated and participated in Jack's race. It was an unbelievable success again!

You may be wondering why did it take Emily so long to tell us about the totals and race day. Jack had a VERY EXTRA Busy race weekend. Friday night he was at McAlister's for packet pick up, Saturday he got up early to go to his race. After the race Jack went straight to bed for rest. Sunday, Jack woke up happy and full of energy so he went to church. Sunday night, Jack had a one of his seizures in his sleep where he stops breathing. He was very tired on Monday. He slept most of the day on Monday. He was still tired on Tuesday but slept less. He rested the reat of the week with Ms. Charlotte and I watching closely. He worries me so very much when he has grand seizures. Worrying about Jack is worry like no other.  It is a worry from deep in my soul. The constant worry rarely leaves me alone.

I took Jack to his Memphis cardiologist today. It was suggested to me that we keep one here in Memphis well infomed and on Jack's team. That sounded like good advice to me so I made an apt. He said he would be happy to work with Dr. Moulik in Houston. Jack's heart is doing fine. Jack had a full cardio work up in Jan. He said he would review the doctor notes and test results from Houston. I told him I wanted to stay on top of all things cardio with Jack. Mitochondrial disease is sneaky. He agreed. He wants to see Jack back in Oct. for another full cardio work up. It may end up saving us an apt time in Houston. We'll see...

This fall...

Dear family and friends,

Thank you all so much to those that have already signed up for the Mito What? 5k. Your support these past 5 years through this mito journey have meant so very much to me. I always treasure your kind words and prayers. This race means so much to me. It gives me hope that a cure or treatment will be found one day when I see so many people coming together on that day.

When Jackson was fist diagnosed with Leigh's John and I were absolutely devestated. We had no idea what was ahead of us.The day in November that Jack was diagnosed with Leigh's,  I remember telling John that I haven't even planned his first birthday party and now the doctors are telling me I need to plan his funeral. It was a heartbreaking time. I am grateful to God for every smile Jackson has because there was a time when Jack didn't smile. I am grateful to God for every moment I have with Jack because I never know when Jack's life will end. Even though I am so grateful for Jack's life there is the reality that Jack has never talked, walked or even played with a toy. There are so many milestones of childhood that Jackson will never experience. It just completely breaks my heart. This fall Jack won't be going to Kindergarten. I will never hear Jackson recite his ABC's,123's or his phone number and address. Jackson won't be star of the week and make a poster with photos.  I will not pack a snacks and send milk money for him. This fall I will not be buying big crayons, fat pencils or even a nap mat. He won't be picking out a new lunch box and backpack to go to school. I always enjoyed watching Elizabeth and Wesley struggle with this decision. I won't have that pleasure this fall. I won't even get to cry when I leave him on his first day of Kindergaten like I did with Elizabeth and Wesley. Instead, Jack will be at home having his diaper changed, enduring physical therapy sessions so his muscles won't atrophy, be on continuos oxygen to keep his O2 levels up, have formula poured through a tube into his stomach to keep him nourished. Jackson will have to be continuously repositoned to avoid skin breakdown. He will have his temperature checked rectally every hour to make sure he temp is normal. If his temp is not normal and he is overheated he will have to be cooled down with icepacks and cool rags. His brain can not regulate his body temp. He will be lifted in/out of his bed, wheelchair, bath chair, stander and carseat. Included in Jackson's physical therapy sessions (which are twice a day) are stretching sessions for his arms, legs, hips and rib cage. He gets strapped into a stander and stands for 45 min to help his internal organs, leg bones, pelvis and spine. Jackson will endure 25 minute or longer chest percussion treatments 4 times a day, just to keep his lungs pneumonia free. He will be going to the neurologist, cardiologist, pulmonologist, GI, immunologist,allergist, kidney specialist,  physical therapist, occupational therapist, speech therapist appointments this fall and as long as he lives. Jackson will be hospitalized and will have IV's when his temp is 101 or greater. He will be pricked for bloodwork this fall and the rest of his life. This fall Jackson will be learning to communicate with a switch. This fall I won't be writing notes to Jackson's teacher. I will be writing letters to insurance companies begging for them to cover the cost of Jackson's needs.

So with all that said I am begging for your participation in the Mito What 5k. You don't have to run (I never run anywhere)! Just register and get a shirt. You can raise awareness just by wearing mito fashion. I am begging from the bottom of my heart.




 

Jack's art party

Last night I went to an art party at  my incredibly talented cousin Melissa Fischer's house. The party was held as a fundraiser for the UMDF. Participants paid a fee to make a craft. After the cost of the supplies were covered the profit is donated to the UMDF. Last night we made a "love bug" for Valentine's Day. We had red velvet cupcakes with cream cheese frosting. They were absolutely the most delicious cupcakes I've ever had! We also had banana punch made by her sister-in-law Ashley. It was soooo very good! It was  a fun night crafting and snacking! We are thinking of doing it again around Easter time. You guys should watch for an invite soon!

Jack has still been fighting bronchitis. We have increased his vest treatments from 2 times a day to 4 or more a day. He is on his third antibiotic. He is still smiling through it. I hope we can get him clear soon.

We are looking forward to the Mito What? 5k coming in March. It is being held 8 AM March 24, 2012.  It will be Jackson's 5th birthday!!! The race will begin and end at the U.S.A. baseball stadium in Millington, Tn. You can register for the race on line at racesonline.com You don't have to be a runner to get a shirt!!!! There are different levels of participation. I know some of you live out of town and won't be able to attend but you can still register and get a shirt. If you can come and you don't want to run or walk just register for
 a shirt come and cheer the runners in. It means so much to me and the other mito families to have your support. Our kids don't have a cure but your participation in fundraising events give us hope that one day a a cure or a treatment will be available one day.

Jackson's Houston Adventure

Last week was Jackson's yearly tripto Houston, TX to see all his mito doctors. I told him he was going to see his #1 girlfriend, Dr. Koenig. He smiled when I told him that he was seeing a girlfriend. He is such a ladies man sometimes. Mom, me, and Jackson got on an airplane Monday afternoon. I was nervous about Jack being around all those germs. We may drive next time but Jack has a hard time riding in the car for over 10 hours. It's hard making those travel decisons.

So what did we learn about Jack.....
Dr. Koenig said that Jackson looks good overall. He is growing. he wiegh's 47lbs. and is 44in. long. Jackson has been fighing bronchitis and has been having lots of jerks lately. I asked her what to do about the increase in the frequency, strength and duration of the myoclonic jerks that I have been noticing in  Jackson having for the past month or more. There is nothing we can do for him. Jackson is taking the max on all his seizure drugs. I can't imagine what things would be like for Jackson if he took nothing for the myoclonic jerks/seizures. At least while he is on the meds it controls them enough where he can participate in his life and make progress developmentally. She added a new vitamin to our mito cocktail. It is called B6. She also doubled his Co-Q-10. Jackson is still on the list to be recruited for the EPI 743 study. It is a study of a new drug called EPI 743. It is similar to Co-Q-10. Doctors that are studying this new drug have seen great results for Leigh's patients. I am excited to find out for about this opportunity for Jack.  The doctors are not recruiting patients at this time so Jack is on a waiting list.  /Dr. K wanted to see him again in 6months. If we lived in Houston she said she would see him every month. Everytime we go to Houston it reminds me of just how fragile Jack's health really is. At home Jack's needs have just become normal to us. Sometimes I forget just how precarious, different, difficult, Jack's life is . He is just my baby.

Later that same day we saw the GI doc. His name is Dr. Rhoades. He also thought Jack looked good. He said Jack obviously doesn't have any trouble getting nourishment. LOL Jackson just has trouble emptying his stomach and his bowels. He increased Jack's Reglan to help with gastric emptying. He suggested dulcolax to help Jackson have a BM. We have been using Fleets but have not having the usual results. We'll see how it works. I think I may try to get a GI here to start to monitor Jack. They may could jsut do the same thing Rhoades did. It would shorten our Houston trip and I am in favor of that. It really didnt offer  anything else to Jack's overall plan of care.

On Wed we saw the cardiologist Dr. Moulik. I really like her because when I first met her she started out her first sentence with.."In my other Leigh's patients I see...." I thought wow she sees other Leigh's kids this is great! She ordered a 24 hr halter, ekg, and echocardiogram. I don't have the resuts of the 24hr halter yet. The EKG and echo showed everything with the structure of his heart and rhythm of Jack's heart to be normal at that time. Jack does have WPW (Wolfe Parkinson White). Which means Jack's heart gets mixed up signals smometimes. Dr. Moulik thought Jack's WPW was not life threatening and has decided that it does not need any treatment at this time. Jackson's heart rate has been dropping occasionally in the night while he has been sleeping. She and Dr.K felt like this is related to the lesions on Jackson's brain stem and there's not anything we can do for it. When he sleeps Jackson is on his monitor.

Later that day we saw Dr. Paechco. She is Jack's imune specialist. She is keeping Jack on Zyrtec for allergies. I asked her about what to do when it is so hard to get an iv in Jack when he is sick. I asked about a central line/PIC line etc for Jack. I don't think we need one now because we are not in the hospital on average 3 to 4 times a year. If we were in the hospital more it would be considered. I am against one for now because the risk of having a life threating infection is WAY to high. She said for now when Jack is sick give more fluids. But how much how oftern...She was going to talk to Dr. K about it and get back with me. I think I will consult the Gtube clinic people that monitor Jackson's nutition about it at our next visit.

Finaly we saw Dr. Smith, the pulmonologist. She is concerned about Jackson dropping his SATS at night. She said it is related to the disease and the lesions on Jack's brain stem. She wants him to continue on his oxygen, proair, qvar, and vest treatments. She is considering trying an apap machine for Jack. It is similar to the cpap and bipap machines for sleep apnea. It is experimental and insurance doesn't cover it. If she decides it is something Jack needs then we will have to make another trip to Houston because no one close to Memphis does it.

I know it seems like alot of trouble to go to just to hear that Jack is doing great. I was worth it to me to hear it from doctors that see Leigh's kids all the time. It put my mind at ease that everything that can be done for Jack is getting done. I couldn't live with myself if I didn't make sure that I was seeking every opportunity for optimal health for Jackson. I can't cure the mito. I can't get rid of that ugly thing that is within the very cells of my child. But I can do everything in my being to give Jack every chance to live the best life he can. I do that my seeking the most experienced Leigh's doctors that I can physically get Jack to their office. That's my job because I am his mother.
Thank you so much for checking in on Jackson. He is a very special mito boy. Please continue to pray for good health for Jackson.  Pray for wisdom for the doctors that see these kids. Please pray for the research that is going on right now with EPI 743. Right now it offers the most hope for Jack and kids like him. Please continue to ask the Lord for a cure for this disease.

Guess what Jack did yesterday!

Jackson has been such a happy and healthy boy lately! He has been staying healthy because he doesn't go anywhere! I can't remember the last time he went to the grocery store with me. He can't catch it unless it comes through our door. I hate that his life (and ours) has to be so isolating for now but I can't allow him to get sick. I can't let him get go through another hospitalization with I.V.'s and fevers. I gotta do what I gotta do to protect him. Cold and flu season will be over in a few months and I can't wait!

School started back on Tuesday for everybody including Jack. His teacher, Ms. Anna, set up his computer with his software. The program choice for the day was a story about a momma cow looking for her baby. The computer would read the page and Jackson would have to click to turn the page. He clicked to turn the page all the way through the story. It was a total of 17 clicks! It was amazing to see him click to turn the page to hear more of the story! After the story was over Ms. Anna hooked his switch up to his switch toy Elmo that sings ABC easy as 1,2,3 by Michael Jackson. With prompting, Jackson clicked his switch and made Elmo sing and he loved it! Ms. Anna walked away to get another switch toy off the shelf. When she came back to the room she and I stood back away from Jackson where he couldn't see us watching him. We watched Jackson wiggled his shoulders, neck and head to try to figure out himself how to make Elmo sing. He was clicking on his own without prompting! Ms. Anna said this showed that Jackson was a natural learner. He wanted to figure out himself. This is one proud momma! Jackson also showed critical thinking skills by clicking his way through the story. Jackson wanted to know what happened next in the story! It was such a great day! All of those skills have been discovered because Jackson is staying healthy.