Ohio Trip March 3-7 2013

We always take two days for driving to Akron. I am sure if Jack was typical, it would be possible to make the trip in one LONG day. Jack is not typical so, Mom, Jack and I set off on the first leg of our two day journey for Jack's third visit to Akron Sunday March 3. It is better for Jack to break up the trip into two days. Once you put Jack in his carseat he stays in that position. Can you imagine the discomfort of not being able to sift your weight or even move  any of your body parts for comfort? OUCH is an understatement. We make frequent stops to rub Jack's back and change his diaper. We traveled to Louisville, Ky which is good half way point on day one. Jack traveled really well! We got up Monday morning and traveled the rest of the way to Akron. It was thankfully, uneventful!

When we got to Akron Monday afternoon there was a Winter Storm warning. Mom and I were nervous about the snow. Growing up and always living in the South, neither of us has any experience driving in the winter conditions that plaque the whole state of Ohio. We watched the weather closely. This trip was a two day visit. The first day was an EKG, and a visit with Dr. Cohen. The visit with Dr. Cohen includes bloodwork, review of meds, and what's new with Jack session that includes the good and bad. We have not experienced any side effects from EPI-743. The only thing that has been "bad" is Jack's weight gain. He started the study weighing 46lbs. He now weighs 52 lbs. Weight gain is bound to happen to a growing boy so I really can't say that it is because of EPI-743. Jack's seizure meds are given according to his weight. Jack experienced a weight gain and the usual dose of seizure meds no longer controls the seizures. The increase in seizure frequency and intensity brings on misery for Jack. We are in the process of adjusting the seizure meds for optimum control.
We returned home Thursday evening. All the way home I had been checking Jack's diaper. He remained dry all the way home. I became very concerned when we had been home a little while and Jack still had not voided. I called the doctor on call for advice. While waiting on the call backs, I decided to give Jack a vest treatment. He voided during the treatment. I was so relieved!  The nurse called back and said to take Jack in to Dr. Frizzell the next morning. By the time we got to the Dr. office Jack had not voided since the night before. He had not voided for another 12 hrs. The nurse was preparing Jack for a cath to get a urine sample to check for infection when he decided to finally pee. Jack has never done anything like that before. I have been so worried about this new thing Jack has decided to do that my nerves are a wreck. Just as quickly as he stopped peeing he.started peeing on a regular basis again. I have several theories. 1) He is experiencing increased muscle tone in other parts of his body why not in the muscles that control voids. He may be confused at this new found control. 2) His brain is just not sending the signal to his bladder. Either way it is another symptom of mitochondrial disease that we will watch and treat as needed.  Please consider donating $20 to the Jackson Culley Mito-What 5k. You get an awesome t-shirt out of the deal and help Jack at the same time. The 5k is going to be March 23, 2013 at 8am in Millington, TN. Go to www.racesonline.com to register.

Something normal

Last night, Jack had a visit from the tooth fairy!!! That's right you heard me, the tooth fairy! I noticed one of Jack's bottom teeth looking crooked earlier this week. When I felt it it was loose! I was immediately excited! Jack would get to experience a normal childhood milestone!  I was also immediately worried that the tooth would come out on it's own and Jack would get choked. This is the type of normal that is so precious to us! I was told when Jack was first diagnosed (dx) that these moments would never happen for Jack. I was told to take him home and love him because there was nothing anyone could do for Jack. You can imagine the grief that followed. That grief is what makes just a simple loose tooth so sweet! The tooth fairy did not bring Jack the usual monetary gift. Jack has no concept of money. So, the tooth that is assigned to him brought him Francesco from Cars 2.  Jack loved it!

The TenCare Battle of feb 2013

You all my have heard that I fought tncare and lost recently. So here's my side of the story...

Soon after Jackson was dx with Leigh's I applied with the Social Security Dept. for some help for Jackson. I think we can all agree that Jack's health situation and many others similar to it our why we all pay our taxes. We want our tax dollars to help those people in difficult situations. Well, I applied we received benefits a whole $30 a month and tncare. I didn't care about the money I just wanted the tncare coverage for Jackson. At the time the lady told me if our income increased by $5 we would no longer qualify for SSI benefits. You may ask would I want Tncare. Why would I want the government involved in Jackson's healthcare if Jackson has a private health insurance policy? Good question...
In our case, our private health insurance does not pay for Jackson's private duty nursing (Jack only gets 40hrs a week), diapers (forget about them paying for wipes), some of Jackson's durable medical equipment and has restrictions on physical, occupational and speech therapy. The private insurance called the second wheelchair that Jackson needed due to growth a convience item. They wanted him to continue using a wheelchair that was too small for him. As you can imagine using ill fitting equipment on a daily basis can cause a slew of other medical issues.  I am so glad we had tncare coverage at that time because they paid in full for Jack's wheelchair. They also pay for Jack's diapers that the insurance says is not covered in the policy.  There is a myth I would like to attack right now. Just because someone has a private health insurance policy doesn't mean that all their medical needs are met/covered by the policy and that person has no worries. I challenge all of you to really look at your health insurance policy. Read the information they send you, including the fine print. You will be surprised. The days of 100% coverage on all things is gone my friend. It is an 80/20 at best insurance world and don't forget about those outrageous individual and family deductibles that have to  met before they will even pay the 80% of the allowable charge and in some cases you don't even get to count your copay against the deductible. READ my friends, READ!

So Jack got TNcare through SSI. We had an small increase in income and lost the SSI $30 payment but got to keep the tncare. He was put in the transitional medicaid category. They are transitioning you off medicaid. The transitional category only lasts 12 months. After the 12 months you must reapply for tncare. I reapplied after our 12 months was up and was told we did not qualify for tncare but we might if we had enough unpaid medical bills. I submitted all our unpaid medical bills to see if we qualified for the Spend down category and we got to keep medicaid for another year. Oct. 2012 it was time to reapply again. This time when I submitted unpaid medical bills they didn't count. I was told they were to old to count. "What" I said. Come to find out, when you submit unpaid medical bills for spend down the bills must be incurred in the month of application or 3 months prior. I disagreed with that decision so I appealed. I scrambled around the house for 2 weeks planning my side of the case. I found every receipt I could that would count for spend down. When we got to the hearing I listened to the states evidence and their side. then I got to tell mine. I told about Jack's health condition and all the receipts I had pulled together but it still wasn't enough to meet the magic spend down number. The judge said I should have submitted the receipts at the time of application. I argued that all I was told to submit was unpaid medical bills. No one and no where on the application does it tell you what counts for spend down. I had to search on the Internet myself and call a lawyer with the law and justice center to find out the rules for spend down. None of that mattered to the so called judge. He asked me what my witness would testify to and I already knew what she would say and knew it would not change his mind.  After I realized none of my hard work mattered to him and he had already made up his mind I decided to make the hearing last as long as I could and waste his time. I was so angry and I had no way of doing anything about it. So I decided to talk as much as I could so I could delay his docket for the day and hopefully get way  backed up. I think it worked and I hope he had a crappy day like I did. I know that is not the right attitude to have but it is honestly how I felt at the time. I was a desperate, angry mother and just wanted help for my child.

For those of you that may not know difference between medicaid and medicare, SSI and SSDI, I am going to teach you.
Definitions
Medicaid-
 The federal government gives money that you paid to it called taxes to all 50 states to help cover the part of the cost of state run health care called medicaid. Each state also uses state taxes to fund their medicaid program. Each state calls their medicaid program a different name, in Tennessee it is called TNcare (tencare). Each state runs their medicaid program according to their own state laws. They have rules concerning who can qualify for medicaid, what they will cover and how long you can keep it. So who is eligible in New York may not be in Tennessee. What is a covered cost in Tennessee may not be in Ohio. Medicaid has a financial eligibility component to it. You must meet their strict guidelines concerning income and resources in order to get TNcare. They have a computer program that does it for them because it is so complicated. In Tennessee their are a bunch of different categories that one may qualify for after you meet the financial part. In other words it is mainly geared to help the low income get healthcare. They don't care what your dx is they just want to know how much money do you make. You must be under 65 to get medicaid.

Medicare-
Is run by the federal government. States have no authority with medicare. You have to be 65 to get benefits or be a dependent of a parent that is receiving benefits to get coverage. Again your dx doesn't matter. The only thing that matters is age and your pocketbook.

Spend down-
So now you know their is a financial component to medicaid. Your next question should be... What do families do if they make to much money to qualify for tncare but not enough to be called independently wealthy and have a sick child. In other words you ain't broke enough for the state to help but you still broke! There is a category called spend down. You "spend down" your "extra income" to meet the financial guidelines. You can count health premiums, copays, OTC, prescriptions, nursing costs, and mileage (.42 per mile) round trip to doctor and pharmacy.

SSI-Supplemental Security Income-
Income people receive from the government (again your tax dollars). There are again financial components and mathematical formulas to determine eligibility.

SSDI- Supplemental Security Disability income
There are not any financial components to this but you must prove disability. In order for children to receive benefits they must have a parent that is retired, dead or disabled and receiving benefits.

I could be wrong on some of this but this is how I understand the system to work today. Sometimes you win and sometimes you just limp away from a battle, lick your wounds and fight again another day. I am forming another plan as I type this. I will keep you posted to let you know if my plan works.

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