Reagann’s Blog (written by her mum BJ)

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I will start my first blog off saying how heartbroken our family was to hear of the tragic loss of a young lady at the age of 22 who lost her battle with ROHHAD this week, Sarah Blake. I had never spoken to her or her family, although I wish I had. She was a member of our support group but we had never corresponded. We send our love and prayers to her family.

I’ve thought for some time what I would write about in this blog. I’m committed to helping raise awareness of ROHHAD but at the same time our family values our privacy. We don't want this disease to define us or especially Reagann. It's a balance that I haven't quite figured out yet. I've said for some time that Reagann has ROHHAD but ROHHAD does not and will not have Reagann.

This past weekend we went to a store about 50 miles away from home. A woman who worked in the store recognized Reagann and commented that she had seen Reagann on facebook. She was very nice and heartfelt. She recognized Reagann but she wouldn’t have been able to tell us anything about ROHHAD, but most people, including close family and friends, couldn’t really tell me much about the disease if I asked. How do I change that? The disease is SO complex, it takes us parents years to try to understand it and have any sort of grasp on it. But maybe that’s not the goal. My goal has always been to find others who could have the disease. I don’t ever want anyone to go through what I went through….begging doctors all over the country to listen to me that there was something wrong with my child. And for months, years even, no one would listen. It’s still hurtful nearly five years later to think of all I went through to try to get help for my child.

The biggest question I get asked from people is “How is Reagann doing?” I never quite know how to answer this question so I usually just smile and say that she is doing great. It’s just easier to say that. If I tried to explain it all to someone who asked, it would take hours. I do not take Reagann’s health for granted, overall she is doing quite well, and I am very thankful. But every day truly is a battle. We have to watch and monitor everything Rea does. We keep spreadsheets of her daily fluid intake, oxygen levels, heart rate, etc. I keep a spreadsheet with all of these numbers on them, and track averages; so if her numbers are off trend or higher or lower than average it sticks out.

I use a lot of the skills I’ve learned at my office to track everything Reagann. It would seem to most people that it’s a ton of work and hard to do, and I don’t minimize it, it is hard but it’s worth it. I can tell you anything Reagann usually by memory. I study, analyze and memorize numbers, spreadsheets, doctor notes, clinic notes, medical records to be able to help my child. Is it overwhelming? YES! But I do feel that it’s in her best interest? Absolutely. Our doctors actually request my files periodically.

Reagann’s biggest battles these days are with her electrolytes, mainly her sodium levels. The last few months have been very hard with her sodium levels being elevated. Any sort of stress or sickness will affect her sodium levels and she shows no signs when her sodium is high or low. The only trigger is if we notice that she’s not drinking as much as normal or if we have to push her to meet her daily fluid goal. We are actually lucky in the fact that she is pretty much able to drink as much as she wants. We are not on a strict fluid regimen like some children are, Reagann has partial diabetes insipidus, which means that she has some working thirst mechanism, so basically her brain doesn’t tell her all the time to drink to keep her body hydrated, but it does tell her some of the time.

If her thirst mechanism worked fully then her brain would know when she was getting dehydrated and signal her to drink more. Her current fluid goal is 50-60 ounces daily. When her sodium is over 150 we get a fluid goal from her pediatric endocrinologist. Her fluid goal is calculated on her weight. When her sodium is over 150, our fluid goal is typically around 90 ounces a day for 2-3 days and then we will have her re-checked . We are chasing more and more high sodiums in the last few months and we are unsure what the cause is…it could be because she’s gained a little weight; disease progression or a number of other things. I refuse to say it is disease progression.

Reagann normally gets her sodium checked every two weeks. If she’s in the normal range (135-145) then we get a two week pass before re-checking, if she’s high, then we have fluid goals, and recheck every two days or so until she’s back in normal range.

Some days are harder than other days. This life is a very complicated, often lonely, mis-understood life. When you have a child with a chronic health illness (you will NEVER hear me say terminal because I don’t and won’t believe or accept that) it seems that you will always be accused of trying to get attention. I wish people understood that most of us would give anything for our children to have normal lives….for us as parents to have normal lives. But until you live this life daily, you will never understand. You are on constant high alert, and live every day, every hour of your life trying to give your child as normal a life as possible.

I’ll never stop fighting for and believing in Reagann. I’ll never give up. Reagann is a miracle and I praise God every day for what He has done in her life and in our lives.

ROHHAD will truly not ever win with our family.

Please continue to help all of the families that fight ROHHAD back, raise awareness of ROHHAD, and keep all the families and children in your thoughts and prayers. These truly are the most special people in the world. There’s a reason why our kids are 1 in 70 million.

God Bless,

BJ Duenne

Jaxon’s Blog (written by his mum Shanel)

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Since Jaxon's birthday things have been very busy. We've had several adjustments in meds and with the ventilator. Jaxon has started cortisol supplement when he is sick or his body is under stress. His ventilator pressure has been increasing. There are times even with the increases that Jaxon is having sever obstruction and his body is accepting 0ml volume of air with his ventilator pressure maxed out. I'm going to have to learn how put it a tube (down his throat past the obstruction)and bag him in an attempt to get him breathing again. This is very scary. We are also going to be meeting members of the local fire department so they can meet Jaxon and know his care plan, in the event they are first responders to an emergency with Jaxon and his breathing or cardiac issues.

We have gotten a new Armadillo back for Jaxon's wheelchair(see pic above) to help give him better support in an attempt to alleviate some of his back/neck/leg pain.

The public school board has decided his needs are too complex for them to even try to meet. As a result I have started a relationship with an agency who helps advocate for inclusion for individuals with disabilities. I've also had meetings with different people in local government in attempt to address some of the shortcomings with programs for people with complex needs.
Jaxon also decided on his make a wish. He wished for a camper trailer. He's adamant that it will be decorated with superheroes everywhere and that he will take his superheroes to visit the ocean this upcoming summer.
Like I said, life has been very busy. Never a dull moment. And in amongst all the chaos, my superhero still needs to find time to just be a 5 year old boy.

‘Angel’ Emilee’s story continued as written by her Dad Charlie

Emilee 2Well time to say a little more about my lovely daughter from where we left off.

Thinking back to when Emilee started school, it should be the happiest days of her life however at the same time she is getting sicker and sicker and none of the Doctors can tell me what is going on .

I can tell you it was very frustrating when all they can say is watch what you are feeding her even though you know that is not the problem. In Kindergarten she was doing very good and still had a few friends but at school not so much. I can see Emilee is still gaining more weight and now her temp will not stabilize it ranges from 106degree F( 41.11 degrees C ) to where it does not even register on the low end of thermometer and again Dr s do not know why.

Academically Emilee is still achieving 100% on all her school work even though she misses a month straight being in the hospital.

Having tests done and trying to keep her temp normal is mostly why she is spending so much time in the hospital for at this point. When we have to go to the hospital our local one they send her to a bigger hospital that is a 2.5hr drive for me and I still have Tony her brother to take care of. As a single parent it was hard to juggle it all and sometimes I would keep him with me and go to hospital to be with Emilee. This meant he was also missing school and for now he was doing really well in his work at school too.

Then sometimes I would have to stay at home and Emilee would be at the hospital by herself; I hated both situations take Tony with me and he misses school or leave him with someone and I go to hospital with Emilee or leave her alone in hospital. None of those decisions were easy and such a difficult choice to make neither is very good for any of us.

For now we where getting through it . Many times Emilee would end up falling asleep at school, we know now this was due to ROHHAD. I would go and collect her and bring her home and despite this her 1st grade work was excellent.

Her body and mind was getting worse and worse, she became affected by Diabetes which was really difficult and some violent tendencies were starting, her behaviour was changing before this she would never have hurt anyone or anything ever. As all of this was progressing and her body was changing so much, still Doctors could not tell me what was going on.

In the summer months Emilee spent 2 weeks at her mom’s and while she was there she had a seizure this was to be her first one of them with more to come. Her mom lives 8 hrs. from me so I went straight down there to see what was going on and still the Doctors had no answers for us they knew she was sick but did not know yet what was causing everything.

I was so scared of what was happening to my beautiful Emilee and then her mom decided she is going to try and take her from me. Her mum went to children's services to try and keep her. I loved my daughter with all my heart and I knew that staying with her mom was not in her best interests given that I was her main guardian and her father, I fought hard for Emilee as I knew it would be very hard on my baby girl. I went to a lawyer and managed to have Emilee returned home to me were she belonged.

Not long after she returned home her teachers wanted to test her for gifted classes because of how smart she was and how good she was in school . The problem was getting it done because she was spending more and more time in hospital and missing so much work because she was still having so much trouble staying awake at school. When Emilee did manage to take the test so when she did very well but just missed out by a few points.......................................

I will write more again soon, this is enough for me just now; none of this is easy on me but I hope it helps or comforts some people more to come soon.

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