Wednesday, June 19, 2013

Sophie's Journey

This is our friend Sophia.  She has been a long time friend of Haven's and we love her family dearly.  Lately Sophia has been struggling with getting help from doctors and has been waiting to start treatment.  I have included a note from her mom below and there is a link to her Facebook page.  Please check out her page and keep yourself up to date on Miss Sophia.

Sophie's Journey

I would like to tell you a little bit about my Sophia. She is the youngest of six and was born on October 19,2004, by C-Section. She was only a C-Section because i had one before her. She weighed in at 9 pounds 6 onces and was 21 inches long. Sophia was healthy but she was diagnosed with kidney reflux at 3 months old and antibiotics took care of it. She would out grow the kidney reflux by her second birthday. When Sophia was 18 months old she was tiny at 20 lbs but by the time she was 22 month she had gained almost 25lbs her pediatrition sent us to the OWL program at childrens hospital that's when we met Dr. Rhodes. i explained Sophia's symptoms - the throwing up, the thirst, the constipation. So after 2 long years of medical appointments and testing Sophia has been diagnosed with ROHHAD syndrome.

( Rapid onset Obesity With Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation).

We have a long road ahead of us and not knowing when her body will stop working is very difficult. Just knowing that is a daily struggle. I will try and update Sophia's progress daily in her journal and will soon add pictures and medical updates and appointments. I want to thank everyone for the support I have received since receiving Sophia's diagnosis.

We are almost 2 years into our diagnosis and things have progressed with Sophia's symptoms and she has gotten more symptoms and has lots of medications to take each day. I have meet some great ROHHAD families since her diagnosis and the internet has brought all of us closer together. I will continue to spread the word about ROHHAD in hopes that more families will be able spread the word. And to help other families who are looking for a diagnosis for their child.

Tuesday, June 18, 2013


So we have a new Endocrine doctor.  I'm becoming a pro at new doctors.  She has worked with a ROHHAD child and understands the ins and outs.  I have all of Haven's latest labs printed out.  Her last Endo doctor wanted to get her on Growth Hormone shots immediately as she is seriously low.  So I will bring all of these labs with me and hopefully have a great discussion.

We hope to get on a good routine with this Endo doctor and hope Haven likes her.  We are also waiting on Pulmonary to call.  This is just because we haven't had a sleep study in two years and it's time for another one just to get a base line reading.

Wednesday, June 12, 2013

Nurse Patel ----- 2009 and 2013

As Reagann was getting ready to enter in to isolation at Hopkins I told her about a wonderful nurse Haven had in 2009 during her isolation stay.  This is Nurse Patel.  She was amazing and we truly loved her with Haven.  She even dealt with Haven's crazy sleep.  Well I received a photo from Reagann today and I thought I would share.

This is Haven in 2009 and Reagann in 2013.  We are so happy she got to meet Nurse Patel.  We are sure that Reagann will be well taken care of.  Patel has stayed in our hearts and minds for many years. 

She should be nominated as best nurse at Hopkins! 

Reagann is on her 3rd of 4 doses of chemo and has started to lose her hair.  She was able to join in a part at the hospital and she donated her hair to Locks of Love.

Amazing Reagann....we can't wait to see more photos!

Tuesday, June 11, 2013

Pediatric Oncology

If you've never had to go in to a Pediatric Oncology room or visit a floor you should count your lucky stars.  It's one of the worst places we have been in for the past 6 years.  You meet children and you play games and crafts with them and then the next time you visit you find out they didn't make it.  Or you see a newborn or a 6 month old baby receiving chemo and it just breaks your heart.  WHY?

Just very very sad.

Today they had a party for a little girl who was receiving her last dose of chemo and she got to ring the bell.  I was so happy for her and her parents.

IVIG Day.....spending the day at Primary Childrens

We are spending another day at Primary Childrens.  Today is for a round of IVIG.  Haven's levels were around 220 this time.

We've been doing this now since 2007.  Yes it does get old.  My daughter was 3 when we finally found the tumor and got a diagnosis and she has endured surgeries and chemo since.  I spoke with our oncologist in Connecticut and she informed me that the reason we are still doing IVIG is not because of the Hi CY but because of the Rituxan.  Which kinda shocked me.  I figured it was from the Hi CY we did in 2009 when we wiped out her immune system.  But she had about 9 or 10 doses of Rituxan and you're supposed to only do 4 - 5.  Since it's a new chemo there isn't much history on it....not to mention no one else has used it for ROHHAD the way we have.  So as of now no one knows when she will be healthy enough to not have IVIG.

In March we ran a lot of labs to do a full work up and found out that Haven's calcium was scary low - normal is 9 and she was at a 6.  This can cause heart failure and such. So she was immediately put on an enormous amount of calcium and then was retested a week later.  She has since been fine.  But we did find out that her growth hormone has dropped even lower and we will be meeting with Endocrine to start on growth hormone shots.

During the past few months Haven was living with her Grandma in Virginia and she started this no sugar - extremely low carb diet plan.  Amazingly - Haven has lost 24 pounds and just lost another 2 pounds this past week.  She has started walking 1 mile a day on the treadmill and is even starting to play games and throw balls with her sister and brother.  This is insanely different than the "usual" Haven.  Her blood pressure has stayed around 98/60 and her O2 levels haven't dropped below 97 in a year.  We will be doing a sleep study in the next couple of months just to get another baseline reading.  But I do not foresee any issues there.  We are so proud of her.

So I'm finally writing in this blog.  I figured with the amount of ROHHAD families that still find me it is best to keep this updated and informed and hopefully help more families along the way.  I will be putting our family updates in my family blog - keeping medical stuff separate.  (Wylene - make sure you check there.  If you need our address for Haven let me know - we've moved a couple times this past year).

Again - I would like to have everyone to please put these kids in your thoughts and say an extra special prayer for Reagann as she will be receiving her 2nd dose of Hi CY at Hopkins tonight.  Please send her a card or really cheers these kids up.  I will keep you updated on Reagann and other kids as I can through this site.  I'm starting to get a few minutes to myself each day and get to turn on a computer!

Love to all!

Monday, June 10, 2013


HI FRIENDS...Reagann has just had surgery for her PICC line and is doing great. She has an address in isolation room at Hopkins now. This is the same place Haven was in 2009. PLEASE help Reagann keep her spirits up with a card. Please note DO NOT SEND STUFFED ANIMALS OR FLOWERS. They are not allowed on the floor and could make a child seriously sick.

Her address is:

Johns Hopkins children's center
1800 Orleans St.
Reagann Duenne
Bloomberg 11S, Room 7
Baltimore MD 21287

She will be losing her hair (just like Haven) and will need some lovely hats and scarves. One of Haven's favorite things to get in the hospital were puzzles.

Thank you to all my friends for keeping her in your thoughts and prayers.

Thursday, June 6, 2013

Another day at doctors

Well I haven't had to say we've been at the doctors much lately.  But I seem to be spending some time in there lately.  Haven has some crazy infection in her toes - back in 2009 we had surgery for this.  We had her CMP checked and everything came back normal.  Oncology called over to the pediatrician with info on Haven.  I did the cliff notes version tonight with him about ROHHAD and Neuroblastoma Cancer.  The medicine he is thinking of putting her on is extremely hard on the liver and she would have to be monitored constantly.  So I'm not really into that.  Haven has done amazing since treatment and doesn't go in to the hospital really anymore.

Her IGG labs came back at 225 this time.  So she will be getting her IVIG on Tuesday.  She has been moved in to a 12 week schedule now instead of an 8 week schedule.  Which is amazing and one step closer to not doing this at all.  At least we can hope.  I talked with the doctors at her previous hospital and they said the reason she is having so many issues with gaining her immune system is due to the many doses of Rituxan not the Hi CY.  We really like the Rituxan when she was doing it.  :)

Anyway, we have a few more doctor appointments to figure out this infection.  I was very happy that her doctor is going to take a few days to research ROHHAD and the medicine he wants to put her on.  We should know more by Tuesday.

Wednesday, June 5, 2013

Haven visits Oncology

Yesterday we spent the morning at Primary Children's getting more labs.  We checked her IGG levels, Calcium, and did a full panel on the liver.  There are a few questions I had on some things that are requiring a liver check.  We should find out today if we need IVIG this week or not.  They want to push her to a 12 week schedule instead of every 8 weeks.  We found out that she is still on IVIG due to Rituxan - we thought it was the Hi CY.  We are hopeful she does not have to do this for many more years.  She's tired of going.

We are also getting set up to meet with Endo on a growth hormone stimulation test.  They want to start her on growth hormone shots.  She will also be meeting with Pulmonary soon to schedule a sleep study that is long overdue.

As of now Haven is doing great.  She is finishing up 3rd grade and going to 4th next year.  She has definitely overcome many obstacles and we're so proud of her.  She continues monitoring her own foods and watching her sugar intake.  She keeps to under 20 carbs a day and carries a carb calculator with her everywhere  ---- which can drive you crazy!  :)  So far she is down about 25 pounds and doing amazing.

Catching up on Haven

I feel like I never have time to write in her blog anymore and it's always a catching up thing....with three kids it's a bit harder.  Now that the other two are becoming more independent I think I can start diving back in to this! :)

Here are some photos of our last couple of months:

 Miss Haven with her grandma in Virginia.  She practically moved in with Grandma and Grandpa for about 9 months and had an amazing time learning and growing.  She started a no sugar diet and has lost almost 25 lbs now.

 Haven at the hospital working on crafts while waiting to get her IVIG infusion.

 The hospital staff fell in love with her and decorated her face and took photos for their paper.

All decorated up

As usual Haven does her own blood withdrawals before starting IVIG

I honestly can't remember when Haven had an Easter egg hunt and was able to do anything on Easter.  Thank you to Grandma and Grandpa for taking her out!

 Hanging out in Maryland visiting Grandma and Grandpa there.  We had a great time watching movies and hanging out.

A beautiful smile while getting IVIG treatments in April 2013.  

You can see from this photo that Haven has lost a lot of weight.  She just looks amazing but more important is that she has more energy and is actually sleeping much better.

Miss Rohnin protecting and watching over her sister while the nurse takes her vitals

 And then there are the babies.......this was in April.  They are really growing up.