ROHHAD in Ireland

 I received this story in my email today. It still shocks me that my daughter has ROHHAD. It's a horrible disease that destroys a child's life. There is no cure. Most doctors, if they take some time to learn about it, pass you around until some specialist says yes, they'll take you. Our daughter, Haven, is now 19 and has been living with this for almost 17 years. She was also diagnosed with Neuroblastoma Cancer, which is how we even found out about her ROHHAD. She's done experimental treatments to stop the progression. We believe it has helped tremendously but ultimately there is no stopping this disease. 

Please take a moment and learn about Caoimhin Adams, an 8 year old boy from a small place in Ireland. 

ROHHAD IN IRELAND

My condolences to the family. 

ROHHAD Life

It's been a long time...guess since February of this year.  Haven's had quite a few quiet years.  Last January we met with Oncology and did a lot of scans and located a spot near where here original tumor was.

But it was so small no one was really concerned and we let it go and decided we would watch it.  We went through life with a Neurosurgeon for a tumor in the pituitary in March.  That had been resolved and what seemed to be going well has changed.

She has continued with her C-pap and sleep studies.  We pretty much live at Primary Children's Hospital in SLC but one thing we were super excited about this year was her Pulmonary doctor from Hopkins moved out here.  Happily he is her Pulmonary doctor once again.  He is amazing and we love him.  Good to see a familiar face too.

She did a PFT - Pulmonary Function Test.  Which wasn't too great.

Haven has been on Growth Hormones for a few years and has also still been doing her IVIG every 4 weeks.  We've been doing IVIG since she was 4 years old and now she's almost 12.  Luckily she has a nurse that she absolutely loves and that cares so much for her.  She even comes in if Haven happens to need blood work on her days off.  We do our IVIG on the weekends which helps me with working.

In June I started noticing Haven's left shoulder drop and by September it had dropped a good couple of inches and her walk was a bit twisty.  So I called her Endocrine doctor - which to my surprise had retired since we saw her just 3 1/2 months ago.  So now she's getting injections and walking weird with no doctor to follow up with.  So I started calling all of her other doctors to find out what's going on and to start making appointments with them.  It's October - we still don't have an Endocrine doctor BTW!

Last week Haven met with her Immunology doctor and we went through all of her lab work and refilled IVIG for the next year.  But I brought up the shoulder.  She ordered an X-ray and instantly I knew what was up.

I snuck a photo of the X-ray.  We received a call confirming what I saw.  In the past 8 months Haven has developed scoliosis and it's actually pretty severe with a 47 degree curve.

Next up was Oncology on Friday to do another CT Scan of the area we looked at last January.  Now this I figured would be a waste of time but it's better to get it done and see what's going on inside this girl.

BAM ---- it's a tumor and it's grown quite fast in 8 months.  Immediately we are referred to a surgeon and went over the scan with him.  They do not believe it's another "blastoma" but he is concerned it could now be Lymphoma Cancer.  Due to Haven's being at an increased risk of infection she will not be doing the major surgery to remove it.  However, we will be going in to surgery for a biopsy on Oct. 29th.  That will be sent to Pathology to confirm what it is.

new tumor is circled

Orthopedic Surgeons will be seeing Haven on Nov. 3rd to review her back and options with everything else going on.

While doing all of this her doctors will be meeting with a specialist in Cincinnati at the Children's Hospital on the possibility of Gastric By-pass surgery.  The doctor in Cincinnati is the one who devised the procedure for extremely high risk children with diseases - especially of the Hypothalamus.  But she must be reviewed to see if she can handle this.

It's a lot and that's not even all of it but that's been our first 3 days in October.

THANK YOU MATT'S HELPERS FOUNDATION!!!

 

http://mattshelperschildren.org/

HUGE THANK YOU TO MATT'S HELPERS FOUNDATION!!!!

They have been there for Haven and our family since our diagnosis in 2007.  We've never had to ask them for anything but they always seem to know what we need and when we need it.  From gifts for Haven to gas and grocery gift cards!!!!

YOU ARE ALL AMAZING!!!!

Childhood Cancer Awareness Month - SEPTEMBER

SEPTEMBER IS CHILDHOOD CANCER AWARENESS MONTH!!!!

My daughter has strength I only wish I had. Now usually I discuss my daughter's other disease, ROHHAD Syndrome, but she was actually diagnosed with two and the other is Ganglioneuroblastoma Cancer.

After over 2 years of asking for a diagnosis we received it in 2007 - December 28th at 7:30pm. It's a phone call you can never forget.

"Mrs. Fowler, your daughter has cancer. You will be contacted by an Oncologist in the next 24 - 48 hours."

Only to find out after visiting many doctors that she actually is diagnosed Ganglioneuroblastoma Cancer and ROHHAD Syndrome. To get hit with one of these is devastating.......but two different life-threatening diseases! How the hell can this happen?

Our daughter underwent a 14 hour surgery to remove a tumor in her abdomen that was wrapped around both the Inferior Vena Cava and Abdominal Aorta....it was pretty close to cutting them off from supplying her body with blood. Two major arteries. During this surgery she also had a cath-o-port put in and a spinal tap with a bone marrow withdrawal.

Some general facts about childhood cancer:

• The National Cancer Institute’s (NCI) federal budget was $4.6 billion. Of that, breast cancer received 12%, prostate cancer received 7%, and ALL 12 major groups of pediatric cancers combined received less than 3%.
• Each day, 46 children are diagnosed with cancer
• One in 330 children will develop cancer by age 20
• 35% of children will die from cancer
• Cancer remains the number one disease killer of children; more than genetic anomalies, cystic fibrosis, and AIDS combined
• On average a treatment for childhood cancer diagnosis is two years
• The causes of childhood cancers are largely unknown

Neuroblastoma Cancer Facts:

• In the United States, about 600 children are diagnosed with neuroblastoma each year
• Every 16 hours a child with neuroblastoma dies
• For children aged 1 to 4 years at diagnosis, 5-year survival rate improved from 35% during 1975-84 to 55% during 1985-94. Survival at 5 years from diagnosis was essentially unchanged over these time intervals among infants (83%) and children 5 years or older (40%).
  

Cancer Research Funding

• Nationally, childhood cancer is 20x more prevalent than pediatric AIDS.
• Pediatric AIDS receives 4x the funding that childhood cancer receives.
• In one month there are 2x as many deaths from childhood cancer as pediatric AIDS for the entire year.

Cancer Warriors - Thank you Rachel

I want to send a huge thank you to Rachel Graham for including our princess in the latest Cancer Warriors video below. She is an amazing woman helping families share their story. I met her on facebook and it is great to see these kids and see their story through photos.

Our dear friend Jessica Magnus is also featured in the video! We love you Stana and Jessie! Please check out the video and if you are on Facebook please become a friend of Rachel Graham and learn about our children. If you do look her up the profile picture is the ribbon above!

Thank you again Rachel.

Confirmed to go

Haven's usual Oncologist, Dr. Paz-Priel, came in to see her today. I had a feeling he might show up and then I told Kris that I thought I saw him in the hallway.

He came in to talk to us about the Hepatitis B and what we would do if it came back positive. We passed back and forth a few jokes about how he just likes to make sure we're worried enough. He said a few more grey hairs wouldn't hurt! :)

He looked Haven over and checked her breathing. Everything looks great. He told us that the lab is not usually open to run these tests on a Saturday - which we were worried about. But they were able to call in a favor and the lab person came in to make sure we knew asap.

About 45 minutes later he came back in to the room with our Inpatient Oncologist, Dr. Farah, and said ALL TESTS ARE NEGATIVE!!!! Yeah! So we are good to go with Hi CY.

Haven should begin Hi CY within the next 2 - 3 hours. Our other Oncologist, Dr. Chen, will be checking in on her tomorrow. Haven will be required to go potty exactly every 2 hours after the initial receipt of the Hi CY. Even at night - the nurse will come in to wake her up every 2 hours to make her go to the bathroom. We can not leave the Hi CY in her bladded any longer than that or we will have major complications.

Also with the Hi Cy we are required to put this paper in to her diaper to check the amount in the urine.

We are extremely excited she does not have Hepatitis B. I'm really nervous now about the future blood transfusions she will have while on this treatment. But I really have no choice - I can't deny the transfusions.

Haven is finally sleeping. She did not sleep at all last night. She had the fevers off and on and was just very anxious to not be in her bed. So I am very happy she is finally out of it.

ROHHAD: Cardiology -- Pulmonology

These are the two next big doctor appointments we have.

Cardiology has been added as an extreme importance. Haven's resting heart rate has been staying around 150. It has been constant around 130-150 for over two months. But I remember her in the hospital last year and it stayed around 140. We should find out next week when this appointment will be made. She will wear a monitor around her chest to monitor her heart rate while awake and active. I received some emails about heart rates and emailed NIH and our doctors. The first number being on the low end (some of our friends stay around this when resting) and the second number being on the high end.

EDIT: Just heard from the doctor - they use the Harriet Lane Handbook for resting heart rate calculations:

Ages 5 - 7:

Low End: 65
Average/Normal: 100
High End: 133

According to NIH resting heart rate:

Newborn infants: 100 - 160 beats per minute
Children 1 to 10 years: 70 - 120 beats per minute
Children over 10 and adults (including seniors): 60 - 100 beats per minute
Well-trained athletes: 40 - 60 beats per minute

Her doctors are also putting in a referral for a Pulse Ox monitor. We didn't believe we needed one only a month ago but on Friday I received an email from her doctor and the referral has been placed. She will wear this at night when she is sleeping - it will monitor her heart rate and oxygen. I am very relieved to have the doctors ordering this. I have been extremely concerned about her during her sleep. This will at least ease my mind a little.

Pulmonary will be on April 16th. This will be a very important meeting and our first with the doctor. We will discuss all of Haven's sleep studies, the ABG, and other information that I have received and noted from the other mom's that I am concerned about.

Endocrine will be on May 4th. This will be our 6 month follow up meeting.

Oncology also informed Kris that every Friday Haven will now have full labs done - not just Cyclosporin levels. They will test the hypothalamic hormones, Cyclosporin levels, sodium levels, and other needs for the lab testing. This is more than Haven usually has but is needed for the records and to see if anything is changing, good or bad.

Haven will continue on this chemo treatment for one year. At 6 months the Oncology team will review all of her results with the medical board.

There is the small quick version of it all.
Thank you to everyone
Love ya

Grandparent's Week

Haven had a busy week last week as her grandparents came in and out throughout the week. After April 1st we will be keeping the house locked down pretty tight as we get deeper in to Haven's chemo so everyone had to come see the Princess.

We were missing a couple of the grandmas though - Grandma Sing was hanging in Utah and Grandma B-Honey is in Korea. We have a few Great Grandma's out there that we want to say HI too...we don't see them much at all but we wish we could.

We had a blast the entire week and Haven kept everyone busy.

Grandma and Grandpa Virginia came out early. Grandma stayed with Haven for the entire week and they were constantly working on crafts when Haven wasn't at the hospital. Although they only had 2 full days the entire week together they stayed really busy. As you can see in the background they worked on the tree. Now I haven't had time to take it down and we joked about having a tree for every holiday. Haven is now working on a new tradition - we have the first Easter Tree. As you can see they made egg ornaments, bunny ornaments, and a big easter egg bow is on the top of the tree. It's absolutely adorable.

They also made a St. Patrick's Day set up with a leprechaun on the side table. He is still there and she has been reading her books with us each day. I'm sure all of this will stay where it is until Grandma comes to change the holiday look. :)



Later in the week, Grandpa Beard Off came to visit us from Utah. He brought with him a most amazing quilt made by Grandma Sing. There are sections on the quilt for all of her family members. There are also hidden messages sewn in to the quilt that say "kisses" "hugs" "Haven" and flowers and butterflies. The quilt is absolutely amazing. There are also little charms sewn on to match different family photos. Haven has been asking to sleep with it but mom has put it away in a zipped back to keep it safe. Haven will absolutely cherish this quilt as she grows older. THANK YOU SO MUCH GRANDMA SING (and grandpa for your computer skills)....WE LOVE IT. We hope you can visit next time.

Haven randomly likes to try out new smiles. She practices them in her floor length mirror and this is one of her new ones. She says she doesn't do big smiles anymore because she's a little bit bigger now. I know, I have no idea what that means but I don't really argue with her much. But Grandpa certainly doesn't have a problem with big smiles! :)

Haven and Grandpa stayed in this position for two days. Haven asked me on Saturday night if she could stay up and "party". Of course I rarely say no to her and her Grandpa hasn't seen her in over a year so she partied. She and Grandpa worked on this sticker book that was sent to her from a MACS friend. They started the book right after chemo at 8pm and we had to beg Haven to go to bed at midnight.

On Sunday, She and Grandpa started early. We couldn't tear these two apart. I thought we were going to have to pay Haven to let Grandpa take a break. Haven's friend Mason sent her a 1st grade workbook that is about 500 pages.......They worked on this book from 8am until 11:30pm that evening. I think Grandpa took two 15 minute breaks to help Kris with the trash (sorry to put you to work Gpa). I'm not even sure Grandpa ate the day.

PS: They almost finished the entire book!

Before Grandpa Simon left with Grandma on Sunday we had to have a Grandpa photo. These are Haven's Grandpa's and some of the men that love her like no other in this world. You have Grandpa B-Honey, Grandpa Simon, and Grandpa Beard Off.

Wow, doesn't my girl look older! She has no make up on but she looks like she does. She has changed so much over the past year. She is our world and our princess.

Thank you to everyone for visiting our girl. Everytime we see her smiling and laughing and having a great time we just take it all in. We stop whatever we are doing just to hear her laugh. She has never giggled and laughed as much as she did this week.

All our love to our families for the support and care you have shown Miss Haven.

PS: we have lots more photos on Haven's flickr site at the right side bar. Just click the box and you'll see them or click a photo above.

Pre-Op appt

Haven and I met with her Pediatrician today. It was a great meeting - we talked with her doctor for about an hour about everything that has been going on and how I feel and how Kris is doing.

Haven's Pre-Op exam was good. Mainly it's to review all of her medications, anesthesia she is not allowed to have, any past issues we've had with surgeries or anesthesia.

I received some info from Haven's medical files. Which is always nice to have them in our files.

It's weird......the doctors tell us that Haven has a serious disease and all that. But it's not until I read it in her files from last week where her doctors states "serious illness which is life-threatening" that it actually hits you. ROHHAD is a disease my daughter could easily die from --- not her Cancer.

All of our children with ROHHAD are in a life-threatening position that we have no control of. Our children may look fine today and be in the hospital for months at a time tomorrow.


Miss Haven is in Chemo all day today. This will be her 2nd dose of the Rituxan. We have been closely monitoring the toes for Tuesday's appointment.

Hope everyone has a happy Friday the 13th!

Chemo and Infections

Haven started her first dose of this other chemo last Friday. By Saturday, there is an infection in her big toe on the left foot. By Sunday morning, there are three more toes on the right foot that are infected.

By infected - I mean green, gooey, swollen.

By Sunday afternoon her big toe on her right foot is cracked open and bleeding. The bleeding has continued and during chemo yesterday at the hospital it was bleeding again.

She had 3 doctors look at her feet yesterday and the decision was made that she needs surgery on the toes.

Haven has less than a month to do this surgery because at that point her immune system will be gone and the risk increases significantly.

Considering that we already have surgery scheduled for her eyes on March 26th this only leaves me with next week available. Haven has an appointment with the surgeon on Tuesday, March 17th and hopefully we can get the surgery done next week.

Making it two surgeries in two weeks. If we're hard to reach over the next few weeks you will know why.

Neuroblastoma Tumor

MRI results came back today - Kris reviewed them with Haven's doctor and EVERYTHING IS GREAT!

The tumor that still remains has not grown since last May so we will continue to focus on ROHHAD and monitor the cancer.

Thank you to everyone thinking about Haven! She is currently in chemo at the hospital. They have been there since 8am and she should be coming home today around 5pm - as long as it all goes well.

Love to all

Comparison photos - ROHHAD and Neuroblastoma signs

So - randomly I go through photos and remember when everything was normal in our lives. Here are some photos of Haven right before everything went crazy. These are from 2006 - I have uploaded a bunch of photos to her flickr site. You can click HERE to see them.

Haven working on bikes with her dad in the garage. This was in May 2006. She was 2 years and 4 months old.

June 2006 - Uncle Kevin's graduation.

Haven with big sister Kendall and Grandma Sing

Haven with Grandma Virginia.

June 2006 - Having fun with Kendall

July 2006 - the very next month. Gained about 25 pounds in one month.

With dad - July 2006

Haven and Kendall - December 2006 - after many multiple doctor appointments. Diagnosis - mom overfeeds child reduce calories to 1,000 per day.

December 2006

Haven's 3rd birthday - January 26 2007. Begins having major stomach pains putting us in the hospital and doctors thinking mom is insane. Doctors start treating me like I have Munchausen Syndrome. Begins to have blood in the potty and doctors diagnose that she is pushing to hard give her prunes.

3rd birthday party. Wearing size 6 at this time.

Gained approximately 35 total pounds (up and down) in 6 months.

Haven's ROHHAD Update 2-12

Today was a long day. We headed down to Baltimore for our first visit with the Orthopedic Surgeon that will be watching Haven's bones as she starts to grow. We've also had many severe back pains that seriously just paralyze Haven and she falls to the floor in a ball grunting.

If you remember I took her in on Dec. 5th for a CT Scan of her back to check for fractures. We were clear on that. Then a few weeks later it happened again when she was playing.

I took some photos of her xrays today from the computer screen. Sorry they are from my phone so they are not too clear. But I did detail them so you can see what is what.

This is an xray of the front view - sorry I didn't get pictures of the side view. Haven really wanted them too. This xray shows you the Port, tumor on her back, curve in the spine, and original location of the neuroblastoma tumor. You can really see how big the tumor on her back is from this xray.

A normal spine of a child at her age is 45 degree angle. Haven is currently at a 51 degree angle.

I realized that most people probably don't know what I mean about a port. She has a port in her chest which is essentially like a little rubber stopper. It is sewn in under her chest with about a 3 inch scar.

To access Haven's port they use a 1 inch needle each time that goes in to her chest at the port. Then there are little butterfly wings that clamp the needle down on to the chest to stay in for the day for medicine and IV fluid. The port has a line it in that goes through her artery up in her to her neck. She has a small incision in her neck to pull the line through. You can feel the line but you can't see it.

From today's meeting we now know the status of Haven's spine. We also learned that her bones are very brittle and as of now still a bit spongy. They are starting to squish together as the discs between vertebrate get smaller. This is causing her the pain. He did not see any fractures but with the shape of her spine and the weight he said she could easily have a fracture at any time.

He mentioned the possibility of Haven having Brittle Bone Disease due to the long term use of chemotherapy, high levels of steroids for over a year, and now more chemotherapy. She has not been allowed to have any vitamins for over a year and we removed milk from her diet due to high sodium levels. She has been drinking milk for over a month now.

Tuesday, February 17th, Haven will have a bone density scan to check her for Osteoporosis. Her Endocrinologist will handle it from that point unless surgery is needed.

Wednesday, February 18th, Haven will see Dr. Price (original surgeon that removed most of her Neuroblastoma Tumor) in regards to the tumor on her back. He will examine her and discuss surgery to remove it. 90% of the time these tumors are not cancerous (we're told) but they will remove it and have it tested.

We have another sleep study planned for this weekend in Mt. Washington Pediatric Hospital. This will be Haven's 3rd study. They will be videoing her the entire night this time.

Ultrasound is February 24th

Eye Surgery is March 26 - along with Blood Gas testing

As everything unfolds or we get more news I will keep everyone updated.

We hope all of you have a great Valentine's weekend. Haven has received many Valentine cards from around the world. Thank you to MACS site for all your help with all our children.

Thursday visit to Arnold Palmer Hospital

Haven's levels needed checked - even on vacation. So we headed to Arnold Palmer Children's Hospital Pediatric Clinic. Haven was very good and she helped the nurse out a lot.

!
I received a phone call that evening. Haven's levels are too high again. So we have adjusted her chemo to a lower dose until we can get back home and back to Johns Hopkins!

Thank you everyone at Arnold Palmer for helping my baby! :) ooohhhh....I mean big girl! We were all corrected many times that she is no longer a baby - she's 5!

Update on Haven

Quote from Haven's Oncologist that sticks with you: "I feel a lot of pressure. Chemo is a slow methodical process that you have to tweak to make right and it takes time. ROHHAD is a scary syndrome and can become a life threatening emergency overnight. Time is against us with Haven".

Haven had a full day of chemo on Friday. Dad and Haven got in to the hospital around 7:45 am to get ready for Haven's 8:00am blood test for her chemo levels. Then immediately after that started the other chemo for the day. I heard that after they gave her the Benedryl she passed out for a while. Let me tell you, when we do all day chemo treatments at the hospital and Haven is just laying in the bed waiting for the bottle to empty (6 hours later) it can be a LONG day! So sleep is always welcomed......I always request it to be taken through the port instead of orally! It hits her faster and helps her sleep quicker. Plus throw a hot blanket on her (they have these heated like from the dryer at home) and she is OUT!

Kris met with Haven's Oncologist for quite a while in the morning. Then I got a call around 11am to let me know what they had discussed and if there was anything I needed to add or if I had questions. I had to work so I couldn't be there. I know Kris gets nervous going in by himself because I'm the one the keeps track of dates, surgeries, doctor appointments, and research of ROHHAD. So I sent him a text of a few key items that he needed to discuss.

Haven will be having another surgery planned. We're waiting to hear from her surgeon as to when he feels it should be. Her eye surgery is March 26. This other surgery will be for the massive lump on her back. No one knows what the lump is but it is bothering her a lot lately. So once I hear more about that I'll update everyone. We will also be meeting with an Orthopedic doctors for her back and starting physical therapy as she is having too many back pain issues.

I received a printout from the doctor of Haven's weight and height since last January. I wanted the comparison for my records from the past year. It also shows her blood pressure each week and temp. So this was very exciting to me! I'm easily excitable!

We are now required to do a daily log of any changes and anything we notice. So we can look back on the records in one month and decide if she should be off this chemo and put on something else.

Haven's chemo dose was increased on Friday and Haven and I will be at the ER at Johns Hopkins on Monday morning at 7:45am! Whew.....luckily I have the day off and don't really like sleeping in! :)

Love to all!
M

ROHHAD Updates

Well I haven't updated everyone in a while on all the angels with ROHHAD. There are quite a few things happening. I'll start with my baby first!

Haven with a Paula Deen autographed picture

HAVEN: She is at the hospital right now getting her levels checked and has a full day of chemo. They will be meeting with her doctor to discuss how everything is going. They will also be discussing possible back surgery for Haven, upcoming eye surgery, upcoming pulomonology appointment and anything we've noticed while on this daily chemo. Haven is currently sleeping about 4 hours a night with constant wake ups and major pottying problems. She has gained 4 lbs since the beginning of December and 2 lbs in just the past 12 days. I have been keeping Haven's FLICKR site updated with photos from years ago to now. We're also excited to have Haven featured on MAKE A CHILD SMILE for January. We're working on her birthday party for next weekend and then we're off to Disney for a week!

Mason and his dad on New Years Eve

MASON: I talk with his mom daily! He is doing pretty well. He was scheduled to see Dr. Weese-Mayer on January 12th but that fell through with insurance not accepting. ROHHAD is a rough syndrome - no one knows about it and insurances don't want to pay for something that isn't known about. Mason has been slightly congested and since he is on the ventilator whenever he sleeps there house can get crazy! I love the stories. Every time he coughs or sneezes his ventilator alarm goes off and everyone is immediately in his room. They are on the same schedule as we are...wake up about 4 to 5 times a night. Funny story: Mason was sleep walking about a week or so ago and they woke up with alarms going off and all this craziness. Mason remained connected to the ventilator and dragged it down the hallway with him. I could not stop laughing.....I know what we go through at night and I could imagine waking up to all that! We also found out that Mason is a huge fan of Minnie Mouse! :) His baby sister, Cameron, is always stealing his Minnie. PS: I'm pushing Julie to get a site up for Mason!!!

Sophia and her big sister

SOPHIA: Miss Sophia has had a REALLY bad time through the holidays and beginning of the new year. I keep in touch with her mom as her schedule allows. On Dec. 27 Sophia had her sleep study and slept the same as at home. Waking about every hour, talking and crying out in her sleep and as she normally does everyday, woke up at 5:30am and threw up. On Dec. 30th Sophia had her MRI for the full body - to locate any masses. This trip ended up lasting 6 days in the hospital for Sophia. The conclusion of severe sleep apnea led them to rush Sophia into surgery to remove her tonsils and adnoids. Due to her low oxygen level Sophia remained on oxygen most of the time. During the MRI they noticed that Sophia's lung was full of fluid and was essentially getting squished. They suctioned it out and then again during surgery they had to suction it out again. Today Sophia is doing a bit better. She has another sleep study at the end of February, meeting with behavioural medicine. Her Cortisol levels and Prolactin levels have been pretty high so they are waiting on word regarding those. You can check out Sophia's web site HERE or on the side bar of Haven's.

Josh and his two cousins

JOSHUA: As you know - just at the holidays Josh was headed to the hospital for some major testing and meeting with his Cardiologist. It seems as though Josh has developed a pretty major sinus infection that alerted the doctors to start a test for Cystic Fibrosis. They don't believe he has CF but it's definitely best to rule it out - who knows what this syndrome brings with it. His Pulmonary tests showed more evidence of airway obstruction and he will now also use a steroid inhalent. Josh is on the ventilator 24 hours a day. He is allowed to take small breaks from the ventilator but he is watched closely. He has had a few high temperatures over the past week. We're constantly checking in on Vanessa's blog to make sure Josh is doing well and the entire family. They have some great photos up that are awesome. I love to see everyone.

NIKKI: I have not heard from Nikki's mom in Australia since right before the holidays. We hope to hear from her soon! Hope all is well down there and Nikki is home or on her way home.

Haven's Schedule

Kris took Haven in to the doctor on Friday to have her levels checked again. I honestly didn't know what high levels meant or what we were even looking at. After having her levels checked Kris met with Dr. Paz-Priel to review everything.

Haven is now scheduled to be in the hospital to have her levels checked three times a week - Monday, Wednesday, and Friday. She still takes the chemo two times a day (8am and 8pm) with her other medication. We just lowered the dose temporarily. Once she is stable they will again increase the dose.

Normal levels on this chemo (which I just found out on Friday) are at 150. Haven was at 300. So double the normal. I haven't listed the name of her chemo because it's too confusing when you read on it. Usually this chemo is given to bone marrow cancer patients or Leukemia patients. So it doesn't make sense to the normal person when they check Wikipedia or something. But we understand why she is on it and how they came to decide to use this one.

This week:

Bloodwork - Monday
Full day chemo - Tuesday
Bloodwork - Wednesday
Pulmonology - Thursday
Blood work/Dr. Paz-Priel - Friday

Kris will take Haven every day and I will meet with him on Thursday at the hospital. I have never met the Pulmonology doctor and have a list of questions for him.

ROHHAD / Neuroblastoma Treatments

Here is an update on Miss Haven – it’s good with the bad

Cortisol levels are great. Haven is producing Cortisol on her own which is AWESOME! We were a little worried about this but maybe this means we won’t need to do growth hormone shots in 09! It’s always a plus when Haven has good news. We hope to see her start growing now.

Bad news: Haven’s body wasn’t handling the new chemo very well. She had her levels checked today and I received an email from her doctor that they were way too high. So we were instructed to skip tonight’s dose of chemo and to only do half the dose tomorrow. She will go back to the doctor at 8 am on Friday to get her levels rechecked. We hope they level out or she may have to switch chemo treatments already.

I have been asking the doctors about her symptoms but I still have not gotten an answer on that. Since starting this new chemo last Wednesday she has not slept for more than 4 hours in a night. Her gums are always sore and I have been loading her up with bubble gum flavored Ambesol. She has been peeing like crazy and she is achy all over. We were a bit concerned this afternoon. Haven fell asleep around noon and could not wake up. Kris tried waking her up many times and she just couldn’t do it. He finally got her to get up around 5pm but Haven sleeping like that is very unusual. I was sure we’d be at the hospital but she seemed fine while I was home.

I’ll update again after her levels.

Kris did take some video yesterday of her sleeping – I guess it was pretty crazy. I haven’t looked at the videos yet but I’ll post them up.

The New Year: 2009

We hope everyone had a wonderful holiday break. We definitely had a nice enjoyable much needed break. We're trying to get back in the swing of it now and get back to "real life" as much as possible.

We have so many people to thank for everything they have done for Haven this year. We want to thank everyone that has donated to her - everything is much appreciated. To everyone that sent her gifts and cards - THANK YOU. To Johns Hopkins Riverside - THANK YOU! Our Disney trip is right after Haven's birthday!

Over the break I RARELY looked at the computer (can you believe it) but here are a few donations that came in that we want to say THANKS!

Aunt Dottie and family - thank you for the donation
Rick Family - thank you for the donation
Stilwell Family - thank you for the donation
Shockey Family - thank you for the donation

Now back to your regularly scheduled program!
Love to all

ROHHAD / Neuroblastoma Treatment Plan

We have been waiting about 2 weeks to find out what Haven's treatment plan will be for 2009. We were certain it would be an elevated and more frequent dose of Rituximab but this is not the case. I spoke with Haven's doctor on Tuesday and we are starting her first dose of chemo tonight at 8pm. It took 24 hours to get the medicine made and ready for us.

Haven will be on this plan for probably a year but can be longer if necessary. During this time I will keep a journal of everything happening with Haven. This includes her eating pattern, her food requests, her pottying, number of diapers used in a 24 hour period, her temperment, her sleep/non-sleep patterns. Any little changes we notice will be documented.

Just a reminder THIS IS NOT A GUARANTEE that it will even do anything. We have no cure for ROHHAD so we're hoping to see some changes or we know it's just not working.

Rather than go in to all the side effects and details of the medicine I will just let you know it's generally the same as all other chemos: breathing problems, kidney issues, high blood pressure, etc etc. So we will also be keeping close eye on her.

Her schedule will consist of:

• 2 doses of chemo every day at exactly 12 hours apart - 8:00am and 8:00pm
• mix with shot glass of whole milk (I wasn't excited about the whole milk part) at room temperature
• 2 times a week Haven will go to JH for blood work by 8:00am. Her chemo will be brought with her to take immediately after the blood work.

Why are we doing blood work 2 x a week. This medication has the possibility of throwing her "levels" out of whack all over the place. So they need to keep track of it closely.

So going in to 2009 - we are thankful to still be bossed our by our little princess and we are trying to look at the positives as 2008 was filled with so many negatives. This is going to be a VERY hard year. Kris will be taking Haven to 95% of her doctor appointments as I have to work and we'll need to keep on each other to give her the chemo at exactly the same time every day at 12 hour intervals! We're buying an alarm clock tomorrow that will be the "Chemo Clock".

OH - We find out on Friday if she is making Cortisol! I SUPER DUPER hope so!