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.

Alaska Beauty Queen helping in Childhood Cancer Research

Honestly you hear A LOT about people doing stuff for cancer research.  Breast Cancer ribbons are on just about EVERYTHING you can imagine.  It's almost used so much that no one pays attention anymore.  But about as rare as childhood cancer seems to be is as rare as you hear about someone doing a cause for it.  So I am to post a wonderful story I read this morning about an Alaska beauty pageant winner helping in childhood cancer research.

A few years ago I met an Alaska beauty pageant winner - Aleah Scheick (photo above from her beautiful wedding) -  we have been amazing friends since.  After winning she was diagnosed with Non-Hodgkins Lymphoma.

I would like to say thank you to Miss Alaska's Debbe Ebben (photo above) for shaving her head and raising money and awareness for childhood cancer! 

Miss Alaska Shaves Head for Childhood Cancer

Welcome 2011 and IVIG

Yes it is a brand new year and I'm telling myself to be positive that it will be a GREAT year. We definitely have a lot of exciting stuff happening this year.

We definitely rang in the new year with a bang.....I was asleep by 10! :) Doesn't mean miss Haven let me sleep for too long but I did try.

We did finish the year with two great movies - Gulliver's Travels and TRON. Haven really enjoyed them but liked Gulliver's Travels more. We had a wonderful Korean New Years dinner at Grandma and Grandpa's house.

Haven was scheduled to go in for her chemo boost on January 3rd. She's had a pretty good cough since the beginning of November that seemed to taper with her last dose of IVIG. But it came back right at the holidays. So we were anxious to get in to Oncology - especially after talking with Pulmonary and her pediatrician during the holidays.

This is a photo of Haven's port while it is being accessed for chemo. She has had this cath-o-port since her first surgery in Feb. 2008. She had two ports at one time and thankfully the Hickman line was removed (which was the other side of her chest). She was a bit sad to miss the first day back to school - she couldn't wait to get in and see Mrs. Petz and her friends. Plus the constant reminder to get her birthday invitations ready this coming weekend.

These are the "tubies" that connect in to her port to give her the medicine.

As you can see to the right of the photo - Haven won a prize yesterday from one of the nurses. She used a thermometer under her tongue for the first time EVER! So this was a major deal. She usually has it put under her arm. Why she can go through multiple surgeries, tests, scans, chemo and not be able to put it under her tongue is the Haven mystery, as she would say.

Haven also had a chest x-ray which we are waiting to hear if anything comes of it. At this point it's been almost 24 hours so I doubt anything is wrong. We have a prescription for her from her doctor but we are waiting a few days to see if the IVIG helps. Her Oncologist says this isn't how IVIG works but as parents we noticed a difference on the last dose. She did sleep ALL night last night - first time in 3 weeks. So if it is IVIG then I like IVIG because I get to sleep!

We also have an appointment set up with the ENT on February 1st. I'm still waiting for the call back from Pulmonary to have Haven fitted for a cpap. At least as a temporary check. We want to do 2 weeks to see if there is any improvement - if not then we will discuss discontinuing this with her doctors.

We hope everyone had a safe and happy new year! Bring on 2011

ROHHAD sucks

As with most mothers of a child with Cancer I should be stressed and worried about this and it coming back. If you sit and think of one of the worst things that could happen to your baby it would be that they would be diagnosed with Cancer that randomly selects who it wants to attack. However, then you throw in ROHHAD and I don't even have time to think about weather or not Haven will have Leukemia before she is in her mid 20's or if all the chemo she has taken will cause another type of Cancer we have yet to hear about.

Haven has done a couple years of experimental chemo that has completely regressed some symptoms and helped her in so many ways. She is a completely different child now than she was when we first entrusted Hopkins to save her life.

In 2008 (at age 4) we were told - Haven is on the progressive road to stop breathing on her own by age 7.

I have been terrified of this number since then....almost 3 years. As it is Haven is set to turn 7 on January 26th.

In early November I scheduled a meeting with her Pulmonary doctor and told him that I had noticed Haven was snoring a lot and very loud. I also noted to him that her chest was caving in a bit while sleeping like it was hard for her to breathe. Her sleep walking episodes are also from 4am - 7am. Sleep walking should start around 3 hours after falling asleep in general.....not when you are about to wake up. So she was evaluated by two doctors and quite frankly when I request a test they usually don't argue if I bring some facts.

So Haven was scheduled for sleep study immediately and we did her test on November 26th. During that time we changed phone numbers a few times and my numbers weren't in the Hopkins system so they couldn't get me. Finally Haven's pediatrician got the info and sent an email to me to urgently call her and come in.

So on Dec. 22nd I rushed over to the Peds office and reviewed Haven's sleep study results in detail with her for about 45 minutes. She gave her suggestions and listened to what I wanted. Then we conference called her Pulmonary doctor to review the tests and figure out where to go from here.

Comparison (normal CO2 being between 35 - 45) :

Feb-09 Aug-09 March-10 November-10 Oxygen 98% 97% 98% 93% CO2 49 50 45 51 Resting HR 160 145 90 85

So you can see in the above before and after chemo - those are just some basic stats of Haven. It's hard enough for family and friends to understand and comprehend anything about this disease so we'll keep this easy! :)

So in 2009 I was told it is VERY difficult to jump up from 50 to 51. That it is a big jump to go up one point and they didn't think Haven would do it any time soon. Then after he chemo she actually dropped 5 points. Which was amazing and Haven has never been diagnosed with an apnea in any of her million sleep studies.

November 2010 report:

• CO2 jumps up 6 pointsO2 drops
• Approximately 5 episodes every hour
• Sleep efficiency rated at an 81%
• Abnormal gas exchange
• Prolonged period of wakefulness from 3:30am until 4:30am (not related to seizure but is parasomnia)
• Snoring is a level 3 out of 4
• No significant central apneas
• Paradoxical inward rib cage motion (her chest is caving in when trying to breathe)

Haven is now diagnosed with Moderate Sleep Apnea from zero apneas.

Obstructive apneas occurred during REM sleep
Desaturation episodes occurred during non-REM sleep


So what is next???

Chemo and meet with Oncology on January 3rd (due to Haven's drop in IgG levels she will have chemo for her next few visits no matter what her labs are)

ENT - we have been referred to one of the top specialists at Hopkins for Pediatric ENT. I will schedule this today to have her Adenoids and Tonsils checked. You know tonsils but adenoids are hidden here:



Pulmonary - fitting Haven for a C-pap machine, however, discussing my growing concerns with this as well. If you haven't seen one it looks like this and she'd wear it every night at bed time.

If ENT concludes that Haven's adenoids and tonsils are too large and wants to do surgery as of now we have decided to decline this. It has to be an extremely good reason that a C-pap wouldn't work. This would be Haven's 8th surgery and who knows how many times she's had anesthesia for MRI's. Each time Haven has anesthesia she is in the hospital longer and on Oxygen longer. Yes I know this is a basic surgery and whoop de doo......but Haven's last surgery was only 30 minutes long but left her on oxygen for 2 days.

Fitting for C-pap will happen after the first of the year - which will then include multiple sleep studies to make sure it is at the right pressure. And yes I know a C-pap is a whoop de doo thing too....but not for a 6 year old. So if you are age 45 don't say "oh I have one of those, it's nothing".....did you have one when you were 6? We haven't even decided if this is what we want to do either.

Anyway.....this was our latest sleep study. Usually I forget we even had them and am reminded by a doctor when they say it was uneventful. But this time I had her doctors hunting me down.

Yeah.....on to 2011

Delayed updates!!!

Wow...I'm a slacker but I assure you I am extremely busy!!!! We've also just been enjoying living lately and Haven loves to have her foot and leg massages every night! Sometimes I'm massaging her for an hour at a time!

Thank you to everyone for continuing to send her gifts and cards. The above picture was from a girl scout troop and was filled with lots of goodies. Thank you Matt's Helpers Foundation for the gas cards, gifts to Haven, and her Target card. She bought quite a few craft projects with her gift card. Thank you to Casey Cares Foundation for the wonderful Dutch Wonderland passes - we plan to use them when the heat goes away! :)


Well, Miss Haven went to the dentist a few weeks ago and was given an A+ for her teefies. Even her adult molars are already in. However, we seem to be missing the entire front section of teeth! I believe the tooth fairy is now filing bankruptcy - we've lost 5 teeth and 4 were in the last 6 weeks.

Miss Haven is having a very good summer - much better than last year. We love our doctors, nurses, and hospital but it's so much better NOT to be locked up in isolation. We did forgot about an appointment in Oncology last week - she was to get her IVIG. So she is there today - I'm just waiting for news and to hear about her counts. We can tell her counts are low as she has had a couple infections. Seems the toes are coming back to haunt us again.

Miss Lauren has been hanging out with Haven most of July. She comes over 3 times a week. She is moving to Hawaii in August so Haven is getting as much time with her as possible.

Haven goes to cancer camp in 2 weeks. She has asked if she can spend the entire week there and not come home at night. This makes me very nervous but if she was going to stay over night with anyone besides mom and dad who better than her nurses and doctors and kids who are just like her. We are still deciding what we want to do. Not like we wouldn't go pick her up at 2am if we received a phone call.

Then shortly after that Haven will begin 1st grade. This will be a big change for her. We are going to have her go a full day - 8:30 until 3:45. No nap and minimal snacks....she may panic at first. But again we are less than a football field away from the school so it's easy to go grab her.

She has Endo and Pulmonary coming up before school starts and one more sleep study. I am fairly certain all will be good news. Since the chemo last year we have not reversed back to the bad side....everything just keeps getting better. It's a slow process but a good one. I'm very happy we took the chance and trusted our doctors....Haven is such a different child now.


Love to all - will update with anything from Oncology! OH we have lots of new ROHHAD friends out there in the world. I will be updating the Friends of Haven blog soon!

TWO YEARS

IMPORTANT DATE: JUNE 6, 2010 --- NATIONAL CANCER SURVIVORS DAY


Haven's stage of cancer is noted to have a 70 - 90% five year survival rate.

There are less than 5 children out of 1,000,000 children born who have Ganglioneuroblastoma Cancer.

Well it's been exactly 2 years today since Haven received her first dose of chemotherapy - RITUXIMAB. She began with about 2 months of chemo 4 days a week that tapered over the next year.

Exactly 1 year ago Haven began her 5 years to become cancer free!!!! So in April 2014 we will have a huge party at the house as Haven marks her 5 years!!!!

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.

ROHHAD and RARE DISEASE DAY

Just wanted to remind everyone - this Saturday, February 28th is Rare Disease Day.

RARE DISEASE DAY is not only in the USA. There are events planned all around the world. Check out the web site and check out your location. UK starts on the 25th!

While I am constantly researching ROHHAD and Ganglioneuroblastoma Cancer I sometimes run across new sites that I have never seen before. Mainly because they have my daughter's name in them or they list something relating to ROHHAD. About a week ago I found Little Miss Hannah's web site. Hannah is 6 months old and is fighting Gaucher’s Disease Type 2 or 3. Since her mom shared Haven's link to bring awareness to Haven's rare disease I wanted to share with you Hannah's link.

I would like to thank some other brave women who have emailed or sent me letters about their child's stuggle with ROHHAD. Although they are worried that it may push me to lose all hope to hear their stories I am overjoyed to hear from these moms.

Although some doctors want to make this disease be our fault and accuse of us causing this disease we have to be strong in knowing we did nothing as parents to make this happen. We all have the same story from our child's birth. I am reading your letters and everything is like deja vu - I'm sure all of you feel the same when I send you Haven's story.

For 2 years we were told it was our fault and we were overfeeding our child and we were doing everything wrong. I have been angry for 3 years now and even when I tell myself I'm not going to be angry anymore I am. ROHHAD SUCKS! It's horrible....I hate it. No one understands it and it seems like you are alone. None of us have slept in years due to worry about our children. We all pretend to go on like everything is normal....but when I take my daughter shopping for clothes I am reminded it is not normal.

People stare and believe it or not, people say stuff. People come up and say stuff about how our children are fat and that we should be ashamed of ourselves as parents and how we are ruining our child's life. I'm sorry but I want to go insane on these people but out of respect for my daughter I keep it as civilized as I can. I can't promise that I'm always civilized. I know all of us as parents with ROHHAD children are going through this. AND I shouldn't have to lock my daughter in her house so she won't be stared at by ADULTS! Anyway, I hear all of you and I completely understand what all of you are going through...we just have to be there for each other.

I took Haven shopping Saturday to buy some new clothes - as she has again grown out of everything we bought only a couple of months ago. She didn't want to try anything on - she didn't even really care to look at the clothes. She told me "I don't know why we come shopping - nothing fits me". This is extremely difficult for me to hear and even more for her. I don't know what to do here. She's 5 years old and wears a size 12/14 shirt.

April 1st Haven's doctors meet with the medical board again about her ROHHAD and where to go. Until then I will be working on some reports and charts for the doctors to take with them. I am collecting data from all of the mom's I talk to. I have a few that do not want their name or daughter's names used - and that is fine. They are still sending me all the info I need to make my report.

Some day there will be a treatment and cure for ROHHAD.

All our love to our wonderful friends fighting ROHHAD:

Mason Byrom



Joshua Wooten


Sophia Adamo

Taylor Brooks Foundation and Vassago Cycles

So we are always busy and we’re constantly doing something. I’m not sure we have “free” time….as some might say. I have 5 sites that I maintain – one being Haven’s. But there are a few things we don’t usually discuss on here – which is our donations to causes. Working with bikes we donate many frames to many races across the USA. We donated a custom pink frame to the American Cancer Society and we also donated a frame to the Taylor Brooks Foundation. I wanted to share with you a bit of the story of The Life Bike.

Taylor Brooks Foundation

On Friday, July 13, 2007, 6 days before her 14th birthday, Taylor was diagnosed with Desmoplastic Small Round Cell Tumor cancer. This is a very rare type of cancer and is usually found in boys so it is very unusual for it to happen to a girl. She had treatments that went 5 days in a row, each day was approximately 6 hours of intense chemotherapy. She did this every 3 weeks for 4 rounds – a total of 20 days of chemotherapy.

Taylor worked to support her treatment hospital through fund raising and patient parties as well as acting a a poster child for a blood drive during her treatment. Sadly Taylor lost her battle with cancer and won a place in heaven just after 3AM on April 1, 2008 while her parents held her with her sister by her side.

While we are unable to donate money to causes, we were able to donate something that raised money for the Taylor Brooks Foundation. In early 2008, we were contacted by Dirty Spokes Productions and asked if we'd donate a Vassago frame for a raffle for childhood cancer. IMMEDIATELY we said YES! Soon after we heard that one of the Vassago bike shops, Addictive Cycles, was also going to donate a Vassago frame. Well yesterday I received an email from Jeff Brooks, Taylor's father. Here it is:

Dear Dirty Spokes, Addictive Cycles and Vassago,

All of you may or may not remember me. My name is Jeff Brooks. I rode in the Conyers event to raise money for the foundation started in my daughter Taylor's honor. All of you were gracious and donated your time, support, money and frames for raffles. The day was a giant success!

The Taylor Brooks Foundation was the beneficiary of approximately $12,000 that day. In the first year of operation because of the help people and organizations like yourself have provided the foundation has;

- delivered new patient diagnosis kits to the AFLAC Children's Cancer centers at Egleston, Scottish and the bone marrow transplant area.
- delivered holiday gift bags to Scottish Rite on Mothers Day, Fathers Day, 4th of July
- Given a Christmas party at all of the facilities above.
- AND COMMITTED to Sponsor a new fellow from Emory University to work in the AFLAC Children's Cancer group for Children's Healthcare of Atlanta.

I found the email below tonight as I was catching up on personal messages. I think you all will be touched. I plan to contact Seth and Linda and help them get started with this incredible project.

I would like first THANK EACH OF YOU for the success and the seed that has been planted. I would also like to ask each of you for your ideas on how we could take this idea of the Owen's national?

WHAT IS THE OWEN'S PLAN?????

see.....below.........