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.

ROHHAD and NEUROBLASTOMA Update on Haven

Currently - I just wanted to update everyone on what we're doing and what we're waiting for.

Through the holiday break we have slowly been decreasing Haven's dose of steroids and will end on January 1st. On January 2nd at 8:00 am we will have blood taken at JH to check Haven's Cortisol levels. I am hoping she is producing Coritsol on her own and we won't need the steroids.

January 13th - Haven will go in for Chemo and meet with her doctor to discuss the future treatment plans. We are waiting to hear if we will go back on Rituximub (chemo) for another 6 months.

January 15th - Meet with Pulmonology at 8:30am to evaluate Haven's breathing - really I don't know what they do. This will be the first time we've seen Pulmonology since our initial 10 day hospital stay in October 2007.

Setting up an appointment with Audiology - WHY? Josh lost his hearing after being diagnosed with ROHHAD. So since we know very little about this syndrome we are getting an initial marker on Haven's hearing and will be able to watch if it starts to decline.

Still waiting from Haven's eye doctor - I've called 3 times and haven't heard back. I'll call again after the holidays. Now that she is going to be getting more chemo I'm not sure if it's the right time for surgery. Haven's doctors will have to tell me on Friday.

Christmas photos are coming! I have so many projects in the works right now I barely have time to do anything!

OH - had some CRAZY doctor dreams over the break. I remember one very clearly.
-- Haven was in a lab room (all white of course) with lots of doctors. They frantically tell me that Haven now has Hypoxia. I was upset (although when I woke up I didn't know why) and I kept asking them how do they know. They kept telling me "it's on the list". I was crazily searching for the list and they had it on the white board and were check marking Haven's symptoms - just going down the list. They kept telling me to look at the list over and over. --

I woke up and searched for my list. Hypoxia is NOT on the list. I was't even sure if it was a word or medical term. BUT it is!

HYPOXIA: is a pathological condition in which the body as a whole (generalized hypoxia) or region of the body (tissue hypoxia) is deprived of adequate oxygen supply.

Haven does NOT have Hypoxia! Whew! :)

ROHHAD and NEUROBLASTOMA CANCER

So we spent pretty much all of yesterday at Johns Hopkins. We had a great visit with both of Haven's Oncologists. We had a lot of questions and we received a lot of answers. I will have to post an update probably next week sometime when we have a more clear picture of the immediate future for Haven. Here is what I do know:

1. She is very tricky and very complex - ROHHAD and Cancer mix
2. Chicago doesn't have a "treatment" or really a test as no one really knows about this syndrome
3. Her Oncologists have a plan of attack however, they will meet with the conference again next Tuesday to confirm this is the way they want to go and then will get our approval. Once we approve I will let everyone know what they are doing.
4. We are still waiting on the sleep study results!!!!!! It's now Day 10!

Something we are doing right now - which we hope will show us a good sign. Haven will be off the steroids by January 1st. We are weening her off starting yesterday. I will take her in to the hospital on the 2nd for an 8:00 am Cortisol test. If her body starts making Cortisol on it's own we will be HAPPY - if it doesn't then we will go back on steroids.

I want to say one thing that made me VERY happy. Yesterday we met with the social worker at the hospital as well to review anything that we need and to discuss assistance with Haven. As we all know - unless you live with a ROHHAD child you really don't understand what the heck we're talking about half the time! :) Not a bad thing it's just the way it is. The SW, Lisa, came in to the room AFTER reading about Haven and studying ROHHAD. She even read Haven's blog. I was very happy about that. It seems like most people come in and don't check on her before hand and ask questions that we really feel they should know already. THANK YOU LISA!!!

Oh - Thank you to the Ped. Oncology and Nurse Katie for Haven's Christmas present! Of course, anything about the Princesses is a favorite!

Really sick....

Well from the last round of chemo Haven was super sick for 2 weeks straight. Throwing up and fevers....severe infections in three of her toes and a very bad bone thrush. This week chemo was no better.

After 3 weeks she still has infections in 3 of her toes. We bought the foot bath, took the Amoxicillin, and cleaned every night with peroxide. Still about every other day she would have green puss coming out from her toe nails. Normally a hangnail wouldn't bother anyone...but someone without an immune system it's a major issue.

Both of her big toes have been swollen beyond belief. I bought her new open toed shoes (which I am not a fan of at all) so they wouldn't squish down on her toes. I bought her some new cute socks but after a day of wearing them she was in so much pain we've gone back to bare feet.

She now has new medicine for the infections and this medicine must really be bad. We have a hard time getting her to keep it down. She almost throws up every time she takes it - and that's 3 times a day. It's a powder we have to mix with applesauce. Well that made her not want to eat applesauce again. So we tried pudding....nope - didn't work. Now she and her dad have come up with a crazy mix for the medicine:

Strawberry Jelly
Lemon juice
Sweetner
Medicine

This is a little better...she gets the chills really bad when taking it and the gag reflex kicks in. So I have Crystal Light ready to wash it down and then another snack. There is also a cream we have to put on all 3 toes and then we bandage them up for the night.

The past 2 nights since chemo have been really bad. I know she will wake me up at 2am to potty and then again at 3am soaking wet from breaking a fever. The past 2 nights she has been completely soaked and her hair dripping as if from the shower. I get her changed, change the sheets and pillow and dry her off. Then that can usually get her sleeping until about 5:30am when she wakes me again.

We were able to clear up the blisters with her medicine. That was one good thing that has happened.

She has still not grown any - still only 39 inches - same as last November. But they said she will not grow until we are done with chemo.

Her sleep walking has started up again and I sometimes catch her running through the halls and bedrooms. She has no idea where she is and is just spaced out.

We're doing as best as we can with her and she gets a lot of special treatment....Lauren is here twice a week and that is the biggest treat for her. She absolutely loves her friend coming over and gets all dressed up when she sees her.

I have a few pictures to post tomorrow. Love to all - M