IVIG received - much needed

 On Tuesday we headed out to our other new hospital - Huntsman Cancer Institute in St. George.  This is where Haven will get her port flushed every 4 weeks and then every 6 weeks she will get her labs drawn and then every 8 weeks she will get IVIG.  So, we actually get a one month break and then we're in ever two weeks for a month....then another month break.  Then every few months she will head to SLC Primary Children's for a full run down of labs and check ups.

 Her room was very nice at this hospital.  It's bigger than my labor and delivery room and she had a private bathroom.  I was very surprised and happy.  She was very well taken care of.

 Here is Haven hanging out with the Child Life specialist.  He was great and they talked about everything.

 Haven's first time having a male nurse.  Jason was great...he was very willing to let Haven do her own flushing and everything.  Accessing the port was easy and she did very well.  She of course made sure he did everything the way her other nurse did it.

 Nap time.  I let her stay up watching Happy Feet Two the night before so she'd be nice and tired.  She fell asleep about 20 minutes in to the infusion of the IVIG.

 Oh....5 hours later we are done with the IVIG...now to do a flush of saline...wait 30 minutes...flush again and close up with Heparin.

 

 If you've worked with Haven you know she wants to do her own flushing, blood draws, and Heparin.  I was happy that Jason would let her since he'd never worked with her before.

She did extremely well.....at the end when the took the needle out they hit a small vein which caused blood to pour out everywhere.  When I say pour out it means I thought I was going to pass out seeing so much blood.  Haven, of course, was amazing.  She grabbed napkins and put pressure on it and just asked everyone to get her stuff to help.  Her nurse Jason was already on it...mom did not do so well and was not overjoyed at seeing so much blood come out of her child's chest.

My Haven is amazing...she constantly blows our minds and she is a true rockstar.  Love you Roo!!!!

Primary Children's Oncology visit

 Headed to SLC yesterday with the girls for Haven's appointment.  It's about a 600 mile round trip drive!!!

 During the drive Haven decided to take a nap and Rohnin decided to take a few bites in to Haven's orange.

 The view from the parking lot was breathtaking - so beautiful!

 Haven getting her weight and vitals done.

 Blood pressure was great.

 Working with her new nurse Tiffany.  She was showing Haven a new way to numb the port.  They have a cold spray that works instantly.....ultimately Haven decided to go with the cream and not try something new since it was her first time there.

 Prepping to access the port.  Haven was not happy that the needle was different looking but it was the same - 1" needle.

 Hoping the port still works....we flushed

 and flushed!  They have a medicine to unclog the port if needed....but after a few flushes...

 We were in business....what does that mean!?  NO SURGERY!  Yay.....Haven did amazing as usual.  We had lots of blood taken.  Some of the labs we checked were her IGG levels, prolactin, sodium, IGF-1, and CBC.  They of course checked other oncology labs but that's what I can remember. 

Her doctor was great...we really liked her.  She listened to both Haven and I. She had also read up on ROHHAD and knew about Haven.  She had the latest info from Hopkins on Haven that we had sent up to her. 

We did not receive chemo yesterday as we needed to get all of her labs done first.  I am to receive a phone call today with the results and she will have her chemo on Friday this week at Dixie Regional Hospital.  We got a prescription for numbing cream and for more DDAVP. 

 Haven wanted to do a few photos from the campus.  This is front of the Painted Horse.

 Haven the Gnome

 Being the bird

She is just too cute!!!!  She did amazing.....we are very proud of her.  Will update again after chemo on Friday!

Spring round of IVIG

After Grandma and Grandpa left we had a day of chemo.

Haven has started to not want to see the needle to go in to her chest. She uses a 1 inch needle and her numbing cream. Since doing her Hi CY she has begun to feel a little bit of pain. Not much but a slight amount....so she prefers to not see it now.

However, she is still able to do her own blood work and to flush her port.

Haven is usually awake for about 1 hour out of the 4 - 5 hours of chemo. She slept quite a bit during this round. We canceled her Physical Therapy this day as this usually wipes her out for 24 hours.

We met with her Oncologist and Haven is doing well. Her counts are up (finally) and instead of going every 4 weeks we have been moved to every 6 weeks for a flush (next visit June 27) and every 12 weeks for IVIG (so sometime in early August).

Haven is not scheduled for any more MRIs!!! Her doctors are very confident about her tumor not growing!

Next up is Kennedy Krieger Institute for Neuro-psychology evaluation and Endocrine.

IVIG needed

Miss Haven was in Oncology on November 15th and did her labs and had a full check up with her doctor and we were told that they wanted to see what her body was doing and that they would like for her to go until January 3rd before receiving her next treatment. Which sounded great to us.

Then the next day Haven started with a horrible cough which I had no idea what to do. She has never been sick and this was her first cough. Believe it or not - everything we have all been through with tumors, cancer, chemo, ROHHAD - I had no idea what to do now. We made her tea every night with honey, I would wake her up around 2am and give her another spoon of honey, and then more tea in the morning. We don't really like to give her medication when we don't have to.

That seemed to help for about 3 days then it started getting worse. So I bought every child's cough medicine they make and 4 different types of cough drops....nothing helped!

Then I got a call from Oncology that Haven's IgG level was too low and she needed to come in for IVIG. Which seemed to make sense to me - she's never been sick and no cough - 14 weeks without IVIG and now she's coughing - so she went in yesterday for her treatment.


I believe she read for about 3 hours - every book she could find. Then the hospital has bingo on the TV every Wednesday. So she played and WON! She decided she wanted the drawing desk and she had to leave before it was delivered so the nurses are holding it for her to pick up.

She still has the blisters on her face from the tape - we use Benadryl lotion every evening for that. Tomorrow will be a week since she had the study - can't believe how long these are staying this time. They usually go away after a couple of days.

Haven was asleep by 6pm last night and off to school this morning. Hopefully without falling asleep in class!

Oncology November 15th Visit

Miss Haven went in to Oncology for her 6 week visit on November 15th. We were told at her last visit on October 4th she would need IVIG. So we made a plan to be there the entire day - this is usually a 6 - 7 hour dose for Haven. However, Haven's nurse Katie (above photo) called and said that Haven's IgG level tested at 500 (normal is between 700 - 1400) and that her doctor wanted to skip IVIG this time and see how her body handles itself and see if it can boost up on it's own. So Miss Haven did her labs and was out within a few hours.

Her next Oncology appointment is in January. To explain a bit of IVIG -

Intravenous immunoglobulin

IVIG is: a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months.

IVIG is used mostly for the following diseases:

• Allogeneic bone marrow transplant
• Chronic lymphocytic leukemia
• Idiopathic thrombocytopenic purpura
• Pediatric HIV
• Primary immunodeficiencies
• Kawasaki disease
• Chronic inflammatory demyelinating polyneuropathy (CIDP). Only the "Gamunex" brand manufactured by Talecris is approved for CIDP (in 2008), under the U.S. Orphan Drug law provisions
• Kidney transplant with a high antibody recipient or with an ABO incompatible donor

However, it is in Phase 3 testing of Alzheimer's Disease but can be used in these and others as well:

• Autism
• C. Diff
• Guillain-Barré syndrome
• Muscular Dystrophy
• Stiff person syndrome
• Opsoclonus Myoclonus

Haven really has never had any side affects from IVIG except she does sleep the entire day it is received - but back to normal the next.

IVIG and realizing normal doesn't mean normal

Miss Haven had IVIG today. She was at the hospital and hooked up by 9am. Some reason this dose really wore her out. She slept most of the visit at the hospital. But she did a great job with her port.

We did talk to the doctor about some of our concerns and although everything is going well with Haven and her treatments have worked amazingly Dad and I are still stuck with this number in our head that one of Haven's doctors threw out there. Age 7 - the number given due to elimination of symptoms that Haven will stop breathing on her own. With only 8 months to go we are more and more nervous. But then we were reminded yesterday - this age was given due to her symptoms at the time and average age of onset of respiratory failure and it was BEFORE she did any treatments. They have hope that this has helped her and she won't go in to respiratory failure. So this helped to remind us to let it go to the back of our heads and let her live life.

We had her set up to skype with her grandpa in Utah but with chemo and homework she passed out. We barely made it through homework with her eyes open.

Something that made me a bit sad and made me realize that our version of "normal" really isn't normal. Haven had her first day of school on Tuesday and although she had fun she wasn't very comfortable with the kids. It will take time. But that evening Haven came to me all excited and saying how she couldn't wait to go to chemo and see all her nurses and doctors and friends. That she missed being at chemo. This is Haven's comfort zone and this is what she knows as normal.

I also made a scrapbook for her school that from our "normal" point of view it's cute and sweet and highlights what she has gone through. But I guess when you are a teacher that is going to present it to a class of kids it's a bit much and not normal. But I think this is dad and my warped sense of normal too.

It is hard for a person that hasn't dealt with any of this to understand a family that has or is going through this and what they consider normal. But I don't think we comprehend what life is supposed to be like when you're normal either. And that's just fine with us.

IVIG and Immune testing

While I'm still waiting for results to come back from Haven's sleep study and MRI I did receive a phone call from her Oncology nurse that her IGG levels have dropped down to low and she will need to come in for a dose of IVIG. I can't get in to what an IGG is....way too complicated but it's the immune system. :)

The IVIG dose usually runs 6 - 7 hours - depending on how her body wants to react to it. Sometimes she's good and can take a faster drip and sometimes she needs to take it slow.

Haven also failed her immune test done in February. It takes at least 6 weeks to get this test back. This is, however, what the doctors expected as she is only at 9 months since Hi CY.

We will be at Hopkins all day Monday. We will first visit with her therapist at 11am and then at 1:30pm head to Endocrine for the 6 month follow up. I have a few questions for him and want to review some theories so it should be a good visit.

I figure if I don't get results from him on Monday for both tests then I'll start calling around again. Even if a test is good I think some one should call and say so.

IVIG

Miss Haven had a full day of IVIG today. I heard she did excellent with her port again. Since she started feeling pain she has been more nervous about her port. She still doesn't feel "full" pain but definitely feels a little now. Still we have not seen her cry and has never had tears. But we can tell when she is sad.

After IVIG Haven is usually exhausted. She had a bit of energy when she got home but it didn't last. We started to watch the movie UP and by 6pm she was nodding off. I was able to keep her awake until about 7pm when she was finally allowed to crawl in to bed. If I put her in too early she'll have me up at 5am. She has been peacefully sleeping for a few hours now.

After IVIG she is always really cold with chills. I was laying on the couch and looked over and she had wrapped herself up! So cute. She will be pretty tired until Sunday....this is how she always is after IVIG. Luckily, she will be back to her normal self for Turkey Day! :)