Finding Oncology, Pulmonary, and Endocrine!!!

Tomorrow we will be meeting one of Haven's doctors and having a full review of everything "Haven".  I have copies of all of her latest work and labs for this doctor. 

We will be moving asap towards another Oncologist and setting up for the year of chemo treatments - will be nice to have them on the calendar instead of figuring out when the next one is.  We have at least one or two more surgeries coming up.  If one of the surgeries is soon I will push to have it after school is out in June.  She is doing amazing at this school and to her it would be a punishment if she had to be out for a week or two. 

I have been in contact with people about a Pulmonary doctor and getting her next sleep study. I tell you I can definitely give you the results at home!!!  I just bought a mega ton humidifier for her room - it helps a bit but I need a Pulmonary doctor to check her CPAP machine and get her working on it.

Endocrine will be the more difficult doctor - they do not have one for children in St. George.  So we will have to travel to Salt Lake City for this for each appointment. 

The probability of having to go to SLC for Oncology, Pulmonary, and Endocrine is pretty high and the hospital we would be going to is: Primary Childrens Hospital.  Which is the children's hospital for Utah, Idaho, Wyoming, Nevada, and Montana.

We have a few other ROHHAD patients attending this hospital and one is undergoing part of Haven's treatments for ROHHAD. 

I will be happy to get with an Oncologist regarding her IVIG and hope Haven can fully recover in the next year or so. 

I will know more tomorrow and hopefully be able to give a clear update on Haven...right now we've just been doing little bits.  I will get with the other parents using SLC and find out who their doctors are.  Maybe even plan a visit with these other children!!!

Until I know more........................

Vet, Pulmonary, Therapist, Oncology, Endocrine and Child Life

Well I definitely made great use of my day off work yesterday. Only one of the above in the title did not belong to Haven! :) Deezy had to be spayed yesterday. She is recovering well and Haven has been hand feeding her...SPOILED!

After taking Deezy to the vet at 7:30am we headed to the hospital. We started our day with Pulmonary and reviewing Haven's latest sleep study and setting up our follow-up appts.

PULMONARY: sleep study - my main concern is Haven's CO2 levels

• February 2009 - CO2 level at 49 - was told that jumping to 50 would take a lot and they didn't expect it.
• July - September 2009 - Hi CY treatments and Isolation
  
• August 2009 - CO2 level at 50 - doctor said he will start being concerned at 55 and she will be watched closely.
• October 2009 - HD Dex and IVIG
  
• March 2010 - CO2 level DROPS to 45 - doctor extremely happy and not expected. 35 - 45 is normal!

Pulmonary agrees and is doing more research on sleep instability linked to respiratory syndromes. Possible theory is that her sleep walking kicks in when she has a good length of pausing in her breathing. This could be keeping her levels at a normal level and may be why we are seeing an increase in sleep walking coinciding with a lower CO2. Who knows....but it's a good theory. I honestly don't mind sleep walking because I know she's breathing - I can hear her yelling!

THERAPIST: Honestly this may be good for some but I'm just not sure it fits in with us right now. Haven has no anxiety about doctors, needles, procedures. She's been doing this since age 2. Maybe it would've been better in the beginning but I'm not sure where it will help her now. The only issue Haven has is leaving the house but once she's out she's fine. I don't think trying to treat her like a "normal kid" at this point will be beneficial....the girl is already too adultlike.

ONCOLOGY: Received a call from them regarding Haven's MRI.

• Brain: clear and perfect --- as with all of her Brain MRIs - no tumors and anything out of place
• Abdomen: tumor still present but has not changed shape or size. Very stable and will be monitored as usual.
• Pelvis: perfect nothing out of the ordinary and nothing new showing up

IVIG is coming up and another round of immune testing. We are currently working on getting Haven in to school. There are a lot of factors here.

ENDOCRINE: This was our 6 month follow-up visit and through emails we had discussed other tests I wanted to run.

• May 2009 - Haven had no growth. She hadn't grown in 1 1/2 years. She was a negative on the growth scale and was about 38 inches tall.
• July - Sept 2009 - HI CY treatments
• September 2009 - Haven shows growth potential - grew to 39 1/2 inches tall - no growth hormone shots and testing again in December
• October 2009 - HD Dex and IVIG
• December 2009 - Growth hormone tested - show an increase
• March 2010 - Haven has GROWN now to a solid 43 inches tall!!!!

I had discussed doing Cushing's testing again - after talking with the doctor - Haven has shown tremendous growth and this isn't usually the case in a Cushing's child. She also didn't have the red striae noted on Cushing's patients. Doctor again said IF I really want to do the testing he will order the salivary cortisol testing and if I really wanted he would do the other tests but it requires a lot from Haven and there really isn't any evidence leaning towards Cushings. So I have decided not to test and will review again in 6 months.

Prolactin - seeing the brain MRI showing no tumor present at the Pituitary tells him it just confirms the mis-communication between hypothalamus and pituitary. He will be concerned on this hormone around age 9. Until then they will keep monitoring.

Leptin/Adiponectin - As we can guess - Haven will have a high Leptin level - she's obese. But even is she is Adiponectin deficient there is nothing that can be done at this time. To run the testing would just be for the books and nothing done with it. So we will let this go for a while.

Brain Stem - I wanted to discuss this but Endo doesn't work with the brain stem and was unable to answer my questions. He is consulting with a Neurologist for me and possibly will set up a meeting with Neuro at some point. But as he said - if there is nothing Neuro can do about it and can't fix it then it's probably not beneficial to have them on the appt list when they can just review her results in the system.

CARDIOLOGY: Happy to report!!!!

• Before all of our treatments Haven had been on BP medication for 2 years --- she was taken off in September 2009 --- BP has stayed around 109/65 and no medication!!!!!!!
• Before all of our treatments Haven's resting heart rate range was 140 - 165 ---- she is now consistently staying between 80 - 90!

CHILD LIFE: We're working on getting Haven in to school a few hours a day and with her currently home school teacher joining her. This will help her slowly get in to the rhythm of school and not overwhelm her. There is a lot going on here first before we finalize everything.


As you can see I am very happy! I'm very proud of Haven for kicking butt the last 4 years and I'm very thankful to our doctors for fighting to do these treatments for her and now we're actually seeing some results! I'm looking forward to our 1 year mark and hopefully a full immune system! :)

Love to all -----

Meeting with Dr. Cooper

Today I had an afternoon meeting with Dr. Cooper, Haven's Pediatrician. We meet about every 6 weeks to review Haven, where she is and was. We go over any notes that I have and get them in order for the other doctors Haven visits.

We reviewed the notes from Cardiology and Pulmonary and what the doctors had written in their reports. I got my copies for my files. She said I could probably copy their upper notes to take with me to any new doctors so we do not have to go through it all over and over again! Good idea.

We're still waiting for anything from Cardiology. I mailed the halter back to them on Monday so I'm not sure when they would've received it.

We have a meeting with Endo on May 4th so we reviewed some medical info from Susannah's mom and thought it would be a good idea to mention to our Endo. Susannah just recovered from surgery for Cushing's Syndrome. Which Haven has been tested for a few times. She copied me on an email she sent to my favorite mom in Europe and a few areas caught my attention!

We are doing well on the Pulse Ox machine. We know how to use it and I am confident in the results now - after talking with Pulmonary earlier this week. I keep a detailed log of her stats throughout each night.

I discussed Chicago with Dr. Cooper today and it really helped to talk to her as she has no bias to go or not go. Kris and I discussed it tonight and I believe we have come to a decision. Kris will discuss it with Oncology tomorrow and then we will confirm what we are going to do. We have been thinking hard on this decision and I have been working with our case manager.

I found some of Haven's old records yesterday while digging through boxes. Some reason I feel like I already said this but don't remember. Anyway, we have her blood records from July 2006 and two of her MRI reports from 2007. So I will scan that in and email to all of her doctors.

Until the next doctor appointment tomorrow - that is today's update!

Love to all
M

Exhausted

Well....I am exhausted. Friday was a long night with checking Haven's stats on the O2 monitor. Then last night I did a check at 9pm, 10pm, 11pm, 12am, 1am and then finally fell asleep to have some crazy weird dreams. Woke up at 3am and checked her readings again. Heart rate was around 110 and oxygen at 94. Then Haven got me up at 4:30am because the alarm was going off on the machine. At that point I realized I never heard the alarm which is a good test and means if there is an emergency I won't hear the alarm.

So we're gonna have to work on that tonight. I believe it alarmed because I'm trying to spare the use of the bands. We only got 5 with the machine so I have to find out how I'm supposed to get more. I really wish someone would've explained something to us about the machine and who we call for supplies.

This week should be a calm week. Haven will be in Oncology on Friday. We'll be discussing Chicago and our thoughts and feelings regarding that. We're still discussing how we feel about it and are still not sure what we want to do. There is a lot of work to do with insurance. I spoke with our case worker on Friday and there are a lot of hoops to jump through to find out if it's even a consideration. That will be discovered throughout the week.

We hope everyone had a wonderful weekend. Love to all.

Pulmonary, Oncology, Cardiology - Oh My!

Well we made it through. The past two days have been filled with lots of doctors and lots of talking!

PULMONARY:

We were very happy with this appointment. I really like our doctor. He listened to everything, explained the difference between obstructive sleep apnea vs. central hypoventilation and how they see it in a sleep study, he had talked to Oncology before meeting with us so he already knew about Haven and pulled up her records while we were in there. It was great. We spent over an hour with him.

A few areas discussed:

Haven's breathing and where she is right now: he noted that her sleep studies were pretty good right now and that we would continue to monitor her closely for any changes. He said her last sleep study showed her highest CO2 level at 49 and when she starts to pass 50 is where the concern would be. We have a sleep study scheduled for August 15th and a follow up appointment with him on July 23rd.

He did say he didn't expect Haven would suddenly stop breathing in the next month or even the next 6 months but then reminded us that he can never say that for certainty because we don't know how ROHHAD acts in every child. At most, he said she would probably only need to go on a bi-pap machine in the next 6 months but didn't think we should worry about that yet.

He mentioned bringing Neurology in now to monitor possible seizure activity during her next sleep study. Doesn't mean she is going to have a seizure but Haven has a history and since this hasn't been monitored in over a year he would like another record of her brain in that department.

He is a bit concerned with her sleep walking and talking. More for her safety than anything plus we were sure to explain to him that although her sleep walking drives us crazy and doesn't let us sleep - it also comforts us because if she's screaming and talking we know she's breathing.

He ordered a pulse oximeter for Haven to use at night while sleeping. They said it would be set to alarm if she reaches 94....I am sure we will hear many alarms as Haven is usually around 95-96 and a little drop will cause an alarm. Kris received a call tonight about the delivery! I did briefly discuss her sleep walking and machines but he was not worried. We will have to spot check her stats - we'll have different times at night that we will have to record them.

We discussed her ABG results that were done while under anesthesia. He is almost discounting them because the readings would be altered by the intubation and oxygen given to her. So we will have to have an ABG test while awake. We did not do this today - as he wanted Haven to meet him first without pokes and it hurts a lot.

He is highly recommending us to go to Chicago to meet with Dr. Weese-Mayer. He said he has talked to her and discussed Haven. Oncology has also talked with her about Haven. He is concerned that there could be something they are missing and wants to have a full evaluation done by Dr. Weese-Mayer and then get the results back here. He also would like to have Haven's full studies in the research paperwork to help other children with ROHHAD. I have emailed the team at RUSH (thank you Julie for all the paperwork). We'll discuss this with Oncology next week.

We discussed Haven's allergy to Ketamine. He absolutely doesn't believe it's specifically Ketamine related. He told us that a ROHHAD episode (Respiratory Failure) can easily be triggered by ANY anesthesia. Haven must be required to be intubated without question at any procedure done. He told us to make sure when she does have anesthesia that we require them to monitor her CO2 levels while giving her oxygen. He said CO2 levels can rise dangerously if not monitored at the same time in a ROHHAD child. We have agreed to have her intubated on every anesthesia procedure due to her seizure activities and respiratory failure a few times while under anesthesia.

The funniest thing we heard: he said he bets that Haven can stay under water for a couple of minutes and have no problem at all. She lacks the function of panic that would tell her she needs to get to the surface to breathe. She can withstand a high level of CO2 - which a normal person can not and they would rush to the surface. We didn't take him up on the bet!


ONCOLOGY:

We went to Oncology following Pulmonary. Haven needed to have her levels and blood work done. When we met with Cardiology yesterday he wanted to have her tested for Anemia. So this was done today as well. We will not have results back until next week. Everything went as planned here.

Child Life talked to us about Sunrise Camp for Kids with Cancer. So we are going to sign Haven up in the 5 year old camp. It's a daily camp (she doesn't stay over). It's only for kids with Cancer. There are Pediatric Oncologists and Oncology nurses on site. Our own Nurse Katie volunteers at the camp and Kim at Child Life is there. We think it will be really good for Haven. It's free for her to go and she will have a blast doing crafts. This will be in August.

CARDIOLOGY

Next we went over to Pediatric Cardiology to get Haven hooked up on her holter. We are monitoring her heart rate. She runs high so the doctor said he would like to see her have fluctuations and get down in to the 80's during sleep. We will get the results from him next week. He doesn't want to put her on medication because she is already on so much. He wants to have an Echocardiogram done every 4 - 6 months to monitor her.


Here is Miss Haven with the heart monitor on. She was happy it was in a little purse. We were worried it was going to be too big but they said this is the size everyone gets. The monitor is in the little bag and it hangs around her neck. She has to sleep and everything with it on.

Here are a few of the leads. These are on her upper chest. There are more that are up on the side of her neck and all on her belly area. She is doing pretty well but she keeps saying the tape is pulling. Luckily we don't have a hairy chest to worry about. She is definitely concerned about taking all of them off.

She is always concerned about someone putting something sticky on her port and messing it up. So she always tells nurses and doctors not to touch her port and to make sure stickies don't get on it. Luckily - we didn't have to have any there.

Well, this is long but it's been a long day. I'm going on my 4th day straight of a constant migraine. I hope Haven sleeps tonight. And I'm super excited it's Friday tomorrow and NO DOCTORS!!!!

Love to all!

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

Chemo Plan

Friday Kris and I met with Haven's Oncologist and discussed the Neuroblastoma Cancer and that they will keep a watch on the tumor and monitor that. We also discussed ROHHAD and Haven's current condition.

We have seen some positive results over the past 2 weeks with the Rituximab. She has been going potty by herself and she has been sleeping....or at least she did for a week. Since Friday's dose of Rituxan she has not slept. I really hope this evening is much better.

We also learned that she will be on her current chemo plan for about the next year. They will reevaluate in 6 months and continue on for another 6 months after that. So we are looking at hopefully going off the chemo this time next year.

Haven's Oncologist also gave me a bit of homework - which I haven't started yet. There are a few questions we need to ask some of the other moms to maybe come up with another theory and may require more testing for Haven.

As for now Haven remains under the care of Oncology (as she will for a long time), Pulmonary, Endocrine, and her Pediatrician.

Medical Updates

Haven has Rituxan tomorrow - her full day of chemo at the hospital - and it has been requested by her Oncologists that I join Kris and Haven at this meeting to review everything. I do not normally go to the chemo appointments but since her doctors are urging me to be there I will join them.

(PS: Hi Oncology Doctors - they told me they read Haven's blog so this will be a test!)


What I know:

Haven will remain on Cyclosporin twice a day for a minimum of 6 months
Haven will remain on Rituxan for a minimum of 6 months

They will not alter this schedule or make any changes. I have been and will continue to keep a daily log of Haven and monitor her for any changes - positive or negative. At the end of her 6 months her doctors will discuss her files with the medical board for further review.

Haven is still on schedule for surgery on March 26th. Haven is at a high risk on this surgery due to the ROHHAD and the chemo. The biggest concern for her doctors is her being in anesthesia. The risk of her stopping breathing is significantly higher due to the ROHHAD. We have already been called about Haven staying over for observation - but I have requested that this only be done on an as needed basis and not automatically.

I also spoke with Haven's nurse today - the ABG (Arterial Blood Gas) will also be performed during surgery. She has to be under anesthesia to have this done so we had to make sure it happened at the same time as the other surgery. Haven's ABG will be done through her wrist. I am told she will have more pain from this than the actual surgery.

On March 27th - Haven will be back at the hospital for her full day of Rituxan.

April 9th - We will meet with Haven's Pulmonary doctor - Dr. McGinley. He will begin to handle Haven's case from this point forward - re: respiratory functions. He will review her ABG results with us and begin to keep a constant monitored file for Haven.

Early May - We will meet with Haven's Endocrine doctor Dr. Cooke - for her 6 month check up. We will look at her growth and talk about her immediate future where he is concerned. He has been working close with Haven's Oncology team and the Orthopedic doctor.