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 -----

Doctors....not all bad

About 75% of Haven's life has been in doctors offices and hospitals. Over the years we've been through a lot of doctors - some bad, some good, and some that are truly lifesavers. I get upset with doctors and when we first came to MD I was every doctors enemy. I didn't trust any of them even if I had just met them.

CA was horrible. We spent 2 years wasted on doctors and hospitals. Constant blaming of us for our daughter's issues and constant dismissal of symptoms. This made me hold a grudge against ALL doctors.

Moving to MD was probably the best decision I made to save my baby. We've lived at Hopkins for over 2 years now and we've made it through some rough patches with doctors and diagnosis. I've had many arguments and disagreements with our doctors in the beginning and it was hard to take what they were telling me.

But now I can't tell you how much I respect our doctors and how much gratitude I have for them. I can honestly say without these doctors I probably wouldn't have my baby with me. I probably would've lost her and never knew why.

There are major players in Haven's life who have kept her alive and fighting and have helped me along the way.

Dr. Cooke - Endo - he has been there from day 1 when Haven was rushed to Hopkins from Upper Chesapeake. He didn't give up and tested her for everything. He listened to what we had dealt with prior in CA and he broke it all down. He found her Ganglioneuroblastoma Tumor and ultimately diagnosed her ROHHAD by putting all the pieces together.

Dr. Paz-Priel - Oncology - he's been with us since December 2007 when we were first informed that Haven had cancer and a large tumor in her abdomen. He has been the best doctor I could ever hope for. He is always there when I need someone to listen to some wacked out theory I have or to listen to all the notes I take on Haven. He watches the videos I send him. If it weren't for his aggressive attack on her cancer and ROHHAD I wouldn't be here showing the world my baby and her improvements. He has never given up on Haven and has fought through many doubts on her treatments. Seriously I could never thank him enough.

Dr. Price - Surgeon - well....without him and his knowledge of these tumors and surgical skills I could've lost my baby in February 2008 when she underwent a 14 hour surgery. He has been there when I need him and for anything I have questions about. He is the only surgeon I would ever let dig deep in to my baby. He watches for Haven and when we see him at the hospital he always stops to say hi to her. Amazing!

Dr. Cooper - Pediatrician - she has been there for more than just Haven. She has been there to help us out as parents. She has collected articles written by other doctors for me that I can't get because I'm not a medical professional. She has helped us get Haven the appointments that are difficult with specialists. She's even helped me get in to doctors!

There are people that will never see Haven again that I'm thankful for their honesty - Upper Chesapeake. They stabilized Haven during her seizure and did a CT scan...ultimately came out and were honest and said "We can't help you - we're calling Hopkins". I am so happy they didn't just try to guess and openly admitted they didn't know.

It took me a long time to trust in a doctor with my baby and now I can't imagine not having them in her life and watching over her. I am very happy they put up with my cockamamie theories and my many emails.....most at that level wouldn't even pay attention. And believe me, I email them a lot!

Sleep study and MRI

We are ready!

Spoke with Pat at Mt. Washington Sleep Lab and we're all set to go. They have Haven's room ready so we'll be there by 8pm Saturday evening. Pat said she'd missed us because it's been 6 months - I had to reschedule this appt a few times.

Confirmed with MRI today for Haven's appointment on Monday. We have to be there at 7am. They have promised that it IS in her files to use her PORT ONLY for the IV. I better not see a bunch of needle holes and cables wrapped around a leg or arm when she comes out. I already know they will intubate her during the procedure. This is going to be a 4 1/2 hour MRI which is too long for her to be under anesthesia without assistance. So I have promised them that I will not argue about it as long as they don't threaten to automatically keep her overnight!

And they have Haven listed as a "Frequent Flyer" in her records at MRI. So I'm wondering if after so many hours does she get a free MRI????

We also have an Endocrine appointment this month on March 22nd. I'm excited to get in there to talk with him. Oncology never talks to us about Haven's growth hormone or prolactin or anything like that because it's not their deal - it's Endo. Plus I want to discuss my many random theories. Plus my MRI request was for the head and particularly the pituitary area so he'll be able to discuss that with us.

After that it's another trip to Pulmonary and our normal Oncology visit! I have to set Pulmonary up after the sleep study.

Endocrine

We met with Endocrine on Monday. I was concerned about this meeting - this would've been one to cause arguments. But instead we received happy news!!!!

Haven is growing - however slow - but she is growing. She is in the 5th percentile right now and showing signs of continued growth. The doctor was pretty happy with that. I was concerned we would have to start giving her growth hormone shots as this is what was said in May prior to Hi CY. But since then she has grown almost an inch!

He also stated with her growing and growth hormone levels at least not below normal she did not have to do the cortisol testing right now. He would put a request in to Oncology to have both growth hormones checked in December during labs.

So we are very happy and excited we do not have to give her a shot 6 days a week! :)

Endocrine Meeting

Well we had a very good meeting with Endocrine today. It was the best meeting we've had with Endocrine. We had quite a few areas of discussion today. Our next appointment will be in 4 months instead of 6 months.

Beginning with Haven's blood pressure being slightly on the rise and today at the doctor her blood pressure was 125/67 and a heart rate of 125. Which the heart rate I'm used to but I noticed last night her blood pressure was 124/63. Which is pretty high. We discussed with Endo and it's possible that her blood pressure medication may need to be reevaluated and increased. We will discuss with Oncology on our next visit. Cyclosporin can also raise the blood pressure.

Next up was Haven's growth. While I have been happy that between January and April she has grown from 39 1/2 inches to 41 1/2 inches it seems as though this is not enough. Haven is around the 5th percentile for height. We had a pretty long discussion as I really do not want to have to put her on growth hormone shots. Our friend Josh is on them and I believe he gets 6 shots a week....I think Haven would beat me up if I tried to do that! :) The doctor said he would not put her on this yet but he said he had to see her grow a lot more in the next 4 months than she has in the last 6 months. If she does not grow a lot over the next 4 months she will have her IGF1 retested and probably begin the shots. Haven's current IGF1 level is 55 and normal is between 57 - 260.

Haven has her Thyroid checked about every few months and as of right now she is looking good. Actually I had to have some blood work done last week and her Thyroid is better than mine! So this is great news. Kris has been concerned with Haven's sodium levels - which over the past 3 months of lab work she has remained in the healthy zone - not high like she was before we started back on chemo.

NORMAL PROLACTIN: LEVEL 0 - 18

The above chart is something I made with the info received today about Haven's Prolactin level. In the early months of 2008 we didn't really monitor this very close. In October I began a lot of research and was extremely concerned about the Prolactin level. The doctors weren't really monitoring it because nothing has ever really been noted about Prolactin in ROHHAD children. I was very emotional about checking this constantly that the doctors have now started to look closely at it. The graph above shows the dates Haven's Prolactin was checked and the level it was at. This shows us that the Hypothalamus is not communicating with the Pituitary.

We discussed the time in the graph where Haven was at her lowest on Prolactin level. This was the best Haven has ever been in 3 years. She completely stopped wearing diapers, she was refusing food and never asked for it, she was sleeping soundly, never sleep walking or talking, behavior was awesome. This was when Haven was on Rituximab and Cytoxan. So as I have been questioning over the past few weeks in my head with the minimal progress we've made on Rituximab. Was it the Rituximab? Was it the Cytoxan? Was it the combination of both at the same time? I believe this is a hint to what we will find out in 4 more months from Oncology. I won't make a guess here but I have a pretty good feeling I'm right.

Cortisol - Cushing's testing -- We discussed retesting Haven for Cushing's Syndrome with the salivary test. Her last Cortisol reading was done in January when she was taken of Prednisone and it was at a good level. The doctor pretty much left this decision up to me and what I wanted to do. I talked to Kris and then told the doctor that I felt I would like to wait until our meeting in 4 months to do the test. High cortisol will stop you from growing. Since Haven has grown this may be more that she doesn't need to deal with right now. He agreed. So in 4 months if Haven has not grown almost twice as much as she has in the past few months we will test the Cortisol.

At most our Endo doctor will only do the salivary testing for Cushing's. If we find that her levels are not normal he will refer us to a doctor at NIH for further testing. He is not familiar with all the types of Cushing's and the doctors down there specialize in this. Knowing Susannah also went to NIH makes me feel more comfortable with going there to be tested. The reason we can not do the pee test on Haven is because she is still in full-time diapers. She would have to be admitted to the hospital for a week on a catheter to get all of her urine. I do not want her to have to do this if it's not necessary. So in September we will move forward with this.


I feel really great about our meeting and the decisions we've come to together. Kris said he felt very comfortable with it all. We continue with our current list of medications, checking her blood pressure twice a day, and monitoring her on the pulse ox machine at night.

Thank you to everyone for all your help.

Cardiology update

I spoke with our Cardiologist today. A few notes from him:

• She has premature beats in the upper chambers - slight arrhythmia - not concerned with this at this time
  
• Haven's heart beat is a constant fast beat and he was unable to distinguish between her being awake and being asleep
• Around 9:30pm and later in the evening she had gone up to 150 bpm while sleeping
• Around 2pm and 6pm he said she was down to 90 bpm while awake

He will request an annual thoracic echo be done.

Due to her heart not having a real pattern and to continually beat high while sleeping but having variations he has concluded that her problem is more Neurological than Cardiac. He is not concerned of her going in to cardiac arrest or any issues like that. He is recommending Neurology and Pulmonary monitor her closely and that we try to fix her sleep patterns.

We will meet with her Oncologist this Friday and review his notes and discuss where to go next. I am happy she is monitored so close by everyone. We'll definitely see something bad before it happens.

Haven currently sees:

• Oncology
• Pulmonary
• Endocrine
• Cardiology
• Orthopedics

And thanks to Haven's Pediatrician - we are able to keep track of everything and get all of the doctor reports we need every 6 weeks.

She has MRIs to monitor her tumor every 6 months and will continue with Cyclosporin and Rituximab for the next year.

Next appointment is Endocrine on May 4th - we will discuss having her checked again for Cushing's Syndrome and checking her growth and need of growth hormone shots.

Love to all

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.

Letter about ROHHAD

I'm sure many of you don't read the comments written by others to Haven's site. But I found this one from Vicki - Leigh Ann's mother - very important. I would like to share this with everyone.

Misty,

It is good that you have doctors at John Hopkins who are concerned for Haven. A doctor who listens and believes the parents about the symptoms of their ROHHAD child is very important.

The first pediatrician for Leigh Ann would not listen or believe me. I quit him after Leigh Ann returned home in Oct. 1992 from the children's hospital in KY. In Nov. 1992 I changed to an Evansville, IN pediatricain who was located 8 miles from our home. This pediatrician was open-minded and he would listen and believe me.The Evansville, IN pediatricain is the one who diagnosed Leigh Ann with LO-CHS/HD now called ROHHAD in Nov. 1993. He then sent her by air life-flight back to the children's hospital in IN. This children's hospital was 200 miles from our home.

Leigh Ann had been a patient in this hospital several times since Nov. 1992. Also we had made several trips for clinic visits at this children's hospital. But they had failed to diagnose LO-CHS/HD. The Evansville doctor had a very difficult time trying to convince the doctors at the children's hospital that Leigh Ann had LO-CHS/HD.

In Feb. 1994 the doctors wanted to transfer Leign Ann to a rehab hospital that used their doctors. But due to the problems we had experienced at this hospital, I had Leigh Ann transferred to a neurological rehab hospital in Michigan. There were good doctors at the rehab in Michigan who had a better understanding about LO-CHS/HD. Because of the problems with the children's hospital in IN, we started taking Leigh Ann to Vanderbilt in Nashville, TN. The local pediatrician in Evansville, IN asked the help of the Vanderbilt endocrinologist to explain LO-CHS/HD and to assist the other Vanderbilt doctors with understanding LO-CHS/HD.

The Vanderbilt endocrinologist had started Leigh Ann on growth hormone shots just prior to her death. She was treating Leigh Ann for her SIADH problem as well as other endocrine problems related to what is now known as ROHHAD.

All ROHHAD patients need caring doctors. An over-confident doctor who thinks ROHHAD symptoms is only a problem of too many calories can do more damage than good for the child.

There is still more knowledge that needs to be learned about ROHHAD and its treatment. However, it seems to me that at least Haven's doctors are trying and care about her.

Love & Prayers, Vicki