Cpap Class!

Luckily I had Monday as a holiday because the Peds at Home crew showed up to demonstrate how to use Haven's Cpap machine.

Since Haven's treatments with chemo she has done so remarkable that we are starting to have a "normal" life...whatever that means! :) But she is only regularly scheduled for hospital visits for IVIG every 6 weeks and then has her Endo and Pulm appts scattered throughout. Much better than 2009-2010 when she was at the hospital 4 days a week.

Meeting with her Pulmonary doctor last week was definitely good news. Talking with him - if Haven didn't have ROHHAD he would probably not put her on a cpap machine at all. Her numbers were within normal levels for him but with ROHHAD he just feels it's better to be preventative than reactive.

In 2006 we started going crazy wondering what was going on and then in 2007 ROHHAD and Cancer took over our life....everything we did was reactive. A symptom would pop up and we'd have to attack it with no time to think about what we were fighting and where to go after. Now that Haven is at a point where she is stable and we aren't chasing symptoms we're able to jump ahead of ROHHAD and kick it's butt where possible.

Tackling the Cpap machine:

In an effort to be preventative we have begun to work with Haven on a cpap machine. It is more like a testing phase for her than a necessity. Since she isn't having any major breathing issues and is showing no signs of central apnea at this point we all feel it's good to be precautionary.

A Cpap machine has a setting of 4 - 20. With that, 4 is the lowest it can go. Haven's machine is set at a 5. So just one point above the lowest setting. I am much happier knowing this!

• For the first week Haven has been instructed to wear the mask (without oxygen) while watching TV for 15 minutes each night. Just to help her get used to feeling it on her face.
• For the second week Haven has been instructed to wear it for 30 minutes with oxygen while watching TV. Again just to get her used to it.
• The third week she is to wear the mask for 30 minutes while sleeping with oxygen.
• The fourth week and so forth she is to wear the mask for 1 hour while sleeping with oxygen.

At most Haven will stay at 1 hour each night until her next sleep study. At that point they will monitor her to see if it is at all affecting her sleep. She definitely will not go on a full night sleeping with the cpap until all tests are done and we know it's not harming her. I've mentioned before that I am not a fan of the cpap and that I do believe it can cause harm with the ROHHAD kids if not watched very very close. Just my beliefs after speaking with a few other parents and her doctors.

Although Haven's breathing has definitely gotten better since the inhalers we are still moving forward with this. However, her dad and I are slowly having doubts that she even needs the cpap at all yet. But it's always better safe than sorry.

Once we start actually testing the machine at night I will update everyone on how she is handling it.

Flovent inhaler working! :)

Well....after having a couple of weeks since my initial meetings with Haven's doctors and getting in to fit her on the cpap we are feeling much more comfortable with Haven's recent sleep study and the results.

Since Haven's treatments (which you can read about to the right of the screen) she has changed drastically. Many of her symptoms regressed or went away entirely. Again telling us what we already felt and that we are happy with the decisions we made to go further with her chemo. But then we had a bad sleep study....right?

When I spoke with her doctors over the Christmas holidays I was definitely concerned that her sleep study had changed so drastically from only 8 months previously....but then I was reminded of a few areas:

1. This was the first sleep study EVER that we agreed she slept like she did at home. None of her other sleep studies really made sense to us since she barely slept.

2. January 3rd Haven had a chest xray showing a bronchial infection of some sort. She'd had a cough since November. Her pediatrician gave her an antibiotic to take for 5 days. It did help a little.

So maybe Haven has been the same throughout all the years but never really had a good sleep study. Plus she's never had a cold or cough in her life so this was new to us.

Haven did the PFT (Pulmonary Function Test) - first without an inhaler and the second try with the inhaler. This gives the Pulmonary doctor info as to whether or not her bronchial tubes are narrowed in due to inflammation or something - possibly like Asthma. Although we have been told she does not have Asthma.

Haven did test better AFTER the inhaler of Albuterol. So it was confirmed that she would take two different inhalers -

Flovent - which is a steroid to open the tubes and she will use this twice a day for one month. Then we will check to see if she is doing better.

Albuterol - just for emergencies - like if she is having a coughing fit at night or something.

So I'm fine with these - nothing major. My brother lived on an inhaler for years due to asthma so I understand them.

The doctor said it could take 3 weeks of Flovent to notice a difference.....

Medically I can't say there is a difference because I don't have her hooked up to a test each day but after one week Haven has minimal coughing during the night and is definitely feeling much better. We haven't had to use the Albuterol yet - which she is happy with because she says it tastes funny.

Now if we can just have a full night sleep - sleep walking is amazingly draining!!!

Visit with Pulmonary

In November 2010, Miss Haven had her 1 millionth sleep study (well not millionth but it feels like it). This was the first time she's ever shown signs of anything. It was minor but enough changes to warrant the need to have her on a Cpap machine - at least to see if it even helps.


She met with the nurse and respiratory therapist to fit a few masks and to figure out which one she liked best. Her mask will only cover her nose - which is nice. The one in this picture is not the one she chose. She didn't like the way it touched the top of her lip and she didn't want to see the blue. We let her make the decision of which one was best for her.


Ultimately she chose this one. It is completely clear and fit perfectly on her nose. I told her it looked like she was heading in to space. She will need to wear this each night once she has built up a tolerance for it. Which I'll explain that in a few.


After being fitted for her cpap she had to have a Pulmonary Function Test. This is a great test to check many things - some are:

Air volume going in and out of the lungs
How fast the air can be moved in and out
How stiff the lungs and chest wall are

The first part of the test was just Haven doing it on her own without stimulation to the bronchial tubes. We were also looking for signs of asthma with her recent chest xray results.

Once she finished the first part of the testing she was given two puffs of Albuterol. Albuterol is used to prevent and treat wheezing, difficulty breathing and chest tightness caused by lung diseases such as asthma and chronic obstructive pulmonary disease (COPD; a group of diseases that affect the lungs and airways).

Then she had to redo the tests again to see if there was any difference in her lungs.



Here is a video of Haven doing the second part of the testing. She did awesome.


Looks like a 2 year old scribbled on the computer but this was Haven's breathing results.

Ultimately it showed us that she is in the almost normal range...she was still low on her testing but not too bad. Then it showed us the difference the Albuterol made in her test - which was quite significant.

We ended up getting an inhaler - Flovent - which she will use twice a day. This is a steroid to open the lungs. We will use this for one month and see if there is any change.

Then we got another inhaler - Albuterol - which is only used as an emergency type inhaler. This is for the coughing attacks that she might have in the night - they can last for 2 hours at a time and she will sleep through it all but I can't!!! It will help with the wheezing as well.

The Cpap machine will be delivered tomorrow. We will start her off slow - as I'm not even sure I want to do this. Too many variables that no one knows about with ROHHAD and the breathing machines.

Initially she will use it while watching TV but without the machine hooked up. About 20 minutes a day - just to get the feel of the mask. Then we will move to hooking the machine up for about 20 minutes while awake and supervised. Then she will move in to sleeping with it for 20 minutes while we are awake and can check her. Pulse Ox machine is required to be used during this time as well. Slowly we will graduate to 1 hour. She will remain at 1 hour until her next sleep study is concluded and the tests have been read.

I am happy with this decision. I like taking it slow and if I see anything I don't like we can quit and call the doctor. Haven is happy with it too.

Next up is her ENT appointment to check her adenoids and tonsils - but with the statistics of it only helping 60 - 80% of the patients we're not really in to adding another surgery right now.

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

Sleep Study 11-26-10

The day after Thanksgiving Miss Haven was scheduled to go in to sleep study....which means a lot of driving for mom while dad stays up all night watching her. It's a 2 hour round trip drive and I do it twice each study - they have to be there at 8:30pm and have to be picked up at 5:30am.

To get ready for the study - if you've never had one - is about a 1 hour and 20 minute prep.

First they pretty much scrub her head, face, chest, and legs with this gritty stuff (see photo). It smells kind of like toothpaste but is very sandy. Then the chest and stomach straps are placed around her with the monitors hooked to that. Then she has both toes wired up for heart rate and oxygen levels. Once those are all in place and working they can start with the wires.

Here you can see Haven's chest strap and face wires. The wires are stuck to her with a glue and then taped on. Only problem - Haven is allergic to tape! Doesn't matter what tape it is...she is allergic to it.

After they wire up her face they wire up the head. The glue is horrible and I'm sure not at all comfortable to sleep with. I can't imagine if my child was not cooperative......say like some of my nieces and nephews!!! I can't imagine them sitting still for 1 1/2 hours while getting wires glued to them and then having to sleep with them on. Luckily my daughter is better than I would be at this.

As the night wears on you can see the happy child! This shows both straps and all the wires so far hooked up behind her. Haven did have extra wires this time due to the monitoring for seizure activity.

Finally the gluing has stopped and she is wrapped up and ready to lay down.

This is a sensor they put on her throat to monitor the snoring. Haven has had some pretty extreme snoring the past couple of months and even causing us to miss some sleep lately.

All of her colorful wires. They always let Haven pick which colors she wants to put on first.

And it's night night time


We are hoping for some good results from this study. This was the most Haven has ever slept at a study - she usually only sleeps 1 - 2 hours....this time she slept the entire time. She was also snoring with in 2 minutes of falling asleep and that stayed constant all night. Luckily they also video tape her during the sleep study as she was a crazy sleep walker (glad they have bars on the beds to keep her in). She was screaming so much and so loud the technician kept coming in asking if Haven was ok. Kris kept telling her yes that this is exactly what we've been waiting to show the doctors.

Saturday after we got home I gave Haven a long soaking shower to remove all the glue and tape residue but it didn't help. Still two days later she is blistered from the tape and has a bad rash on her face.

We won't have results for at least 2 weeks but am hopeful they can get to us sooner - we just have to wait for Neurology to look over her results first. Over this weekend she had us very concerned as each breath while sleeping also caused her chest to sink in pretty deep. We checked on her about every hour.

Pulmonary November 4 visit

Last Thursday was my turn to take Haven in to her doctor appt. These things always run about 2 - 2 1/2 hours....plus drive makes it a full day! We left at 9:30am and got back around 2pm!

We met with the student doctor first - they always do this at Hopkins. I understand the need to teach but I hate repeating every aspect of this horrible syndrome! However, this time was different - may be it's because I've walked out of doctor's offices before because they didn't even take a minute to look at her file...but this student knew about 75% of ROHHAD and all of Haven's symptoms. I was very impressed!

After getting a thorough check up we met with her regular Pulmonary doctor and discussed her sleep walking habits and a few new issues that have popped up. Well not that sleep walking for her is new (she's been doing it for almost 4 years) but it is no longer at 11pm start. It starts between 1 - 4 am now and sometimes last for over an hour at a time. Even as I write this - last night (november 8th) she was going ALL NIGHT! There was a break here and there but it was pretty much non-stop. Even when I left for work at 6:15 this morning she was still going.

This is where the doctor is concerned on the sleep walking. Normal sleep walking should happen around 3 or so hours after falling asleep. With Haven's starting 5 hours after or not even starting until 5 or 6 am it raises some concerns. His main concern is seizure activity. He is concerned that it is possible that there is some seizure activity happening in her sleep causing the sleep walking to occur at these times. He made sure we have her emergency seizure medicines and what we need to do. But Haven hasn't had a seizure since 2007.

Then there is a new development in her breathing while sleeping......the past 3 months she has begin to snore. It started off small...random snores. I tried making the house colder, hotter, sleeping sitting up and changing her pillow. Now she is snoring every night and practically all night long. Even when taking naps on the couch she is snoring.

So with both of these issues happening around the same time the doctor is pushing her sleep study up to get us in quicker. There is usually a 3 month waiting period. He will be involving Neurology on this sleep study and adding an extended EEG to the test. So it will take a bit longer to get the results back as Neurology will need to read their section. If she is showing seizure activity she will start seeing a Neurologist regularly....but let's just hope that's not the case.

Haven's last sleep study showed tremendous improvement in her CO2 and sleep habits. She had dropped from a CO2 level of 50 to a 45 and wasn't really going crazy in her sleep. So once this is scheduled and done I will feel much better.

Next up in regards to doctors for Haven is Oncology on November 15th - she is due for another round of IVIG and then hopefully a quiet holiday!

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.

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