Tuesday, December 28, 2010

ROHHAD sucks

As with most mothers of a child with Cancer I should be stressed and worried about this and it coming back. If you sit and think of one of the worst things that could happen to your baby it would be that they would be diagnosed with Cancer that randomly selects who it wants to attack. However, then you throw in ROHHAD and I don't even have time to think about weather or not Haven will have Leukemia before she is in her mid 20's or if all the chemo she has taken will cause another type of Cancer we have yet to hear about.

Haven has done a couple years of experimental chemo that has completely regressed some symptoms and helped her in so many ways. She is a completely different child now than she was when we first entrusted Hopkins to save her life.

In 2008 (at age 4) we were told - Haven is on the progressive road to stop breathing on her own by age 7.

I have been terrified of this number since then....almost 3 years. As it is Haven is set to turn 7 on January 26th.

In early November I scheduled a meeting with her Pulmonary doctor and told him that I had noticed Haven was snoring a lot and very loud. I also noted to him that her chest was caving in a bit while sleeping like it was hard for her to breathe. Her sleep walking episodes are also from 4am - 7am. Sleep walking should start around 3 hours after falling asleep in general.....not when you are about to wake up. So she was evaluated by two doctors and quite frankly when I request a test they usually don't argue if I bring some facts.

So Haven was scheduled for sleep study immediately and we did her test on November 26th. During that time we changed phone numbers a few times and my numbers weren't in the Hopkins system so they couldn't get me. Finally Haven's pediatrician got the info and sent an email to me to urgently call her and come in.

So on Dec. 22nd I rushed over to the Peds office and reviewed Haven's sleep study results in detail with her for about 45 minutes. She gave her suggestions and listened to what I wanted. Then we conference called her Pulmonary doctor to review the tests and figure out where to go from here.

Comparison (normal CO2 being between 35 - 45) :

Feb-09 Aug-09 March-10 November-10
Oxygen 98% 97% 98% 93%
CO2 49 50 45 51
Resting HR 160 145 90 85

So you can see in the above before and after chemo - those are just some basic stats of Haven. It's hard enough for family and friends to understand and comprehend anything about this disease so we'll keep this easy! :)

So in 2009 I was told it is VERY difficult to jump up from 50 to 51. That it is a big jump to go up one point and they didn't think Haven would do it any time soon. Then after he chemo she actually dropped 5 points. Which was amazing and Haven has never been diagnosed with an apnea in any of her million sleep studies.

November 2010 report:
  • CO2 jumps up 6 pointsO2 drops
  • Approximately 5 episodes every hour
  • Sleep efficiency rated at an 81%
  • Abnormal gas exchange
  • Prolonged period of wakefulness from 3:30am until 4:30am (not related to seizure but is parasomnia)
  • Snoring is a level 3 out of 4
  • No significant central apneas
  • Paradoxical inward rib cage motion (her chest is caving in when trying to breathe)
Haven is now diagnosed with Moderate Sleep Apnea from zero apneas.

Obstructive apneas occurred during REM sleep
Desaturation episodes occurred during non-REM sleep

So what is next???

Chemo and meet with Oncology on January 3rd (due to Haven's drop in IgG levels she will have chemo for her next few visits no matter what her labs are)

ENT - we have been referred to one of the top specialists at Hopkins for Pediatric ENT. I will schedule this today to have her Adenoids and Tonsils checked. You know tonsils but adenoids are hidden here:

Pulmonary - fitting Haven for a C-pap machine, however, discussing my growing concerns with this as well. If you haven't seen one it looks like this and she'd wear it every night at bed time.

If ENT concludes that Haven's adenoids and tonsils are too large and wants to do surgery as of now we have decided to decline this. It has to be an extremely good reason that a C-pap wouldn't work. This would be Haven's 8th surgery and who knows how many times she's had anesthesia for MRI's. Each time Haven has anesthesia she is in the hospital longer and on Oxygen longer. Yes I know this is a basic surgery and whoop de doo......but Haven's last surgery was only 30 minutes long but left her on oxygen for 2 days.

Fitting for C-pap will happen after the first of the year - which will then include multiple sleep studies to make sure it is at the right pressure. And yes I know a C-pap is a whoop de doo thing too....but not for a 6 year old. So if you are age 45 don't say "oh I have one of those, it's nothing".....did you have one when you were 6? We haven't even decided if this is what we want to do either.

Anyway.....this was our latest sleep study. Usually I forget we even had them and am reminded by a doctor when they say it was uneventful. But this time I had her doctors hunting me down.

Yeah.....on to 2011


  1. Misty can I copy this and take it in to Nikki's doctors please {I know you'll say yes} as I also get the "uneventful" talk too but this time I would like more. I have read up so much about ROHHAD now that I want some answers and I wanna know why Nikki's doctors think she doesn't have it anymore!
    Oh and yes I've met alot of adults that say "oh I sleep with that or my husband sleeps with that". Nikki has slept with it since 2 Sep 08 and not much of a complaint except she hates it when she can't talk very loud with it on! :) I think they just accept it and know that they need it...I know Nikki does...God bless her!

  2. Dear Misty,

    Thank you for the medical update on Haven. I pray that whatever procedures that Haven has to have that they will help her.

    Still keeping Haven and family in prayer.

    With caring prayers,


  3. Misty, I should send you some copies of Josh's flow charts showing his O2, CO2 and heart rate. We have an end tidal CO2 monitor hooked up when he is on the ventilator. The numbers you showed aren't to me all that terrifying. Josh's CO2 and O2 numbers are all over the place. We actually make frequent adjustments to his ventilator settings based on the numbers on the monitors. The numbers change constantly. For example, Josh can start off with CO2 in the mid to upper 50s and it can go down to the low 30s in like an hour or so. If he gets a little bit congested, it can go back up within basically seconds to the 40s or 50s and as soon as he coughs and clears, it will be back in the 30s almost immediately. What I'm saying is I wouldn't panic over the numbers they are giving you. I wish I could calm your fears further, but I know just the thought of a possible respiratory failure is extremely frightening and nobody can possibly guarantee that won't happen to Haven. But just remember, nobody can really say it will happen, either, no matter what treatment she has or doesn't have. I pray for her to only get better and better as she grows - and it looks to me like she is doing just that.