Saturday, February 22, 2014

Sleep Study Results

Finally got the results from our sleep study and have to say it was what I was expecting.

Her doctors here are able to contact her doctors at Hopkins and actually work very close with her old Pulmonary doctor from Hopkins as he travels here every few months and will be here again this summer.  They were able to view her last report from October 2011 which showed she only had evidence of sleep apnea with a Respiratory Disturbance Index (RDI) of 4.6 events per hour.  On December 2013 (2 years later) she showed Severe Obstructive Sleep Apnea with a RDI of 30.8 events per hour.

It might be easier to bullet point some of the highlights.  It's a 3 page report that I'm taking the notes from.  This test has kept me up for the 4 nights now.

  • total recording of the sleep study 515 minutes.
  • total time Haven actually slept 276 minutes (due to choking herself awake constantly as you will see)
  • total sleep time in stage 1 - 9.6%
  • total sleep time in stage 2 - 73.8%
  • total sleep time in stage 3 - 15.7%
  • total sleep time in REM - 0.9%
  • She slept for 244 minutes on her left side
  • during 276 minutes of sleep she woke up 62 times at about 13.5 times per hour
  • no evidence of seizures
  • during her 276 minutes of sleep she had 140 obstructive events resulting in about 30.8 events per hour
  • central apneas at 0 per hour (at least this is good news)
  • mixed apneas at 0 per hour
  • loud snoring throughout testing and nasal pressure/end tidal CO2 poor during most of study
  • notes from doctor: respiratory events may be underestimated due to frequent episodic oxyhemoglobin desaturations
  • during 276 minutes of sleep Haven had 216 desaturations (drop in SpO2)
  • desaturation average of 46.9 per hour
  • SpO2 desats during sleep down to 70.9%
  • average respiratory rate rage 14 - 26 bpm
  • max end tidal CO2 in NREM at 108 mmHg (98% of sleep time)
  • max end tidal CO2 in REM at 45 mmHg (2% of sleep time)
  • capillary blood gas at beginning of study: PH 7.44, pCO2 of 39
  • capillary blood gas at end of study: PH of 7.39, pCO2 of 38
  • regular sinus rhythm
  • minimum resting heart rate 66.7
  • maximum resting heart rate 168.2 



She is currently on oxygen, C-pap machine, and pulse oximeter at home.  

Labs March 8th.  
Surgery is March 10th for tonsil, adenoids, and brochoscopy.  
IVIG on April 5th.  
April 10th follow up from surgery.
Endocrine on May 1st.  
May 3rd labs.  
Repeat sleep study on May 3rd.  
May 22nd Pulmonary follow up.  
Immunology follow up June.
Cardiology will follow up every 6 months unless Pulmonary requests sooner.

Pulmonary follow up

 Last week I took Haven back to Pulmonary for her follow up after being in the hospital for the week.

The doctor didn't like her being on the full face mask so we tested out a bunch of masks.

She definitely didn't like the nasal pillows and ultimately went with the first one pictured above.  I'm not   too sure how much I like the nasal mask.  Haven is a crazy sleep walker.  She is constantly screaming in her sleep and breathes through her mouth.  So having just a nasal mask concerns me.  But we will try it out.  

We also ordered her a chin strap which is to help keep her mouth closed when she is sleeping.  We also have her pulse ox machine set up and have ordered new probes to keep watch on that while sleeping.  Kris got me some baby monitors so I would stop wandering the halls at night and can listen to her at the side of my bed.  So now I can wake up and check the pulse through the baby monitor.  Very nice.

Getting fresh air after hospital

After a week in the hospital we wanted to get Haven out for some fresh air and a walk

Not to mention the babies needed to get out and get the sillies out

We did a small hike and played in the rocks

Weston was having a blast playing in the dirt and rocks and pointing out all the poop to me

Rohnin was having fun jumping from rock to rock and trying to find the babies running around

It was great to get Haven out.  She'd been in a bed all week.

Goofing around in the truck

Oh these kids……they are so hilarious

Thursday, February 20, 2014

Tonsillectomy and Adenoidectomy --- UPCOMING SURGERY

What does it mean?  Tonsils are the two round lumps in the back of the throat. Adenoids are high in the throat behind the nose and the roof of the mouth (soft palate) and are not visible through the mouth or nose without special instruments.

Haven's surgery has been scheduled for March 10th.  She will have her Tonsils and Adenoids removed, she will have a Bronchoscopy done, and she will have her ears suctioned out.  Normally this is an outpatient procedure but because Haven is a rare and unique person she will 99% positive stay overnight for observation and respiratory monitoring.  She also does not do well with anesthesia - none of the ROHHAD child do.

They also say there is generally a 2 week recovery period for "normal" people…..Haven does not have an immune system to aid in recovery so we're not exactly sure what her recovery period will be from this surgery.  She will be kept in a clean environment and we've already ordered some masks that are cooler than the hospital ones for her to wear.  It normally takes her about 30 days to recover from a mosquito bite… this will be a new one for us.

Grandma will be flying out from Virginia to stay with her and pretty much be her nurse during this time.  Which is great because I will be flying back out to Asia and Bangladesh.  It's very hard having a child with a rare illness and no family nearby but it's very nice to know Grandma will fly out every 6 weeks and stay for a few weeks at a time!  LOVE YA GRANDMA!!!!

On May 3 Haven will have a repeat sleep study to see how her C-pap is working after surgery.  Her pulmonary doctor wants her to have a full two months recovery from surgery before making any evaluations on her.  We agree.

C-pap and Home Oxygen

Haven is now required to use a C-pap and oxygen at home.  So that day that we were discharged from the hospital we met with a respiratory therapist at the house.  He was able to train both of us on these machines.  Haven learns very fast and knows all of the controls now.

We were able to set up her room really nicely with all of the machines.  The big blue box is her oxygen.  This hooks up in to the silver machine which is her C-pap machine.  That has the mask which goes over her face so she can breathe at night.  Then she has the third machine which is her pulse ox machine to monitor her oxygen levels and heart rate.  This sounds of the alarms through the house if she desats.  

So far she is doing very well with it all considering it was a very fast transition.  Mom and dad - not so well.

ER & Admitted.Pneumonia to Pulmonary to Cardiology - ROHHAD

Friday February 7th, Miss Haven went to bed at her normal time after getting her shot - around 8pm.  Around 10am on Saturday she was still sleeping and having very labored breathing.  I was home alone with her.  I contacted the other ROHHAD parents on Facebook and then I sent a text to a ROHHAD mom close by.  She reminded me to check Haven's SPO2.  I put her pulse ox machine on and I almost passed out………

Her resting heart rate was around 150 and her SPO2 was dropping into the 70's.  I moved her and moved her….I tried to wake her for the next couple of hours.  Daddy came home around noon and for the next 5 hours he continued doing the same.  By 5pm on Saturday we still couldn't wake her.  It was at that point that I decided to take her to the emergency room.  Once we arrived they immediately took her in.

Here we are waiting in the ER for her port to be accessed. She received two different antibiotics while in the ER room.

We went down to X-ray to check for Pneumonia

Wouldn't you know it…….she has Pneumonia……antibiotics and admitted to hospital.

Within minutes of being admitted Haven falls asleep.  She just could not stay awake for more than 5 minutes at a time.

And within seconds of falling asleep she would desat.

They ultimately put her on oxygen for the remainder of her 5 days in the hospital.  She only had to use it when sleeping so that was nice.

During the day she was able to have Child Life come by and hang out with her.

Haven has a pee issue…she loves to pee….as do most ROHHAD children.  So the nurses weren't moving to Haven's required speed.  They weren't noting how much pee she was producing quick enough so she thought she would do it for them.

Finally after two days she was up and walking around.

Cardiology came in on the Monday and did an EKG to check on her heart.

Then we were wheeled down to follow up with an Echo

Everything looked great and now it's just a matter to figure out why the heart rate is so high when resting.

While in the hospital we were able to go ahead and get her IVIG taken care of

This was great so we didn't have to go back in two days later to do it all over again

While waiting for her respiratory therapist Haven could not hold on.  It honestly did take 2 hours for the therapist to show up and no one else could put the c-pap on her.  For some reason the nurses didn't think to put the oxygen on her.  I had to bug them do to do it.  That was a bit annoying and probably the only issue we had.  I stayed up until 5:30am that night I was so worried they were going to forget her again.

Finally after a 2 hour wait for Resp Therapy she had a c-pap and was sleeping much better.  At one point around 3am she did stand up in the bed and try to jump off.  Luckily I was there to catch her and put her back in the bed.  This has always been my worry about this machine.

Before leaving the hospital Haven was interviewed for the hospital Radiothon.  She has done this previously for Hopkins so she's pretty much a pro.

Again giving her interview.  She also did a round of Bingo and won 4 times.  She's amazing.

We have a follow up appointment with Pulmonary on the 19th - looking forward to that.  She went home with a heart halter on for Cardiology.  She has her follow up with Endocrine on May 1st.  Cardiology follow up in 3 weeks.  Surgery will be scheduled to remove Adenoids and Tonsils.  She will also have a Brochoscopy done and suction out her ears.  

Labs for Birthday

Haven with one of her favorite nurses Danielle.  She only likes Danielle accessing her port.  So we make sure all labs and IVIG is done on Danielle's weekends.

Just a few blood draws today.

For her birthday she got a few gifts from Danielle.  So sweet!

Haven's 10th Birthday

Miss Haven wanted to have a Minion party for her 10th birthday with a mix of girly girl.  So this was the theme.  It was a very nice birthday.  Grandma from Virginia came to visit for about a week and gma/gpa from St. George came to visit.

Lots and lots of girls.  We had a lot of fun.  The girls did a lot of crafts and played lots of games.

Even grandpa had to join in!

Grandma and Haven spent a day together at a beautiful hotel tea room….

Miss Haven at the Garden Tea Room ordering from the menu

Grandma and Haven together

The manager brought Haven a beautiful rose and specially made macaroons

Sleep Study

Miss Haven hasn't had a sleep study since October 2011.  That was done at Johns Hopkins before we left Maryland.  So it was time.

We met with her new Pulmonary doctor - Dr. Tan - at Primary Childrens and got her in very easily.  This was a great study.  I felt very satisfied and had a true showing of Haven's real sleep pattern of how she sleeps at home.

Here she is all prettied up and ready for bed.  Still not sure how she can sleep with all this mess on her.

This was mom's bed…..not like Hopkins but that's ok.  

Just a little screen capture of Miss Haven's sleep…I have no idea what it means.  

We won't get the results for a few weeks.

Monday, February 17, 2014

Ear Infections!!!!

My little boy has endured 10 ear infections in 6 months.  So immediately following my latest trip to Asia he was scheduled for ear tube surgery.  This leaves only one Fowler (including dog) that hasn't had surgery in this family.  Haven still reigns supreme on number of surgeries received.

As explained by the doctor.  The reason babies have so many ear infections is because the Eustachian tube lays flat instead of angled down like in an adult.  This causes the fluid to not drain properly.
The tubes are small and are put into the ear drum.  They will eventual push themselves out.  

I have to say….if I had known that putting these tubes in would stop by son from vomiting 3 - 4 times a day and having constant fevers of 102 or higher I would've done it a lot sooner. He has been such a different little boy.  He doesn't moan the halls at home crying and whining….he actually smiles and plays.  It's insane the difference.  

I have to say ---- DON'T WAIT UNTIL 10 INFECTIONS!!!  Get the child some relief sooner - you'll get to sleep much better!

Hong Kong January 2014

My trip to Hong Kong usually includes Japan.  I love this scheduled flight because it's the 787 Dreamliner!  If you have a chance to fly on this plane - take it!!!!  I generally love the stop over in Japan but this time we had a 1 1/2 hour wait for security……HORRIBLE!!!!

I did get to do some shopping while in Japan……had to pick this dude up for the hubby.

While my first stop over in HK was supposed to be for one night at a hotel and then off to Bangladesh it ended up being a sleepover at the airport.  My flight out of Japan was so delayed that they no longer had shuttles or anything to get to my hotel.  Since I had to be back at the airport in 6 hours I decided to stay and work.  At least until my computer died and then I found a nice metal seat to take a nap on!

The airport was decorated so cute.  As it usually is. 

Swarovski Christmas Tree

Cute little reindeer and tree