Still in Limboland

We had an appointment with the ENT on the 9th.  The appointment went well.  As I expected the ENT wants to do surgery to remove Riley's tonsils.  She is also going to look to see whether Riley's adenoids have grown back and if they have she is going to take them again too.  We have scheduled this for the 29th of the month, however the plans may change.

We still do not know about whether the neurosurgeon is going to want to do anything and we won't until we go an see him this Thursday.  I will do a more in depth updated after that appointment because then we should have a full plan, but I wanted to do a quick update today.

Days 119-130 of 365 Days of Thankfulness

5/8: I am grateful for quiet evenings spent with Kyle and Riley.

5/9: I am grateful that we have such a good relationship with our ENT.  She is leaving for another state and was completely honest with us about which of her partners we should start seeing.

5/10:  I am grateful that I am able to work from home on days that Riley has doctor's appointments.

5/11:  I am grateful to whoever came up with the idea of massages.  I went for a 1 hour one today and it was awesome.  I need to do that more often.

5/12: I am grateful my mother on this Mother's Day.  She is the most awesome woman I have had the pleasure of knowing.  And I am lucking that she is my mommy.

5/13:  I am grateful for my father.  Today is my Dad's birthday.  He is an amazing man.  He is always willing to help anyway he can and has taught me what it mean to be generous.  I love you Daddy!!

5/14: I am grateful for my father-in-law.  Today is his birthday.  Thank you for creating Kyle.

5/15: I a grateful to the beautiful weathI er that we have been having.

5/16: I am grateful to Kyle for taking Riley to her doctor's appointment today and handling the aftermath of it.

5/17: I am grateful that I am able to work a flex schedule.  I had to go home early yesterday and I was able to make up several hours today by going into work early and staying late.

5/18: I am thankful for the two strangers that helped me without be asked today at the grocery store.  One held my cart will I tried to get Riley in and the other return my cart to the cart return after I loaded my bags.  It helps to restore my faith in the human race.

5/19: I am grateful that Riley decided to sleep in this morning because it meant that I got to sleep in too.

Limboland

I have been putting off writing this post for a few weeks.  First I was trying to do more research about it and then I decided that I did not want to worry everyone who follows this for an extended period of time.  But we are hopeful that we will have a few answers by the end of the week so I decided to post this now.

Riley has decided that we needed a visit to Limboland. I hate Limboland. Limboland is a place where you know something is wrong whether it is a serious something or a not so serious something. It is a place where you do not have the answer to the most important questions: "How do we fix this?" "Where do we go from here?" "What can we do RIGHT NOW?" "Has is caused damage already?" "Are we going in for another surgery?" "How long will we be in the hospital this time?" "When can I get in to see the doctors I need? No it can't wait a month; she needs to be seen now." "Does she need a trach again?"

These are not fun questions but they are the questions that have been running through my head since I talked to Riley’s pulmonologist. Riley had a follow up sleep study on the April 12th from her shunt revision back in September. And to say it did not go well would be the BIGGEST understatement I have ever made. Riley’s apnea is worst then it has EVER been.

Before I go into the results I want to define a few of the terms that I am going to use throughout the rest of this post.

Apnea: is a period of time during which breathing stops or is markedly reduced.

· Obstructive apnea (OA): is when air cannot flow into or out of the person's nose or mouth although efforts to breathe continue due, the airway collapses completely allowing no air to pass through during sleep causing the individual to snort and gasp for breath

· Hypopnea (H): is breathing that is shallower or slower than normal. This is a partial obstruction, the airway collapses partial it allows some air to pass through, however much smaller and it is accompanied by a arousal from sleep (either a complete arousal where the person is awake or an arousal where they simply come out of REM sleep) or a desaturation of oxygen for 20 seconds of more.

· Mixed apnea (MA): is a combination of central and obstructive apnea and is seen particularly in infants or young children who have abnormal control of breathing. Mixed apnea may occur when a child is awake or asleep

· Central apnea (CA): occurs when the brain fails to send the appropriate signals to the breathing muscles to initiate respirations

· Hypoxemia (Oxygen Desaturation): occur when oxygen in blood drops, meaning an abnormally low partial pressure of oxygen, content of oxygen or percent saturation of hemoglobin with oxygen, in combination with each other or individually (I refer to this as destat throughout my blog)

Polysomnography: (sleep study) is a comprehensive recording of the biophysiological changes that occur during sleep by monitoring many body functions including brain (EEG), eye movements (EOG), muscle activity or skeletal muscle activation (EMG), heart rhythm (ECG), the breathing functions respiratory airflow and respiratory effort indicators and peripheral pulse oximetry during sleep.

Respiratory Disturbance Index (RDI): is one very important measure of the severity of the sleep disorder. The RDI represents how many times per hour breathing stops or becomes very shallow. This index is important because it is often associated with disruption of sleep and dangerous drops in blood oxygen levels.

Arousal: abrupt transition from a deeper stage of sleep to a shallower stage

Continuous positive airway pressure (CPAP): is a treatment that uses mild air pressure to keep the airways open.

Hypoventalation: Abnormally slow and shallow respiration, resulting in an increased level of carbon dioxide in the blood.

Arnold Chiari malformation: is a structural defect in the cerebellum, the part of the brain that controls balance. The lower rear of the skull is smaller than normal, and thus the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination

Tracheostomy: is a surgically created opening in the neck leading directly to the trachea or the breathing tube. It is kept open with a hollow tube called a tracheostomy tube (or trach as I refer to it throughout this blog).

Now that the vocabulary lesson is over let get back to what is going with Riley.

I think this may be the first time that I might have reached Red Alert status since coming home from the NICU.  I have wondered what it would take to get me to Red Alert status and the numbers from the sleep study seem to be doing it. When I first heard the new numbers I was freaked out but then calm came over me.

I told myself that we would figure it out. We would circle the wagons, gather the medical team (pulmonary, ENT, and neurology), make a plan, and fix this. So got on the phone and called the ENT to set up an appointment with her. I emailed the Neurosurgeon who we already had an appointment with for 5/23 to see if he wanted us to come in earlier or wanted any more tests before we came in. ENT can see on 5/9, and Neurology did not think that we needed to come in sooner. He also did not want an additional testing. I am on Red Alert and her doctors don’t seem as concerned.  Now I love Riley’s ENT, Neurologist and the Pulmonologist, but I wanted to get in and be seen as soon as I got the results.  But that would not be the case.  So instead I started searching online for my own answers. I did not find any, so I resigned myself to waiting.

When Riley was in the NICU she had 4 different sleep studies all with around the same results. I will these numbers with the most recent results.

12/28/09: RDI- 68.7/hr. (obstructive apnea (OA) -38.6/hr, hypopnea (H) -15.6/hr, mixed apnea (MA) -3.0/hr, & central apnea (CA) - 8.4/hr)

2/12/10: RDI- 40.5/hr. (H-35/hr, MA-4.9/hr & CA-.3/hr)

3/13/10: RDI- 59.6/hr (OA -33.4/hr, H-22.7/hr, MA-3.5/hr & CA-1.7/hr

3/16/10: RDI- 52.5/hr (OA-30.9/hr, H-17.5/hr, MA-3.1/hr & CA-.9/hr

The sleep study after the trach was placed was the best one:

5/6/11: RDI- 5.7 hour (OA-.2hr, H-2.2hr, & MA-.7hr)

Then we had a repeat test in January of last year that showed the apnea was coming back. Our ENT decided that it was time to take out Riley’s tonsils and adenoids; though when she went in Riley’s tonsils were not big so she left them in.

1/20/12: RDI- 16.6hr (OA-2.0hr, H-10.3hr, MA-.8hr, & CA-3.5hr

Then we had another follow up last summer that showed that it was still not getting better so we tried Riley on the CPAP machine. Which was not a success. Shortly afterward Riley’s shunt stopped working. It was then thought that the apnea had come back because the shunt was not function properly.

7/2/12: RDI-26.6/hr (OA-14.0/hr, H-10.0/hr, MA-1.6/hr, & CA-1.0/hr

Then we got the most recent results….

4/12/13: RDI-105.7/hr (OA-6.0/hr, H-98.2/hr, MA-1.3/hr, & CA-.2/hr.)

Yeah they are as bad as they look. When you look at these the RDI is the total number of times Riley’s breathing either stops or is shallow to the point where she rouses or has a destat. The other numbers are a breakdown of the 105.7/hr. So basically 6 times an hour Riley is not getting any air going through her airway, 98.2 times an hour her breathing is shallow enough that she is rousing from a deep sleep (33.7 times out of the 98.2) or her oxygen level is too low.

The hypopneas are more concerning then the full obstructions at this point. Every time that you are roused from a deep sleep to a shallower level of sleep your blood pressure goes up. And every time your blood pressure goes up you increase your chances of developing heart and lung problems.  So Riley’s blood pressure is INCREASING 33.7 TIMES AN HOUR. Come on…Really.  33.7 times an hour!  THIS IS A PROBLEM.

The other thing that is not expressed in these numbers is Riley’s CO² levels.  It is normal to have CO² in your blood; a normal level is between 35-45mm of Hg. Anything above 50% is considered hypoventilation. Riley is peaking at 65% and 68% of sleep study above 50%.  So this is bad.

The only good news in this study is the number of central apneas has gone down, meaning that it is not a problem with Riley’s brain telling her to breath.

So at this point we do not have a plan of action other than trying to get Riley use the CPAP machine again.  We have ordered a new CPAP mask because the one we have is not working for Riley.  I will do a separate post about the CPAP machine. And Riley masks once we get the new one in.

Wordless Wednesday

One of the other blogs that I read does this wordless Wednesday.  I thought I might try to start doing this. So this one is not completely wordless.

"Typical' Toddler

I spend alot of time thinking about how different Riley is from other children her age.  But this weekend and tonight she reminds me how the similar she is to children her age too.

On Sunday we went over to the playground that is across the street from our house because two of the other kids who are around Riley age and understand Riley's differences were out playing. After swinging on the swings and sitting and watching the other kids run around (Riley loves doing this) it was time to go in and have dinner.  So we said goodbye and the other kids were going in too.  Well Riley was not ready to go in.  She is threw a fit.  She pulled my hair and tried to bite me.  It made me realize that this was behavior that other "typical" 3 years would do.

Then tonight at bath time Riley decided that Mommy needed bath too.  She would splash around then turn and smile at me, then splash me again.

She amazes me everyday!

Days 106-118 of 365 Days of Thankfulness

4/24: I am thankful to have a rook over my head.

4/25: I am grateful for good friends.

4/26: I am thankful that the Dogwood tree in my backyard seems to be coming back.  It is starting to bloom now.

4/27: I am thankful to my mom who came over on a Saturday to make bow for my sister-in-law's wedding.

4/28: I am thankful for the Nook Kyle gave me several years ago for Christmas.  It has allowed me to read more than ever before.

4/29: I am grateful to have dinner with my girl friends from high school.

4/30: I am grateful that Kyle and I can fight and talk through our problems.  Ever marriage has it ups and downs and as long as you both are trying to make it work it will.

5/1: I am grateful that Riley and Ella, our 85lb Rotti, are such good friends.  And that Ella is so good with Riley.

5/2: I am thankful for how Macy makes it to return and exchange things.  I brought a great dress that had weird stitching in it and they made it super easy to exchange it for another without my receipt.

5/3: I am grateful to Riley's wonderful teachers and the staff at her school.  They had Field Day to today and it was so fun to watch Riley interacting with the other kids in her class.

5/4: I am thankful that Kyle took off of work so that I could go to a concert for my sister-in-law's bachorolette party.

5/5: I am grateful for a quiet Mommy and Riley day.

5/6: I am thankful that the weather held out for us yesterday so we were able to go to the Zoo.

5/7: I am thankful for how happy Riley is when I come home from work.  It makes even the worst day better.