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.
