Cariyana's toil

At a Pulmonary appointment in December 2010, I registered my ongoing concern for Cariyana's sleep patterns. I was told the doctor would discuss it with the sleep specialist and get back to me. I never heard anything. Since then, I have learned he did in fact discuss it with others. "They" are not sure that there is much more to be done to increase the size of Cariyana's airway. She has already had her tonsils, adnoids, and uvula removed.

Prior to March 20th, we had been concerned about Cariyana's worsening night time breathing for about 5 days. Loud, restless, talking in her sleep, get her up to the toilet and she falls asleep on the toilet and even fell off the toilet! I called Iowa City Childrens specialty clinic with my concern for Dr. Smith, pulmonologist. They would have he and/or the sleep specialist call me. The nurse advised for me to tell them my credentials to be sure I was heard. She remembers Cariyana from her birth.

March 21st--I had Cariyana sleep on my office floor so I could keep an eye on her and her pulse oximeter readings. I did everything I could to try to get her numbers to stay up where they should (greater than 90) including jacking up the oxygen. She was so restless, arching her back (trying to open her airway), she was just miserable, but desperately tired. I wondered at this point if our pulse oximeter was accurate with some numbers as low as 68%. I finally turned off the pulse ox and went to bed. The next morning, she awoke as usual, and went to school. Only one thing was different. I found her sleeping on the bedroom floor. Nothing different than usual really, occasionally she has been known to fall out of bed. She must have fallen out, and decided to just sleep there.

Tuesday, March 22nd--The school nurse called. Cariyana had been sent to her due to coughing. She was not herself--quiet (she is usually VERY chatty), appeared lethargic, and her pulse ox was 82%. A room air pulse ox for Cariyana when awake of 82% is unusual. I made an appointment for 11:30 with Dr. Anderson and rushed out of the office to pick her up. Dr. Anderson took one look at Cariyana sleeping in my arms and said for the staff to call the ambulance and told me she would get a helicopter ride today. Cariyana went by ambulance from the doctors office with low pulse oximeter readings (in the 80s). She was fine as long as she was awake. Sleeping she was so much worse. Our first stop to get to the University of Iowa where we needed to be was Genesis East ER.

Dr. Anderson's last words to me were, "be sure she is evaluated for recurrent brain stem compression causing any of this apnea". Cariyana had decompression surgery at around 9 months.

Dr. Dela Cruz, a long time ER doctor at Genesis, was not overly impressed with Cariyana's condition. Again, as long as she was awake, she was fine. And of course she was interested in what was going on (especially if there were needles involved!). After labwork and an x-ray, we were sent on to Iowa City by ground ambulance.

Once we were triaged in the ER there, we were admitted to the inpatient pediatric floor. Dr. Volk and Dr. Smith saw us almost immediately. I was impressed at the attention paid to us and the speed of their arrival. Of course, it was nearing 5 pm! Dr. Smith stated he had received my message. But, he did not have any ideas or suggestions after speaking to the sleep specialists. All that could be done, had been done. He just had not had time for 2 days to call me. At least he was honest?

Dr. Volk declared the tentative diagnosis was sleep apnea and we would try a CPAP apparatus at bedtime. It would take a little experimentation to see if we could find a mask to fit. We would also undergo allergy testing the following day related to the last one month of runny nose. I asked for a crib instead of a regular hospital bed like most 6 year olds could use. I would not be able to sleep myself if I had to watch her fitfully sleep again tonight.

They tried CPAP, the mask would not work, it was too big, or too small, or she would toss and turn so it would not stay on. They tried to rig it with extra straps, to no avail. I was concerned she might have to go on the ventilator. There was also discussion about a tracheotomy as the only remaining treatment. There were so many at her bedside all night long! Her nurse Alex was really worried. I was there the whole time. But to me, what I was seeing was no different than the night before. I guess I was oblivious to the nearing gravity of the situation. What was different was our ability to monitor her vital signs and labwork, especially her venous blood gases which at that point I was not paying much attention to.

Cariyana tossed and turned. Envision a fish that you just pulled from a lake flipping around on the ground. When she was apneic, she would arch her back to self correct the position of her airway. No wonder she often complained of a headache in the mornings with her positioning. And of course the snoring respirations. The pulse ox kept lowering over the night. Initially just in the 80's, then 70's. But I had seen 68 the night before at home. She was in a crib thank goodness, to keep her safe. She wore out somewhere between 9 and 1 that night.

The pediatric fellow had me call Steve at 1 a.m. to discuss resuscitation status because of her deterioration. That was not a discussion we were planning on having. It took us by surprise. But she was bad. I now wish I had video of her thrashing. Would have been great to add to my Cariyana case study I usually do for students in the spring!! (Sad, huh, I am always thinking of how to spice it up for them!)

Now they had my attention. Code status. That meant she was in jeopardy of arresting or perhaps even dying. What does that mean? I called Steve. What do we want to do? Clearly we do not want a child on the ventilator, nor with a long term trache. We have been through so much already. It would grossly change our family life again. I called my dear friend Kenna who offered to come and also drive Steve who had been working 10-12 hours a day and who rarely got enough sleep working 3 a. m. to 3:30 p.m. I was afraid for him to drive. I was afraid we would need to make decisions on the phone while he drove. I called Joann, the only other one who truly knows what it is like to care for Cariyana and a chronically ill child with a complex medical history. She did not offer much advice other than to try to hold off until morning to make a final decision.

By 2:30 a.m. Cariyana went to the PICU (Pediatric Intensive Care Unit) with pulse ox's dipping into the upper 50's and 4 -5 episodes of apnea per minute. Nothing they tried including high flow oxygen and cpap made a difference. They even tried stomach positioning (on a tower of pillows) to get her tongue to drop forward. It became clear that the tongue was the issue. It was obstructing her airway. Her CO2 level is now 100!

Finally, in the PICU they placed a very narrow nasal trumpet (long tube into the nose that holds the tongue forward) with instant improvement. Her heart rate dropped from 160 to down into the 90's. Pulse ox 90-100% consistently. No stridor (loud snoring respirations). Finally relief for her!! It was instantaneous. Within 30 minutes her CO2 was back down to 68. By the time Steve and Kenna arrived she was resting comfortably.

Wednesday, March 23rd--They sedated her and she slept the entire day. She did not awaken to move a finger or turn. Sleep deprivation, exhaustion. I don't believe she needed the sedation. She would have slept regardless. She was so tired just from trying to breathe. Each time we awoke her to give her medicine, she was so hard to get awake and stay awake. Like she was drunk.

They removed the sedative around noon. About 4pm yesterday they moved us to a monitored pediatric bed. She slept through the entire transfer. At 5:30 she woke up and was awake about 40 min and did not fall asleep while sitting up. A first for her!

She remained on 6 L hi flow oxygen (hi flow oxygen is something you usually see in infants). The plan was to sleep, then slow weaning of the oxygen to make sure fluids go to the appropriate spaces, placed her on sulfenadil (its VIAGRA!!) for her assumed recurrent pulmonary hypertension. She now has significant cardiomegaly (enlarged heart) since her last chest x-ray in October. They performed an ecchocardiogram (ultrasound of the heart), it does not appear much different than last year at least unofficially. That is very good news.

A plan was made for Thursday. A nuerology consult will talked of to assure there is no recurrent brain stem compression causing any of the apnea of last night. She was to just sleep. She needs to catch up. The crisis portion of our stay is over. The nasal trumpet could be something we do at home for a temporary fix to keep her tongue out of her throat for better breathing. But we need a more permanent solution. Perhaps some surgery in the future to make her airway slightly larger. Bring her mandible forward, do "something" to her tongue. This next step would be planned well with a team. Cariyana is very complex.

Thursday, March 24th--Cariyana's blood gases were much better with a CO2 of 48 vs. 64 of yesterday. She is still on the hi flow oxygen but they may start to wean it down. I hope the trumpet can come out or at least be moved to the other nare (nostril). Its bleeding and sore. And all Cariyana did was sleep today.

Friday, March 25th--My link to the world during this hospitalization was my laptop. I cannot imagine what it would have been like without it.

Cariyana is off oxygen and saturating 94-96% on room air. They did not remove the nasal trumpet yesterday. The wanted to do a slow wean of oxygen first. I am getting bored and a little hungry for adult conversation!

Cariyana has a fever 101.4. I am thinking atelectasis related to her not doing her CPT vest all this week. That is one thing we do religiously each day and if she were ill I would do it 2-3 times in a day based on how her lungs sounded. I suggested CPT to the resident yesterday....not ordered or not done. What do I know? The nurse got me a manual percusser this morning and I did it myself. They removed the nasal trumpet after lunch today. I will get to reinsert it tonight before bedtime.

Cariyana's blood pressure has been running 150/110's with a rare normal one thrown in here and there. A nephrologist has been consulted. To my knowledge her blood work has all been normal. The nephrologist does not want to treat her blood pressure. He said there is concern for her lungs if we do. With his preliminary view of the records it does not appear the hypertension has affected her systemically. Instead, we will monitor her blood pressure twice a day and FAX the results in to the office. She underwent a kidney ultrasound late today. I did not get any results but assume it was normal.

Saturday, March 26th--Insertion of the nasal trumpet by mom last night went well. Her oxygen tubing helps to hold it in overnight. Cariyana did not have much anticipation of the event (her co2 was 100 the last time it was inserted) and it went well. It took her about 20 minutes to adjust to the sensation (cough and gag0. She left it in all night. She barely moves now when she sleeps. I have never seen her rest so well.

No fever through the night (We did CPT yesterday).

IF the next two or three nights at home go as easily as last night we may even go with the rest of my family to Texas as planned for Spring Break.

Cariyana and I finally made it home Saturday night. As it ends up the pulmonary and cardiac docs could not agree on treatment (viagra or not).

So, we came home with f/u apts in a few weeks from ENT, pulmonary, and cardiac.

Cariyana's saturation on room air is back to baseline. In order to sleep now we must insert a nasal trumpet. She hates the insertion of course and it takes her about 20 min to get over the gag and find a restful place in order to fall asleep. So far so good. We inserted it "family meeting" style last night. Steve and Nick will need to learn. The other kids will likely participate with the removal over time.

We have issues to sort out and clarify--cardiomegaly, pulmonary hypertension, whether to resume suldenafil (viagra), and she has now developed marked hypertension (150/110) which they do not want to treat due to its potential effect on her lung status.

In the end now it has been made clear. Her chronic elevations of co2 related to her obstructive sleep apnea is what lead us to this point. The sleep apnea cannot be treated with cpap--its a tongue issue. (In the future I still plan to request a cpap trial to convince me that high pressure will not help. I think I need to be convinced before allowing them to suggest what seems to me to be pretty invasive surgery like removing part of her tongue.) Apparently the face grows during adolescence in normal persons. If that is the timing for achondroplasia children, that is 6 years off. The nasal trumpet until then seems harmful to me and to all but the ENT doc who was quite flip with me in the PICU.

If I were advising someone else about leaving on vacation I would tell them no. We are going to prepare to leave but trim our time gone. Charley will be broken-hearted if we don't go to Texas to see his birth family. My husband does not relish the trip without me as the coordinator/communicator. He is a great support person but takes a back seat to many of these kids birth family connections. I truly believe Charley will be a better teenager with this visit completed.

So, that is the story. We are heading to Texas. It won't be the trip we planned but we will have some fun seeing the Oklahoma City Memorial and at the Fossil Rim Wildlife Center. Plus the whole birth family thing.

Dr. Anderson has been updated. He is not supportive of the trip exactly but said "you do know where the medical centers are along the way, don't you?" Iowa City docs said I could go if I am comfortable.

This has been very therapeutic for me to write out this experience and it certainly clearly outlines how complex Cariyana really is medically.