The trach

Birth is a powerful experience.  Many women report discovering untapped reserves and strength they never knew existed.  This was the case with Bekah's birth.  It was a 33 hour long labor and a fully natural, unmedicated birth.  I felt so powerful and so strong after she was born.  I knew I could face anything after that.  But here I was six weeks later listening to a doctor telling me that if Bekah's next extubation doesn't go well, she would require a tracheostomy.  Translation; they were going to cut a hole in my baby's throat!  There was nothing I could do.  We were at the mercy of her respiratory muscles.  Hope of bringing her home in tact was fading.

After the breathing tube was removed, Bekah only breathed on her own for about half an hour before she became tired and needed intervention.  They put the breathing tube back in, allowed the machine to breath for her and made the final decision. Her operation was scheduled for just a few days later.

The tracheostomy is a fairly simple procedure.  A small incision is made in the neck below the adam's apple, a little fat is removed, and a tube is inserted.  Sutures are tethered to the shoulders to ensure the aperture stays open.  I kissed Bekah goodnight as she was wheeled into the operating room a second time.

I waited in the sunlight outside.  The most profound sense of peace came over me.  It is the kind of feeling one can only experience in the utmost defeat.  The moment the mouse, still living, lays limp in the cat's clutches.  There was nowhere to go from here, no fighting to do, no pleading, or hoping.  Bekah was getting her trach.  HER trach.  It was now a part of her life and it belonged to her, helped to define her and would hopefully sustain her life.

When she returned from surgery, she was still sleeping, bloated from the extra fluids they gave her, and completely paralyzed.  They had given her a medication to ensure she wouldn't move during the procedure.  Her tongue was the first to awaken.  I watched as it slowly began to move, seeking comfort.  She was eager to suck but she wasn't strong enough to hold the pacifier in her mouth.

diaphragm plication

The surgeons weren't optimistic.  They didn't think that she would be cured by a surgery that would tack her diaphragm down on the right side and  make more room for the lung.  The doctor told me that if it didn't help, Bekah would have to have a tracheostomy.  I hated the idea.  Jeffrey hated it even more.  'No way I'm going to let them mutilated my daughter if they aren't going to at least try to fix the diaphragm!'

No one is born with a trach.  It was bad enough that she needed surgery to begin with.  I was the kind of mom who uses cloth diapers and coconut oil hoping to let the child wean from the breast when ready even if that meant they were old enough to use full sentences to request nursing.  A trach meant such a departure from my aspirations for what childhood would be like for Bekah.  It's an artificial airway made by creating a whole in the throat through which a tube is inserted.  It's like a very severe body piercing but with a purpose.  It meant careful ongoing care.

I found myself nearly begging for the diaphragm plication.  I even sought an advocate at the hospital to ensure it.  Reading up on the surgery, I learned that most children with paralyzed diaphragms are weaned from the ventilator after three days.  I was holding out hope that this would cure her and we could take her home in tact.

It was a long surgery.  It took more than three hours.  I was told that a small camera was inserted into her chest cavity, her lung was deflated, a second "tube" was inserted to do the procedure.  She would have a few small scars if everything went well.  The process was like laproscopy.  But LAProscopy applies to the abdomen.  She was having a THORAscopy.  It was in her thorax.

I walked with her to the operating room and kissed her goodnight as she was taken behind the double doors.  My oldest child had had surgery when he was less than three months old.  I knew the process and wasn't too concerned.  I just held my breath that this would be curative for her.

Jeffrey and I enjoyed some sunshine while we waited.  It was too disturbing to be in the hospital room with out her.  I finished the season finally of "Breaking Bad" and, as the credits rolled, a surgeon appeared at the door.

Not far behind him was Bekah in her crib, still asleep from the anesthesia.  She had a tube in her chest that drained fluid.  That was pretty gross, watching the reservoir fill up with reddish fluid, then brown, then clear.

She was still intubated.  I knew her next trial would be soon.

code blue

Bekah's next extubation attempt was to be handled with kid gloves.  She would be given steroids to minimize swelling of her air way days in advance.  She would be given more time off the fentanyl to allow her to be more alert.  She would also be placed on a high-flow machine.  This is a breathing machine with a nasal cannula that basically blows air up your nose.  The idea is stimulate breathing.  Think of what it's like when you hang your head out the window of a moving car.  The force of the air in your face stimulates the inhale.

Once the necessary precautions were taken, Bekah's breathing tube was removed and she was transitioned to the high flow  machine.  She didn't like it at all.  She was very agitated.   Dr. L came in and said she must be having withdrawals from the fentanyl.  "We are going to start her on methadone."  "METHADONE??!!  What is this the Betty Ford clinic?  We left Venice so we would never have to face this."  Jeffrey and I laughed.  We couldn't believe our 1-month-old infant needed a drug with such a stigma.

If you haven't noticed a theme in the previous posts, everything in the hospital takes hours.  Hours to set up the high-flow machine, hours to coordinated care for extubation, hours to do a blood gas.  What happened next took mere seconds.

The nurser administer the i.v. dose of methadone.  Bekah let out 2 little squeaky sounds.  Her eyes rolled back in her head and she turned grey.

"She doesn't look good."  I told the nurse and ran to turn on the light.  When I returned to the bedside, the nurse was giving my baby a little extra oxygen.  I grabbed the face mask, so Bekah could be bagged.  She was unconscious.  Alarms began to blare.

"Call PICU RT!"  the nurse told her intercom device.

"Calling PICU RT." the mechanical voice responded.

By this time, the nurse had the mask over Bekah's face and she was receiving artificial respiration and the respiratory therapist was on the way.

What happens next is a blur.  Someone called the code.  The room filled with nurses, drs, and respiratory therapists.  The head of the pediatric department was there looking very nervous.  Someone started chest compressions.  There was a security guard standing outside the door.

"Does she have a pulse?"

"She has a pulse."

"GET THE CODE SHEET!  GET THE EPI!"

I don't know who was talking at this point.  Every voice seemed disembodied except the voice of the alarms warning us that Bekah was near death.

"Epi's in."

"No response."

"Get the adrenaline!"

"Got a line in!"  someone had given her another I.V.

"I wish we didn't have to wait. The nurse is getting it from the med room."  Someone had spoken this to me.  It was no comfort.

The head of pediatrics approached me as I squeezed my husband's hand to near numbness.  "She has a pulse.  Her heart is beating very slowly.  We always start chest compressions when the heart rate drops below 40 beats per minute."

"Adrenaline is in."

My baby's arms were sprawled and jerked with every compression.

"No response."

"Get the narcan."

I knew this drug.  I remembered studying it in school.  It blocks opiates and opioids like heroine at the cellular level so that the drug cannot penetrate the cells.  It is an emergency room medication that is administered to overdose patients.

"We have to wait for it to come up from the pharmacy."

"I wish we didn't have to wait."

CPR continued.  Somewhere outside, a siren blared adding to the chorus of alarms and tense voices.

The narcan was administered.  The alarms stopped and there was a collective sigh.

"Cancel the code?"

"Yes cancel the code."

The head of pediatrics looked at me and expressed his relief.  "She's ok."

Dr. L. approached me and said something I don't remember.  Probably something about adverse reaction and narcan and withdrawals, heart, rates, pulses and "She's ok now."

one the room emptied of nurses, doctors,  and respiratory therapists until even the security guard was gone.  I was cold and shaking, trying to catch my breath after holding it for so long.  I looked at my baby.  She was beautifully pink and sleeping soundly.  It was the best she had looked since the day she was born.  I took a picture.

One The nurse offered me a cup of tea.  Usually, I don't care for tea, but at that moment, it was the best thing I had ever had.  A soothing cup of chamomile was perfect.

Down the rabbit hole

There is such a thing as ICU psychosis.  Patients lose touch with reality due to the 24 hours of activity, lights, alarms and stress.  It also takes its toll on parents of patients in the pediatric intensive care unit.  During Bekah's 3 week long intubation, there were times I wasn't sure she was alive, times when a  near dream state threatened my sanity and times when I was sure I was dying too.  She was in complete respiratory failure. She had several blood draws a day to ensure the vent was set adequately.  She had breathing treatments and every time a nurse went to change her diaper or suction her secretions, her heart rate would plummet and her oxygen saturation level would drop.  She turned blue frequently and needed resuscitation.  Alarms were a constant as more and more tests were scheduled daily.

She had a tube that went up her nose, down her throat and into her stomach.  This is how she got fed. I pumped breastmilk and it was given to her.  Feeding was sporadic because she needed tests that required anesthesia which meant her stomach had to be empty.  My constant question was, when can we restart her feeds?

She was asleep most of the time on a fentanyl drip.  I learned that this drug can be hundreds of times stronger than street heroine.  When she would awaken, she was given ativan to lull her back to sleep. I missed holding her.  I stroked her head to soothe her when she would awaken before the ativan took effect.

The first extubation attempt took place a little over a week after our arrival in the PICU.  Her fentanyl drip was turned off an hour before the trial so she would be awake.  When the tube was out and she was off the vent, I was finally able to hold her.  She was just on oxygen, but she sounded like she was breathing through a crushed rusty pipe.  Her breath was ragged.  She would go 24 hours before she began to show signs of distress.

The next day she was reintubated and put back on the vent.  She had daily chest X-rays that showed her right lung looked small.  Her genetic test came back during week 2.  She does not have CCHS.  Finally some good news.  But that still left her diagnosis dangling beyond reach.

One day, dr so-and-so approached me with a theory that there might be something wrong with her diaphragm.  We would have to wait out the weekend for the fluoroscopy.  This is a live X-ray, like a video instead of a snap shot.  Not long after the study, there was finally some news.

Her diaphragm was paralyzed on the right side.  This meant that when she breathed, the left side would contract normally, but the right side would push up into her lung causing it to collapse.  There's a surgery for this.  It's called plication, where they draw the diaphragm together on that side like you would draw a curtain and tie it back.

I was finally relieved.  It seemed a cure was in sight

Intubation, just the beginning

I knew what intubation was.  My sister had been intubated just before her death.  I knew the sound of the ambu bag forcing air across the vocal chords.  I was sure this meant my baby was dying.  I nearly collapsed.  I had to leave the room as the doctor slid a tube down Bekah's throat and connected it to a ventilator so she could breath.

When I saw her again minutes later, she was asleep, doped up on ketamine.  A paramedic transport team was on its way to stabilize her and transport her to Kaiser Sunset's PICU.  I kept telling myself, if they can stabilize her she can live.  My sister had died in preparation for transport.  I knew how difficult it could be to stabilize a patient.

The paramedic team arrived with a gurney that had everything Bekah would need to live for the next event.  There was a glass box used to stabilize her body temperature which had dropped to a frigid 96 degrees.  There was a ventilator and several different monitoring devices.  I couldn't wait to get her in the box and warm.

The team worked for hours to ensure that transport was completely safe for her.  When she was finally loaded into the back of the ambulance, I breathed a sigh of relief.  I rode in the front and could hear that things were going well in the back.  Bekah was stable and tolerating the transport without incident.

We arrived in the isolation room at the PICU surrounded by drs and nurses.  One of them approached me and asked if I had ever heard of CCHS or Ondine's curse.  I signed a convent form to run genetic testing for this horrible disorder and was told that my baby may need a teach for years.  Later, I would research CCHS (Congenital Central Hypoventilation Syndrome) and learn that for some families,  this means their child doesn't breath during sleep.  It would be weeks waiting for the results of the test.

not long after that

The paramedics arrived in short order.  It is lucky we live just around the corner from the fire station. We were on our way to Cedar's Sinai in no time.  When we arrived, she was dusky and crying.  They ran an echocardiogram that took hours, urine tests, blood test, you name it.  I was distraught.  Trying so hard not to cry or panic in front of my 2 week old infant.  If I was scared, she was certainly terrified.

Because we are Kaiser members, we were to be transferred.  This took nine long hours.  I was hungry, tied and very very worried.  The pediatric room in Kaiser Sunset was a welcome solace to the 12 hours we had spent at the e.r.

In the morning, we were visited by a number of specialist and the head of the pediatric department.  She would be observed carefully over the following week.  She had an ultrasound of her brain, more blood tests, and eventually a sleep study.  This was the most revealing.  It determined that she had central primary sleep apnea.  Basically, she would stop breathing for more than a minute when she was asleep.  The head of pediatrics diagnosed her with "exceptional periodic breathing."  His hypothesis was that she breathed like a premature baby, sometimes faster and sometimes slower.  He expected that she would out grow it by two months of age.

We were sent home on an apnea monitor that strapped around her chest and supplemental oxygen.  This monitor was about as useful as a broom at the beach.  It would alarm that she had quit breathing.  I would look at her and see that no such thing had occurred.  It would alarm if she got sweaty, which happened quite frequently.

Two nights after coming home, the monitor alarmed.  She was breathing.  It alarmed again and hour later.  I cleaned the leads.  It alarmed again.  This time my husband awoke and Bekah was crying.  I told him to stay up with us.  It had never alarmed so frequently and I was becoming concerned.

Bekah was becoming more and more agitated.  I couldn't calm her.  I couldn't sooth her and she couldn't nurse at all.  Hours passed as Jeffrey and I took turns tending to her.  She became cold and sweaty.  Her head bobbed with every breath and the skin around her collar bone retracted each time she inhaled.

We called the advice nurse at Kaiser and were told to call 911.  When the paramedics arrived, she was finally sleeping comfortably and her blood oxygen saturation was 100%.  Maybe she was just colicky.    
So we sent the paramedics away hoping not to return to the long wait at the e.r. needlessly.

The sun began to rise and I realized that Bekah hadn't fed in nine hours.  I offered her a bottle, she just cried as the milk ran down her chin.  I realized that she would have to be seen today.  Determined to avoid the e.r. at Cedars, we  prepared for the short bus ride to Kaiser West LA, just around the corner from our apartment.

She was sweating, so I wrapped her in the moby and headed out for the bus stop down the street.  The morning was overcast and foggy.  I looked at the baby on my chest.  She didn't  look good.  "Does she look blue?"  I asked Jeffrey.  "It's just the morning light."  We were fortunate not to wait too long.  The bust arrived within minutes of our arrival. Bekah was in and out of sleep during the 10 minute ride, crying when she was awake.  If she's crying, she's breathing...right?

We were in the e.r. in no time and were escorted to a room.  Shortly, a Dr arrived.  "Your baby looks sick.  We have to intubate her."

late one night

Breathing is something we all take for granted.  We don't give it a second thought until something goes wrong.  In fact we never consider the constant ins and outs.  I remember seeing Bekah's fetal breathing patterns during an ultrasound and being excited for her upcoming life outside the womb.  I had no way of anticipating at that moment that I would later wish she could go back in utero where she was fully safe.

When she was born, I immediately noticed that she had trouble nursing.  That first night was really rough.  She cried endlessly.  The nurse put her crib on an incline so she was nearly sitting up.  This was the only hour or so of peace we had that night.  The lactation consultant assumed that Bekah's difficulty nursing stemmed from a tight frenulum.  That's the little piece of skin beneath the tongue that attaches it to the jaw.  A short or tight frenulum prevents a person from sticking out their tongue and can cause difficulty latching on.  We had her frenulum snipped in a very simple procedure just before discharge.  I had every hope that life would improve.

Upon arriving home, I was patient as she struggled at the breast, not sure why her problems persisted.  As days went by she began to grunt and sweat.   I noticed breath sounds as she was sleeping.  One night, I was sleeping next to her and she was in a bouncy chair.  It bobbed with every breath she took.  I couldn't imagine that this could be a good sign.  I was getting very concerned.

Not too much longer, I attempted to nurse her again and as she slept in my arms her head bobbed with each breath so much that it nearly rolled off my arm.  I had never seen this before.  I did a quick Google search.  There was a youtube video of a baby in respiratory distress bobbing its head in identical fashion as Bekah.  We were having an emergency.  I called out to Jeffrey as he slept demanding with urgency that he wake up as I called 911.

Home again.

Bekah is home from the hospital again.  This last time was her shortest visit yet.  She was in the pediatric intensive care unit (PICU) two days for observation.  She was admitted for respiratory failure again.  This time was unique.  She is dependent on a ventilator and has had a tracheostomy since October 12, 2013.  She was 6 weeks old when she got her trach.  This time she was supposed to go to the pulmonologist for a follow-up after her last discharge.  She gets non-emergency medical transport for all her medical appointments.  The ambulance came to pick her up and the moment she was wheeled into it, she desated and bradyed turning blue.  Her heart rate dropped to 60. The respiratory therapist took her off the vent and used an ambu bag to breath for her.  She came back up but was still uncomfortable.  The r.t. tried repeatedly to put her back on the vent.  Bekah wasn't having it.  Her oxygen saturation levels kept dropping as she gasped for breath.  She was bagged all the way to the dr.'s office.  I wish you could see the look on the faces of people as we came into the office.  Here's this 4-month-old baby on a gurney being bagged and surrounded by paramedics.  It really looked like we mistook the office for the e.r.  The gurney wouldn't fit in the elevators so the e.m.t.s had to keep unloading it so the back of it would fold down.  Meanwhile, people are staring at us aghast.

We met Dr. S. in the hallway and he escorted us to his biggest exam room.  After a brief conversation, we decided she should go to the emergency room.  Of course!  We piled back into the elevator again. Down stairs, we went code 3 (lights and sirens) across the street to the e.r.

As soon as she was put onto a hospital bed, she was put back on the vent, smiling, blowing raspberries and looking around.  Seems she just detests the car seat.  They kept her for observation anyway.  She did just great during her 2 day stay.

While there, the drs determined that she needs to be sedated for all future transports.  Hello benadryl! They also decided that once home, she isn't to go anywhere for the next two months.  After that she will make all of her follow-up appointments in the PICU.  No going from office to office for follow-ups.  The drs will visit her in the hospital.

She is coin well now, her first night home was uneventful.  She is recovering from a painful muscle biopsy, the fatigue of being in the hospital and now the poor thing is teething.  She just can't seem to catch a break.

I will go into her history in a later post.  I am hoping to share her journey with other parents and caregivers of medically complex kiddos.  I welcome family and friends to keep up to date on her well-being. Please feel free to ask questions, share stories, and offer advice and support.