Life with One (1) Medically Complicated Infant: The Tricky Bits

In my last post on this subject., I gave a rundown of what an easy day with my son was like when he first came home from the NICU.  Those days were pretty straightforward if exhausting - but I managed to get between 8-10 hours of sleep a day (although none of it was in chunks longer than 4-5 hours), eat three meals, and generally get a chore or two done around the house.

If it was a tricky day, I got 8 hours of sleep, a meal eaten while doing something else, and prayed that my back wouldn't be permanently damaged from any of the gymnastics.  These days also meant that I generally had no downtime between two feeds or I did a tube-feed with Jack in the backseat of the car while one of my parents were driving us to or from the doctor's office.

Here's what I remember from three tricky items: bathing Jack, medical appointments, and in-home therapy visits.

Bathing the Baby:
We bathed Jack about once every 7-10 days.  I'd wipe him down with wet-wipes every two-three days, but eventually he'd get grungy enough that he needed immersion in water.  The tricky bit was that Jack had a feeding tube and nasal cannula taped to his face - well, actually there was a base layer between his face and the tape, but you get the idea.  Removing the wires, feeding tube and nasal cannula was simple especially since we only removed it when the base layer was coming loose on its own.   The exhausting bit was replacing everything after the bath. 

Expanded for details.  The beige layer visible on his cheek is duoderm which had a sticky bottom layer that wouldn't remove skin and a textured top layer that we stuck surgical tape and tegaderm on.  The white layer is surgical tape; it was the adhesive layer that held everything in place.  On top of the surgical tape was a layer of tegaderm which is like a sticky saran wrap to make it harder for Jack to pull anything free.  He's got his orange NG feeding tube in his right nostril.  The small black and white cords are attached to his chest.  The thick white cord is attached to the oxygen monitor on his big toe.

Here's how I'd bathe Jack:

Pre-bath prep:

1. Turn off monitor.
2. Remove taped layers from Jack's face.
3.  Cut the feeding tube and nasal cannula free of the layers of adhesive.
4. When Jack was on 24 hours a day of oxygen, replace the cannula using the slider on the tube to hold it in place.  If he was being bathed during a oxygen wean, hang the nasal cannula on the flow meter or throw out if it needed to be replaced.
5. If the NG tube was >14 days old, throw it out.  If under 14 days, take the NG tube over to the kitchen sink.  Use 10mL syringe to force 5-10mL of whatever pop we had on the counter through the feeding tube to dissolve any proteins that had coagulated in the tube.  Drop the tube into about 1/4 of a cup of pop to clean the outside.  
6. Take off Jack's clothing.  Remove the chest leads and stick them to the diaper wipes container.  Remove the toe lead and lay it in the box of assorted medical items. 
7. Swaddle the baby in a bath towel and lay him in his bassinet.
8. Put the baby bath on the sink and fill it.  Get soap and washcloths ready.
9. Remove Jack's diaper.  Put Jack in bath.

Bathing him was pretty standard; if he was attached to the nasal cannula, that would trail up and out of the bath tub.  I'll skip those steps and start back up with Jack laying on the dinning room table we converted into a changing/medical station wearing a diaper with a large receiving blanket or dry bath towel under him.

Post-bath work:

1) Fish the feeding tube out of the pop and rinse it in water.  Carry it back to the baby.

2) Cut a length of surgical tape about 4 inches long.  Rip in half.  Attach the pieces to the flow meter so I won't lose them.  Tear 3 one inch pieces and attach to the flow meter.

3)Use the towel or receiving blanket to straight-jacket Jack's arms.

4) Measure the depth that the feeding tube needs to be inserted by placing the tip at the nostril that it will be inserted in, run the tube to the earlobe on that side of the head, then to a point halfway between the xiphoid process (bottom of the sternum) and his belly button.  (Jack traditionally thrashed his head around during this part which made it insanely complicated.) Twist one of the pieces of surgical tape all the way around the NG tube leaving two sticky ends free.  Lay the NG tube out of the way where neither the baby nor I will get the tape stuck on us.

4) Take out a sheet of duoderm, a roll of tegaderm, and scissors.  Cut the duoderm into a barbell-shape where the thin bit fits on the skin between Jack's upper lip and nose without touching his upper lip.  Remove the backing.  Pin Jack's head in place while placing the duoderm on his face without stretching it.   (Angry growls from the baby should be ignored.)

5) Lubricate the tip of the NG tube with saliva.  Insert the NG tube by pointing Jack's chin upward and aiming the tube for the dark space in his nostril.  Quickly and smoothly push the tube inward until the tape is reached.  Attach the tape to the duoderm under his nose.  (Counter-intuitively, Jack never put up much of a fuss during the actual insertion - probably because from his point of view I wasn't messing around with his face.)

6) Check the tube for placement in the stomach by attaching a 10mL syringe and suctioning.  When it is in place, stomach fluid or formula will appear in the tube.  (Honestly, you'll know if it goes in the trachea LONG before this point.  It happened once.  Jack's face turned bright red, he started frantically waving his arms and thrashing his head.  We pulled the tube out in less than a second and he was fine.)

7) Lay the NG tube along the tegaderm on his cheek.  Use the 1 inch pieces diagonally across the tube to secure in place. 

8) Lasso the nasal cannula around his head.  Tighten the slider in the back.   

9) Cut a length of tegaderm.  Remove backing and place over the NG tube and nasal cannula on one cheek.  Cut another length.   Use to secure the nasal cannula on the far cheek.

10) Replace the toe electrode.  

11) Replace the chest electrodes.  Cut two short lengths of tegaderm and place over chest leads to secure.

12) Turn on monitor.  Redo toe electrode as needed.  

13) Dress baby.  Look at clock.  Start feeding routine.

Medical appointments:

7-10 days before:

•  Get one of my parents to come to help wrangle Jack's equipment.

Night before:

• Check his medical go-bag to see that it contains an entire NG replacement set (NG tube, duoderm, surgical tape, tegaderm, scissors, 10mL syringe), a replacement nasal cannula, a 2oz syringe for feeds and an unopened bottle water.
• Make sure the diaper bag has diapers, wipes, Vaseline, a spare outfit, a burp cloth, a pacifier and toys (once he got old enough to be interested in toys.)
• Check that the travel tank in the shoulder bag has enough oxygen for the trip.  Replace if needed.

After the last feed before the appointment:

• Put car keys and wallet in pockets.
• Mark bottles with feed times that will occur during the appointment plus one extra. Place medications in the correct bottle.  Place bottles into diaper bag (which is an insulated lunch bag.)
• Place diaper bag into the giant bag.  Bring giant bag into dinning room.
• Put baby on changing table.  
• Detach monitor from electrical outlet.  Verify that the battery is working.  Put monitor in giant bag.
• Use regulator on the travel tank to set the oxygen flow to 0.5L. Detach nasal cannula from oxygen concetrator.  Attach to travel tank.  Place travel tank in giant bag.
• One adult picks up baby.  Second adult secures cords onto the first adult (so that the first adult doesn't trip on them) and picks up giant bag.  Walk out of the house, down the steps and to the car.
• Put the giant bag on the floor behind the car seat.  Secure Jack.  One adult drives; other adult sits in back with Jack and is in charge with dealing with the monitor if it goes off during the trip.

Upon arrival at the doctor's office:

• Adult who is not driving removes stroller from the trunk, arranges the oxygen tank, monitor and diaper bag into storage under the stroller, removes Jack from the car seat, and secures him in the stroller.  Driver watches for anyone who might not see the adult running around the outside of the car and parks the car after Jack's brought inside.

The actual appointments are pretty standard. 

After the appointment, all of the previous steps are reversed.  Tube-feeding in the car was the same as tube-feeding at home except that the adult had to hold the 2oz syringe over Jack's head to allow gravity to feed him so you have really tired arms at the end.

Therapist Visits:
Ok - truth in blogging time.  I like home-visits by therapists.  I get time to talk with an adult besides my parents or spouse plus the therapist is someone who was totally used to babies attached to medical equipment so I didn't have to explain anything to them.  Since I did home-bound tutoring when I taught, I know that therapists aren't mentally judging my housekeeping or organizational standards; it's not part of their job description.

The down-side: Babies can smell a therapist coming from a mile away and work at thwarting their evil aims.

• Jack behaved like his physical therapist was trying to kill him when she placed him on an exercise ball or laid him on the floor on a heap of mats and blankets.  
• He'd go completely limp and pretend that he didn't know how to support his head.  
• Jack had an entire act where he'd slump over while being held in a sitting position and cough dramatically 3-4 times.  Using his last bit of strength, he'd slowly raise his head up and bravely make eye-contact with the PT or OT before giving a weak, deathly ill baby cough.   I would burst out laughing and "verbalize" Jack's performance as "Lisa, I really want to work with you today - I really do.  But PT is making my multy-dwug wesistant tuburkuosis (multi-drug resistant tuberculosis) worse...so I just....can't.  I'm so weak...."  That would make Jack swivel his head towards me and glare at me because for siding with the PT.    

His antics were hilarious - but it's exhausting to have a baby acting like he's being killed for the better part of an hour even if you know he's fine.

Plus, no matter when we scheduled an appointment, Jack would be hungry and cranky by the end and I'd have no downtime between two feed cycles.

 For the first two months after he was discharged from the NICU, Jack averaged 0.5 doctor's appointments between his "normal" well-baby visits and specialists visits to the pulmonologist, cardiologist, neuro-developmental pediatrician, and optometrist  plus two therapy visits per week.

We were lucky.  Jack "graduated" from weekly PT at the end of the first two months and out of SLP at the end of four month into a single monthly appointment with a PT/OT early childhood specialist through Early On.  Our SLP acted as a medical liasion guru who got Jack into specialists earlier so that his care would be transitioned faster.  The optometrist found no signs of damage to his eyes so we just need a single yearly appointment until he's verbal enough to be checked at school.  His lung issues and persistent PDA didn't lead to pulmonary hypertension so his next cardiology appointment is when he's three.  We see the pulmonologist twice a year.

For kids with more substantial delays like Verity and Katie Musser or Lina, Olyvia, Rachele and Avi Carpenter, they could be entitled to three therapy visits a week each until they were enrolled in school (Speech, occupational therapy, and physical therapy) on top of well-child checks, any visits to the doctor for illnesses and quarterly/yearly appointments with medical specialists.  Additionally, if the kids are eligible for Medicare and receive specialist visits, the kids are often eligible for home care aides to help with daily hygiene function which adds an entire different layer of scheduling to the calendar.

Just writing this out is exhausting - so that's why I am extremely skeptical that families with multiple children with complicated disabilities are able to cope with a single stay-at-home mom and whatever resources they can cobble together - especially if they refuse governmental services as many CP/QF families do.....

Charlie Gard: What the Spin Misses

I don't want to write this post.  At all.  Not now, not ever.  But I think I need to.

Charlie Gard's story has ripped my heart into tiny bits.

Charlie and my son are medically mirror opposites.  My son Jack was born critically ill from extreme prematurity but has been slowly getting healthier and  healthier.  Charlie was born a big healthy term baby boy and slowly got more and more ill until he died.

Under the surface, though, the similarities are creepy.  Charlie and Jack didn't have medical conditions that could be treated.  All doctors and nurses could do was to use technology to support their bodies while the disease processes worked themselves out.

What killed me, though, was the pictures of Charlie.

I can ID all of the lines attached to his face - ventilator inflow, ventilator outflow, NG or NJ 8 or 10 French tube.  The way the teddy bears or blankets cover PICC lines or IVs.

Let me tell you what the nurses and doctors can't tell you: the technology keeping a child alive involves pain.

Ventilator tubes and nasogatric tubes rub at the nasal passage, mouth, and throat.  Doctors expect to see irritation similar to an active cold when examining a intubated child.  This is the reason children who have needed breathing support or nutritional support are at high risk of developing oral aversions; they've learned that having objects in their mouth causes pain, not pleasure.  We've been lucky because Jack would suck on his ventilator and oral-gastric tubes like a pacifier and has no signs of an oral aversion.

Ventilator tubes move slightly in the trachea while they work.  I don't know how well Charlie's tubes fit his trachea; Jack's was a horrible fit.  His ventilator leaked all the time which is a fancy way of saying that air escaped up his trachea instead of leaving through the tubes.  This increased the amount of gas in his stomach giving him gas pains most of the time.  The doctors had some options, but none of the options were great.  A larger tube would probably rub against his trachea more and make his thoat more sore; it also increased the risk of scar tissue forming that would require surgery to replace his trachea before he could get off a ventilator.

Which reminds me of the third ventilator problem: mucus.  Humans produce a lot of mucus in their lungs to protect against bacteria and fungal infections.  When a person is healthy enough to cough forcefully on their own, the mucus is expelled into the pharynx and swallowed.  Ventilated babies don't cough so the mucus can build up and block the ventilator tube.   Every few hours, Jack's nurse would have to suction his lungs and mouth to remove mucus.  Once he was old enough, he cried because it hurt.  Before he could cry, his blood oxygen would tank and his heart would race.

Twice, my son managed to move his ventilator tube.  If the tube isn't in the right spot, the ventilator can't inflate the lungs and blocks the airway instead.  Jack had normal muscle tone so we had to keep him swaddled into a ball with his head mostly immobilized so that he didn't thrash himself free of the ventilator.   I don't think Charlie had that problem; his limbs are too limp.  In fact, I only saw Jack that limp once - the first time he moved his ventilator tube which caused him to turn maroon, purple, blue-purple then gray as he passed out from lack of oxygen.

We were lucky in one respect- Jack didn't need many IV's or PICC lines.  The problem with any line that goes through the skin is that it increases the chances of an infection exponentially.  Jack's one PICC line lasted less than a week before it got infected.  The doctors removed the line and gave him a week's course of antibiotics.  Going through a course of antibiotics is never fun; Jack was a cranky baby.. He needed four blood transfusions which took a good sized IV in his leg.

They never mention the game of electrode roulette.  Jack's oxygen sensor on his foot always burnt little blisters into his toes. We change the position daily and try to find less sensitive areas but all we've really ended up doing is giving him several deep calluses.  As he moves more, he's ripped the skin around his toe with the sensor on it when he kicked with enough force to make the edge of the sensor act like a knife blade.  He cries.  I cry, too, because I can't stop it from happening.

All of these things involve tape on infant skin.  There are lots of ways to try and minimize the damage to the skin - but removing the tape always irritates my son's skin.  On the other hand, leaving the tape in one place increases the risk of developing an allergic reaction and an itchy rash.  So far, his face tape seems to be in the sweet spot of not ripping his skin and not having an allergic reaction.  Too bad he's allergic to his chest electrodes.  

Jack and Charlie both had their bodies growing while kept in unnatural positions.  Jack spent 3 months exposed to gravity and able to fully extend his muscles that he was supposed to be smushed into a ball.  Charlie hasn't been able to contract his skeletal muscles to move and put tension on his bones like a 3-11 month old baby is supposed to do.  There are very talented physical therapists who worked with Jack - and I assumed worked with Charlie - to mitigate the effects, but there's only so much a PT can do with a kid on a ventilator.

I can talk about the medical issues all day.

What I can't describe is the level of denial a parent needs to survive.  I knew about all of these issues while Jack was in the NICU; I also refused to think about them.  I couldn't.  Jack's chance of survival were around 90% - but the doctors and nurses had to do painful things to keep him alive.  If I let myself feel - really feel - the sadness, fear and helplessness that were always lapping at the edges of my consciousness, I'd collapse.    I'd follow my instinct to grab my son and run away from the NICU.  I'd stay at home instead of holding him for 2-4 hours a day while those damn alarms kept going off.

I couldn't accept that my son's lungs were severely damaged from his birth for the first three months in the NICU.  Just plain couldn't deal with that idea.  I heard what I wanted to hear - he was a difficult case, he was showing improvement, he did things on his own timeline.   I somehow managed to not hear that being on oxygen after 36 weeks gestation = severe BPD.   I was willing to have a long NICU slog - but the thought of a ongoing medically complicated baby with oxygen and feeding tubes at home was a place I could not go.  So....I didn't.

I nursed that dream as long as I could.  I think I made it to about 38 weeks before I had to admit what was really clear; the doctors and nurses were figuring out how to send Jack home on oxygen.

My denial was over a fairly simple medical issue - Jack's on the most simple style of oxygen support and needed a NG feeding tube to give him calories when he was too exhausted from the work of breathing to eat enough.

How much worse is coming to terms with the fact that Charlie was dying?  That his body was starving at the cellular level?  That his body was sacrificing high energy demand tissues that weren't critical for life - like his skeletal muscles and eventually his brain - in a desperate attempt to keep his heart, lungs and digestive system going?

And then - a ray of hope appeared.  A doctor has a possible treatment for mitochondrial disease.

How could his parents rationally and dispassionately assess the claims of the doctor?  I'm not Charlie's parents, but I suspect the words "possible treatment" drowned out any other words - and I can't blame them for that.  In an emergency, any possible salvation will do.

Charlie's doctors, though, could assess the treatment and its risks.

The first red flag was that the doctor did not have an open clinical trial running on the treatment.  I do human research on educational topics.  To do any human research, scientists have to show that the subjects of the research can be informed of the benefits and risks of the trial.  Here's the problem:  I can't imagine that a research protection committee would have allowed a consent form for Charlie's parents that had any potential benefits to Charlie listed.  Charlie was more physically depleted and had a more severe form of disease than any of the previous patients.  If Charlie's parents believed there was a benefit to Charlie from the experimental treatment, informed consent could not be given because the parents had an unreasonable expectation of benefit to Charlie.

The second red flag is the term "clinical improvement" instead of "therapeutic improvement".   Clinical improvement is worthless from the standpoint of patient well-being; it means that a test shows either a slowed progression of the disease or an improvement of a single marker.  Therapeutic improvement means the patient is improving medically.

Let me give an example: My first blood test when I was diagnosed with HELLP showed I had a platelet count of 44,000 when a normal range is between 400,000 to 120,0000.  If my platelets increased to 48,000, I would have a clinical improvement because a test showed that my platelet count went up.  That increase would not be a therapeutic improvement because I would still be at too high of a bleeding risk for low risk anesthesia during surgery.    When my platelets went up to 100,000 before surgery, I had a clinical and therapeutic improvement; I could receive epidural anesthetic which was safer for me and my son than general anesthetic.

According to the doctor who offered the experimental treatment, Charlie had between a 10-56% chance of a clinical improvement.  That's underwhelming to start with.  Add in the potential increased discomfort and increased risk of a chaotic death during cross Atlantic transfer of a comatose, severely compromised infant - and the argument for the experimental treatment being attempted falls apart.

Babies like Charlie Gard are born and die every day in every community.  The only difference is that Charlie's parents stayed in a state of denial longer than other parents of dying infants and children generally do.  

No one is served by pretending that doctors, nurses, the UK justice system and the EU Court of Human Rights are evil while Charlie's parents are saints.

The truth is more simple and more sad.  Charlie's parents loved their son and didn't want him to die.  Charlie's medical professionals loved their patient and didn't want him to be in pain while his body broke down.   People can love the same person deeply and disagree on what the kindest action is.

To my first-born son on the day we expected you to be born

Dear Jack,

You are thirteen and a half weeks old - and yet today is the day we expected to meet you.

I didn't have a birth plan.  I didn't know if I wanted skin-to-skin, delayed cord clamping, or rooming in.  I hadn't packed a bag for the hospital and we hadn't picked out an outfit to bring you home in.  I thought I wanted to breast-feed, but our class was scheduled for early February.  I was actively looking for a sardonic stuffed animal to bring with me to the breast-feeding class; I had ordered a stuffed methicillin-resistant S. aureus (MRSA) toy which was awesome, but too small for the class.

None of that stuff mattered, thankfully.

I had been waiting my whole life to have a baby - and I was so thrilled to have you.  I cried when I got a positive pregnancy test.  Seeing you wave your arms and legs around at the 12 week ultrasound blew my mind.  Your dad and I joked for weeks that you were saying "Look!  I have fingers!  Look at my fingers!  Ooh!  Legs!"  I breathed a sigh of relief at the 20-week ultrasound when your heart, spine, and brain looked good; I worry too much since your Uncle David died so young. Watching the technician visualize the blood flow to your kidneys and your umbilical cord left me gasping with delight.  You were so perfect - and completely you.

And then - all of a sudden - you had to be born far too early to save us both.

I spent the night before your birth in frantic prayer.  I wanted you to live.  Live and be happy.  Please, let him live and be happy.  I'll do anything.  Please.  I don't need my organs to work perfectly after he's born; damage me before hurting him. Please.  He's so small - I'm grown so let me take the damage.  Please.

When Dr. Erinn was delivering you, I could hear her talking with someone.  Apparently, you were up to some tricks.  The delivery team couldn't get ahold of you to keep you in place while she opened the uterus.  Well, and then you tried to stay put by shoving a foot into a Fallopian tube.  You are clearly my son and the son of your Dad.

I didn't hear your actual birth; I was throwing up rather loudly.  Eventually, I started looking around to see if you were born.  I heard Dr. Haines say to Dad "You want to trim the cord, Dad?"  I let out a breath that I had been holding since the night before; you had to be doing well if the NICU team could let Nico trim your cord.

I stared at the drapes and then threw up again. (Really, vomiting while having an epidural in place isn't too bad.  That was a nice surprise.  Bet you love that bit of information :-P )

All of a sudden, a voice says "Melinda. take a look at your son."  I turn my head towards the voice and there you were.   No one had told me that I might be able to see you in the OR.  You were perfect - and crying!  Dr. Prentice had told us yesterday not to worry when we didn't hear you cry - you were young, the cries are really quiet and most preemies don't cry.   You were crying!  The hat for the CPAP mask covered most of your face so all I could see was your chin and cheeks - but you were perfect and making the most heart-breaking and adorable "wah, wah, wah" sounds.

I told you what was in my heart: I love you so so much.  I'm so glad you are safe.  You are totally worth it.  The disembodied voice - I still have no idea who it was - told me I could kiss you before you went to the NICU.   I felt like a kid on Christmas; I got to see you and kiss you!?!?  I gave you a little kiss on the chin.

Later that day, I got to see you in your isolette.  I wanted to see you but was scared, exhausted and hormonal which made me feel crazy.  Your dad brought me down in a wheelchair with a nurse in tow since I was still on one-to-one nurse coverage.

I wanted you to live and thrive and grow.  I knew that you might not make it - 26 weeks gestation is so little - but I hoped to see a sign that you were here to stay.  I wanted you to know how much I loved you, how much your grandparents, aunts, uncles and cousins loved you.  I wanted you to know how amazing the world was - flying kites, learning to swim, petting kittens, watching fireflies on a summer night - in hopes that you would be able to stay here.

I wanted to give you hope and strength, but you were the one who gave me strength.

I saw teeny, tiny, itsy-bitsy you in the isolette.  Dr. Prentice had told us not to worry if you didn't move much since you weren't used to moving against gravity and you needed to save your strength.  Apparently, she forgot to tell you that piece of information.  You were waving your arms in the air.  You were grabbing the cords to your heart monitor with your toes - as smoothly as if your toes were fingers!  Your tiny hands were exploring the new textures.  You grabbed your CPAP mask and clutched a piece of it for a few heartbeats then tapped your fingers along a different piece of plastic.  All of a sudden you reached your eye-protection which was a strip of flannel.  You paused for just a second, then tapped your fingers along the flannel discovering a new texture.

In that moment, I had the hope I needed.  You were exploring this world with gusto - so however long you were with us you would experience life to the fullest.  That's all I wanted for you.

You've grown into a strapping, healthy baby now.  We tell people that you are a few days old - which is true in a semantic sense - and watch their eyes pop at a 8 pound baby who can hold his head up for a few seconds while we hold you in a sitting up position and focuses on objects.  (A nurse kindly - but firmly - asked us to stop doing that to people in the NICU.  It was messing with parents of actual newborns who thought their newborns were slow.  We must have forgotten to give the punch line of "He's 3 days old but was born 14 weeks ago.  Oops.....)

I love you, little man.

Mom

PS.  Yes, I know I finished this a few weeks later. What can I say?  You were so cute I got distracted.