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.

Update from the Disappearing Blogger :-)

Enjoying finally looking pregnant during the first week of November

Looking at my old post dates, I'm having a hard time believing that I haven't posted since early November which seems like yesterday....but a lifetime ago, too.

I guess I should just jump in.  Figuring out where to start is the hard part, though.

On November 18, 2017, my husband's family had a small party for Thanksgiving.  I had a blast.  Since the food at holiday parties is richer than I'm used to and I was 25 weeks pregnant, I was annoyed, but not surprised that I started having gas pains after the meal.  That night was miserable, but I adjusted my diet, got more exercise, and took some pregnancy safe anti-gas medications.  I was still much more gassy than usual, but that's pregnancy for you.


On November 23, 2017, I was working on a blog post when I smelled rust.  While I was trying to figure out what the hell was going on, blood started gushing out of my nose.  I ran to the bathroom, grabbed a ton of toilet paper and applied tons of pressure.  The bleeding stopped within a few minutes.  Honestly, it took longer to clean up the blood on my face, hands, shirt and the drips I lost control of when rushing through the house than it did to stop the bleeding.    I told my mom about the bleeding - it reminded me of the one time I broke my nose - and she asked if I had had elevated blood pressure at all during the pregnancy.   I hadn't had any elevated blood pressure.  In fact, my blood pressure had been 110/70 at my last OB check up about 10 days before.

Thanksgiving came!  My mom and brother were going to meet my husband and I for dinner at a local Chinese restaurant.  Nico had to work on the farm so it was just Mom, Mike and I for dinner.  The restaurant was closed so we went to IHOP.  I haven't been there in years and was thrilled to have Red Velvet Pancakes.  My stomach was acting up a bit from time to time, but a heavy starch sounded amazing.  My super-kind mom left a huge tip for the waitress since the restaurant was slow.  The waitress was touched and we all chatted for a bit.  I made some kind of joke about the fact it was completely dark at 6pm at night; did I mention winters in Michigan suck?

That night, my stomach was still gassy, but around midnight I started having sharp pains by my belly button.  The pain was keeping me from sleeping.  I wondered if I was having Braxton-Hicks contractions, but it didn't feel like my entire uterus was tightening, just the uppermost part.  I spent an hour trying various remedies that my OB had sent home with me - walk, rest, drink water- and realized around 2am that I was still awake, miserable, and - oh, yeah - the pains seem to have a rhythm to them.   I dug out the emergency number for my OB's practice and called.  One of her partners strongly recommended that I go to the hospital to get checked out since premature labor needs rapid treatment.

I went to the hospital.  I wasn't having contractions - just some disordered Braxton-Hicks.

The nurses took my blood pressure.  It was 206/115.  I had developed severe pre-ecclampsia in 10 days.  

I was attached to an IV, had blood drawn, the works.  The nurses had a hard time getting my blood pressure to drop;  turns out I inherited paternal family's ability to ignore beta-blockers.   Eventually, my blood pressure went down to "higher than ideal, but not dangerous" and I was sent up to L and D for a few hours of monitoring and then they would send me home.

That was the plan, anyways.  There was one piece of information missing: my blood work hadn't come back.  When it did, everything changed.

The blood work showed that my liver enzymes were extremely high while my platelets were dangerously low.  Not only did I have severe pre-ecclampsia, but I had developed an extremely rare syndrome that can occur with pre-ecclampsia known as HELLP.   My liver was becoming extremely overactive.  This causes it to start digesting itself while destroying red blood cells and platelets.

I knew what HELLP was.  HELLP kills women when left untreated from seizures, strokes and uncontrolled bleeding from liver ruptures.  HELLP kills babies by having the placenta detach before birth and leads to torrential bleeding.

I had walked into the hospital critically ill. If I hadn't gone to the hospital, Jack and I would have been dead in hours to days.

There is one cure for help: immediate delivery of the baby.  The problem was that I was 26 weeks, 1 day pregnant.

The doctors and nurses worked a miracle.  They were able to stabilize me long enough that I received two doses of betamethasone to speed my son's lung, skin and intestine development.  I delivered Jack by emergency CS 28 hours after I got the first dose of betamethasone.  That's 32 hours after I called my doctor or 40 hours after we left IHOP.

Jack's baptism at 4 days old. He weighs 1 pound. 5oz.

Jack weighed 1 pound 12 oz and was a whopping 12 inches long.  The neonatologist held Jack next to me for a few minutes so that I could give him a kiss and tell him how much I loved him, how glad I was that he was safe and that he was totally worth all of the pain of the last 28 hours.

I can't really describe the last 11 weeks.  Maybe someday, but not now.

Jack's done well.  His lungs were still very, very underdeveloped.  He spent a month on a ventilator followed by two weeks on NIPPV and three weeks on CPAP.

The pictures are easier to focus on and I do like showing off my handsome son.

Holding Jack for the first time.  He's a week old and weighed about 1.5 pounds. That was the day after I went home from the hospital.

The smaller the baby, the more people needed for basic care.  Notice the arm waving free in spite of my husband and my best efforts.......

"Feisty" is good; Having a 2 pound baby who tries to lift his head and pull his ventilator tube out....not so good.  Hence the hand I have clamped on

Doing "containment" to help Jack feel secure.  He always likes having hands on his feet because he could kick at them.

Jack's around 3 weeks old or 29 weeks gestation in the picture.  He's on a ventilator still.  

Jack's first Christmas as Santa's newest elf.

Jack dressed as a vampire in honor of his fourth blood transfusion.

Jack's sharing some bonding time with my husband.

Jack's on NIPPV or CPAP in this pic.  He started regulating his body temp at a very young age so he'd overheat during skin-to-skin if we put a real blanket on him.  Hence the bandanna.

The most "Jackian" photo we have so far!  Dude does not like having his arms contained and will break free of any swaddle known to man...

Actually, this might be the most "Jackian".  Notice the hand shoved free of the swaddle and a leg held at a 45 degree angle.  When your kid is attached to monitors, you can use a weighted beanbag to make him feel safe....and keep his pacifier in place.

You really do get used to all the tubes and cords attached to your kid.  When I look at this picture, what I remember isn't the medical things but starting to cry when I came in Jack's nursery because Jack was in a real crib.

We dressed him for the first time at 35 weeks gestation (or 9 weeks after he was born).  It took me 20 minutes mainly because I'd never done a kimono style onesie before

When Jack was switched to a nasal cannula for oxygen, I felt like we had a changeling baby; I had gotten so used to the hat and mask for the NIPPV and CPAP that seeing his whole face and head was startling and wonderful.

Reading a book aloud on sustainability for my Master's.  The neonatologists were always telling us how good it was that we read to Jack; I've never had the heart to tell them that I read aloud because staring at a monitor while Jack was asleep was very, very boring.

Being able to pick up and hold my son without needing medical backup has been amazing!  Holding him when he was on a ventilator required at least 1 respiratory therapist and two nurses to safely transfer him to and from the isolette.  When he switched to NIPPV/CPAP, we only needed one nurse.  Now, I can pick him up whenever we want to or whenever he needs some cuddles.

Jack's Mafia enforcer face  :-)

"No, really, Dad.  The nurses said I can stay up all night.  Don't you believe me?"  I think we'll be seeing that face a lot :-P

Jack's sleeping in his dinosaur position in his dino jammies!