Life with One (1) Medically Complicated Infant: An easy day.

Being a bit over a year into my son's wild and crazy life, his medical needs have dropped down to "baby who needs asthma-control medication and judicious choice of outings during cold and flu season".  IOW, he's so close to being a normal 9 month (adjusted) baby that I feel like I can breathe.

I'd breathe more easily if I hadn't gone back to child hoarding adoption blogs. 

See, I have no idea how many of these families are even pretending to function.   My single medically complex son pushed my skills at organizing, sticking to a schedule, staying calm, ad-libbing, communicating and not losing my mind to the brink.

I didn't home school.  I didn't work outside the home.  I didn't do more than a handful of chores a week.  I didn't cook meals from scratch.  I relied heavily on my husband, my parents and a few family friends to keep our lives running well enough that we could keep my son healthy and growing.

I don't think people get a clear view of what a medically complicated kid looks like from those blogs - so I'm going to outline what I remember doing at various time intervals.

I also feel compelled to point out that my son was much easier than many medically complicated kids.

• The doctors know exactly what is wrong with him.  
• I live 30-60 minutes away from a city with a level 4 NICU (that's the kind that is attached to a pediatrics hospital that can do all sorts of tricky surgeries), a top rated children's hospital that has an out-patient clinic where my son is being monitored by a series of pediatric specialists, and a family doctor who thinks she's the luckiest doctor in the world to get to have my son as a patient.   
• My son weighed between 8-16 pounds so I could move him without assistance and without being likely to injure myself.
• My son is a super-good, mellow baby.  His personality is as easy-going as they come.
• My husband and I learned how to use Jack's oxygen (O2) and feeding tube (NG) easily.
• I'm really good at formulaic paperwork and I find playing phone-tag with insurance companies vaguely enjoyable.   
• My husband makes enough money that we could simply buy a lot of odds and ends that has made our lives more simple while he was working half-time.  
• Most importantly, my parents were on-board and ready to go with helping out with Jack.  

Here we go.  I'm going to start with an "easy" day.  This is any day that does not include a bath, having to redo taping, out-of-the house visits or any in-home therapist visits.

Average Day Schedule with a newborn Jack:  (Jack had issues with severe choking during reflux episodes not associated with feeding so an adult who could do infant CPR had to be with him at all times.)

9am: Wake up.  Get dressed for day. Stretch legs.   Eat breakfast while getting an overview of Nico's time with Jack.

9:30am:  Take over Jack's feeding so Nico can get ready for work.   Say goodbye to Nico when he leaves around 10am.

10am: Get Jack settled into his Rock'N'Play.   Throw in a load of laundry. Tackle one of the weekly duties.   Nap/ down time for me if possible.

11am:  Change over laundry.  Fold yesterday's load.  Give him his dose of Zanac at least 30 minutes before noon.

Noon: Begin a feed with Jack.
Feed routine:
1) Change Jack's diaper (and clothes if needed).  He's got wires on the toe of one foot, two chest leads, one oxygen cannula and a feeding tube.    I always felt like I was trying to put a diaper on an octopus.
2) Put Jack in safe location while I get his bottle out of the fridge.
3) Put warm water in his bottle warmer.   Put bottle in warmer.
4)Look up what medication/vitamin he needs at this feed.  Measure medication and add to formula.
5) Assemble the Dr. Brown's bottle system.
6) Stack pillows on the couch so that I can feed Jack in a left-side lying position without too much discomfort for me.
7) Feed Jack stopping to burp after each ounce.  Each choke, gag or reflux flinch is "one strike".  After three strikes, we need to stop the oral feed and switch to his feeding tube so that he doesn't learn to associate feeding with pain.
8) Finish by feeding tube.

• Put baby in rock'n'play or his crib.
• Get a 10ml syringe, 2 oz syringe, and a length of string.
• Tie the 2oz syringe to the string.  Tie the string to a piece of furniture that will hold the syringe at a constant height of between 6-12 inches above Jack's head.
• Pull the feeding tube out of Jack's onesie. (This was often the hardest part; I swear that kid wrapped the tube around his legs on purpose.
• Attach the 10mL syringe to the feeding tube. Draw out any air that's accumulated in his stomach.  Detach the syringe from the tube and blow the air out.  Repeat until the syringe shows stomach fluid or formula.
• Attach the 2oz syringe to the feeding tube.  
• Hang the 2oz syringe from the string.
• Fill the syringe with 2oz of formula.
• If the tube isn't flowing, use the plunger to push a few milliliters of formula through the tube.  
• Repeat the last two steps until he's finished the bottle.
• Detach the 2oz syringe from the feeding tube.
• Use the 10mL syringe to draw out air as above.
• Cap the feeding tube.

9) Wash up all of the items used during this section.

Feeding Jack takes between 1-2 hours.  Jack's usually pretty tired by the end and often will fall asleep.

1pm: One or both of my parents would come over.  They would finish Jack's feed while I caught an hour to two hour nap.  I'd need to add medication to his 3pm feed if they were staying before I fell asleep.

3pm: Wake up from nap.  If my parents are staying, I do an out of the house errand or exercise.  If not, Jack's ready for his next feed which will last until between 4-5pm.

4pm: Nico comes home.  He goes to sleep for two hours.

5pm: Eat lunch if I haven't done it already.  

6pm: Nico wakes up in time to take over for Jack's 6pm feed.  I go to sleep in the bedroom until 11:30pm.  

9pm: Nico feeds Jack.  He also gives Jack his nebulizer treatment which Jack has strong feelings about.  He catches a nap on the couch where he can hear Jack's alarms.

11:30pm: I wake up and take over Jack for the night shift.  Give Jack Zanac right away in his feeding tube since it is most effective on an empty stomach.

Midnight: Jack never wakes up for this feed - ever.  Since infants aren't really active participants in physical therapy, I do his PT on him after I change his diaper, skip the oral feeding portion and move right to feeding him through the tube.  It takes about 30 minutes to feed by tube only and 15 minutes to change his diaper and do PT when he's asleep.

1am: Work on blog post if I'm not sleepy.  Nap on the couch near Jack's bassinet  where I can hear and respond to his alarms if he chokes if I'm sleepy.

3am: Question all of my life choices; I'm not a night owl.  Play a bit with my wide-awake baby, remember that this isn't going to help him differentiate day and night, and get down to feeding the baby.  

Between 4-5am: Finish feeding Jack.  Fall asleep on couch.

5:30am: Nico wakes up and starts making coffee.  I wake up, mumble "I love you" and go into the bedroom where I can sleep more deeply until 9am.

That was an easy day with one kid and three-four caregivers.  The next post will be on the repeating issues that cause days to go from "easy" to draining.

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.