Chapter 9: Baby's First Surgery

A baby's first few months of life should be filled with many exciting firsts that proud new mothers eagerly document in their baby books.  First bath! First solid food! First trip to church! My babies memorable firsts looked a bit different... First ambulance trip! First IV placed! And... First Surgery! Not nearly as fun to record for the baby books but no less significant of milestones in their lives. 

My C section was my first ever time to have surgery.  My mom had her first surgery at 56 years old.  My dad had a knee surgery long before I was born.  My brother had tubes put in his ears but I would not remember that.  Surgery was anything but a commonplace occurrence in our lives.   Other than the hours I devoted to watching Grey’s Anatomy, surgery was about as far removed from my life as possible.  I still find it hard to believe that in just 2 short years, there have now been 27 times that one of my children have gone under general for a surgery or procedure.   It is no longer a rarity but unfortunately a pretty frequent part of my life. I’d love to say therefore that it has gotten so much easier and that it’s no longer stressful or emotional.  But, there is still something terrifying about leaving your child in the OR and I still hate surgery days more than any other days. 

She was 5 weeks old when Addie had her first surgery.  It was a small surgery, just having a Broviac or central line placed.  Which of course did not sound small to me when I understood that someone was going into my tiny perfect little girl’s chest and threading a piece of plastic into the main artery near her heart.  It sounded downright horrific and I was petrified and saddened by the whole process.  I suppose though that living in a NICU surrounded by sick babies does if nothing else, provide helpful perspective when needed.  So while I grieved that my sweet girl was going to have this surgery done, I also knew there were babies in her room who had endured open heart surgery within a week of being alive. And they were okay and their parents were okay.  So I knew God could protect Addie and that she could be just fine.  I knew there were worst things to have to endure and I took whatever solace I could in this as they wheeled her away in the tiny portable incubator to receive her first of many scars of honor. I waited and I worried and I prayed and I preoccupied myself with her adorable brother anxiously awaiting her return.

And I remember her return like a photograph, I remember her wearing a purple onesie and my marveling that the IV that had been so prominent on her tiny body was now gone.  She was now receiving medication through a small line hidden under the onesie.  It almost seemed better.  And while I had prepared myself for her to be asleep or in some sort of pain, it was to my tremendous joy that within an hour of her return, she was happily snuggled in my lap, drinking her bottle with a ferocious appetite and oblivious that anything significant had occurred in her life that day.  And relief washed over me.  We had just survived our first surgery day!  And it had not been horrible.  And my little girl came back as precious as I sent her.  And suddenly when I thought of the picture Dr Doom and Gloom had painted, of many surgeries to come, suddenly it actually seemed like maybe it wouldn’t be as bad as I had feared.  In that moment holding her in her purple onesie feeding her, I stopped feeling quite as scared.

Conquering my fear of surgeries was, however, short lived.  If I could give some advice to me two years ago, among the morsels of advice I’d hand to her would be to understand that every surgery is its own risk, its own experience and its own fear.  Do not fear some because they seem bigger and do not become complacent and stop praying and preparing through some because they seem small.  Just stay calm, stay busy, and stay prayerful and meet each surgery experience square on as if it was the first and only one.   But this was 26 surgeries ago and such wisdom perhaps only comes from experience.  Because after facing Addie’s surgery with sheer dread and trepidation only to find it far less traumatic than I had feared, I now developed a very relaxed approach to Max’s upcoming turn.  I was now a central line placement expert and it was going to be just fine.  No biggie.

Except well it did not exactly turn out that way.  Max’s surgery was delayed by a couple of weeks because he had been having some difficulties breathing and was still requiring CPAP a machine that went over his nose to give his breaths some extra force.  But replacing peripheral IVs in his hands and feet almost daily was becoming problematic so it was time for him to get his central line.  A swallowed a lump as he sped past me in his mobile isolette and then waited patiently for him to return and come sit on my lap and stare up at me and be as happy as Addie had been.  As the hours passed and he had not returned, I became increasingly less confident and more anxious.  We got word that his blood pressure had dropped and he had required fluid and things had not gone as smoothly.  As a result they were going to leave the breathing tube down his throat and bring him back sedated and intubated.  No sitting in my lap drinking a bottle.  No cute onesie covering up the new plastic in his chest.  He came back looking very out of it and sad and suddenly the things I had feared two weeks ago sending Addie off were there right before my eyes.  And surgery seemed like a really big deal again.  And years of many surgeries seemed heartbreaking and insurmountable.

It would be six long days before Max would come off the ventilator and start to seem like himself again.  And he would get worse before he got better.  Within 48 hours of his surgery, he started running a high fever and seeming quite sick.  His blood work indicated his blood pH had dropped and his urine output dropped and his blood pressure was unstable.  The nurse had to call and tell us that Max had developed a urinary tract infection that had spread to his bloodstream and he was now septic and in danger.  It was definitely the sickest we had seen either of them.  We spent days sitting by his bedside unable to touch him or hold him, reading him stories, holding back tears and praying he would get better soon.  He finally seemed to turn the corner.  One doctor told us that if he had not received a dose of immunoglobins IVIG the day after his surgery, we would have lost him.  Statements like that would just take our breath away.  We were literally living in a life or death world, daily.  And we had no choice but to just be grateful every single time life prevailed and a crisis passed.

These two experiences were our introduction to the world of surgeries.  Sometimes it goes so smoothly you wonder what on earth you ever worried about.  Other times you spend days wondering if you will even survive it.  Looking at it now, their first surgeries, their line placements, were actually perfect foreshadowing of their transplants down the road.  Addie would have to go first and we would worry ourselves sick.  And she’d bounce right back and completely shock us and make us laugh that we were ever so concerned.  And then Max would follow, and we would cling to Addie’s miracle for hope, and once again, he would have to fight for his life afterwards and leave us wondering how we would ever make it through.  

Chapter 8: Dr Doom and Gloom

There seems to be at times this irrational fear of denial among some doctors.  We have dealt with enough doctors now to know they are as different and unique in their personalities and approaches as any other group of people.  There are the cheerleader 'Be positive!' doctors, the funny 'humor is the best medicine!' doctors, the soft spoken 'we'll let you know only what you really need to know' doctors, the arrogant talk over your head using big words doctors, the compassionate put themselves in your shoes doctors and then there are the serious, 'I really need you to understand how serious this is' doctors.   It is the last group that I have always struggled with the most.   They seem very concerned that we have a full depressing understanding of all the daunting things our future has in store.  Any false optimism or perceived cheerfulness in light of our circumstances seems to send off a red flag that it is time to sit us down and force that worrisome denial right out of us.  I would come to feel that almost all neurologists belong to this last group and dread any meetings with them as a result.

At times I just want to explain to them that denial is in fact a coping mechanism.  Freud identified it as one of the ways we protect ourselves when we cannot deal with present realities.  Some degree of denial may be a necessity, and when they drive it out with their facts and figures and truths, we can walk away perhaps more realistic but inevitably stripped of a much needed way of coping with the unimaginable.

Our first of what would come to be many denial bursters occurred the afternoon following the ambulance ride.  By this point, we had been awake some 36 hours and had experienced seeing our daughter become sick instantly before our eyes, we had followed an ambulance at 4 in the morning and then we had been overwhelmed with this big new hospital and new procedures, new doctors and so many questions.  I remember clearly that I had on maternity leggings and an oversized purple shirt with no make up and crazy frizzy hair and in my sleep deprived emotionally wrecked state, I know I looked like a pitiful mess of a character.  This was the demeanor I had to take on for my first ‘family meeting’ that afternoon.

Sitting in the conference room was Al and I, the nephrologist (kidney doctor) at the time and a whole slew of doctors, nurses and social workers.  We had no idea who was a senior doctor and who was a medical student, there just seemed to be so many of them.  Somewhere in that room was a wonderful nurse practitioner who we would come to know and love, who would be there at the kid’s first birthday, who would come up and see them every time they were admitted long after they left the NICU and who closely follows their lives through Facebook.  We would come to see her familiar face as a source of comfort.  But that day she was just another stranger's face that looked sorry for us.  Somewhere in that room was a funny young doctor who wore chucks with his Scrubs and that we would affectionately call JD after the main character on Scrubs.  We would come to be glad for his rotations on for his friendly, relaxing and personable approach.  But that day he was just another face, no different from the many others who I have never seen again.

The meeting was conducted by the nephrologist who was on service at the time.  We would come to know 4 main nephrologists and value each of them for their unique strengths.   This particular man was on service at the time so had the misfortune of being the doctor who would have to diagnose the kids and explain to us what all that meant.  For playing this role, I would come to call him ‘Dr Doom and Gloom’ for a very long time.  Quite irrationally it always felt like because he told us what they had, he had somehow brought this tragedy upon us.  So through no fault of his own, following that meeting I would always feel quite anxious when he was on service.  It took me a long time to reach the point where I truly respected him, not just because he was very much an excellent doctor, but because he wanted us to know what we were up against and own it and face it.  His goal of banishing our denial was painful at the time, but arguably necessary in the long run.

As I sat there in my leggings with my wild hair and tired eyes, he drove home the point that we were talking about a very serious medical condition that would change the course of their entire lives.  Although we had worked out from our move to the big hospital that going home was right around the corner, truly nothing had prepared us for him telling us he expected us to be there for another 6 months to a year.  While we had seen firsthand how dangerous their condition was, the long list of possible complications from strokes to line infections to countless surgeries felt like a punch in the stomach. Suddenly dialysis became not just a scary possibility that we did not understand but an inevitable heartbreaking part of their future.  His most positive statement of the whole hour was when he said they knew of one patient who had survived to 21 with the condition.  He looked like this was supposed to bring me some sort of solace but somehow all I could do was think about what had happened to all of the others. 

I suppose the meeting can best be summed up by the following exchange:  Dr Doom and Gloom tells us that this condition will alter the rest of their lives and be something they will always deal with.  In a small shaky voice I challenge that surely once we transplant them in a couple of years, everything will be okay.  Like wearing your heart on your sleeve, I threw out there my remaining morsel of hope I was clinging to for dear life.  We’ll transplant them and everything will be okay, it was my attempt to make peace with this awful diagnosis.  And as soon as I offered up my heart’s hope to them, he replied with ‘Transplant is just trading one set of problems for another.’

And my little small fire of burning optimism against the odds burned out right then.  The goal to make me face my reality was a success and I felt drained dry of all positivity, hope and comfort.  Maybe it needed to happen in order for me to begin rebuilding a new vision and optimism rooted more firmly in reality.  Maybe I needed to truly demolish my ordinary dreams I had so clutched to.  Maybe being stripped bare is the only way you can really start over like you need to.  It was probably a necessary pain. But when I think about that day, I don’t think I will ever remember anything but the empty hurt we left with.  Robbed of our denial with nothing to put in its place.  Forever changed.

Of all the things we have been told by doctors, there are few we quote as often as the fact that transplant is trading one set of problems for another.   Having been through two transplants, I have at times scoffed at this quote and at other times, reflected on the intense wisdom contained in it.  Inherently it is true because transplant is not a magic cure all.  At the same time, I believe one of the best decisions I ever made was to ultimately reject this prophesy he laid out.

Over the coming weeks and months, as I began to accept our journey and our battle, I chose not to accept that transplant was merely an exchange of problems.  I built up a new dream whereby transplant would be my finish line.  We would keep them alive and we would give them our kidneys and we would achieve our happily ever after.  

No doubt it was the most important decision I made, to have something to hold onto.  To refuse to abandon all hope.  To choose to fight for some light at the end of the tunnel.  To insist on dreaming of a healthier future.   So while I now appreciate that he made me come to terms with dialysis and that he helped prepare me for their incredibly long hospitalization, I am also glad I chose not to be defeated by that first talk and that God helped to rebuild hope in me, a hope that was as necessary as air itself to sustain me for the years ahead.  

Chapter 7: Not in Kansas Anymore

After being blindsided that Saturday morning, I decided I needed to be more prepared for doctors coming to have talks with me.  So the next day when a nurse practitioner came to talk to me, I was very well prepared with proper clothing, a fed and snuggly baby in my arms and a serious and calm look on my face.  It was a demeanor I came to perfect and with very few exceptions, I was able to maintain through a lot of crises.   But on day two of my new reality, my cool calm and collected act was still a work in progress and I know I was shaking as I tried to sound intelligent and unafraid asking her questions about what exactly this kidney condition actually meant.

It was in that conversation that I started to really understand that dialysis might be a reality for my children, even though I still had absolutely no clue what that actually meant.  It was the first time someone mentioned a kidney transplant to me and mentioned that the babies would have to be a lot bigger for that to happen.  But most significantly it was the first time I understood that we were talking about a life threatening situation.  Because really hearing that there is a lot of protein in their urine does not really sound that terrible.  But as I sat there and listened to her, it started to sink in that this was no small foe we were up against.

The main thing I will always remember from that conversation on the day after was her telling me that the babies having been early might have saved their lives.  Her logic was that by being in the NICU, we discovered this kidney condition from bloodwork and not from symptoms or a serious infection.  She explained that some babies have their condition but it is only discovered once the baby is very sick and it is too late.  It was a very sobering thing to understand that we were talking about something that serious.  Something children died of.  I sat calmly and nodded.  And then sobbed the whole way home.  As I would do many more days in the weeks that followed.   There were days I just could not wait to get to my car so I could let down my facade of the emotionally composed mom I was using all my energy to keep up.  My car would be my little sanctuary where I allowed myself to feel and cry.  

I suppose the life threatening nature of what we were dealing with would have been more clear to me if I had gone home either of those two days and googled it.  I am no stranger to consulting Dr Google so I cannot totally explain why it was literally weeks before I would type Congenital Nephrotic Syndrome into Google.  I think I just knew it would be bad and I wasn’t ready to see it in black and white like that.  I didn’t want to believe it was something so concrete that it could be looked up.  I did not want to see words like dialysis and transplant and accept that reality.  So not only did I not google it but I forbid my parents from telling anyone the actual name of the condition so they couldn’t google it. As long as we just called it ‘a kidney condition’ and focused on the positive, all that life threatening part would just never happen.

That worked really well for 48 hours.  It was two days after the red robe day and I was very busy not thinking about kidneys and dialysis and pretending that life had not changed.  We were up at the hospital holding babies and Addie was uncharacteristically fussy.  She kept crying and the nurse was trying everything to console her and kept telling us that it was probably just gas.  I felt really unsettled about the whole thing and was worrying about her when we decided to leave for an hour to grab some dinner and then come back up for night feeds and bedtime rituals.  We were in the car coming back to the hospital when we got a terrible phone call that made no sense to me.

She started running a fever.  Quickly she got sicker.  She kept forgetting to breathe.  We had to put her on a ventilator.  She is very sick.  Her bad kidneys are removing her antibodies she needs to get better.  This is Very Serious.  She is not stable.  You may need to be at a bigger hospital with more pediatric doctors.  Get here as soon as you can.  This is a Very Big Deal.

There are a handful of images that to this day still haunt me.  One of those is walking back into that NICU where I had left Addie in a sweet pink onesie crying and moving and feisty and finding her now stripped only to a diaper, still and pale, with a tube down her throat.  It was the most horrible thing I had ever seen and I could not stop crying.  I did not understand what was going on.  I couldn’t even think clearly enough to realize I was worried she was dying.  I just felt hysterical fear.  I sat on Al’s lap and cried in his arms while we waited for what seemed like forever for the doctors to come talk to us. Those moments of raw emotion and grief became more rare as I got used to horrible images and scary twists and turns.  But that night is in my memory like a photograph, I can close my eyes and still see every detail.  And even now, knowing that everything will turn out okay, I still feel my heart rate accelerate and I still feel a lump form in my throat when I think back to that night.  Intellectually my life changed on the Red Robe Day.  From that fateful morning onward, I knew my life was different.  But emotionally the change happened two days later crying on my husband’s lap.  It became real seeing her like that. Nothing was the same as it used to be anymore and it never would be. That was the moment when I became the mom of sick babies.  I changed that night.

I also understand what the nurse practitioner was telling me when she told me that being early had saved their lives.  Because as much as I had wished Addie had been home with me in her perfect crib, I now realized that if she had gone from fussy to so sick she couldn’t breathe in a matter of hours at home, we probably would have lost her.  My empty arms those nights meant she was surrounded by nurses and doctors who were equipped with the machines needed to save her.  Everything that had seemed so sad now seemed like part of the plan to keep her alive and I had a whole new perspective on everything.

It was a crazy night.  Addie got better almost as quickly as she got sick.  The ventilator took away the concern about breathing and the IV antibiotics started to attack the Strep B infection she was battling. By the middle of the night, she seemed to have achieved at least a tenuous stability.  Once we were all breathing a faint sigh of relief, the doctors talked to us about moving to the big hospital in the city.  Up to this point, we were at the local Woodlands hospital where they were born.  It was 15 minutes from our house and while it had a good NICU, there were many things the big downtown hospital had that our smaller one just did not. There were kidney doctors there.  There were surgeons there.  There were infectious disease doctors there.  There were neurologists there.  There were 100 sick babies there.  Our small close by NICU just would not be able to provide the care Max and Addie would need.

So at 4 in the morning, Al and I followed the ambulance in his mustang to our new hospital, scared and overwhelmed. Within one day we had gone from feeling like this whole kidney thing surely wasn’t that big of a deal to seeing our daughter critically ill and realizing that driving an hour away to the big scary hospital was now part of our new very unwanted and terrifying reality.

Things were going to get better.  Quite quickly actually. We would settle into the big hospital and be so glad we were there. Addie would recover just fine and go on to have a great few months there.  Max would get help, great care and love there. But you could not have convinced us of that during the silent trip following the ambulance. Never had our beautiful ordinary dreams seemed so far away and never had our new reality seemed so unfathomably sad and frightening.

Chapter 6: The Red Robe Day

If they ever make a movie of my life, I would like to play the day we found out about their kidney condition very differently.  I would like to be calmly sitting at their bedside dressed in nice clothing, looking focused and asking intelligent questions.  I really hope they do not portray me quite like I was- as this emotional, confused, distracted crazy lady wearing only this red fleece robe and trying feabily to nurse a tiny neonate all while getting life changing news.  I really wish you could get a little warning that your life is about to turn upside down so you could prepare to look appropriate for such a pivotal moment. But of course you rarely realize you are about to be forever changed in advance.  I didn’t even realize it at the time.  It wasn’t really until a few days later that it really occurred to me that life would never be the same again after the morning of the Red Robe Day. 

One reason why I was so unprepared for this turning point was that we stumbled upon their kidney condition quite accidently.  Perhaps if there had been a build up of symptoms and we knew the search for the answer was on, I would have seen being sat down with news by the head doctor coming.  I would have known to always be wearing more than a red robe while awaiting results. 

Ironically their kidney issue was discovered while looking into Max’s respiratory and neurological issues, which would end up taking us a full year to actually diagnose.  While no answers were showing up to explain his breathing or tight muscles, some bloodwork showed there was low protein in his blood and high protein in his urine, when it should be the opposite.  The doctors got in touch with the kidney team.  The kidney team quickly recognized the problem and felt that he probably had congenital nephrotic syndrome or leaky kidneys that dump protein that should be in the blood into the pee.  These were conversations that at this phase we did not know were occurring.  The kidney team also knew this was a genetic condition so recommended they run a blood test on his twin sister.

Prior to the red robe day, I knew Max would be a while still before coming home.  Breathing did not seem to be his strength and I had already sobbed the whole way home from the hospital one day when they told me they doubted he would make it home by his original due date.  But at three weeks old, Addie was getting closer and closer to coming home.  She was just mastering eating and seemed to be champ at breathing, regulating her own temperature and being adorable.  It was likely only a week until the perfect crib with its pink bedding and fairy mobile was inhabited and my empty nighttime arms were filled with her. 

For that reason the part of the red robe day that really caused me to lose it was this incredibly unexpected and devastating news that Addie too was sick.  I will never forget sitting there awkwardly trying to pretend I was mature enough to nurse my baby while talking to the senior most doctor and trying to digest this strange news that Max had some problem with his kidneys and protein.  And then he told me Addie had it too.  And I had to give Addie to the nurse because I could not stop the tears anymore.  And if there is anything more embarrassing than sitting in a red robe breastfeeding, it would have to be crying in a red robe breastfeeding.

That day I did not really understand what I was being told.  I think he may have said the word dialysis at some point but even if I had paid attention to that I certainly did not know what dialysis was.  The idea of transplant was never mentioned.  There was just a lot of talk about losing protein and growth problems, poor immune system.  He looked sad a lot.  The only thing I really understood leaving that day was that this was not good and that my bundle of pink would not be coming home in a week.

We did what you do when your world starts to crumble, you surround yourself with loved ones and hold onto as much denial as possible.  We had lunch with my parents and tried to explain it to them.  We all agreed that losing protein did not really sound that bad.  How bad could it be?

But for all our attempts to stay positive and dismissive, we also went home that night and I cried while Al moved the crib back up to the nursery.  Our dream had vanished and we couldn’t wake up to the reminder of what we had come so close to.