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Not the Plan
Sitting in that cold, sterile NICU room, I was trying to take it all in.
The wall behind the incubator was painted a sage green. It was a calm color, oblivious to the chaos occurring a few feet away.
“That’s my sister Rebekah’s favorite color,” I remember thinking.
The floors were a tan color, forming a cold, hard surface that seemed to spread out endlessly in every direction. I blankly studied the floor as my knees threatened to buckle, wishing I could just wake up and have this bad dream be over.
This was not the plan.
I was supposed to be enjoying cozy snuggles with my new little newborn, in my own bed.
But that’s not what happened. The calm events following the births of our first two children were not repeated with our third.
After giving birth at home, gently into water, I pulled my sweet baby up out of the birth pool. He was facing away from me as I lifted him to my chest and the midwife near me spotted the defect on his lower back at the same time I did.
It was a brownish-purple area the size of a half dollar. I knew immediately that a bath wasn’t going to make that go away.
The atmosphere quickly changed from one of relief to frenzy. 911 was called for transport to the hospital, and the ambulance arrived at our house 5 minutes later.
“We think it’s Spina Bifida,” said one of our midwives, giving us a tight squeeze as we ran out the door with hastily packed hospital bags. She told us that God knew, and prayed with us on the sidewalk by the back door. The words in her prayer have been long forgotten, but being swallowed in her hug meant the world.
Time turned into sludge. Seconds ticked by, each one heavier than the last.
My 15-minute-old baby was speeding away from me in an ambulance. Please God, this has to be a dream.
We jumped into the van to burn a trail of rubber to the local children’s hospital.
“Is it a boy?” I asked the midwife in our haste.
Yes. It’s a boy.
There hadn’t even been time for an anatomy check.
Up to this point, I’d been very certain that God agreed with my ideas of what I was capable of handling – and surely he knew that we were not the type of couple that could care for a special needs child. It would be too much. He knew that. Smugly sending Him the memo to form all of our children perfectly, I expected Him to do just that. “Only healthy and whole babies, God” …with a milkshake and a side of fries.
But that’s not what He gave us. He formed my third baby with a hole in his back. One of the most severe birth defects that’s compatible with life, in fact. And that was devastating.
“Spina Bifida.” The midwife’s words echoed in our brains as the miles flew by under our tires. “Don’t Google it,” Micah told me, but it was too late.
We crossed the bridge into the city, and each truss remains etched on my mind as I tried to absorb that this was really happening…
I read the WebMD overview.
“There was no sac on his back, Micah. It’s the worst type of Spina Bifida possible.”
One of the biggest concerns on our minds that day was that our child would be crippled. His legs would not work due to nerve damage, and he would need a wheelchair to be mobile. Little did we know that only 6 days later, a much larger issue would rear its ugly head – and the team of doctors caring for him would fail to identify it.
This silent lurker, biding its time, waited until his 7th day of life to start demanding attention. Hydrocephalus is its name.
Hydrocephalus made our concerns about being “crippled” seem like a walk in the park.
Hydrocephalus has single-handedly created the need for 10 of the 11 surgeries our son has endured in his 3 years of life.
Hydrocephalus is a misunderstood monster.
Our baby spent over 9 months in my womb, growing and developing, forming a little body that looked perfect from the outside – except for that small lesion of neural tissue, poking out at the base of his spinal column – a “neural tube defect” known as Myelomeningocele Spina Bifida.
About the time I was getting a positive pregnancy test and sharing the news with family, our son’s neural tube was not forming normally. Instead of closing and staying inside his body, it remained open to the outside. This prevented the vertebrae in his lower spine from developing properly, and exposed his nerves to amniotic fluid. The fluid that protected him in the womb was also the “poison” that gradually damaged his nerves beyond repair.
Shunt or No Shunt
Thirty minutes after giving birth, I was looking down at him in a NICU incubator – unable to hold him, and uncertain of his future.
His wristband said “Boy DeJarnatt,” since he’d arrived unnamed on the ambulance, with one of our midwives at his side. We chose the name Jotham on the harried drive over, which means Jehovah is perfect.
The neurosurgeons came into Jotham’s sage green room to brief us on their plans. They said they would do surgery the following day to close the opening in his back. This would prevent infection, but wouldn’t improve the expected paralysis in his legs. They planned to suture together the flattened neural tissue to create the tube of the spinal cord, as it should have formed. Once they had a watertight cylinder of spinal cord, they would pull muscle and skin over the defect, enclosing the lesion.
And then we would wait.
Our waiting would show if he needed a ventricular shunt, they said, insisting it would be very obvious if he did.
They told us that if a shunt was needed, there would be expansion in the ventricles of his brain, and this would be visible via ultrasound.
We had no reason not to believe them. This was our first rodeo, after all.
And it was going to be a doozy.
Many kids with Spina Bifida need a tube (called a shunt) to be inserted into the brain to drain their spinal fluid (CSF) through an alternate route. In our son’s case, his body learned to make a lot more CSF than normal while he was forming inside me (since the fluid was continually flowing out the hole in his back). Closing the hole surgically meant there would be nowhere for the extra fluid to go. Some babies’ brains can compensate for this, avoiding shunt dependency, while others need the ventricular shunt to drain the fluid to another location in the body.
Little did we know what kind of wild ride was ahead of us. This first hospital stay was about to be the best education we could possibly get on how our son responds to head pressure – but it was going to be at his expense.
The neurosurgeons told us that ventricles in the brain enlarge when there is too much fluid for the brain to handle.
This can be a true statement. But what DIDN’T they tell us?
They did NOT say that it’s possible for there to be NO ventricular enlargement when there’s too much fluid for the brain to handle.
Head pressure that’s not visible on imaging (also known as a “sneaky shunt malfunction”), is a possibility that many in the neurosurgical profession don’t acknowledge. We didn’t know this at the time.
Clues of Head Pressure
It made us sick to think that our son might need a shunt, and every day that passed without one seemed like a good milestone to reach. At the time, we didn’t realize his body was actually suffering from no longer having an outlet for the extra CSF. He had an increasing number of signs and symptoms indicating a problem as the days passed. But the neurosurgical team said his symptoms were from other things, not hydrocephalus.
Five days after the closure surgery, his body gave a monstrous clue that it was not handling his CSF well at all: his surgical closure site leaked spinal fluid.
Leaks like this can occur when there’s too much internal pressure –CSF always follows the path of least resistance. However, the neurosurgical team did not connect the dots, and were instead concerned that the wound wouldn’t heal if fluid was leaking.
Later the same day, Jotham suddenly started having apnea and breath-holding spells that were so severe, he needed high-flow oxygen. The neurosurgeons insisted it couldn’t be hydrocephalus because his ventricular size was not enlarging.
When Jotham was close to 2 weeks old, he developed inspiratory stridor, which is a fancy term for “noisy breathing.” Each time he inhaled, there was a little “whoop” sound.
In this same time frame, his eyes began to move weirdly, which I later learned was nystagmus. But this was brushed off by the NICU medical team.
The neurosurgical team kept looking at his ventricle imaging saying he couldn’t have head pressure, because his ventricles weren’t getting bigger.
So they blamed these symptoms on other things.
First they said it might be lung immaturity (even though he was born full term). We trialed Jotham on doses of caffeine, which they typically give to babies that are born prematurely, but it failed to help.
Next, they decided the symptoms were probably from spinal meningitis, even though they couldn’t find evidence of it in any lab work. They gave him 14 days of hefty antibiotics “just in case” – and it was all for naught. Fourteen days later, nothing was different. The symptoms were still there.
The apnea symptoms would wax and wane, mild on some days, and very severe on others. We’ve since learned that atmospheric pressure changes have a huge impact on our son when he’s experiencing brain pressure(these barometric pressure shifts are especially intense when a weather front moves through).
A Solution & A Letdown
In the middle of the “just in case” antibiotics and the ongoing symptoms (stridor, nystagmus, and unexplained apnea spells), the neurosurgeons said our son’s ventricles had grown just a tiny bit – large enough to warrant a VP shunt.
So we agreed to the surgery, and the shunt was inserted. We prayed for the best.
At first, we were very encouraged. Our son’s apnea symptoms improved immensely! But it was short-lived. He breathed wonderfully for a few days following the surgery, but then the apnea kicked back in with a vengeance 3 days later.
(In hindsight, the shunt catheter going into the brain probably occluded very quickly. There is a high chance of blockage after any shunt surgery.
The surgery itself can create extra debris in the brain,
and it can also stir up the debris that’s naturally already inside the ventricles).
When the shunt didn’t fix things long-term, the 3rd thing the neurosurgical team chose to blame symptoms on was a structural abnormality of the airway. However, scopes and tests were performed, and his airway structures were declared normal (besides some vocal cord paralysis, which we now know was due to brain pressure).
On the days baby Jotham had the most apnea spells, he also had trouble with pacing himself while feeding. I had to pace for him, removing him from the breast every few suckles to take a breath. If he was left to feed without my intervention, he wouldn’t take breaths properly, making his oxygen saturation drop, and the alarm on the hospital monitor would blare.
No End in Sight
So there we were, sitting there with a medical team that had no idea what was occurring, and no idea what to do to fix it. We were anxious to go home with our baby, and they were also anxious to get us home – they hoped he would improve faster in our home environment, since it’s much easier to heal at home than in a hospital. His symptoms miraculously lessened enough for him to be discharged from the NICU for a few days, but then 5 days later he was readmitted to the PICU with worsening symptoms again (during a huge storm – that barometric pressure shift).
As a last resort, while saying they would probably end up recommending a tracheostomy and gastrostomy, the neurosurgeons wanted to do a very invasive brain surgery. The procedure is called a Chiari Decompression, and involves opening up a part of the brain that is very vascular (i.e., very risky). A piece of mesh is placed inside to create a larger opening for CSF to flow around the brain. We agreed to the surgery, seeing no other option. Our son was 6 weeks old at the time – the youngest infant they’d ever performed a decompression on in that small children’s hospital.
During the very hard recovery from the decompression surgery, his VP shunt developed a bubble of fluid around the outside of the valve (underneath his scalp). The surgeon was puzzled by the results of a shunt tap, so he did exploratory shunt surgery, and found a blockage inside the shunt catheter going into the brain.
Many of our baby’s symptoms resolved after that shunt revision, and it makes me wonder if the decompression surgery was even necessary.
There Are Options and Alternatives
This is just the beginning of a story that is still ongoing – so far, we’ve had a long 3 years of VP shunt “adventures” (he had another shunt malfunction when he was 6 months old, and then very severe issues when he was 2 years old).
In some ways, I wish I could go back in time and talk to the “me” that was hovering over that NICU incubator… I would like to tell myself a few things that could have influenced our son’s care for the better. Here are 3 things I wish I knew:
1. I wish I knew how much “say” we actually had in our son’s care.
As the parent, if we don’t agree with a procedure or method of care, our word carries tremendous weight. The small children’s hospital near our home at the time was NOT good at communicating this. They should have made it plain to us that we were the ultimate authority in our child’s care, but they tended to operate like we were just wallflowers in the room, unless we asked what they were doing, or requested they explain things.
Since this was the first time we’d ever had a child in the hospital, it was a steep learning curve. I didn’t know how to communicate our expectations to the medical team, and I didn’t understand the “hierarchy” of medical staff either.
Not knowing who was who, or the difference between “attending” and “resident,” I would have very detailed discussions with the doctors-in-training (residents), only to realize as they left the room that they were not the “top doc” I thought they were. Then the “attending” would come in, asking all the same questions, and I was thoroughly confused.
We’ve since developed a system of communication that meshes with the hierarchy of practitioners, and brings our questions straight to the top of the ladder:
1) When the resident visits the room first, we tell them our questions for the attending doctor (e.g. “I’d like to discuss such-and-such with the team”).
2) They go communicate those questions to the doctor that’s overseeing our child’s care, and this gets everyone on the same page a lot faster. It allows the attending doc to be pondering our questions before coming in to visit us, and they can put some forethought into treatment options.
3) I inform the nurse of my questions for the team and my goals for rounds, if I have any. This enables them to jump in and help me communicate if things go south, or if I forget a question. There have been many times the doctors have misunderstood me, or the nurse has sensed a lack of quality communication. By offering their perspective, as the only medical professional in the room that’s had hours of hands-on experience with my child, valuable strides have been made.
4) If the social worker gives quality advocacy, I keep them very tightly in the loop on what’s happening, how things are (or aren’t) progressing, and the quality of communication occurring between myself and the treatment team.
Some doctors welcome questions better than others, and when asking questions, we can learn very quickly if they’re the type to “put us in our place,” or if they will be willing to work with us as the head of our son’s care team.
During Jotham’s first few months of life, interacting with doctors created a lot of stress for us. It was our goal to be actively involved in the care choices being made, but some doctors did not appreciate that. We thank God for the doctors that actually listen and brainstorm with us to provide good care for our son.
Since those first few hospitalizations, we have learned how to advocate for our child more effectively.
We don’t assume *anyone* knows what they need to know about our child (which means lots and lots of repeating).
We are not afraid to say we need some time to think about a treatment, or request extra discussion time with the attending doctor if we need it.
The effectiveness of this “care team” working properly is almost completely dependent upon having a quality attending doc on service. I’ve been needlessly threatened multiple times by doctors that I have respectfully disagreed with. Some doctors will do anything to feel in full control of their patients – even to the child’s detriment. It’s not an easy road to walk – asking questions, researching options, and discussing treatment plans in-depth. Sometimes this means standing up for what we believe is best, contrary to what a doctor thinks. But it’s our child’s health and well-being we’re advocating for, and we get to live with the consequences of every decision that’s made, for better or for worse.
2. I wish I knew that I didn’t have to feel “stuck” with one doctor.
Doctors in other hospitals and practices may offer more experience, and completely different viewpoints (though getting there could require a complex transfer to another hospital if the child is inpatient).
When we were researching the idiosyncrasies of VP shunts, we came across a surgical procedure called the ETV/CPC that could replace the need for a ventricular shunt in children.
Our excitement to find this surgical option was quickly dashed when the pediatric neurosurgeon at the small children’s hospital said he could not do an ETV. It comes with a steep learning curve, so not every neurosurgeon offers the procedure. He said our only available option was to put a VP shunt into our son.
If I had known that we could arrange a transport to a larger nearby hospital where neurosurgeons *did* offer the ETV/CPC, I would have worked toward making that happen in a heartbeat. We were SO uncomfortable with the high failure rate of VP shunt systems (and we were right to be apprehensive, with how that’s played out for Jotham). But since we were told there were no other options, we did the best we knew how, and agreed to the VP shunt.
There’s always another doctor out there that can give a second opinion (or third, fourth, fifth, sixth, seventh…). We’ve consulted with so many neurosurgeons, it amazing how many we’ve had to go through to find one that understands our son’s needs.
3. I wish I knew that some kids with hydrocephalus can be EXTREMELY sensitive to pressure changes (both intracranial and atmospheric).
There are two types of VP shunts – fixed pressure, and programmable. VP shunts allow fluid to flow out through the tubing when the patient’s intracranial pressure (ICP) reaches a preset number.
If a person has a programmable shunt installed, there are multiple pressure settings to choose from in one valve, and the setting can be changed with a simple adjustment (using a magnet) in the neurosurgeon’s outpatient clinic.
With a fixed pressure shunt, there is only one pressure at which the valve will open, allowing fluid to flow from the brain. If the neurosurgeon makes the wrong guess when choosing a fixed pressure valve, then it takes a surgery to change the valve to a different pressure setting.
The neurosurgeon that cared for our son in the NICU thought that fixed pressure shunts were best, and that there was no added benefit to having a programmable shunt. We trusted his opinion at the time, but have since learned that there is DEFINITELY a benefit to our son having a programmable shunt. Brains are not all created equal!
Our son has had 3 different valves, and 2 of them were programmable. From our current perspective, we think that being stuck with a neurosurgeon’s “random guess” doesn’t seem very logical when a programmable option is readily available. The programmable feature has been very helpful for Jotham in the past.
The barometric pressure, or pressure of the atmosphere, played a huge role in the roller coaster of our son’s symptoms listed above. They were ebbing and flowing, and at the time we had no idea they were connected to the weather happening outside. Most neurosurgeons will deny a valid link (“I can’t prove that with physics,” is an exact quote by a neurosurgeon from our recent past). I haven’t consulted with a neurosurgeon yet that will admit to the connection between hydrocephalus symptoms increasing and the onset of adverse weather outside. Nor can I find much in the medical literature to give credence to this phenomenon. But as we all know, the medical field has its limits, and since it’s common knowledge that neurotypical people can suffer from atmospheric changes as storms roll in (all we have to do is talk to a migraine or arthritis sufferer about how they feel when it storms), it’s not much of a stretch to assume that the atmosphere’s pressure has a great influence a brain that’s already struggling to manage the fluid inside it.
Our son now has a valveless shunt, which is somewhat of an anomaly. He developed low-pressure hydrocephalus (LPH) after experiencing severe global brain damage. This type of hydro can’t be found on any of the major medical websites at the time I’m writing this.
Experience has been one of the most brutal educators. We had the “disadvantage” of not being aware of our son’s condition before he was born (though there are quite a few reasons I’m thankful for that). The crash course we’ve had on Spina Bifida and hydrocephalus has been a wild ride, and the scars run deep. But those wounds have helped us understand Jotham and his individual needs, so I’m thankful for the added knowledge.
Our personal experience has been valuable, but the help and input of others has been priceless. God has put people in our path at the just the right time and place, in all different capacities. Hospital counselors, our church family, fellow parents of children with similar challenges, our relatives, doctors with an ear to listen – all have played key roles in helping us cope, and obtaining clarity and direction.
I’ve spent many hours processing things with hospital counselors and social workers…
…multiple late nights standing over the hospital bed on the phone with other parents of special needs kids that have walked the same road.
…many detailed conversations on Facebook and via text with parents that have blazed similar paths.
…countless visits with friends and family, the atmosphere ranging from broken silence to laughter & tears.
If I could go back in time and talk to myself when I was standing over that NICU bed, questioning life to its very core, I would want to warn myself that it’s going to be hard.
I’d want to say that I would be forever changed by being privy to an ebb and flow of unfair pain and suffering.
I’d be temped to share that my heart would be ripped to shreds over and over; wrenched by the suffering I couldn’t take away from my child.
My dreams would be haunted by traumatizing events, set to a symphony of beeps and alarms from hospital machines.
I would almost lose him due to doctors not listening. His brain would be severely damaged, and his precious soul stuck inside a body that he could no longer control.
But if I’d known all of this three years ago, I may have been crushed. It was crushing enough to look at the “unknown” stretching before us, and there’s a strange mercy in only being allowed to live one moment at a time, without knowing the future.
And yet, if I hadn’t experienced all that “hard” above, I wouldn’t know that he’d be a happy little guy that adores his mommy and daddy, just like his siblings did as babies. …his joy would emerge just as strong, on the other side of obstacles. …his sweet personality would shine bright on the good days, drowning out those dark nights. And even amidst deep losses, through Jotham’s tough grit and determined spirit, God would give little bits of him back to us, piece by piece.
That cold, white floor in the sage green room was a personal nightmare come to life. And it was just the beginning.
And yet, through the nightmares of the past 3 years, I have been changed.
My heart has hurt more than I ever thought possible.
I have learned what it’s like to see so much value and worth in someone that many view as a worthless drain on society.
I have been stretched beyond the boundaries of my simple, guarded heart, learning what it means to love fiercely.
My desire to see the effects of sin be overcome, and suffering to be no more, has been deeply renewed.
I’ve been drowned in the outpouring of love from family and friends, resurfacing strengthened, again and again.
This is the 1st of several articles I’ll be publishing on Hydrocephalus. If you’d like to know when future posts go live, please subscribe to our newsletter and I’ll email you each week with an update on the latest content!
How have you been impacted by hydrocephalus or a child born with special needs?