The father of a new hydrocephalus patient shares his story…

We recently met Tom Coury, a rehabilitation physician, triathlete, and recent father to a daughter with hydrocephalus.  His daughter Amelia received her first shunt at 17 days old, and Tom was kind enough to reach out and share their story.  He’ll also be swimming Alcatraz with us this summer!

We are so touched and grateful to have the Coury’s as part of the Team Hydro family. Their story, still just beginning, already captures so much of the uncertainty that comes with a diagnosis of Hydrocephalus. By the same token, the strength, optimism, and indefatigable resolve of Amelia’s parents speak to the very best of humanity.  Taken together, this uncertainty and this resolve embody exactly why we do what we do at Team Hydro.

We love you, Coury’s!  Go get ’em, Amelia.  We’re cheering for you.


Amelia’s Daddy’s Log

6/18/2017 – I’m taking a shower, and Heidi tells me the best Father’s Day news I’ve ever received.  She is pregnant again.  This will be Baby #6.  See, we have a baby around every 3 years.  We’ve had a baby in 2003, ‘06, ‘09, ‘12, ‘15, and now 2018.  The birth order so far is B – B – G – G – B.  I ask Heidi to keep the sex of the baby from me.  We had always known prior to birth, but I had never been surprised.  She was good to keep the surprise from me right up until the birth.

12/7/2017 – Heidi had her second ultrasound.  For background, our last two births have been at TMC Birthing Center with nurse midwives.  We’ve found that the birthing center provides a good balance between over- and under- medicalizing the process of childbirth.  Our last baby came out at 10 lbs 2.5 oz.  Heidi did that at the birthing center without an epidural. 

The initial ultrasound this pregnancy showed everything as normal.  But due to her age (38), the midwives recommended an additional ultrasound.  This second one was done at 28 weeks.  This time, the measurement for the lateral ventricles was 10.3mm (upper limit of normal is 10mm).  This put the baby in the category of ventriculomegaly. 

I went into a small panic.  My head began to race with thoughts of surgery after surgery after surgery, and shunt after shunt after shunt.  Thoughts of infections, and explants, and wounds.  I’m a physician who specializes in Physical Medicine & Rehabilitation (PM&R or physiatry) and I have taken care of several patients over the years with shunts.  The patients I see never seem to do well.  The shunts are continually failing, and the patients seem to be in the hospital multiple times a year.  One of my patients has over 20 no-shows to her clinic appointments with me, the vast majority of which are due to her being in the ER or hospital.  My role in her care is primarily pain management.

1/10/2018 – Another ultrasound is taken, showing the ventricle size at 10.3mm (no change).  I take a deep breath in.  Perhaps this is one of those abnormalities that are never found unless you look for them.  People walk around all the time with unusual this or that.  Maybe the ventriculomegaly will self-correct.  In fact, didn’t I read somewhere that enlarged head circumference is possibly a weak marker for autism?  We already have two kids on the autistic spectrum.  Yep.  This is, as a pediatric neurologist once said about my oldest, while looking directly at me, Familial Macrocephaly. 

2/7/2018 – Anther ultrasound is taken.  The ventricle measurement has increased significantly.  This is no longer ventriculomegaly; this is hydrocephalus.  At the time of the ultrasound, the technician had the Maternal Fetal Medicine specialist do a quick consult.  She recommended we get in with “neuro” soon after the birth (Was that neurology or neurosurgery?  Somehow my brain skipped neurology and went straight to surgery.)  That evening, I spoke with the MFM specialist for about an hour about hydrocephalus, what to expect, and where to go from here.  At this point, we were no longer in the realm of the birthing center.  We would be delivering at TMC at 39 weeks.

2/19 – I cleared my schedule expecting her induction and delivery to go quickly.  What I didn’t expect was that TMC was so busy there was no room for us at the inn.  We were initially supposed to be called at 5:30 in the morning to come in.  We got the call at about 10:30 that night.  The induction was started just before midnight. 

2/20 – I enjoy the birthing center experience, but that’s a lot more work for me.  At the hospital, she gets an IV, an epidural; we turn on some TV, kick back, and wait for the magic to happen.  We asked for the Neonatologist to do a consult.  Obviously the NICU was pretty busy, because he didn’t come around until about noon.  He again went over hydrocephalus, what to expect, etc.  He left, and the nurse midwife checked Heidi again.  She saw the baby’s head, so she figured it’s time to push.  The plan was to have the NICU on hand in case there was any distress.  Heidi gave about 4 good pushes in a row, and then took a deep breath, ready to start the hard labor.  That’s when the midwife handed her a 7lb 11oz beautiful baby girl (with a big head).  The baby was just fine.  She went straight to mom.  The NICU nurses looked confused and asked, “Why are we here?”  We excitedly shared the news with the rest of our family and friends that Amelia Margaret Coury was here, and she was rock solid stable. 

2/22 – Heidi and Amelia got to go home.  Prior to discharge, Amelia had a post-natal brain ultrasound.  I totally geeked out, being able to see so many brain structures that are normally only visible with an MRI.  (Baby skulls are not hard bone yet, so the ultrasound can peer down pretty far, though not as far as MRI.)  Her scan showed a Grade 1 intraventricular hemorrhage (bleed) on one side and a Grade 2 hemorrhage on the other.  We were all a bit puzzled, as IVH is normally a complication of premature birth.  But Amelia was a full-term, good sized baby.  The plan was to go home and follow up with her pediatrician, and get a referral for pediatric neurology.

2/26 – Her pediatrician, Dr. Carl Roberts, checked her out and felt she looked good.  He made the referral to pediatric neurology and ordered a follow-up ultrasound.

3/5 – The follow-up ultrasound showed her ventricles had enlarged again.  Dr. Roberts gave us the results on the phone. 

3/6 – Her fontanelles began to bulge.  It looked like a goose egg on the top of her head, like when you smack your head on something hard.  Dr. Roberts made the call to Phoenix Children’s Hospital neurosurgery team, which is the pediatric division of Barrows Neurologic Institute. 

3/7 – Heidi and Amelia (along with my parents) got to meet Dr. Taryn Bragg for the first time.  Amelia’s head circumference had not changed much from Dr. Roberts’ visit.  She ordered an MRI and told Heidi to plan on surgery the following week.  I spoke with Dr. Bragg for about an hour that evening.  Amelia’s presentation was not clear.  She had the hydrocephalus, but she also had a very large posterior cyst, which appeared to be communicating with the ventricles.  This cyst could be putting pressure on the posterior brain structures (cerebellum and brainstem).  She told us to watch for signs, such as sun setting eyes, projectile vomiting, or lethargy.  I cleared my schedule for the following week. 

3/9 – I came home from work.  This was a Friday afternoon.  I held Amelia in my arms and looked in her eyes.  Something wasn’t right, but I couldn’t quite explain it.  She had disconjugate gauze (the eyes look in different directions), but in brand new babies, this can be normal.  However, it also could be due to compression on the parts of the brain that control the muscles of the eye.  I took some videos to send to Dr. Bragg.  Later, Heidi was changing Amelia’s diaper, when her eyes started to set.  Heidi got scared.  We immediately dropped our plans for the evening and headed straight to Phoenix Children’s. 

3/10 – Amelia had her VP (ventriculoperitoneal) shunt placed.  Dr. Bragg elected to place a nonprogrammable, medium pressure shunt made by Medtronic.  The surgery went well.  We got to visit with Amelia while she recovered in the PACU and awaited a bed in the hospital.  Her bandage wasn’t sticking to her head, so Dr. Bragg had fashioned a big, beautiful coban bow for her.  Her vitals looked good and she was resting. 

As part of the normal post-surgical protocol, at one point the nurse checked her eyes for a pupillary reflex.  When you shine a light into one eye, you should see both that eye and the other eye constrict to adjust for the light.  Amelia’s eyes didn’t respond.  At all.  Another nurse came by to check, and they didn’t respond.  I checked them myself, and they didn’t respond.  The nurse called Dr. Bragg who ordered a STAT CT of her head.  The CT was normal.  We had no idea why her eyes were not responding. 

She was taken to the NICU.  This day took the lead as the scariest day of my life as a father, just barely passing that time Heidi was in my truck with my two baby boys and was hit by a car.  I waited in the ER for 45 minutes for the ambulance that day, and was meticulously picking out pieces of glass from their hair in the bath that night.  This day was scarier than that day. 

The NICU staff was great.  In fact, the whole staff at PCH was great.  Pediatric hospitals are magical places, where everybody is there because they want to be there.  From techs, to nurses, to docs, to staff, there wasn’t a grumpy face in the place. 

Heidi and I hadn’t slept more than a few hours the night before surgery.  She was put in contact with Ronald McDonald house so we could take turns getting some shut-eye.  I went off to dinner at the cafeteria not knowing what we were dealing with.  Was there permanent brain damage?  Was this just a delayed response to anesthesia?  Was Amelia blind?  I started to doubt my memory of her vision.  I had only known her for 17 days, but maybe I was wrong to think Amelia was looking at us.  I pulled out the videos I took of her the day before.  No, in fact, Amelia was looking right at me as I held her.  Her eyes constricted and dilated with the light as I moved.  Her eyes were working, less than 24 hours prior.  Something had had happened. 

I wanted to do something for Amelia.  I wanted to help in some way.  I felt helpless.  Sure, I could be a good father.  We would take good care of her, no matter what the situation would be.  If we had a blind child, then we would deal with that.  But maybe there was something else I could do.  Maybe I could give back in some way.  One of my hobbies is racing triathlons.  Maybe I could put together a race team, like they do for breast cancer and blood cancers.  I even thought of a great name – Team Hydro.  Excitedly, I searched for that name, and discovered that such an organization already esisted.  And this wasn’t some flash-in-the-pan charity.  Their funding had already yielded legitimate bench (basic science) and translational (lab-to-clinic) research. 

Heidi went to Ronald McDonald house first.  Fortunately, she had pumped plenty of breastmilk to get through the evening.  Unfortunately, she left the breast pump at the hospital, so I knew she would be back in the middle of the night. 

3/11 — I’m fuzzy on the details (sleep deprivation will do that), but at some point in the middle of the night, Amelia’s eyes started to respond.  It was a weak response initially, but it was there.  Heidi came back around 2 AM and we switched out.  By the time I got back to the hospital around 6, her eyes were back to normal.  We still don’t know what happened or why. But we were grateful they were back.  As Heidi drove part of the way home, I sent a text to Dr. Bragg thanking her for her work.  She responded kindly, and added, “drive safely”.  It occurred to me that relative to the drive between Phoenix and Tucson, a VP shunt placement is a pretty safe procedure. 

3/20 – Dr. Roberts evaluated Amelia and gave her an all-good bill of health. 

3/22 – Follow-up with Dr. Bragg and a repeat MRI in Phoenix.  The posterior cyst membrane appeared to be descending along the brainstem.  She recommended watching clinically and follow-up in a month.

3/24 – Her fontanelle appeared to be bulging again.  We worried about shunt failure.  Dr. Bragg recommended keeping her head upright.

3/30 – Amelia gave her mom some projectile vomiting, exorcist-style.  Heidi was rightly concerned.  We now noticed that her fontanelle was quite sunken, and that the skull bones were even overlapping a bit. I was worried about over shunting. Dr. Bragg recommended keeping her head lying flat.

4/2 – Amelia gave her mom another projectile vomit. 

4/3 – One more episode of vomiting, and Heidi took Amelia to Dr. Roberts office.  Dr. Roberts checked her over for non-neurologic sources, and felt that all was fine. It must be coming from her neurologic situation.

4/4 – Amelia vomited again.  Heidi called Dr. Roberts, who promptly called Dr. Bragg.  I went to work on Friday, 4/6, fully expecting that Amelia would have her first revision the next day.

4/6 – Heidi took Amelia up to PCH for a new MRI and a visit with Dr. Bragg.  The imaging looked good.  No surgery was recommended, and follow-up made for one month. 

At this point, there are more questions than answers.  Why are Amelia’s fontanelles shifting so quickly with positional changes, yet the MRI does not show a dramatic change?  Will she need a revision before her fontanelles close at 18 months?  Why did her eyes not respond, and should we be concerned about anesthesia in the future?  And why does she have hydrocephalus in the first place?  We haven’t yet seen a pediatric neurologist, but Heidi made an appointment with one.  Dr. Bragg mentioned that this might be something called a Dandy-Walker variant, which is probably defect in neural tube formation. 

4/7 – I got to meet Sam and Peter Finlayson on a conference call today.   Together, they formed Team Hydro, and their family sits on the Hydrocephalus Association board.  I’m excited to have these guys to lean on, (and to give back to as well) as we continue this journey. 

Amelia is developing well.  She looks at her daddy and mama, and is beginning to coo.  She has the beginnings of a social smile.  She can occasionally bring her hands to her mouth to suck on her fists.  Her pupils react and accommodate well to light.  She has other normal baby issues – reflux, cluster feeding, wanting to be held all the time, that the rest of our kids have been through.  It’s 10:40 PM.

I’m going to go give all of my kids a kiss goodnight.