A Broken Heart

35,000 children are born with heart defects every year (American Heart Association)
Only 1% of those children have truncus arteriosus (many sources, including the Herma Heart Center where Turnip may be treated)
That means about 350 babies are born in the U.S. with the same condition as Turnip every year. (330 according to the Morbidity and Mortality Weekly Report from jan 6, 2006)
Surgery usually happens at 6-12 weeks (EPA, page 4)
15-50% of children require another surgery within the next five years (i.b.i.d.)
All heart valves are replaced after 12 years(i.b.i.d.)
Newborns with truncus arteriosus stayed in the hospital for an average of 30 days during 2003 (CDC, table 2)
The hospital charges for this stay (not including lab tests and doctor fees) ran to $200,000 (CDC, table 2)
In a small study published in 1996, 6% of infants died before surgery and 10% died during surgery (EPA, page 4)
In a national survey, 20% of newborns with truncus arteriosus died in the hospital in 2003 (CDC, table 1)

Saturday, December 31, 2016

Finally asleep

Just finished Harry Potter #8 and evening meds.  Fell asleep holding my hand.

Today's totals:
Only took 1 nap
Did number two, 2 visits from big sis
Took 3 walks, ate 3 good meals, played Magick with 3 friends

Sleep all night
Less out of the chest tubes
Lower blood pressures


1. Poop!  #1 done
2. Walk more.  #1 done, plus 1/2 hour sitting
3. Work on BP control.  In progress.  134/68 now.
4. Eat fatty foods.  Ate eggs, 3/4 of a turkey sausage.  Still no cloudy output.
5. Eat protein.  See #4.  Also currently getting an infusion of albumin.
6. Increase heparin a little.  I'm assuming this is done.  

Currently watching Harry Potter #7.  I must say I much prefer his movie selections this time around.  Last time I think we must have watched Frozen 20 times.

POD (post op day) 4 plan

1. Poop!  To help he had miralax in his prune juice this morning.
2. Walk more.  This will help move any fluids that have accumulated in his lungs or his tissues either out of his body or back into his circulation.
3. Work on BP control.  He's back on his home dose of enalapil and will get a dose of furosemide today.  The furosemide will also help him pee more, put less out of his chest tubes, and offload some of the extra fluid that he's currently holding on to.
4. Eat fatty foods.  Although he did not put any fat out through his chest tubes yesterday (which is reassuring), there is still a small chance that his thoracic duct (which takes fat from his intestines and delivers it to his blood stream) was damaged or irritated during surgery.  If it was, the fluid coming out of his chest tubes would become cloudy.
5. Eat protein.  He is healing and needs extra protein.  He is also losing protein from the fluid that is coming out of his chest tubes.
6. Increase heparin a little as his anti Xa levels were not therapeutic.  He'll stay on heparin until his warfarin levels are in the therapeutic range (2-2.5).

Boring night

Lots of sleep, peeing more, pushed a lot of fluid (1.4 L) out of his chest tubes (probably because he was up and about more yesterday).

Friday, December 30, 2016

Warfarin diet

 So, it looks like our family's diet will not need to change as much as I feared. I talked to a nutritionist today who said we need to be consistent in our consumption of foods that are high in vitamin K, such as kale and broccoli. She also reminded me that vitamin K is stored in the body's fat, so if in the past we've eaten brocolli once a week and kale every other week, then continuing to eat those vegetables at the same frequency will be perfect. Papa is off exercising and showering...and picking up some kale at the grocery store so we can feed Small boy the same foods in the hospital that he'll be eating

Also,  I brought him some chicken tiki masala from his favorite restaurant for dinner.

Progress on day 3 after op

So far today...
He ate half a scrambled egg, graham crackers and milk, a couple ounces of gatorade, and some prune juice.
He had all of his packing lines removed.
He had his arterial line removed, so he got his left hand and arm back.
He walked around the hospital, which took the help of his nurse and two physical therapists to support him and move all the tubes and wires.
His stats look good.
He's now sitting in a seat napping after watching much of Harry Potter 3.

POD 3 plan

1. Eat
2. Walk
3. Poop
4. Start heparin (goals: anti xa 0.3-0.7, PTT 50-70), continue warfarin (goal INR: 2-2.5)
5. Get a platelet transfusion (due to his platelet dysfunction).
6. Start an oral blood pressure med.
7. Take out his arterial line, non-functional ventricular pacing wires, non-functional atrial pacing wires
8. Change his dressing
9. Cardiology will start working on getting us home INR monitoring equipment.

Ready to make one little boy very happy


PICU doc confirmed small boy is back to a normal sinus rhythm.  He will not need cardioversion.  He can go back to drinking clear liquids once he wakes up...which will make him so happy!

What happened overnight was scary, but was how the doctors expected things to go if small boy escaped his funny heart rhythm on his own.

BP is still a bit high, but his temperature has remained stable within normal limits, he has great oxygen delivery to his fingers and toes (and everywhere else), and he continues to make urine.

I hope that today he is able to start eating solids again by the end of the day without any puking or problems with his heart rhythms.  The PICU doctor also mentioned removing his arterial line soon.

I am so looking forward to sleeping when I get home!

HR holding in the low 70s...

Even when he was sleeping.   I think he may have converted to a normal sinus rhythm around 6.  I think I can see P waves...also maybe U waves.

Ventricles in the drivers seat

Small boy's heart is beating (HR=60-70 in a junctional rhythm).

External pacing wires not working.  BP high, oxygen good, in pain, getting Tylenol and morphine

Backup: AED, medications, placing a temporary internal pacemaker

I agree with small boy: "I want to leave"

Losing capture

The pacemaker is no longer signaling small boy's heart.


HR came back up to 65 (possibly with the return of P waves) before the docs on site reached an electrophysiologist who helped them get the pacing wires to work.  Now being paced at 89.  Esmolol is off.  During the time right after he came out of atrial flutter, he maintained his blood pressure, he maintained his oxygen saturation and he looked good.  I think this was just him breaking the cycle. Not that it didn't get my heart racing.

He is now comfortably sleeping...and probably will for a while as he got morphine right before this all started.

Broke atrial flutter...

...now low heart rate.  Started at 20.  Now low 50s.  BP good.  Oxygen stating at 99.  Pacing wires not capturing. Pads on for cardioversion if necessary.  EKG being done.  All PICU docs in room.

Thursday, December 29, 2016


Small boy decided he wanted to sit up in his chair. It took two nurses 10 minutes to get him moved to the chair.  He almost didn't wait for them.  15 minutes later he decided he wanted to move back to the bed. It took two nurses 10 minutes to move him back to the bed. Then he fell sleep.

 His blood pressure's are still high, his heart is still in atrial flutter, and he is still thirsty--but not as bad as yesterday, which is good as he is NPO (not allowed to eat or drink anything) in anticipation of his cardioversion tomorrow.

Try, try again

 Small boy Is still in what they are now calling atrial flutter. His atria are still signaling super fast. ventricles are only contracting every other time his atria tell them to.  They tried another round of adenosine this afternoon. That didn't work. They also tried over-pacing his atria with his external pacemaker. That didn't work and it hurt because it also paced his abdominal muscles.   They just switched his medication from esmolol to amiodarone, in the hopes that it  Break water cycle. If it doesn't work, will sedate him and try to electrically switch his heart rhythms (cardiovert) tomorrow.

In other news, he sat up for an hour in his chair and ate graham crackers dipped in milk when he got his Coumadin. He did both of those things without puking.

Good nap

Small boy just took a nice nap and gave me a smile and a kiss before I left to go nap at home.
Physical and occupational therapy or just  arriving as I left. Papa will supervise this activity.

His heart rate is sitting at 130. There is no variability.

Good news: small boy's temperature has remained within normal limits for the last day. He has also started drinking water and Gatorade again without puking.

Future plans: CT surgery will consider removing his chest tubes after he starts eating and he's putting out less than 5 mL per kilogram from his chest tubes and the fluid he's putting out is clear, not bloody or cloudy (chylous).   This probably won't happen for another few days, as small boy has not started eating solids yet.

Trying medication

The adenosine test  showed that small boy's heart rate is not due to abnormal communication between the lower and upper chamber of his heart (reentrant tachycardia).  Instead it is due to some of the cells in his atria (upper chambers) having a creating an abnormal, extra fast signal (creating an ectopic atrial focus), the docs are trying a medication (esmolol) that should slow down the rate at which all of the cells in his atria can send signals.  The hope is that we will slow down the cells that are beating super fast...enough that his normal pacemaker cells can take back over at a more appropriate rate.  The risk is that this drug slows down all of the cells in his atria, so when his normal pacemaker (SA node) takes back over, it may be sending out slower signals than we want.  The other risk is that if we slow his heart down too much, his blood pressure may drop.  So, niteoprusside is off and nicardipine is going down.  Those are BP drugs.

'Started at 50, rate down to 144.  Up to 100, rate down to133.

Woke up comfy.  Watching big hero six.

Some things...

... I'd rather not watch.

Hooked up to EKG.  PICU, CT surgery, and Cardiology in the room. Pushing  0.1 adenosine to try to break the rhythm.

Adenosine works by temporarily stopping the heart, in the hopes that when it restarts it will restart in his normal rhythm.

Didn't work.

Getting echo (ultrasound of heart)


pushed him into a tachyarrhythmia(fast heart rate = 152).
EKG here, CT surgery here, PICU attending here, Cardiology attending here.
Flipped back into normal rhythm with a cough.
Back into svt with another puke.
Teams back in the room.

POD (post-operative day) 2: plan

1. Get up and move more.  He has been sitting straight up with pillows to support him since yesterday afternoon.  He will have PT and OT 2x per day starting today.
2. Advance to a general diet.  I ordered him toast with peanut butter, scrambled eggs, and turkey sausage for breakfast.  I also ordered graham crackers and milk for dipping as well as string cheese for a snack.
3. Poop.  He got miralax this morning and has s glass of prune juice waiting for when he wakes up.
4. Allow him to drink more.  After surgery, many people, including small boy, get inappropriately thirsty.  The medical team calculates how much fluid his body needs in a day.  This is called his maintenance fluids. This is the amount of fluid he is able to take in over the course of 24 hours.  Small boy would be very happy if he were allowed to drink his entire maintenance fluids.  However, any fluids he receives through his IV are subtracted from the amount he is allowed to drink.  Unfortunately for small boy, he has gotten quite a bit of fluid through his IV: antibiotics, replacement calcium, blood pressure medications, and blood products.  Today the CT surgery team loosened his restrictions.  He can now drink his entire maintenance fluids.  Any IV fluids will not count against his  fluid intake.
5. Better blood pressure control with BP meds (nifedipine, nicardipine, milrinone) and pain control (Tylenol, oxycodone, fentanyl)
6. Continue with anticoagulation:  full dose of Coumadin today. Start heparin tomorrow.  INR = 1.4, PPT = 28 (was 29.5 yesterday).

Morning update: blood pressures

Oliver slept poorly and had (expected) high blood pressures over night.  He is now on two blood pressure medications: nifedipine and nicardipine.  The high blood pressures are an expected reaction of his heart adjusting to its new configuration.

Before surgery, a lot of his blood would flow backwards from his body back to his heart through his incompetent aortic valve between every heart beat.  This meant that his heart had to work extra hard to pump out the extra blood every time it beat.

His new mechanical valve prevents blood from flowing backwards into his heart between beats, so there is less blood for the heart to pump out to his body.  However, his heart is still getting used to this reduced work load and is pumping hard...which makes his blood pressure high.

Eventually, his heart will adjust to its new, more efficient setup, but until then the doctors will use blood pressure medicine to keep his blood pressures low in order to protect the suturing that is currently holding his heart and his new heart parts together.

Wednesday, December 28, 2016

Peacefully sleeping

Small boy has done well today, so the doctors advanced his treatment plan.  Since he is tolerating small amounts of oral intake, has started Coumadin and switched over to (oral) oxycodone, which seems to be working well.  The plan is to start his bridging dose of heparin tomorrow.  His INR is currently 1.6.

His temperature has been trending downward.

Excellent care

I forgot how much of a difference excellent nursing care can make.  We have had three thorough, kind nurses so far who have advocated for small boy with the rest of the medical team and been super responsive to his needs and our questions.

Doing fine - THIRSTY!

He's unhappy that he can't drink all the water he wants, and I think the pain level just went up. They gave him some morphine, and he's napping now.

Continuing to do better

Junior has had the pacing wires out for over an hour now and rhythms and pulse rate are still looking good. He also just had a popsicle, which he was very excited about (the occasional ice chips and sips of water weren't cutting it). Of course, he downed it super fast and now has to wait for more fluids, which he's not excited about. The PICU doc just came by before leaving, and he noted that he didn't expect Junior to be doing as well as he is at this point.


As I was leaving, the nurse stopped me to tell me that small boy was periodically making his own heart beat.  After I got home, Papa Texted me that they had stopped pacing Small boy's heart to see what it would do on its own.  Things look promising.


Cerebral oxygen saturation patch off.
Hand/arm restraints off.
Pressure bandages (mepilex) off.
Foley catheter out.
Watching Harry Potter #1 with papa.
Begging for water.

Post op day 1: plan

After 21 people rounded on small boy (including two electrophysiologists and the entire CT surgery team), we have a new plan.   The plan takes into account Olivers funky heart rhythms, which are unexpected post surgical complication. They're expected to resolve within a week. However, during that time he will need some pacing.

It seems as though Olivers external pacemaker was set too low. That's why it stopped pacing his heart when he coughed.  The pacemaker is currently turned up higher. Also, the two electrophysiologists were able to once again pace his atria.  Pacing these upper chambers of his heart will be the backup plan if the ventricular pacing wires stop working.The CT surgery team is also on standby to place additional pacing wires if these wires fail ( which is not expected ). Their equipment is on a cart outside of small boy's door.

 in addition, small boy was given fluids ( albumin ) to help with his low blood pressure. His pressures are now beautiful in the 90s over 50s.

His temperature is also trending downward and is now 37.5.

If everything remains stable until noon, we will attempt extubation. But for now he still has a breathing tube in, and is heavily sedated.


change of plans: no extubation yet.  Heart rhythms funky. Pacing wires not consistently capturing.

Post operative update 5

Getting ready to extubate.  It's the one thing small boy keeps indicating that he wants.
His heart rhythm continues to evolve.  Currently in heart block with an intrinsic rate of 20, ventricularly paced at 100.  Atrial pacing wires no longer capture heart but still capture diaphragm.
It is thought that decreasing his precedex will release his respiratory depression and heart rate depression.  He should then be able to breathe on his own and move toward beating his own heart.
His temp is still elevated to 38.6, but he is on antibiotics, and the current thought is that the temperature is due to inflammation in response to all of the scar tissue that was cut through.

Post operative update 4

Small boy's belly and diaphragm are no longer being paced, but his heart is: ventricles only.
He is not easily sedated, so he keeps getting doses of sedatives.
The sedatives may be contributing to his low blood pressures.
The low pressures may also be related to fluids moving out of his blood vessels and into his tissues.  His eyelids are quite puffy.
His temperature is also creeping upwards (not surprising).

Tuesday, December 27, 2016

Post operative update 3

Down to fentanyl, precedex, and milrinone.
Still intubated.
Still paced...and his pacing wires are pacing his diaphragm.  It looks like hiccoughs--only 90 every minute.  The cardiologist on call, who just happens to be a whiz bang electrophysiologist, is on his way in to try to help.

Post operative update 2

Nifedipine for BP control
Amicar for bleeding (standard)
precedex for sedation
Fentanyl drip for pain

Post operative update 1

The surgery was everything the surgeon hoped it would be.  As expected, there was bleeding.

The surgeon ran bypass through small boy's carotid instead of his aorta as small boy has a very short aorta and all of the "prime real estate" was used during prior surgeries.  Brain oxygenation was monitored throughout surgery and was fine.  The carotid was repaired after small boy came off bypass and has good flow.

There is a small leak between the sutures used to attach the valve to the aorta.  Our surgeon consulted with the adult CT surgeons who do many more aortic valve replacements than he does.  They said to leave it alone.  It is smaller than the built-in leak in the valve.

The surgeon removed a hunk of muscle from below the new valve to prevent small boy from developing an obstruction in the future.  He said it was left over from previous surgeries.

Mechanical valve size: 21 (adult) st Jude
Conduit size: 22 (adult)
Paced: yes.  His conduction system is working...but his heart was not beating as fast as they wanted it to, so they are keeping him paced for now.

Intubated due to bleeding due to scar tissue due to prior surgery.  Surgeon believes he could have been safely intubated already, but is being extra cautious.  He expects the PICU docs will extubate him in a few hours when he wakes up.

How is the team doing? They were done at 1:30 pm but kept him downstairs in the OR to make sure there would be no bleeding once he came to the PICU.

intra-operative update 7

Closing the incision.  
Small boy is doing well.

Intra-operative update 6

Surgeon still working.
Papa and I are heading back to the hospital to wait.
We both got in a good nap today in preparation for the week to come.

Intra-operative update 5

Small boy is off bypass.  Surgery going well.

Intra-operative update 4

Small boy now has a shiny new "robot" valve at the base of his aorta and the surgeon is replacing the tube that takes blood from his heart to his lungs (RV to PA conduit).

Intra-operative update 3

Small boy is on bypass.  Surgery is progressing well.

Intra-operative update 2

Surgery is progressing.  Small boy is doing well.

Intra-operative update 1

Surgery has started.  Small boy is doing well.


I don't know how other parents do this.

Papa just goes with the flow and assumes things will work out.

I ask questions and question answers.  I ask if there is no better way, if we can't avoid the scary stuff, if maybe there is some forgotten something that will make everything easier.  And then, when the time comes to push back for surgery, I feel I have done everything that I can, exhausted all easier options, and I chose to trust the experts who are a part of small boy's team.

They are thorough, detail-oriented and very, very good at what they do.  They do not cut corners or take short cuts.  They have contingency plans in place.  I trust that, whatever the outcome, they and we will have done our very best.

In the OR

After a little midazolam, small boy got pushed back to the OR.
He took mama, Sarah (his stuffed penguin), and blankie with him.
He chose to breathe root beer flavored anesthetic, reached his hand up to put it on his mama's cheek, and was out.
We should see him after 7 this evening.  

Monday, December 26, 2016


#1.  As we are walking down the hallway after today's lab work
Small boy:  I wish I didn't have this.
Me: Have what?
Small boy: All of this:  the blood draws, the surgery, the heart stuff.
Me:  Me, too.  I wish you didn't have it too.

#2.  On the car ride home
Small boy: you know Baymax in the movie about the little boy?  The one who is "your personal healthcare assistant?"
Me:  big hero six?  Yes.
Small boy:  I wish he were real and I had one of him.
Me:  what would he do for you, give you squishy hugs?
Small boy:  No, he would give me shots, and give me surgery.
Me: You know, you already have a baymax,
Small boy: huh?
Me:  You know how papa checks his blood sugar?  After you start your new medicine, you will have to check your blood, too.  Your papa will help you, just like baymax.
Me:  Do you know how old your papa was when he had to start Checking his blood sugar?
Small boy: eight?
Me: yep.
Small boy: really?
Papa: Yes, really.
Small boy:  I'm eight, too.

CT surgery instructions

Do a pre-op scrub at home tonight
No meds in the morning

Anesthesia instructions

No solids or cloudy liquids after midnight
No clear liquids after 0530
Skip morning blood pressure meds
Ask CT surgery if we should skip morning antibiotics
Ask CT surgery if we should do a scrub at home
Arrive at 0615
Surgery at 0730

Wednesday, December 21, 2016


I had both email and phone conversations with some of small boy's doctors today:

1) EKG changes
--nonspecific ST changes in precordial leads (V1, V2, V5, V6) suggestive of (more likely) increasing heart strain or (more scary) pericarditis
--Premature Atrial contractions...which I'm pretty sure he has had in the past, but there was some trouble finding his old EKGs to compare with.
--He now has a 48-hour Holter monitor and we scheduled an Echo and EKG on Friday to follow up on these findings.

2) This sucks!
--Yes, yes it does.  All of it.   There are no good solutions.... Every fix brings its own set of problems.  --All of the problems (and all of the solutions) are scary.

3) The rash seems to be receeding
-- we'll continue to monitor till Thursday

4) Anticoagulation
--Will be small boy's life-long companion after Tuesday -- because I will have agreed to it.
--His doctors will come up with some more clear guidance for us before our newly scheduled appointment on Friday: 1) a nose bleed plan, 2) a head bonk plan, 3) a list of high energy sports that are acceptable. 


So, apparently, small boy has some non-specific changes in his EKG that was done on Monday.  ST and T wave changes and some PACs.  I'm not sure the direction in which those waves have changed...or in which leads....the nurse just called to schedule a 48-hour holter monitor starting today and another EKG and echo on Friday.  That is NOT reassuring at all. 

But, I think it makes sense to get his CXR before attaching the holter, so I shipped big sister off to a friends house and small boy and I are going to start our medical perigrinations again.


Monday, December 19, 2016


So...for the past few months, small boy's dad has been managing his medical appointments.  On Monday, we switched.

All I knew was that small boy had an appointment at 9 am with Hematology and then "there were a whole lot of other appointments on the reminder message, but I only set up the 9 am one."
me: "What were the other appointments?"
him: "I don't know, the message was really confusing."
me: "o.k."

In addition, I knew I wanted to schedule an appointment to get his rash checked out since it hadn't cleared since Thursday.

Of course, as soon as it was time to leave the house, small boy had to pee, and sing and pose in the mirror while he washed his hands, so we started off a bit late.

Then, while I drove in to Hematology, I called small boys regular doc to set up an appointment, only I didn't know when we would be able to be there because I didn't know which other appointments he had scheduled for today.  So, I guessed at took the 1 pm appointment.

When we checked in at 9 am, I learned that small boy had appointments at 11 and 12...but not in the children's hospital, in the heart and vascular center in the main hospital...so I couldn't check in.

I called to try to figure out if we would be able to make our 1 pm appointment, cause, you know, having open heart surgery when you have a rash all over your chest seems like a bad idea.  The lady I spoke with assured me we'd be fine.

And then the Hematology appointment involved squirting something up small boy's nose (which required a full body restraint courtesy of me) and getting a blood draw (no big deal).

I thought the blood draw was everything he would need for surgery.  The phlebotomist didn't have any other orders....however, we learned later that there would be more draws, at a different facility due to insurance requirements (boo, hiss!)

The Heme folks gave small boy Battleship, which he really likes. The tests they ran showed that DDAVP corrects his platelet dysfunction...so we went home with some....but we were told not to use it after he is on warfarin, so I'm not sure what good it will do, since warfarin should start in 8 days.

While we were at his Heme appointment, we noticed that his rash had spread to his left sideburn and right cheek. 

Then we checked in for his 11 am appointment and were told that his noon appointment would take an hour, so I called to cancel his 1 pm appointment, and rescheduled for 5:15 pm.  15 minutes later, when she was taking up back to the US suite, the tech told us the entire appointment (11 and 12) would take only 1 hour....So, I called small boy's general doc back to reschedule for 1 pm.

The US showed wide open veins in his neck and groin, and retrograde flow in his abdominal aorta (his aortic valve is not working).

We got lunch at the "mama cafeteria"and then visited his general doc, who gave us oral and topical antibiotics for his skin rash and did the EKG that was scheduled for tomorrow.  We thought we were all done....

...but when I got home and read the material the CT surgery team had sent, I realized that small boy would need a chest Xray and another blood draw before the 26th..... 

Thursday, December 15, 2016


So.... small boy now has a red, bumpy rash on his chest where his gymnastics singlet does not cover.  I'm not sure what it is.  We noticed it after gymnastics tonight.  Kevin took him to urgent care.  They thought it was a heat rash, but said to bring him back on Monday if it wasn't better.

Tuesday, November 22, 2016

Surgery date: Dec 27

Will replace truncal (aortic) valve with mechanical valve, replace pulmonary homograft and start blood thinners.

Thursday, October 20, 2016

not quite sticky enough

Today we visited a pediatric hematologist to see if she could come up with a reason for small boy's nose bleeds (I was also sort of hoping she would come up with a reason why he would not be able to get a mechanical valve).  Small boy had some fancy pants blood work done a few days ago, and we went in so she could explain the results. 

It turns out that there is a reason why small boy's nose bleeds last so long.  Blood has lots of different ingredients: some of them help carry around oxygen, some of them help fight germs, and others help plug any blood vessels that get leaks by forming a clot.  There are lots of different ingredients that help blood clot.  Small boy has a problem with two of them. 

 Sweet Gum Balls from my yard. First are the platelets.  Platelets are like sticky sweet gum seed pods.  When a blood vessel gets a hole in it, they stick to the edges of the hole...and then they stick to each other and form a temporary plug.  Small boy's platelets are not as "sticky" as expected.  That means they form a less stable plug more slowly than expected. 

Then there is factor IX.  Factor IX is one of many ingredients that create the "glue" that coats the platelets after they form that plug.  The "glue" helps strengthen the plug made out of the platelets.  Oliver has less factor IX than expected.  This means his platelet plugs have a harder time staying stuck together after they form. 

We don't know why small boy's platelets are less sticky than expected or why he has less factor IX than expected.  His unusual heart anatomy may be making his platelets less sticky.  He may be using up more factor IX to make up for his less sticky platelets (Use more glue to hold the flimsier clots together).  He may have a mutation in the gene that codes for factor IX.  We don't know.

What we do know is why he has such long nose bleeds.  We also have more information to think about as we consider how to proceed with his next surgery.   There is a medicine (DDAVP) that may help when he gets long nose bleeds, but overall, having an explanation for why he gets the nose bleeds does not prevent him from getting nose bleeds...or from getting a shiny new mechanical heart valve for his birthday this year. 

Saturday, August 6, 2016

special birthday present this year

After small boy's echo and cardiology visit in early June, he got sent for a stress test with doppler echo, and then a cardiac MRI.  Long story short: it looks as if he will be getting a new heart valve for his birthday this year. 

I had hoped to avoid the blood thinners for a while longer--at this point mostly because he still occasionally has some really scary nose bleeds.

His new valve won't change his activity restrictions as we've already adopted the guidelines that he will be on after he gets a mechanical valve.  We may have to change our diet to avoid some of our favorite foods that are high in vitamin K, though--like kale.  

My chest gets all clenchy, my stomach ties in knots, and my upper arms get all tingly and hard to operate when I think about it.  While typing this out, the area around my lips has gotten all tingly and I'm having a hard time convincing my fingers to hit the proper keys. 

Monday, April 4, 2016


Today I am grateful to sit on the edge of small boy's bed, holding him on my lap while he reads a book.  The unnaturally loud think of his heart beat is quieter than usual right now.

Sunday, February 21, 2016

how to compare pediatric cardiothoracic surgery programs

Apparently, there is a way to compare the success of different hospitals ... that isn't based on how much the parents' of the children who were treated liked the doctors (US News and World Reprt).  The Society of Thoracic Surgeons has compiled a database comparing different pediatric cardiothoracic surgery programs around the country.  The database is searchable at:


This particular link does not contain all of the data... but it does allow me to compare our local children's hospital with others around the country.

Monday, February 15, 2016

triannual cardiology appointment

The good news: no surgery for now.

The details: Small boy's heart has not changed:  It is still enlarged and ineffecient but functional.  For now, his medical treatment will be guided by his behavior.  Unless he starts showing signs of heart failure, he will continue visiting his cardiologist every four months for an echocardiogram and evaluation.

If anything changes, he will get an MRI.

His next surgery will focus on both his pulmonary trunk and his aortic root.