Ok - so what now??

Max has always had a small bump on the bridge of his nose.  When he was a baby, it was a slightly more pronounced vein and as he has gotten older it has started to protrude similar to an aggravated mole.  A few weeks ago, Max rubbed it to a point where it bled slightly – no big deal.  Then, on Thursday while he was in his bed taking a nap, he accidentally rubbed it to a point where it must have burst open. 

Thankfully, our nanny randomly decided to go upstairs to check on him.  Unfortunately, she found him in a pool of his own blood.  Where the blood was coming from was obvious, because it wasn’t just leaking out – it was gushing upwards like a small fountain (seriously, what is up with the horror film blood scenarios lately?!).  Multiple attempts to stop the bleeding were unsuccessful to the point that Max got his first ambulance ride and then, only by applying a clotting agent, was the Emergency Room staff finally able to stop the bleeding. 

ER visit #1

Back at home, we honestly thought the worst was behind us as we put him to bed that evening.  But when Todd went to check on him at 1am that evening, he found him lying in a heart-stopping pool of blood having accidentally pulled off the bandages.  We knew the drill – called my Mom and headed straight to the ER.  Fortunately, this time we were able to get the bleeding to stop without significant medical intervention, but we still had no idea how to prevent it in the future. 

ER visit #2

Our solution was to protect the wound by applying multiple band aids and having Max sleep with socks on his hands (yeah, he was not thrilled), which worked – for a while – until Sunday night when Max managed to somehow rub it just right and cause it to burst while still wearing the band aids AND THE SOCKS!  If it wasn’t totally traumatizing, I would have been impressed. 

So Monday we went into full attack mode to avoid future bleeds.  The problem was we didn’t even have a plan of attack.  We didn’t know what this bleeding “mole” was even called, how to treat it or even who we should contact.  Luckily, we have a village.  Todd’s Dad called a friend who called a friend who called a friend so that we could be squeezed into a plastic surgeon (even if that plastic surgeon now specializes in tummy tucks and weight loss, it would at least give us some direction!).  In the meantime, our pediatrician worked to schedule us with Dermatology only to find out once a plastic surgeon had looked at it, that what we really needed was a pediatric plastic surgeon.

When I did finally talk to the scheduling assistant for pediatric plastic surgery, she commented “Max must be a very special boy.  I’ve gotten A LOT of calls about him today.”  Ya damn right, Linda. 

We met with the plastic surgeon this morning and surgery is scheduled for Tuesday, so we have a plan, but my anxiety is still sky high.  Max is basically on 24 hour surveillance with Todd and I taking turns sleeping beside him to make sure we have no more accidents.  Now I love Max, but he sucks as a bedfellow so we’re very tired and extremely cranky – including Max. 

A cross between Nelly and a Real Housewife post-nose job

I feel like we’ve crammed a year's worth of trauma into the past few weeks and I can’t help but worry about the financial ramifications of this tiny “mole” – one ambulance ride, 2 ER visits, one out-of-network plastic surgeon visit – but you know what they say – Mole money! Mole problems!  (get it? LOL! Why don’t I have friends?)

So what is it?  Initial diagnosis from Dr. Tummy Tuck (who was lovely and very helpful) is a pyogenic granuloma – a gathering of capillary blood vessels prone to profuse bleeding.  Because it is directly on a capillary, the blood will gush vs. flow. 

Why is it bleeding so much now?  Max has issues with blood clotting linked to PBD.  He does take Vitamin K for this issue and was given Factor 7 in the ER to help as well.  As to why it has started to bleed, who knows and jury is still out on whether it is related to the esophageal varices issue. 

Is he in pain?  No, I don’t think so.  For him, it’s just as if a scab came off, but we do have to hold him down for a substantial amount of time to stop the bleeding and that is unpleasant for all parties involved. 

What do you need from us?  Really nothing.  Just to vent.  The fact that a tiny skin bump could jeopardize Max’s health is so incredibly frustrating and sad.  Over the past months, it has truly felt like it is one thing after another with little breathing room.  Since it’s my birthday week, I’m also allowing myself a party – a pity party. Todd and I had planned to attend Rare Disease Week this week in DC and were really looking forward to experiencing that together, but we obviously felt uncomfortable leaving Max in his current condition, which is the right decision, but I can’t help feeling disappointed. 

On the positive side is that Max is no worse for the wear.  All the ER trips and lost hemoglobin have done nothing to break his spirit.  If anything, it may have made him just a little slap happy 

Thanks for reading - Corin

So how is Max?

I did this to myself.  I spent all of Saturday, December 28^th doing nothing.  Todd hung out with Max and Ellie hung out with a friend so that I could spend the full day putting together a 1,000 piece puzzle.  It was incredible.  Then, I did the unthinkable – I got cocky and posted about my relaxing day on Instagram. 

So really, it should have come as no surprise when Sunday evening Max threw up enough blood to make our kitchen look like a triple homicide.  In that moment, surrounded by that much blood, what can you do but call your Mom and just say “I need you to come over.”  I’m not sure Todd and I even talked about it, we just immediately started packing for a hospital stay. 

One quick side bar – while Todd and I packed and my Mom helped clean up Max, my Dad cleaned up the kitchen and I gotta say that if you do happen to commit a triple homicide and need someone to clean it up, Greg Garbe might just be your guy.  Five stars.  Would recommend. 

When we got to the hospital, just as the nurses started to question exactly how much blood he had thrown up (I said a lot – I meant A LOT), Max promptly threw up another large amount of blood helping to jump start the level of care we received.  Bless his continued sense of timing. 

When we initially talked to the doctors, we assumed that the bleeding was due to an inflammation of the ulcer Max had suffered from a few months ago.  Despite all the blood, Max seemed to have bounced back fairly quickly.  He was a bit pale and seemed to have some lingering stomach pains, but was otherwise acting normally so we didn’t overly worry.  When we had been hospitalized for his prior ulcer, we had decided not to do an endoscopy since the medicine seemed to have alleviated any symptoms, but we knew with this additional episode it would absolutely be necessary.  What we weren’t expecting were the results.

The endoscopy showed that Max had most likely suffered from a ruptured esophageal varices.  Esophageal varices are enlarged veins due to obstructed blood flow to the liver and are usually associated with advanced liver disease.  We had spent so much time in 2019 trying to get his seizures under control, we totally forgot that he also had the liver of an 80 year old barkeeper.

So what do we do now?  When the varices ruptured, it alleviated the pressure that had built up, so Max feels great right now, but there’s really no real solution to stop the same thing from happening again.  We are meeting with his gastroenterologist to explore a possible beta blocker, which may help with bleeding, or exploring rubber band ligation to essentially cut off the varices.  However, neither of these options are 100% effective and this diagnosis has the feel of a ticking time bomb.  

So how is Max? He’s happy, playful, and as ornery as ever.  How are we?  Overall, we’re scared and this super sucks, but I suppose it’s just one more rock on the shit* mountain of PBD.  

 

So here is my prayer:

Dear God, thank you for each new day where you protect us and love us.

Thank you for providing us the kindness and support we need to persist

Please give us the continued strength to find the joy and happiness in all situations

And to fight for Max and all those that need a voice.  Amen 

*Here is an article correlating swearing with intelligence that I stand by: 

Swearing is a Sign of More Intelligence, Not Less Say Scientists 

Also, Max came extremely close to receiving a blood transfusion, so just a gentle reminder to give blood if you can.  Preferably, O positive if ya got it :)