Reiss: Uniquely Uncommon

SLCH Visit

It turns out that Reiss’ heart condition – the combination of all 5 defects, as well as the placement of her heart within her chest, and the placement and size of the components of her heart – is rare.  On top of that, her hemoptysis (coughing blood) is rare. IMG_3510[1] Additionally, she has migraines, which is rare for 2-year-0lds.  Reiss is rare and unique in every way. So much, that we have learned to look out for the rare and unique at this point.

Her Heart

We talked to Reiss’ heart surgeon this week about Reiss’ heart.  They believe left ventricle is small and the ventricular septal defect (VSD) is large.  They will know more once they get the 3D heart model printed (estimated done date:  8/12).  If he were to patch the VSD, trying to make Reiss’ heart a 2-ventricle heart, the patch would take up two-thirds of her left ventricle.

Also, she would need a conduit and valve for the 2-ventricle option.  These would fail and cannot be replaced with a cath procedure.  More surgeries equals more scar tissue.  More scar tissue equals longer time in surgery each subsequent surgery.  If she ever needs a heart transplant, this would also cause issues.

Lastly, for the Fontan, she would only be on the heart-lung machine for 15 minutes.  With the 2-ventricle option, it would be more like 1.5-2 hours.  Longer surgeries lead to a higher chance of blood transfusions.  Which each blood transfusion, Reiss’ body creates antibodies against the foreign blood.  In the case of a heart transplant, this would increase the possibility for rejection.

Hemoptysis

Reiss’ hemoptysis has the SLCH team of doctors baffled.  The pictures from the bronchoscopy should have shown more, given the amount of blood they saw.  (At least they were able to see the episodes in person.  Prior to this weekend, they could only go off of our iPhone pictures and verbal record.)

There are some studies that have shown that veso-dilators have helped not eliminate the episodes, but decrease the amount of blood in these episodes.  The thought is that the medicine will help relax the vessels carrying blood to the lungs.  This would decrease the pressure in the vessels.  If there are collaterals coming from these vessels, the pressure to them would be less and, therefore, less bleeding into the lungs if a collateral did burst.

They want to keep her on this medicine for at least 6 months to see if it works.  There are only about 100 published cases using this type of medicine and very few of those cases have the exact anatomy of Reiss.

Migraines

Reiss has been complaining of headaches almost daily for about two months.  One even woke her up in the middle of the night and made her puke.IMG_3491[1]

We relayed this information to our doctors while admitted into the hospital.  Afer a neuro consult, they scheduled Reiss for an EEG.

The EEG came back normal.  While it is uncommon for 2-year-olds to have migraines, it is not completely unlikely.  Roy’s family has a history of migraines and this is probably hereditary.  Additionally, there are things that show up on an EEG with people who have migraines – these were present in Reiss’ EEG.

What do now? Help reduce triggers.

  • Make sure she is hydrated
  • Make sure she has enough sleep  she should be getting 10 hours.
  • Stress and anxiety can be a triggers.
  • We can give her tylenol, but only 2-3 times/week.  Anymore than that can create a tolerance and even increase the headaches
  • Reduce caffeine intake
  • If she is having a migraine, move to a dark room and rest.

We will also be watching for food and drink triggers along the way.

Pre-Surgery

Reiss will go back to St. Louis for a cath procedure and echo on August 11.  They want to measure the size of the VSD, as well as look for collaterals that need to be plugged.  If IMG_3500[1]there are any collaterals that cannot be plugged through the cath, they will make plans to do so during the open heart surgery.

This visit should only be two days.  However, we will be prepared for a lengthy stay just in case.  As mentioned above, the 3D heart is supposed to be done by 8/12.  We have asked to sit down again with the surgeon to discuss Reiss’ case in-depth.  We are hoping the 3D heart will be available during these discussions to help with understanding.

Surgery

Reiss is scheduled to be in the OR on August 26th.  Her Fontan recovery will be 7-10 days.  We will know more details about surgery closer to the date.

Shirts

Surgery shirts are available!

Order your shirt between now and Tuesday, August 4.  I will need to rush our order to receive them prior to Reiss’ surgery date.  You can still order after August 4.  However, I will not be able to guarantee delivery by August 26.

Each order will be followed by an invoice emailed to you through paypal.  Invoices must be paid by August 4th.  Invoices paid after that date are not guaranteed delivery by the surgery date.

ORDER NOW!

A portion of the proceeds will be applied to Reiss’ heart walk team.  Everything we raise goes directly to CHD research and education through the American Heart Association.

Below is a screenshot of the website.  Reiss requested an orange shirt, like we thought.  The artwork in the background of the site is what will be on the shirt.  Once I have a proof, I will send it out.

shirt

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