A Lil' Boy's Journey to Live….

Heart Camp. ❀️

Some pics of Riley having an Amazing time at heart camp last!! Thank you to Dr O’Connor, Cardiac Center at CHOP and all of the staff who donate their time to give our kids an Unforgettable week making memories. ❀️

Riley is off for a fun filled week at Heart camp with the Children’s Hospital of Philadelphia!!!

#CHD #Heterotaxy #LoeysDietz #Transplant #Warrior #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

Friday Riley had his FEES study at Children’s Hospital of Philadelphia. The first thing I want to say about the study is how Amazing Riley did! We are so Proud of him for his handling of everything from the numbing of his nose to inserting the camera to his participation in the study. He truly did Fantastic!
The study did show similar results to the swallow study in that Riley still doesn’t have great control over thinner liquids. We could see the ‘penetration without aspiration’. Years prior that was true of thin or thickened liquids but Friday we tried thickener again with much better results. The liquids went right down without any pooling along the way.
So the plan for now is to let Riley drink thinkened liquids. Dr Ruiz would like to follow up with a scope in about 3 months.
There are still some things we’re trying to make sense out of. For instance .. over the last 3 years Riley hasn’t really drank other then sips that were considered a treat. Usually soda was his treat of choice. Well in speaking to Dr Ruiz we realized soda doesn’t contain anything that would increase lipid laden macrophages in Riley’s lungs. Hmm. So that would suggest the increase isn’t caused by Riley’s sips. However if Riley is drinking or eating and refluxing that could be a cause, but than there’s the fact that Riley had a Thal fundoplication and is on reflux meds. Dr Ryan (GI) did share with us at our last visit with him a study that shows no connection between aspirating and increased lipid laden macrophages. So that leads to the ultimate question .. what is causing increases in lipid laden macrophages in Riley’s lungs? If we are able to rule out aspirating and reflux .. where does that leave us? Don’t have those answers right now. What we do know is Riley is able to control thickened liquids and therfore is able to drink if it’s thickened. So right now we have a happy 14 year old who is drinking some super thick Sprite!
Monday Riley has a orthodontists appointment and next Monday Riley leaves for heart camp. Riley LOVES going to heart camp, but to be able to drink their lemonade Riley says makes it even better!

CHD #Heterotaxy #LoeysDietz #Transplant #Warrior still #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

Update to today’s appointments with Dr Ryan (GI) and Dr Ruiz (ENT).
Dr Ryan shared an interesting study done in Boston that shows that lipid laden macrophages can be present in the lungs with or without reflux or aspiration. We have forwarded that information to Dr Josephson (lung transplant). Of course we know in years past that when we completely held fluids the lipid laden macrophages decreased in Riley’s lungs but more recent studies have shown that Riley wasn’t aspirating yet there was an increase in lipid laden macrophages. So the question is are Riley’s truly caused by aspirating or reflux or caused by something else all together? That’s what we are going to try and figure out.
When we met with Dr Ruiz he suggested doing FEES studies. It is similar to a laryngoscopy where they insert a small camera through the nose however with FEES Riley would drink liquids dyed green and they would watch through the camera what happens when he swallows. Where exactly does the fluid go. It was suggested to do it several times to get a better overall picture versus a single snapshot in time.
Now that Riley is 14 we can’t really be the ones to say yes .. he has to want to do it. In the office he wasn’t sure. Dr Ruiz did explain though that this could be the answer. Doing the FEES study could show if aspirating is really the problem and if it’s not it could open the door to being able to drink again. Of course once we got to the car Riley agreed to do it. So we’ll call tomorrow to schedule.
There was also talk about doing an endoscopy and placing an impedance probe, but we are going to do the FEES study first and go from there.
Fingers crossed that we are on the road to finding some answers.

#CHD #Heterotaxy #LoeysDietz #Transplant #Warrior #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

End of the school year!

Today was Riley’s last day of 7th grade!!

#CHD #Heterotaxy #LoeysDietz #Transplant #Warrior still #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

Riley’s appointments on Monday went well!
Labs, echo and EKG all looked Good. We met Dr Berger, while seeing Dr Rossano, who is joining the heart transplant team. Dr Berger will be a Great addition to the team. Very thorough.
Riley’s PFT’s (pulmonary function tests) were comparable to his last. Which is good. We discussed the results from the swallow study. Riley was quick to tell Dr Josephson that the study didn’t show aspirating, but the reality is during the last study it didn’t really either and a month later his lavage showed lipid laden macrophages. This time we aren’t repeating a bronchosopy so we really need to find the source before giving the go ahead to drink. Hopefully when we see Dr Ryan and Dr Ruiz next week we’ll come up with a plan.
Last appointment was with Dr Anari in orthopedics. Another amazing doctor we’ve added to Riley’s team. He too was very thorough. We spoke of Riley’s cardiac history and his newer diagnosis of Loeys-Dietz. He said it made sense because Riley presents more like someone with LDS than with cardiac history. There was a slight increase to Riley’s curve (scoliosis). He now measures at 21. Intervention occurs if the curve measures over 25. So we will keep an eye and see orthopedics again in 6 months and repeat an xray at that time.
Again overall the appointments went well. Riley will see both transplant teams again in 3 months and orthopedics in 6.
In the mean time Riley has about a week left of 7th grade!! Craziness! In the beginning of the summer Riley will go to Liberty Lake Day Camp for 2 weeks and the last week of July he has been invited back to heart camp with Children’s Hospital of Philadelphia! Riley is Super Excited for both!!

CHD #Heterotaxy #LoeysDietz #Transplant #Warrior still #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

Swallow study.

Update. Riley’s swallow study actually looked similar to the one he had a few years ago when he was cleared to drink, but without really using any techniques. He used a small straw, a regular size straw and no straw all with similar results. Chicken nugget seemed to go down without issue. The speech therapist was actually quite pleased with what she saw given Riley’s history.
We are not making any changes to Riley’s intake though until after we talk to his team and further investigate the cause of the ‘fatty cells’ in the lungs.
Given that Riley’s heart has looked beautiful for 9 years we have decided to start seeing both transplant teams on the same day (every 3 months) instead of one every 6 weeks. So June 6th he’ll see his transplant teams and then June 15th he’ll see Dr Ryan (GI) and Dr Ruiz (ENT). They may want to repeat an impedance probe. We shall see.
Until then .. Happy Memorial Day Weekend. πŸ‡ΊπŸ‡Έ

CHD #Heterotaxy #LoeysDietz #Transplant #Warrior #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

Results are in ..

The good news is the lung tissue that was biopsied was Negative for acute cellular rejection. Yay!
We know from the CT scans that there is some chronic rejection, but that is to be expected at this point post transplant.
The not so great news is the lavage showed increased (25%) lipid laden macrophages. This would most likely be a result of aspirating. Riley does not drink like the rest of us, for this reason, however for quality of life reasons he does take ‘sips’ on occasion. This may be the cause of the increased lipid laden macrophages. That being said Riley still struggles with eating. He pockets food in his cheek, and can sometimes take 45 minutes to clear his mouth. So it’s also possible that he’s aspirating some of the food. Another possible cause could be reflux. Most of Riley’s diet is through his gtube. Riley did have a Thal fundoplication after transplant to try and prevent refluxing, but a Thal is a partial fundoplication unlike the Nissen. Dr Nance was unable to do a Nissen because of Riley’s heterotaxy. So that would leave some room for reflux. Right now we just can’t be sure.
The other thing that was discovered from the bronch is low grade lymphocytic bronchiolitis. They aren’t overly concerned. It is believed to be caused either by the recent pneumonia or aspirating.
The plan is to head back to GI and see Dr Ryan and to also see Dr Ruiz in ENT. Dr Ruiz is newer to CHOP and specializes in swallowing. Riley will also have another swallow study done to see if he’s aspirating and does he also aspirate some food when he eats.
Everything in Riley’s world is a balancing act. Trying to do everything to give him the longest life possible .. while trying to give him quality of life now.
Happy Friday to you ALL and as Always
Thank you for being on this Journey with Riley and our family!

CHD #Heterotaxy #LoeysDietz #Transplant #Warrior still #BeatingtheOdds Everyday. β€οΈβ™»οΈπŸ’œ

Update .. πŸ’™

Update from Dr Drant ..

~ Good news! The measurements of Riley’s aorta from his recent cath are stable and show that his aorta is not dilating out of proportion to his body growth. The measurements of the ascending aorta (between the donor aorta and his aortic arch) fall into the upper limit of normal-mildly dilated range.

Still waiting for results from pulmonary. Emailed Dr Josephson who actually is in Boston at a Heart/Lung Transplant Conference. She is reaching out to the team back at Children’s Hospital of Philadelphia. Hopefully we’ll have results soon.


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