A Lil' Boy's Journey to Live….

Riley had an appt with heart transplant and GI on Monday with a side of PFT’s.
Riley’s heart looks Beautiful!! Echo, labs and EKG all looked Great!
Unfortunately Riley’s lung function has not showed any improvement after switching to the inhaled steroids and finishing off the Augmentin. Not much we can do as of right now anyway. Riley will have a CT in August and will be have a bronchoscopy done during the triple scope August 15th.
Riley’s appt with GI went as expected. They also want to be a part of the triple scope. They would like to check Riley’s malrotation (post Ladd’s procedure), check the condition of his Thal fundaplication, do some biopsies and place an impedance probe. The probe is placed for 24 hours to record pretty much what’s going on with Riley’s gut .. like if he’s refluxing. Riley will be admitted for the 24 hours. The doctor we saw mentioned if Riley’s still refluxing changing his Gtube back to a Jtube like he had right after transplant. We explained to the doc that a Jtube would be another Quality of Life debate. He agreed and said it may be possible if need be to then just switched Riley’s existing Gtube to a GJtube instead of just J. Our experience has been with just a Jtube that they are hooked up 24/7 to a feeding tube. That’s not the direction we’re hoping to go but won’t know until August 15th.
Before August 15th Riley has some exciting weeks ahead! The week of July 22nd Riley is headed to the eastern shores of Maryland with CHOP’s heart camp for a week! Then the next two weeks after heart camp Riley is going to Liberty Lake Day Camp. We had to move up Liberty Lake camp because of August’s procedure but Riley is just Happy it didn’t effect his ability to go to camp!!
We don’t know what the future holds for Riley but for now we can promise him a Great summer!!!

#CHD #Heterotaxy #Transplant #Warrior #BeatingtheOdds Everyday .. 💙♻️❤

Update … Riley’s lung function has continued to decrease some over the last year or so. During PFT’s he did 2 puffs of Albuterol which seemed to help so he is going to start using an inhaled steroid twice a day moving forward. We did a respiratory panel while in pulmonary and are starting an antibiotic.
Next Riley saw ENT. The Laryngoscopy showed that Riley does not have vocal cord paralysis. The hope was that he did so it could be fixed and he could drink again. However where we thought this was the end of the road .. it’s not! Dr Zur said she can not rule out that Riley has a laryngeal cleft through the laryngoscopy. A laryngeal cleft could also be causing Riley to aspirate. So we signed consent today for Riley to have a bronchoscopy done by Dr Zur. If she finds that there is a laryngeal cleft we have also signed consent for an injection with prolaryn. This would be a temporary fix. Possibly up to a year at which time we could do it again or not or opt to surgically fix it. In the mean time Dr Zur would like Riley to see GI while also consulting with Dr Nance his general surgeon who did Riley’s Ladds procedure along with his various feeding tube placements over the years. Dr Zur would also like Dr Jackson (Riley’s plastic surgeon) involved given his raised pallet.
It’s possible Riley could have what they call a “triple scope” if GI and pulmonary would like to get in on the bronchoscopy.
Right now all of those details are still up in the air. Hopefully tomorrow we’ll know more.
For today .. there’s still Hope!!!

Tuesday Riley had his appt with lung transplant.

Labs all looked good. Still waiting on EBV. Riley did struggle some with pulmonary function testing so we will repeat the PFT’s in about 6 weeks when he sees heart transplant. Hopefully he can get his numbers up some. There will also be a repeat CT scan done towards the end of summer.

Riley’s weight is up to 63lbs from 58 when he was in for his bone infusion therapy last month. Can’t say we’re surprised given the added nutrition he’s being getting through his gtube since having to stop drinking by mouth due to the aspirating. No one is really quite sure as to what the goal is when it comes to Riley’s weight. The first 5 years he was in the one percentile. After transplant he bulked up to 60 percentile. As he learned to eat by mouth and was weaned down on steroids he went back down below the 10 percentile. Today he’s at 18. So for now since we are utilizing the gtube more since he can’t drink we are going to aim for 25 percentile and go from there.

We have not given up on Riley’s ability to drink again. He is working with speech to try and improve his technique. We are also in the process of trying to get Riley on Dr Zur’s (ENT) schedule for evaluation of vocal cord paralysis. She is already aware of Riley’s case per heart transplant and would like to see him. If Riley does have vocal cord paralysis there is a procedure called Renervation with EMG that could correct the problem. The last thing we want is to put Riley through another surgery but the last thing Riley wants is to never be able to drink again. That being said .. the phrase ‘quality of life’ was said more then any person would want to hear in an appointment. The reality is if we want to let Riley drink we could. He Will Be damaging his lungs. It’s almost looked at as he already beat the odds of being here 6 years post transplant and some people I guess would be happy they had that and therefore let Riley be happy Now by letting him drink. Knowing it will shorten his life but again we don’t know how much longer he’ll beat the odds so give him that quality of life now. That choice is on our shoulders. As Riley’s parents we’ve decided to not give that up yet. There are so many other things in Riley’s life that make him happy besides the ability to drink. We’ve taken away one thing with the hopes of finding an answer. We are praying that Dr Zur is that answer. If she’s not and the speech to improve his technique also doesn’t stop the aspirating .. we will then revisit the drinking versus quality of life debate.

We are in the process of planning a family meeting with Riley’s team. Also waiting to finalize an appt with Dr Zur. Riley has an appt with the bone health doctor in June and we will schedule his next heart transplant appt along with his CT scan and PFT’s.

Thank you all for being on this #Journey with Riley and our Family.

❤ Mom

#CHD #Heterotaxy #Transplant #Warrior #BeatingtheOdds Everyday.

Riley has been discharged! Some adjustments were made to his nutritional intake unrelated to the infusion therapy. He’ll return Monday to check labs. 💚

The face you make when your nurse is still able to draw labs off your IV.

~ Riley’s had another pretty good day. His calcium level is still fluctuating some so they are pumping him full of calcium. If we can get to Friday evening without any added side effects he should be able to be discharged. If not they’ve mentioned Saturday because Riley got the infusion so late on Tuesday. As always we will do whatevers best for Riley. ❤

#CHD #Heterotaxy #Transplant #Warrior #BeatingtheOdds Everyday.

Riley is finally settled in. Unfortunately after arriving Tuesday afternoon we had to wait a couple hours to actually get to the floor. By then shifts were changing and there were more delays. Riley finally started his Zometa infusion at 10:40pm after doing labs and starting a tylenol prophylactic. He does not appear to be having any allergic reaction .. Thank God. The possible side effects usually take about 24 hours before appearing. Obviously the Hope is they don’t appear at all! If they do we will deal with them as they come.
Thank you ALL for being on this #Journey with Riley! ❤💚

#CHD #Heterotaxy #Transplant #Warrior #BeatingtheOdds Everyday.

Riley had his Swallow Study today. Unfortunately it did show that Riley is aspirating when he drinks. At first it was thought to be thin liquids but now they are not sure if he’s also aspirating thickened liquids. Given the results Riley can not take Any liquids by mouth until further notice. As you can imagine Riley isn’t happy about not being able to drink. The team will discuss the results and get back to us on Wednesday with a plan. As mentioned in a previous post aspirating can cause chronic rejection. Riley has had mosaic air patterns in his lungs for a couple years now. At his last CT in March the mosaic air patterns were noted as being more prominent. Not sure what all of this means moving forward but will update when we know more. ❤

~ Pic is from while waiting to do the swallow study.

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