A Lil' Boy's Journey to Live….

Riley’s Tuesday with Lung Transplant went pretty good!
PFT’s (pulmonary function tests) looked about the same as last time.
Riley is weighing in just shy of 70lbs. So he has finally gained back the 15lbs he lost and added a few more. Of course this is because of his added nutritional boluses he gets everyday via his gtube. They would like to get Riley to the 25th percentile so he has about 7 more pounds to gain before we visit pulling back on any boluses. At that time he’ll need to be able to maintain and gain weight which Riley seems to struggle with just eating orally. We’ll cross that bridge when we get ‘back’ there.
We discussed more about the possibility of Riley having Chiari malformation. As mentioned previously the plan is to do a MRI when Riley’s inpatient in October for his bone infusion therapy. We talked a little more about Riley getting headaches. Before transplant Riley saw Dr Licht in neurology so Dr Goldfarb is going to loop him in with the MRI.
Still waiting on some lab results but what was back when we left looked good.
Riley did get his flu shot today and it played out much like when he gets an IV .. Riley freaking out! It’s crazy because just a few hours earlier he had about 10 vials of blood drawn and sat by himself with a little help from Buzzy and had no issues. As upset as he was he understood he HAD TO get the flu shot. He knows how Important it is to Protect his Precious #GiftofLife.
Monday Riley has appts with Plastic Surgery and Orthopedics. Followed by an appt Wednesday with Endocrine (bone health).
Thank you All for being on this Journey with Riley.

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

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Update .. 💙♻️❤

Update .. Heard from Dr Ryan (GI) and spoke to Dr Piccoli (GI) and Dr Goldfarb this week. GI docs are in agreement that Riley’s impedance probe and biopsy results looked pretty good. There was some minor inflammation in the esophagus and the probe showed some reflux. It was pretty minimal time over the 24 hour period. So the Thal is doing it’s job. We are going to add back a reflux med in the continuing effort to protect Riley’s lungs.
Dr Goldfarb said Riley’s BAL (lavage done during Bronchoscopy) was improved some since March. This is most likely because Riley isn’t drinking and therefore isn’t aspirating as much. There is still some cellular evidence of aspirating/refluxing but hopefully adding the reflux med and with Riley still not drinking that will continue to improve.
Riley has several appts coming up in September including plastic surgery, bone health, orthopedics and pulmonary. In October Riley will be admitted to CHOP for his next round of Zometa (bone infusion therapy). During that admission Riley will have the MRI to check for Chiari malformation.
Until then .. Riley is excited to be back in school and in the 5th grade!!

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

Update .. 💚

Update .. We are still waiting on the final results from Riley’s procedures last week. After some more research and talks with the team we are going to go ahead with the MRI to rule out Riley having Chiari malformation. This will be done during Riley’s admission in October for his bone infusion therapy. Riley does have several of the many possible characteristics listed under chiari malformation. Could also possibly explain Riley’s frequent headaches located at the bottom back of his head. We don’t know but MRI is the only way to find out. If Riley’s doesn’t have chiari malformation then it will be another thing we will check off the list. It’s horrible to have to continue to expose Riley to all this radiation but HE wants answers as much as everyone else. So we will continue to do whatever we need to do to give Riley the Best quality of life NOW!!

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

~ EDIT .. have learned since posting this that MRI’s don’t use radiation! Thank Goodness! Riley has been exposed to a lot of radiation already in his 11 years.

Riley’s triple scope was done on Thursday. Unfortunately there isn’t a cleft. We will have to wait till next week for the rest of the results from pulmonary and GI. Now we will go back to plastic surgery and see if surgically repairing Riley’s raised palate could help. Also going to schedule a MRI of the brain to see if there’s some abnormality that could be causing his delay in swallowing. This will be Crushing to Riley but we can technically tell him we’re still gonna try plastics, MRI etc. Just keep buying a little more time until we need to make quality of life decisions. ❤♻️💙~ Riley is still working through the whole process of getting an IV but he wanted to share how Great he is at getting it out with a little help from #OldTownRoad .. 😉

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.

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