Time to try some Duocal! The plan is to stop Riley’s morning feed and instead to eat breakfast. Then eat lunch at school with the rest of the kids. After school he’ll get his gtube feed with Duocal added along with some vitamins. Riley will still need to go to the nurse at school for a water bolus since he is not able to drink for hydration due to aspirating. Fingers crossed that Riley is able to maintain his weight/growth. Last time we tried transitioning to eating orally Riley continuously lost weight. As Riley’s parents, though, we believe it’s time to try again and made the request. We will monitor closely and if he struggles with eating enough or starts losing weight we will reevaluate.
Riley’s appt with Dr Josephson (lung transplant) went well. Pulmonary function test results were about the same as they have been. Labs all looked good except for the tacrolimus level (immune suppression) which was a little high. So we have lowered the dose and will check it again next week. When Riley did labs they also collected for the Covid vaccine study. Hopefully sometime in the fall we’ll have a better idea of how much protection the booster shot is providing. We don’t know anything in regards to the renal ultrasound other than you could see they were measuring cysts. Question is how do things look compared to the previous ultrasound. Surely if there is anything concerning we will hear from nephrology. If we don’t hear anything Riley has an appt already scheduled for October. For now .. Riley is Super Excited to go back to school (in person) on Thursday! Yay to some kind of normalcy not just for Riley but for All the kids!
Quick update .. Riley’s heart transplant appointment went well! Labs, echo and EKG all looked Great!! Always good when there isn’t much to update. Riley’s Super Excited that today was his last appt before heart camp next Monday! Yay!!
Riley’s visit to CHOP went well. He will see Dr Mancilla (Endocrine) again in 6 months. Prior to that appt he will have another DEXA scan to check his bone density. Endocrine is extremely happy with the results of Riley’s previous Zometa infusions but with Riley still growing any new bone growth will not have been treated. So for now he will skip another infusion and see how the scan looks in 6 months. Thankfully no new fractures have been noted from CT and they’ll check the spine again when Riley has his next orthopedics appt and xray for his scoliosis. Riley also had ‘research’ labs done as part of the Covid vaccine study he’s participating in along with additional labs that Endocrine ordered. Flex xrays of Riley’s neck were ordered by Dr Drant (cardiologist seen for Loeys-Dietz). Pretty sure this was to get a baseline but if there is anything of concern Dr Drant will be in touch. Riley’s next appt is in July to see Heart transplant. Then soon after that Riley will be off to Heart camp!!
Riley appointments on Tuesday went well. PFTs and labs were all within ‘Riley’s range’. Dr Josephson asked if Riley would be interested in being part of a study on the antibodies after receiving the Covid vaccine in solid organ transplant recipients. 100% Yes to that!! So far studies have shown that SOT recipients appear to not have many antibodies even after their second dose. We already figured that would be the case but participating in the study will give us Riley’s actual numbers and if the vaccine offered any other type of protection. Also to be a part of what could be done to offer SOT recipients more protection. Riley also saw Dr Drant in cardiology. We further discussed Riley’s Loeys-Dietz diagnosis. Really nothing new to add to what has been previously posted. We were able to visually see though the blood vessels in the back of Riley’s head, as seen in the first picture, that have tortuosity. Uncommon for the average person but common with LDS. The good news is the vessels don’t tend to change over time. So they won’t get worse. The second picture is of part of Riley’s aorta. From the root which is donor to his native aorta. One can see from the picture that there is some dilation in Riley’s native aorta. We are being assured that the measurements are not concerning at this time but will be continuously monitored through echo and when Riley has cardiac caths. The type of SMAD2 mutation that Riley has, so far, has shown to cause more issues a little later in life. That being said Riley is still case 1 of 1 when it comes to the SMAD2, Loeys-Dietz, Heterotaxy, Complex Congenital Heart Disease and heart lung transplant in a patient. So we will continue to watch closely and deal with any issues as they may arise. For now Riley is headed into summer one Happy kid!! He received an invitation back to heart camp CHOP. As long as there is enough space for any new kiddos to attend Riley will be going to heart camp in July! He also is Super Excited to be going back to Liberty Lake Day Camp for 2 weeks in August! Riley has spent 95% of his time at home since March 2020. He’s ready to get back out and Live this #GiftofLife that he has been given!!