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!!
Riley’s CT and CTA results are in. The CT for his lungs did show some increase to the air trapping or mosaic air patterns. What that means is there appears to be more bronchiolitis obliterans or chronic rejection then at the last CT in October. Thankfully Riley is not symptomatic so for now we’ll continue to monitor. If symptoms started to appear that is when an open lung biopsy will be discussed but thankfully we are not there at this time. The CTA showed arterial tortuosity or curvy vessels towards the back of Riley’s neck. Though it’s rare this is a typical finding with Loeys-Dietz. There was no aneurysms present. We did inquire if this could be the cause of Riley’s frequent headaches but have been deferred to Dr Licht (neurology) for those answers. So for now like pretty much everything else in Riley’s life we monitor. Riley will continue to Live the Life he so Loves to Live and we’ll cross each bridge when we get to it.
Update .. Riley’s genetic testing that was pertaining to his kidneys has come back with no new genetic findings. The thought is that the kidney cysts are related to the previous findings of SMAD2 but aren’t completely sure at this time. Further research is being done. What does this mean for Riley’s care as far as the kidney cysts? At this time we will wait to see nephrology again until October. People can live with the cysts forever without issue. For others it can turn into a bigger problem leading to kidney disease. We don’t know at this time which way this will go for Riley. Honestly this is really the case with everything Riley! We just don’t know! So we’ll continue to face each battle as they arise and Riley will continue Loving the life that has been so Generously gifted to him.