I wanted to have a blog post soley devoted to Jaylie and her Pulmonary Vein Stenosis journey thus far. Jaylie is 15 months old right now and started her journey with PVS at 8 days old. Because of the history we had with her brother Jude, we decided to have an echo on Jaylie for peace of mind. Much to our horror and shock, at 8 days old her echo was preformed and showed she also had PVS!! When we got that diagnosis that day, my husband and I thought for sure we would be losing yet another child to this disease because that is all we had known. We did our research and called several doctors, desperately trying to find the right place to treat Jaylie. PVS to the majority of the medical field is considered a death sentence and wont even try to treat your child. Through a sweet mom and freind, we were put in touch with a doctor in Colorado who then directed us to a doc at Texas Children’s Hopital in Houston. This genuinely nice doc was a God send for our Jaylie. When Jaylie was 4 weeks old she was careflighted to Houston to be treated by this doctor. Jaylie was honestly knocking on deaths door when she met Dr.J. Without the heart cath and intervention, Jaylie would have only lived a few more weeks. She had all 5 pulmonary veins narrowed with 2 completely occluded. (most of the time there are only 4 pulmonary veins but some people have 5) She was also having a battle with jaundice but after the first cath and having her veins opened up, it cleared up within a few days. Here is a rundown of her 8 heart caths to date:
Cath 1: 4 weeks old– (5 drug eluding stents, one in each vein)
Cath 2: 8 weekd old– 5 more drug eluding stents, one in each vein to extend out past the end of the original 5 stents bc she had more narrowing right beyond the stents.
Cath 3: 3 months old– Ballooning of all 5 veins, she had narrowing inside the stents and a little beyond. At this point we were given a choice: start Jaylie on a drug called Sirolimus with the hopes that it would help keep the veins open and slow the growth of scar tissue build up or go down the path of lung transplant. With much prayer we decided to try the Sirolimus but also do all the work up for lung transplant, just in case it ended up coming tp that.
Cath 4: 4 months old– This cath had to happen in somewhat of an emergent fashion so we did not have our normal cath dr and one of his partners treated Jaylie. He used drug eluding balloons on all 5 veins.
Cath 5: 5.5 months old– Ballooned all 5 and put an additonal stent in 1 vein. She needed a blood transfusion this time around. After this cath, she started on an iron supplement once a day.
Cath 6: 8.5 months old– Ballooned all 5 and made the stents bigger.
Cath 7: 11.5 months old– Ballooned all 5 and made the stents bigget.
Cath 8: 14.5 months old– NO intervention needed!!!! Some slight narrowing but not enough to intervene this time. WOW!! This was a shock and a huge blessing, thank you Lord. Right after this cath she got her first Mehta Cast for scoliosis. This is another part of Jaylie’s journey I will be blogging about a little later.
With each cath in the beginning, she always had narrowing just beyond the stents but not in the stents. After we started the Sirolimus the narrowing stopped beyond the stents and only narrowed within the stents. Our Dr’s belief is that the Sirolimus is controlling the progression of the narrowing to a certain degree. She does have narrowing within the stents each cath but that is something that can be addressed with ballooning. We have HOPE!!! This is not something we had much of when she first got her diagnosis. We praise God daily for Jaylie’s life and how He orchestrated every detail to get her to where she is now. PVS is so unpredictable and we know this, but we also know we serve a mighty God who has given us more time with Jaylie than we had with Jude or even dreamed of having with her. We were privileged to celebrate her first birthday, that in and of itself is a miracle. God is still in the miracle working business and our baby girl is living proof!!
My hope with this blog post is to tell our story with PVS in hopes that it will one day help another family that is researching how to treat thier child. Our daughter is our hero and she is kicking some PVS booty!! Her story is far from over but this is her story to date.
Cath #9: 17 mo old—enlarged all stents a little and dilated some narrowing. Her RV pressure was 39….this is the lowest it has ever been!! The cardiologist was so pleased and surprised. Jaylies 2nd Mehta cast was placed after the heart cath. She will have another cathertization in 6 months. She is also still taking the Sirolimus daily.
Cath #10: 24 months old—enlarged all stents and 3 of the stents were cracked open, bc they had hit their max size in diameter. 2 of the cracked stents stayed open but one had to have a regular bare metal stent placed inside it to keep it open. Her RV pressure is still holding steady at 39!!! She did have some tissue build up in all the stents. After this cath, she had her 4th Mehta cast put on.
Cath #11: 2 years and 8 months old– 6th cast
Cath #12: 8th cast . intervention on only the left lower vein, cracked stent and enlarged vein. the ballooning caused a psudo anyerism so a stent could not be placed at this time.
cath #13: no cast, follow up from cath #12 to put a stent in Left lower vein.