Baby steps.
In support of
The Gurbal Fam
View Support Registry
The Gurbal Fam
When we last updated one of our major concerns was John’s adrenal function after months of steroid use. The endocrinologist ordered the labs and said a cortisol of 2 would be “very very bad“ and a result closer to 15 would be best case scenario.
We had the labs done and his cortisol was 5, which felt like a punch to the gut and was not the news we wanted.
Since he had not been off steroids for long at that time, I asked the doctor if we could wait a few weeks and repeat the labs just to see if his adrenal glands would bounce back on their own. After a two week wait and the wait for the results we are ecstatic to report that his cortisol level is now up to 13!!!!!!!!! His doctor had already diagnosed him with adrenal insufficiency, and he may still carry that diagnosis, but the fact that his cortisol levels rebounded so well on their own is an incredible sign. They will continue to monitor the same labs, but it is our hope that he can remain off of prednisone and that his adrenal function will remain normal.
From a nose perspective- we saw his ENT doctor last week and the report we got from her was slightly confusing. The good news is that when she looked in his nose, she said that it is definitely open (but very small!) on both sides as far as she can see, and there is still no inflammation. He still has an extensive amount of scar tissue that will only ever go away with surgical removal. Because of his age and size and all that he’s already been through, surgical removal of the scar tissue is not a good option anytime soon. The doctor feels like he should be making more progress with his feeding based on how his nose looks on the inside.
The ENT doctor agreed that since they don’t feel like there’s much they can do to help at this time they want to bring in another team of doctors who specializes in craniofacial defects. On September 5, we will meet with a pediatric plastic surgeon that specializes in kids with defects similar to John’s. In preparation for this appointment, will have a CT scan of his face so that the doctors can get a better view of the deeper parts of his airway that are very difficult to visualize with the camera. The scan will take a few hours and he will have to be under general anesthesia (for the 9th time…) to make sure they get quality pictures. We were thrilled that they were able to get him in quickly and his scan is this Thursday. The results of the scan will be reviewed with us at his appointment with the plastic surgeon the following Friday.
Generally speaking, John is an extremely happy, go with the flow baby, and although some of the days are very intense and require lots of interventions, he is making improvements in every area. We finally caught up on all of our physical milestones, we finally learned how to roll over and can sit by ourselves for a few seconds. We like to stand up when holding onto things, and we don’t love being in a room by ourself. Since he can roll over, he can now safely sleep on his stomach, which is a huge help in terms of his breathing. His oralmotor strength continues to improve and we are working with his speech therapist to make sure he stays on track. At one point, not that long ago, he was getting 12 medicines a day. Now he is only on ONE medicine that he gets just 3 days a week.
He still really struggles to drink a bottle and requires suctioning at least 5 times a day (sometimes more like 15…). He gets 95% of his nutrition through his G tube, which isn’t ideal, but we are very thankful that we moved forward with placement so that we have a reliable and safe way to get him all the calories that he needs.
If you’re still reading, I’m sorry for the novel. His life has been very complicated from the beginning, and there are so many moving parts. Even on the hardest, most stressful days, we are extremely thankful for all the people supporting us through this difficult journey and pray one day this will all just be a “remember when.” we are even more thankful to live just outside of Atlanta where we have every kind of doctor that we could possibly need to help get back on track.
Specific requests at this time:
-A successful CT scan on Thursday without complication, with results that provide some amount of clarity.
-Extra patience and strength on the really hard days.
-For LJ’s life to continue to get easier.
We cherish you all. 🩵
We had the labs done and his cortisol was 5, which felt like a punch to the gut and was not the news we wanted.
Since he had not been off steroids for long at that time, I asked the doctor if we could wait a few weeks and repeat the labs just to see if his adrenal glands would bounce back on their own. After a two week wait and the wait for the results we are ecstatic to report that his cortisol level is now up to 13!!!!!!!!! His doctor had already diagnosed him with adrenal insufficiency, and he may still carry that diagnosis, but the fact that his cortisol levels rebounded so well on their own is an incredible sign. They will continue to monitor the same labs, but it is our hope that he can remain off of prednisone and that his adrenal function will remain normal.
From a nose perspective- we saw his ENT doctor last week and the report we got from her was slightly confusing. The good news is that when she looked in his nose, she said that it is definitely open (but very small!) on both sides as far as she can see, and there is still no inflammation. He still has an extensive amount of scar tissue that will only ever go away with surgical removal. Because of his age and size and all that he’s already been through, surgical removal of the scar tissue is not a good option anytime soon. The doctor feels like he should be making more progress with his feeding based on how his nose looks on the inside.
The ENT doctor agreed that since they don’t feel like there’s much they can do to help at this time they want to bring in another team of doctors who specializes in craniofacial defects. On September 5, we will meet with a pediatric plastic surgeon that specializes in kids with defects similar to John’s. In preparation for this appointment, will have a CT scan of his face so that the doctors can get a better view of the deeper parts of his airway that are very difficult to visualize with the camera. The scan will take a few hours and he will have to be under general anesthesia (for the 9th time…) to make sure they get quality pictures. We were thrilled that they were able to get him in quickly and his scan is this Thursday. The results of the scan will be reviewed with us at his appointment with the plastic surgeon the following Friday.
Generally speaking, John is an extremely happy, go with the flow baby, and although some of the days are very intense and require lots of interventions, he is making improvements in every area. We finally caught up on all of our physical milestones, we finally learned how to roll over and can sit by ourselves for a few seconds. We like to stand up when holding onto things, and we don’t love being in a room by ourself. Since he can roll over, he can now safely sleep on his stomach, which is a huge help in terms of his breathing. His oralmotor strength continues to improve and we are working with his speech therapist to make sure he stays on track. At one point, not that long ago, he was getting 12 medicines a day. Now he is only on ONE medicine that he gets just 3 days a week.
He still really struggles to drink a bottle and requires suctioning at least 5 times a day (sometimes more like 15…). He gets 95% of his nutrition through his G tube, which isn’t ideal, but we are very thankful that we moved forward with placement so that we have a reliable and safe way to get him all the calories that he needs.
If you’re still reading, I’m sorry for the novel. His life has been very complicated from the beginning, and there are so many moving parts. Even on the hardest, most stressful days, we are extremely thankful for all the people supporting us through this difficult journey and pray one day this will all just be a “remember when.” we are even more thankful to live just outside of Atlanta where we have every kind of doctor that we could possibly need to help get back on track.
Specific requests at this time:
-A successful CT scan on Thursday without complication, with results that provide some amount of clarity.
-Extra patience and strength on the really hard days.
-For LJ’s life to continue to get easier.
We cherish you all. 🩵
Comments
Nanacolefive
Susan Tindal