I apologize for not updating in so long. Life got very heavy for a while there. Kaylee was really struggling in school, we were struggling with juggling all of the out-of-town appointments, and she kept getting sick. Things did take a little break which has allowed me to recharge and reset. 

Over the last month a lot has happened. Kaylee had her 6th upper GI scope and a colonoscopy. We are waiting for a telehealth with the GI doctor from Mayo to go over the results of that. She had an episode of dizziness combined with vision loss causing her to fall. This is the first time she has ever fell when symptomatic. This landed her a trip to the ER since any falls require a CT scan because of the von Willebrand's Disease. Everything turned out fine and she got a liter of her super juice (saline)! Her ferritin is trending down again. The HTC at Mayo wants her to receive iron infusions when her levels drop below 50 but her HTC at Children's doesn't want to do it until levels drop below 20. Since Mayo is her primary HTC but Children's is the one that can order it they came to a compromise and will treat her once levels fall below 30 and levels will be checked monthly. 

Kaylee's pulmonologist worked so hard to get her in sooner than the 20-month waiting list with the geneticist. Dr. Starr with MMI in Omaha agreed to see her via telehealth, pro bono, on her own time! Kaylee had this appointment last week. Dr. Starr agreed that genetic testing was necessary. She will be testing for Primary Ciliary Dyskinesia, Immunological disorders, Autoimmune Disorders, some Liver Disorders, as well as casting a "wide net". Today we received the testing kits in the mail. They will test Stephen, myself, and Kaylee. I believe it takes about a month for them to get the results. We have truly been blessed with some fantastic specialists along this journey! 

Additional immunology testing yielded more puzzling abnormal results. If genetic testing doesn't provide any answers her immunologist would like to refer her to a more specialized immunologist. There are three in the country. One at the Jewish hospital in Denver, one at Harvard, and one at the National Institute of Health. She has had 5 sinus infections, 2 ear infections, and one or two upper respiratory infections since February, and she is currently sick again. We have to figure this out!

Friday, she has a follow up with the Boystown doctor. June 12th, we head back to Omaha. She has a liver MRI at 6:45am on the 13th then will see the endocrinologist. Endocrinology will investigate if there is an issue with low aldosterone. She has a follow up appointment with her pulmonologist in July, and her 6th month follow up with her complex care pediatrician at Mayo in August. The end of September she will finally have her appointment with the liver specialist. 

Monday of this week Kaylee had her 3 month follow up with Dr. Klaas, her complex care pediatrician at Mayo. I cannot explain the connection they have. Kaylee is so comfortable with her. Dr. Klaas validates her, hears her, and sees her. This allows Kaylee to be active in her medical care and me to take a supporting role. The original medication that Dr. Klaas prescribed for Kaylee's POTS is helping! It has brought her blood pressures up and her heart rates down. Unfortunately, she is still experiencing lightheadedness, dizziness, and vision loss. As I do with every other specialist, I asked Dr. Klaas why Kaylee's body doesn't seem to hold onto the salt and fluids that she takes in. Dr. Klaas gave Kaylee 3 options, increase the med she is already on, add a mineral steroid that will tell her kidneys to hold onto the fluid she takes in (this should improve the dizziness and vision loss), or do nothing and see how the summer goes. She told Kaylee the decision was hers but if she didn't want to make the decision then we would make it for her. Kaylee made the decision to add the new med AND she told her why she was making the decision. I love love love seeing her making informed decisions for her own care!!! Dr. Klaas and Kaylee talked about how Kaylee needs to prioritize things in her life taking her health into consideration. She explained that this is Kaylee's life now and that she will need to adjust her choices on a daily basis in order to try to live as normal of a life as she can. Given that Kaylee is now taking 12 pills every day, and 18 pills on days when she's been bleeding, I asked if there was a chance of ever getting off of the meds. Dr. Klaas said it was unlikely and that Kaylee will likely need these for the rest of her life. This was something that was a little difficult for both Kaylee and I to digest. BUT, there may be a glimmer of light at the end of the tunnel. We were talking about Kaylee's upcoming endocrinology appointment. I said that they would specifically look at conditions that cause low aldosterone. When Dr. Klaas said that this new medication acts as aldosterone in the body I swear you could see a light go off in all three of our heads! This new med (essentially aldosterone) is supposed to help Kaylee keep the water and salt she takes in and improves blood pooling in extremities. What if Kaylee has always had a condition that causes low aldosterone, and it left untreated has caused her Dysautonomia? If this is the case, and the condition is treated, will her Dysautonomia go away? We are cautiously optimistic, but this is the first time there has been dots to connect!  Kaylee will need to go off of the med a couple days before seeing the endocrinologist, so it doesn't mask anything during testing. This medication can throw off her potassium and sodium so these will have to be added to her monthly ferritin checks as well. 

Overall, I think this little break in appointments/procedures has done us all some good. We've been able to rest a little and get caught up on other pressing matters. We have both been able to recharge a bit and a good appointment with our favorite specialist always helps too! It's disappointing that her appointments with Mayo are not covered by insurance but the care they provide is priceless and worth every last penny!