Here we are with another update.
My Gallstone is still there and still causing pain. I have two surgical consults coming up. Hope to have a plan forward pretty soon regarding all that craziness. There is only one stone, but it’s a large stone. Who knew one stone could cause so much havoc.
Addyson had a repeat orthopedic appointment today to discuss her wrist. She has had pain for weeks in her wrist. We went 2 weeks ago, they said, do another two weeks of bracing and come back and see the wrist specialist. So Randy took her today. The dr believes that her body had another FMF Flare (her fever syndrome flare), but like a feverless flare, as she never got a fever. However her joints are inflamed. They said it’s like rheumatoid arthritis. If the flare lasts 6weeks or more, then come back.
Recap on FMF: Familial Mediterranean fever (FMF) is an autoinflammatory genetic disorder that mainly affects people of Mediterranean origin. FMF is characterized by recurrent episodes of fever and serositis (chest, abdomen, joints), leading to painful attacks early during childhood. Amyloidosis is the most fatal complication of FMF. Early initiation with colchicine leads to improvement in the attacks and prevent amyloidosis.
They do feel that her daily anti-inflammatory meds are providing coverage for the FMF to keep her safe from amyloidosis (With amyloidosis, the protein amyloid A, which is not typically found in the body, builds up in organs ⸺ especially the kidneys ⸺ causing inflammation and interfering with their function).
Addyson is monitored closely to make sure her dose of Colchicine is working well, because if not there can be life long Complications. The Inflammation can lead to complications such as:
Kidney damage. Amyloidosis can damage the kidneys, causing nephrotic syndrome. Nephrotic syndrome can lead to blood clots in your kidneys (renal vein thrombosis) or kidney failure.
Joint pain. Arthritis is common in people with FMF.
Infertility. Untreated inflammation caused by FMF may affect the reproductive organs, causing infertility.
Other complications. These can include inflammation in the heart, lungs, spleen, brain and superficial veins.
We are also doing all the needed research to decide who will be doing addyson’s big chest wall repair.
So APRIL is still crazy, with required testing for the next surgery. We are hoping surgery can wait until the fall (OR MAYBE even next year) however, we know that her “dent” is getting larger and starting to shift her vital organs, so we are monitoring things closely. This surgery stresses me and Randy out more than any other surgery we have done to date.
Thank you to everyone who has helped me with my spreadsheet (yes, I’m using one to track our options). To everyone who is helping us weigh our options. offering to help us juggle things for when we actually do the surgery.
This surgery has so many unknowns. Like can they do it through the side, or do they end up cracking her chest. Does she end up needing her aortic root fixed at the same time? Aortic root repair is open heart surgery. And we still haven’t gotten to the root of why is her aortic root dilating all of a sudden? Cardiology is still trying to figure this out. This surgery is just terrifying — Knowing they are so close to her heart/lungs/etc. We will use no surgeon who isn’t a thoracic surgeon, but feel safer with a cardiothoracic surgeon. This surgery comes with a lot of concerns even post surgery. We have been following multiple families who within 3-6 months post op had collapsed lungs and fluid around heart/lungs, so even getting home doesn’t mean the worst is over. This girl likes to test Randy and I’s nerves. People keep saying we aren’t given more than we can handle but I do not believe this. Why put a kid through these illnesses, these journeys, these battles. There is no need for this for anyone. 😦 Yes, I’m just in that kind of funk right now. I promise I will move past it, eventually.
Ethen is getting ready to turn 13 (I still can’t believe it) so we have a few things coming up for him. A fun weekend in BOSTON, watching the REDSOX/O’s game at FENWAY!!!! Plus he has his team impact signing day on the 7th!
Below are our upcoming appointments. This is just a glimpse into our schedule. We are both also working full time, and Randy coaches 3-4 times a week. Dealing with a chronically ill child is a lot, but we love her so much.
4th: Addy cardiology
5th: Michelle surgical consult
7th: Addy PFT/MST Test & Ethen’s GW Signing Day.
10th: Ethen DR Visit
11th: Michelle surgical consult
25th: Addy CHOP visit (GI and CT)
28th: Addy CHOP Visit (AADP and Surgical consult)
3rd: Addyson metal testing
17th: Ethen genetics
7th: Addy endo
16th: Addy cardiology
23rd: Addy pulmonary
28th: Addy Dr Appt
We also have a friend whose daughter is in the hospital. We are thinking of them and sending them so much love. They are already on day 14, with no discharge date in sight. We love you all Sofia and Pam!!!