Brooklyn's Battle with JEB: Your Help Matters!

Hello!

Brooklyn was born with Junctional Epidermolysis Bullosa. It is a genetic skin condition. We did not know about it while I was pregnant. We found out about it after she was born. We got the final diagnosis from genetic testing about 2-3 weeks after she was born. She was born on June 5th, 2023, and was transported a few hours after being born to the U of M Masonic. We really had no idea what was going on, but it was apparent to my delivering doctor there was some issue with Brooklyn. She had a hard time keeping her oxygen up for one, and she was missing skin on the bottom of her right foot, her head, and bottom, and was missing 3 nails. They transferred her a few hours after being born. It was definitely a scary moment for us. I remember getting to the cities with my fiancé (Aaron, Brookie’s dad) completely unsure what was ahead or the outcome or anything for that matter. We were scared, worried, unsure, anything of that matter you name it. It was very overwhelming when we first got there, all of the specialty doctors seeing her and checking her. She was only in the NICU for 5 days, then we were sent home with a whole lot of things on how to care for her skin and things to be using for her and things to not use on her skin. Unsure if we were ready and what the road ahead looked like, we were headed home regardless. Being home was scary for a while, but then ended up being fine. We were always on edge and always worried about her, or more so the JEB and what could happen. After 12 weeks, I went back to work, not really wanting to leave Brookie because again the JEB/what could happen when I wasn’t there. She has always been an amazing, easy-going baby. She had always had a baseline stridor; it was never addressed that it was a real big issue, just something to monitor during follow-ups in the cities and with her primary care physician. Fast forward life to January/February 2024, Brookie had been having worsening stridor upon doing tasks she didn’t like: for example, suctioning her nose out. It had gotten to a point where she would get so upset her stridor was worse, and she would turn a grey/blue and was covered in sweat. On February 23rd, 2024, a Friday, we had brought her into the ER in the cities, and she ended up being intubated and sedated on the spot. It was the most horrific experience I had ever seen bedside in a hospital. I was asked by many clinical staff if I wanted to go out of the room with her dad. I kept telling them, “No, I need to be right here with her! I can’t leave her!” I remember both Aaron and myself sobbing and hearts breaking and again in that same spot of not knowing what was to come for her and the future. She was intubated and sedated that time for 8 days. She had a PICC line placed and TPN for nutrition. They had her sedated to let her airway heal and to self-dilate. After she was woken up, she sounded a lot better, and her wounds were looking better also. But then she started having mini seizures, which was alarming because that had never been an issue prior. We consulted with neurology to make sure this was not something new to add in with everything else. Testing was done, and everything was normal. They stated that it could have been happening from decreasing sedative medications too quickly, so they adjusted them again, and things were okay. After 21 days, she was released with hopes of just doing dilations every 3 weeks to keep the airway open. This was the game plan going forward. We went home on March 15th, 2024. We had follow-up visits planned for April 15th, 2024, which we had gone to. ENT was her last to see for the day, and he was very concerned with her stridor and it being more severe than usual. He admitted us into the ER and had told them to not sedate or intubate her right away, to hold off this time. The plan was to sedate and intubate in the OR the next day. So, on April 16th, 2024, she was sedated and intubated again for a Laryngoscopy with Bronchoscopy. After this was done, they had shown us how small and restricted her airway was. It was measuring too small, and that had been the reason she was going through the turning grey/blue and being very sweaty. She was breathing through an airway that was too small. Ideas were discussed with her team on what to do; they mentioned dilations still and/or a tracheostomy tube. There had been an incident in the early AM where B wasn't in a deep enough sedation, and she had moved too much, and her intubation tube fell out. CPR was performed, and they had gotten her re-intubated. Neuro was consulted again after this happened, and luckily nothing with her brain function was compromised. We were not there when this happened; we were staying at a hotel close by. They called us around 5:30 am (or so?). Now that this had happened and her team discussed more in-depth, her long-term realistic best option would be for her as dilation didn’t seem like a great idea for her anymore. They stated a tracheostomy would be the best route for her airway and for her health overall. They also stated that a g-tube should also be placed for nutrition as well. This news was a very heartbreaking one to hear. Like that this was having to be done for our daughter. When they told us, my heart literally felt like it fell out of my chest. I cried a lot. It was hard to hear for us at first, and we heard all the pros and cons to it and decided if that’s the only route, then it needs to be done. Which looking back now, I think I was scared to care for a tracheostomy tube because I had no idea what to do! Also, adding on, she had a central line this time as a PICC did not work for her as it had the time prior. TPN was her nutrition source again (till the g-tube was placed). We had A LOT of training on the trach and CPR with a trach. Cares and everything needed to care for a trach and keep her healthy/healing after it was placed. They told us about supplies and how to keep her skin safe and protected with a trach. We had training on her g-tube as well, of course! This time after she was woken up after sedation, they weaned her off slowly since the time prior was not ideal, to say the least. Before we got to go home, we both needed to have completed multiple trach tie changes/cares to show that we were comfortable doing them at home. I remember it being so stressful learning all of this and crying behind closed doors. 45 days later, we were discharged on our way home with a goal of only going down for follow-up visits!! Yes, this is a short summary of things, but without going into a deep, deep dive, these are things that are important. She had her 1st birthday on June 5th! We celebrated with a party that weekend with family and close friends! We did 3-ish month follow-up visits. Seeing her PCP as needed. Has a great RN. And still now, we are doing the same thing (basically) going to the cities for follow-ups! We have added on seeing the specialty dentist that works with the U of M Masonic as well. She is anemic, and her hemoglobin is low, so we have also added hematology to our list of specialties we see!! So we do see them every 4 weeks! Which is fine because infusion days aren’t super hectic, they just aren’t Brookie's cup of tea. Getting an IV is not anyone’s idea of a good time. But she always does amazing and is in good spirits after it’s placed!

We are nearing her SECOND birthday soon!! We cannot wait to celebrate her.

Today, right now, as I am writing this, I smile, now. Getting the trach and g-tube placed SAVED her. I do believe that! God is always walking beside her and keeps her safe! Everything about Brooklyn is perfect. She’s an amazing toddler and is always growing and learning new things! She is tough, resilient, strong, sweet, loving, sassy, feisty, smart, and so much more. We are so blessed beyond words can explain!

Like I said before; this is nowhere near everything that has ever happened to her. There are other things in between it all, but this is a general overview of her! As life is more complex and more in-depth!

Anyways, I just wanted to finally set up a GoFundMe (I have been asked multiple times to do so!) for her as there are things that medical doesn’t cover and is an out-of-pocket expense for us, we have gone back and forth on another medication dermatology has suggested, it isn’t guaranteed to help as nothing ever is, but her insurance doesn’t cover it for that reason. If we decide to give it a go it is a bigger expense for us out of pocket. We aren’t wanting to reach any certain goal. This is more if something comes up and insurance doesn’t cover it and it’s a larger expense, we will have this to help out!

Thank you for reading and being a part of Brookie’s JEB journey! We appreciate you!

Blessings and much love from our family to yours!