Emery's Angels

Hi, my name is Laura, and I am setting up a GoFundMe for my beautiful niece, Emery and her family. As many of you know, Emery was born on April 11, 2022, with an extremely rare genetic disorder called Primary Hyperoxaluria Type 1, which attacks multiple organs, including but not limited to the kidney, liver, and heart. Emery will require a dual transplant of the liver and the kidney.

It all began on July 2, 2022, when Sam and Janine brought Emery into the emergency room at Children’s Hospital because she wasn’t feeding as she should and was vomiting large amounts. They were worried about dehydration. While there, they performed a sonogram of the abdomen. Although they didn’t find what they were looking for, Emery’s kidney lit up like a beautiful blue crystal, signaling medical staff that they needed to look more into the kidney.

Emery’s first hospital stay in the PICU was 19 days long and included many tests, x-rays, sonograms, blood transfusions, surgeries, multiple blood draws, dialysis and more. Every time the doctors thought they had figured out a diagnosis, Emery would present with another symptom that would throw out the current diagnosis, and they would start from scratch. July 8, 2022, was her first surgery for a dialysis port and a kidney biopsy that would be analyzed and sent for genetic testing. On July 10, 2022, doctors suspected a diagnosis of an extremely rare and life-threatening diagnosis of Primary Hyperoxaluria Type 1. Official diagnosis would be a few weeks after genetic testing returned. Emery, at only 3 months old, was in end-stage renal failure. The disease had attacked and destroyed her kidneys in her 3 short months here on earth. Again, on July 10, Emery underwent surgery for another dialysis catheter placement.

Janine learned of a new medicine on the market that can help reduce the amount of oxalate produced by the body to cut down on some of the damage it causes. However, to get the costly medication, insurance must approve it, and then the drug must be sent to our local pharmacy. This process could take up to two months while Emery still suffers at the hospital. Days later, they discharged Emery to go home on July 21, 2022, and return daily for her dialysis. At least she could go home and see her older sister Evelyn (3) and her dogs Krueger and Louise.

A month later, on August 10, 2022, insurance approved the medication without having to conduct an appeal! There are only two pharmacies in the country that the pharmaceutical company will release the drug to; the medication was overnighted to one of them, and then that pharmacy will overnight it to our Children’s hospital. As the days passed by, Emery was getting sicker and sicker. Her nutrition was down, and she wasn’t eating well. The difficult decision was to place a G-tube to have nutrition directly given to her.

On August 16, 2022, Emery was back in the hospital and having surgery again. Hopefully, this would provide the nutrition and growth she needs to be 22 pounds for a kidney transplant. However, it led us to another side effect of the PH1…a bleeding (clotting) issue. She was discharged on August 18, 2022, for only 3 hours, when she started vomiting and defecating blood. Back to the hospital, she went. The bleeding stopped on August 20, 2022, and the good news was received. Oxulumo (the new medication) was here in Buffalo and at the pharmacy!

Emery was discharged home on August 22, 2022, with the idea that Oxulumo would be administered on August 26, 2022, after her dialysis appointment. Emery started bleeding again on August 24, 2022, throwing another wrench in the plan for the medication. Back to the ER, they went. Emery was throwing up blood again. Janine and Sam fought extremely hard to allow Emery to receive the Oxulumo, as the doctors wanted to wait until she was more stable. On August 26, 2022, Oxulumo officially flowed through Emery’s tired and overworked body! They returned home as a family again, only to return to the hospital on August 29, 2022, because Emery turned blue and lifeless that night. They couldn’t make it to Children’s Hospital in time, so they went to a closer hospital, where they intubated Emery and stabilized her. They were then taken by ambulance to Children’s Hospital, where she was again admitted to her second home, the PICU. Her blood pressure was too high, and with the amount of fluid, her body worked too hard and got tired. Again, a new medication was started and added to her already extensive regimen. Emery was extubated on Aug 30, 2022. On August 31, 2022, Emery had an echocardiogram showing her heart was enlarged, and she was now in acute heart failure. More medication was added again to the regimen.

September 2, 2022, Emery needed to be intubated again to undergo a CT scan to look at the anatomy of her heart. Again, that didn’t go as planned. She was too swollen and had to be given steroids to calm the inflammation to allow the tube to pass through. Due to this, she wasn’t able to be extubated right after surgery but was able to be extubated later that night. However, a few days later, on September 7, 2022, Emery’s little body was overworked again. She turned blue yet again, was re-intubated, and has stayed intubated until this current update.

Janine started reaching out to other hospitals to see if there was somewhere more knowledgeable on Emery’s condition and could provide more insight and different treatments for their beautiful baby girl, who deserves none of this. Pittsburg Children’s Hospital agreed to admit her, and now it was time to go back to the insurance company and have them approve the transfer. The approval came on Friday, September 9, 2022! Emery would be transferred as soon as a PICU bed opens at UPMC. On September 10, 2022, Emery was transferred via Mercy Flight alone because the seat reserved for a parent was taken by a doctor. After all, Emery was unstable. Her family traveled via car to meet her there. Emery remains in the PICU, where more tests, scans and procedures are being performed to see how they can help this deserving, beautiful baby girl who never asked for any of this, doesn’t deserve any of this and continues to show us she is a fighter.

We ask that you become one of “Emery’s Angels” and help her with a donation to support the extensive, expensive, much-needed care that Emery is receiving. If you are a parent, going to be a parent, have a niece or nephew, or have grandchildren, you understand how fragile a child’s life is and how it takes a village to help raise them and provide them with the love, care and support they need. Emery’s parents haven’t been working, so they can stay at Emery’s bedside at all times and take care of their other toddler, Evelyn. We are all in this together, and every little bit helps! Emery needs you as her angel right now. Please say a prayer and donate to allow her treatments to continue. We thank you, love you, and appreciate every one of you! Updates will continue so that you can follow Emery on her PH1 journey!