We had one perfect month before she passed away. I think God gave us that perfect month with Quinn. For one month she had no seizures she took her feedings well, and she did excellent in her occupational therapy. She was taking some pureed foods by mouth; a huge accomplishment for her. I really thought she’d make it through the first year. I knew that the majority of children with AGS don’t make it through the first year…but she was doing so well, I had no doubt. She was only 3 days short of turning 11 months old. She passed away on OCT 25, 2013, at 12:37 p.m. It was a Friday; I think it’s the worst day I’ve ever experienced in my life. I hope there’s never a day that tops it.
Quinn died on a Friday. I remember that the weekend before she’d been sick. She’d vomited a few times. This was frustrating, but not unusual. She vomited a lot. She was fed through a feeding tube that connected to a port in her belly that fed right into her stomach. She wasn’t able to tell us when she was full and her feeding pump just kept pumping at set volume formula right into her little belly. When it got too full she’d vomit, nature’s pressure relief valve.
I woke up OCT 21st, Monday morning, exhausted. Quinn had been fussy through the night. She hadn’t slept well, and so Barlow and I had tag-teamed through the night to hold her close and comfort her. We’d take turns closing our tired eyes for a short amount of time until the other would be deliriously tired and at his/her wits end. Barlow left for work by 6:45 a.m. I got Quinn dressed, planning on taking her to her Grandma’s. Barlow’s mother and father were watching Quinn during the day while I was at work. Shortly after I got her dressed she threw up. I cleaned her up and got her dressed again. I was trying to fix my hair when I heard her gagging again. I ran over the pick her up before she chocked. She vomited on her new outfit and on my work clothes. I cleaned her up again and laid her on the middle of our bed while I change my cloths. She then vomited on her clothes and on our bed. That was it! I broke down in tears. I called my work and explained I couldn’t come in because Quinn was so sick. I was really trying not to miss more days. I’d missed a lot work for appointments, occupational therapy, and hospital admissions. The Doctors and staff that I worked with were amazingly patient with me. I’m so grateful that they were so flexible. They had worked with special needs children and knew how hard families had to work to care for them. The rest of the day we rocked in the chair in her room and I gave her tiny amounts of formula through her feeding tube. She kept it down and started to perk up.
Tuesday morning she looked much better. She was making wet diapers and she kept most of her feedings down through the night. She did great in her occupational therapy with Heidi in the morning. She only vomited once a tiny bit. I took her to her grandma Hardy’s around noon. A Short time later Grandma Hardy texted Barlow concerned that she’d been vomiting. I’d asked one of the Doctors I work with look at her, since there were no openings at her Doctor’s office. We all thought she had gastroenteritis (stomach flu). Barlow took her home, and when I arrived he was pretty frustrated. She could only keep down 1 mL of liquid at a time. We had a hard time feeding her even through the tube after we got ~ 10 mL in she'd vomit. Tuesday night we set her up on her pump at 15 mL/hour. We had her in her bassinet in our bedroom at 11pm we heard her vomit and we found she was vomiting blood. I looked like coffee grounds, which wasn’t a great sign.
I was pretty scared. We had been through some pretty scary experiences with Quinn by now, seizures, not eating, fevers and 3 hospital admissions to Seattle Children’s. Barlow asked me, “On a scale of 1-10 how concerned are you?” I rated this as an 8.
We took her to the ED. They gave her an IV because of course she was dehydrated. They did some blood tests and the only thing abnormal was her potassium which is not unexpected. She perked right up after she got some fluids through her IV. The ED Dr. thought she had a gastritis that sometimes causes bleeding in the stomach. I hooked her up to her feeding in the ED and she took ~ 80 mL no problem so they sent us home with some Ondansetron (an anti-nausea medication). They usually use it for patients who are vomiting when getting chemotherapy.
We saw her regular Dr the next day (WED) they thought she had Mallory Weiss tears which is when you vomit so much that you break some blood vessels in the esophagus. That is how they explained the blood.
Thursday she started breathing fast. I rationalized that it was because she'd been vomiting a lot. She did this a lot when she wasn't feeling well. Grandma Hardy called again when watching her and was concerned. We brought her in to check her oxygen at the clinic I work at and she was saturating fine.
Friday was our anniversary. My in-laws were going to be out of town so we planned to celebrate our anniversary on Thursday night. After stopping by the Dr’s office she seemed to settle down. We took her to Grandma and Grandpa Hardy’s and we went out for a quick anniversary dinner.
Grandma Hardy texted me that she was worried about Quinn. Barlow was not too happy when I told him we had to end our date and go get her. When I saw her I felt sick. She looked gray she was breathing hard. We took her to my clinic I couldn't get an oxygen reading on her we went to the ED. I thought she'd aspirated (inhaled some vomit into her lungs). When I was checking in with the ED receptionist one of the nurses walked by and saw how gray she was. She picked Quinn right up and took us back to a room. . They had no rooms open so they kicked a poor woman with a sprained ankle out of a room so we could be seen. They hooked her up to oxygen and she pinked right up. We had some men from our church come give her a blessing. They did a chest X-ray and found her heart was enlarged and it looked like she had pulmonary edema. About 4 weeks earlier she’d been treated for a small pneumonia and her x-ray hadn’t had any signs of a heart problem.
It was a real struggle to get an IV in her because she's so chubby and she was dehydrated. The nurses tried to place the IV using ultrasound and we finally got a good line. They planned to transfer us to Seattle Children’s. Barlow went home to get clothes for us. The staff had planned on sending us to the hospital us by ambulance to Seattle. (It was too foggy for us to go by helicopter). We had to wait for Barlow to come back, because he had the car seat in the car. I didn’t know that they used the car seat to transport a baby in the ambulance. She didn’t like being strapped in and she let out a little whimper… that’s the last time I heard her cry…
I rode in the ambulance with Quinn and Barlow drove our car. The ride out went smoothly. I was still in denial about how sick she was I thought she was going to be fine. We'd been through things like this before and she'd been fine. I had to ride in the front so I couldn’t see Quinn. She was quiet and seemed relaxed through the drive. I chatted with the Ambulance driver and I tried to distract myself for worrying.
When we got to Seattle they got another IV in her and started doing some tests. They did a blood gas on her and I knew the numbers they read of were bad. They hooked her up to an epinephrine drip to help her heart contract more effectively. They did another X-ray I could see it this time. After I saw her X-ray…I knew we were in big trouble. Her heart took up nearly ¾ of her chest. There was no room for her lungs to expand. They put a tube in the breath for her, put in more IVs and did more tests.
The Dr. Sat down with us and explained that her heart was only working at 25%. They explained that they were doing all they could but the next step was ECMO (where they bypass the heart and lungs, they take the blood out oxygenate it and pump it back in to the body). I knew that once we got on ECMO the next step was a heart transplant (Quinn was not a candidate due her AGS) or they would have to fix the underlying problem with her heart. When they told us that I knew... I knew that she was going to die.
I tried to explain it to Barlow and he was understandably upset. I was still really numb. I started calling my mom (my father passed away 2 years ago) and my sister they were going to get the first flight out of SLC that they could. Barlow called his parents to come out to Seattle Children’s as soon as they could.
After talking to some specialists and Quinn’s primary doctor on the phone, we decided not to do ECMO. They thought that was her neurological condition (Aicardi-Goutieres) was the cause of the cardiomyopathy. The odds of them finding a way to fix the underlying problem were very slim. On ECMO she may have been in more pain, and it would prolong the inevitable. It was a hard decision; because I didn’t want to give up hope. We weren’t ready to let go; I don’t know that we ever would be.
We had some people from church give her a blessing and a name. Barlow's parents made it in time to hold her. We held her and we had them extubate her (take out the breathing tube), give her pain medicine and stop the epinephrine. She didn't cry and she didn't struggle. She just didn't ever wake up...
They let us hold her for a long time before one of the doctors finally came in and called her time of death. She’d been gone for several minutes before he’d come in… I think they were letting us hold her one last time before hearing those words. The nurses took out all the IV’s. The volunteer at Seattle Children’s made imprints of her hands and feet for us. Another wonderful Volunteer from Solumination took pictures for us. At some point they asked about doing an autopsy, and we gave permission to use any of her organs that they could. Barlow’s parents went to the airport to pick up Barlow’s brother who’d boarded a plane in SLC as quickly as he could. Barlow was exhausted and fell asleep on a couch in her room I called my mother and my sister.
I was alone with Quinn for quite some time. The nurse came in and asked if I’d like to wash her. I removed all of the bandages from her little body. I washed away all the iodine. She had so many little puncture wounds in her little arms from all the IVs. I was still in such shock it began to wear off and I had a little break down I cried and cried. I woke Barlow up sobbing and begged to leave. He checked with his parents they were on their way to the hospital with Abram and wanted to see her one last time. I asked Barlow to stay with her while I walked around. I didn’t want her to be alone, but I couldn’t stay in that ICU room for one more moment. I somehow made it outside and walked and walked. Seattle Children’s has some nice grounds and was grateful for a place to walk. Eventually Barlow’s family came back and saw her one last time. Grandpa and Grandma Hardy started the long drive home. Abram stayed home to drive our car. Barlow and I had been up for well over 24 hours and were no condition to drive. We said our sad goodbyes and left Seattle Children’s. We had spent so much time at Seattle Children’s over the last year it had begun to feel like our second home. I think that made it a little easier to leave her, because we knew she’d be treated well and with respect. We picked up my mom and sister from the airport and Abram drove us home. It was and is the most painful thing I’ve ever been through.