On August 8, 2001, I had my fourth child, AMBER LEE HEISS. My pregnancy was normal and the birth of Amber was normal. At around six months, I started noticing little things that weren’t quite right. She trembled when reaching for a toy. She wouldn’t bear weight on her legs and as the days went on, she started crying lots. At 1-1/2 years old, Amber was losing skills fast. She was crying ALL THE TIME. At 18 months she was diagnosed with Rett Syndrome. I started writing about our journey here and here. For more information on Rett Syndrome, go here. RS is a neurological disorder. There are around 1 in every 10,000-12,000 Rett Syndrome births all around the world.
Amber never walked. At four years old she got a Fundoplication and a Gastrostomy Tube. Amber continued to have problems with constipation and seizures. Other than that, she was quite healthy. I know that sounds crazy, but she was only on one medication for seizures and seldom got sick.
At 12 years old in April 2014 we found a lump on Amber’s neck. It was diagnosed as a Thyroglossal Duct Cyst. At that time Amber also started keeping her mouth open ALL the time. This made it extremely difficult for her to chew food and swallow. Although she had a g-tube, she was often able to eat. Liquids were much more difficult for her. In the last year she seemed to be more and more uncomfortable. Something definitely was going on. After numerous doctor appointments and differing opinions, it was decided the cyst needed to be removed and she needed Scoliosis Surgery. She needed the scoliosis surgery because her organs were being crushed because of her inability to sit up straight. The doctors all agreed her mouth was left open because of compensating to breathe. I was told it was like when we run, we breathe through our mouth, not our nose, to compensate.
We were able to get the cyst removed in November 2014. Amber was home the same day. On the way to the hospital I received a call. They told me there was an opening the following week for scoliosis surgery, instead of waiting for the scheduled February 2015 surgery. So we took it. On November 26th, 2014, Amber had scoliosis surgery. The surgery was about 7 hours. After much prayer, she was able to successfully come off the ventilator after 20 days. She was in Pediatric Intensive Care for a total of 26 days. Amber was able to come home four days before Christmas. Those first 48 hours were quite scary. I slept in the same room with her those first couples days. But she continued to get better. She was being weaned off of methadone and ativan during those weeks.
It’s funny all the things that didn’t really seem to matter, become so very important. Amber seemed to have a little trouble breathing four days before she died. I was a little concerned and even gave her two breathing treatments and was able to borrow a pulse/oxygen machine to monitor things. (It is important for me to note the doctors never suggested we use that machine. I borrowed the machine from another mom). She got better. I no longer used the pulse/ox machine. The third and second day before she passed away she would have episodes every couple hours of deep crying with tears that lasted about ten minutes. It was a sadness type of cry, not a cry because of pain. It is something we have never seen before. The intensity of sadness was heart-breaking. We attributed it to being weaned off the medications.
The day before she died, Amber was SO happy and really starting to get back to her normal self. It was so noticeable that her dad and I actually took pictures of her that day. We hadn’t seen much of her smiles in the last couple months, so it was a wonderful sight to see.
That night Amber was being silly. I held her in my lap (which wasn’t the easiest being she was 13 years old), and just loved on her more than usual. At 10:30 pm I put Amber to bed. I kissed her and told her I loved her. I laid her on her side. My son checked on her at 1:30 am before heading to bed.
The next day was going to be my first full day at work after missing quite a bit because of Amber. I awoke at 5:15 am and laid in bed for an extra half hour. I then got up at 5:45, let the dogs out and put wood in our woodburning stove. I then went to take care of Amber. I rolled her over and realized she wasn’t breathing. NIGHTMARE! Her body was still warm. I started yelling for everyone to get up. I started CPR and 911 was called. We all were begging Amber not to leave us and begging Jesus not to take her.
Paramedics came and took over. I rode in the front seat of the ambulance calling back to Amber to “Hold on. Mama’s here”. It was snowing out and the roads were very bad. My husband, Karl, followed in the car. At the hospital, we were told they did everything they could and asked if they could stop. We said yes. It was just like on tv. Everyone stepped back from Amber at the same time, the doctor looked up at the clock and announced the time of death.
Although it felt like hours, from the moment I found Amber to the moment she was pronounced dead, it was exactly one hour.
We now believe Amber was already gone when I found her.
Girls with Rett Syndrome do die in their sleep or suddenly. We just never thought it was going to happen to us. This was totally unexpected. We chose not to get an autoposy. Her cause of death was declared complications from Rett Syndrome.
