Update 5/16/2015: This subject is so painful, that I never updated this page with the results of the autopsy. Erin died from Anaplastic Large Cell Lymphoma. Mr. Shaun, Erin’s dad, talked to the original St. Jude’s doctor that she saw and learned that it isn’t always detectable and that they tested for that more than once. He also learned that if she had been diagnosed and lived, she probably would have gotten it again. She was tested for cancer many times and it never showed up until the last few days of her life. We are, of course, devastated, and never knew that she was dying. We miss her terribly and thank you all for your continuing support and love.
As I mentioned, we still as of today 8/30/2014 do not know what specific illness Erin had, or what ultimately caused her death. But let’s start at the beginning.
In October of 2013, Erin started having a stiff neck. The previous October, she also had pain under her arm. We thought she had pulled a muscle (then) at gymnastics, and told her to stretch. When it got bad, we took her to the family doctor who found swollen lymph glands and prescribed antibiotics. 5 weeks later, they did an ultrasound and they were huge, but around a week later the pain subsided and the swelling went down too. We never thought, a year later, this would be the case again. This time she was in karate, having quit gymnastics after sitting out 2 months the year before. But on November 14 as I was putting her to bed, I saw a huge lymph node above her collar bone and I got her out of bed and went to Huntsville Hospital Women’s and Children’s ER.
We probably spent 5 hours there for the doctor to tell me that her CBC was absolutely normal, but that he was pretty darn sure it was some sort of infection and so he prescribed 10 days of augmentin. I felt better and we breathed a sigh of relief. However, in 10 days another lymph node had swollen as that one went down. This time we made an appointment with an ENT, who prescribed another 10 days of antibiotics. When that didn’t work, we made poor Erin have a needle aspirated biopsy, which she had to be put to sleep for, on her birthday December 11, 2013. That was the first appointment they had, and by this time we were worried and needed some closure. I still feel bad about that, but it made us feel a little better at least.
Let me stop to mention, that during this time the pain in Erin’s back near her shoulder blade had become very bad. It kept getting worse, and she woke up all night crying. She also developed a small rash on her left wrist area, which the doctor said was too small to bother with. And no one was concerned with her back pain.
You can imagine our confusion when that came back “negative.” Negative for what? Really all that we got out of it was that they tested for bartonella (Cat Scratch Fever) and cancer, but needle aspirated biopsies don’t always find things so we were referred to the HHW&C’s St Jude Affiliate Clinic. We made an appointment in early January and saw a Hematologist/Oncologist there.
This doctor was flabbergasted at the size of Erin’s swollen lymph nodes. They were pretty huge, but that was her pattern. One went away and another came up – huge – during this time. Her ESR, CRP, and Ferritin levels were also very high. This doctor frustrated us because her only focus was on cancer. If she didn’t have lymphoma, she didn’t know what she had, and didn’t even bother to refer us to anyone else.
Again, the back pain was severe at this point, but they would say “Oh, it is just scoliosis” and dismiss it as not related. A full lymph node biopsy on the left side of her neck was scheduled for late January, and the results came back on Valentine’s Day 2014. Not cancer – WHEW! But wait, that was the worst night for pain she had and she screamed all night. By this point, she couldn’t move on her own hardly and certainly couldn’t get up and lay down (which required back muscles), and she had been out of karate since early January.
We arrived at HHW&C ER, again, around 10 am on February 15, 2014. The ER doctor was rather dismissive, asking why we were there. She said, this is for acute situations and emergencies. Pain isn’t an emergency. And so we told her what we had been through. Sometime around 3pm, they had spoken to the radiologist who did the pre-biopsy CT, and to the doctor at the affiliate clinic, and they agreed to do an MRI. It took 3 hours, because Erin was in so much pain she couldn’t lay still. Around 8pm, the doctor came back and said she has osteomyelitis and we are referring you to Children’s in Birmingham. The reason: we have not got the expertise to finish a diagnosis or to treat this.
We left Huntsville for Birmingham and arrived there around 10:30 pm the night of 2/15/14. The doctors there were also baffled, but finally they decided she needed a biopsy of her T2 vertebrae because that’s where the “bright spot” was on the films. They got neurosurgery to agree to do it, and she had her first “real surgery” on 2/18. They got a sample, and they were going to release us on 2/19 but the surgeon wanted one more CT scan prior to release. That’s when he saw a “gelatinous mass” and requested to go back in. On the morning of 2/20, he got it all and she did so well we were released that day. She was still in pain, so we spent the next two days at a Hilton Garden Inn in Birmingham to let her rest before the 2 hour drive home. We were hopeful. And look how good she looked – this is a video of her helping my mentally disabled uncle walk my cousin’s dogs on 2/22/14.
They didn’t get anything from a culture, so a few weeks later they put her on Clindamycin. 10 days into that, she ran a fever for the first time, and they readmitted us mid-March and inserted a PICC line, and put her on Vancomycin. We were there 6 days so that the PICC would settle in the right place. She was on the Vanc for about 10-14 days before she was again running a really high fever, so they pulled the PICC and put her on Zyvox. Around this time she was feeling pretty great, and she had gained the weight she lost back (nearly 15-20 lbs). But her labs still didn’t look great. By the way, in March they told us that the only thing that grew from her T2 biopsy was something called P. Acne, which is considered a contaminant because it’s on everyone’s skin. It actually causes acne.
She was on the Zyvox until May7. She had been short of breath and continued to complain of pain in her upper chest area on the right side. An x-ray showed an opaqueness there, and so they readmitted us. On May 9 she had a surgery to biopsy the area, but it turned out that her upper right lobe was stuck to her ribs. Removing that caused a lot of blood, so they had to insert a chest tube. She also had a broncoscopy which showed great looking lungs. But she recovered rather quickly, keeping the tube for only 2 days. And they got 20 samples of bone and tissue. Great right? No – nothing grew from any culture that we were aware of.
Her condition was deemed inflammatory at that point, although the infectious disease team didn’t let us go. Rheumatology and ID wanted us to go to Iowa to see a specialist in a disease called Chronic Recurrent Multifocal Osteomyelitis, or CRMO. It is very rare, and the expert in the US is at the University of Iowa Children’s Hospital. Erin was put on Indocin (a strong NSAID) and our appointment in Iowa was made for July 17, 2014.
She did great all summer, and we had around 2 months between the May hospital visit and the July appointment. We had to put off a lot of stuff as time went on – “we will do that after Iowa” I said more than once. The Saturday before we left for Iowa on Monday, she started running a fever – for the FIRST time in her illness – at night. This time there were no drugs that could cause it. I thought, great her body is fighting whatever this is. That was echoed by the doctor in Iowa, who did a full body MRI and told us immediately that day that she believed Erin had a bad infection. Her entire torso was opaque almost on the MRI. She said, let me consult (multiple people) and come back in the morning. We did, and she told us to drive back to Alabama and make arrangements to go to Children’s. She said, if she gets worse along the way, check into the nearest ER and have them medflight her back to Birmingham.
That day we drove to Illinois to stay the night with one of her two best friends, who had moved there a year or so ago. Erin really wanted to see her house, and I’m glad we got to do that. When we pulled into Huntsville on Saturday 7/19, expecting to go to Birmingham on Monday, we got a call asking us to come that evening. So we went home, grabbed clothes, and left for Birmingham. That was the last time that Erin would be home. I think she knew, because she cried as she hugged the dog. And she had not done that at any point prior to that.
We checked into Children’s, and they decided to start over with everything. So multiple groups got called in. They were going to do another biopsy, and wanted to plan it very carefully, so it wasn’t scheduled until Wednesday of that week. She was getting visibly sicker each day, but I didn’t know how sick she was until it was upon us. Fluid was building up in her chest cavity, compressing her lungs, and I didn’t know it but that meant the inflammation and illness was causing her capillaries to leak. She had the biopsy, and immediately she had lungs that sounded like she had bronchitis. They said, that’s normal. She also developed a marbilized looking area on her chest. They said, that’s a rash, we will keep an eye on it. She held her own for a few days, but the fluid built up and so on the next Wednesday she was scheduled to have fluid removed via needle. They removed a lot, and yet her lungs were still full. This is where she began to decline in earnest. She wasn’t hungry, wouldn’t eat, and was in increasing pain. At this point she had another PICC line too and was on some strong antibiotics. They had re-cultured a hot spot on her T2, and it again grew P. Acne. This time it wasn’t a mistake. Still they said her lungs were normal in being “full of stuff” but they prescribed respiratory therapy to break it up.
On Friday of that week, she had to have more fluid removed. It was A LOT. I forget how much now. She had chest tubes and had been meant to go to intensive care due to the amount, but they successfully extubated her and sent her to special care instead. She was on morphine with two chest tubes, and so she wasn’t awake a lot. I still did not understand how serious this was. How did I miss it?
Sometime during the night, the nurse was bugging her because she still had not urinated and she had been on fluids. Erin was talking, though, and awake. She gave herself some morphine and let the nurse do a quick catheter, and then as I was helping her with something she said “Mommy, you have bad breath” and she smiled. I said I bet I do! She gave herself another dose of morphine, then her breathing was not very good. We thought she had too much medicine, but the nurse was worried. Before we knew it the ICU team was taking her out of the room, pumping her full of fluids to stabilize her blood pressure, and we didn’t see her until after 6am.
That day, Erin was on some sort of breathing mask. It was hard for her to talk, because you couldn’t hear her. She was still struggling to breathe, but I still thought it was temporary. My aunt and cousin came that day, and I spent part of the day in the waiting room with them. They had brought a wrapped gift, and before they left we went in to ask Erin if she wanted to open it then or later. She wanted Michelle to open it for her right then, and when she did Erin smiled at her. It wasn’t long afterward that Erin could not breathe very well at all, and her vitals showed that. She was desperately trying to tell us something and her voice was so weak I could not hear her. Shaun gave her a piece of paper to write it to us, but she could only write gibberish. It was Sunday evening, and later on we realized that she was asking us to put Big Brother on the TV for her to watch.
They told us around 10pm that they were going to intubate her, and that they would have her sedated when we came back. When we returned, they had braided her hair to get it out of the way, she had a breathing tube, and she was paralyzed and sedated. I guess at that point I was worried and shocked, but I still did not doubt she would recover.
The next ten days were a blur. About 4 days in, she was doing a bit better and they unparalyzed her and woke her up. That was the last time she was awake. She moved her legs, her arms, and opened her eyes. She was in pain and was able to nod and tell me she was. I thought she was getting better, and I spent the night at a hotel. Shaun told me early the next morning before I left, that they had to re-paralyze her. I still didn’t get it.
Erin died around 12:20 am on Thursday August 14, 2014 (Wednesday night). On Tuesday, they told us that she was circulating oxygen but that her mitochondria in her cells wasn’t using it. I had a terrible feeling that day, and I got into the bed depressed at 8pm and didn’t get out until almost 10am on that Wednesday morning. I kept seeing an image of her in my mind, one that I ignored, of her at around 3-4 years old holding her bunny and in some person’s arms. She was facing me, and looked like she was sad. I rallied that day, and I said I know I have to be strong for her. She actually did better that day and they were hopeful, but around 4pm her lung started bleeding. They told us they had one more hail mary, and that was to put her on an oscillator with some substance that would help the lungs to absorb oxygen. She was on the oscillator all evening, and vaguely improved once but then her vitals all started crashing. Around 11:30pm or so, I was laying down half-asleep and I heard as clear as a bell “Bye Mommy.” I immediately got up and asked Shaun what we should do. We both knew it was time to talk with the doctor, and he said that at this point it was not survivable. When they turned her life support off, she died within a few minutes. It was so quick. Her little fingers started turning blue almost immediately, and I knew she was gone.
I have put off writing that last paragraph for several days, because it is very painful to relive. We told the nursing staff that we did not want to stay for them to clean her up. The last few days had been utter hell, and I knew her soul had left her body, and I just wanted to leave. There was no point in staying another half hour to put ourselves through another round of the hell of saying goodbye. We were both holding her hand when she died, and that was it. Leaving the hospital with our two wagons of our stuff and her stuff, was surreal. I had said I would not leave without my baby but there I was, at 1am leaving without her, never to see her again. You cannot imagine what that was like unless you have had to do it yourself.
I never thought, until her last day alive, that she would die. If I had been afraid of that I might have done something differently, although I don’t know what. I do know that I feel as if my ignorance of that deprived me of saying goodbye to my baby, and of helping her through what might have been scary. I spent days with her helping her go to the bathroom, watching her decline, yet I never thought she would die. Maybe that’s how it was supposed to be, because being in ICU was hard enough. Maybe we all got what we needed. But I will forever second-guess and I will never kn