This time of year, from March through May, I am flooded with memories. Memories that are in direct contradiction to each other, memories that have completely different emotions to them. Back in March 2017 Ariella had just begun her treatment. Our days were awful, our lives put on hold. We were scared and sad and feeling helpless. And we had so much hope. Less than a year and she would beat this thing. We could get through a year and then we would be back to normal. Her prognosis was good. In between hospital visits, when Ariella was in between treatments, those brief periods when she was feeling well, we could live our lives. We had a lot of fun. We even had fun times in the hospital. We had some terrible, terrible times in those early days, especially just trying to figure out this new life, adjusting to our new normal but I never felt completely defeated. Honestly I followed Ariella’s lead. I can’t say she never complained but she mostly had a great attitude. She never doubted she would get better and she cared more about comforting other children. And I would do anything to go back there. For better or worse those hospital rooms were our second home. We knew the doctors, nurses, child life staff and volunteers. We got to know other families. We decorated the rooms and brought comforts from home. Even when times were better, when Ariella finished her treatment, I missed those days. We had fun but there was also a sense of safety. While Ariella was there, we were fighting the cancer, keeping it at bay. Once we were out of those rooms we were doing nothing to stop it from coming back.
In March 2019 everything had changed. The promise of a cure from a bone marrow transplant quickly vanished when Ariella had to be admitted to the ICU, and then a week later was intubated. Of course we still held onto hope because without hope, you just can’t continue living. But I knew, I just knew if she was intubated she was not going to come off life support. I tried not to despair. We had some steps forward. But ultimately with each step forward there were larger steps backwards. As the weeks went on I lost more and more hope and began to fear more and more that we would lose her. Days and nights were spent staring at her vitals, listening to the beeps. We were terrified and exhausted and completely helpless. Ariella would have a great day here or there which inevitably would be followed by another setback. There was nothing fun about those days, just constant vigilance. I do not look back at those days with any sort of fondness. Even when Ariella’s room was overflowing her last day, visitors spilling out into the hallway, waiting to say their goodbyes. I’m glad people got to say their goodbyes. But following those moments I have to remember the machine being turned down, step-by-step until it was no longer keeping her alive, hearing Ariella take her last breaths, and feeling her leave us.
With the calendar having turned over to May I’m not filled with the sense of renewal that the promise of spring and warmer weather often brings, but instead filled with dread about facing the anniversary of the day Ariella died. Remembering those awful days leading us to making that impossible decision. Leaving the hospital without our girl after spending over 2 1/2 months there just hoping we would be leaving as a family, only coming back for check-ups. We went into the hospital on February 19, 2019, one week before her transplant, filled with hope and excitement, ready to celebrate her re-birthday. We left the hospital May 9, 2019 as a broken family of two with a hole that can never be filled.
I’m sure everyone is familiar with Chaos Theory. That one very small change early on can cause a much larger difference in a later state. I often wonder how this plays into Ariella’s illness and death. If we had caught the cancer sooner, when she first complained of leg pain, would this have changed her outcome at all? Would she have responded better to the chemo? Would she still have relapsed or would have starting treatment earlier changed that? Her scans that showed the relapse were done on a Friday. If we didn’t have the scans until the following week would that have changed anything? Treatment would have started a bit later getting meaning her bone marrow transplant would have been a bit later. Would that have changed the ultimate outcome? Maybe she wouldn’t have been exposed to the flu. Maybe she wouldn’t have gotten so many infections. Maybe, maybe, maybe.
We of course will never know if the outcome would be different. But these “what ifs” torture me. They frequently pervade my thoughts. I replay all the various scenarios over and over. If only we had done this, or if this one thing didn’t happen then maybe the rest of it wouldn’t have. It seemed like we were in the midst of this perfect storm where Ariella was getting bombarded with every possible infection and complication. What if just one variable had changed? What if just one thing had been done differently? Would we still be here without our daughter?
People are going to say to stop doing this to myself. It was nothing we did or didn’t do and tormenting myself about it won’t change anything. Logically I get that. Running these thoughts through my brain over and over does not serve any purpose. And yet I cannot stop. Because the thing is, Ariella dying never once crossed our minds when we were considering bone marrow transplant. I fully believe that one small change initially would have altered the outcome drastically. Whether it was the timing of transplant, administering of different meds sooner, a different doctor on duty earlier on in the ICU stay, etc. And this only contributes to my anguish. Because her death, at least following transplant, did not have to happen. And I will never come to terms with it.
If only we could turn back time. I’m actually not sure where I would turn it back to. Maybe to when she very first started complaining of leg pain. If we caught the cancer sooner maybe she wouldn’t have had any of those micrometastatic cells that didn’t respond to chemo. I’m not sure I would turn it back to when she relapsed. I don’t know that we would do anything different except maybe start alternative treatments sooner. I would turn it back to BMT and not do it. But once again, who knows what effects those changes would have on the greater outcome? I am sure that if we could go back and not do the transplant Ariella would still be with us. I can’t say for how long but could have been a lifetime.
Not only am I facing a lifetime without my daughter, without the person that completed me, but I am also facing a lifetime of questioning every decision that was made. If only, if only, if only.
Ariella trusted us. From the time of diagnosis she trusted that we were making decisions based on her best interest. She dreaded chemo, dreaded radiation, dreaded all of it but she never really argued about it because she trusted us when we said it was what she needed to do to get rid of the cancer. We never kept things from her. When she was getting ready for her limb salvage surgery she knew it was a possibility she would wake up with part of her right leg amputated. But still she trusted us that the surgery was the right decision. We never promised her she would be cured. But we always said the doctors were doing everything they could. When treatment was working we shared that and when it wasn’t we shared that as well. She never lost hope because there were always other options. I remember one day getting scan news that wasn’t what we wanted. When I picked her up from school and told her about it and told her treatment would be changing she said “that’s okay, at least I still have options.”
When it came time for the bone marrow transplant Ariella was understandably scared. She knew about the radiation prior and the high dose chemo that would once again make her feel lousy. But once again she trusted us when we told her she would get through it just like she got through all her other treatment and it was her best chance to get rid of the cancer once and for all. And she went into the hospital with a positive attitude. Up until she started feeling the affects of the chemo she was dancing and singing with friends, playing games, roaming the hospital, and playing pranks. She was no longer scared. She trusted us and the doctors that all would be okay.
This is one of the images that plagues me. Ariella so hopeful and optimistic in the hospital, acting like she didn’t have a care in the world. Ariella receiving the bone marrow without complaint. Ariella and I watching TV and hanging out in her hospital room just talking and cuddling in bed together. Getting texts from Ariella when I wasn’t there. All that time just waiting until her blood counts came up so she could get out of there and get on with her life. She trusted me when I talked about all the things we would do once she had clearance from the doctor. She had no doubt at that point that everything was going well and it was just a matter of time before she would be sprung from Hopkins.
I guess this goes along with my regret and guilt. Ariella trusted us that the bone marrow transplant was the right thing to do and yet it ended up being the cause of her death. We let her down. We made the wrong choice. Yes I know hindsight is 20/20 and we made the best choice we could have with the information we had at the time but that still doesn’t change the fact that the two people who are supposed to keep her safe failed to do so by authorizing a treatment that has known severe complications. I see her happy and okay one minute and then everything changed in the next when she was intubated and scared she was going to die and always had sad eyes. And we couldn’t effectively comfort her. We couldn’t keep her safe and we couldn’t make her feel better.
Along with a lifetime ahead of missing Ariella and missing the memories we will never get to have, I have a lifetime ahead of having to live with those images of Ariella with sad eyes in the ICU, wondering how it all went so very wrong. She thought she would be fine, she’d get through BMT and have a lifetime of fun and friends and dancing ahead of her. At some point in the ICU she realized that was not the case and she might not be okay, even though we told her she would be. I made it a point to never lie to her about her illness but I didn’t think I was lying when I told her she would come through BMT just fine. I never thought death was a possibility. I just hope she never blamed David or me during that time. I know I blame myself enough. If only, if only, if only.
July is sarcoma awareness month. In honor of that I am going to share some facts and share Ariella’s story for those who have only gotten to know her in the past several months.
From the Sarcoma Alliance: What is Sarcoma? Cancerous (malignant) tumors of the connective tissues are called “sarcomas”. The term sarcoma comes from a Greek word meaning fleshy growth. Sarcoma arises in the connective tissue of the body. Normal connective tissue include, fat, blood vessels, nerves, bones, muscles, deep skin tissues, and cartilage. Sarcomas are divided into two main groups, bone sarcomas and soft tissue sarcomas. They are further sub-classified based on the type of presumed cell of origin found in the tumor. They all share certain microscopic characteristics and have similar symptoms. Sarcomas can develop in children and adults. For children under 20 approximately 15 percent of cancer diagnosis are sarcomas. Although rare, there are approximately 15,000 new cases of sarcoma diagnosed each year in the United States. In general sarcomas are divided into the large groups: soft tissue sarcomas, and bone sarcomas.
Ariella had Ewing’s Sarcoma (from Stanford Health Care website) What causes Ewing sarcoma? The majority of Ewing’s sarcomas result from a chromosome rearrangement between chromosomes #11 and #22. This rearrangement changes the position and function of genes, causing a fusion of genes referred to as a fusion transcript. Over 90% of individuals have an abnormal fusion transcript, involving two genes known as EWS and FLI1. This important discovery has led to improvements in diagnosing Ewing’s sarcoma. Similar to osteogenic sarcoma, trauma or injury is sometimes involved with the site at the time of diagnosis. However, this trauma is thought to bring the condition to attention rather than to have any causal relationship. Some doctors classify Ewing’s sarcoma as a primitive neuroectodermal tumor (PNET). This means the tumor may have started in fetal, or embryonic, tissue that has developed into nerve tissue.
Ariella began complaining of pain in her right leg in September 2016. However this was following an injury, she had accidentally gotten hit by a chair in school. Ariella was a dancer and she pretty much only complained of the leg pain at that time after she had been dancing. We did have it x-rayed which didn’t show anything and we were advised to have her rest the leg for an extended period of time so it could heal. Since the pain did get better with rest we just figured it was injured further with dancing and didn’t think anything of it. At the same time, Ariella had lost an alarming amount of weight which we were working with her pediatrician to figure out. As part of diagnosing the cause of the weight loss blood work was done. Blood work came back normal. Sarcomas cannot be detected in the blood. We did not at all connect the leg pain to her weight loss. Her pediatrician was unaware of her leg pain since it came and went, not sure if he would have made the connection or not. Rest seemed to help so she continued dancing but by the end of January the pain was constant, not just when she used her leg. It was also red and swollen. She saw an orthopedist who was concerned with what he saw on the x-ray and sent us next door to his office for an MRI. Not 20 minutes after the MRI I received a phone call that she had a tumor, it looks malignant, and a referral to the orthopedic oncologist. We saw the oncologist the next day, January 27, 2017 and had more tests and scans. She went in for a biopsy Monday January 30, 2017.
I actually don’t remember the exact day we had the official confirmation of cancer but it took several weeks after that to determine that it was Ewing’s Sarcoma. Here we are, anxious and wanting to start treatment immediately but had to wait for the type of cancer because all cancers are treated differently. February 27, 2017 we got confirmation. Luckily the cancer was localized to her right tibia. We then had to meet with the pediatric oncologist who would detail the treatment plan.
Ariella began her treatment in March, 2017. It started with placing a Hickman Catheter, which ended up causing a pneumothorax requiring a chest tube. This delayed treatment further as it took a good 5 days at least for the pneumo to heal and the chest tube to be removed. Then finally, treatment started.
Ariella endured 17 rounds of chemo which required her to be inpatient every other week in the hospital for at least 2 or 5 nights. She pretty much had every side effect you could have; hair loss, nausea, fatigue, neuropathy, etc. She had fevers several times requiring ER visits and an infection that required her Hickman to be removed and replaced, causing another pneumo requiring another chest tube. However when she was not in the hospital you would never know she was sick. She continued going to the dance studio (even though she couldn’t dance), spending time with friends, and having sleepovers.
After the 6th round of chemo the chemo was paused so Ariella could have her limb salvage surgery to remove the tumor. Rather than using an implant the doctors decided to place an external fixator to allow Ariella to regrow her own bone.
Ariella had the fixator for 51 weeks but eventually learned to walk without crutches and even dance in her recital.
Ariella rang the end of treatment bell December 28, 2017. She had her Hickman removed January 2, 2018 and returned to school that same week. The kid was amazing. She jumped back into her life like nothing happened. We were relieved when her first set of routine scans came back clear and devastated when the second set of scans June 1, 2018 did not. This time the cancer came back in her lungs, acetabulum, and sacrum. Ariella did have her fixator removed the following week.
This time Ariella had a port placed instead of a Hickman, which allowed her to have a decent summer. Treatment was outpatient and we were still able to go on our planned trips to Disney World, LA, and Ocean City. Ariella even went to school when on this chemo. She went through 2 different chemo regimens which were not working and finally went on an oral pill. She also had radiation to all the various spots. The pill and radiation were what got her stable, showing no evidence of active disease. We got this great news on January 25, 2019 and we began preparing for bone marrow transplant. The hope was that her new bone marrow would kill any residual cancer cells.
Ariella was admitted to the hospital on February 18, 2019 for pre-transplant radiation and chemo. The transplant was on February 26, 2019 with her father as her donor. Though she didn’t feel well after her transplant things were going well, until they weren’t. On March 7, 2019 she was transferred to the PICU. She had strep pneumonia and also the flu. She was septic with a low blood pressure (the reason she was transferred) and requiring oxygen. She began to show improvements, weaning down on the oxygen but then had nosebleeds and began to require more oxygen support requiring intubation on March 15, 2019. I’m not going to go into all the ICU details and complications, but just say that we were so hopeful because she would show improvements. But then she would have a setback. This was the pattern for her entire ICU stay. Until finally she couldn’t recover from her setbacks. Ultimately she died on May 9, 2019 from respiratory failure and kidney failure.
If you want to read Ariella’s story from the very beginning you can at https://www.caringbridge.org/visit/ariellastein This will take you from diagnosis through ringing the bell at end of treatment. Ariella wanted to be able to share her own pictures and updates so we started www.facebook.com/ariellapaigestein near the end of her first round of treatment in November 2017. You can see the rest of her story there.
Just a note, after I finished writing I realized it may be too graphic for some to read. I didn’t go into much depth but I talk about images of Ariella in the ICU. If that’s too much for you I advise you to skip to the last paragraph.
Sleep. Sleep continues to be elusive. Once I fell asleep last night I mostly stayed asleep but it took a very long time for me to fall asleep and I was up early. I have a reel of images running through my head and I can’t turn them off. These images mostly attack at night, though I do get flashbacks of them throughout the day. All images of Ariella in the PICU. Shattering images of her scared eyes when they were prepping to intubate her. Crushing images of her with her sad eyes and miming that she wanted to drink. Devastating images of her writing on her white board “What if I die?” and “I want to die. This is horrible. I might as well be dead.” Asking “am I getting worse?” Heartbreaking images of Ariella on her side when they were cleaning her, in obvious discomfort and a tear dropping from her eye. Distressing images of Ariella practically lifeless in bed, barely opening an eye even when being poked and prodded and moved around. These images do not leave me. I feel such anguish that this is how she spent the last two months of her life, mostly awake and aware. She wasn’t in pain but she was extremely uncomfortable and miserable.
Also very sad to remember are the few positive moments we had in the ICU because at the time, they were positive. Ariella being so excited for a taste of popsicle and Rita’s that she wanted me to tell everyone about it. Ariella being so excited for her tracheostomy surgery because it would mean the tube would be out of her mouth and she would be able to drink and talk. That was another milestone she wanted me to share with everyone because she was so happy about it. When Ariella woke up after her surgery she gave me a big smile when she realized the tube was out. We all thought that was going to be the turning point to get her out of the ICU. We had talked about what Ariella should get following her hospital stay as she deserved something big. And she said, well wrote, “how about new shoes?” All the hell she went through and was going through and she asked for shoes. Broke my heart. The last thing she had looked forward to was finally getting water. She was able to get just 5mL at a time about 3 times a day to start but boy was she happy to get just that. That’s how horrific it all was, that drops of water and tastes of a popsicle could bring that much joy. She had everything all planned out once she got out of the ICU. A big glass of water in the purple cup she won playing bingo, milk, Rita’s, and a shower. And then a big steak dinner when she finally got out of the hospital. None of those dreams were realized. She was on the schedule to have a swallow study completed but it never happened because she took a turn for the worse. After that she never fully recovered. The only comfort I take from this is that she was sedated near the end so no longer uncomfortable and scared.
To contradict myself now, I am glad Ariella was sedated for her sake but I regret not having the death talk with her. When she asked earlier in her stay “what if I die?” everyone thought she would recover and come off the vent, her lungs just needed more time. We did not promise her she wouldn’t die but we did say the doctors were doing everything they could to prevent that. When it became apparent that there was a good chance Ariella wasn’t coming home with us she was mostly sleeping so we never got to talk to her about how she was feeling about dying. By then was she scared to die? Or was she ready to die? What did she think would happen once she died? Where did she think she would go? What, if anything was she worried about? We have talked about death before and what happens after someone dies and Ariella believed in Heaven so I just hope with all my being that by then she was no longer scared to die and was ready to go. Because it kills me to think she might have been scared about dying near the end but didn’t tell us.
I started this post about sleep. This lack of sleep along with the grief is leaving me in what I would call a fog. Concentrating on anything for longer than a few minutes is impossible. I get distracted so easily but usually not by anything, I just end up staring into space. I am extremely forgetful and am having trouble just getting through routine tasks from start to finish. I find myself frequently losing my train of thought when in conversation. I wasn’t going to write today but I know if I don’t have something to do I will fall asleep and I am worried that a nap will make it even harder for me to sleep tonight. But maybe I should take sleep when I can.
Everything is just so hard. I can’t even get my thoughts out right now. Maybe it’s the silence, though I have the TV on constantly for the noise. But that’s the wrong kind of noise. It still seems so quiet. I can’t escape my thoughts, running through my mind in a constant loop. The images change but they are all of Ariella, who she was, her life before and after cancer, the hospital, what might have been. Anytime it hits me that she’s gone forever I panic, I can’t catch my breath, I start shaking. This just can’t be, and yet it is and I have no choice but to live with it.
Ariella of course was our world but she is my world more now than ever. Before she ended up in the ICU, even after she had been diagnosed with cancer I was able to think about things besides her throughout the day. She didn’t consume my thoughts and emotions. Maybe as a baby but I also had my own life to think about, separate from her. Now I feel so intertwined because of her absence. Every single thing makes me think of her, whether it’s something she would like, something we did, or something she never got to do, anything, makes me feel her absence. Even mundane daily chores. One of her chores was to set and clear the table. I think of that every time we have a meal. Cooking. I can count on one hand the number of times I have cooked dinner since Ariella died (actually since mid-February when she went into the hospital) and cooking for 2 is not so different than cooking for 3 except that I don’t have Ariella asking “what’s for dinner?” and I don’t hear her complaining about homework or grumbling about setting the table while I’m cooking. So it is completely different. It’s like that with everything I do.
I’m having a hard time getting out my thoughts today. I’m having trouble concentrating. The emptiness, the silence, the stillness, her absence is ever present and it’s stifling.
There’s not a whole lot in my life that I regret. Even before Ariella was diagnosed with cancer we tried to live life to the fullest. We spent time together, went on vacations, played games, imaginary play (so much imaginary play which I did not enjoy but would give anything to have her begging to play with me now). Sure we were busy and had to do the regular day to day stuff but family and home always came before work for both David and me. So I can’t say that I regret not spending more time together or saying I love you more or giving hugs, or things like that because I always did. I have always known life can change in an instant from losing friends in high school and college and somewhat unexpectedly losing my dad (he had heart issues but I certainly didn’t expect him to die when he did). I never wanted to be the person who regretted missing time spent with loved ones. And I wanted to spend as much time with my girl as she would allow. I always used to joke with her that one day she wouldn’t want me always around, that she would get annoyed with all the hugs and kisses and not want to sit on my lap. She always disagreed, saying she would always want hugs and cuddles and to hold my hand, and it was true, even at 11 she often held my hand when we were out, she loved to cuddle together in bed, and had not reached the phase where she didn’t want to be seen with her parents. We certainly saw the pre-teen attitude and eye rolls but mostly Ariella liked having us around.
Once she was diagnosed of course we saw things differently. We always thought she would beat it but of course the thought was always there, what if she doesn’t survive? So we did even more. Gave her as many experiences as we could. Said yes to all the opportunities that came our way. We did not put her in a “bubble” in between rounds of chemo and instead let her go to the dance studio, have sleepovers, go to Build-a-Bear of course, and do all the things a 9, 10, 11 year old should do.
So no regrets with how we lived our lives and spent our time before and after cancer. But I do have a couple regrets and they are big ones. I regret not trying to cuddle more with Ariella when she was in the ICU. It was pretty challenging because of the vent and tubes and they said I could not lie in bed with her (I regret not fighting that too) but we both were aching to give each other hugs and just couldn’t. I regret not trying harder to give her physical comfort. I regret not trying harder to wake her to hear me when she was so sleepy but not yet under actual sedation so she could hear me tell her I love her and goodbye every time I left the room, especially to go home.
But the biggest regret, and I know I am going to get all sorts of arguments and comments about this, is choosing to do the bone marrow transplant. It was not guarantee of a cure, it is not standard treatment in sarcomas (it was a trial), and she was currently no evidence of active disease and having a good quality of life. Of course we don’t know if the meds she was on would have kept the cancer at bay but I am certain she would have lived longer and would have had a better quality of life. If she did decline due to cancer we would have actually been able to tell her goodbye, she could have told us her goodbyes, we could have gotten more hugs and kisses. She could have been made comfortable but maybe not as scared. Instead she had 2 of the crappiest months a child could have, only being able to communicate by writing, not being able to eat or drink, being uncomfortable with complication after complication, not being able to say goodbyes or know or be aware of the love that was surrounding her at the end. She was scared she was going to die. We never promised her she wouldn’t. But we redirected and instead just tried to reassure her that that the doctors were doing everything they could. While of course we were worried about the same thing. Cancer is traumatic. A dying child is traumatic. But there is so much more trauma with the way that Ariella died than I can ever put into words and I will never not regret doing the BMT and nothing anyone says will change my mind. The 3 of us walked into Hopkins together hopefully optimistic that the BMT would keep the cancer from coming back. But only 2 of us walked out. Wasn’t worth it. It’s this, this is what plagues me a good amount of the time. The actual medical and emotional trauma Ariella went through and that we went trough watching it. The not only not being able to help or comfort your child but feel like you’re the reason she’s in the pain and discomfort. I know we made the best decisions we could at the time given the information we had and we thought we were acting in the best interest of Ariella and everything else everyone is going to say about blaming ourselves. Intellectually I know it. But the regret will always be there.
As I said at the start, I don’t have a lot of regrets. But I can’t let go of the one I do have. And I’m sure I never will.
