Updated: Jun 10, 2021
BEWARE: Because I have not updated the blog since October-- this is long (sorry Mom) and emotional-- I apologize!
Taggart Lake-- May 2016
I have dreaded the process of writing an update because I am not sure if I have the ability or emotional stamina to recap the past few months. While the outcome is fine, I have experienced some of the most profound grief of my life (which after the past few years is saying a lot). Mike and I have returned to an almost “normal” life, but I have been reminded (once again) of the fragility of our existence, and it is hard not to live in fear of grief.
For the first thirty years of my life, I waited for something terrible to happen to me. My sister and I always recognized the fact that no one escapes the deep, agonizing sorrow experienced with loss or heartbreak or radical change. But, ultimately, despite a few bumps in the road, my daily life was exciting, productive, and happy. I had sympathy for my friends who had lost parents or suffered terrible accidents or been traumatized by their parents’ divorce, but I can say now that there was no empathy. I truly had never experienced the gut-wrenching, sick-to-your-stomach-can’t-eat-for-days, debilitating, paralyzing pain associated with grief. Undoubtedly, it is called suffering for a reason. I did, however, always know that one day I would-- some time, somehow, some way. There are obstacles and challenges everyone must face, and some people have considerably bigger hurdles than others. But at some point, we all have to overcome something. Here is the update on the hurdles my short little legs have been jumping lately.
Rose at 15 months--18 lbs, 27.5 inches tall. My perfect peanut!
Rose is the cutest, if I don’t mind saying so myself. She is definitely small and growing on her own curve-- but growing and making progress nonetheless. We have had huge success with the speech and physical therapies, and Rose is totally coming alive. She loves to laugh, play, kiss, give high fives, eat solid food, chew on her hearing aids, and give all of us immense pride and joy. Like all things that become familiar, we are so comfortable with her hearing aids. We love them. We know they work. They are a part of who she is-- and she is awesome.
In October (just after my last update), we discovered that she had fluid in her ears. For some fateful reason, we ended up seeing a new ENT and he asked if we had seen an opthamologist, had an EKG and echocardiogram, an ultrasound of her kidneys, blood work, and genetic testing. My heart sank. What kind of parents were we? No. We hadn’t done any of it. No one had suggested that we needed to go through such lengths-- she seemed fine.
There is solid research (which I googled and found immediately) linking ear deformities to kidney malformations and from there to genetic syndromes. This new doctor said that his top priority was to get an accurate diagnosis because he didn’t believe it ended at hearing loss. He made us an appointment for an ultrasound of her kidney, an eye exam with an ophthalmologist, he scheduled blood work, and he referred us to Primary Children’s Medical Center (PCMC) in Salt Lake City, Utah.
The first radiologist we saw told me that Rose did, in fact, have a kidney malformation. She had a horseshoe kidney-- which is when the two kidneys fuse together and cross over the spine in some way. He told me to ”google it and use Medscape because that was a reliable source”. He said probably coincidence, not uncommon. She would be fine. But I didn’t particularly care for what I read about horseshoe kidneys, and it seemed like a bigger deal than he made it out to be. However I liked what he said: “she would be fine.” I had been liking those words since the ENT initially bet me a million dollars, when Rose was five weeks old, that she wouldn’t have permanent hearing loss.
Hours after my Medscape reading, I called the new ENT’s office, sobbing. I am quite sure they all think I am crazy, but I was now in a panic. What else was wrong with Rose that we hadn’t discovered yet? The doctor called me back later that day and could not have been more helpful. He had already called PCMC in Salt Lake City, and he said that we would be hearing from the genetics and urology departments to schedule appointments. He said he spoke with his colleagues there and they suspected Rose had a genetic syndrome known as branchial-oto-renal syndrome. Branchial refers to the branchial arches in the neck and Rose did not display any of those symptoms. The oto is the ear and hearing loss. Renal-- the kidney. To us, seemed like a match. The good news was that this syndrome was not associated with any cognitive delays, so she should continue to progress well so long as we monitored her hearing loss (and we got that down!) and her kidney.
Our plan was to get through December, and our appointments would commence immediately after the New Year. In the meantime, our physical therapist noticed that Rose had an oblong shaped iris in her left eye. Once she pointed it out, it was so obvious. How had we not noticed? So I took to the computer again, and YIKES! A misshaped iris is called a coloboma and it can indicate a problem in the retina. The problem is not correctable and means that the person can be blind in that eye. WTF!
So we spent the month trying not to worry about Rose’s strange abnormalities, and we had a Hallmark-movie-holiday. We put our dog down just before Christmas because he was old and ready. We mourned his loss with lots of tears (he truly was the best dog in the universe), missed those family members no longer with us but relished in the Christmas spirit. We played in the snow (skied, went sledding, made snow angels), opened presents from Santa, ate amazing food, celebrated with great friends and extended family, and cherished our time together. The girls loved every moment-- from the fiasco of putting up our 20-foot Christmas tree to the day we had to say goodbye to Nico, our elf on the shelf. The magic of Christmas was in full existence at our house. And then the New Year hit.
Rose and Mason
On January 4th, we met with the ophthalmologist. Before the appointment, I soaked through my shirt in the waiting room. I was shaking I was so nervous. Rose appeared to see just fine, but my confidence was shot-- what the hell do I know? We feel good about hearing loss and so grateful for all the technology and opportunities that Rose will have, but I didn’t think I could also handle it if Rose was blind in one eye. It just didn’t seem fair that she has to deal with all this. But the quirky doctor put us at ease, and he said she had a “trace coloboma” that did not go all the way through to her retina. She could see! Thank god.
Rose waiting for ear tube surgery
The following day Rose was sedated for the first time for ear tube surgery. While it is a very routine procedure, it was heart wrenching for us to see Rose taken away in the littlest hospital johnny they make. She was so cute and happy prior to the surgery--ignorance is bliss, but when she came out she was mad. As the anesthetic wore off, she fussed and moaned and snuggled and threw up. But the end result was that the tubes were in and two days later they were making a world of difference.
By the end of the week, I was the happiest I had been in months. Rose was sitting in her high chair at night babbling consonant sounds trying to partake in the conversation. She searched for our attention using her voice, and she was ready to talk! Though her immediate progress proved to me that she probably had not been hearing very well over the holidays (which broke my heart), I was ecstatic thinking about how much progress she would make moving forward. Onward and upward!
So that weekend we packed the car and drove to SLC to spend time with my mother, my sister, her husband Chris, their 2-year-old Ripley, and new baby girl Freddy (10 weeks) before our genetics and urology appointments at PCMC on Monday.
This is when things took a nosedive. The genetics appointment was positive. Our doctor said she suspected branchial-oto-renal syndrome but was thrown off by Rose’s small stature and flat nose bridge. To start, she was going to do some blood work to try and confirm the diagnosis, but overall she was impressed with Rose’s personality and progress.
The urology appointment involved another ultrasound, and the doctor changed her diagnosis. She does not have a horseshoe kidney. She only has a functioning right kidney and the left kidney is dangling off of it like an earring. But no big deal! We only need one kidney and her right kidney is working perfectly. The doctor felt confident that Rose can do all the things we all do and suffer no ill effects. Thank god.
Then the blow: While looking at the kidney, the ultrasound tech noticed tumors all over Rose’s liver. The radiologist called the urologist and suggested we go immediately to oncology. Obviously, I started to shake and cry. Oncology? Why oncology? The doctor marched us over and the oncologists came in right away. They wanted to get started on blood work and we needed to move quickly to determine whether or not Rose had liver cancer-- aka hepatoblastoma.
It was like we were in some fucked-up time warp. Is this what we were really doing? It didn’t seem real and even as I type this and conjure these memories my fingers are shaking. Rose has so many tumors on her liver, not just one or two, but an “innumerable” amount in all sections of her liver, so the doctors explained she would need a liver transplant. But we had to confirm with blood work and an emergency MRI of her liver which was schedule for the day after the next because Rose would need to be sedated again which requires fasting-- which is so painful for a baby, especially a baby who is so small she is not even on the charts. The oncologists connected us with one of the top pediatric liver specialists in the world. Luckily for us-- she happens to practice at PCMC. This doctor is like a celebrity, and her wisdom, power, experience, and kindness is palpable. The team of oncologists explained that they defer to her until a complete diagnosis is made-- and she is the one to examine blood work, MRI, and symptoms (of which Rose had none).
Thank god we were staying with my sister and my mother was also there. After leaving the hospital, we went back to their house to cry, freak out, and put on a brave face for Neve-- who really didn’t understand what was going on. This is when I basically stopped eating. The good news is that I have lots of stored fat-- like a whale-- so I could probably survive for a long time without food. My mother tried to force feed me enough so that I would not get sick. But I could not do anything but pound my fists on the bed and kick and scream like a toddler. It was like my job-- just to freak out. Panic. Cry. Throw a tantrum. Cry some more. The only phrases I could continuously mutter were “what the fuck” and “it just can’t be.”
Waiting to be prepped for MRI
Mike and I faked it well around Neve and Rose, and we had to be so strong for the MRI. As always, Rose was an incredible trooper through the fast, sedation, and recovery. She melts my heart. But the results of the MRI were not in right away as the radiologist had not completed his report and we had to wait for the liver specialist to read them as well. The blood work showed an elevated cancer marker, but it was not too elevated. Just enough to give the doctors concern, and me-- more anxiety. In the meantime, the liver doctor scheduled the biopsy for Tuesday, January 19th. She wanted us to go home, regroup, get some clean clothes, and come back on Monday night. She would call us later with the report from the MRI.
At 9pm, the phone rang and I immediately had a physical reaction. But alas, she told us the results were still not in and someone from her office would call the following day. More waiting. Did I mention I am impatient? I am impatient in general, but hyper-impatient doesn’t even describe my state while waiting to find out if my daughter had severe liver cancer.
The following day one of the graduate students working in the liver doctor’s office called, and I am reluctant to relay this story because it is NOT indicative of the quality of care from this specialist. But the student said that the radiology report favored “hemangioma” which is like those dark, red wine-ish, raised birthmarky-like stains you often see on people’s skin. Of course it is different when it manifests on an organ. On the liver they are vascular tumors but benign. I almost screamed with relief. Over analyzing everything at this point, I felt very confident that they would not have given us this bit of good news if they were not very confident themselves. Finally, we caught a break and, for the first time in days, I felt like I might actually survive this disaster.
A few hours later, the oncologist called and asked how I was doing. I said I felt great and considerable relief as we prepared for the biopsy. She paused. There had been a mix up. The student who had called to deliver the radiology report had made a mistake and mixed up the words hemangioma and hepatoblastoma. The oncologist forwarded me the report from the radiologist. It had words like “morbid edges” and “decay” in it. It specifically stated that it could be infantile hemangioma or multifocal hepatoblastoma. “I favor hepatoblastoma,” he wrote. To translate, the radiologist thought Rose had liver cancer.
Immediately I called the surgeon’s office thinking that she knew more and had a different opinion, but the student-nurse reread the report and acknowledged her mistake. Big mistake. Huge. So later that night the liver doctor called to remind us that nothing was conclusive until the biopsy results/pathology were read. She still didn’t lean one way or the other. So we spent the rest of weekend at home stewing over the fact that she most likely had liver cancer. We didn’t leave the house. I could not go out in public. Our incredible friends brought us food, checked on us, and helped with Neve. Needless-to-say, we were devastated.
I was so anxious to hit the road on Monday so that we could move forward and tackle the next challenge. I wanted to make progress, whatever that meant. If Rose needed chemo right away, I wanted to start. If she needed to gain five pounds in order to have the transplant, I wanted the plan, the calories, and to pack on the pounds. The geneticist had ordered more blood work to see if that could help provide some answers. I wanted to get to the bottom of this. I wanted my poor baby to get healthy.
So Rose was sedated again--this time with general anesthesia. Her third sedation in less than three weeks. Mike and I held hands in the surgery waiting room, and Mike kept asking me why he felt so sick. We carried barf bags, and Mike thought he actually had the stomach flu. Never in his life had he experienced this level of stress. How can a parent not be on the verge of puking when their child is having surgery to determine whether or not they have life-threatening cancer. Occasionally, I glanced around the waiting room and wondered about all the other families in there. It is heartbreaking place.
An hour into the surgery we started to get impatient (shocking)-- she should have been out any minute. But the minutes passed, and we didn’t hear a thing. Other families were meeting with their surgeons and we felt a flutter every time the door opened, but the doctor or nurse never came for us. Finally, we could bare it no longer and we asked the reception desk to call the operating room. It had been two hours, and they were finally done. The doctor was on her way out.
She sat down between us and took out her cell phone. She showed us pictures of a few of the larger tumors. She told us they actually resected an entire tumor in order to ensure the most accurate biopsy. But now we had to wait. She said she did not know what the tumors were-- didn’t favor one diagnosis over another-- so we had to be patient. I was such a disaster that she recommended I chant the Alcoholics Anonymous Serenity Prayer-- and then she forwarded me the words. God grant me the serenity to accept the things I cannot change; courage to change the things I can; and wisdom to know the difference.
Waiting for biopsy results and recovering from surgery
Mike and I met Rose in recovery where the nurse asked us if she had a voice and could cry. Yes, of course. But she was so hoarse from the sedation and intubation that she could not squeak out a peep. She tossed and turned and looked terrible lying in a rolling crib connected to tons of tubes. Finally she made a few noises and her vitals were stable. We moved to our recovery room where we would stay for two days.
Since my father died, I have always looked for signs that he is near. He has sent us a few incredible messages through friends who have visited mediums, and I take great comfort in reading and rereading those messages. I talk to my father regularly, but during this process I begged him for help. Whatever the outcome (which I know he didn’t have control over), I just hoped he could give me the strength to survive. Pulling into SLC, we drove underneath an arch with a bald eagle which I had never noticed before. Trees and birds remind me of my father, especially bald eagles because of his stunning white hair, and deep down I hoped that was a sign. When we walked into our hospital room, I was stuck by our room number- 3009. My birthday-- March 9th. And while I was rocking Rose and staring at the wall in front of me, I noticed the housekeeping placard-- extension 24444. My dad’s birthday is August 24th, and his password to everything: 24444. Crazy. For a few minutes, the pit in my stomach vanished and I felt comfort. I felt my father. But I talked myself out that craziness and started imagining what I would do when the doctor came in to tell me my daughter had cancer--the most likely scenario.
Mid-morning on Wednesday, the doctor and her entourage opened our door and marched in. “Hemangioma,” she said and I immediately broke out in hysterical (and I mean hysterical) sobs. How did we get so lucky! She told us what the next steps would be, but ultimately Rose could survive with hemangioma. She would need monitoring and an immediate echocardiogram (another sedation) to confirm that there was no shunting (tumors sucking blood from heart), but hopefully the tumors would recede and she would outgrow it. Hemangioma can be managed. As long as it wasn’t cancer, I felt relief. I cried for about three hours-- embarrassing sobs that mortified my husband and, finally, he asked me to close my mouth and get it together.
Perking up after surgery
Neve thinks all trips to Utah are a blast! Here she is with my mother and Ripley at Wheeler Farm.
My spirits soared, yet at the same time I was now profoundly aware of how all the other parents in the that hospital felt. Most likely, at the very same time we received our good news, a family received the worst news of all. In the next room, the child (who we never saw but heard) had endured a bone marrow transplant. Spend a few days at a children’s hospital and it changes your perspective forever.
Immediately, I was hungry. I could finally eat. I went down to the cafeteria and on my back way up, I stopped at the hospital chapel/meditation room. I felt this overwhelming need to give thanks, to say my gratitudes, to make promises, to never forget how challenging life can be. And even in the face of all the sadness I have felt over the past few years, I recognize it can always get worse. I can’t even let my brain go there-- but the core of me knows this to be true.
I have never felt older or any different after a birthday. But lying with my father when he took his last breath changed me. It’s almost as if I have met a celebrity named Death, and it is cathartic to share the story of our encounter. After this experience with Rose, I feel different as well. I have had to think about how life would progress with a child with cancer. Nevermind the fact that Rose’s health and life expectancy would contradict everything I have hoped and dreamed for her, I also felt guilty and heartbroken for Neve. I pictured my older self sitting in a rocking chair reading her memoir, and it read something like this: “My life was full of joy until I turned four and my sister was diagnosed with cancer and my mother sunk into a terrible depression and we had no fun after that.” (Except in my nightmare-- Neve was a much better writer.) I know myself and I know that with the help of my family and friends, I would have dug deep and found the strength and courage to do whatever was needed. But I am beyond grateful that I don’t have to do that. But there are people (for a million different reasons) who do have to muster that courage or flash a smile to a child when they just want to bury their face in their hands and sob. I am crying tears for them right now because I now have an understanding (even if it is just a slight understanding) of how they feel, and it kills me. We got lucky this time, and I know it.
After a few days recovery and a normal echo (thank god!), we all came home and relished in Rose’s good fortune. All was great and we were moving forward. But we had to go back to Utah in two weeks for check-ups, and Rose’s oblong shaped pupil seemed to have become more pronounced which all the doctors told me would be impossible if it was a coloboma. Conclusion: I was going crazy!
When back in Utah, I had to be super aggressive to get an appointment with a pediatric ophthalmologist at PCMC. As mentioned, colobomas do not change so there didn’t seem to be any urgency. I continuously called the geneticist’s office but didn’t get a response-- until February 9th. While having dinner at my sister’s, the geneticist called me and her tone was somber. She asked if I was with people and told me she had some significant news.
“The microarray chromosome test was abnormal,” she explained. “Rose has a large duplication and small deletion on chromosome six.” Two abnormalities-- not just one. A very big deal in the genetics world. Immediately, I thought she had to be wrong. We had done genetic testing on the embryos--this had to be a mistake. “Are you okay?” It was obvious she was devastated just delivering the news. “What does this mean?” I asked. And the answer then and now continues be--nobody knows. She was brutally honest and said that she needed time to do research and a complete investigation, but her initial response was that this explained Rose’s clinical symptoms: eyes, ears, hearing loss, liver, kidney, small stature, flat nose bridge, and gross motor delays. She said Rose might have significant cognitive impairments-- never be able to live independently or speak or walk or drive a car . . . . OR perhaps she might be okay and just have minor learning disabilities. She didn’t know. So we scheduled a follow up for March 3-- and I hung up the phone with the strangest feeling that she was talking about someone else’s child. Not mine.
In the meantime, she scheduled an appointment for me with the eye doctor at PCMC because there is a risk of glaucoma associated with this genetic abnormality, and she was concerned since I indicated that Rose’s eye was changing (or at least I thought it was). The following day we saw a fabulous pediatric ophthalmologist who did in fact change Rose’s diagnosis. As for me-- I was not crazy! The pupil was changing. She does not have a coloboma but a “something something corectopia”. It is symmetrical--meaning it is in both eyes--but Rose’s right pupil had not started showing the symptoms yet- it has since manifested in both eyes. (FYI-When she was born, it was not apparent.) At the moment, she has perfect baby vision, and aside from the risk of glaucoma (which is treatable), this diagnosis is much better than a coloboma. Good news. Grateful for the good news.
While I was petrified to google chromosome six abnormalities, I did a minimal amount of research and determined that everything I read did not fit the description of my little girl. The internet can be a very scary place, and all the reading did was give me a pit in my stomach. And even recapping all of this is giving me heart palpitations-- and I am ready to be done writing it (probably as much as you are ready to be done reading it! Never a good sign if the writer isn’t even engaged!).
So lots transpired between the chromosome diagnosis and mid-March. Rose and I spent the night of her first birthday (Friday-2/26) in the hospital in Jackson because Rose had the stomach flu for a few days and, after determining that she was not herself, I took her in at 8pm. I pulled away from the house sobbing, and Mike was nervous that I might not be able to drive. But alas, it was Neve’s birthday on Sunday, and she deserved a little normalcy. The ER docs thought she seemed fine--showed no outward symptoms of dehydration or infection-- but because I now speak doctor language I demanded (in a nice way) bloodwork and an ultrasound of her kidney and liver. Unfortunately the tests showed that she was severely dehydrated and had some sort of virus-- so we were admitted. Of course the only place they could get an IV was in her head. So she had quite the festive birthday hat.
We met with a neurologist, had a brain MRI because structural brain abnormalities are associated with her chromosome duplication--her brain is perfect, checked on her liver (which is healing beautifully and the tumors are acting just like hemangioma), met with the oncologists to review blood work, met with a comprehensive care doctor who can help us coordinate and manage the ten million doctors we see at PCMC, and we met with the genetics team again to get more information. This time they were very specific about the genes that were duplicated and deleted. The nine genes that have been deleted are associated with a syndrome called Axenfeld-Rieger Syndrome which involves the eyes and ears. The duplication is so rare (many geneticists will go an entire career and not see these genes duplicated on chromosome six) it does not have a name. The doctor really didn’t have much to tell us except to wait and see how Rose develops. So the big takeaway-- I have to be patient. God damnit.
While these results are hugely significant, we are living life like everyone else. Rose does not exhibit many of the symptoms associated with the duplication, and I have promised myself that I would STAY AWAY from the internet. I have not googled a thing since the initial call. I am definitely suffering from some PTSD, and I have had to take all the information from the doctors and put it away-- in a folder, in a drawer, where I can only get to it if I really need it. Because, ultimately, it doesn’t matter. It is not terminal. (I suppose that is the silver lining of the cancer scare? A little perspective.) And second of all, however Rose develops, she is still my perfect baby and we will love her like crazy no matter what. Rose is going to be who she is. And we are so lucky that she is here. Fortunately, she is progressing just like every other child, and she is crushing it.
Rose is almost walking independently but walks with a walker like a champ (which I think might be harder than regular walking?) She is into everything-- and especially loves to crawl into Neve’s room and find the Barbie shoes. She is ahead of the game with her speech and language-- saying mama, dada, na for neve, cat, dog, cow, up, out, down, tree, apple, ut-oh, and the list increases every day! She is smiley and happy and engaging and silly and so full of personality and love. Neve is the best big sister, and every morning she climbs into Rose’s crib before we come down and they share a few very special minutes together. They sit up and face each other and “talk” and giggle like mad. Mike and I watch on the monitor-- and usually I have tears in my eyes.
Now that the trauma of the winter is over, I am in heaven. As Mike reminds me, there is no reason to stress, not right now anyway. All is well-- and we are just so grateful to everyone who has supported us, and cared for us, and ensured that we stay focused on what really matters-- our health and our happiness. I still talk to my father and my godfather Brook-- who I truly believe are helping me through this tough time. As mentioned I always look for signs. The sign from Brook: one of Rose’s liver scars is on her belly button and it looks like a perfect hurricane symbol. (For those that don’t know- Brook owned Hurricane Restaurant in Kport, and his wife Luanne still does.)
Being a parent is the hardest job in the world because loving your children literally hurts your heart. No one knows what the future holds, so I am trying hard not to waste my time making predictions or speculations. So I will end with the quote that we shared at my father’s memorial service from the book John Goffe’s Legacy by George Woodbury: “Tomorrow will be another day and tomorrow will be different, but we will not know the particulars until then.” So here is to the now!
That said, I am off to play with the girls, take them to the pool, plan our trip to the beach, and enjoy every second--even the not so fun moments. Next blog update: probably next winter- at the rate I am going. xox