Our journey, her battle, our life without her.
May 26, 2021
It all started in April. Sloane had two ear infections that weren't cleared by 3 different antibiotics. After multiple visits to the ENT and pediatrician, the decision was made to get tubes. We thought that after surgery, she would bounce back and we would have our little girl again.
It has been a very different story. Since then, she has gone slowly downhill. We weren't sure what was going on because she wasn't herself at all and seemed to be getting worse, not better like we thought she would. After blood work and a CT scan, they found a mass at the base of her skull, right by her brain stem. We were admitted right away to Nationwide Children's Hospital. She had 3 MRI's to determine what the mass is and where it is connected. The tumor is in a category called embryonal tumors. They think it could be a chordoma. It is in the dura of the brain, and it is pressing on the brain stem. It appears the tumor is sitting on two cranial nerves and pushing onto the pons portion of her brain stem. One is the facial nerve which controls her facial muscles and the other is the vestibulocochlear nerve which controls her hearing and balance. Makes sense that she is experiencing paralysis in her face and has lost the ability to sit and stand independently based on the nerves that are being compressed.
They are planning for surgery next Wednesday, June 2. They are getting the team of neuro-surgeons, neurological oncologists and ENTs to perform the 12 hour surgery. They will go through the back of her mouth as it is the best way to get to the tumor. She will be in the PICU after surgery for a couple of weeks and will be on a ventilator and feeding tube until she fully heals. We will have a pathology report 3-4 days after surgery to see what stage the tumor is and what treatment she will need. The goal is to get most, if not all of the tumor in one surgery but that may not be an option dependent on how wrapped up the tumor is with other parts of her head. This tumor is in an incredibly difficult spot to reach, which makes full removal that much more difficult since there are so many nerves and blood vessels connected to her brain stem.
We are very scared about what this means for our little girl. There are so many unknowns that can’t be answered until after surgery on Wednesday. We are trying to stay as positive as possible, but I won’t lie, we are having more dark moments than light right now. Each day the reality of our situation sinks in a little more.
First Brain Surgery
June 2, 2021
Surgery day. Originally they discussed trying to get as much of the tumor as possible. They have changed the plan a bit based on what they now know from her multiple MRIs. They will be taking a more conservative approach. The goal was to get as much as they could safely, without disturbing the nerves and arteries too much.
The initial pathology report states that it appears to be a Poorly Differentiated Chordoma. Chordomas are rare and this type is the rare of the rare. Samples, pathology reports and imaging are all being sent to St. Jude’s for more opinions on how to treat this tumor. The doctors are also consulting with other neuro-oncologists down at Cincinnati Children’s Hospital as she may need radiation as well, and they have the proton beam radiation machine there. We will begin chemo a week from today. After one round of chemo, they will do another MRI to see if chemo has any effect on treating it. If it does, we will keep up with the chemo treatments. The hope is that the tumor will shrink enough to either have it all disappear or make it easier to remove the tumor without doing any permanent damage. If not, we will cross that bridge when we get there.
Some days the grief is so crippling, while other days we push through. We will keep on fighting for Sloane every single minute of every single day. Shakespeare said it best, “Though she be but little, she is fierce”.
The MRI showed that the tumor has grown since surgery. We are starting emergency chemo now instead of waiting for final pathology results.
Sloane had her first dose of chemo (ifosfamide and etoposide) while laying in Mat’s arms. She will have a dose every night for the next four days, after which we will give her body some time to recover and then she will receive more (cyclophosphamide, vincristine, doxyrubicin ) for two days. There will also be an MRI at some point in the next week or week and a half to see how the tumor is responding to chemo. She is stable, but still sedated and on a ventilator. The NJ tube and PICC line are getting her the much needed nutrition she needs to stay strong and fight hard. She has some color back which made us take a sigh of relief.
We received the final pathology report back from the Mayo Clinic and St. Jude’s. It is confirmed to be a Poorly Differentiated Clival Chordoma. This is a positive because these tumors have been known to respond to chemo and radiation, but they are also very aggressive. Once again it is a waiting game to see if the tumor will shrink as a response to the chemo. All of her pathology reports, imaging and charts are being sent to St. Jude’s and The National Cancer Institute for those doctors to study. This is an extremely rare tumor, and even more rare for someone of her age.
Sloane had an EVD (external ventricular drain) placed and they found that the drain is helping a bit and the fluid in her ventricles are decreasing. Now any time the pressure builds in her brain, the drain will relieve that.
We just keep telling ourselves that the chemo is going to work by attacking those tumor cells and stop it from growing…and then start shrinking it. She is on a very aggressive chemo treatment plan, but this is a very aggressive tumor. We saw a few signs of life yesterday. She opened her eyes when we were talking to her and she started to flex her right hand and move her right leg some. We will take any win we can get right now, but this emotional roller coaster is taking a toll on us. Sleep is hard, our bodies ache from being tense all day long and our stomachs hurt with the anxiety we feel from hour to hour.
Living in the PICU is not for the faint of heart.
Countless MRIs, CT scans, NJ tube placements, suctioning, ventilator, nasal cannulas, arterial lines, PICC lines, chemo port, IV lines, hourly finger sticks, hourly neurological checks, 2 brain surgeries, 2 EVD placements, chemo drugs so toxic that they have to hyper-hydrate her so it won’t damage her internal organs and that isn’t everything she is going through.
We sleep at the hospital every night in fear we are going to lose her. Machines are constantly beeping and nurses are in and out frequently. Sloane is hooked up to so many machines and IV’s it is hard to even hold her for fear we may pull something loose.
They gave us a big bed so we can lay with her. This makes it a little easier on us and also on her….any way we can be near her is all that we want.
Sloane came off the ventilator. We actually got a few half smiles out of her. She gave us some high 5’s, and even played a little bit by trying to pull our masks off.
She has been working with PT, OT, Speech and Recreational Therapy to get her to interact and play again. Today she sat in a tumble form, which is basically a molded foam seat that supports her head, for 30 minutes. That is the most she has sat up since the end of May. She played with some toys and even threw some that she didn’t want on the floor. She was exhausted afterwards, but she loved every minute of it.
She tried some applesauce. She kept asking for more, which is a good sign, but we can only do that when the Speech Therapist is there to make sure she is swallowing properly. If she sees us eating, she signs “more” and points to the food.
This amazing girl is holding her head up now, using both arms and legs, and will even try a little sitting with some back support. Instead of just laying in our arms, she is starting to squirm again like the 16 month old she is. She is playing with and looking for toys too. She even danced to some music for the first time since mid-May.
The facial paralysis is still there and her vocal chords are partially paralyzed, but it is encouraging to see this progress. As she gains strength and more control over her body, we can do and try more to regain movement that she has lost. Little by little, she is doing it.
She actually sat unassisted for about 30 seconds! She was pretty wobbly, but she did it. We played with bubbles, looked at pictures of her sisters and even got to have a lollipop.
Sloane has been working so hard with PT and OT. We saw her actually crawl, stand while holding onto the couch and even trying to pull herself up to standing. She was exhausted and shaking after all of that work, but she is going after it and getting it! Her progress over the last three weeks had been incredible. She is sure showing that tumor who is boss.
Big day again! As soon as I arrived she pointed to her play mat and was ready to play. She crawled without being prompted, basically she wanted a toy and went after it. She tried to stand on her own from a seated position…didn’t go so well, but she tried. She even stood while holding onto the couch to play. Then PT got out the gait trainer and she walked all around the halls waving to anyone who stopped to talk to her. She was so exhausted after all of that, she took a super good nap. She woke up happy and pointed to the gait trainer again so she could walk down the hallway and wave to everyone. They all call her “The Queen” as she waves while walking.
What is going on?
Sloane has been in good spirits, which is so nice to see. We had a CT scan yesterday to check for any type of bleeding because she has lost most of her strength and function of her left side again. The CT was clear, so doctors are hoping it is just swelling from the tumor as it may be “pissed off” (docs words) from chemo. We are really hoping this is temporary. It is so hard to see her look at her left hand and try to move it and she can’t or she is able to lift it, but not able to really grab anything.
Another MRI was done. It showed that the tumor is swelling and pressing on her brain stem. In turn, her brain stem is being compressed. This is what is causing the left side weakness/partial paralysis. They will be treating it with steroids in hope that it will decrease the swelling.
Sloane’s condition has deteriorated. She was taken back to the PICU. She is currently on high flow oxygen and being monitored very closely. She has lost movement on her right side and a CT scan was ordered. The scan showed that the tumor has grown and she has more swelling. The doctors said that the CT isn’t as accurate as a MRI, so not to worry about the apparent growth.
Trying to Save Her
They did another MRI. Sloane needed to be intubated and put back on the ventilator because her breathing was not even and she was doing a lot of gasping. She is stable and resting now that she is getting full breaths. PICU docs think it is tumor related. Oncology docs think it is virus related. Both don’t know. Radiology believes it is tumor growth they are seeing. Oncology believes it is tumor swelling and blood from the tumor hemorrhaging. Either way, the tumor is pushing on Sloane’s brain stem and has caused her full body paralysis and breathing difficulties. Either way, our baby is on life support for the second time.
It has been confirmed, the tumor has grown despite the chemo and there is a lot of swelling around her tumor and her brain stem.
After talking with everyone, we have decided that the best course of action is to operate. They will be making an incision behind her right ear to gain access. They will retract part of her cerebellum so they can reach the tumor. At a minimum they will take a portion of the tumor for analysis to determine if it is new growth or dead tissue. If there is blood, they will try to evacuate as much as they can. The goal being to not cause any unnecessary damage to the surrounding nerves and arteries. They will try to safely get as much as possible. The will also be placing her EVD (external ventricular drain) back in to help with any pressure after the surgery.
We are meeting with the team to talk next steps. Radiation is on the table. They are thinking of transferring her daily to Ohio State University Medical Center for photon radiation (traditional) since they have an ICU that can keep her on the ventilator. Our discussion today was about this plan and preparing us for the very real possibility of our greatest fear.
To say that we are broken is an understatement. We have been crying for several days now and the news doesn’t seem to get any better, just worse. The possibility of losing our little girl seems more real than it ever has. Trying to find the words to tell her sisters about what is going on is excruciating. Knowing we may never be a family of five again has devastated us in a way that no person should ever feel.
This picture is from June. This is the only picture we have as a family of 5 in 2021. This is when they thought we were going to lose her and they let the girls come into the hospital to see her. We didn't realize we should have taken more pictures as a family. We never imagined a life without Sloane, but on September 14, 2021, that all changed.
The world keeps moving forward around us and we are stuck in a place with no end. We will forever be grieving for Sloane. There is no light at the end of this tunnel. No matter what we do in her name, no matter how much money we raise for pediatric chordoma research, we will never be done grieving. It will always feel this way inside. We will just learn to live with it and grow around it. It is hard to see everyone moving on as if nothing happened, but that is because nothing happened to them. It happened to us. It happened to a little girl who was as pure as sunshine and didn’t deserve to die from a one in a million tumor.