Vienna’s Story

Site created on October 9, 2019

Vienna Elizabeth enriched our lives with her arrival August 16, 2019 at 7:37pm. She weighed 7 pounds 12 ounces, measured 20 inches in length, born with beautiful thick black hair and big dark brown eyes. From the beginning of her life she has been our little firecracker! The nursery nurses would bring her back from testing or examinations and tell us “she’s a feisty one”.  Her name comes from Ashley’s grandmother, Metta Vienna and Ashley’s middle name, Elizabeth. All three share August birthdays a week apart. From the beginning of her life feedings were always a struggle. Initially we struggled due to lack of breastmilk when we discovered her significant weight loss at her 4 day checkup with her pediatrician on August 20th weighing only 6 pounds 13 ounces. We continued with breastmilk feeds and began supplementing with formula where breastmilk was lacking. Upon our return to her pediatrician’s office August 22nd we were thrilled when she was back up to 7 pounds 4 ounces. She continued to always seem to be one that took her time eating with feedings lasting 45 minutes to 1 hour. When she was around 10 days old she began to be fussy for a few hours every evening so we considered colic the cause. Upon her 2 week follow up with her pediatrician on August 30th her formula was changed to one that sometimes can help with colic. She ate 2 bottles without problem but then seemed to start refusing the formula that night. We returned to the original formula for supplementing. That following week she began to cry usually from about 5p/6p until 12a/1a/2a and a couple of times until 3 am. The first week of September her crying began to increase to the afternoons as well and continued increasing with mornings. By the time we returned to her pediatrician for her 1 month follow up on September 19th Vienna cried most of every single day. That week we decided out of desperation one night to re-attempt the special formula for colic. She seemed to take this without much problem this time. Her only peace in life by this point was night when she would usually have 2 good (for her) feedings meaning no or very little crying during eating. She continued to take her time with her “good” feedings, eating 2 ounces. Her daytime feedings were usually running back to back and no real time between eating, still only eating 2 ounces. During her 1 month well check on September 19th we agreed to start her on Pepcid as she seemed to be a classic case of reflux with weight gain but not proper weight gain (weighing only 8 pounds 13 ounces), crying through feedings, long feedings, and still crying after feedings while never being content. After starting Pepcid we had hope we were on the right track and Vienna was off to a great life as she had 2 good days. That quickly faded on the 3rd day when we returned to our normal crying and never content. The last week of September her crying progressed into the night and we lost any “good” feedings. Vienna at this point of her 5 week life was crying most of a 24 hour day with some sleeping at night from exhaustion and 10-30 minute naps here and there in daytime, nothing of a typical newborn. By this point so many different medications, bottles, diet changes for breastmilk concerns, blankets, swaddles, walking, anything you can think of tried with very little to no help or only temporary help. 
       The night of September 22nd we noticed some redness to her lower right gums. Since around 7-10 days of life she always had prominent teeth on the lower right gums to the point we and others commented on this interesting feature. The following morning September 23rd the redness was worse and her lip laying over that area had slight protrusion with crying. We called the pediatricians office and they saw her that morning. Vienna incidentally had a fever to which we were sent to the pediatric ER and admitted overnight. Her workup fortunately was without any etiology of her fever. During this time the area continued to grow at a rapid pace. On Wednesday September 25th we saw Dr. James Forsen with pediatric Otolaryngology who also consulted his colleague Dr. Clarry while we were in office. All physicians agreed this appeared to be a hemangioma. The treatment for hemangioma is an oral medication called Propranolol. Dr. Forsen put us on this x 1 week as a diagnostic tool. If this area responded to the medication which is what we hoped for then it definitely was a hemangioma and we were good to go with simple medication. Upon our return to Dr. Forsen the area had continued to increase in size and obviously not respond to the Propranolol. We proceeded with a biopsy under sedation the next morning Thursday October 3rd.  Dr. Forsen and we were still hopeful that he would do the biopsy and discover it was indeed a hemangioma that just needed an increased dose of Propranolol. Unfortunately when Dr. Forsen came out of surgery he informed us this was definitely not a hemangioma but rather a “mass” of some nature and malignancy could not be ruled out. That weekend was the longest weekend of our lives as we waited for the pathology report. Dr. Forsen called us daily to check in and let us know the status of the pathology report. On Tuesday October 8th we finally had a preliminary report back reading out “Melanotic Neuroectodermal Tumor of Infancy”. Dr. Forsen the morning of the biopsy informed us of research he had done and mentioned this rare benign tumor as a possibility but just wasn’t sure what we were dealing with as he had never seen anything like this. He did advise us because this was a mass affecting her jaw bone and no longer a hemangioma which respond to medication management we would be looking at surgery to remove this but that he would be sending us on and not performing the surgery. The day of her biopsy Dr. Forsen started making phone calls to get us in touch with the physicians who would be taking care of us whether this was malignant or benign.
Once we had the pathology report we knew we had to move quickly despite this being a benign (not cancerous) tumor. Dr. Forsen had names and numbers for everyone we needed to speak to immediately. We reached out to Dr. Jason Rich’s office that same morning and were scheduled to see him the next day Wednesday October 9th. Dr. Rich is a head and neck surgeon with Washington University who deals mostly with adults but does some pediatric cases as well. We met with Dr. Rich who discussed the plan with us. They had plans for surgery October 21. Based on Vienna’s weight struggles and her lack of pain control as well as us explaining the rapid growth of this they offered to attempt the following Monday October 14th. We said “absolutely!” When he asked how this date sounded. While in the exam room he texted the other physicians on the team and we immediately went back over from Wash U to Children’s to meet our other physicians on the team. We were treated like VIPs being escorted by our two ENT physicians. We met with Dr. Moulter (pediatric ENT), Dr. Leonard (pediatric ENT), and Dr. Patel (pediatric Plastic Surgeon). They reviewed the CT scan together and discussed their plan with us. Every physician has told us this has never been seen here in St. Louis but Dr. Moulter said to me “Even though we’ve never seen this exact tumor we do these same procedures all the time for other things.” We were admitted that night for pain control and nutrition. We compromised on inserting a nasogastric tube by getting pain under control first and seeing how eating went. Pain is finally under control with Tylenol and Gabapentin. We are set for surgery on October 14th. 
The surgery will start with placing external fixation to stabilize her jaw. Then they will remove the tumor, her jaw bone on the right side,and a nerve that is encompassed by this tumor. They are able to leave her joint as it is not currently impacted by this tumor. They will place a spacer to the area where the jaw bone was to fill that space until reconstruction at a later date. This is needed to prevent the existing jaw from shifting and filling this empty space.  She will under go reconstruction at least 3 months out from this first surgery to ensure recurrence does not occur to this area. The recurrence rate is fairly high and higher in infants diagnosed under 2 months of age. The physicians will have to take good tissue with the affected tissue to decrease the risk of recurrence. When reconstruction takes place they have a couple of options and haven’t decided which route they will take quite yet. She will always be without teeth to this part of her mouth and will need implants as an adult.  She will require a feeding tube of some nature during recovering to help keep up her nutrition. This tumor is very rare with less than 500 cases worldwide and there are no leading experts in the country. This tumor often occurs in the upper jaw with only 15 documented cases in the lower jaw like Vienna has. 
We know we have the best team we could have and each physician on our team was hand picked by another respected and talented physician. We know that Vienna will be okay but that she has such a long road ahead. We are very grateful this is not a malignancy and threatening her life. It is a relief to find an answer to her 8 weeks of struggles and to be on the road to helping her have a better life. We have seen the tender mercies of God all along this journey from her getting the best night sleep of her life the night before biopsy, being placed in Dr. Forsen’s care who has then placed us in the care of Dr. Rich, Moulter, Leonard, and Patel, the urgency that everyone has seen in our case, the pain relief she most recently is experiencing, and the rapid nature that The Wash U and Children’s teams have reviewed her case and got us scheduled for surgery. We have great hopes for Vienna to have a great and fulfilling life. 

Newest Update

September 4, 2021

Journal entry by Ashley Akhtar —

We finally made it to craniofacial team at Children’s last Friday. We saw Dr. Patel (our pediatric plastic surgeon), a dentist, and a speech therapist. Dr. Patel is happy with how Vienna looks as well as how she is doing. He notes there is some movement between the two pieces of jaw due to scar tissue building up and prohibiting the bones to completely fuse together. He said he’s okay with this for now and is still very happy with the results from her reconstruction last year. Her dissolvable plate would be dissolved by now and not providing us any stability. He hadn’t told us at the time but told last week that when he went in last year she had built up quite a bit of scar tissue between the two jaw pieces. He said this is why the jaw bones still can’t completely connect, there is scar tissue between the jaw bones. His plan for Vienna is to leave her jaw alone for now. He will plan to do another bone graft and place a titanium plate for more stability and to solidify the jaw as one piece when she is 8-10 years old. He said in the meantime if these two completely fuse together she won’t need that surgery. We as well as he hope this will happen to save her from that surgery. When she is 15-16 years old he will plan to then reconstruct the jaw to give her the proper jaw line. As with all things and especially Vienna’s journey our plans can always change but for now Vienna is doing well and Dr. Patel is happy so we will leave her jaw alone. 
I did get the chance to ask Dr. Patel what he learned from Vienna and what he would do different now reflecting on things. He said he definitely would have started reconstruction immediately when they removed the tumor. He said waiting allowed the scar tissue to build up and ultimately caused her more surgeries in the end. Her risk of recurrence was very minimal now in hindsight. The literature available for this type of tumor report the tumor has a high rate of recurrence, especially when diagnosed young like Vienna was. This is why we waited for reconstruction, to ensure she would not have recurrence. What we all now realize is most of these tumors occur in the upper jaw which requires the surgeons to resect only the tumor and risk leaving cells behind. However, one of Gods many Tender Mercies to us was her tumor being located in the lower jaw. This allowed them to get a good amount of healthy tissue when they removed the tumor which means her rate of recurrence is less that what is found in literature. I have wanted to know what he learned from Vienna so it was good to hear what his perspective is now being on this side of her journey.  
The dentist would like to see her in 6 months to keep a close eye on what we think is a tooth in the very back on the right side. It doesn’t look normal and initially we thought it was exposed jawbone but now it appears it could be a tooth. I did inquire into her molars not being in the correct place and he said they definitely aren’t but there isn’t much that can be done until Dr. Patel reconstructs the jawline. I have to say the dentist is the least impressive doctor we’ve seen on this journey so far. It came out in our conversation that he is planning to retire in the near future and it was very evident to me that he’s pretty much checked out. There are 2 other dentists that he said we could see if we wanted when we return. I will plan to do that if possible. 
We saw an amazing speech therapist, Lynn. She spent an hour with us. Vienna was the most talkative around a stranger that she has ever been. She stays very quiet even around people she knows, except with Kamal, Miss Melissa, and myself. I was so surprised and super excited with how talkative she was in front of the speech therapist. Lynn was excited also to be able to hear her. She is referring us to a feeding team at Children’s. Vienna doesn’t always chew food before swallowing like she should. I’ve noticed it and mentioned it. Lynn thinks it would be good to have a team who specializes in feeding evaluate this. As I told Lynn, if they evaluate her and tell me there is no concern then I’m good with that. Like most parents I want to give Vienna every opportunity to have the best outcome in life. I said to Lynn, this is why I have said through our entire journey “It takes a village” because it does. The more people we have to help us the better off Vienna will always be. 
Vienna started school 3 weeks ago. The school is called “United Services for Children”. The program is a preschool program specifically for children in the Missouri First Steps program. They have different classes for the different needs of the children in First Steps. Vienna’s program is called “Play and Learn” with mostly children needing speech and social skills. I am so happy with the growth I have seen in Vienna’s speech. It’s so amazing. I wish everyone could be a witness to the transformation in her just in the last 3 weeks. Her speech is unbelievably improved. Every day I am so amazed at a new words she says. Our speech therapist saw her yesterday  for the first time in 3 weeks. She was so shocked at Vienna’s speech. She just kept dropping her jaw and looking at me in disbelief. I kept saying to her “I know. I couldn’t wait for you to see her today.” I sincerely wish people could witness this transformation in her. I have to give the credit to going to school now. 
We continue to find joy in our journey and be very grateful for our purple warriors. I hold gratitude daily when I watch Vienna for how far we come. I give credit to her fierce spirit💜. Loving Vienna💜. 
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