Viola Marie's Heart Journey
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Just a little background: our sweet Viola Marie was born on July 29th, 2015. She was evicted at 41 weeks gestation and was a wonderful little baby with ALL the hair. Before the age of 1, it was suggested to have her genetically tested for a possible syndrome. The doctor had no specific syndrome in mind but based his recommendation on physical features and a gut feeling. After nearly a year wait, we started the process at the University of Iowa Children’s Hospital. After months of waiting on results, it was found in March of 2018 she has a duplication of material on chromosome 16 affecting 179 genes. Because there is no other documented exact duplication, other than her expressive speech delay and a few mild physical features, the doctors were unsure what all it could affect as she showed no symptoms of health problems aside from picking up viruses like many toddlers.
In May of 2018, Viola was to have an endoscopy to test for celiac disease which the doctors were questioning from some blood results. The gastroenterologist wanted her to have an echocardiogram (ultrasound of the heart) as a precaution for being put under anesthesia for the procedure due to her chromosome disorder. The results changed our world forever.
On May 23rd, 2018, we were told Viola has a congenital heart defect called a bicuspid aortic valve. The doctor said this CHD wouldn’t cause many problems in her life at all. The devastating part was we were told she has a “severe” case of primary Pulmonary Vein Stenosis (PVS), a rare, serious, and frequently fatal condition in which there is an obstruction in the pulmonary veins that bring oxygen-rich blood from the lungs back to the heart. It can be isolated to a single pulmonary vein, but most often occurs in multiple veins simultaneously. On that date, we were told it was in the bottom two veins for sure and also possibly in one top vein. The stenosis occurs due to abnormal cell growth which narrows the walls of the veins. PVS frequently progresses. As a result, partial loss or even total obstruction of flow to a vessel/s may occur.
After a lot of waiting for appointments, having more tests, and waiting on results, we have more definitive answers. We found out from a diagnostic heart catheterization done on August 1st, 2018, that her bottom two veins are, in fact, severe, her upper left vein is open and clear, and her upper right vein is nearly occluded (closed). Also, as a result of her PVS, Viola has developed Pulmonary Hypertension (PH), an abnormally high blood pressure that occurs in the arteries of the lungs. As a result, the heart works harder to pump blood through the lungs, causing pressure in the pulmonary arteries to rise.
We were given the plan of a balloon catheterization in four weeks for the lower veins and to attempt the upper right vein. After I relayed the results and plan with a fellow PVS mother on Facebook, she contacted the PVS team at Boston Children’s Hospital (BCH) on our behalf because after having been dealing with this disease for years in her own children, she was worried about the slow approach knowing how fast PVS can move. BCH is one of the only hospitals in the country with a formal program for this specific condition. The nurse of the PVS team was leaving the country the next day, but still gave her number to be passed on for us to call that night if we so desired. I spoke to her late Thursday night (8/2) explaining Viola’s condition and results found thus far. After hearing everything, she said she believed Viola should be seen at BCH quite soon. She called the BCH PVS team the next morning to make her a priority and get the ball rolling which has let us to where we are now. The BCH doctor told me Viola is sort of in a category of her own with this considering she was diagnosed with primary PVS at nearly 3 years old and not because of showing symptoms. It usually hits hard and is aggressive in the first six months of life. So for this, we are truly blessed.
Viola will undergo an open heart surgery called a sutureless repair where the surgeon will open the pulmonary veins and remove as much of the obstruction as possible to allow blood to flow more efficiently back to the heart. This should also correct the PH. They will analyze the removed material to get an idea if the cells are active or more like scar tissue, which would indicate it may not progress further.
While our hope and prayer is this surgery will open her veins and she will stay stable for good, we are aware this is not a guarantee as there is no cure. The doctor said she will likely start a chemotherapy drug after surgery. The chemo is used to treat the proliferation of cells causing PVS the same way they would treat the growth of a cancerous tumor. Until very recently, it was considered an experimental trial, so we’re not sure how much will be covered by our insurance. We’re hoping it will be a decent amount since its cost as part of the trial was around $4,000 a month. But either way, she will have medical expenses the rest of her life to monitor the disease with echocardiograms, lung scans, and probably heart catheterizations.
For this leg of her heart journey starting next week, Charlie and I will need to take four to six weeks off for the duration of her stay. For those that don’t know, one of the downsides to my career as a closed captioner is we receive no paid leave. Charlie will exhaust all of his paid leave and dip into FMLA which is also unpaid. So your support not only helps cover the costs of Viola’s surgery and extended treatment, but also to help maintain our household bills (mortgage, cars, insurance, utilities, etc.) along with travel, lodging at $200+ per night, transportation, and necessities that will add up very quickly in Boston while we lose our income.
We are very obviously terrified of the diagnosis, surgery, and financial burden, but we have faith God is with us and will help us through. We are extremely grateful for any and all donations, and would appreciate it very much if y’all would share this fund raiser to cast it far and wide for our beautiful little girl’s journey and raise awareness of this very rare and awful disease. Subtle symptoms you wouldn't think of, and everything looks fine until it's dire unless caught like Vi's. Even if you can’t donate, we would gladly accept prayers for Viola. Thank you so very much!!!
-Tracy, Charlie, Viola, and Livonia Steele
In May of 2018, Viola was to have an endoscopy to test for celiac disease which the doctors were questioning from some blood results. The gastroenterologist wanted her to have an echocardiogram (ultrasound of the heart) as a precaution for being put under anesthesia for the procedure due to her chromosome disorder. The results changed our world forever.
On May 23rd, 2018, we were told Viola has a congenital heart defect called a bicuspid aortic valve. The doctor said this CHD wouldn’t cause many problems in her life at all. The devastating part was we were told she has a “severe” case of primary Pulmonary Vein Stenosis (PVS), a rare, serious, and frequently fatal condition in which there is an obstruction in the pulmonary veins that bring oxygen-rich blood from the lungs back to the heart. It can be isolated to a single pulmonary vein, but most often occurs in multiple veins simultaneously. On that date, we were told it was in the bottom two veins for sure and also possibly in one top vein. The stenosis occurs due to abnormal cell growth which narrows the walls of the veins. PVS frequently progresses. As a result, partial loss or even total obstruction of flow to a vessel/s may occur.
After a lot of waiting for appointments, having more tests, and waiting on results, we have more definitive answers. We found out from a diagnostic heart catheterization done on August 1st, 2018, that her bottom two veins are, in fact, severe, her upper left vein is open and clear, and her upper right vein is nearly occluded (closed). Also, as a result of her PVS, Viola has developed Pulmonary Hypertension (PH), an abnormally high blood pressure that occurs in the arteries of the lungs. As a result, the heart works harder to pump blood through the lungs, causing pressure in the pulmonary arteries to rise.
We were given the plan of a balloon catheterization in four weeks for the lower veins and to attempt the upper right vein. After I relayed the results and plan with a fellow PVS mother on Facebook, she contacted the PVS team at Boston Children’s Hospital (BCH) on our behalf because after having been dealing with this disease for years in her own children, she was worried about the slow approach knowing how fast PVS can move. BCH is one of the only hospitals in the country with a formal program for this specific condition. The nurse of the PVS team was leaving the country the next day, but still gave her number to be passed on for us to call that night if we so desired. I spoke to her late Thursday night (8/2) explaining Viola’s condition and results found thus far. After hearing everything, she said she believed Viola should be seen at BCH quite soon. She called the BCH PVS team the next morning to make her a priority and get the ball rolling which has let us to where we are now. The BCH doctor told me Viola is sort of in a category of her own with this considering she was diagnosed with primary PVS at nearly 3 years old and not because of showing symptoms. It usually hits hard and is aggressive in the first six months of life. So for this, we are truly blessed.
Viola will undergo an open heart surgery called a sutureless repair where the surgeon will open the pulmonary veins and remove as much of the obstruction as possible to allow blood to flow more efficiently back to the heart. This should also correct the PH. They will analyze the removed material to get an idea if the cells are active or more like scar tissue, which would indicate it may not progress further.
While our hope and prayer is this surgery will open her veins and she will stay stable for good, we are aware this is not a guarantee as there is no cure. The doctor said she will likely start a chemotherapy drug after surgery. The chemo is used to treat the proliferation of cells causing PVS the same way they would treat the growth of a cancerous tumor. Until very recently, it was considered an experimental trial, so we’re not sure how much will be covered by our insurance. We’re hoping it will be a decent amount since its cost as part of the trial was around $4,000 a month. But either way, she will have medical expenses the rest of her life to monitor the disease with echocardiograms, lung scans, and probably heart catheterizations.
For this leg of her heart journey starting next week, Charlie and I will need to take four to six weeks off for the duration of her stay. For those that don’t know, one of the downsides to my career as a closed captioner is we receive no paid leave. Charlie will exhaust all of his paid leave and dip into FMLA which is also unpaid. So your support not only helps cover the costs of Viola’s surgery and extended treatment, but also to help maintain our household bills (mortgage, cars, insurance, utilities, etc.) along with travel, lodging at $200+ per night, transportation, and necessities that will add up very quickly in Boston while we lose our income.
We are very obviously terrified of the diagnosis, surgery, and financial burden, but we have faith God is with us and will help us through. We are extremely grateful for any and all donations, and would appreciate it very much if y’all would share this fund raiser to cast it far and wide for our beautiful little girl’s journey and raise awareness of this very rare and awful disease. Subtle symptoms you wouldn't think of, and everything looks fine until it's dire unless caught like Vi's. Even if you can’t donate, we would gladly accept prayers for Viola. Thank you so very much!!!
-Tracy, Charlie, Viola, and Livonia Steele
Organizer
Tracy Jo Steele
Organizer
Urbandale, IA
