Surviving & Thriving with VATER/VACTERL Association
One mother’s journey. One young woman’s story of perseverance.
A baby girl is born with congenital defects
She came out kicking and screaming. They cut the cord and swooped her off to the nurses’ station to clean her up and check vitals. I could hear whispered chatter, concerned tones. “What’s going on? Is she OK?” The OB looked at us and said… ‘her anus hole is closed. We have to run tests immediately.’ And then she was gone. I barely saw her. I don’t remember if I held her. I couldn’t fix it.
I was 27 years old. It was my first pregnancy. She carried so high I could prop a cereal bowl on my belly and walk around. I looked like I was about to birth a beach ball. With swollen ankles, a vitamin deficiency, and terrible lower back pain, I eagerly awaited the arrival of this cherub in June 2000. She came 10 days early after three days of intermittent-yet-intense labor. They broke my water out of pity from my endless labor. After the epidural was in and I was feeling no pain, she finally arrived. We named her Sawyer.
It wasn’t how I pictured it. The movies told me that I was supposed to be gorgeous, glowing, and photo-ready — not puffy-faced with stringy hair desperately in need of highlights. My husband would be there beaming; my mother holding my hand lovingly; my father serving as an assistant coach and at-the-ready with a video camera. Instead, I had a husband scrambling to hold one leg, my poor mother struggling with the other, my father outside the room about to vomit, two in-laws covering their faces, and because it was a teaching hospital, we had the added bonus of six medical interns in a semi-circle looking horrified at my south end.
After she was born it was chaos. My rectum tore during delivery so the OB was frantically trying to sew me up while juggling the afterbirth. Nurses were a frenzy around us and the six interns were frantically writing notes as the OB barked some mix of medical jargon and instructions. My parents and in-laws were in shock and my husband was beside himself in the corner. “Good God. What is happening?!”
For the next two hours, a stream of doctors and nurses came in to talk to us — one after the other with worst-case-scenario horror stories.
She may have one kidney, two over-sized kidneys, or no kidneys at all
She may wear a colostomy bag for the rest of her life
She may never walk
She may never have children
She may be in a wheelchair
She may have an enlarged heart
She may have Hirschsprung Disease
She may have spina bifida
She will need at least 10 surgeries
She may not have a normal life
She may not live through the week
One nurse had such a terrible bedside manner my mother-in-law had to be held back from punching her in the face. Another doctor rattled off at least five terrible options in a row and then abruptly left. In the midst of it all, my husband was given a Valium while I was consulted by a breastfeeding specialist.
My father is an engineer so he immediately got into project manager mode with his mechanical pencil and grid paper in-hand. He took notes, asked questions, served as our champion because I was still in a haze of post-delivery and pain medication. I don’t remember if I slept that first night. We were not allowed to see her. She would be in back-to-back tests for the next three days and when she wasn’t in exam rooms, she would be in the NICU getting held by loving nurses who were still not me.
MRI
EKG
Upper and lower GI
Blood work
Urine tests
X-rays
Brain scans
Hearing tests
Lung tests
This poor 8lb, 6oz, 19inch infant was worked over, poked, prodded, and spit out the other side. Thank God she doesn’t remember. Wearing full protective gear, her father and I were allowed, one-by-one, to hold her and bottle feed her briefly in the NICU.
After the first line of on-call doctors and nurses finally left, they were replaced with actual, knowledgeable specialists who shied away from worst-case-scenario speak and instead focused on facts and solutions. We were in for a 12-year long haul.
The condition
Sawyer Reed Moss arrived on June 22, 2000, around 10pm at Deaconess Medical Center in Spokane, WA. She was born with VATER Syndrome/VACTERL Association — acronyms used to describe a series of characteristics that typically coincide:
V: vertebral (abnormalities in the spinal column)
A: imperforate anus (anus does not open outside the body)
C: cardiac (abnormalities with the heart)
T: tracheal (abnormalities with the windpipe)
E: esophagus (abnormalities with the esophagus)
R: renal (abnormalities with the kidneys)
L: limbs (anomalies with limbs)
After her litany of tests, it was determined she had “two points on the VATER” — as the specialist said. She was born with an imperforate anus and her L4 vertebrae were misshapen — something that would eventually cause severe scoliosis if not addressed with surgery. Her heart, trachea, esophagus, kidneys, limb, and brain were all in good shape.
To add fuel to this little fire — it appears that God has bad aim. She was born with an anatomically correct vagina but what was supposed to be her rectum formed in the wrong place. Her rectum was closed — it was unclear if she had a sphincter. But this other hole formed in the middle between her vaginal opening and what was supposed to be her bum hole. This is where her BMs passed. Without it, she would have needed emergency surgery immediately after birth. As her father said after this news “Rectum? Nearly killed ‘em!” Ugh.
OK — so it could be worse. She would walk, she would talk, and by all accounts, she will hit all the wonderful milestones of healthy babies and toddlers. But there was more. We were told…
- She will have her first surgery to correct her imperforate anus as soon as she is 10 lbs.
- During the pre-surgery time, we will have to use an increase in diameter “dilation rods” to keep the God’s-bad-aim opening from closing so she can continue to pass BMs. These will need to be boiled between uses. She will bleed.
- She will need at least one surgery to correct the imperforate anus but likely two.
- She may not have a sphincter — they will need to surgically create one. We won’t know until surgery.
- If she has a lower imperforate anus — she will not need a colostomy bag. If she has an upper imperforate anus — she may have to wear a colostomy bag for the rest of her life. We won’t know until surgery.
- Her vertebrae abnormality won’t be an issue until she starts growing. She will need to be assessed by Shriners immediately and stay with them until she is 18. We won’t know much until she’s at least two.
- Assuming she isn’t wearing a colostomy bag, potty-training will be difficult. Expect that she won’t be out of a diaper before she is 4 years old.
- She will need a VATER specialist, spinal surgeon, and pediatrician who specializes in kids with this type of condition.
We learned all of this in our first meeting with her VATER specialist. Thank God we lived in a community that had access to exceptional care. Dr. H would be her VATER surgeon, Dr. O would be her spinal surgeon at Shriners, and Dr. R was a pediatrician who just happened to specialize in VATER kiddos. We were covered.
The first two years with VATER
At this time both my husband and I worked full-time. We were lucky to have two sets of grandparents in the vicinity who were willing to help out, as well as a wonderful daycare up the hill from our house. Sawyer had four surgeries in her first two years of life…
Surgery #1: 10wks (Aug 2000)— VATER surgery #1 to correct the imperforate anus. They discovered it was a lower imperforate anus (praise sweet baby Jesus) and no sphincter was present so they had to surgically construct one. The surgery took five hours with a 3-day hospital stay. Even at 10lbs, she fought so hard, it took three nurses to get an IV into her foot. The use of dilation rods would need to continue to help reduce scar tissues from forming.
Surgery #2: 5mo (Dec 2000) — VATER surgery #2 to correct scar tissue issues the dilation rods couldn’t prevent. The surgery was three hours and outpatient. Dilation rods continued for two more months.
Surgery #3: 18mo (Dec 2001) Unrelated to VATER but connected to family history — she also had chronic ear infections since birth. She had to get ear tubes put in. They worked. She was rarely sick after that.
Surgery #4: 2yo (June 2002) Spinal surgery to excise L4 and fuse L3 and L5 taking bone from her hip. The surgery took eight hours at Shriners. We were in the hospital for 10 days. She would be in two body casts over six months.
Taking a step back…
Anticipating one surgery for your tiny infant born with congenital issues is emotionally daunting. Knowing that the baby would go through multiple surgeries was flat out crushing.
I gained 30lbs with the pregnancy. I lost 40lbs in her first two months of life. I could not bring myself to eat yet needed to because I was nursing. As mentioned earlier, my rectum tore while giving birth. I bled for five months before alerting a doctor. It was discovered I had a fissure that was not healing. I, too, went into surgery when she was five months old and sat on a donut for a month afterward. Butt-twins unite!
I worked full-time through it all and took sick leave during surgeries and recovery time. My employer was supportive. I was lucky. The dilation rods were challenging but we got into a groove with them. She did bleed a few times and that was scary.
This sweet little baby was a trooper from get-go. She had the best disposition and always found her smile after each procedure. She was a beast when she came out of anesthesia though — kicking and biting and fighting — those poor nurses. On top of all of this, she was also born with her mother’s skin sensitivities. The poor girl was allergic to her own urine and feces so she had terrible, bleeding rashes and cradle cap. We gave her a lot of “open-air” time and lathered her in Desitin on the bum and Neutrogena TGel on the scalp.
She was loved by her medical teams and we never felt unsupported by the countless nurses, doctors, interns, and family that remained close by and on-alert.
The hardest procedure and recovery was spine surgery. The surgery and time at Shriners were nurturing but very challenging emotionally. Every day we met families dealing with lifelong issues like muscular dystrophy, spinal cord injuries, and cerebral palsy. We saw so many children that will never walk, will be 100% dependent on care their whole lives. Sawyer walked and talked and sang. She would go to school, dance, have friendships, and lead a full life. Being there helped me maintain perspective.
The first cast Sawyer was given was 7lbs. She weighed about 25lbs (25th percentile given her age). It covered 75% of her body: full torso and down one leg with a hole in the belly area for expansion. She chose purple Scotchcast in honor of Barney the dinosaur. She fought so hard during casting that it took nine hours and even the tech had to leave the room for a time because he was so affected by this tiny creature. She was the first 2yo they ever cast. They would do it differently next time. She started with a walker but eventually learned to walk with it on her own. She couldn’t sit so she had to lean on things or lie down. She couldn’t fit in a car seat so we strapped her in using pulley straps similar to those used by skydivers.
The second cast (after 3mo) was also 7lbs but allowed her to sit for short periods. She chose a bright green Scotchcast in honor of Mike Wazowski from Monsters, Inc. I lobbied hard for her to be sedated on casting #2 so it would be less traumatizing for all. She fared well, found new ways to sit, and could fit into a car seat. Much better.
After the first casting experience and hospital stay, I was left in a fetal position on my basement floor. I have never experienced anything so emotionally and physically draining. I eventually picked myself up because this little girl was not stopping. She wanted to go back to daycare immediately so she could show off her new cast to her little friends. We persisted…
Potty-training with VATER: a 9-year gig
Sawyer was ready to potty-train around age three. It did not go well. We had the potty seat, the potty books, and the potty music. We read all the articles, listened to doctors’ counsel, and even formed a small team of cheerleaders that included her grandparents and daycare workers.
She eventually caught on but there were a lot of accidents. Over and over. We went back and forth with Pull-Ups. We never went anywhere without a change of clothes and plenty of wipes.
Age 3, 4, 5, 6, 7, 8, 9 we dealt with this pattern. Sometimes OK, often not. BM accidents at daycare, then school, then after school programs. Her little brother arrived when she was 5-years-old. He potty trained immediately by age 3 and never looked back. This was hard on her and it was exhausting.
Then came the lying and hiding. As she got older she became embarrassed and a little sneaky so she would hide her soiled underwear. I would find them under couch cushions, under her bed, in trash cans.
We decided to consult yet another specialist — someone who could go at it from a psychological perspective. Dr. G was fantastic. She worked with Sawyer for a year, meeting with her weekly to help her work through the psychological side of the condition, find accountability, and create new ways of dealing with accidents. It helped with the sneakiness and lying but did not fix the problem with accidents. What are we supposed to do? Will she still be having accidents on her wedding day? How are we supposed to explain this to her future boyfriends?!
At age nine we tried another specialist. This clinic specialized in constipation and incontinence — mostly in the elderly. For weeks we tried a rectum stimulation therapy designed for individuals with colorectal disorders (aka electrical stimulation therapy) where they put her in a chair and stuck little sensors to various spots on her bum with tiny electrical pulses designed to stimulate bowel function. It was a total bust. She would fall asleep but really liked getting a “prize” at the end of each session.
At age 10 Sawyer was heading into 6th grade at a new school in a new state. My husband and I were going through a divorce. It was a disjointed and disorienting time for everyone. The accidents remained inconsistent and seemingly unattached to anything physical or emotional. We could not negotiate, medicate, or locate a solution. Nothing worked.
Finally, at age 11, Sawyer and I went to a check-up doctor appointment with a spinal specialist from Children’s Hospital Colorado. She asked if we ever tried “bowel management.” We listened and learned and got a referral. A new era was about to begin.
Overcoming VATER through Bowel Management Week at Children’s Hospital Colorado
It was 2011 and we were living outside of Denver, CO. Sawyer was in 6th grade and doing well socially, scholastically, and physically other than sporadic and unpredictable accidents. We had a program — she carried plastic bags, wipes, extra clothes wherever she went. She knew how to deal with it. She had a healthy outlook. She was in school, had tons of friends, was a ballerina with the Arvada Ballet Company, and just signed on as a student with the Rocky Mountain Theatre for Kids. This little spitfire was busy and fulfilled despite the chaos in her private life. Her little brother was 6yo and struggling with the effects of divorce. Their dad moved out of state and was unable to visit often. She helped with the house, chores, and caring for her brother while juggling her own priorities. We added an after-school nanny on our team and I was also in a new relationship with the man that would become my husband in 2012. There was a lot of adjustment.
Sawyer and I attended the orientation for Bowel Management Week in Denver at Children’s Hospital Colorado. There we met about six other families with kids with imperforate anus. Sawyer was the oldest child in the cohort. There were some with upper imperforate anus, two with Hirschsprung Disease, another born with spina bifida, two more with lower imperforate anus. Some wore colostomy bags full-time, a toddler was in a wheelchair, a baby was still in diapers. It was the first time we met anyone with this syndrome. It was the first time I met a parent going through the same thing. We wept.
They began with a thorough exam and in-hospital enema. To fully clean out her tiny body, it took about five hours and more than four liters of enema solution. We found out her body liked to “store stool.” Wonderful. We also found out that the reason she was having accidents was due to nerve damage from her early surgeries. This nerve damage caused her sphincter to atrophy and therefore turned off all signals to the brain that told her she had a BM-a-brewin’. So no wonder she had accidents — she couldn’t FEEL anything. Because her body liked to store stool, she also lost her sense of taste and smell. And one more thing — her growth was stunted because her body had grown toxic. It was a lot to process.
Our team at Bowel Management Week was amazing. They treated the kids like celebrities — gave them a swag bag with a portable DVD player and DVDs, books, toys, and healthy snacks. We met the GI surgeon Dr. B and learned about a bowel management system first developed by a specialist from Cincinnati. The program was an intensive full week so Sawyer and I prepared to fight for a solution.
Here is the program…
- It required the administration of specially designed gentle lavender soap enemas on a daily basis so her body was trained to evacuate each day.
- We were told it may be for life or possibly adjusted as she her body acclimated and if her sphincter re-engaged and began functioning again.
- She could also reduce the need for enemas through a mix of diet, exercise, and vegetable laxatives.
- The enema was a one-hour process involving an IV bag, a liter of saline, soap, and catheter.
- I played nurse — setting up her space, preparing the solution, inserting the catheter attached to the IV bag solution, and completing the enema process.
- She would have to hold in the solution for a timed 10 minutes while watching a show on her portable DVD player or reading a book.
- Then she would spend at least 30 minutes evacuating while continuing with her show or book. Sometimes I stayed in the room and other times she wanted privacy.
- This was repeated every night for a full year. Then it went to five nights/week, then three, then two, then one, and then finally after two years she was done.
The result of the enema program was the elimination of toxins, a clean large intestine and colon, reactivation and engagement of her sphincter muscle, the loss of perpetual bloating and hard stomach, and newfound energy, color, and appetite.
In case you are keeping track, it took 11 years, 6mo, 9 days, and 10 specialists to find a solution.
Life after VATER
By age 13 — Sawyer was cured of the effects of VATER and imperforate anus.
In the first year after starting the enema program…
She gained 20lbs
She grew five inches (with stretch marks to prove it!)
She started puberty
She regained her sense of smell
She could taste food for the first time
In 2012 we started a new life and new family. I re-married and we joined a family with two boys. Together we make six and we are celebrating our 8th wedding anniversary in July.
Sawyer thrived through the rest of middle school and high school.
She was active in school, choir, and theater.
She was a leader among her peers and loved by her teachers.
She was the lead in her senior play.
She went to dances, had boyfriends, sang in choir concerts, and mentored younger students.
She got her driver’s license at 16 and her first job that same summer.
She went to college across the state and is majoring in business and entrepreneurship.
She worked 40 hours/week last summer to save up for her first car.
She came home at the start of #COVID19 and stayed three months to help with her younger siblings and around the house while she finished her final quarter of her sophomore year.
She is loving, caring, talented, and giving.
I was one of many partners with her in this life’s journey. Many others also deserve credit for her success. Her father, grandparents, stepfather, siblings, aunts and uncles, friends, childcare workers, and of course, the many doctors, anesthesiologists, and nurses who treated her along the way.
I know she will always rise above. She credited surfer Bethany Hamilton as her inspiration as she, too, was a young girl who persevered through the toughest of obstacles- losing her arm to a shark as a pre-teen.
Today, June 22, 2020, is Sawyer’s 20th birthday. I will forever be in awe of her amazing spirit.
“Courage doesn’t mean you don’t get afraid. Courage means you don’t let fear stop you.” — Bethany Hamilton
In her own words
In 2019 Sawyer was in a speech class. She was given an assignment to speak about something that had a big impact on her life. She bravely chose to share her story about being born with VATER. Here it is — in her own words.
If you are a parent, relative, or individual living with VATER/VACTERL Syndrome, please know that you are not alone. Here are some resources and support groups that can help. Be educated. Stay informed. Seek support.
- VACTERL Association Support Group (website)
- VACTERL Association Support Group (Facebook Group)
- VACTERL Network (website)
- VACTERL Network (Facebook Group)
- VACTERL Association (private Facebook Group #1)
- VACTERL Association (private Facebook Group #2)
- National Organization for Rare Disorders (NORD) and VACTERL Association resources
- National Center for Advancing Translational Sciences (NIH)information about VACTERL association
Read the next installment in the series: VACTERL Diaries: Newborn
