Tracy & Tyler Wright's Story
“I believe your daughter has Infant Botulism. There is a treatment, but it is only made in California. She may get worse before she gets better. She may lose her breathing.” These words are still very vivid to us to this day. These are the words that were spoken to us only a short time after our five-month-old baby, Madison, entered the Emergency Room at GBMC. Dr. Jenna Hammond was on call that evening. She saved our Madison’s life! We would like to share our story with you.
We took our five-month-old daughter, Maddie, to GBMC's ER at 10:00 p.m. on Friday, January 9th. Two days prior, she had gotten her first two teeth. We first noticed a few subtle differences in her behavior, but over the next couple days we watched our daughter’s normal, happy and energetic demeanor change into a very weak and sick child. We could not figure out why she would not nurse and why she had these sad, droopy eyes. She was very lethargic and weak, to the point that she could not hold her head up. She also was very fussy, but her cries were becoming weaker. We associated all of this to a bad case of teething, and thought that dehydration was causing her to be so lethargic. We had spoken to our daughter’s pediatrician’s office several times and had even been to an ER at another nearby hospital. All tests came back negative and she didn’t have a fever so we were sent home. Thankfully, we stuck with our own instincts that something was very wrong with our daughter, and we took her to GBMC. GBMC has a separate pediatric ER, unlike many other hospitals.
Within minutes of stepping in the door, the Pediatric ER triage nurse examined Maddie and noticed her poor state. She took vitals and immediately sent us to a room where nurses were already waiting. Maddie's vitals showed no fever, her lungs sounded clear, and she had a good oxygen level and good pulse, but she was very pale and her eyes were closed and appeared sunken in. The soft spot on top her head was also sunken in and her body was limp, like a rag doll. It was very clear that she was extremely dehydrated.
Almost immediately after we arrived in the room, Dr. Jenna Hammond came in to see Madison. She immediately commented on Maddie's lack of head control and her dehydration symptoms. She also checked her eyes and commented that her pupils were not dilating. We could tell that she was concerned and questioned us about her weak cry and if she was constipated. In fact, she had been constipated for several days, but that was not that unusual for Maddie. Then, Dr. Hammond looked at us and asked a very unusual question, “Has she had any honey?” We were taken aback by this since we thought it would be an odd question to ask. Plus, we would never offer honey to her at her young age. Dr. Hammond left the room and told us that she would be back after she made some phone calls. This is the moment that we knew that something serious was wrong with our daughter.
Maddie laid there in a lifeless state on the hospital bed with many nurses working on her. Due to her severe dehydration and lack of circulation, the nurses had a difficult time putting an IV in Maddie. Finally after multiple attempts and a few frustrating moments, a NICU nurse was able to get an IV in her. They quickly took her blood for testing and then hooked her up to IV fluids. After almost two days, Maddie was finally able to get some much needed hydration, which gave us a sense of some relief.
By this time, our heads were spinning. We were confused and exhausted. We were up the entire night before at the ER and were in the midst of having a second night in a row with no sleep. We were drained, but we were trying to stay calm so that we could process everything that was happening. We wanted to call for support from our family members, but with the urgency of everything happening around us, we couldn’t. The staff at GBMC continued to attentively work on Maddie so that they could run tests to determine what was wrong with her.
It was not long before Dr. Hammond arrived back into our room and delivered us her preliminary diagnosis. She said the poor dilation of her eyes and lack of body control suggested it was a neurological and muscle issue. She wanted to do more tests to rule out meningitis and head trauma, but Dr. Hammond was fairly confident that Maddie had a different illness, one that was very rare. Dr. Hammond believed it was Infant Botulism.
We sensed the urgency from the concerned look of Dr. Hammond’s face and the tone in her voice. She said that Infant Botulism (IB) is a very severe and rare bacterial infection that causes weakness and loss of muscle tone in young babies. It causes paralysis of their entire body and blocks the nerves from working. The illness typically begins with constipation and is usually first noticed by difficulty feeding due to poor sucking and swallowing. Babies also have a weak cry, diminished facial expression, and poorly dilating pupils. Maddie had all of the tell-tale signs of IB.
Dr. Hammond did say there was a treatment in California and that if we needed it, it would be flown here for us. She also told us that we would not be staying at GBMC. Many babies with IB lose their ability to breathe and we needed to be at a hospital such as Johns Hopkins or Sinai where they would be able to give her the respiratory support that she may need.
We asked Dr. Hammond if she was confident in her diagnosis and she said she was reasonably certain even though she had only seen one prior case the year before. Dr. Hammond wanted to rule out the other conditions by doing a round of tests, which included a CT scan and lumbar puncture which is similar to a spinal tap. We quickly were transported to another room to get prepped for the tests. I was allowed to stay with Maddie and hold her hand during the CT scan. For the lumbar puncture, we stayed with her while they prepped her. The nurses asked us to wait in the waiting room during the procedure. While in the waiting room, this is the moment when all of the emotions that had been building over the course of the last 24 plus hours finally hit and I started sobbing. I knew something was severely wrong with my baby and wasn’t processing it. Dr. Hammond said that she thought it could be Infant Botulism, but how? My husband quickly started doing some research on his smart phone.
He found that babies contact it from swallowing botulism spores, which then produce toxin in the baby's large intestine. The toxin poisons the baby and can cause paralysis. Medical research does not fully understand all the factors that make a baby susceptible, but they do know honey is a source of the botulism spores. It was also concluded that most IB patients acquired the spores by swallowing microscopic particles found in soil and dust. The IB condition is so rare that only 75 to 100 babies contract the illness in the US each year.
What baffled us was that we didn’t give Maddie any honey and she wasn’t near soil! It was January 9th and there was snow on the ground and it was frozen! We hadn’t even left our house many of the days prior to her symptoms. Despite Dr. Hammond and my husband thinking that it was IB, I still had my doubts. I sat in the waiting room crying and wondered if she would ever be the same happy little girl ever again. I looked at her, unable to move her body or extremities, with her pupils dilated, and thought that permanent brain damage had already happened.
By 1:00 a.m., Maddie had received all her additional tests. Her CT scan came back normal and the spinal tap did not show any abnormalities. Although IB would not be confirmed for another week through analysis of a stool sample, Maddie was going to be treated as though she had it. An ambulance soon arrived to take Maddie to Sinai Children’s Hospital.
Maddie spent five days in the Pediatric Intensive Care Unit (PICU) at Sinai and an additional seven days in the regular pediatric unit. The $45,000 antitoxin treatment, called BabyBIG, was flown to us the following day. By the time it reached us, Maddie was in a very poor state. She was put on an NG feeding tube for the majority of her stay. She could barely move, her eyes were permanently dilated, she couldn’t cry but would whimper, and her breathing was labored. She lay in a lifeless state, and was hung in a sling-like fashion on her bed so that her lungs would be open, allowing her to breathe easier. Thankfully, she did not need to be intubated and put on oxygen. We associate this to the quick reaction of the nurses at GBMC and Dr. Hammond’s quick diagnosis. Although we were only at GBMC for a short three hours, it was the most important three hours of Maddie’s recovery. Had we waited any longer, or if she hadn’t been diagnosed so quickly, the treatment would have been delayed, putting Maddie at even more risk of losing her breathing.
It was an emotional and a physically draining time for my family. We stayed by her side day and night and watched her get better each day. Her improvements were very subtle things at times, such as opening her eyes, or moving her arm. We were even happy when her moaning and whimpering got louder. With each small improvement, it gave us strength and hope that she would be okay.
We were discharged to go home on Wednesday, January 21st. It was the day before Maddie turned six months old. We were happy to be going home, but nervous at the same time. Maddie had made tremendous progress in those two weeks. She could move her legs and arms and her head, and her eyes were responding to light. She still had droopy eyes, but every day they looked a little better. She still lacked head control and had poor muscle tone in her core area. We knew holding herself upright was going to take some time. The NG tube had also been taken out and Maddie was starting to nurse again, although it was still a challenge. She had to relearn how to suck and nursing was very tiring for her. We tried a bottle, but she would become frustrated and refuse to drink it. The doctors planned to admit Maddie to an inpatient rehab center until her feeding got better, but fortunately Maddie started to make better progress and Sinai allowed us to take her home. Sinai arranged for a home nurse to come see her three days a week and she was also going to receive physical therapy services three days a week. In addition to that, we would be seeing her pediatrician on a regular basis – everyday in the beginning to check her weight.
The first couple of days home were physically and emotionally draining. Maddie wasn’t eating well and she wasn’t gaining weight. We were very close to having her re- admitted to the hospital. Thankfully, she started to nurse better and slowly started gaining weight. After those initial first few days home, she started to recover very quickly! The home nurse and the PT were very impressed with her progress and were able to scale back their visits. Maddie was soon able to sit in her walker and even jump in the jumperoo! We were all amazed her how quickly she was bouncing back to her happy self again!
Today, two months after the IB symptoms started, our lives are basically back to normal! Maddie sits by herself, and can walk forward and backwards in her walker. She loves her two-year-old big sister, Lexi, and is already working hard to keep up with her! Starting baby food was a little delayed, but Maddie is finally starting to like it. Sleeping through the night is still a struggle, but with time that, too, will get better.
Most importantly, we are thankful that our little girl is okay and will be able to live a normal and happy life! We are so grateful for all of the support that we received from our family and friends, but mostly all the wonderful nurses and doctors that took care of her. We give most of our thanks to Dr. Jenna Hammond at GBMC for her quick diagnosis, and the BabyBIG treatment that gave our baby her life back!