IDAHO FALLS — It was a fight for two lives: A woman with an aggressive lung disease was trying to survive for her unborn daughter. In August, she was able to give birth safely and she and her baby are alive.
However, her baby was born with a condition that she and her husband want to raise awareness about called MCAD deficiency, which is a rare metabolic disorder.
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Jasen, 35, an Army veteran, and Ambrosia Howell, 30, lived in Idaho Falls. They moved to Utah over the summer for specialized care at the University of Utah in Salt Lake City when they learned Ambrosia had a high-risk pregnancy.
At the time, an online fundraiser helped Jasen and Ambrosia.
“My family is so grateful to the community for all their love and support, and to the people who have donated to us. We are truly forever grateful,” Ambrosia said.
Doctors told them the risk of death for their unborn daughter and the risk of death for Ambrosia was high. Their daughter’s due date was September 5.
“I wanted to have my little girl and give her a chance, even despite my own health. And everything worked out, you know?” » said Ambrosie.
Aurora Rosalie Ayla Howell was born at the University of Utah Hospital on August 19 at 2:43 p.m. She weighed 6 pounds, 14.4 ounces.
Aurora is currently just over two months old.
“I feel a lot of joy and love… We did it. It was the best possible scenario,” Ambrosia said. “(The doctors and staff) really took great care of me.”
“I’m so proud of my wife who has been strong through all of this. People like her family know about her illness, but no one really sees how much she struggles and how much she has had to overcome and overcome. She has been so strong her whole life,” Jasen added. “She’s a fighter.”
Background
Ambrosia was diagnosed with stage 4 bronchiolitis obliterans last year in Pocatello when she was just 29 years old. This is the most severe stage.
According to the American Lung Association, it is a rare chronic lung disease that worsens over time. This leads to inflammation, irreversible damage and scarring. She has been on oxygen as an adult since 2024.
Click here for a previous article written by EastIdahoNews.com about Ambrosia.
Fast forward, Ambrosia and Jasen had an unplanned pregnancy, and this would be their first child.
She had met with maternal-fetal medicine doctors in Utah, and they told her there was a chance she wouldn’t make it and that she was considering aborting the baby.
“I understand the risks. This is our first baby. I love our baby and I think as long as we have a good team, I think our baby deserves a chance to live,” Ambrosia said in a previous interview with EastIdahoNews.com.
Ambrosia said Jasen was worried during her pregnancy. He had nightmares.
“Because they had told us about the risk of death for the baby and the risk of death for me was very high, he was worried about that. You know, ‘Am I going to come home to a wife and a baby? Am I going to have one or the other? Am I going to have both? Am I going to have neither?’ He was really scared,” Ambrosia said.
Pregnancy
Doctors were able to closely monitor Ambrosia and her pregnancy in Utah and performed several non-stress tests to make sure her baby girl was okay. According to the Cleveland Clinic, a non-stress test (NST) is a simple, painless prenatal screening test that measures your baby’s heart rate and reaction to movements or contractions.
This is usually done in high-risk pregnancies to check if the baby is healthy and getting enough oxygen.
The plan was to have Ambrosia induced on August 30 and she would have an assisted delivery with forceps.
The Cleveland Clinic states that a forceps-assisted birth is an “operative vaginal delivery in which a health care provider uses specialized metal, spoon-shaped or forceps-shaped instruments to gently cradle and guide the baby’s head through the birth canal during the second stage of labor.”
“By the time I got to 8 months (pregnant), I was getting to the point where I was extremely struggling. I had asked my doctor if there was a way to be induced earlier because they told me they wanted me to go as far as 39 weeks as possible,” she said.
It was difficult with her lung disease and she was in pain.
Delivery
Ambrosia asked her doctors if she could be induced on August 19. She went for a non-stress test on August 18.
“When I was there and they were looking at our baby, there were times when her heart rate would drop a little bit before it came back up again, so they were wondering if she was getting enough oxygen,” she said.
She was then sent to the obstetrics department at the University of Utah to make sure the baby was okay and it was decided she needed Aurora. Ambrosia worked for 13 hours.
“I was very nervous. It was really hard to see her in so much pain,” Jasen said.
There were many people in the room, including maternal-fetal medicine doctors, a pulmonologist, an anesthesiologist and nurses.
Once Aurora was born, doctors put her on her mother’s chest for a minute or two. She was rushed to the pediatric table because she didn’t have enough oxygen. She spent 12 days in the neonatal intensive care unit (NICU).
Diagnosis of MCAD deficiency
“They did a newborn screening,” Jasen said. “When he came back, he came back with MCADD, where the second D stands for deficiency.”
They had never heard of it before. When they were informed of the gap, they were provided with documents.
“When I read those documents, I cried and Jasen had to console me because I didn’t realize it was that bad. I became really anxious and worried about our baby and what the future was going to look like,” Ambrosia said.
Amber Wright, a board-certified pediatric hospitalist, works at Eastern Idaho Regional Medical Center in Idaho Falls. She explained to EastIdahoNews.com that MCADD is an inherited metabolic disorder. It stands for Medium Chain Acyl-CoA Dehydrogenase Deficiency.
“Medium-chain acyl-CoA dehydrogenase is the protein that breaks down medium-chain fatty acids. Basically, medium-chain fatty acids cannot be metabolized due to a mutation in the gene that produces the protein needed to break down fatty acids,” Wright said. “The problem is that you can’t metabolize fat for energy, so you can get dangerously low glucose levels and a toxic buildup of partially digested fat.”
She said most patients with MCADD are diagnosed from newborn screening. She explained how it works if a baby tests positive. The metabolic geneticist receives the positive test and immediately notifies the pediatrician who contacts the family. Wright said that’s when the family works directly with the metabolic geneticist and pediatrician for treatment.
Wright said knowing about an illness through newborn screening before symptoms develop not only saves lives, but will save babies from suffering serious, life-threatening neurological damage from a metabolic crisis that could have been avoided.
“I cannot stress enough the importance of newborn screening. If a couple has a baby with MCADD, it is very likely that at least one parent carries the gene. These couples should definitely speak with a genetic counselor to be aware of the chances of having another child with MCADD so they can prepare,” she said.
It is a lifelong illness. There is no cure, but life expectancy is normal if managed well.
“The most important thing is to make sure people with MCADD don’t have hypoglycemia, so don’t go too long without food. Babies need to be fed every two hours. As children get older, they may go longer without food,” Wright said.
She added that metabolic geneticists typically give families a take-home letter with instructions for doctors on how to treat MCADD, as well as their contact information for further advice. People with MCADD may end up in the hospital.
MCAD deficiency triggers sudden and potentially fatal energy outages. If Ambrosia and Jasen need to go to the emergency room because Aurora isn’t eating when she’s supposed to or gets sick, she has an emergency metabolic disease protocol that was written for her by the University of Utah Metabolic Genetics Clinic.
Aurora has already been to the emergency room twice.
Part of Aurora’s protocol sent to EastIdahoNews.com reads:
“Parents have been instructed to take the child to the emergency room if she is unable to eat or has a high fever, vomiting and/or diarrhea. MCAD deficiency can cause sudden cardiac arrest and sudden death in cases of illness or metabolic stress if not treated appropriately. This child must be seen by the emergency doctor as soon as possible.“
The letter states how doctors should treat it and what tests should be done.
“It’s financially stressful, but also emotionally devastating, because even though we’re extremely exhausted like all new parents, we have to keep going,” Ambrosia said.
Aurora needs to eat when she needs to eat. At the tired parents stage, Ambrosia and Jasen set alarms to make sure Aurora didn’t miss meals.
“If we miss that wake-up call to get up and feed her because we were taking a nap, then her levels will drop too low and crash…and then she’ll have a…
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