New mom tackles lethal illness

Comprehensive Lung and Breathing Program helps provide lifesaving treatment
June 22nd, 2017

The young mother lay in the Intensive Care Unit, color draining from her body. Her organs were failing. A team of pulmonary and heart specialists raced to try and save her life after a helicopter brought her to UCHealth’s University of Colorado Hospital.

Weeks earlier, before the birth of her second child, Danielle Soto, then 24, had been energetic and fit. Her husband was serving in the Army at Fort Carson in Colorado Springs. Danielle worked at a dialysis center, went to school and loved working out, hiking in the mountains and taking their son, Nico, to the Garden of the Gods where he could climb on little rocks while she gazed up at a cathedral of red spires.

Danielle Soto with her husband, Sean. Photo courtesy of Destiny Marvin.

Now Danielle could barely breathe. She held her mother’s hand and asked a question that was haunting them both.

Danielle Soto, right, with her mom, Destiny Marvin, at Red Rocks. Photo courtesy of Destiny Marvin.

“Mom, am I going to die?”

Destiny Marvin did not know the answer, but refused to succumb to their worst fears.

“She was blue. I’m holding back tears. I’m panicking inside,” Destiny recalled.

Still, Destiny willed herself to be strong and fight for both of them.

She squeezed Danielle’s hand and said, “You are still alive. Let’s stay positive. We have tons of people praying for you and you have the best doctor.”

Mystery illness

Danielle had just given birth to her baby girl, Haven, on May 10 of last year. After coming home, severe exhaustion overwhelmed her. She lay in bed around the clock, nursing Haven and cuddling with Nico, who was then 4. Her husband, Sean Soto, then 23, tried to be home with his wife and children as much as possible. But he had to go to work too. When Sean was away, Danielle remembers forcing herself to get out of bed every few hours to try and make sure she and Nico kept eating. Even getting up to go to the bathroom took extraordinary effort.

Danielle son plays on rocks with his cousin. Photo courtesy of Destiny Marvin.

Danielle knew something was wrong and kept checking with her obstetrician, who wondered if she had lost too much blood during the birth and was anemic.

Or perhaps she was dealing with post-partum depression. Danielle tried medication for both conditions, but continued to decline.

Over Memorial Day weekend, Danielle tried to go to Target with Sean to do a little shopping. They put Haven in the cart in her baby seat and Nico skipped in with his dad. Danielle could barely make it from the parking lot to the front of the store. She told Sean she had to sit and rest, that he should go ahead.

“I was walking like a 90-year-old woman. I could see my heart beating in my chest. I was watching everyone else walking by and thinking, ‘How can they walk so easily?’ Then it hit me. I waited for my husband and told him, ‘I need to go to the hospital right now.’”

The couple returned to their Colorado Springs home and waited for Danielle’s grandmother to come watch the children. Then Sean took his wife to the nearest hospital.

“They knew something was wrong, but didn’t know what it was,” Danielle said.

She spent a miserable night struggling to breathe.

Danielle and her husband, Sean Soto. Photo courtesy of Destiny Marvin.

 

“I literally had to tell my brain to take a breath in and out. It was like someone was smothering you or an elephant was sitting on your chest or you were at the bottom of a dog pile.”

In the morning, a cardiologist conducted an echocardiogram to assess Danielle’s heart function and within five minutes said he needed to rush her to UCHealth University of Colorado Hospital (UCH). He told her she had a rare illness called pulmonary hypertension.

“You’re going to be on Flight for Life. I know a doctor who specializes in this. He’s going to help you.”

There was no room for Sean on the helicopter. Everything became a blur for Danielle. Sean called her mom and told Destiny to race from her home south of Denver to Aurora so she could meet Danielle.

“It was surreal. When I got to the ICU, there were probably 15 or 20 doctors and nurses, and my mom was waiting,” Danielle said.

Among them was Dr. David Badesch, the man who would help save Danielle’s life.

Research fuels lifesaving treatments

Badesch heads UCHealth’s Pulmonary Hypertension Program at the University of Colorado Hospital. It’s one of the leading research centers for the disease in the world. For decades, the Comprehensive Lung and Breathing Program has led and participated in vital clinical trials for drugs that have dramatically boosted life expectancy for patients suffering from pulmonary hypertension.

“Back in the 1980s, when the disease was originally being described, the survival was only 2.8 years,” Badesch said.

Now, thanks to extensive research, median survival has improved to seven years or more. And some patients do much better.

When Danielle arrived for help, she was in grave shape.

“She was at considerable risk of dying. If she had come in even a few days later, it might have been too late. She was already in shock with liver and heart failure,” Badesch said.

“We see many sick folks and she was among the most ill at the time of presentation. Her case necessitated a very fast evaluation and getting her immediately on to therapy. We have a fantastic team of nurses, physicians and nurse practitioners,” he said.

Almost immediately, doctors threaded a catheter through Danielle’s jugular vein in her neck, then through the superior vena cava and the right side of her heart into the lungs. Known as a right-heart catheterization, the procedure allowed the team to directly measure blood pressure in the lungs and blood flow generated by the heart. They confirmed that Danielle was suffering from pulmonary hypertension. As with many of the most severe cases, the cause for her case is unknown and therefore called idiopathic pulmonary hypertension.

It’s possible that the hormonal changes in pregnancy aggravated her illness; given that her heart had swollen to two times its normal size, doctors told Danielle that they were amazed she managed to give birth to a healthy baby and survive herself.

Once the diagnosis was confirmed, Badesch and his team began giving Danielle a medication that they helped develop and test at UCH more than 20 years ago called prostacyclin. They started with a small dose and increased it gradually.

While Danielle was finally receiving lifesaving treatments, she was not out of the woods.

Her mom remembers keeping a close eye on her coloring and vitals.

Danielle gets a visit from her little sisters during her treatment at UCHealth’s University of Colorado Hospital. Photo courtesy of Destiny Marvin.

At first, her nails were chalky from lack of oxygen. Her kidneys were failing to produce urine.

“I remember looking at that bag and saying, ‘Please God, let there be urine. Let her organs start working again,” Destiny said.

Destiny was unrelentingly positive with Danielle, but sometimes had to take breaks in a nearby sanctuary room.

“I had to walk into that room. I dropped to my knees and cried my eyes out. But somehow she pulled through. Her organs started to pull through,” Destiny said.

Danielle was coming back to life, but was angry. She wanted to be with her baby, son and husband. She wanted to be a normal young woman again. She wanted to be able to go to the gym and do cardio and strength training like she always had. She didn’t want to face the idea that she was stuck with a scary, chronic illness.

The caregivers understood.

“Dr. Badesch and his team were so gentle. They went over the top to be supportive of us as a family. Danielle really is alive because of how fast they moved,” her mother said.

Making peace with a new life

The medicine that keeps Danielle alive is also her pesky companion. Along with the accoutrements of all moms — like diaper bags, snacks and car seats — Danielle must carry a little backpack full of medication and backup doses everywhere she goes. The backpack contains a pump loaded with a cassette of prostacyclin. The medication travels from the pump through tubing to a catheter in Danielle’s chest. It delivers infusions directly to Danielle’s heart.

While the medication keeps Danielle alive, it requires great attention to detail. Danielle has to keep everything sterile and carefully load new doses of the medication each night.

As she recovered, she had to find the will to live with permanent restrictions.

For instance, doctors often warn patients with pulmonary hypertension not to spend time at high altitude, but Danielle loves the mountains. To begin to feel like herself again, the family took a brief trip to Summit County last August. Danielle had to monitor her oxygen levels, but as she breathed in the scent of pine trees and gazed out of over Lake Dillon, she began to make peace with her losses.

“I was still sad about the whole thing, but it showed me I could still go to the mountains,” she said.

The family recently celebrated Haven’s first birthday. These days, Haven is saying da da, mama and baby as she carries baby dolls around. She loves dancing and adores her big brother.

Danielle has learned to relish every moment with her children.

“I would hate to miss all the special things they’re doing,” Danielle said. “I’m grateful to be able to take care of them.”

She also has begun to think about the future. She’s eager to return to school someday to earn a master’s degree in education.

“I want to teach at a Christian elementary school,” Danielle said.

And she’s dazzling Badesch with her fitness levels. She’s back to working out and each time she comes in for a follow-up visit, she completes a step test to prove her mettle. During a recent visit, Danielle asked if she could run during the test. Badesch gave his approval.

“I don’t care how you get there,” he said.

Danielle loves working out, including with her boxing trainer. Photo courtesy of Destiny Marvin.

“OK,” Danielle joked. “Next time I’ll bring my roller skates.”

Danielle said she is forever grateful to Dr. Badesch and his team.

“They made sure I understood all that was going on. I always have their support. They made it a little easier for me to accept (my illness),” she said.

As for Dr. Badesch, seeing a patient like Danielle thrive fuels the motivation for groundbreaking research.

“It’s a huge challenge to actually cure this disease. Right now, what we have are treatments that are successful in controlling it,” Badesch said.

The onset of pulmonary hypertension is often between age 20 and 50. While the idiopathic form is rare and Danielle’s case was especially severe, Badesch has treated other young mothers who were stricken. Decades ago, when young women received the diagnosis, they often were advised to “get their affairs in order.”

“Young women essentially were being given a fatal diagnosis. But now (with improved treatments) and continuity of care, we see many of these women back 15 or 20 years later. Their kids have grown up. That’s what excites me most about what we do,” Badesch said.

Danielle continues to do remarkably well and the team will keep a close eye on her.

Said Badesch: “Hopefully, we’ll be able to see her raise her baby.”