The Monahan Triplets: Managing Cystic Fibrosis Times Three

When she got word that her newborn triplets had cystic fibrosis (CF), Hannah Monahan thought it was surely a mistake. The test results must be wrong, she thought.

Already a mother of four, her other children did not have the disease, despite her being a carrier of the genetic mutation that can cause CF.

The medical news didn’t change, however. Mabel, Eden and Ender Monahan indeed had CF, a progressive, genetic disease that affects the lungs, pancreas and other organs. They join about 40,000 adults and children in the U.S. living with the disease.

In people with CF, a genetic mutation causes a protein meant to move chloride (a part of salt) to the surface of a cell to malfunction. The Cystic Fibrosis Foundation (CFF) says without the chloride to attract water to the cell surface, the mucus in organs such as the lungs, pancreas and liver can become thick and sticky.

In the lungs, that can make it difficult to breathe. In the pancreas, it causes digestive issues.

For the Monahans, who live in Jim Thorpe, having one more child turned into having three more, all with a condition that requires daily medication and treatment.

“It’s not easy,” Hannah, 33, explains. “It’s chaotic and stressful and very hard mentally sometimes.”

When Hannah and her husband Andrew went to see their obstetrician, they first found out they were having twins. A month later, at another appointment, the third baby was found. Two girls and a boy.

“I went from high risk to even more high risk,” Hannah says. “I was elated and honestly just so flabbergasted we were going to have three babies at once.”

Unfortunately, the girls developed a circulatory problem while still in the womb where the veins in the placenta were fused together and one baby was getting too much blood, while the other wasn’t getting enough. Laparoscopic surgery at Children’s Hospital of Philadelphia successfully fixed the problem.

On Dec. 27, 2022, Hannah had a scheduled appointment with her obstetrician that took a surprising turn. Ender’s heart rate was low and Hannah ended up having an emergency cesarean section at an area hospital because Ender’s umbilical cord was wrapped around his neck. The babies – nearly two months early – weighed a little over three pounds each and were healthy, but in the hospital’s neonatal intensive care unit.

Shortly after Hannah was discharged, she ended up back in the emergency room with what was determined to be preeclampsia, a potentially life-threatening blood pressure condition she didn’t experience in her prior pregnancies. While being admitted she missed a phone call from the doctor, whose voicemail said all three triplets tested positive for CF.

“My world was rocked. I felt so helpless and trapped laying in that bright, cold room,” Hannah recalls. “Of course, I didn't know all that it (CF) would entail, but I have learned a heck of a lot since. We’ve been in survival mode for over two years. All we can do is take each moment at a time.”

“I was already overwhelmed with having triplets and worried that they were premature. I didn’t even really process their diagnosis because I didn’t know what CF was,” Andrew says.  

Caring for the magnificent Monahan seven, who range in age from 10 down to the triplets who will be 3 on Dec. 27, is hard work to say the least. Caring for children with CF means constant monitoring and medication.

Hannah says life is chaotic and stressful, but notes she and Andrew couldn’t do it without the help of family, including grandparents. Both Hannah and Andrew work opposite shifts at the Marion Hose restaurant in Jim Thorpe. Nurses come to their home several times a week to help administer medication and treatments, Hannah says.

The Monahans say the only outward sign of the triplets’ CF is a cough. The babies must always get medication before they eat.

With a large family, and triplets with CF, Hannah says she tries to take mini breaks to recharge. She also tries to get the children outside as much as possible.

“I cope from knowing it’s the right thing to do,” Andrew says. “I was raised to be a good person so I’m going to be a good dad. Having four kids before the triplets got me ready to take on having triplets.”

Hannah says the triplets are doing well. “It’s amazing. I think they’re doing phenomenally,” she says.

The Monahans say they do everything they can to make life as normal as possible for all seven of their children. There’s a lot of splitting up, a lot of coordination, but they’re making it work with the help of others.

“After having two kids, you get used to the responsibilities of becoming a parent and you get into a routine of being a parent,” Andrew says. “It’s one day at a time. They are our kids and we have to take care of them.”

Hannah says her family has felt very supported. “The whole community has been great. It’s been amazing,” Hannah says.

“I’m so thankful for all I have learned, especially medically,” Hannah says. “I’m so thankful for all the amazing people we have met, especially our CF center team! We have felt so cared for and supported throughout this whole journey.”

Jennifer Logan, MD, heads up the CF center, and says the facility’s holistic care model includes all resources recommended by the CFF. Dr. Logan says the center has various specialists, including physical and respiratory therapists, nurses, a pharmacist, social worker, dietitian, outreach coordinator and behavioral health specialist.

Dr. Logan says a CF diagnosis is tragic news for parents. “Families are typically very upset. It takes a long time to come to terms with the diagnosis,” she says. “The first year is the hardest because the babies are the most fragile and the parents are learning. We support them and let them know they are not alone.”

Hannah and Andrew say the CF center team is a caring group who always has a smile on their face and is always accessible and helpful. “I have the personal cell numbers of a lot of people in the clinic. I can text them anytime,” Hannah says. The CF Center team adopted the Monahans for Christmas 2023, providing gifts for everyone.

“They’ve been more than helpful and I totally trust their judgment on how to handle this medical condition,” Andrew says. 

Dr. Logan says the most important thing for parents with children with CF is to reach out when they need help. In addition to a CF center like the one at Lehigh Valley Reilly Children’s Hospital, there are parent and support groups, as well as education.

“We want parents to know we’re here and that we want to help. It’s hard sometimes to let down barriers and let people in,” Dr. Logan says. “But that’s why we’re here. It’s the whole point of having a care team. We want parents to know they are not alone. CF is so complex, you’re not going to learn everything in the first year. We tell parents to trust in the care team. Taking that weight off your shoulders is the most important thing in the beginning.”

According to the CFF, a person with CF typically inherits two copies of the cystic fibrosis transmembrane conductance regulator (CFTR) gene that contain mutations – one copy from each parent – to have CF.

According to the CFF, each time two CF carriers have a child together, the chances are: 

• 25 percent (1 in 4) the child will have CF
• 50 percent (1 in 2) the child will be a carrier but will not have CF
• 25 percent (1 in 4) the child will not be a carrier of the gene and will not have CF

Cystic fibrosis affects more than just the lungs. It was first identified in 1938 by Dr. Dorothy Anderson as “cystic fibrosis of the pancreas,” Dr. Logan says. 

“The whole body is affected,” she says. “It affects the balance of salt in the body. The imbalance of salt causes mucus layers to be dehydrated. That causes various problems, including breathing, but also digestive problems because it affects your pancreas and reduces the ability to absorb fat.”

Dr. Logan says the pancreas is often the first organ affected. Before modern medicine and treatments, and before a lot was known about CF, she says many CF babies would die of malnutrition in infancy. The Monahan triplets initially had difficulty with bowel movements because of the effect of CF on their digestive system. One had emergency surgery.

Dr. Logan says about 70 percent of babies with CF are now diagnosed through newborn screening. Since 2010, every state in the country has included CF on their newborn screening.

Considered rare by disease characterization standards, CF is the most common inherited disease, Dr. Logan says.

She says one of the biggest misconceptions about CF is that it’s a Caucasian disease. Almost 25 percent of CF patients in the United States are non-white. About 1 in 25-30 Caucasians are “carriers” and harbor a mutation in the CF gene that could cause the disease. CF carriers do not have disease and would not know they are carriers without genetic testing.

Treatments for CF have advanced rapidly in recent years. Dr. Logan says modulator drugs are providing symptom relief and increasing life expectancy. The current average life expectancy is 61, but Dr. Logan says that will undoubtedly increase as research continues. Gene therapy is in clinical trials.

The median survival age has increased nearly 30 years in the past quarter century, according to the CFF.

The Monahan triplets are on a modulator drug, which modifies the function of the CFTR protein so that it can serve its primary function related to salt balance. The modulators basically correct the underlying problem in CF by fixing the CFTR protein.

Dr. Logan says modulators are essential in treating the underlying conditions created by CF. She says the drugs work for 80-90 percent of patients. About 10-20 percent aren’t eligible or can’t tolerate the medicine. “They [modulators] are phenomenal in the way they can improve the lives of people with CF,” she says.

Two of Dr. Logan’s patients have had their pancreas function restored and others have been able to be taken off a lung transplant list.

Dr. Logan says with the help of CF medication, the Monahan triplets will live long, full lives.

The future for those with CF is getting brighter, she says. “It’s going to look completely different. Everything is changing in the world of CF. I tell my parents there will be a cure for CF in their child’s lifetime.”

Hannah says it’s hard to see the triplets go through so much with medication, treatments and lab work.

“I want them to live long and happy lives. I don’t want them to feel different,” she says.

With such a large family, and with three children with CF, Hannah says she’s worried she won’t have enough of herself to split up among all her children. She and Andrew do everything they can to make life as normal as possible for all seven.

“There are really good days, too,” Hannah says. “It’s worth it. They are worth it.”

Andrew says he hopes the triplets have the best childhood he and Hannah can give them. “I hope they live to see a cure for their medical condition,” he says.