Hannah Moraski has always been independent, courageous and someone who exemplifies the word tattooed across her forearm: Fighter.
She has vast appreciation for her newfound freedom (even in a pandemic), a new prescription drug, Givlaari, that has changed her life and Justin Smith, a handsome young man whom she married on Aug. 21.
On a recent camping trip with Justin, one of the first she has been able to take in years, Moraski, 26, spent several minutes taking in the glory that is Colorado’s Collegiate Peaks near Buena Vista.
“Just looking out over the mountains and knowing that you are alive and being grateful,’’ she said. “… I am so happy to have my life back.’’
Five years ago, Moraski became suddenly ill. Bedridden, she couldn’t eat, shower or walk. Over a few months, she made frequent trips to the Emergency Department and took painkillers for debilitating pain in her back, legs and abdomen. She was miserable.
Eventually, Moraski was diagnosed with a rare blood disease – Acute Intermittent Porphyria – a genetic disease that affects only five in every 100,000 people.
“You’d be hard-pressed to find any of us in the community who have severe acute intermittent porphyria patients,” said Dr. Uchenna Njiaju, a hematology and oncology specialist who cares for Moraski at UCHealth Memorial Hospital Central. “So, there’s nobody, probably, except for expert geneticists, a couple around the country, who can lay claim to having treated a hundred over two years or that sort of thing. We all have a sprinkling, because it’s so rare.”
In most people, symptoms never manifest and people live their lives not knowing they have acute intermittent porphyria. But, Njiaju says, Hannah had a rather aggressive presentation and course with AIP. For Hannah, the attacks sent her to the hospital for week-long stays. During one stay, she suffered cardiac arrest.
For the past five years, Moraski has been shackled to twice-a-week visits to UCHealth Memorial Hospital, where she received infusions of panhematin, a drug that helps replace what her blood can’t produce. Each session lasted 2 to 3 hours, and she passed the time reading, knitting and talking to her nurse.
Moraski could never travel far from the hospital. She got used to thinking she’d never be able to take a vacation, hold a full-time job or even go camping for more than a few days at a time. The need to be close to a hospital to receive the hours-long infusions was her first priority.
In January, a drug called Givlaari was released. Now, instead of spending at least 6 hours a week sitting in a chair to receive infusions, Moraski gets a shot once a month. It takes about 30 minutes.
“It’s just a little shot and I don’t have to sit here,’’ she said. “With everything going on in the world, it really couldn’t have happened at a better time.
“It didn’t occur to me until I got this drug what I was really missing in life and what I now have back,’’ she said. “It’s really hard to take anything for granted and little things like camping, just looking out over the mountains and knowing that you are alive and being grateful that this drug exists.’’
Moraski is working again. Previously, she could work only about one day a month. She dreams of returning to college and pursuing an education in psychology/mental health.
“I really want to help people in that way, and I think my own personal struggle has made me realize that I want to help people that much more. It’s more than just helping, it’s connecting and understanding and being a voice for people who do not feel as though they can be a voice for themselves,’’ she said.
For her doctor, watching Hannah’s progress has been a joy.
“Hannah has really come a long way. Her strength and resilience through challenges that would demoralize most other people, are truly inspiring. Hannah always has a lovely smile on her face. It’s been such an honor to partner with her on this journey. I’m just so happy to see her healthy, having less frequent infusion appointments and more time away from the hospital to live her life,” Njiaju said.
In recent weeks, the biggest thing in her life has been her wedding on Aug. 21 to Justin, who has stuck by her from the day she was diagnosed.
“Justin and I had always had to plan things around my infusions, everything was controlled around infusions. That’s the biggest thing, I’m so grateful for his support. It’s changed his life too,’’ she said.
“He has always stuck by my side and now he can enjoy things in his life too. I am so lucky to have him, and I can give back to him and I feel like I am actually going to be the wife he deserves. He has done so much for me over the years and now I can give back, I can finally do it thanks to the drug.’’
The two traveled to Vermont, where Moraski was born and raised, for a small wedding among family and a few friends. It’s the first time that Moraski has been back to Vermont since she moved to Colorado five years ago. Because of the coronavirus pandemic, the couple will opt for a smaller crowd and delay their honey moon until the environment for travel is safer.
Acute intermittent porphyria
Dr. Uchenna Njiaju says the main problem in AIP is that there is deficiency of an enzyme called porphobilinogen deaminase. This enzyme helps to break down molecules called porphyrins that form in the process of manufacturing heme, the main pigment that enable red cells to carry oxygen.
When porphyrins accumulate excessively in AIP, they cause attacks and problems in the nervous tissues. The main way the genetic code instructs the body to make proteins is through molecules we called mRNA.
Givlaari is a small molecule, that interferes with the genetic message (mRNA) leading to manufacture of nerve system poisons in AIP.
“By using Givlaari, we have fewer of these toxic molecules floating around and so we can prevent AIP attacks,” Njiaju says.
“Possibly next year, we want to plan a big honeymoon. Possibly next year we will be going and doing a cruise, which is something that I’ve never done. We never thought it would be possible to travel, to go on an actual cruise.’’
“This new drug opens up a whole new world. It’s literally living life. It’s like I had these couple of years of struggling and it has paid off,’’ she said.
At first, Moraski admits, she was nervous to take Givlaari because she was not sure her body would accept the drug. Some people have side effects – high liver enzymes or certain rashes – around month 3 or 4.
“I want everyone else to be able to get the medical care they need and experience life, whether it is with AIP or any other disease. Often, disease goes unnoticed and people just suffer in silence and I don’t want those people to suffer in silence,’’ Moraski said.
Moraski has been influential in social media circles among people who have AIP. When the newly diagnosed are looking for a physician, she often recommends Dr. Njiaju.
“Dr. Njiaju has really made a name for herself. She’s become an expert on AIP. She’ll call me personally and she takes phone calls from my mom. The two of them are in constant communication and I don’t think I would be able to go through this without Dr. Njiaju.’’
Moraski has always appreciated Njiaju’s direct but compassionate approach.
“She’s always kind of told me straight up. She’s been honest and straight-forward but also very loving. And that’s what you need in a doctor. She knows what she is doing and she is incredibly intelligent. She’s an amazing person.’’
After regular visits to UCHealth Memorial Hospital two times a week for five years, Moraski is happy to be able to take time for herself, work and go camping with Justin.
“I could not have asked for better nurses. The people here, they have seen my journey and they’ve been through the progress with me,’’ Moraski said. “They are so happy for me. I love them to death, but I am so happy that I don’t have to see them.’’
Moraski was radiant as she walked down the aisle. She and Justin exchanged rings and recited their vows, After their wedding in Vermont, they began their journey together, free to go anywhere their hearts desire.