Valentina: Overcoming Pancreatitis & Diabetes, All with a Positive Attitude

Valentina (2nd from left), her mother Sonia, her father Juan Pablo, and her sister NicoleSeventeen-year-old Valentina is an extremely talented high school senior living in southern Florida. She has many interests and would like to attend a university in Florida to major in international studies. She is currently participating on the school cheerleading team, student government, and National Honor Society, and she volunteers for Relay for Life (American Cancer Society) and Best Buddies. However, since the fall of 2017, she has faced—and overcome—life-threatening medical issues related to chronic pancreatitis and its associated treatments, including a major surgery called total pancreatectomy-islet cell autotransplantation, or TP-IAT. “I was always positive,” Valentina states. “I never really focused on the bad things. I just kept pushing through. I think that’s one of the biggest things that helped me get better.” Her proud mother, Sonia, states simply: “It was just amazing how she overcame her disease!”

An Overhead Conversation Leads to a Diagnosis

Valentina started having pancreatitis symptoms in second grade, but she, her mother, father Juan Pablo, and younger sister Nicole, now 13 years old, did not know it was pancreatitis at the time. “I would just have a lot of stomach aches,” Valentina remembers, “I would feel really sick occasionally. It got to the point where we would have to go to the hospital and they would just tell us that I had gastritis or that I was constipated.” Gastritis is a condition in which the stomach lining is inflamed, or swollen, and is often managed by reducing dietary acid intake. Valentina was told to follow a low-acid diet, which helped her. She remembers having flare-ups on rare occasion, but nothing too bad.

That all changed in October 2017 when she was in 10th grade and had a major flare-up. “We thought it was just going to pass away as it always does,” Valentina recalls, “but the flare-up lasted for a week, and it got to the point where I was vomiting blood. That’s when we were like ‘ok, we need to go to the hospital.’” At the hospital, the staff asked Valentina for her family history to help pinpoint the cause of her illness. By a stroke of luck, she had recently overheard her parents saying that her father may have pancreatitis based on a chance finding from a medical examination, although Valentina did not realize at the time that he had not been diagnosed, and he did not have symptoms. Thus, when asked about her family history, “I told them that my dad had pancreatitis,” she states. “Because of that reason … they tested me for it and it came out positive.” The doctors told the family that they typically do not test children for pancreatitis because it is so rare, so it was fortunate that this conversation Valentina overheard—and decided to mention to the doctors—led to an accurate diagnosis of her illness so quickly after her severe symptoms started.

About Pancreatitis

The pancreas is an organ located behind the stomach that has many important functions. Tiny clusters of cells in the pancreas, called islets, produce hormones such as insulin that regulate blood sugar (glucose) levels. The pancreas also produces fluid that is released through ducts into the intestine and contains enzymes that are necessary for digestion of food. Usually, these powerful digestive enzymes are inactive until they exit the pancreas and enter the small intestine. In cases of pancreatitis, however, digestive enzymes are activated prematurely while still inside the pancreas, resulting in damage and inflammation, and symptoms of abdominal pain, nausea, and vomiting. As Valentina explains, “My pancreas is actually digesting itself." Chronic pancreatitis is rare in children and is often associated with a genetic mutation. People with genetic forms of pancreatitis have a higher risk of developing pancreatic cancer later in life.

After she was diagnosed, Valentina was admitted to the hospital for 5 days and then went home, but she was not there long. “She got very, very sick again, so we had to take her back to the hospital,” says her mom. At that time, the gastrointestinal doctor was on vacation, so there was no specialist available to help them. Sonia, speaking through tears, remembers how terrifying the experience was. “I couldn’t do anything for her. I was so worried because she couldn’t eat,” she says. “It was awful…. She was dying in the hospital. She was so, so sick, with a lot of pain.”

Not knowing what else to do, Sonia spent her nights searching the internet for a doctor who had expertise in pancreatitis and could help her daughter. She found a pancreatitis specialist at University of Florida (UF) Health, but the doctor was about 6 hours away and only treated adult patients. However, Sonia was adamant that Valentina desperately needed this doctor’s help, and her persistence paid off: the doctor made an exception and agreed to see Valentina very quickly. Reflecting on that experience, Sonia has an important message for other parents: “Be your own advocate.”

Sonia finding that doctor at UF Health changed the entire course of Valentina’s illness. It was there, in late November, about a month after her diagnosis, that Valentina underwent her first surgery to treat her pancreatitis: “Endoscopic retrograde cholangiopancreatography, or ERCP,” she explains. “What they did was remove what was blocking the pancreatic ducts, and placed stents to keep the ducts open and allow the enzymes to flow to the small intestine." After that surgery, she was feeling better, and was even able to eat a little bit.

It was also there that Valentina found out that she had a genetic form of pancreatitis. She explained that the genetic mutation is in a gene called PRSS1. Because of the increased cancer risk associated with her form of pancreatitis, their doctor at UF Health recommended that Valentina have her pancreas removed via TP-IAT, and their doctor also suggested that Valentina be treated by TP-IAT experts at the Medical University of South Carolina (MUSC).

Undergoing TP-IAT Surgery

In the first stage of the TP-IAT procedure, the pancreas is surgically removed and the gastrointestinal tract is reconstructed. Removing the pancreas would result in lifelong diabetes because the organ contains the only source of insulin-producing beta cells in the body; without insulin, the body cannot regulate blood sugar levels, so the patient would become insulin dependent. However, in the second stage of TP-IAT—islet autotransplantation—the islets that contain the beta cells are collected from the patient’s pancreas and infused back into the portal vein of the liver. The islets then become lodged in blood vessels of the liver where they settle, grow, and begin producing insulin. Thus, the TP-IAT surgery serves the dual purpose of removing the source of severe pain and also eliminating the increased cancer risk while preserving some insulin production, reducing the risk of developing diabetes.

Additionally, because the islets are from the patient’s own pancreas, the body does not recognize them as “foreign” and mount an immune response to them. Thus, people undergoing TP-IAT do not need to take immune-suppressing medicines, which can have serious side effects. This is in contrast to islet transplantation alone, which is an experimental procedure that uses islets from deceased organ donors to treat some people with type 1 diabetes. Notably, people undergoing TP-IAT do require lifelong supplements to replace their pancreatic digestive enzymes.

When hearing about the possible TP-IAT surgery, Valentina didn’t have any reservations: “I immediately felt that this was something that I had to do…. I surprisingly wasn’t afraid.” Her parents, on the other hand, were not thrilled at the thought of their daughter undergoing major surgery to remove a vital organ. As Sonia explains, “Valentina was doing better with the stent. She was able to eat more, so we were kind of more relaxed.” Valentina remembers that her parents were also worried about the prospect that she could have diabetes for the rest of her life, so Sonia and her husband thought about waiting a year before considering the surgery. “But then Valentina started getting sick again, even with the stent, and we had to take her back to the hospital,” Sonia remembers. Because Valentina was barely able to eat, she lost 50 pounds during the course of her illness—weighing only 82 pounds at her lowest. She also had to stay in bed nearly all the time because she was so sick and weak. As a result of Valentina’s worsening health, the family decided to move forward with the TP-IAT evaluation to determine by the MUSC’s doctors if she was a candidate for this type of surgery.

For the evaluation, Valentina underwent 3 days of intensive testing and meetings with various doctors at MUSC. For example, “we had a meeting with an endocrinologist, a psychologist, and a nutritionist and they explained to us how a diabetes life would work,” recalls Valentina. Even though the hope was that the islet autotransplantation component of the surgery would reduce her risk of developing diabetes, there was still a possibility that her own transplanted islets would not function properly, or not make enough insulin to regulate her blood sugar levels. Thus, it was possible that, after her surgery, she would have to manage diabetes by counting carbohydrates when she ate, monitoring her blood sugar levels, and administering insulin.

After the evaluation, the family returned to their home in Florida and soon found out that Valentina was a candidate for TP-IAT. The surgery was scheduled for January 2018—only 3 months after she was diagnosed with pancreatitis. Now the family was faced with the fact that they had to separate, as Valentina’s surgery was far from their home and her sister and father couldn’t come with her due to school and work. Her mom was able to obtain permission from work to take time off for Valentina’s surgery and for her recovery in South Carolina. Her father took care of her younger sister and provided all the financial support. The family rented a house near MUSC so that they could be near Valentina for the surgery and during the 1 month of recovery period.

When hearing about the possible total pancreatectomy-islet cell autotransplantation (TP-IAT) surgery for pancreatitis, Valentina didn’t have any reservations: “I immediately felt that this was something that I had to do…. I surprisingly wasn’t afraid.”

“The day of my surgery, I remember waking up very early. Honestly, it was very peaceful…. In the car ride there [to the hospital], I prayed the whole ride. It was kind of me saying I had an understanding that if it was time to go, that I was OK with it,” Valentina recalls, even though she was only 16 years old at the time. Her surgery lasted a total of 12 hours. During the first 6 hours, the surgeons removed her pancreas, along with nearby organs such as her spleen and gallbladder. After that, it took another 4 hours to isolate the pancreatic islets and 2 hours to transplant them into her liver. Throughout the procedure, Valentina was in excellent medical hands—both she and her mother raved about the MUSC doctors and nurses, who they said were outstanding, explained everything, and made Valentina feel comfortable.

Learning to Manage Diabetes

After the surgery, Valentina spent a few days in the intensive care unit before being transferred to another floor of the hospital. It was a difficult time. She was hooked up to numerous machines and had to take medicine to manage her severe post-operative pain. Even talking was difficult. Also, because it could take several months or longer to know whether Valentina’s own transplanted islets were going to function properly, she had to be treated for diabetes. The nurses checked her blood sugar levels around the clock and administered insulin. However, Valentina’s positive attitude shined through even under these most challenging circumstances: “She walked the second day after her surgery,” beams her proud mother. “She did so great, walking with pain every single day, three or four times per day to recover fast to leave the hospital.”

“I am pain-free ever since recovering from my surgery,” Valentina says.

After Valentina was released from the hospital, the family stayed in South Carolina for a month to be close to the MUSC doctors. During that time, Valentina remembers that, “It was hard getting used to everything, especially the diabetes part. In the beginning, we were so lost … with the counting carbs [carbohydrates] and understanding that a certain amount of carbs meant a certain amount of insulin.” She said things only got harder over time. “At first it wasn’t too hard,” she states, “because after such a big surgery I wasn’t eating very much. But … when my appetite started going up, that’s when we had the most difficulties.”

Achieving Insulin Independence

Coming home after a month in South Carolina, Valentina was still recovering from her surgery and managing her diabetes. Sonia had to return to work after taking 4 months off to care for her daughter, so the family hired a nurse to help Valentina during her long and challenging recovery. Valentina returned to school that summer, after missing nearly an entire school year. That transition was also difficult because she was not fully recovered from her surgery, got tired easily, and had to learn how to manage her diabetes while at school.

However, as time progressed, she made a welcome discovery: “We started noticing that my blood sugars were getting lower and lower every day.” During one of her regular endocrinologist check-ups, the doctor told Valentina not to take insulin if her blood sugars were within a healthy range. “It got to the point that I didn’t need any insulin,” she states happily. As of November 2018, Valentina has been insulin independent. “Now, I only check my blood sugar when I’m feeling sick,” she explains, which is roughly once every couple of weeks. If her blood sugar levels are elevated, she lays down or drinks water and that helps her levels come down without needing insulin. “We don’t have insulin in the house anymore,” says Sonia.

As Valentina notes, “My diabetes was obviously very different from most diabetes,” since it resulted from the removal of her pancreas. Therefore, TP-IAT is not an option for treating other forms of diabetes, like type 1 or type 2 diabetes, because the underlying cause of the disease is different.

Living a Pain-free and Diabetes-free Life

Her successful surgery and recovery have enabled Valentina to look toward the future, with her plan to pursue international studies or law when she begins college next year. At the time she was interviewed for this profile, it had been 1½ years since she underwent her TP-IAT surgery. “I am pain-free ever since recovering from my surgery,” she says. She has also recently been able to be more active, including cheerleading for her school, playing tennis and volleyball, and doing a lot of volunteer work.

Valentina says that before her total pancreatectomy-islet cell autotransplantation (TP-IAT) surgery, which could cause diabetes, the doctors “told us there was a possibility that I wouldn’t be dependent on insulin, but I don’t think I really had high hopes for that…. For me to get past my expectations of where I was going to end up after the surgery is just mind-blowing.”

One of the complications after her surgery is that her body cannot adequately absorb iron, so she has to get intravenous iron infusions. She also has to spend a lot of time keeping track of her diet to figure out what foods make her feel good and which ones make her feel unwell, and she takes enzyme replacement supplements at each meal, something she will continue to do for the rest of her life. And, even though she’s feeling much better physically, the entire experience has taken a mental toll on her. “I don’t really feel normal,” she explains. “Thinking about it mentally, I feel like a year of my life has been stolen from me, where I’ve been forced to become more mature and aware of everything.” However, Valentina’s positive attitude is still apparent: “But I think I’m appreciative of that, of what I know and what I’ve yet to learn.”

Additionally, Valentina says that before her TP-IAT surgery, the doctors “told us there was a possibility that I wouldn’t be dependent on insulin, but I don’t think I really had high hopes for that…. For me to get past my expectations of where I was going to end up after the surgery is just mind-blowing.” Valentina is grateful for the islet autotransplantation portion of her TP-IAT surgery—the extra 6 hours it took to isolate and transplant her own islets has given her freedom from diabetes.

Hope Through Research

NIDDK has supported much of the clinical research on use of TP-IAT for treating chronic pancreatitis in adults and children, and currently supports research to improve outcomes after TP-IAT surgery, including at MUSC and other sites. For example, MUSC researchers are testing a strategy to enhance survival and function of the transplanted islets in adults with chronic pancreatitis undergoing TP-IAT to further reduce the risk that people will develop diabetes after surgery.

NIDDK has also supported other research related to pancreatitis conducted by individual investigator-led teams, as well as larger, multi-center studies, such as the North American Pancreatic Study Group and, more recently, the Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer, that have led to important discoveries of genetic risk factors and other advances in understanding and managing pancreatitis in children and adults.

“If we didn’t have this research, Valentina wouldn’t be here today,” her mother, Sonia, exclaims.

Valentina and her mother value and appreciate the role of NIDDK-supported research in Valentina’s improved health. “If we didn’t have this research, Valentina wouldn’t be here today,” Sonia exclaims. “I’m so thankful to the Lord Jesus for the technology, the doctors, the researchers.” Valentina adds about the research progress: “I think it’s incredible.” Also incredible is the fact that Valentina, at such a young age, has faced formidable health issues with tremendous maturity and positivity—there is no question that her future is bright.