Jackie Whitlock of Modesto and her 9-year-old daughter both have suffered from the chronic pancreatitis that runs in the family.
Jackie had surgery on her diseased pancreas as a child, but her daughter's operation two years ago at University of California San Francisco Medical Center was different.
Mackenzie had her entire pancreas removed and today she's doing just fine without the organ.
"It is amazing that she was so sick and now she is doing so well," Jackie Whitlock said. "She could not do anything, could not go to birthday parties. We held her back in second grade because she missed so much school being sick."
In the United States, close to 300,000 people a year will be hospitalized with an acute attack of pancreatitis, most often caused by gallstones. Fewer people suffer from the debilitating symptoms of chronic pancreatitis, which often results from the heavy scarring of alcohol abuse in adults. In children, the rare disease is hereditary and is almost always misdiagnosed the first time.
Mackenzie became a candidate for a pancreatic islet procedure that is done at UCSF and a few other centers in the country. Fewer than 20 younger patients have braved the procedure at UCSF in the last five years.
For the Whitlocks, the decision required careful consideration because some patients who have their pancreas removed will need to take insulin the rest of their lives.
"People with pancreatitis have tremendous pain and the risk down the line is they can develop pancreatic cancer," said Dr. Andrew Posselt, director of islet transplantation at UCSF. "The most recent approach is taking the whole pancreas out."
The pancreas serves a couple of purposes in the human body. About 95 percent of the organ is dedicated to making enzymes for good digestion of food, Posselt said.
The remaining 5 percent consists of cell clusters, called the islets of Langerhans, that are a natural source of insulin controlling sugar levels in the blood.
A person without insulin has terrible diabetes, so the team at UCSF removes insulin-producing islets from the diseased pancreas and transplants them to the liver. The Bay Area center has done the auto-transplantation procedures on a regular basis since 2012.
"These islets just happen to be in the pancreas," Posselt said, noting they're also capable of living in the liver and other places in the abdomen. "We take the islets from the pancreas after it's removed, isolate them and give them back to the patient."
Symptoms of pancreatitis include upper abdominal pain, nausea, vomiting and weight loss.
Jackie said her daughter's attacks caused by an inflamed pancreas erupted frequently when she was 6 years old. Doctors assumed it was gastritis or indigestion. A severe attack sent her to Kaiser Modesto Medical Center, where tests showed she had internal bleeding.
Transported to a hospital in Santa Clara, Mackenzie received a blood transfusion, underwent tests and was fed through a tube. An endoscopic procedure was done to clean out waxy material that blocked the ducts of her pancreas.
After her release from the hospital, Jackie said, the same procedure had to be done every two or three months. The student at Coleman Brown Elementary School became so sick she was back in the hospital for more than 20 days.
"When she had a pancreatic attack, she was throwing up bile and curled up in a fetal position crying in pain," Jackie said.
During appointments with her doctor, Jackie urged the physician to add another test to the normal blood panel to check for signs of pancreatitis.
Jackie had the genetic disorder as a child, her father had surgery for pancreatitis in his 40s and a grandmother had pancreatic cancer. Mackenzie's blood test came back positive, leading to an imaging scan that revealed dilated ducts in her pancreas.
Mackenzie was put on a special diet, with no greasy fried food, and the family was soon discussing options with specialists at UCSF. Beside the chronic symptoms, Mackenzie was on addictive medication for the pain, her mother said.
The islet transplantation is considered when it's evident that other treatment or types of surgery won't relieve symptoms or restore the patient's quality of life. But the total pancreatectomy is a gamble.
Removing the diseased pancreas eliminates the persistent abdominal pain, allowing a large majority of patients to come off painkillers. Medical journals report that 60 percent of the patients are not dependent on insulin for the long-term, though the percentage is higher at UCSF, particularly among children, Posselt said.
In some cases, however, the transplanted islets don't function well enough and the patient requires an insulin pump for life.
"There are a lot of ways to treat the disease," said Posselt, who works with adult patients and children. "What you don't want to do is wait too long until the islets stop working."
Harvesting islets from a heavily scarred pancreas is difficult and time increases the risk of deadly pancreatic cancer.
"Mackenzie was so ready to be out of pain," Jackie said. "We prepared by talking with other families. One of the parents in an online group had her son send Mackenzie a picture of his scar."
Mackenzie was approaching her 8th birthday as she faced the transplant surgery in April 2016. The day before the operation at UCSF, a hospital team that works with children came into Mackenzie's room and drew a picture of the human body on the wall. They dipped a nerf dart in paint and had Mackenzie shoot the pancreas with the dart gun.
After the patient is put to sleep in the operating room, Posselt said, the challenge is removing the pancreas, which is surrounded by organs, and maintaining the blood supply until right before it's taken out. The islets start dying if the blood supply is cut off too early.
After it's removed, the pancreas is dissolved and healthy islets are taken out and put in an IV bag. The solution is run into a portal vein and the islets migrate to the liver.
Good outcomes are determined by how well the surgeon isolates the islets after removal of the pancreas, Posselt said.
Following 18 days in the hospital, Mackenzie and family stayed in the Ronald McDonald House near UCSF and Mackenzie soon was playing on a trip to the beach.
She was on insulin for months after returning home to Modesto and had tests every three months while the islets were taking root in her liver. The cell clusters, like a nest of baby birds waiting to hatch, eventually wake up and start producing insulin, the doctors say.
At one follow-up visit in fall 2016, the Whitlocks were told Mackenzie may not come off the insulin she was taking. Later that December, she was taken off insulin and has never been on it since, Jackie said.
The 9-year-old takes a digestive enzyme before she eats and is free of pain medication, Jackie said. She likes tumbling, riding a scooter, rollerskating and eating the food she likes.
“I am happy I can be with my friends and enjoy school and go places,” Mackenzie said.
Posselt said that follow-up studies show good long-term results for functioning islets in auto-transplant patients. The cell clusters are transplanted from one place in the patient's body to another, so there's no need for drugs to prevent tissue rejection.
Mackenzie has told her story through Facetime to other kids awaiting the surgery. About two-thirds of patients at the UCSF program are adults with chronic pancreatitis and a third are children, though the numbers are not large. Fourteen patients between 4 and 22 years old have undergone the auto-transplant procedure at UCSF in the last five years.
Jackie, a medical assistant for Stanislaus County health services, has two other daughters and a 20-year-old son in the Army. Some time ago, a test showed her 24-year-old daughter has the gene mutation of pancreatitis but she has no symptoms.