Pancreas Care

Total Pancreatectomy with Islet Autotransplantation (TPIAT)

TPIAT is discussed when treatment options such as medical management and endoscopic treatments do not relieve the symptoms of pancreatitis. It is an option only when other treatments have failed to relieve the pain and suffering related to the disease. The goal of the TPIAT is to provide pain relief and improve quality of life. TPIAT involves two steps in one operation:

1. Total Pancreatectomy (“TP”) - This involves removing the entire pancreas and reconstructing the gastrointestinal tract. To prevent postsurgical complications, the appendix and gallbladder
   are removed. The spleen is removed because of shared blood vessels with the pancreas.
2. Islet AutoTransplantation (“IAT”) - After the pancreas has been removed, an “islet isolation team” takes the pancreas to a laboratory where the islets are isolated and recovered. The islets have beta cells inside them. The beta cells are responsible for producing insulin. The team returns the islets to the operating room. The surgeon transplants them into your child’s liver. The hope is that these cells will continue to produce insulin as they did in the pancreas. We want the islet cells to make insulin and provide good blood glucose control. We will not know for several months if this will happen.

Patients with severe, debilitating pain caused by chronic or acute recurrent pancreatitis may benefit from TPIAT. Your child’s doctor may recommend it if other treatments have failed.
Patients who undergo TPIAT typically have been experiencing this type of abdominal pain for more than six months. They are unable to go to school or be active, and have been hospitalized one or more times for the pain.

Patients with severe, debilitating pain caused by chronic or acute recurrent pancreatitis may benefit from TPIAT. Your child’s doctor may recommend it if other treatments have failed.

Patients who undergo TPIAT typically have been experiencing this type of abdominal pain for more than six months. They are unable to go to school or be active, and have been hospitalized one or more times for the pain.

Our care team will provide a full evaluation to decide whether TPIAT is the best way to treat your child’s pancreatic problems. We first want to give an overview about what the pancreas is supposed to do. We want to explain how TPIAT may help people who are suffering from acute recurrent pancreatitis or chronic pancreatitis.

The evaluation visit can be time consuming. It usually takes multiple days to complete. It can include appointments with:

• Gastroenterology (GI)
• Surgery
• Endocrinology
• Pain Management
• Behavioral Medicine and Clinical Psychology (BMCP)
• Physical Therapy
• Social Work
• Infectious Disease

During the evaluation, our care team will collect all the information we need about your child’s condition. It is a chance for our care team to get to know you and your family, and for you, to learn more about us. Our goals for this visit are:

• Get to know you and your child
• Evaluate your child’s cause for pancreatic disease and assess the severity of the disease
• Test the function of the pancreas
• Understand what you and your child can expect from our team
• Share our thoughts about your child’s pancreatic disease stage
• Give you and your child a chance to ask questions and share concerns
• Decide if further testing is needed
• Determine whether TPIAT is a good option for your child

We will also want to make sure that you, your child and family know:

• Details of your child’s illness
• What TPIAT involves
• Risks, benefits, and both expected and unexpected outcomes of TPIAT

Your child will most likely need testing as part of the evaluation visit. These tests might include:

• Blood tests
• Genetic testing
• Mixed meal test (MMT)
• Imaging tests, such as magnetic resonance imaging (MRI), computed tomography (CT) and ultrasound
• Endoscopic retrograde cholangiopancreatography (ERCP)
• Endoscopic ultrasound (EUS)

You will meet with the surgeon to talk about what the operation involves, what the surgeon expects the outcome to be, and what your child’s recovery will look like. You and your child will also meet with an infectious disease doctor. This is because the TPIAT operation involves removing your child’s spleen. It is important that your child has certain immunizations before the TPIAT to reduce the risk of getting a serious infection called sepsis. The infectious disease provider will discuss this with you.

After the evaluation is complete and all of your child’s test results are available, the PCC team will meet to decide what course of treatment is best for your child. The team will answer these questions before making a decision:

1. What caused your child’s pancreatic disease?
2. How severe is the disease?
3. Will TPIAT improve your child’s quality of life?
4. Are there any medical issues that may prevent a successful surgery?
5. Are there any other treatment options besides a TPIAT that could benefit your child?
6. Does your family have the support needed to get through the TPIAT process, including the hospital stay?
7. Does your family have the resources needed to meet your child’s needs during this challenging time?

After the team finalizes the recommended treatment plan for your child, the gastroenterology nurse will contact you. The recommendation will be one of these options:

• Approved: Your child is a candidate for TPIAT. We can proceed with the scheduling process.
• Deferred: Your child is not a candidate for TPIAT at this time. The team will work with you and your child’s primary gastroenterologist (GI doctor) to create a treatment plan that does not include TPIAT for now.
• Decision Pending: Your child needs further testing or evaluation before the team can make the decision.
• Not a Candidate: The child is not a candidate for TPIAT.

The team will talk with you about other treatment options for your child. You will be able to talk with the doctor or nurse about the treatment recommendation and next steps.

TPIAT can last the majority of the day. Our surgery nurse practitioners play an important role during this long day. They will provide your family with updates from the operating room and spend time with you and your child in the pediatric intensive care unit after surgery.

Your child will stay in the hospital for about 2 to 3 weeks. This includes stays in the pediatric intensive care unit (PICU) and diabetes unit. Along each step of the hospital stay, all members of the PCC
team, will see your child to discuss progress and plans with you and your family. These family-centered rounds typically take place in the morning.

After their operation your child will recover in the PICU. All TPIAT patients stay in the PICU for about one week. During this time, the team works to maintain good blood glucose control. They will monitor your child for any concerns.

The PICU staff will use special equipment to care for your child. This equipment may include:

• IVs that give medicine or fluids
• A tube in the nose to suction out fluid from the stomach
• A breathing tube in the mouth
• A urine catheter
• Tubes in the abdomen to drain extra fluids
• A GJ feeding tube in the abdomen

Your child also will have a dressing covering the incision from the operation. For the first week after the operation, your child’s body may look swollen. This is from the extra fluids needed during the
operation. The puffiness goes away within five to seven days. Your child may need a medicine called a diuretic to help get rid of the puffiness.

In the Diabetes Unit

After your child begins to require less testing and medication, you will move to the diabetes unit while your child continues to heal. This is where the endocrinologist and the diabetes education team will focus on diabetes education. Be ready to dedicate about 20 hours to education during your inpatient stay. You will be learning about diabetes, an insulin pump, a continuous glucose monitor, the feeding tube, and new medications. This might feel overwhelming, but with hard work and the team’s support, you will learn what you need to know.

The weeks and months following surgery are very important for your child’s long-term success. We ask that your child remain in Greater Cincinnati for about four to six weeks after leaving the hospital. Our guest services team is available to assist you in planning for the temporary relocation needed for the procedure.

During those first four to six weeks, you and your child will return to the Pancreas Care Center for frequent follow-up appointments with our multidisciplinary care team. Our team will:

• Monitor your child’s blood glucose control and islet function.
• Adjust medications as necessary.
• Make sure your child is getting the nutrition he or she needs before and after the feeding tube is removed.
• Wean your child from pain medications.

 After this initial follow-up period, your child will return to the clinic less frequently for check ups.

Most patients report significant pain relief and an improvement in their quality of life following TPIAT. In the months following surgery, they typically can return to school, social activities and even sports. Many patients are able to dramatically reduce or eliminate their need for narcotic pain relief.

Knowing whether your child will remain on insulin therapy takes longer—up to a few months to a year or more. At Cincinnati Children’s our data for patients one year after the operation shows:

• 30 percent of children produce enough insulin on their own (no longer need insulin)
• 50 percent of children need to take a low amount of insulin (your child’s body is producing some or most of the insulin on its own)
• 20 percent of children need to take a moderate to high amount of insulin (your child’s body is producing little or no insulin on its own)

Younger children are more likely to stop needing insulin completely. Approximately two-thirds of children under age 8 do not need to take insulin at one year after their operation. During your TPIAT
evaluation, your endocrinologist and surgeon will discuss your child’s possible insulin needs. Even if your child stops needing insulin, you will still stay in contact with your endocrinology team.

From now on, your child will have to take medication to replace certain digestive enzymes that are normally produced in the pancreas (pancreatic enzyme replacement therapy). However, your child will not need to take anti-rejection drugs after TPIAT, since the islets are transplanted from his or her own body and not from a donor.

Our nationally-recognized specialists provide online second opinions for families. They will review your case and answer specific questions about a diagnosis or treatment options. It’s easy, convenient and secure. Plus, you don’t have to leave home. Our case manager will collect your medical records and guide you through the process.