We have a vital and active Hepatobiliary Surgery Program. Our surgeons perform a range of hepatobiliary operations, including various shunt procedures for portal hypertension, resections for pediatric liver tumors, Kasai procedure for Biliary Atresia, and ileal exclusion and biliary diversion for Progressive Familial Intrahepatic Cholestasis Syndrome. These procedures can not only delay the need for liver transplantation and afford our patients a better quality of life, but also allow some children to survive without liver transplantation.

Children with liver disease are dealing with a very serious medical condition, and may develop further complications. Their families need to recognize the warning signs and what to do if complications occur. Signs and symptoms may include:

• Jaundice (yellowing of the skin and eyes)
• Itching
• Pale or clay colored stools
• Dark or tea colored urine
• Ascites (an abnormal accumulation of fluid in the abdomen)
• Easy bruising
• Failure to gain weight
• Vomiting of blood
• Forgetfulness, confusion

If you are experiencing any of these symptoms, please speak with your primary care physician before contacting us.

You or your child's primary care physician must contact our office to begin the referral process. Our team will then work on getting the necessary insurance clearance so you can start your child's evaluation here at Mount Sinai.

Please bring a copy of your medical history with you or ask your physician to fax it to us at (212) 241-2064 before your visit.

• Referrals to Pediatric Liver Disease, Pediatric Liver Transplantation, or Pediatric Hepatobiliary
  Phone: (212) 659-8060
  Fax: (212) 241-2064

Location
Our outpatient office is located on the 12^th floor of 5 East 98^th Street (between Madison Avenue and Fifth Avenue).

The Kravis Children's Hospital at Mount Sinai is located on 1185 Fifth Avenue. The Pediatric Intensive Care Unit is located on the third floor, and the General Pediatric Unit is located on the fifth floor.

Evaluation for liver transplantation is an ongoing process, which continues even after your child has been accepted onto the waiting list. The main goals of the evaluation process are:

• To find out if liver transplant is the best treatment of choice – on occasion transplantation may not be necessary as another type of surgery may be more appropriate due to your child’s disease process
• To find out if your child is a suitable candidate for liver transplantation
• To evaluate the timing of liver transplantation
• To educate you about the following aspects of liver transplantation:
  - Waiting for liver transplantation
  - Organ allocation
  - Type of liver transplants performed
  - Outcomes
  - Potential post operative complications
  - Commonly used medications after liver transplantation

Your child's evaluation will include an initial consultation with:

• Pediatric Hepatologist
• Transplant Surgeon
• Transplant Coordinator
• Infection Control
• Social Worker

Listed below are some of the "routine" tests that everyone must undergo in preparation for liver transplantation.

• Laboratory blood tests: It is important that we find out your child's blood type, blood counts, blood chemistries and viral studies (this includes checking for the hepatitis viruses, Cytomegalovirus, Epstein Barr virus and HIV virus)
• Abdominal Ultrasound and Doppler: This is a non-invasive imaging study that uses sound waves to create a picture of your liver and the surrounding organs. It also assesses the flow of blood into and out of the liver
• Chest X-Ray: These are used to ensure that there are no lung infections present
• Echocardiogram: This is a non-invasive imaging study of the heart to create a picture of the heart. This allows us to assess how well the heart is functioning
• Psychosocial evaluation: This allows us to find out the individual needs of every patient and family member and help them deal with the stresses of transplant

Occasionally there are additional consultations or procedures that may be required prior to completion of the evaluation process. Pending clearance from your insurance company you will hear from our scheduler notifying you of the various appointments.

Children who have had a transplant or are listed for a transplant, and their families often face many financial, psychological and social challenges. Our team is committed to finding out about the individual needs of every patient and family and helping them deal with all aspects of the illness.

We have specific resources and expertise to help in this process. As part of the standard care in the outpatient office and in the inpatient units, we perform comprehensive psychological assessments supervised by our dedicated social worker and psychologist. This assessment includes interviews and questionnaires, which will be used to inform you about the potential needs or problems as well as guide you through the recovery process and assist in finding specific help if it is needed.

We believe that education is an important factor in ensuring a successful outcome after liver transplantation. Education helps to foster a good working relationship between your family and our transplant team members.

After completion of the evaluation your child will be discussed at our Pediatric Recipient Review Committee Meeting. The committee is a group of professionals from the various departments that are involved with the transplant team to see if a transplant is needed. If your child is accepted as a candidate you will be notified and your child will be placed on the UNOS waiting list.

All patients are entered onto the national transplant waiting list maintained by the United Network for Organ Sharing (UNOS), a national organization that works 24 hours a day, 365 days a year to match donors to recipients. In order to fairly and efficiently distribute this scarce resource, the country is divided into regions, and most of the organs that come to Mount Sinai are from donors in New York State. The amount of time your child spends on the waiting list is determined by many factors, including blood type and the urgency of his/her condition.

UNOS is a non-profit organization that regulates and maintains the nation's cadaveric (from a person who is brain dead, but whose organs are still viable) organ transplant waiting list. The Organ Procurement and Transplantation Network (OPTN) was established by Congress to ensure that donated organs are fairly distributed regardless of an individual's age, sex, ethnicity, religion, lifestyle or social status through UNOS. Both UNOS and OPTN are regulated by the U. S. Department of Health and Human Services. As of February 27, 2002, UNOS implemented the Pediatric End-Stage Liver Disease (PELD) and the Model for End-Stage Liver Disease (MELD) scoring systems for cadaveric liver allocation. UNOS mandates that all transplant centers in the United States use this new policy.

The Model for End-Stage Liver Disease (MELD) is a numerical scale, ranging from 6 (less ill) to 40 (gravely ill), used for liver transplant candidates age 12 and older. It gives each person a "score" (number) based on how urgently he or she needs a liver transplant within the next three months. The number is calculated by a formula using three routine lab results:

• Bilirubin, which measures how effectively the liver excretes bile
• INR (prothrombin time), which measures the liver's ability to make blood clotting factors
• Creatinine, which measures kidney function (Impaired kidney function is often associated with severe liver disease)

The only priority exception to MELD is a category known as Status 1. Status 1 patients have acute (sudden and severe onset) liver failure and a life expectancy of hours to a few days without a transplant. Less than one percent of liver transplant candidates are in this category. All other liver candidates age 12 and older are prioritized by the MELD system.

A patient's score may go up or down over time depending on the status of his or her liver disease. Most candidates will have their MELD score assessed a number of times while they are on the waiting list. This will help ensure that donated livers go to the patient in the greatest need at that moment.

Candidates age 11 and younger are placed in categories according to the Pediatric End-Stage Liver Disease (PELD) scoring system. Again, there is a Status 1 category for highly urgent patients, representing about one percent of those listed. All other candidates in this age range receive priority through PELD.

PELD is similar to MELD but uses some different factors to recognize the specific growth and development needs of children. PELD scores may also range higher or lower than the range of MELD scores. The measures used are as follows:

• Bilirubin, which measures how effectively the liver excretes bile
• INR (prothrombin time), which measure the liver's ability to make blood clotting factors
• Albumin, which measures the liver's ability to maintain nutrition
• Growth failure
• Whether the child is less than one year old

As with MELD, a patient's score may go up or down over time depending on the status of his or her disease. Most candidates will have their PELD score assessed a number of times while they are on the waiting list. This will help ensure that the donated livers go to the patients in the greatest need in that moment.

While on the waiting list, your child will have follow-up appointments with his/her own physicians as well as regular appointments in our office. At this point, we will start vaccinations against certain viruses and bacteria that sometimes develop after transplant surgery when immunosuppression medication increases the risk of infection.

When a suitable donor has been identified, you will be informed and asked to bring your child to the hospital. This call could come at any time and often happens at night so it is important to be prepared. Upon arrival, your child will undergo a series of tests and be given immunosuppression medications and antibiotics as part of your preparation for surgery.

Your family will wait in the Surgical Family Waiting Room and be kept abreast of your child's progress by a member of our transplant team while your child is undergoing surgery. The transplant surgeon will speak with your family after the surgery.

Liver transplant surgery is extremely complex and on average may last six to eight hours, depending upon the patient's condition. Transplant surgery occurs in four stages:

1. Removal of the recipient's diseased liver from its bed.
2. Placement of the new liver and making vascular connections between donor liver and recipient blood vessels.
3. Removal of the vascular clamps and establishment of the blood flow into liver which is called "reperfusion"
4. The last step is the reconstruction of the biliary tract. This can be done either by connecting the donor's and recipient's bile ducts or creating an isolated intestinal (Roux) loop to connect the donor bile duct.

Immediately after the surgery, your child will be taken to the Pediatric Intensive Care Unit (PICU) where your child will be closely watched. Equipment will monitor your child's heart, blood pressure and breathing, and regular tests including blood tests, electrocardiograms and daily chest X-rays will keep the clinical staff informed of his/her condition. Your child will have several intravenous lines providing medications and fluid. The abdominal incision will be in the shape of an inverted "Y" and will be cleaned regularly. As your child regains his/her strength, certain equipment, IV lines, and tubes will gradually be removed.

Visiting hours are open for parents with all other family members allowed between the hours of 9 A.M and 9 P.M. However, since post-surgical patients are at a high risk for infection, visitors with a cold or infection cannot visit during this time. The length of stay in the PICU varies from patient to patient. Your child will be able to leave the PICU to a regular floor when he o rshe is fully awake, able to breathe on his or her own, and has a normal temperature, blood pressure, and pulse.

From the PICU, your child will be moved to the general pediatric unit where he or she will be more comfortable because continuous monitoring will no longer be required. As the nurses help your child through this stage of recovery, it is important that he or she becomes as independent as possible. Independence includes slowly returning to normal activities such as eating, walking and deep breathing. An inpatient nurse coordinator will explain the various medications and discharge planning and will teach you and your family about the post transplant recovery process.

The average post transplant hospital stay is approximately two to three weeks. Transplantation is a complicated process and may require re-admission to the hospital for problems like rejection or infection. Please refer to our post-liver transplantation education manual.

Follow up visits are very important to your recovery. Visits will be more frequent immediately after discharge and less frequent over the course of the following months. Typically, an outpatient schedule runs as follows:

• Two visits each week for four weeks
• One visit each week for four weeks
• One visit every other week for four weeks or until your child is considered sufficiently stable

Visits are then spread to every two, four, or six months, and even annually, with increasing distance from the transplant.

Your child should continue to be followed by his or her pediatrician after transplant for routine well child care. Our team will be communicating with your child's pediatrician to update him or her on your child's condition. Your child should be able to return to school three months after surgery.

Our Pediatric Liver/Liver Transplant Program is actively involved in basic and clinical research. In addition to institution based research, we are actively participating in a number of national/international multi-center research studies as illustrated below:

• Biliary Atresia Research Consortium (BARC)
• Pediatric Acute Liver Failure Study Group (PALFSG)
• Cholestatic Liver Disease Consortium (CLIC)
• Combination Peg-Intron plus Rebetol for the treatment of Chronic Hepatitis C
• Adefovir in Chronic Hepatitis B Virus infection in children
• Modified release (MR) tacrolimus based immunosuppressive regimen
• Adherence/Post Traumatic Stress in the pediatric liver transplant population
• Studies of Pediatric Liver Transplantation (SPLIT)
• Entecavir in children with Chronic Hepatits B Virus infection

The Mount Sinai Children's Center Foundation was founded to help children have the best experience possible, even while visiting or being in hospital. Programs such as the Child Life Program, the Clown Care Unit, Reach Out and Read, and The Zone all help to brighten our children's experience here at Mount Sinai Medical Center.

For more information about these programs or The Mount Sinai Children's Foundation, please log onto http://www.msccf.org/index.html