Monday, October 13, 2008

TP/ICT - My Surgery August 28, 2008

After surgery on August 28th and subsequent hospitalizations (1) pneumonia, (2) vomiting and inability to eat, and then again because of (3) hemorrhaging and needed blood transfusions), I have had the SBFT (Small Bowel Followthrough) and the upper GI that was ordered by my surgeon. I then saw my GI, Hepatologist, Dr. Faragi. Here are the reports from the tests:

Upper GI:

  • Bowel gas pattern is generally in the normal limits.
  • No evidence of esophagitis
  • Patient demonstrates severe spontaneous gastroesophageal reflux with the barium column
  • extending nearly to the thoracic inlet (see fig. 1 & fig. 2)
  • No distinct hiatal hernia
  • Stomach appears to distend normally

fig. 1

fig. 2

Small Bowel Followthrough

The duodenal bulb appears smoothly contoured. There is inflammation of the duodenum. The descending portion of the duodenum is abnormal in appearance with distortion of the lumen of the bowel. This may be due to scarring from the patient's inflammatory bowel disease. There is small diverticulum. (My Crohn's Disease was never in the small bowel...only in the colon and this inflammation and scarring is most likely due to Pancreatitis...according to my GI, Dr. Faraji)
There is free reflux of barium through the ampulla into the common bile duct and proximal biliary tree (When I am refluxing pancreatic fluids and bile in this manner, oh, the pain...right dab smack in the center of my upper quadrants, and radiating into my back!). (see fig. 3)
Beyond the duodenum, the rest of the small bowel otherwise appears within normal limits. A couple of small diverticuli are present on the transverse portion of the duodenum.
There is otherwise no fixed or dilated loop of small bowel. Transit time through the small bowel is normal
Multiple images of the terminal ileum are normal in appearance.

fig. 3

Dr. Faraji says that this reflux through the ampulla where the pancreatic duct meets the duodenum, and then continues to back up into the common bile duct and proximal biliary tree, and the fact that there is inflammation, shows I am in the midst of having an attack of Pancreatitis, even though my pancreatic enzymes no longer elevate due to atrophy of the pancreas. My last EUS showed a tortuous pancreatic duct that was dilated more than 3X the normal size. The duct had clubbed side branches. So there is a problem with the pancreatic duct, the ampulla, and the opening to the duodenum. This is where the Sphincter of Oddi lies, and is no doubt cause of my SOD (Sphincter of Oddi Dysfunction)

I am slated for many lab tests:

  1. Hep A, AB, hep BsAg, Hep BsAb, Hep Bc IgC, Hep C Ab
  2. Antimitochondrial Ab- AMA (To test for PBC - Primary Biliary Cirrhosis PBC , Lupus, andHemochromatosis {see Below #14}) Other Related tests: Autoantibodies, Alkaline phosphatase (ALP), Alanine aminotransferase {ALT}, Liver Panel)
  3. ANA (Also, to test for PBC and other autoimmune diseases)
  4. Anti Liver-Kidney Ab_Live-Kidney microsomal Ab
  5. Anti Smooth muscle Ab- ASMA (Another test for help distinguish PBC from another disease such as Lupus, Rheumatoid Arthritis or thyroiditis)
  6. Alpha-1 Anti Trypsin phenotype-A1AT (Alpha-1 antitrypsin {AAT} is a protein that is produced in the liver and released into the bloodstream. AAT helps to inactivate several enzymes but primarily works to protect the lungs from elastase. Elastase is an enzyme produced by neutrophils and it is part of the body’s normal response to injury and inflammation. Elastase breaks down proteins so that they can be removed and recycled by the body but, if its action is not regulated by AAT, elastase will also begin to break down and damage lung tissue.
    AAT is produced at the direction of two copies of a protease inhibitor (Pi) gene. This gene is co-dominant, which means that each Pi gene copy is responsible for producing half of the body’s AAT. If there is a change or mutation in one or both of the gene copies, then less AAT and/or dysfunctional AAT is produced. If the resulting AAT production drops down to 30% of normal or less, then the affected patient will experience a disorder called alpha-1 antitrypsin deficiency. Patients with this disorder are at a considerable risk of developing emphysema (a progressive lung disease) in early adulthood. If they smoke, or are exposed to occupational dust or fumes, the lung damage tends to occur sooner and be more severe.
    If the AAT produced is dysfunctional it tends to accumulate in the liver cells that produce it. As it builds up in these cells, the AAT forms abnormal protein chains, and begins to destroy the cells and damage the liver. About 10% of those affected with AAT deficiency will be jaundiced as a newborn. Many improve on their own but in severe cases these infants may require a liver transplant to survive. AAT deficiency is currently the most common reason for a liver transplant in the pediatric population.
    The amount and function of the AAT created depends on the mutation inherited. While there are more than 70 different alleles (variations) in the Pi gene, only a few are common. Most people in the U.S., about 90%, have two copies of the normal M gene (MM). The most common of the abnormal forms are S and Z. Those people with:
    • One copy of M and one of S or Z (MS or MZ) will produce reduced amounts of AATbut should have enough to protect themselves. They will be carriers of thecondition, however, and can pass it on to their children.
    • Two copies of S(SS) may be asymptomatic or moderately affected (they produce about 60% of therequired AAT)
    • One copy of S and one of Z (SZ) are at an increased risk ofdeveloping emphysema (they produce about 40% of normal AAT)
    • Two copies of Z(ZZ) are the most severely affected (they only produce about 10% of the requiredAAT) along with those who have one or two copies of rare forms of the Pi genewhich are “null” (they do not produce any AAT).
    Types of AAT tests
    Different AAT tests can be used to measure the amount of AAT, determine which types and concentrations of AAT protein are present, and determine which Pi gene alleles a patient has.
    • Alpha-1 Antitrypsin, this test measures the concentration of AAT present
    • Alpha-1 Antitrypsin Phenotype, separates out the different variants of alpha-1 protein being produced and compares them to known patterns. It also allows an estimation of the amount of each type present. Since AAT is an alpha1 globulin type of protein a regular protein electrophoresis test can be used to screen for a severe AAT deficiency {this is the one my doctor is using for me}
    • Alpha-1 Antitrypsin DNA testing, genetic testing can be done to identify which protease inhibitor gene mutations (Pi gene alleles) are present. Only the most common mutations are usually evaluated (M, S, Z). This test can be used to help evaluate affected patients and their family members.
  7. Alpha Fetoprotrin- AFP (AFP is used to detect tumors that mark cancers of the liver, testes, and ovaries. Patients with chronic liver diseases such as cirrhosis or chronic hepatitis B must be monitored at regular intervals because they have a lifetime risk of developing liver cancer. A doctor may order an AFP test, along with imaging studies, to try to detect liver cancer when it is in its earliest, and most treatable, stages. Increased AFP levels can mean liver cancer, cancer of the ovary, germ cell tumor of the testes, cirrhosis, hepatitis, or other cancers (stomach, colon, lung, breast, lymphoma). {I think this is a very good test for me.})
  8. Serum Ferritin, Iron Sat., TIBC, transferring () retic count (Serum Ferritin: To determine whether your blood iron level is normal. The serum iron test measures the amount of iron in serum, the liquid portion of blood. Iron is an essential trace element. It is necessary for the production of healthy red blood cells, which carry oxygen through your body, and some enzymes, which perform tasks in your body. ) (Total Iron Binding Capacity {TIBC} - measures the amount of iron that can be carried through blood by transferrin. Transferrin is the protein that transports iron from the gut to the cells that use it. Your body makes transferrin in relationship to your need for iron; when iron stores are low, transferrin levels increase and vice versa. In healthy people, about one-third of the binding sites on transferrin are used to transport iron. This number is called the transferrin saturation.)
    (TIBC transferring: Transferrin is the plasma protein that binds to iron and transports it through the circulation. TIBC measures the total amount of iron that transferrin can bind. While the two tests are different and are reported in different units {g/L for transferrin and umol/L for TIBC}, they measure essentially the same thing.)
  9. Serum Ceruloplasmin, copper (Ceruloplasmin is primarily ordered along with blood and/or urine copper tests to help diagnose Wilson’s disease, an inherited disorder associated with decreased levels of ceruloplasmin and excess storage of copper in the liver, brain, and other organs. Rarely, it may be ordered to help diagnose or differentiate between conditions associated with copper deficiencies. It is ordered along with copper tests when someone has signs and symptoms that the doctor suspects may be due to Wilson’s disease such as: anemia, nausea, abdominal pain, jaundice fatigue, behavioral changes, tremors, difficulty walking and/or swallowing, dystonia. Rarely, ceruloplasmin may also be ordered along with copper tests when your doctor suspects that you have a copper deficiency and periodically if monitoring is recommended.)
  10. TSH, Free T4 (Thyroid)
  11. LFT: Alb, T Bili, D BiliAST, ALT, Alk Phos, GGT, total protein (Liver function)
  12. PY, PTT, INR (The PT may be ordered when a patient who is not taking anti-coagulant drugs has signs or symptoms of a bleeding disorder, which can range from nosebleeds, bleeding gums, bruising, heavy menstrual periods, blood in the stool and/or urine to arthritic-type symptoms {damage from bleeding into joints}, loss of vision, and chronic anemia.) (see fig. 4 below)
  13. CBC with diff
  14. Hemochromatosis HFE mutation: C282Y, H63D (HFE gene test - Hemochromatosis is a common genetic disease that causes your body to absorb too much iron. It is usually due to an inherited abnormality in a specific gene, called the HFE gene, that regulates the amount of iron absorbed from the gut. In people who have two copies of the abnormal gene, too much iron is absorbed, and excess iron is deposited in many different organs, where it can cause damage and organ failure. The HFE gene test uses a sample of blood drawn from your arm to see if you have the mutations that cause the disease {the most common is called C282Y}. Too much iron can lead to damage to a number of organs, including the heart, liver, pancreas {where insulin is made}, and joints. The most common cause of iron excess is an inherited disease called hemochromatosis. In this disease, the body absorbs more iron than it needs from the gut, and the excess iron gradually accumulates, causing organ damage over many years. The disease is inherited, usually when you get one copy of an abnormal HFE gene from each of your parents. People with only one abnormal HFE gene show no evidence of the disease. One good point...I have never had heart problems or disease! {Maybe I do not have the HFE gene. However, I do have the pancreatitis and liver problems. I have the same fingers, hands, knees and shoulders arthritis, I have the fatigue and many other of the symptoms. We shall see! }
    Many people who have hemochromatosis will have no symptoms for their whole life, while others start to develop symptoms such as joint pain, abdominal pain, and weakness in their 20’s or 30’s. Heavy alcohol use seems to increase the amount of iron absorbed, while women are somewhat protected because they lose iron every month with their menstrual period. There is now a test to detect the abnormal form of the gene; this can be used if you have unexplained high iron levels or if you have a family history of hemochromatosis.)

Some Clinical Manifestations of Hemochromatosis {the symptoms in bold are ones I have}:

  • Malaise;
  • Liver cirrhosis (with an increased risk of hepatocellular carcinoma.) Liver disease is always preceded by evidence of liver dysfunction including elevated serum enzymes specific to the liver;
  • Insulin resistance (often patients have already been diagnosed with diabetes mellitus type 2) due to pancreatic damage from iron deposition;
  • Erectile dysfunction and hypogonadism;
  • Decreased libido secondary to the above;
  • Congestive heart failure, arrhythmias or pericarditis;
  • Arthritis of the fingers (especially the first and second joints), hands (especially the first and second MCP joints), but also the knee and shoulder joints;
  • Adrenal gland (leading to adrenal insufficiency)

For a graph of iron problems, see fig. 5 below

Now, I am sick and scared, thinking of each of these lab tests, and hoping I have none of those horrible genes. Well, I take it back! I am not scared as I know my Lord is there for matter what...and He will seem me through whatever comes my way.

Next we will do endoscopy and colonoscopy on October 15th. We are certain my Crohn's has become active, and Dr. Faraji wants a closer look at my upper areas. He may decide on an ERCP after results from the EGD. We are awaiting authorization from my insurance for a Gastric Emptying study to check my Gastroparesis.

So, it seems my GI is in full Super Force, battling the evil and batting down the hatches... looking for anything causing my vomiting, pain, bleeding and sickness.

I am still weak, sore, tired, nauseated and all around feeling totally sick.

But, don't worry! This too shall pass!

Love, hugs, and prayers for you and your family!

God bless and stay well,