My Sister's Story:

In 2002 my sister Malena, then 44 years old, was misdiagnosed as having ovarian cancer, which turned out to be Pseudomyxoma Peritonei (PMP), more specifically, Disseminated Peritoneal Adenomucinosis – origin: appendix (DPMA). Since then I have had the opportunity to study and visit the major U.S. hospitals and doctors that deal with this disease. I have talked to surgeons, oncologists and patients. I have done my homework, knowing that my sister’s life depended on it. I would like to share this information with you in the hope that it may assist you or your loved one in fighting this disease. So, forgive me for the length of the posting, but I want to be sure that I include all relevant details.

The disease comes in two general types: Adenocarcinoma and Adenomucinosis. Of the two, adenomucionosis appears to be somewhat more benign as it is “not technically” a cancer and thus will not usually spread to interior organs (to the lungs and bones, in the rare occasion when it does). Few doctors, however, differentiate between the two. Even at MD Anderson Cancer Center, it is all called “adenocarcinoma.” Dr. Sugarbaker, however, prefers limiting the definition of PMP to adenomucionosis only and excludes cases of peritoneal carcinomatosis from this definition. A good article on this can be found here. But don't be fooled, whether it is Adenocarinoma or Adenomucinosis, it's a serious matter.

This is a rare disease…perhaps fewer than 1,000 cases are known to currently exist. Accordingly, you need a real specialist. Traditional cancer surgeons don’t know enough about the disease to treat it correctly. Even a top oncology surgeon at Memorial Sloan Kettering Cancer Center can misdiagnose it, as it happened with my sister. Having a knowledgeable specialist do the first surgery is perhaps the most important factor in the treatment of this disease, as surgical scaring makes every surgery thereafter more and more difficult.

Most women tend to be in their late 30’s to early 40’s when first diagnosed. I don’t know the average age for men… but have encountered patients from early 20’s to late 70’s. The most common initial symptom is increasing abdominal girth. Men can also show inguinal hernias and women can show ovarian masses.

In the case of my sister, it was an ovarian mass that led to her eventual diagnosis when she was 44 years old. Perhaps more than three years prior to diagnosis, her appendix developed a mucinous tumor. Unnoticed, the appendix eventually burst and spilled out all the mucinous stuff throughout her abdominal cavity (the peritoneum). When her ovary ovulated (i.e. “opened”), some of the mucinous stuff got inside and started growing within the ovary. By the time the doctor noticed the ovarian mass, approximately three years later, it was already 9 inches (23 cm) in diameter. Which brings me to her initial misdiagnosis. After reviewing a CT scan, an oncologist at Baptist Hospital in Miami, Florida diagnosed it as Ovarian Cancer. I had the Chief of the Gynecology Service at Memorial Sloan Kettering Cancer Center review her case. He agreed with the original diagnosis.

Within a few days, we had already scheduled my sister for surgery at Memorial Sloan Kettering. During surgery, the surgeon found such copious amounts of mucinous stuff disseminated throughout her abdominal cavity that he limited the surgery to removal of her ovaries. He thought that, “no further benefit could be afforded to the patient” (i.e. let’s close her up, it is a lost cause). In hindsight, a mistake, but not an uncommon first experience for patients diagnosed with this disease. Which goes to show you that even one of the best oncology surgeons – the Chief of the Department – at one of the best cancer institutions in the world – Memorial Sloan Kettering – can misdiagnose the disease and might not even be able to recognize it under the scalpel. This is the nature of a rare disease. And this is the reason why you need a specialist. In my sister’s case, it was not until seven days after the surgery that the pathology of the tumor revealed the true diagnosis – Disseminated Peritoneal Adenomucinosis – origin: appendix. Here is where my research on the treatment options for PMP commenced.

At Memorial Sloan Kettering, post pathology, the doctors recommended periodic cytoreduction (i.e. debulking). Their approach would be limited to debulking of the tumor through surgery every few years… no chemo, nothing else. But not finding a true specialist on this disease at Memorial Sloan, we decided to look elsewhere. Just for the record, however, I have nothing against this hospital and its doctors. On the contrary, it is a great cancer research center, and should be on the short list of most cancer patients. The only “challenge” we encountered with Memorial Sloan was on insurance issues. If they don’t accept your insurance, be prepared to have to pre-pay for surgery. In any event, we did not see Memorial Sloan at the vanguard of this disease.

Anyone afflicted with PMP would be wise to study all the literature and research of Dr. Paul H. Sugarbaker of Sugarbaker Oncology Associates at The Washington Hospital Center. Certainly, Dr. Sugarbaker is one of the most experienced surgeons on this disease. Most often, Dr. Sugarbaker will only take your case if he believes he has a shot at curing you. He will rate the severity of the case from one to four (not to be confused with the four stages of cancer). In the case of my sister, she was rated a 4+, which meant he would have to remove, along with a bunch of other organs, her entire stomach and colon. This would have left her with a permanent bag instead of her colon (a permanent ileostomy). Even though Dr. Sugarbaker graciously agreed to take her case, my sister was not prepared for such a radical intervention at that time. Had she been at a much earlier stage of the disease, however, Dr. Sugarbaker would have been a very real option for us. He has cured some patients. Others, however, have also died in the process of “going for the permanent cure”.

Among the PMP surgeons, Dr. Sugarbaker is probably the most aggressive and therefore the most controversial. At a very early stage of the disease, Dr. Sugarbaker may be able to cure it. At more advance stages of the disease, surgery for a permanent cure becomes more complicated. A good thing about Dr. Sugarbaker is that he will tell it to you as he sees it. If surgery with Sugarbaker becomes your choice, you’ll have to get into optimal physical shape; as he will tell you, “your survival could depend on it.” One more thing... a few former patients complained about the attentiveness of the hospital staff and suggested that a family member should be with the patient at the hospital at all times. If your disease or health is past the point where Dr. Sugarbaker believes he has a shot at curing it, he will most likely refer you to another doctor (e.g. Dr. Armando Sardi at the Mercy Medical Center in Baltimore, MD).

Before I tell you about other surgeons and hospitals, I need to tell you about IPHC, which stands for Intraperitoneal Hyperthermic Chemotherapy. Not all PMP surgeons use it – that’s why you need to know about it. To explain IPHC, I’ll quote Dr. Brian Loggie. “A major difficulty in treating patients with cancer that has seeded widely on lining surfaces in the stomach cavity (abdomen, peritoneal cavity) is that it is often not possible to remove all the cancer cells.” IPHC is used as “an effort to ‘sterilize’ any remaining tumor cells” after radical surgical tumor removal. “The term ‘intraperitoneal’ means that the treatment is delivered to the stomach cavity. The term ‘hyperthermic chemotherapy’ means that heated fluid (hyperthermic refers to temperatures greater than normal body temperature) is circulated (perfused) throughout the stomach cavity (abdomen).” The heated fluid contains an anti-cancer drug called Mitomycin-C.

So in plain English… the goal is for the surgeon to try to take out as much tumor as possible… and then circulate this heated fluid for a couple of hours around your abdomen (while your abdomen is still open) to kill the rest of the cancer cells. The issue here is that the surgeon will really not know for sure whether or not you are a good candidate for IPHC until the time of surgery. The doctor has to evaluate the plusses and minuses of using it after surgery based on your individual case. As with all treatments, it has risks. If you go to any of the surgeons that use IPHC (which include Brian Loggie from Creighton University, Paul Mansfield from MD Anderson, and Paul Sugarbaker from Washington Hospital), you will receive complete documentation on this treatment.

Now, back to the doctors. At Mayo Clinic in Rochester, Minnesota, there is a wonderful surgeon named John Donahue. My sister loved him. As of 2002, he had performed the cytoreductive surgery on 60 + patients over a 15 year span. He is very knowledgeable and caring. My sister felt very comforted in learning that the surgery was not a one shot deal… that more surgeries could be done in the future if he couldn’t remove all the stuff. We were not as impressed with the oncology team nor with the proposed oncology treatment, however. They suggested an intraperitoneal chemotherapy treatment beginning five days after surgery. We felt that giving the intraperitoneal chemotherapy five days after the operation would not be as efficacious as IPHC due to the tissue scarring that occurs immediately after the operation (scarring prevents the chemo from getting into the tissue). They also proposed some radiation, which can make it a little more difficult to perform more surgeries in the future. PMP patients who have been operated at Mayo feel that this is a great hospital, with phenomenal patient care, totally focused on the patient and not the economics of the business. We loved the doctor, but felt that we wanted a little more aggressive treatment. That left Dr. Loggie and Dr. Mansfield. Loggie was not taking new patients at the time because he was in the process of transferring to Creighton University to set up the clinic there. So, on we went to visit Dr. Mansfield.

Dr. Paul Mansfield from MD Anderson Cancer Center in Houston, Texas performs a procedure similar to that of Dr. Sugarbaker, but because he is not infatuated with “going for the cure” he tends to place more emphasis on the patient’s quality of life post surgery. As of 2002, he had performed 100+ surgeries and only 3 patients had died from complications of the operation itself. He told us about each of those 3 patients (which appeared to be isolated incidents) and he related what he learned from each case. He did not think that he could “cure” my sister by getting all the stuff out… but said he would certainly try his best. He estimated that the operation would take approximately 10-12 hours with an additional 2 at the end for the IPHC.

We scheduled surgery for August 6, 2002 with Dr. Mansfield. We got there about a week prior to the operation itself for a battery of tests, CT scans, and lab work. All done with great care and professionalism. But, despite Dr. Mansfield’s wonderful attention to our case, walking the long hallways from one department to the next and the environment in general made my sister feel as if she was inside a cancer treatment factory, as one more patient going through the “assembly” line. We were comforted, nevertheless, knowing that we were at one of the best cancer centers in the world and under the supervision of a true PMP surgeon specialist.

Things did not go well during surgery. Not because of the doctor or the hospital, but because of my sister’s particular type of tumor. As Dr. Mansfield puts it, “the tumors come in all sorts of different types.” And my sister’s tumor was so encrusted into the organs that too much damage would have been done trying to remove it. He considered removing the entire stomach and colon (as Dr. Sugarbaker told us he would have to do), but opted for not doing so because he would have still left disease in the deep pelvis and up behind the liver. He also opted for not doing the IPHC because, given the abundant amount of tumor still left, he thought it would be better treated with systemic chemotherapy (and doing the IPHC would have delayed the start of the systemic chemo). He did tack the stomach closer to the surface to make things easier at later stages of the disease.

Not much has been posted on the later stages of the disease, so if you don’t want to read about it, skip this paragraph and go on to the next one. The reason why this is a deadly disease is not necessarily because it invades the organs… but instead because it suffocates the organs, preventing them from moving. The entire intestinal tract, in particular, gets stiff, which prevents the food from being digested and excreted correctly. Like a clogged drain, things end up backing-up. This is the reason why he tacked the stomach closer to the surface, so that in later stages of the disease, a port could be more easily installed directly into the stomach and used as “clean-out”. As the disease suffocates the intestinal track, the body is not able to absorb the nutrients from the food. The body, literally, starves to death. The tumor takes over, absorbs all the nutrients from the food and the body’s own muscle (no fat left by then), grows uncontrolled throughout the peritoneal area, and finally, suffocates the patient and evicts him from his own body.

Before I tell you about her initial systemic chemotherapy treatment, you need to know about “establishing a base line” through CT scans and tumor markers. To assess whether a particular treatment is working or not, the tumor needs to be compared to its status prior to treatment. This is done through CT scans and through tumor markers. The tumor markers measure the level of particular proteins excreted by the tumor; the more protein that is excreted, the higher the level of the marker and the higher the probability that the tumor is growing. The markers most used for this disease are CEA, CA19-9 and, for women, CA-125. You can do some research on the specifics of each, but what is important is to see the trend in their numeric values over time. It is difficult to judge the numbers from one month to the next, as the values can fluctuate by quite a bit; therefore, it is best to look at the trend. You should also be careful in comparing the numbers from different laboratories; it is best, if possible, to be consistent in the laboratory doing the test. Finally, you should also know that the normal ranges can vary depending on the test method. For CEA, as an example, one test will have the normal range from 0 to 2.5 for non-smokers and from 0 to 5 for smokers, while another test method will have the normal range from 0 to 5 for non-smokers and 0 to 10 for smokers. So, be sure to compare apples to apples.

We started with systemic chemotherapy on mid-November 2002 (about 3 months after the operation at MD Anderson). Through an IV, she received 300mg of Irinotecan (a.k.a. CPT 11) and 250mg of Oxiloplatinum during approximately a 6-hour period. 21 days later, the procedure was repeated. She was uncomfortable for the first couple of days after each chemotherapy treatment… with headaches, nausea, and vomiting. The second cycle was actually tougher than the first. Her hair thinned out, but she never lost it all. The CT Scans and tumor markers did not show an improvement, and therefore, Dr. Wolff, the oncologist at MD Anderson Cancer Center, switched her chemo treatment to140mg of Platinol through an IV followed by 3500 mg of Xeloda (tablets by mouth) per day for 14 days. 800 mg per day of Celebrex was also added to her regimen. This was repeated every 21 days (i.e. the Platinol given once, Xeloda for 14 days on, 7 days off, and Celebrex uninterrupted). She did three cycles of this routine. Platinol had the similar side effects of headaches, nausea, and vomiting for a couple of days. The Xeloda she tolerated quite well. Celebrex is an anti-inflammatory, which Dr. Wolff started using based on some research done by Dr. Loggie (they all know each other). Again, no improvement was detected either through the CT scans or tumor markers. By now, Dr. Wolff had given up and suspended all further treatment. It was around March of 2003 when I received an email from Dr. Wolff regretfully informing me that there was nothing else that could be done for her.

The most prudent course of action would been to contact Dr. Loggie at this time, but having heard some encouraging results from a few PMP patients taking Thalidomide, we wanted to give it a try for a few months. Thalidomide is the same medicine that was used by pregnant women in the late 1950’s and early 1960’s to combat the symptoms associated with morning sickness. Unfortunately, when taken during the first trimester, the medicine prevented the proper growth of the fetus and resulted in severe malformations of the babies. Thousands of Thalidomide babies were born around the world with missing limbs and other horrific deformities. The drug was pulled off the market, but some years later a group of scientists sought other uses for the drug that had created such atrocious, birth defects. The theory was that because the drug created the malformations by preventing blood from flowing into rapidly growing tissues (as those in a fetus), it could also be used to atrophy the growth of cancer (also a rapidly growing tissue). The drug has now received FDA approval for the treatment of leprosy and is also in various stages of clinical trials for various forms of cancer.

A few years ago, a Doctor, now deceased, recommended this drug to a woman in California afflicted with PMP. She started taking it and after a few months saw her tumor shrink to undetectable levels. This woman also told me that the tumor started growing again after she stopped taking the drug but that it shrank again after she resumed the regimen. After consulting with her then current doctor, a gynecologist-oncologist at Stanford University Medical Center, and evaluating the possible side effects, we decided to give Thalidomide a try. This was in addition to the 800mg per day of Celebrex, which we never discontinued because we knew it was part of Dr. Loggie’s standard regimen (more on this later).

Thalidomide is not inexpensive, in the U.S. the regimen would cost a few thousand dollars a month, which may or may not be covered by insurance. But since my sister lives outside the U.S. (in El Salvador), we were able to obtain it directly from Penn Pharmaceutical in England at a fraction of the U.S. cost. We started at 200 mg per day (at night), increasing the dose by 50mg every couple of weeks until she reached 400 mg per day. She tolerated the drug reasonably well. Side effects can include severe constipation (which needs to be aggressively controlled though diet and stool softeners or it can send you to the hospital), peripheral neuropathy (numbness on body extremities such as fingers and toes – which can become permanent after several months of being on this drug), sleepiness (hence why it is taken at night), and general fuzziness with some memory loss.

At the beginning, her tumor markers showed such a significant improvement that even Dr. Wolff was encouraged by the results and recommended that we continue with it. The good news, however, did not last long. While the CA-125 tumor marker continued to decline, the CEA level reversed its downtrend and started rising once again after five to six months of having commenced with Thalidomide. At about the same time that her CEA level began to increase, her abdomen began to swell and in a matter of eight to ten weeks had already reached the size of a six to seven month pregnancy. Fortunately, a CT Scan revealed that the “abdomen growth” was mostly liquid (known as “ascites”), and we therefore scheduled her for a Paracentesis.

A Paracentesis is a relatively simple procedure used to aspirate the liquid through a fine needle or catheter inserted in the abdomen. Using only local anesthesia, a radiologist generally performs the procedure using an ultrasound to guide the correct placement of the needle. Because the tumor in PMP patients tends to “trap” the fluid in non-connected pockets, it is important that the radiologist be aware of this (otherwise he may not extract all the fluid). In my sister’s case, they extracted 6.2 liters (about one and one-half gallons) of a yellowish/green, free-flowing fluid through two aspirations during her first Paracentesis.

One of the benefits of the IPHC which my sister did not have during her surgery is that it generally prevents the ascites from recurring. Some doctors will also treat the ascites through a non-heated, intraperitoneal chemotherapy. After her first Paracentesis (early part of December 2003) we visited MD Anderson again and at the recommendation of Dr. Mansfield, we met with Dr. John J. Kavanagh, the Chair of the Department of Gynecologic Medical Oncology. Dr. Kavanagh recommended doing the non-heated intraperitoneal chemotherapy at the next “tapping”. That is, waiting until she had more accumulation of ascites (which makes the procedure easier). Basically, in a “reverse paracentesis” format, they would simply inject a chemo drug, known as Taxol, directly into her abdominal cavity. He also had my sister’s tumor tested for EGF Receptors to see if her tumor could be treated with Iressa. Basically, the cells in certain tumors have these receptors that permit this medicine to get inside the cell, and once there, the medicine can destroy the cells from within. My sister’s tumor tested highly positive for the presence of these receptors, and he recommended that she start on a regimen of 500 mg of Iressa on a daily basis. But, stating that treatment with Iressa was a long shot (I believe the FDA actually suspended the use of Iressa in July 2005), I thought it was time that we made a visit to Dr. Brian Loggie at Creighton University Medical Center in Omaha, Nebraska.

I sent Dr. Loggie’s PA (Physician Assistant) at CUMC Cancer Center, 601 North 30th Street, Suite 2321, Omaha, Nebraska, 68131, a complete chronological medical history (everything from a description of her first symptoms, to the official records of all operations, pathology reports, tumor marker levels, and CT Scans). I also arranged for non-stained, tumor slides and tumor blocks to be sent to his office directly from MD Anderson and Memorial Sloan. I believe that helping the doctor treat the patient is part of the patient’s responsibility. And to this end, providing all the medical records well organized and in chronological order is the perfect way of helping the doctor. A detailed cover letter along with a binder containing all the relevant information will go a long way in getting a quick response from a doctor.

Dr. Loggie gave us an appointment to visit with him just a few weeks after receiving all my information. To recapitulate and put the timeline in perspective, she was first operated at Memorial Sloan on April 2002, at MD Anderson in August 2002, had systemic chemotherapy from November 2002 to February 2003, was on Thalidomide/Celebrex from April 2003 to December 2003, and visited Dr. Loggie for the first time on December 2003.

Creighton University Medical Center is located a few miles from Omaha’s airport and an even shorter ride from downtown Omaha. A fairly prosperous town, we liked it from the moment we got there. And unlike the cavernous hallways of most cancer centers, the Creighton Medical Center is a relatively small facility. To us, being Catholic, having the statue of St. Joseph greet us at the entrance of the hospital was very comforting.

We met Dr. Loggie on December 23, 2003 and after a physical examination of my sister and a thorough discussion of her medical history, he reviewed all her CT Scans with us (7 in all). In this process he showed us the progress of the disease. More accurately, I should say the lack of progress of the disease since her operation at MD Anderson back in August 2002, as the tumor had stayed basically the same. It had shrunk a little around the liver and had grown a little around the pelvis, but for all intents and purposes, it was about even.

What I believe differentiates Dr. Loggie from some of his other colleagues in the treatment of this disease is that he will approach it from both a surgical and biological tumor perspective. He will perform surgery when necessary, but as he says, “treating this disease surgically is like hunting with a limited number of arrows; you want to be certain that you use an arrow when you are sure to hit the target.” From the biological perspective, he had his pathologist analyze the tumor and, after the analysis was completed, put her on a daily regimen of Xeloda (2000 mg per day), Tamoxifen (20 mg per day) and Celebrex (800 mg per day). His goal was to put her on a regimen with medicines that she could tolerate well over many years (i.e. not a one shot chemo treatment), and then perform surgery as necessary.

While more recently Celebrex has become a controversial medicine when given at high doses (above 400 mg per day), this medicine is an anti-inflammatory, which, Dr. Loggie believes, helps control the growth of tumors that overexpress Cox-2 (a test performed by the lab on a piece of the tumor) while also preventing inflammation of the organs that have been surrounded by the tumor. As to Thalidomide, Dr. Loggie told us that many years ago he had also prescribed it, but that because the side effects can “catch-up to the patient”, and because of the availability of better treatments, he had stopped prescribing it long ago. We were beginning to see those side effects ourselves - memory lapses, neuropathy, loss of muscle mass, etc. At Dr. Loggie’s recommendation, we stopped Thalidomide.

During the first three months of Dr. Loggie’s treatment (i.e. first part of 2004), my sister’s tumor markers (i.e. levels of CA19-9, CEA, CA-125) continued to increase, but then they dropped drastically and for a period of six months continued to decline. She was a little anemic, but we controlled that fairly well with Procrit (40,000 units, subcutaneous injection, every other week, given indefinitely). Her ascites diminished, but she still necessitated a paracentesis once every three months or so. But then, at a rate even quicker than their decline, her tumor markers shot right up, even past the previously established high water mark levels. Her ascites accumulated at such rate that she was in need of a paracentesis almost every other week. This all happened during the last three months of 2004. Dr. Loggie decided to perform surgery, and on January 4, 2005, she underwent a 12-hour operation.

Between ascites and tumor, Dr. Loggie removed a total of almost 35 pounds (about 14 liters). One of his most difficult surgeries to date (because of the way my sister’s tumor is so attached to everything), he hoped that by removing such copious amounts of tumor and ascites, that it would give Xeloda and Celebrex (now reduced to 400 mg per day because of the recent findings of side effects at higher doses) an opportunity to work better. Unfortunately, this was not to be the case. By May 2005, just five months after this 12-hour surgery, her belly was bigger than ever. The size of an eight-month pregnancy, her belly was growing at such rate that we could physically see it from one day to the next. News got worse. A paracentesis failed – it was not ascites, but tumor and jelly! Her kidneys were beginning to fail, as the tumor was constricting the ureters, preventing the urine flowing from the kidneys to bladder. She could barely move as something was pinching her sciatic nerve, and aside from her distended belly, she was thin as rail.

Lost cause, right? Wrong. With much prayer and Dr. Loggie by our side, we battled one thing at a time. Firstly, Dr. Loggie had a urologist from Chreigton University insert some stents into her ureters. Going through the urethra (the canal from the bladder to where you “pee” from), the doctor “snakes” some stents from the bladder to each kidney. This opens up the ureters, thus allowing the urine to flow from the kidneys down to the bladder (the stents would be replaced about every 3-4 months). Secondly, cortisone was injected directly into her lower spinal cord (much like an epidural) to alleviate her sciatica (this procedure was repeated twice). Thirdly, Dr. Loggie had us visit an oncologist who was about an hour from Omaha – Dr. Qamar S. Khan of Midlands Regional Cancer Center in Fremont, Nebraska. Dr. Khan is a wonderful guy. He spent an hour with us, lifting our hopes by discussing different treatment alternatives. Armed with Dr. Khan’s recommendation (more on this below), Dr. Loggie gained sufficient confidence to perform yet another surgery (note: performing a surgery without a post-surgery treatment alternative made little sense, but with a real, post-surgery treatment option, it was a different story).

On June 2nd 2005, my sister underwent a six-hour surgery. Dr. Loggie removed enough jelly, ascites, and tumor to reduce her abdomen’s girth back to post January 2005 surgery levels (about normal girth, just a little protrusion on her lower belly). His goal with the surgery was to relieve her discomfort but, more importantly, to give the medicine a shot at shrinking the tumor, since it was impossible to remove her entire tumor through surgery (every case is different).

During surgery, Dr. Loggie left a port into her abdomen so the ascites could drain. Here is a picture (click on it to enlarge) of the catheter going into her abdomen. The drainage tube is connected to a plastic receptacle that aspirates the fluid through the pressure created by the vacuum in the receptacle. Note from the picture that a little jelly also drains from around the area where the catheter enters the abdomen.

Dr. Khan recommended increasing Xeloda to 4000 mg per day (14 days on, 7 days off) with 380 mg of Avastin via IV every 2 weeks. Avastin is one of those new drugs (developed by Genentech) that prevents blood from getting to the tumor, and without blood, the tumor gets killed. When combined with chemotherapy (Xeloda is chemotherapy, but oral) Avastin can have very nice results. My sister started on this treatment about two weeks after surgery, along with 400 mg per day of Celebrex and 40,000 units of Procrit, injected subcutaneously, every other week.

I should mention that Dr. Khan had originally recommended putting her on Irinotecan/Camptosar (237 mg via IV, weekly, 4 times), Leucovorin (38 mg via IV, weekly, 4 times) 5FU (950 mg IVP, weekly, 4 times) and Avastin (380 mg via IV, every other week), but given that my sister was so weak after surgery (and so mentally disturbed about starting on chemotherapy once again), he acquiesced to substituting the more aggressive IV chemotherapy (Irinotecan, Leucovrin, 5FU) with Xeloda, which is taken orally.

The Avastin/Xeloda treatment worked like a charm. During the first three months, the tumor marker levels decreased very little, but best of all she experienced no abdominal growth. Another sign that the treatment was working was the amount of daily fluid draining from her abdomen, which decreased from 280 ml per day to less than 50 ml per day. Her appetite diminished, but Dr. Khan corrected that with Marinol (2.5mg once per day). Marinol is a medice derived from Marihuana, which increases appetite considerably. My sister’s appetitive indeed improved on Marinol (but sorry, none of the “other” college-tried, side effects from Marihuana).


Here is my sister Malena during our September 7, 2005 visit to Creighton University Medical Center, at Dr. Loggie's examining table, three months after surgery having gained 12 pounds with no increase in girth (i.e. no tumor growth).


This graph shows Malena’s tumor marker levels since April 2002 (click on graph to enlarge).

The scale for both CEA and CA-125 is to the left. The scale for CA19-9 is to the right. Note the sharp drop since the highest point towards the right. That’s when the Avastin/Xeloda treatment was started.

From a high of 371, 6,914 and 65 for CEA, CA19-9 and CA125, respectively, prior to the August 2005 surgery, the tumor marker levels plummeted to 38, 943, and 29, respectively, within a year. But more importantly is how Malena felt during this time period. She gained (good) weight, was able to return to work, and drive around town. She enjoyed life with energy and optimism, her facial expression radiating happiness. During this time period, her hemoglobin level also increased nicely, probably due to the weekly regimen of 40,000 units of Procrit.

One of the side effects of Xeloda, however, can be redness and tenderness of the feet and hands. Here are some pictures so you can see what I am talking about.

Dr. Khan prescribed 100 mg of Vitamin B6 once per day for the tenderness, but we found that a cream brought to us by a dear cousin all the way from the Dead Sea worked best. The cream’s name is Ahava Dermud, and it is supposed to have some curative mud from the Dead Sea. All I can say is that it worked. It can also be purchased through Drugstore.com.

Around June 2006, almost a year after the Avastin/Xeloda treatment started, Malena developed an infection causing her intermittent but unrelenting fevers. A culture of the peritoneal fluid draining from the catheter identified certain bacteria; this caused us to attribute the infection to the catheter, a double edged sword - on the one hand it was helping to drain the ascites and on the other a foreign body that could cause infections (on the balance, however, it was doing significantly more good than harm). We treated the infection with multiple antibiotics with sporadic success, until fecal material was also identified in the peritoneal fluid around August 2006.

Fecal material was now a clear indication that there was a perforation in the intestine, a significantly more complicated matter. The perforation could have been caused by the catheter itself rubbing against the intestines or, perhaps more likely, a known side effect from Avastin.

Protocol calls for discontinuing the use of Avastin when there is a perforation of the intestine, as a perforated intestine can lead to fatal peritonitis (fecal material spread throughout the peritoneum). But discontinuing Avastin was likely to cause the tumor to resume its lethal growth. Yet, while on Avastin, surgery is not possible as it basically makes incisions impossible to heal; so, repairing the intestine was also out of the question until at least 6 weeks after the last Avastin/Xeloda treatment. All roads were cul-de-sacs -- at least for 6 weeks.

Dr. Loggie discontinued Avastin and Xeloda for 6-8 weeks, but when this time elapsed, despite our unrelenting pleadings, desires, and expectations, he elected to let the intestines heal on their own instead of performing surgery, which he deemed practically impossible. To assist in the healing process, he put her on TPN (Total Parenteral Nutrition – e.g. feeding her through an IV), but within days we could see that the tumor was growing again, and I knew we were beginning to relive the story from a year earlier but infinitely more complicated.

I sent Dr. Loggie and Dr. Khan the following email:

-----Original Message-----
From: Suarez, Jose
Sent: Thursday, November 09, 2006 7:02 AM
To: qamar khan; Loggie, Brian
Subject: Maria Suarez - El Salvador

Dear Dr. Loggie and Dr. Khan,

Since her last surgery on June 2005, when she was facing a multitude of problems, your combined efforts have managed to extend my sister's life by more than one year. During this time she has been able to enjoy her children, grandchildren, parents, home, and more. And for this we are eternally grateful.

It is with an aching heart that I see her now... fighting to stay alive. She is fighting against the tumor that started re-growing after we suspended the Avastin/Xeloda ten weeks ago in preparation for surgical intervention to correct the intestinal fissure.

The Avastin/Xeloda was working so well. Her tumor markers had dropped by almost 90%. We had even noticed a remarkable softening of the tumor tissue.

Given that her heart and lungs are still strong, isn't there something we can do to extend her life by even a few more months? Yes, she is frail, but she has uncanny desire to live and she is a fighter that never gives up.

Isn't there some minor debulking that could be performed that would palliate the pressure the tumor is creating and allow us to resume the Avastin/Xeloda? Without this, we all know she will die... so, even if she dies from complications post-surgery, we are no worse off, and we have a chance at extending her life by even a few months.

Because she is such a fighter, I feel we should seriously consider fighting by her side.

Please consult with one another as, ultimately, the possibility of a few more months of life rests in your hands. If after deliberate consultation with one another, you still arrive to the current conclusion, I will respect it and be guided it. Similarly, if you decide that there is a fighting chance - even for a few more months – I will stand by your side regardless of the outcome.

Thank you for your consideration.

Jose



To which Dr. Loggie responded:

----Original Message-----
From: Loggie, Brian
Sent: Thursday, November 09, 2006 4:23 PM
To: Suarez, Jose; qamar khan
Subject: RE: Maria Suarez - El Salvador

Jose

More surgery is without value in the current situation. Her medical condition currently contraindicates chemotherapy or biologic therapy. She has agreed to a "do not intubate" and "do not resuscitate" status. The biggest problem at present is anxiety, which we will treat. We are continuing with TPN for nutrition. Let us see how she does.

bwl



I knew this was tantamount to a death sentence, which, in fact, was executed by nature merely four days later on November 13, 2006.

I’ll need more time to post on the last week of her life. In the meantime, here is My Eulogy and a few closing remarks:

Her friends, family, and co-workers were critical to our all-fronts battle. Thank you for always being by her side.

I am indebted to all the doctors, nurses, and medical staff that attended to my sister; they are all highly competent and helped us to the best of their ability.

I am eternally grateful to everyone who prayed so much for my sister. These prayers were answered through the extension of her life here on earth and in creating a more fervent union with God while on earth and in eternity.

And most importantly, I thank God for giving us the opportunity to serve Him through my sister's passion.

I hope all this information helps you or a loved one battle this disease. If there is anything I can do for you, you can reach me via the the contact information in the link above or by leaving a comment here. If you need someone to pray for you or a loved one, I have found the prayers of The Sisters of Our Lady of Mercy quite powerful (click on the prayer request link on their site).

May God provide for you and comfort you. Do Not Relent, there is always hope.

Jose Suarez
Malena's Brother

My Eulogy on Malena

32 comments:

Anonymous said...

Jose,

I am so sorry to hear of Malena's passing. Please accept my condolences. I will keep you and her in my prayers. God bless.

LaVerne (from pmpbellybuttons)

lionhearted kat said...

Thank you for your fight for your sister, Jose, but also for the rest of the PMPers around the world. Your powerfully told story will help many others.

My heart is pained for your loss, but may you find comfort in knowing your sister is with the Lord, no longer in pain.

We will continue to pray for Malena's family and for you, her devoted caregiver.

Kat and PMP husband in Omaha

Anonymous said...

Jose,

I am so sorry to hear of your sister's passing, but thank you for your beautifully written account of her illness and treatment, your advocacy for your sister and for many others who have been educated by your site.

Take very good care,

Carolyn
www.appendix-cancer.com

Anonymous said...
This comment has been removed by a blog administrator.
Anonymous said...

Jose,

Thank you for your detailed account of Malena's experience with this disease. I have been living-with/fighting PMP for over 7 years now, and have found your story very informative and reassuring. I was so sad to read that Malena lost her battle with this strange disease. I'm sure she appreciated you as a wonderful brother. I will include your family in my prayers.

2007chaos said...

I've just heard of a friend of a friend who's just been diagnosed with PMP....NO-ONE i KNOW HAS EVEN HEARD OF IT. Your sister's story was very inspiring and your tenacity in helping her fight was an example to allof us who have relatives battling cancer. I hope and pray that our friend's family will help and support her in the same way. a Scottish carer.

Anonymous said...

I have just heard of of dear friend with this disease. My first response was to go the the net and do some research.
Finding you story has touched me. It was powerful and the bond of love that the 2 of you shared is inspirational and meaningful.
I thank you for taking the time to put it all in writing. I would think that in doing so you were torn with pain, yet released by your sharing and your gift of telling a story.
I thank you.

Anonymous said...

Jose,

I am so sorry to learn of your dear sister's passing. I found your well written blog after my husband was diagnosed with appendix cancer in July 2006. I followed your blog, and stopped once I gave birth to my daughter Carmen who was born on November 13, 2006. I have just now had the courage to check your blog to see how your sister was doing.

You are such a loving brother and all the people you will educate on this illness will be a tribute to your sister's life. May God bless your family and give them peace and comfort.

Ayana and Will, Philadelphia

Anonymous said...

Jose,
Thank you for sharing Malena's story. I'm one year post-op and appreciate all the information. Your sister sounds like she was a wonderful person. I send my love and prayers out to your family.

JaNae

Anonymous said...

Information speeds progress, thank you Jose for yourloving march beside your sister,Malena. Her story will help others and I am sending it on to a cousin whose best friend is battling PMP. God bless all those with medical needs.

Anonymous said...

Your devotion to your sister, and your astounding pursuit of her treatment possibilities, tracking each detail with such specificity and unrelenting zeal, has touched me greatly. We have a young friend now battling this disease in Canada, for whom we pray. May it be of some comfort to you and your family that your loving passionate perpetuation of your sister's memory and fighting spirit are reaching and helping many others.

Kylie said...

Your story was very touching. I too, lost my father at the age of 51 to this disease in December 2003 after very similar radical surgeries and chemo. Your story was beautifully written and brought back so many memories for me... Best of luck to you xx

tracey said...

Dear Jose,

I am truly sorry about your sister. My aunt has PMP. She had surgery and the "shake and
bake" almost four years ago. She is now again at Wake Forest Baptist Medical Center where she had more surgery. Her large intestines were removed and a colostomy bag was placed. Unfortuartely, her cancer has returned. Your story was inspirational. Thank you for sharing it.

Anonymous said...

Jose,

I do not know you personally but am very sorry for your loss. Your story is helpful to PMP patients as well as other cancer patients. It gives hope as well as knowledge.

I just found out my cousin has PMP, and found your story. It should have been titled "Our Story", it appears you had a hand in helping your sister find the best care possible and fought for her as much as humanly possible.

I was recently diagnosed with an aggessive form of breast cancer and know the huge amount of effort it takes to find detailed information regarding a particular illness and treatment. We are finding new things everyday.

I have watched my mother battle cancer, she passed a number of years ago and know the toll it can take.

You are a real hero to your beloved sister as are other family members who help cancer patients do research and assist in the care of the patient.

I know we have a long road ahead emotionally and financially. My husband is doing the best he can to research, while working a full time job, cooking, cleaning, taking care of the kids and me. At times he works odd jobs and will take a second job if needed to pay bills. People like you and my husband are hero's that make it possible for us cancer patient's concentrate on our own health with surgeries and treatment. In my husband's words "it is your job to get better and my job to do everything else".

God Bless our Hero's!

Trish-MI

Anonymous said...

Thank you very much for charing with us your sister's story,i,m so sory for here passing.My mother has the same dessease.Ihave read to here Malena's story,but not until the end,because i don't want here to be sad.I want to ask It is better for my mother to strt a treatment with avastin and Xeloda?She already suffered a surgery...
Camelia form Romania!
God help us all!

Anonymous said...

Thank you for this information. Your clear and precise telling of this heartwrenching story helped me to understand my options -as I have been diagnosed with PMP last Friday. So much loss and so much joy. It comforts me to know that God is watching us and just waiting for us to ask for help.... thank you again

tmunoz30 said...

Jose,

Thank you for this blog. The information you've posted is extremely helpful. My deepest condolences to you and your family.

My mother was just diagnosed with poorly differentiated mucinous adenocarcinoma of the appendix. She was admitted to the ER with what the doctors thought was appendicitis, and they found the tumor which extended to two out of nine lymph nodes, which were removed with her appendix. Long story short is that we are now trying to find the best treatment/cure for her. She is a Kaiser patient right now. Although Kaiser has a decent reputation for their oncology dept her in So Cal, we (the family) are very concerned at the way they're handling this. We've looked extensively into cytoreduction surgery with HIPEC. We've spoken to three doctors including Dr. Sugarbaker, Dr. Roy-Chowdhury of Loma Linda University Medical Center, and Dr. Joshua Ellenhorn of the City of Hope, and they all pretty much agree on the use of cytoreduction surgery with HIPEC.

Any comments, suggestions, experiences with any of this or these doctors would be greatly appreciated. We are just beginning this fight and are very scared.

The Harings said...

Jose,

Thank you so much for posting this! My friend is going through the same thing right now as your sister, and your blog has made it easier for us to share with others the seriousness of her situation as it is a complex confusing disease. I am so sorry to hear about your loss. You are in our hearts and prayers.

Joy in MN

Paul said...

I am so sorry for your loss.

Thank you so much for sharing your story.
It really helpful to me (and I'm sure many others) that are helping loved ones through PMP.

All my best wishes to you and the rest of Malena's family

Paul

Anonymous said...

Hello to everyone...I am new to these, blogs etc.... I am hAving a very difficult time finding an answer for a treatment appropriate for my 84 yr old mother diagnosed with appendicial ca... I have gone to several doctors which I am not quite satisfied or maybe just me, I'm still I denial.... My mother has no significant medical history, she is healthy , and strong and the only thing that bothers her is her arthritis... She did chemo the platin and 5fu but the cells did not shrink and doctors didn't want to operate her anymore vs the quality of life she is living now. One HIPEC surgeon however told us she is a good candidate...however I came across , as. I have been searching the internet the very famousd dr Sugarbaker , a certain llse for DrSugarbaker replied to my question and told me 60 yrs of age is the cut off for HIPEC...now this surgeon from Nebraska also told us no chemo therapy. Is going to work at this time bec of the mucus. My dilemma is does this mean we surrender all medical treatments and just wait for that day to come... It already hurts me to think of that time to see my mom suffering and I know I am going to look back and would asked myself have I done enough and the right thing?... I don't know what else to do..we have a pending appt at a uc San diego md dr Andrew lowy.please pray wt us to get the right medical help.... I might lost this page if you have any comments you can ail me at rachelrn105@hotmail.com... Thank you very much for all who posted in here...it is a comfort to be able to meet even in a simple blog to help learn and support each other and to know we have common struggles and pain. God bless you all.

Anonymous said...

I tried to set an appt wt dr Sugarbaker fory 84yr old mother. As per staff dr Sugarbaker is not taking patients older than 60 yr old.... If anybody had an idea why please explain further... I did not fully understood what the staff explained and sound impatient to explain further although she was nice replying to me I didn't want to bother her anymore. All I wanted was to sit down with dr Sugarbaker get his thoughts and opinions regarding my mom's case even if she is not a candidate for HIPEC...guess it is really very hard to touch bases with very prominent people.

Term papers said...

Good Article About "My Sister's Story:"

Brenda said...

I have a friend who has been diagnosed with this same disease. She has had the initial surgery thinking at the time she had ovarian cancer. She is scheduled for surgery and the "chemo wash" depending what the surgery reveals. Surgery will be performed at Vanderbilt Hospital, Nashville, Tn. I had never heard of the disease until she was diagnosed. I applaud you and your courageous sister for a battle well fought against a horrible thief.

Brenda

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JennStar said...

Wow!!!!! What a epic battle, so sorry for your loss.This brought me to tears, she was lucky to have such strong support! I wish you and yours well, and great job at documenting and telling this story.

Belief23 said...

Thank you so much for sharing your Malena's Story. My father was just diagnosed with PMP and we are headed to Mayo Clinic. You commented that people who were treated there had great things to say. I haven't read anything on Mayo Clinic for this specific treatment. Do you have more information?

L said...

Belief23, you might also look into Dr. Greeno at the U of MN (Masonic). The surgeon he works with is Dr. Tuttle.

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Anonymous said...

Thank you so much for your blog. A friend has just been diagnosed with pmp 4 months after having her appendix removed and before finding your blog we struggled to find much information about the condition.
I am so sorry for your loss and wish you all the very best for the future.
Thank you.

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