Diane - Liver Resection
Travel is my passion. I enjoy coming across different areas of the world, finding new people, stumbling upon interesting customs, tasting new foods and communicating with newly found friends. I am a world traveler.
When I became ill with PLD, Polycystic Liver Disease, I could no longer travel. I could no longer enjoy and delight in the discovery of new and interesting foods. I was unable to breathe easily. I could only walk a half a block before becoming breathless, I became so tired unable to go on. If I ate more than a half a cupful of food, the undigested food would pop out of my mouth without warning, without nausea, without being absorbed. The pains and symptoms from my huge cystic liver were becoming unbearable. The tiredness I felt from carrying around 40 extra pounds of organs was exhausting and led to back pain and many of my internal organs being pushed through any available orifice. I had hemorrhoids, back pain, uterine prolapse, varicose veins, bruising and bleeding. I was becoming emaciated, malnourished, with a very skinny upper body and a huge belly with fluid filled legs. I had to do something.
Researchers say they encounter many problems in making the jump from PKD animal model experiments to even thinking about attempts to begin human dietary clinical trials with the alkaline diet. I can understand. The connecting bridge between these studies is oftentimes very wide and difficult to traverse. I am continually jumping to bridge this gap. We with PKD are hopeful that diet clinical trials will become a reality.
I have been on the PKDiet, a plant based diet, an alkaline diet geared toward kidney and liver health, for 40 + years. The formulation of a PKD diet and lifestyle is ever evolving, ever changing. New foods are tried and a reaction can be observed from within a body with polycystic organs. Plus there has been additional feedback from others with polycystic organs along with their own observations.
PKD Diet began as a vegetarian diet. It changed to meat and vegetables; changed again to vegetables coupled with whole grains. This catapulted another search to find different cooking practices to heighten absorption of natural complete whole foods. By soaking grains, legumes, beans, seeds, and nuts, their phytic acid content was diminished, nutrients were enhanced. PKD Diet changed eventually incorporating large quantities of raw vegetables and fruits, sometimes quick steaming to intensify digestibility. This resulted in a slow steady evaluation of cooking and non-cooking methods to improve pure food flavors that strengthen alkalinity. Among the many valuable techniques were those, that sought to avoid the production of acid ferments, alcohols, and vinegars.These were replaced by fermentations that promoted lactic acid. Lactic acid is easily neutralized by the breath from the lungs without further increasing the workload upon the kidneys. Each morning upon awakening, I try (8) eight full deep breathes to blow off any residual lactic acid.
Clearly the greatest changes have been since 1994 when my liver disease manifested aggressively. My current diet has been taken from different bits and parts of past diets as I continually change making note of the current affect upon my polycystic body, kidneys, and live. The results from this experiment of one are conclusive. It has made a difference, a real difference. I am alive today in an amazingly healthy body with excellent kidney functioning, a strong vital liver, my own native organs and I feel great with ADPKD; with chronic kidney disease; with severe PLD. I will always have ADPKD.
I do indeed have kidney cysts, however with a greater number of liver cysts, my liver has given me the most difficulty. Throughout my 65 years, my body has gone through many shapes from being a full figured lady, to looking like I was 9 months pregnant, to a malnourished individual who looked like she just stepped out of a concentration camp, to a healthy vibrant body that I am very proud and more than happy to display in a slinky dress.
I understand that my family history can be very confusing.
Both parents had PKD. Both died from end stage renal disease. And each parent helped pioneer a medical treatment breakthrough.
My Mother was among the first cadaver kidney transplants in the USA. She was the first peritoneal dialysis patient at our local hospital. The doctors were communicating with other doctors via telephone on how to perform peritoneal dialysis. None of the doctors had ever done this before. This was an era way before cell phones, before the internet. There were very few dialysis centers with selection done through lottery. It was before medicare assisted with kidney services.
My Dad was 3 pack a day Camel cigarette smoker. At age 50 he got lung cancer. Doctors from the City of Hope gave him a cure by doing an extremely radical surgery for the times. He was in a heart-lung machine for a few days immediately following this experimental extensive lung surgery. The surgeons attempted to remove as much of both lungs as was feasible and still give him the possibility for continued life. Only a tiny fragment of lung tissue remained attached to his heart. He was able to survive lung cancer through this new cancer treatment. However at 74, PKD had the last say. His kidneys stopped functioning. Long term dialysis was not an option. There were very few dialysis machines in those early days and even fewer transplants were available.
Nearly all my relatives with polycystic kidneys had major events occurring around age 42 from kidney failure, nephrectomies, all ending in kidney Nearly all my relatives with polycystic kidneys had major events occurring around age 42: from kidney failure, nephrectomies, coma, all ending in kidney disease death. My oldest sister died at 42 from PKD and severe PLD. My Mother died at 42. My Father's sister had a nephrectomy at age 42. My Father, born in 1893, lived until 74 when he died from polycystic kidney failure. My youngest sister died at 32 with invasive intraductal breast cancer; she also had both PKD and PLD. I am the only surviving living member of my family with ADPKD. I realize how very lucky I have been to have retained excellent kidney functioning, very good liver functioning without the need for blood pressure lowering medications. According to my doctors, In all likelihood, I will probably never ever need dialysis or a transplant.
My manifestation of this disorder has been an aggressive form of liver cysts which may in some ways, resemble the childhood form of Autosomal Recessive Polycystic Kidney Disease. I have not had the hard hitting kidney disease of my family. No family member has ever developed huge cystic kidneys; all went on to end stage renal disease.
I have been encouraged by my positive lifestyle results so much so that I have wanted to shout to the world that now I have discovered one answer to the question–
I Have Polycystic Kidney Disease, What Can I Do to Help Myself?
Accolades go to my diet, lifestyle, and perhaps the luck of the genes that allows me to remain healthy. In the far distant past, each time I had occasion to speak with doctors, internists, nephrologists, I would ask this same question:
"What can I do?"
The doctors each gave similar responses
"There is nothing to do. You are too healthy. We can provide help when you become ill. Then we have plenty to call upon to help you. We can then treat your high blood pressure. We can then put you on dialysis. We can then prepare you for an organ transplant".
I did not want to wait until my blood pressure started climbing or until my kidneys started failing. My Irish stubbornness refused to allow me to sit by and watch my cystic liver steadily grow. I began experimenting; first with diet beginning in my early twenty's. Now with 40 years of dietary modification, choosing foods that diminish pain, cysts, and symptoms from cystic organ disease, I am one example of what might be possible when someone with Polycystic Kidney and Liver Disease eats a modified alkaline plant based diet, the PKD Diet.
With the clarity of imaging studies today, polycystic kidneys and polycystic liver may be discovered long before young individuals have emerged from the womb. There is a growing body of people much much younger than previous generations with polycystic kidneys and polycystic livers. This current generation with Cystic Organ Disease are more curious and better informed in that their cysts have been discovered at a much younger age. This is a song of courage to each and every one of you who would envision hope that you can live your life fully in health or that there is the possibility of life without dialysis or transplant.
Some fifteen years ago I became extremely ill from my liver cysts. These thoughts may have crossed your mind:
"If she knew so much about diet, why in the world did her liver get so sick? "
It was partly due to vanity. I received an invitation to my 30th high school reunion. I decided to work out twice daily doing aerobic exercises. To my thinking an organic juice fast would easily get me trim and lean. For three months I drank nothing but commercially bottled organic juices. My liver began growing like crazy. This was the fastest growth spurt I have ever experienced with my liver. I have since discovered why this occurred. All bottled juices sit a moment or two before while they are heated to destroy bacteria. This sitting allows a sort of a yeast fermentation to begin, much like the yeast formation that comes about from making wine or ale. I was ingesting a form of an alcohol ferment repeatedly several times a day.
Woman, thy name is indeed vanity! In addition to consuming organic bottled juices for three months, I also purchased a corset that could be cinched very very tightly so I could wear a form fitting dress which gave the impression that I had a tiny waist. Unfortunately this cinching caused one of my liver cysts to burst.
It was at this time that I found Dr. Jared Grantham (University of Kansas) and Dr. Vicente Torres (Mayo Clinic in Rochester, MN), two fine gentlemen from the USA, who have an easy manner and vast amounts of knowledge and experience to call upon when someone needs help with polycystic organ disease. Dr. Torres recommended liver surgery, but I did not want to listen. I still wanted to try to correct the situation myself, try some alternative treatments. I ate a purely vegetarian diet and for the first time I began testing my urinary pH. I thought that a vegetarian alkaline diet would be best. I read a few medical papers and started testing in earnest. The alarming rate at which my liver had been growing decreased, in fact there were times when it shrank back a little, but gradually over the next four years my liver grew and grew and grew. Coincidental to my enlarging liver, there was a sharp decline in kidney functioning. My health deteriorated badly. I could not halt or reverse the process begun by the bottled juice fast. By 1996 it became imperative that I have surgery.
A massive effort from all sources was required to get through the liver surgery and to bring myself once again to health. The doctors will tell you over and over again that a cystic liver will rarely fail. Liver enzyme studies will become a bit wonky in only extreme cases such as mine. I had ¾ of my liver removed, my gallbladder removed, my ovaries and uterus removed. Thirty-nine pounds of organs were removed in one fell swoop. The surgery took 8 hours, with a team of 5 doctors from the Mayo Clinic in Rochester, Minnesota. I will always remember being wheeled back to my room, looking down at my belly which was now concave. It was not only flat, it was going inward, like a muffin tin before it is filled. For the first time in several years I inhaled deeply filling my partially collapsed lungs with a full luxurious breath of air. I was famished, a first for me for several years. It took two nurses to bring into my hospital room all the trays of food I had ordered. I was so happy to be able to eat and take a deep full breath once again. I really missed doing those things.
The next morning I got out of bed and took a few short walks with a hospital walker that held my intravenous solution. There was no pain from the surgery, none. Wonderful! The fabulous anesthesia department had placed an indwelling catheter into my spine. I was numb all over my belly. This was very good, for I was unable to tolerate any pain medication. Medications seemed to give me wave after wave of nausea, followed by the dry heaves. As I took my walks along the hospital corridor, I thought of myself as elegantly gliding along. My husband told me later, that I could barely hold my wobbly head upright, but I looked so incredibly happy. I had pictured myself recovering from this surgery in two weeks time, tops. I had a much longer recovery time.
I was shocked to discover that following my liver resection I developed a very rare complication called Budd-Chiari syndrome or Hepatic Venous Outlet Obstruction, HVOO. Following the removal of my liver drains, my belly filled with fluid and I had to have repeat belly taps to drain off two liters of fluid every few weeks. Each liter of liver cyst fluid was filled with about 40 grams of protein. Both lungs also filled with fluid. I remember watching an imaging study of my lung as it was flipping rapidly in a fluid filled bath. I felt so sorry for my body. It seemed to be putting out the maximum effort it possibly could to keep me breathing. The surgeon was prepared to proceed with a second surgery to correct the situation. There were some risks. I had been referred to a liver transplant doctor (hepatologist). I had only one remaining hepatic liver vein. If during this second surgery, this one vein became scarred, than I would require an emergency liver transplant. I would go to the top of the transplant list immediately. Unlike with the kidneys where dialysis is a possibility, with the liver, at this time, this was not possible.(since then I have read, doctors have developed a liver dialysis machine).
My one liver vein was becoming more and more obstructed by a cystic section within my liver remnant. This portion of the liver had several tiny champagne-grape-size clusters of cysts contained within it. These cysts surrounded the vein and continually pressed and squeezed against it, blocking the blood from flowing freely. The vein tried very hard to move the blood along but what was needed was a new network of smaller veins channeling the blood flow around this obstruction. Working steadily with my doctors, I utilized all the ideas and energy I could to help propel me through this exercise, diet, medications, treatments and a few well chosen herbs and foods. I coaxed my skinny muscle-wasted body to build new blood vessels around this crimp in the vein.
I was so very weak, I could not think clearly. I sought to minimize the amount of energy I put out to make my needs known. My thoughts went to newly born babies and how rough they have it. They can only cry or laugh to let their needs known. I had speech, movement, writing, all sorts of tools available, but at one point all I could do was cry. I was so uncomfortable. I felt helpless, and worse of all, I felt useless. I pulled my energy inward. Weak and fragile as I was, I knew somehow this was what was required so I would heal. I slept continuously.
I used all that was available to me. I climbed onto a machine called the stairmaster. At the beginning I was only able to use the stairmaster barely for three minutes. My body was totally draped over the machine for support. I had not even enough muscle strength left to swallow medication. I could barely hold my own head upright. I pumped the calf muscles of my lower legs, assisting the blood to return to my heart. By doing this I push-started the circulating blood to increase its pace. Exercising my calf muscles caused my pulse to quicken giving additional counter pressure to speed the blood flow forcing it past the obstruction.
My remaining ¼ liver remnant had not yet begun to fully function. My liver was unable to store any nutrients to be used at a later time. What I ate in the moment was immediately taken up by the body. Later when I wanted to stretch or walk, there were no reserves for the body to pull on. The body started depleting my protein stores for energy ( my fat stores were long gone) . It took from the butt and thighs. Within two weeks time, I had gone from thunder thighs (due to heavy edema and swelling pre-surgery) to scrawny chicken legs. I was a walking skeleton, looking as if I had just stepped out from a WWII concentration camp.
My trust never wavered. I knew I would make it. Even as I was hit with wave after wave of nausea followed by daily vomiting for 6 months. This was hardest on my beloved husband. He would try to leave food for me, but the nausea was so overwhelming eating just was not possible. Nourishment became difficult.
I spoke with my doctors almost daily. They were all an unbelievable support for me. I could never have done it without each and every one of them. The doctors started hyperalimentation. This was given via an intravenous catheter threaded through a vein near my clavicle in my chest. My arm veins had gotten so tiny, it was not possible to use these veins even for the daily drawing of blood samples. Continuous nourishment in the form of fats, proteins, carbohydrates, vitamins, and electrolytes were pumped into me. I was allowed carte blanche to eat any food I desired. This was the only way to effect a weight gain until my remaining liver remnant could begin working once again. My weight was 87 pounds and dropping. I became too frightened to get on the scale. Three days later my potassium shot up to 8.5. I was taken to the emergency room and admitted to the intensive care. I had barely recovered from this when the Budd-Chiari hit full on. That’s when I got very serious about my health. I knew I did not want a liver transplant.
My husband purchased a special chair for me called the zero gravity chair. It tipped me upward so my feet were elevated above the level of my heart. Then several different buttons could be pressed giving the body varying types of gentle massage. The chair also heated my weary muscles. It was a delight. I forced myself to walk as frequently as I could. This was only for five minutes within my home. I had physical therapy come over to aid me in exercising. They brought some rocker shoes for me. These stretch the Achilles tendon, the biceps tendon and the hamstring of the lower legs, forcing the thigh muscle to fire so that there is less thigh muscle wasting. This exercise works for painful and weakened knees by rebuilding the support muscles. I was so debilitated that I developed a bed sore, a pressure ulcer. I required help to take a shower each morning. I did not have the strength to place the wet washed clothes into the dryer. They were way too heavy. I was totally lacking in energy. My blood sugar would drop and I would get the shakes. For the shakes I found freshly pressed carrot juice best, even better than orange juice. My remaining liver remnant had not yet begun to store glucagon.
I continued all my treatments. Some close friends have affectionately called me the sweet mule. Stubbornness is a trait I strongly possess. When needed, this stubbornness can be transformed into perseverance. I become like the little Chihuahua that will not let go until the situation is remedied. Daily I utilized saffron tea, fresh cabbage juice with almonds, milk thistle, organic turmeric, artichoke, iron, B complex, B6, B12, folic acid, and rose hips vitamin C. I used cod liver oil compresses externally and the outer leaves of green cabbage plant made into compresses for my liver. The result was overwhelmingly positive. When the date came about for my second surgery, I no longer needed it.
My surgeon, Dr. Nagorney, (has golden hands when it comes to operating on a highly vascular polycystic liver) had written a paper reviewing patients who developed this rare Budd-Chiari complication. There were only ten examples of such patients from around the world. Of the ten, 8 died. Two others required creative shunting procedures done by my surgeon. They are both alive today. I am the only patient he has ever heard of who spontaneously resolved the situation. The doctors were ecstatic! I was successful in getting my blood vessels to lay down new pipeways to transport blood around the blocked vessel. A year later, the radiologist was kind enough to show me on my MRI that the body had enlarged certain vessels and had made new vessels around the obstructed vein. Here is Dr. Nagorney's long term follow up paper on The Mayo Experience with Liver Resections for PLD Polycystic Liver Disease.
Both my kidney cysts and liver cysts are totally stable and have remained so for fifteen years. There has not been any cyst growth since the surgery. My rib cage is permanently deformed from the huge liver I once had. My right lung remains partially collapsed. But for my surgical scar, (which is barely visible in a bikini), one would never know I was snatched from death's door just in the nick of time. I overcame a life threatening illness. I am now absolutely healthy. I am the jump to human clinical trials with alkalinity. I am living proof that long term use of the alkaline PKD Diet works. I took the jump, and I have made it!
More. . .
Genetic testing revealed that I have PKD1, the more aggressive inherited gene. By all standards severe manifestations from PKD should have emerged by the age of 50. Instead at age 50 my liver cysts grew rapidly. I had a liver resection, had my ovaries and uterus removed (for other reasons) and began to lose both liver and kidney functioning. I will always have severe polycystic liver disease. My liver is not normal size. It will never be a small tucked under the ribs liver. It is a hard huge polycystic liver. Rarely liver functioning becomes compromised with PLD, but mine did. It was a struggle to get my kidney functioning to return to the best it has ever been in my entire life. At 63, my serum creatinine is 0.8. My GFR iothalmate is 69. Both these values have improved over the past 3 years. These improvements occurred before beginning octreotide. My liver functioning has improved and is now totally normal. This is a celebration. Genetic PKD researchers have now asked me to participate in a genetic study to see if I have a modification in one of my alleles that has succeeded in changing my PKD prognosis for the better.
Over the years, my doctors have told me that I should likely well out live my polycystic kidneys. I do not know if anyone has improved their kidney functioning as they age. My doctors know of no one. I do know of a few who are no longer taking blood pressure medication when switching to a modified PKD Diet. Each of us has changed the PKD diet to suit them while sticking with a few basic principals:
Cooking your own food
Limiting salt and animal protein
It has been 15 years since my liver resection and my liver cysts have not re-grown. I continue to restrict caffeine, animal products, estrogens, false estrogens, xenoestrogens and estrogen disruptors; rarely experience pains within my cystic liver remnant; and I am in my fifth year of an octreotide clinical trial, a long acting experimental drug that decreases kidney cysts but especially liver cysts. TFor the first time I have experienced a shrinkage of my liver remnant. I am hopeful that octreotide LAR will soon be open to many as a treatment option.
How are my kidney cysts doing? Their function is improving as I age - Unheard of with PKD Polycystic Kidney Disease. This phenomenon has had researchers attempt to see if there is possibly a gene at play or is this phenomenon related to lifestyle? I do not require blood pressure medication to keep my normal blood pressure in the range of 110/70 - 120/80 range. Lipid values are great. I do restrict salt using a very tiny amount of Himalayan Crystal Salt in my baking, less than 1/8 of a teaspoon per day; still on a neutral protein diet calculated at 0.6 grams of protein per kilogram of body weight. For me this is about 30 grams of protein daily.
I try to drink about 3 liters of water daily adding one drop of solé to a full glass of mineral water each morning.
Together, this has been a life changing experience. I have often thought if my liver did not grow so extensively and so quickly that it forced my attention to it, I would be less healthy today. I think if one cares for the liver, the kidneys will follow.
I live near the ocean and walk to the gym each morning for some gentle exercise; then sit down to a breakfast of locally grown fruits, 5 roasted almonds and a cup of roasted grain beverage. Lunch is usually an all greens salad or soup. Several snacks throughout the day consist of locally grown fruits and vegetables. Dinner is usually root vegetables roasted, steamed, or sautéed, with leafy greens, sometimes spelt, rye, barley or corn grains, without yeast. I continually graze all day, snacking in between meals.
While visiting California Wine Country: Napa, Sonoma, and Mendocino, Paso Robles, Santa Barbara and Santa Cruz Mountains; I tasted local wines, spitting out the wine in a spitoon provided by the company. It was a second honeymoon for my husband, one we both enjoyed. After seeing the movie Bottle Shock, I had to sample these wines that beat out the French wines in a blind tasting not once (1976) but twice (again in 2006). I tried to not swallow any, but some was just so very delicious. I am especially fond of Navarro Vineyard Gewürztraminer grape juice. It has a taste similar to a fine French Sauterne–Chateaux d'Yquem. A favorite vegetarian restaurant in Napa is Ubuntu and in San Francisco, Greens. My husband enjoys gourmet eating, matching fine wine to game, fish, and now vegetables. I am vegan. We complement one another. Vive la différence!