Edema: A build up of fluid in the legs. So far Ashley doesn't have Edema.
Fatigue: Ashley does complain of being sleepy more often then not but we attribute it more to her active dance schedule and not her liver disease. Ashley does require more sleep then most 10 year olds.
Jaundice: Yellowing of the skin and eyes. Ashley is not jaundice but this is something we continue to watch as she has been severely jaundice in the past.
Epistaxis: Profuse nose bleeds. Ashley has never had a nose bleed.
Reddness of Palms: At Ashley's list visit to Shands Liver Clinic in August, it was noted that Ashley's palms are red.
Spider Angiomas (Spider veins): Small red spots and tiny lines on the skin. Ashley does have a few spider veins on her face and on her stomach.
Portal Hypertension: This is caused when scar tissue blocks the proper flow of blood from the intestines through the liver, leading to increased pressure in the veins. Ashley has had Portal Hypertension since her diagnosis and it has progressively gotten worse.
Ascites: A build up of fluid in the abdomen. Ascites is the most common complication of portal hypertension caused by cirrhosis. Ashley has mild ascites but is not on medication to control the fluid. Because of the mild ascites Ashley has frequent ultrasounds.
Varcies: Bleeding from enlarged veins in the digestive tract (stomach, esphogus, etc). Variceal bleeding, especially in the esophagus and stomach, is a major cause of illness and death in people who have cirrhosis. Ashley had very small varcies that have recently disappeared. Due to Ashley's risk of developing varcies, she gets an endoscopy done once a year as needed.
Splenomegaly: Increased spleen size. This can lead to a low blood platelet count. Ashley has a very large spleen, it measures approximately 16 cm which is the same size as a healthy 20 year old. Ashley's platelet count is also low.
Spontaneous Bacterial Perionitis or SBP: Infection of the fluid in the belly (SBP develops in 10% to 25% of people who have advanced cirrhosis and ascites. Ashley does not have SBP.
Encephalopathy: Altered brain function. Encephalopathy usually only occurs in people who have advanced portal hypertension. But having a procedure to help reduce portal hypertension (shunting) can increase the risk of developing encephalopathy. Thankfully, Ashley's portal hypertension is not advance therefore she does not have Encephalopathy.
Hepatorenal syndrome: Kidney (renal) failure can occur in cases of advanced liver disease stemming from cirrhosis. Usually, liver failure is the condition that threatens a person's life. But in some cases the liver disease may be stable, while kidney problems are life-threatening. A liver transplant may be necessary to cure renal failure caused by cirrhosis. So far Ashley's Kidney's are healthy despite her left kidney being completely flattened by her enlarged spleen.
Hepatopulmonary syndrome. Portal hypertension caused by cirrhosis can cause lung (pulmonary) problems, such as widening of the blood vessels in the lungs. This widening causes the blood to move too swiftly through the lungs to pick up enough oxygen. Liver transplantation is the most effective treatment for this condition. Thankfully, Ashley does not have this syndrome but it is something we watch due to Ashley's portal hypertension.
Hepatic hydrothorax. Cirrhosis can cause fluid to build up between the lungs and the chest (pleural effusion) and press on the lungs. Treatment can include taking medicines such as diuretics, restricting salt in the diet, and using procedures to remove the fluid. Ashley does not have Hepatic hydrothorax.
People who have cirrhosis may be at increased risk for developing gallstones.3 The more severe your liver disease, the higher your risk of developing gallstones. The chances of Ashley developing gallstones were removed at birth due to the surgical removal of her of gall bladder. However, Ashley can develop kidney stones due to her liver disease.
People who have cirrhosis also are at increased risk of developing liver cancer, mainly hepatocellular carcinoma. Ashley developing cancer is higher then most patients with liver disease because Ashley was born with a precancerous cyst called a choledochal cyst (removed at birth) and because Ashley has 3 unknown cysts/masses in or near her liver. Because of Ashley's increased risk to cancer and her cysts, she has a tumor market test done (through blood work) every 4 months and an MRI or CT scan once or twice a year.
Out of 17 possible complications of end stage liver disease, Ashley only shows signs of 6 of those complications. Her symptoms are manageable which is all we can do right now until she is eligible to be transplanted.
Not mentioned above is a low white blood cell count. Ashley has a low white blood cell count which means she is more susceptible to illnesses/infections. On average we spend at least one day a week at the pediatricians office. Sometimes we can find the source of her illness or infection and medicine is prescribed, other times her illness becomes a mystery that we monitor for more serious symptoms. Ashley is a pro at having blood work, throat cultures, MRI's, CT scans, scopes, etc.
Ashley's other medical complications are joint and back pain, severe reflux and mild inattentive ADD. Ashley is on medication for the joint/back pain and the reflux but takes no medications for her ADD due to the adverse effects of the ADD medications on the liver.
With liver disease, it's truly a waiting game. On the one hand I want Ashley to live forever with her liver disease, on the other hand I don't want to watch her life deteriorate because her liver disease is progressing ever so slowly. For Ashley to get transplanted now she would need to get sicker quicker, but I don't want that either. I am scared to death of a liver transplant but I am also scared to death of watching Ashley slip away because her disease is zapping all the life out of her.
My hands are tied, it doesn't matter what I want for Ashley; her life is in the hands of her doctors. They dictate whether Ashley is eligible for a transplant, they dictate how she is to be treated, what tests are needed, what medications she needs and they even dictate how active Ashley can be. However, as her parents, Joey and I try very hard to give her the most normal life possible.
Fatigue: Ashley does complain of being sleepy more often then not but we attribute it more to her active dance schedule and not her liver disease. Ashley does require more sleep then most 10 year olds.
Jaundice: Yellowing of the skin and eyes. Ashley is not jaundice but this is something we continue to watch as she has been severely jaundice in the past.
Epistaxis: Profuse nose bleeds. Ashley has never had a nose bleed.
Reddness of Palms: At Ashley's list visit to Shands Liver Clinic in August, it was noted that Ashley's palms are red.
Spider Angiomas (Spider veins): Small red spots and tiny lines on the skin. Ashley does have a few spider veins on her face and on her stomach.
Portal Hypertension: This is caused when scar tissue blocks the proper flow of blood from the intestines through the liver, leading to increased pressure in the veins. Ashley has had Portal Hypertension since her diagnosis and it has progressively gotten worse.
Ascites: A build up of fluid in the abdomen. Ascites is the most common complication of portal hypertension caused by cirrhosis. Ashley has mild ascites but is not on medication to control the fluid. Because of the mild ascites Ashley has frequent ultrasounds.
Varcies: Bleeding from enlarged veins in the digestive tract (stomach, esphogus, etc). Variceal bleeding, especially in the esophagus and stomach, is a major cause of illness and death in people who have cirrhosis. Ashley had very small varcies that have recently disappeared. Due to Ashley's risk of developing varcies, she gets an endoscopy done once a year as needed.
Splenomegaly: Increased spleen size. This can lead to a low blood platelet count. Ashley has a very large spleen, it measures approximately 16 cm which is the same size as a healthy 20 year old. Ashley's platelet count is also low.
Spontaneous Bacterial Perionitis or SBP: Infection of the fluid in the belly (SBP develops in 10% to 25% of people who have advanced cirrhosis and ascites. Ashley does not have SBP.
Encephalopathy: Altered brain function. Encephalopathy usually only occurs in people who have advanced portal hypertension. But having a procedure to help reduce portal hypertension (shunting) can increase the risk of developing encephalopathy. Thankfully, Ashley's portal hypertension is not advance therefore she does not have Encephalopathy.
Hepatorenal syndrome: Kidney (renal) failure can occur in cases of advanced liver disease stemming from cirrhosis. Usually, liver failure is the condition that threatens a person's life. But in some cases the liver disease may be stable, while kidney problems are life-threatening. A liver transplant may be necessary to cure renal failure caused by cirrhosis. So far Ashley's Kidney's are healthy despite her left kidney being completely flattened by her enlarged spleen.
Hepatopulmonary syndrome. Portal hypertension caused by cirrhosis can cause lung (pulmonary) problems, such as widening of the blood vessels in the lungs. This widening causes the blood to move too swiftly through the lungs to pick up enough oxygen. Liver transplantation is the most effective treatment for this condition. Thankfully, Ashley does not have this syndrome but it is something we watch due to Ashley's portal hypertension.
Hepatic hydrothorax. Cirrhosis can cause fluid to build up between the lungs and the chest (pleural effusion) and press on the lungs. Treatment can include taking medicines such as diuretics, restricting salt in the diet, and using procedures to remove the fluid. Ashley does not have Hepatic hydrothorax.
People who have cirrhosis may be at increased risk for developing gallstones.3 The more severe your liver disease, the higher your risk of developing gallstones. The chances of Ashley developing gallstones were removed at birth due to the surgical removal of her of gall bladder. However, Ashley can develop kidney stones due to her liver disease.
People who have cirrhosis also are at increased risk of developing liver cancer, mainly hepatocellular carcinoma. Ashley developing cancer is higher then most patients with liver disease because Ashley was born with a precancerous cyst called a choledochal cyst (removed at birth) and because Ashley has 3 unknown cysts/masses in or near her liver. Because of Ashley's increased risk to cancer and her cysts, she has a tumor market test done (through blood work) every 4 months and an MRI or CT scan once or twice a year.
Out of 17 possible complications of end stage liver disease, Ashley only shows signs of 6 of those complications. Her symptoms are manageable which is all we can do right now until she is eligible to be transplanted.
Not mentioned above is a low white blood cell count. Ashley has a low white blood cell count which means she is more susceptible to illnesses/infections. On average we spend at least one day a week at the pediatricians office. Sometimes we can find the source of her illness or infection and medicine is prescribed, other times her illness becomes a mystery that we monitor for more serious symptoms. Ashley is a pro at having blood work, throat cultures, MRI's, CT scans, scopes, etc.
Ashley's other medical complications are joint and back pain, severe reflux and mild inattentive ADD. Ashley is on medication for the joint/back pain and the reflux but takes no medications for her ADD due to the adverse effects of the ADD medications on the liver.
With liver disease, it's truly a waiting game. On the one hand I want Ashley to live forever with her liver disease, on the other hand I don't want to watch her life deteriorate because her liver disease is progressing ever so slowly. For Ashley to get transplanted now she would need to get sicker quicker, but I don't want that either. I am scared to death of a liver transplant but I am also scared to death of watching Ashley slip away because her disease is zapping all the life out of her.
My hands are tied, it doesn't matter what I want for Ashley; her life is in the hands of her doctors. They dictate whether Ashley is eligible for a transplant, they dictate how she is to be treated, what tests are needed, what medications she needs and they even dictate how active Ashley can be. However, as her parents, Joey and I try very hard to give her the most normal life possible.
Comments
For Ashley and Laurie,without trying to give you false hope, there are ways to improve liver's capacity to heal and regenerate. While continuing to see your doctor is the correct option, do not think for a moment that it is the only one! Please, try alternative ways of healing: acupuncture, chinese medicine, herbal medicine. They treat this illness from a systemic point of view, unlike western medicine. What is good for another liver sufferer may not be as good for Ashley. Do not dismiss these options, please. Seeing two types of doctors can only improve her chances to live and have a better life.
I'm 33 now and I almost died of an aggressive form of hep B when I was 6. Doctors had given up all hope in my case, and mom eventually decided to move into the hospital with me, after which the treatment miracuouly began to work. Nobody knew why- there was no rational explanation for why I had begun to heal and eat again. Your love for Ashley can be a healer in itself, and I hope from all my heart that she gets better!
Best wishes and love,
maya
I would also try a bee polen treatment: it is an unbelievably powerful natural remedy for many conditions, including the liver illness (it helps regenerate the hepatic cell). Ask your doctor if there are valid reasons for why she shouldn,t try it, and if he/she agrees, go for it. I,m only concerned about possible allergies, but don,t rely on your doctor's enthusiasm when it comes to bee polen- all you need is the approval, that's all. Most of them have no clue about what it can do in terms of helping the immune system.
yours,
maya