Cookies help us deliver our services. By using our services, you agree to our use of cookies. Learn more

Primary Intestinal Lymphangiectasia

What is Primary Intestinal Lymphangiectasia?

Primary Intestinal Lymphangiectasia is a rare disorder causing protein to be lost from the intestines due to enlarged lymph vessels that supply the lining of the small intestine.

 

Primary Intestinal Lymphangiectasia is a rare disorder causing protein to be lost from the intestines due to enlarged lymph vessels that supply the lining of the small intestine.
Acknowledgement of Primary Intestinal Lymphangiectasia has not been added yet.
Prevalence Information of Primary Intestinal Lymphangiectasia has not been added yet.
Synonyms for Primary Intestinal Lymphangiectasia has not been added yet.
Cause of Primary Intestinal Lymphangiectasia has not been added yet.
abdominal pain, swelling in lower extremeties.
Diagnosis of Primary Intestinal Lymphangiectasia has not been added yet.
Diagnostic tests of Primary Intestinal Lymphangiectasia has not been added yet
Low fat- MCT diet.
Prognosis of Primary Intestinal Lymphangiectasia has not been added yet.
Tips or Suggestions of Primary Intestinal Lymphangiectasia has not been added yet.
References of Primary Intestinal Lymphangiectasia has not been added yet.
Your current therapy Created by Magaliep
Last updated 20 Jun 2016, 07:24 AM

Posted by Magaliep
20 Jun 2016, 07:24 AM

Hello! I would like to know what other fellow people with PIL are currently doing to improve their lifestyle: - Supplementary vitamins -Protein and calorie supplements -Medication Dietary changes besides low fat Im currently on 2x 20% albumin + 1 injection of sandostatin every 4 weeks. I get a subcutaneous vitamin D3 injection in the belly. I take multivitamin zinc tablets aswell as calcium And vitamin K. I use the Ceres MCT 77% OIL I drink isostar protein shakes twice a day and I have started drinking ensure plus twocal. this is 200 ml , high energy, high protein, low fat nutritional drink, designed for people with involuntary weight loss. Since I was young I heavily relied on carbs to gain weight such as bread and pasta which recently made my insulin Levels really high so now Im trying out a gluten free diet. Despite my efforts my weight never stays the same I can go from 60kg to 49kg in a matter of weeks. But I'm definitely doing much better now with this routine than I ever have before.

PIL Created by micrich
Last updated 7 Jan 2014, 12:47 AM

Posted by micrich
7 Jan 2014, 12:47 AM

My daughter has been diagnosed with PIL. It took 13 yrs for us to track down the exact cause of what she has as it has in what I read in previous posts. She had to take the pill with a camera on it to find it. I have looked in many places for others that may have this and really have come up short. I know the one thing that we have done is to use cocnut oil on her food. Like when I stirfry some chicken i will use the oil in that. We also use it as a substitute in baking...tastes good. I also have found a product called Melt. It is coconut oil based and is like butter. They have many different kinds like a veggie spread, a chocolate spread similar to nutella...I have only found the butter type. I found it at whole foods. I believe you can order from their website for the other types. My daughter continues to have pain in her abdomen and swelling especially in her legs. She has had alot of boils and infections do not like her. She had braces for 4 yrs and her gums stayed swelled because of the irritation. There is alot more I will add eventually. We go to the G.I. dr tomorrow and hope to find out more. Its amazing that there is still so little known about this disease. Hope to find more people that struggle with this and hopefully be able to help them too. The more we are finding out about wheat and processed foods the better. I believe our food system has alot to do with these disorders. we are also doing a wheat free dairy free diet along with the low fat MCT diet. As soon as we get back from he doc tomorrow I will post the answers I get from her on here. Hope to help!!!! TTYL!! :)

Toddler with PIL Created by MaryG
Last updated 10 Aug 2013, 02:37 PM

Posted by MaryG
10 Aug 2013, 02:37 PM

Good morning. I came across this web sight hoping to see any new research on PIL. My 3 year old daughter was diagnosed at 9 months old and its been so hard as she as other siblings also. She has been on Monogen since. Her albumin levels were 2.7 I believe in Feb. she had oral surgery and had to have a cpl teeth removed and a few root canals be sues her teeth developed without the enamel. She is very swollen and her belly swollen. We try and stick to her low fat diet as much as possible. She was diagnosed with a sort of ADHD also. Her energy levels are extremely high and gets very aggressive. Daily! We do not know if this is due to the formula she is on or what. She also has some developmental delays and thankful will start school this year because of her special needs. This has been such a struggle for us. I am 41 years old and have a 9 year old daughter at home and also have recently welcomed a 6 year old foster child into our home per emergency cps reasons. Our hands where very full with baby Mary already but could not close our doors to this little one. Mary (our 3 yr old) is huge and I believe all muscle and strong. Weighs a good 54 lbs and almost 4 foot tall now. And that was in Feb. I have not found any recent info on PIL and pediatricians here really do not know much about it. Her legs had a weird swelling almost hollow look to both of them and looked inflamed a cpl months ago and I worried. I appreciate any info you can provide for me. Thank you Any low fat high protein desert recipes would be appreciated too=)

Primary Intestinal Lymphangiectasia Patient Created by Monica1965Gast
Last updated 3 May 2011, 01:30 AM

Posted by jensy1109
3 May 2011, 01:30 AM

Hi My name is Jennifer My four yr old son has P I L. we live in NY and he has amazing md s in NYC. I have lots of info. Please contact me Jensy1109@Verizon.net I am also a Physical therapist who specializes in lymphedema. There are things you can do to decrease leg n abdomen size. I look forward to hearing from you. Jen

Posted by Monica1965Gast
25 Oct 2010, 12:58 PM

To Whom It May Concern, My name is Monica Gast. I am a fourty-five year old woman. I have four children who are all in their 20's now. I am married to a wonderful man named William. (His nickname is Radar.) I ran into your your website by accident, I was doing some research on Primary Intestinal Lymphangiectasia. Because in July 2010 I started having a major episode with my PIL. When I was born the doctors had to drain out lymphatic fluid from my abdomen, because I had so much fluid in my belly. I believe I was told about 10 to 16 ounces that was drained from my stomach. I was underweight. I was just extremely ill. Doctor's told my parents that they didn't think I was going to live long. My parents even had our priest come and give me my last rights at a day old, because they feared that I wasn't going survive. I needed to be in an incubator for over a month. No types of formulas agreed with my system. Doctor's didn't know what to do for me. When I was two years old my parents to me to Shriner's Hospital in Chicago, because of my symtoms where so out of control. Some of my symtoms were: very under weight, swollen extended belly, diahrrea, nausea, vomiting, immunity problems, and developmentally behind in mobility (I didn't walk till I was 3 years old). Needless to say, doctor's at Shriner's Hospital in Chicago didn't know what to do for me. It was the year of 1967 and lymphangiectasia was a realtively new disease, that was discovered in 1961. That meant doctor's didn't have the information of PIL at this time. In 1972 my parents took my to Children's Hospital in Chigaco, because our primary phyician referred me. This is when I was diagnosed with Primary Intestinal Lymphangiectasia. I was seven years old. Doctor's explained to my parents that I was actually born with the condition PIL and malabsorption syndrom. At that time, there was nothing doctors did for PIL, except the Low Fat High Protein diet. This is also the time when my left foot and leg started to hold more lymphatic fluid and swell uncontrolably. I also had a growth that looked like a wart on the side of my right foot that popped open and was leaking. Come to find out, with tests that were done, it was actually a lymph vessel leaking lymphtic fuild. Within a couple of weeks the lymph vessel healed on it's own and closed up. Doctor's were not sure if it would close on it's own or not. With every day, month, and year that passed my lymphangiectasia symtoms continued. I continued to get more lymph vessels growing out of my foot. Especially my big toe on my right foot. The only thing I was to do is to put lambs wool in-between my toes to help cushion and prevent break down of the skin so I wouldn't leak the lymphatic fluid. In 1979 I was then referred to Riley's Children Hospital in Indianapolis Indiana, to have some more extensive tests for my PIL and malasorbtion sydrom. I was 14 years old. This is when my parents and I were told about the middle chain triglycerides oil. As for any other type of treatment for PIL, they didn't have any other to offer. Only the high protein low fat diet with the MCT oil. My developmental in phyical aspects was still behind. Most girls at the age of 9-14 were developing breasts and have had their first menstral cycle by this time. I didn't have any breasts until I was 16 and this is also when I had my first menstral cycle. I remember being the odd girl out, growing up. I was always the thinnest girl with all my friends. I just always felt different because I had health problems, and nobody else I knew had health problems like I did. Emotionally you just don't fit in, because you know your body is different. I felt alone, even though I had parents that were always there for me. My parents tried to treat me as a normal kid, even though I had my health problems. I am truly grateful for everything they given me. They loved me with all of their hearts. My parents were wonderful. I know at times they went through hell... All the trips in and out of the hospital they had to indure with me, while raising 4 other children, but never leaving my side. I remember how hard it was to keep to the diet growing up, because I had four sisters who could eat what ever they wanted. There wasn't to much to choose from with low fat's. Especially when it came to desserts, etc.... When I graduated from high school in 1983, I was 5 foot 7 1/2 inches tall, at a big 90 pounds. I had problems with my right foot and leg swelling to the point it felt as though if you took a pin to pop it, it would pop like a balloon. My right foot was also considerably smaller than my left foot. I would have to say my right foot was 2 sizes smaller than the left foot. I also had the extended belly, because the lymph fluid would settle into my tummy area. After graduating high school I moved on my own right away, and I made the decision not to stick to the low fat high protein diet. You know at that age you think you know it all, at least that is how I was. At 19 I was pregnant with my first child. My pregnacy was not typical, I had to have home nurse care because my body was not giving my son enough nurtrients when I was pregnant. I had to have home IVs and etc. I only gained 5 lbs. with I was pregnant with my son William. When William was born on November 19, 1985 he weighed 5 lbs. 5 oz., so all the weight I actually gained was all baby. I am not going to go into details of each of my pregnancies, but I know that each time I had gotten pregnant my lymphangiectasia became worse. Financially I couldn't afford to cook different meals for my family and then something for myself, so I ate what was made for the entire family. I know this in turn has made my PIL uncontrollable now. In May 2001 my husband and I went on a golf outing and I had broken my right Tibial Plateau Fracture of the leg, from jumping out of a going golf cart. My leg seemed to be healing fine, according to my orthopedic doctor. But, on July 12, 2001 I had woken up in the middle of the night with tremendous pain in my right foot and leg. I also had the symtoms of discolored (purple) toes, and the feeling of my foot and leg being cold. I went to my local emergancy room when this happened and they did tests on my veins to see if I had a blood clot, but it showed that I did not have one. To make a long story shor the ER doctor sent me home, and a couple of days later I had to have my right leg amputated due to a blood clot that formed in the artery. (Not the vein.) Because of the ER doctor not listening to me as his patient, I nearly lost my life. Things had gotten so bad that I had to be put on a ventilator and they were talking about a heart transplant. God must have other plans with me because I survived. I pray to him on a daily basis and thank him for the life he had given me! To continue, In January of 2002 my body gave in to all that had happened to me with the amputation and my instestinal lymphangiectasia, I became so sick that I was hospitalized for over a month. This time I decided to go to Northwestern Hopital in Chicago, Illinois. I wanted to go to a hospital that would dig into my medical history and find out what was going on with me. The Doctor's at NWH were more than I expected. I was grateful for them. They found out I had another problem with my health besides my PIL. They found out I have a condition called antiphospholipid antibody syndrom besides my PIL. This causes my blood to clot, and because I had an injury from the leg getting broken, it formed a clot in my artery causing the muscle and tissue to die. Therefore, the imputation of the right leg above the knee had to take place. It took me 2 1/2 years for my body to recover from everything. In 2005 I was well enough to start living my life as God had intended each of us as individuals. I went to college to get a degree in computer information systems. I graduated college in the Spring of 2008. I missed so much when I was so ill. My children had to grow up faster then most. They in turn had to take care of me, instead of me taking care of them. My husband tried to play mom and dad, even though he is only the stepdad... He didn't have to stay with me... But he chose to... And my parents they have always been a rock when it came to me being ill, always there for me and totally loving in every way... What can I say my Family has been the best, and without them I don't think I would have been as strong as I am! This past July I started having major problems with my PIL and have been seeing doctors from NWH. Of course because I am an adult finding doctors that have dealt with PIL is about zero. So, they have been starting from scratch with me. I am totally their guinea pig. I have been having problems with my bowels, nausea, vomiting, swelling in my left leg and foot, along with swelling in my hands now, fatigue, an extended swollen belly (stomach). (I look like I'm Pregnant!) The list seems to be never ending. The newest symtom I am having, on each side of my ribs towards the center of my chest I have puffy lumps that are soft and mushy like the lymphtic fluid settled in these specified spots. Dr. Olinger is my GI specialist I am going to now. He had to do the colonoscopy and the endoscopy just recently which showed the PIL and malabsorption syndrom, as it did when I was a child. The next step is to start my low fat high protein diet, but the dietition at NWH won't be available until December 21, 2010. That is why I am doing research over the internet. That is what has brought me to your site. I am trying to find people with the same condition so that I can become familiar with all the information as I possible. So, I am asking you to please give me any information about PIL and malabsorption syndrom as possilble? And if possible to give me some names and emails of other patients that were diagnosed with disease? I applaud you for all your hard work putting this website together! There isn't enough information out there for us who are debilitated with the condition. I hope to hear from you soon! Sincerely and With Love, Monica Gast

Community External News Link
Title Date Link
Community Resources
Title Description Date Link

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

 

Expert Questions

Ask a question

Community User List

Im a 23 year old female fro...
Nursing School Instructor
Mother of six...my daughter...
Diagnosed with PIL at 9 mon...
Have a son with Intestinal ...
Our son 3 years old athanas...
My name is Monica Gast. I a...

Start a Community


Don't See Your Condition On Rareshare?

Start your own! With a worldwide network of 8,000 users, you won't be the only member of your community for long.

FAQ


Have questions about rareshare?

Visit our Frequently Asked Questions page to find the answers to some of the most commonly asked questions.

Discussion Forum

Your current therapy

Created by Magaliep | Last updated 20 Jun 2016, 07:24 AM

PIL

Created by micrich | Last updated 7 Jan 2014, 12:47 AM

Toddler with PIL

Created by MaryG | Last updated 10 Aug 2013, 02:37 PM

Primary Intestinal Lymphangiectasia Patient

Created by Monica1965Gast | Last updated 3 May 2011, 01:30 AM


Communities

Our Communities

Join Rareshare to meet other people that have been touched by rare diseases. Learn, engage, and grow with our communities.

FIND YOUR COMMUNITY
Physicians

Our Resources

Our rare disease resources include e-books and podcasts

VIEW OUR EBOOKS

LISTEN TO OUR PODCASTS

VIEW OUR GUIDES

Leaders

Our Community Leaders

Community leaders are active users that have been touched by the rare disease that they are a part of. Not only are they there to help facilitate conversations and provide new information that is relevant for the group, but they are there for you and to let you know you have a support system on Rareshare.