Common Variable Immune Deficiency

I had PSC for 12 years before getting a transplant. My new liver works fine. Starting in December 2012, I have had daily diarrhea, cramps and pain that lasts 3 to 5 hours, EVERY DAY!! UGH!!

My hepatologist scoped me both ways multiple times, ran blood tests for e-coli and c-diff and Alpha-Antitrypsin ( a protein). There is no evidence of disease that would cause these symptoms. He did a blood test that showed the basic building blocks of my immune system, Immunoglobulin A, G and M were off the chart low, indicating a possible cause since a good portion of the immune system resides in the intestinal tract.

He sent me to a noted immunologist at Northwestern Memorial Hospital, who spent two hours testing my blood, taking a detailed history and reviewing the labs from way back to when my PSC was diagnosed and found I had this same immunoglobulin condition. The next time I saw him, he diagnosed it as Common Variable Immune Deficiency (CVID), extremely rare in PSC patients.

My immune system is fighting itself. We all were told how our immune system was attacking our liver causing PSC. Now, I learn, it's up to more mischief. I am symptom free, except for my gut. Low immune system usually allows for easy respiratory and skin infections. It also damages bronchial tubes if not caught early.

I am going to start immunoglobulin therapy, which is healthy immune cells infused through an IV. As for the diarrhea, it may respond in three or four months. No promises.

Have any of you had CVID? I would like to share what it is like and what treatments you are getting

I have psc from March 2010 i got acid colitis from psc which i need to take my pills. i do go to the bathroom quiet often, also i was bleeding from behind and i had to stay in the hospital for a week. they up my does, now i go to the bathroom once a day, it was a scary feeling. I was told there is a drug out there that will work, but we need to wait 2 to 3 years. I don't have two to three years, if it does not get better i will be in surgery.



About my son said:

I have psc from March 2010 i got acid colitis from psc which i need to take my pills. i do go to the bathroom quiet often, also i was bleeding from behind and i had to stay in the hospital for a week. they up my does, now i go to the bathroom once a day, it was a scary feeling. I was told there is a drug out there that will work, but we need to wait 2 to 3 years. I don't have two to three years, if it does not get better i will be in surgery.

I am sorry that you are suffering so bad. I hope they get your gut under control in a short time. I agree with you that it is scary. I have ulcerative colitis and I had PSC. The doctors have checked me out and my UC is not causing the diarrhea. My immune system is causing it, and they do not know how to treat it. My immune system blood test are very low. They are going to start infusions of healthy immune cells into me next week. They said I will need it monthly. They do not think it will solve my diarrhea problem.

Your post is very helpful. My husband is 67, has had PCS for 20 years with very few problems until about 2 years ago. He has had recurring diarrhea now since the middle of January, almost daily. His doctor did many of the same tests you had but was not able to give him any explanations. I am going to print this out and show the doctor. Thank you

Please let me know what your doctor says.

young said:

Your post is very helpful. My husband is 67, has had PCS for 20 years with very few problems until about 2 years ago. He has had recurring diarrhea now since the middle of January, almost daily. His doctor did many of the same tests you had but was not able to give him any explanations. I am going to print this out and show the doctor. Thank you

Read the post... Don't have advice or an experience such as yours but I do want to follow up with you on what exactly happens and how you feel, as supporrt and a way of educating myself. Thank you for the post!

Gloria,

Common Variable Immune Deficiency (CVID) is a sub-class of Primary Immune Deficiency (PID). I am just learning more about this myself, as I was diagnosed with it in July 2013. It is not common. "Common Variable" refers to the scientific characteristic of the disease. I was cured of Primary Sclerosing Cholangitis when I received a liver transplant in 2012. As you know, PSC is a very rare immune system related disease. The symptoms I experience from CVID are fatigue and diarrhea. The treatment my doctor wants for me is called IGg Infusion Therapy. Healthy immunoglobulin cells extracted from donated blood are prepared and flowed into my body by inter venous injection once a month. I have been told it will take 3-4 months before I feel any benefit, and it is not likely going to help with the diarrhea. My gastroenterologist/hepatologist put me on Lomotil as needed for cramping pain, and Healthy Trinity Probiotics (a capsule a day of billions of good bacteria) to help maintain a healthy intestinal tract. CVID weakens your immune system and makes you vulnerable to skin and respiratory infections. I have no history of either, which makes my immunologist scratch his head and wonder why.

This experience is all new to me. I was/am hoping to find other PSCer's that have experienced this and can help me and my doctor. Meanwhile, I will keep posting my progress in the hopes it will eventually help somebody else one day.

Northwestern Memorial Hospital in Chicago is a huge place with a fantastic transplant center called the Kovler Transplant Center. My hepatologist that follows my liver transplant care suspected my immune system was involved in my diarrhea problem. Blood tests showed my immunoglobulin(IGg) A, G and M levels were extremely low. He sent me to the hospital's Immunology Clinic. The doctor there said my hepatologist's workup and testing was extremely thorough and that he could diagnose what I have as Common Variable Immune Deficiency.

As of 9/11/13, he is trying to qualify me for IGg Infusion Therapy, which means they will introduce healthy immune cells from donor blood into me via inter venous transfusion. My insurance company is requiring a different variety pneumonia shot and another Titers blood test (which measures one's production of antigens) before they will consider allowing the treatment. It costs $15,000, yes $15K, each month. I guess I can understand their reluctance. This could add several months for the injection, test and their review. In the mean time, I'm going to keep smiling and gripping the throne's seat!

Thank you, Paul for educating me. I appreciate you sharing your experience. -A

Hi, Paul. Checking in. We're you able to get the infusions you needed? I could not find an updated post on the subject. Hope all is well. -A

I have had five months of infusions of Gammagard, an Iv compounded from human A,G and M immune cells. It is made up of 13,000 donor immune cells processed from 1000 units of blood. It is clear looking and takes 4.5 hours to infuse. Each dose costs $15,000. That's not a typo. I have had an allergic reaction each time I got the infusion. Skin rash and most recently aseptic menengitis. Not fun. Right now, the doctor has me on hold until they can develop a new plan. There is only one person on the national database the hospitals share with the same medical history that has CVID. What they are doing to help me is experimental. I have had more energy as a result of the infusions, but it has only helped a little with the intestinal cramping. I keep posting updates.

Thanks, Paul for the prompt response. I searched your discussion activity but couldn't find updates. The separation of blogs, discussions and personal page comments makes tracking a specific topic difficult, in my opinion. This question may sound ignorant but, how does one donate immune cells? Yesterday I went to an appointment with my fiance to see his Hep specialist at CPMC, SF. It's transplant evaluation time, unfortunately. I know you did well with your live donor (total encouragement by the way). I prayed I would be able to give this gift to him. I was told yesterday I was too small and I would be needed more importantly to care for him after transplant (I disagree, that's what family and NPs are for, but they said no). I want to be able to help. In all honesty, I don't think I could emotionally go through with donating a portion of my liver until after he has his transplant. I need to know he'll be OK first. But with that said, do you know other ways I could help someone out in the mean time?

Immune cells are removed from regular donated blood. Some blood is set aside for processing into various blood products, i.e. plasma, platelets and immune cells. Some blood facilities have a machine they can process into plasma during your donation.

Your fiancé needs to take care of his liver as best as he can. PSC can be slowed, and life can be close to normal. The more advanced PSC gets, the more symptoms occur, such as fatigue, itching and jaundice. For some, PSC remains a chronic disease. Others, will need a transplant. You can read my earlier posts about the Model for End-stage Liver Disease (MELD), which is used uniformly by transplant hospitals to allocate livers. The gist of it is that PSC does not produce the liver chemistry that other liver diseases do. You can be on death's door (as I was) and have a low MELD score. A transplant surgeon at UW-Madison, WI, told me my only hope was to find a live donor. Not an easy thing to do. One of the most inventive ways I heard somebody found a donor was they invited all their friends to a "surprise party, no gifts." The surprise was they told about her PSC and a need for a donor. They got several who were tested and one was a match that became the donor. People are good and they want to help. Another friend in my transplant support group (copy and paste in browser) https://www.facebook.com/pages/Transplant-Support-Group-of-Northern-Illinois/134665889929934 is joining a group of about 100 in Wisconsin that all need a kidney and who have at least one relative willing to be a donor. They will donate to another individual in exchange for receiving a donation. She is just getting started in the process, so I cannot tell you how it is working. My transplant was done at Northwestern Memorial Hospital in Chicago (search for Transplant Village in Facebook for their story), and they are in the process of developing a benevolent donor program through their philanthropy program. This is a medically ethical way for healthy people that want to be a live donor for a complete stranger, to do so at no cost to them and in a way that insures their utmost healthcare. After my transplant, I was contacted by a man in Spain who wanted to be a benevolent donor. There are people out there that are motivated to help a stranger, like the Good Samaritan. If your fiancé is getting very sick, I urge you to look into live donation at a transplant hospital that has a long history of live donor liver transplants. It is very acceptable to be listed at two or more transplant hospitals in different regions. My advice is to not put all your hope in a cadaver transplant. I did for almost four years, and it would have cost me my life. Questions? Any type, any time.

All, please try to read this book. “Healthier without Wheat” by Dr. Stephen Wangen. I am learning a lot myself.
I would try going on a complete gluten free diet. See if this helps rid some of the problems, who knows maybe even all. I am positive it will. All the best.

Do you have chronic diarrhea and cramping related to wheat intake?



Gloria said:

Thanks, Paul for the prompt response. I searched your discussion activity but couldn't find updates. The separation of blogs, discussions and personal page comments makes tracking a specific topic difficult, in my opinion. This question may sound ignorant but, how does one donate immune cells? Yesterday I went to an appointment with my fiance to see his Hep specialist at CPMC, SF. It's transplant evaluation time, unfortunately. I know you did well with your live donor (total encouragement by the way). I prayed I would be able to give this gift to him. I was told yesterday I was too small and I would be needed more importantly to care for him after transplant (I disagree, that's what family and NPs are for, but they said no). I want to be able to help. In all honesty, I don't think I could emotionally go through with donating a portion of my liver until after he has his transplant. I need to know he'll be OK first. But with that said, do you know other ways I could help someone out in the mean time?

U



Gloria said:

Thanks, Paul for the prompt response. I searched your discussion activity but couldn’t find updates. The separation of blogs, discussions and personal page comments makes tracking a specific topic difficult, in my opinion. This question may sound ignorant but, how does one donate immune cells? Yesterday I went to an appointment with my fiance to see his Hep specialist at CPMC, SF. It’s transplant evaluation time, unfortunately. I know you did well with your live donor (total encouragement by the way). I prayed I would be able to give this gift to him. I was told yesterday I was too small and I would be needed more importantly to care for him after transplant (I disagree, that’s what family and NPs are for, but they said no). I want to be able to help. In all honesty, I don’t think I could emotionally go through with donating a portion of my liver until after he has his transplant. I need to know he’ll be OK first. But with that said, do you know other ways I could help someone out in the mean time?

April 4, 2014

Hi Gloria,
Caring for your fiancé after transplant is more important than being a donor, especially if he qualifies for a cadaver liver. Recovering from transplantation is slow. For the first two weeks, someone has to help you with changing dressings (wet, but not bloody. Not as gross as it sounds), bathing, dressing, getting to daily appointments, preparing food, administering oral medications, keeping notes to tell the doctor, doing laundry and keeping track of your regular life. It is hard for the caregiver to get much rest.

You fiancé will be fatigued because the liver is regrowing and using 4000-6000 calories a day. He won’t be much help for the first month. Don’t try to eat that much more to make up for the used calories. My doctor laughed when I told him that’s what I was doing. I got indigestion. He said eat normal. The body will provide the calories. I lost some 15 pounds and then started regaining my weight.

Transplantation is a life-changing, life-improving miracle. Expect to live a normal, happy life together, post transplant. You are experiencing at the beginning of your married lives what I and my wife did after many years of marriage. Your relationship will grow stronger because of what you have to give of yourself to help him recover. If he is anything like me, he will feel there is no way to possibly repay you for the gift you so willingly gave to him. Maybe, some day your wish to be a donor that helps someone will come true. (See our Caringbridge site for more of our story. http://www.caringbridge.org/visit/paulandkathy

When the time comes for transplant, you might want to consider opening a free Caringbridge.org site where you or someone you appoint can post updates about his progress. You will not have enough time or be able to take calls. He definitely will not be able to see visitors for a while. Caringbridge allowed us to keep many people informed, even family in Europe. When Kathy and I were able to write our own posts, people were reassured we were doing fine. The founder of Caringbridge, Sue Mehring included our story in her recent book, “Hope Conquers All.” I know I can only repay what I received by helping others and making the best of the remainder of my life.

Gloria, your question about donating immune cells. Answer: they are extracted from donated blood. Plasma, platelets and immune cells are processed at a blood laboratory. It is an expensive product. It costs $15,000 per month!
Paul