Common variable immuno deficiency (CVID)
Hello folks
Two years later for this is a re-post
Life is greatly improved and infusions are so much easier. I had no idea how long it would take to be normal again. Life is sweet and I am grateful. I also had no idea how many souls are out there with the same issue just floating in a no mans land. Many have found solace here. Please forgive my lasting absence. It took a while to accept through things grieving process. Also within the last 18 months I had a total knee replacement and labial repairs on both shoulders. Life does continue and it is full of promise. It is a joy to me to hear from so many of you who walk dazed in the CVID diagnosis. I will be coming home here to the blog.
Original posted in June of 2010
Genetics are a huge part of our makeup we are a generational being...
The test have returned and this is the diagnosis faced in my life now. The most difficult thing is the sadness regarding the nieces and a nephew who are suffering in the dark as to why their bodies are self destruction. They have no medical intervention. This greatly effects life expectancy. I must inform my siblings, the generation of those there after and so on to be tested and to seek intervention. To do this while guarding the hearts of those who have no comforter.
I have an 8 year old and a 12 year old. The infusions can sorta wipe you out a bit for a few days. Best case only one every 4 weeks. But it sounds more likely that it will ramp up to that. I need courage. I need tenderness to strengthen my heart. Knowing that siblings are in a untreated state, know now what took my moms life. All these thing flood my mind a white wash of thoughts.
The general fatigue and lethargy is so progressive. This has gone un-diagnosed for some time now and many years of repeated resistant lung infections of bronchitis and pneumonia. You know I see the sweet mercy. I asked why? Why do i get all this freaky illness that have left me feeling humiliated, demised,demeaned and folks including medical and family acting as if this has all been in my head. YEARS OF THIS! IT LEFT ME DOUBTING MY OWN CHARACTER AND SANITY AT TIMES. SOMEONE i THINK IT WAS Marsha SENT ME A LITTLE BOOK ON MINISTERING TO THOSE WITH CHRONIC ILLNESS. well at THE HOSPITAL DURING THE INFUSIONS FOR MYSELF THERE i WILL BE...WITH A CHOICE OF THINKING AND BEING THERE FOR ME AND CONSUMED WITH "me" OR EXITING THIS VORTEX INTO THE COMPASSION NOT ONLY FOR MYSELF BUT FOR THOSE AROUND ME.
with THE SAME COMFORT WE HAVE BEEN COMFORTED WE SHALL COMFORT OTHERS.
Must be a lot of folk needed comfort cause here it comes. Please remember to comfort my heart my dear friends and family. I will need the bracing up as I go forward. I can not let this stop who I am within my heart.
Man, it has really been one thing health wise after another for so many years. Understanding is the beginning of knowledge it will be then that I can walk in wisdom once I exercise the knowledge there is for me to gain.
A Wonderful post gave me a nice dose of
God works all things to his Glory
Everything to harm us is turned to glorify Him
May this walk given me to undertake be done in such a way that i remain unfettered by fear, bitterness and the potential effects of treatment.
Treatment usually consists of immunoglobulin therapy, which is an injection of human antibodies harvested from blood donations:- intravenous immunoglobulin (IVIG, most common treatment in the US)[9]
- subcutaneous immunoglobulin G (SCIG, relatively new treatment in the US and UK)
- intramuscular immunglobulin (IMIG, less effective, painful)
IG therapy can't be used if the patient has anti-IgA antibodies but in this case, products low in IgA can be used; subcutaneous delivery also is a means of permitting such patients to have adequate antibody replacement.
IVIG treatment can be received by patients with a complete IgA deficiency if the IgA is completely removed from the treatment.
Disease Information
Hypogammaglobulinemia - Overview
Hypogammaglobulinemia essentially means antibody deficiency, and includes numerous primary immune deficiency diseases such as Common Variable Immune Deficiency (CVID). Unlike AIDS or chemotherapy-induced immune deficiency, primary immune deficiencies are considered inherent, perhaps genetic.
One of the rarest forms of hypogammaglobulinemia, Severe Combined Immune Deficiency, is nicknamed the "boy-in-the-bubble" disease - a somewhat popular example known to movie viewers.
Both Common Variable Immune Deficiency (CVID) and IgA deficiency, whereas antibodies are decreased but not absent, are much more common. The approximate rate for CVID is 1:50,000 persons and the rate for IgA deficiency is as frequent as 1:750 individuals.
As the immune system is quite complex, CVID often involves additional defects in the immune system.
Common Variable Immune Deficiency (CVID) is mild to life-threatening: dependent on resistance to infection, treatment success, and if complications occur. Significant risk of developing certain cancers and autoimmune illness also exists for patients.
Inherited Immunodeficiencies: Common Variable Immunodeficiency (CVID)
Alternate Names: CVID
Definition
Common variable immunodeficiency (CVID) is an immune system disorder which typically affects males and females in the third or fourth decade of life. However, it may also be seen in children. It is characterized by low levels of antibodies (another name for immunoglobulins) in the blood stream and an increased susceptibility to infections.The diagnosis of common variable immunodeficiency is suspected when the patient has low levels of antibodies in the blood stream, a poor immune response to vaccines, and a history of recurrent infections. Patients with CVID develop recurrent infections of the sinuses, ears, nose and lungs. They may also develop enlarged lymph nodes (palpable glands in the neck and groin) or an enlarged spleen. Infections of the gastrointestinal tract may also occur. Individuals with CVID have an increased incidence of malignant lymphomas and autoimmune disorders such as lupus, rheumatoid arthritis, inflammatory bowel disease and autoimmune hemolytic anemia.
Influencing Factors
Pattern of InheritanceGenetic factors do play a part in the development of common variable immunodeficiency. However, there is no single gene mutation (mistake) that can be identified. Environmental factors may also influence the development of CVID.
Treatment Strategies
Treatment for common variable immunodeficiency includes monthly infusions of gammaglobulin (IVIG) and oral antibiotics to prevent infections. Prognosis is variable and may depend on the severity of lung disease prior to diagnosis and the occurrence of autoimmune disease or malignancy. Patients are encouraged to lead a healthy lifestyle, which includes good nutrition and regular exercise.More detailed information can be found here.http://emedicine.medscape.com/article/1051103-overview
19 comments:
glad you have a diagnosis, sweetheart.
Sweet sis, I love you, and you will remain in my prayers.
I can only imagine the flood of thoughts and emotions that you are having- with dealing with this news. I had never heard of this before. I will be praying for you - that God will give you the strength and the courage that you need,
Lisa :o)
On one hand I am happy you have an answer on the other hand I am sorry that your going through this, your a very strong lady and I know your strength, prayers and your love for our Lord will help assist you through this, God has blessed you with such an awesome husband and he will be of great help to you. I have felt like people thought and still think at time's that my depression I have a tendency to suffer with from time to time is all in my head and it is so hurtful, so I know what your feeling. I will pray that our Lord will be with you and to continue to keep giving you strength.
LOVE YOU
Hugs to all~
Annette
Certain diseases have been passed on in my family specially heart, diabetes, arthritis and ear diseases. Gotta just live with them.
Arthritis is a immunity deficiency blood disorder I read.
I the US you have all the info and medical expertise avaialbe in the 3rd world its either non-available or too exppensive.
I'm glad you know what is going on, but I'm sorry you're sick.
I came across your website as I was looking for info on CVID. My 9 year-old grandson was diagnosed when he was 10 months old. It's been a hard battle for us.There have been times when I thought we were going to loose him. The Lord has brought him through each illness. In Feb. 2010 he had C-Diff. After prayer with a friend he was released form the hospital in 3 days.I know God answers my prayers. He does it every day.His doctor has never suggested the treatments and that is what I was looking for. He said that as long as Kobe had some immune factors he could not receive treatments. I'm trying to learn as much as I can. For 2 years he seemed to have outgrown it tn this past year he stayed sick for months. He can no longer attend school. I was looking for financial aid to enroll him in a private school on line. All I have found is help for college students. God be with you on your journey.
I also have cvid. I spent my life being told I was making it up, being dismissed by doctors and family. I am declining and still being treated in a patronized manner. Unable to take IvIg due to reaction. Last week saw a derm for a weird skin rash and node on chest. Was told nothing. Oddly same day made appt with reg doc and had high fever and lung infection. Have been ill whole life and do not know where to turn. Have Lymphoma like symptoms but no work up. I am ill. I am tired and no one will listen. I need a caring immunologist. Any ideas? Have many autoimmune issues and no response to pneumovax. Docs do not get we are seronegative. Thus say labs fine. Am 61yo and lung damage and left side very affeted, Derm had nerve to say cvid mild disorder. I am not. Sonja
THIS IS FOR SONYA. POSTED APRIL 2,2011.
PLEASE DO NOT GIVE UP ON BEING DIAGNOSED. I WAS JUST DIAGNOSED IN THE SPRING OF 2011 AFTER MANY DIFFERENT DIAGNOSES. --- MY PULMONOLOGIST REFERRED ME TO AN IMMUNOLOGIST WITH SO MUCH COMPASSION. ---SHE IS LOCATED IN PORT CHARLOTTE, FLORIDA AND HER NAME IS DR. USHA CHANDRAHASA,MD ---SONYA,IT IS MY PRAYER GOD WILL LEAD YOU EXACTLY WHERE YOU NEED TO BE. --- PEACE
I have CVID and have been doing treatment now for 7 years I started on IVIG and 6 months ago started SUBQ my levels on SUBQ are so much better. I had all the ITS IN YOUR HEAD too, and I understand your anguish. You will feel somewhat better the IVIG just takes time it took me 6 months to a year. I still get the wierd infections but antibiotics work faster now. No one in my family as CVID so it was hard for me to believe I had what they think is a genetic illness, although Ive heard from many that Epstein Barr is common before the onset and I did suffer a severe case in my late 20's at 31 I had my 1st and only child and started feeling ill 6 months later,it took over 3 years to get diagnosed and they said that was quick. Not quick enough for me. (my Daughter is ok Ive had her check) I tooo live in FL I was diagnosed at SHands in Gainesville but now do my own SUBQ and See a good doc in Lakeland at Watson Clinic. I wish you well and if you need any help on anything dont hesutate to ask. I love the title by the way.Tracy
my 22 year oold cousin died recently from his cvid complications, Yes I am sad for this loss but I am also confused, Why him and not me. This bit bybit dyiing, tumors being removed spinal surgeries, nasal procedures, and all the drugs,,,, enough is snough I wish I had the balls to end this horrific life,
Oh gosh, am I glad I found you!
I have been ill nearly all my life - strep, staph, sinus infections, shingles (x4!!!!), pneumonia (x9!!!!!), constant bronchial issues, asthma-like symptoms that had me feeling awful but left my allergist scratching his head, and on and on and on.
Then, last August I suddenly became *very* ill. Very, very, very ill. I started losing ground and fast. By December I was so ill with this THING that I have only been able to consume Boost most days - nausea, vomiting, GI issues. I was also bruising badly - ALL over my body - and in two separate admissions to the hospital the staff social workers tried to badger me into stating my husband had caused them! MY HUSBAND? ARE YOU NUTS? I admit I looked as though I had been severely beaten - some nights I could go to the bed and awaken in the morning with up to 80 bruises - everywhere. Large, small... multifocal, singles... and of all different colors. None of them hurt and they all went away in a matter of a few days. The symptoms and illnesses just piled on and on and on. I went to the Cleveland Clinic to seek help - and I had a doc tell me, "You know, it is common for women who have been raped to develop odd symptoms later in life and I think you are this; I just don't think you have or will 'get over it'." @@
Um.
So, I came back home, having spent THOUSANDS of useless dollars. Upon returning I basically collapsed for a month. Went back to the hematologist and he said the same thing - somatoform. Then my primary care doctor's office KICKED ME OUT OF HER PRACTICE because I was "too complex" for what they offered.
In January, within a week, I had come down with pneumonia, a kidney infection, the flu, AND a mycoplasma infection of the lungs. Heaven help me!
Summer brought the second case of pneumonia this year (and I had had Pneumovax four times in three years; when I was in the hospital in October, I was so sick that I could not communicate that I *had* had my pneumovax and flu shots already, so they stuck me again. After all that I *still* don't show any antibodies to them. We're doing one more set of titers and a vaccine challenge now - they all know it won't actually show any acquired antibodies, but you know - insurance companies require a lot of hoops, and especially so if they are going to have to pay out every three weeks to the tune of roughly $5,000 each. And, because I have had such problems in the past five years I have spent an unreal amount of time in the hospital. 29 days in April 2007 was insane - they ran semi-diluted or non-diluted phenergan into my IVs 24/7 for roughly 17 days; after that they put in a PICC. My veins are shot (I cannot tell you how many thousands of needle sticks I have had over my life, and the phenergan was the last straw, so insurance is also going to have to chip in for a port-a-cath.).
Anyway, I am 44 and things have just been becoming harder and harder, and I have become very frail - not "thin" but rather gaunt and malnourished, and just not "ME"
Finally, one doc I see on a semi-regular basis saw me Thursday early afternoon and was stunned at the changes she saw in me. She got quite angry, told me to go wait in the waiting room, and we would discuss this further. I sat there thinking, "OH MY GOD! Is ANOTHER doctor going to fire me?" Well, she came back out about ten minutes later and she had arranged (and I have no idea what sort of political capital and pressure on friends she had to use to do this) for me to be seen within the hour by a rheumatologist-immunologist and to GO. NOW.
Fortunately, I drag all my med records with me to every doc appointment (17 or 18 CD/DVDs with imagine and other test records; file folders of information sorted first by the major system involved (neurology, physical therapy, OB/GYN, etc.) then by date within each. I have been going through old boxes in the attic to try and find even more, but I do have *at least one* blood test for every year of my life - and the past five years of them makes for an amazing stack of paperwork. WOW. So, when I asked the new doc while I was driving what all they would need from my records they were first stunned that I had come to this, and then when I told them, "Yes. I have that with me." for EVERY SINGLE THING THEY WANTED it was easy to just roll the file box to him and let him merrily dig.
And yes. I *do* have CVID. We're already on protocols required by the FDA and insurance company. However, he said I am so ill that the next 8-10 weeks will basically be make or break. It's not only a matter of getting to the point of having the IgG replacement therapy - but also one of simply surviving until then (and even more so, surviving on some of the meds the insurance company requires we try first. I HATE it when pencil pushers make medical policy. Don't get me started). He was quite frank: I am ill enough that, if I catch another bad infection - the flu, pneumonia, mycoplasma, whatever - I could potentially die within forty-eight hours.
So, I am house-bound for the next 10 weeks. I may go buggy, but if that's the price I must pay to FINALLY have quality of life AND regain my career (I have about 8 months left in my PhD), then it is worth it.
I just cannot tell you, though, what running across your blog entry has meant to me. It's one thing to be the "Boy in the Bubble" - and another thing entirely to not know why or how long or even if.
Thank you. Thank you so very much.
And Nancy? I COMPLETELY understand the bit-by-bit dying thing. It has felt like this for years, and there have been weeks on end where I have simply willed myself to breathe because if I didn't, well, I don't think I would have.
Awful.
And I am so very sorry you lost your cousin to this devastating ... THING.
Hang on and hold hope. That tiny little filament is sometimes the only thing we have.
Rhonda -
I am a faculty member at a university, and I also received one of my Master's degrees fully online. LOVED it.
My husband is an educator in the city schools.
Between the two of us and you, surely we could find something. Surely?
If you would like to touch base, call my Google Voice number at 901.730.6762.
:)
Wow, I am reading through all the comments and I am in awe of how this illness is actually so real. I can't say enough that I know this blog doesn't cure us but sure helps us not feel so alone. Thank you for all of your comments.
I'm a 58 year old woman diagnosed with CVID ten years ago. I am so interested to read the reports from my fellow CVIDers--well named, "an uncommon life."
I was an elementary special ed teacher who suffered for 18 years with undiagnosed allergies and asthma literally since my first year of college after getting pneumonia my senior year of high school. Got sicker and sicker, than finally was diagnosed with asthma and drugged appropriately and started allergy shots, which helped a lot. However, that didn't stop the near-constant sinus infections, colds turning into bronchitis, etc. that I would pick up at school from my students. I would take loads of prednisone to get breathing again and get back to work--and of course, prednisone impairs the immune system...
Finally, 12 years after my asthma diagnosis, in December of 2001, I got sick (which I did roughly every month to six weeks), but this time I did not get better. I couldn't pull out of the asthma attack and sinus/lung infection regardless of the amount of antibiotics and massive doses of prednisone and other meds I was taking. After trying to go back to work by going one day a week, then two days a week, and so on, I got to one week of working four days, and that was all--in March 2002 I relapsed and was sicker than I had been the previous December. That was my last week of work, ever. I had had my blood levels checked in the mid-90's to find them low normal. This time, my doctor checked my levels, and they confirmed the CVID.
I remember thinking, when I was first told about the IVIG, that that was the cure--just a few IV treatments, and I would be good to go. It was quite a punch in the gut when I realized that this condition is incurable.
For five years, I did the IVIG once a month, then got on a trial for subQ, which I've done now for five years, and I love it. I feel much, much better--but I am also extremely isolated. I do not go out much, don't travel at all. (Fifty miles to an acupuncture doctor is the farthest I've gone in 30 years--and she is unhappy with me because I have to cancel so often because of asthma flare-ups). I use a heavy face mask from fall to spring during rare trips out in public during flu season. I only eat in restaurants from spring to fall during off-times (dinner at 4:30, anyone?) when no one else is in the restaurant. When mosquitos are active, I must be very, very careful, as West Nile is in my area. It would take a house fire or me or my family being carried out in an ambulance to get me outside between dusk and dawn from May through October!!!
I only have regular face-to-face contact with my husband and mother, (no children--I was always too sick to even consider it) and they know enough to stay clear if they feel ill at all. I lead a very, very lonely life--but the tradeoff is that I am reasonably well (considering I take 14 meds, not counting the subQ, on a regular basis for other conditions). I have asthma flare ups often because of weather or pollen (even staying indoors...), but I've not had a serious asthma attack in six years, and have only had to use prednisone for a week or two a few times in the last few years to get me through a bad flare up.
I've done this self-imposed exile from the world willingly after being so sick for so long--but it is a very strange (uncommon?) life, very isolated. I know that some people could not afford to do this--go on disability and become a hermit. But it has been my salvation. An odd life, an uncommon life, but finally, literally, since I was 18 years old, a relatively healthy life. I'll take it. It's my only option!
I hope u come across this and this helps u.. I was reading up on cvid bc I have it..33f, diagnosed in 2009 after 9 months in hospital, over 30 surgeries, more than 14 blood transfusions, 18 months on iv antibiotics, mrsa infection in my bones, sepsis, and much much more.I got ivig treatment immediatly after diagnosis 3/09 until 10/12 when my insurance just started not wanting to pay bc of cost (my gammagobulin is almost $12 thousand /3 weeks,)..I am appealing, fingers crossed!! Basically I am reaching out to u to let u know that YOU have to be agressive about ur own care, in this case ur grandsons. If I left it up to drs I would be dead. I had to ask the same questions over and over for months , basicly demand testing, treatment, ansewers throughout my care and that is the only reason I was even diagnosed...I was getting infections over and over scince 2001, got pneumonia in 2007, surgerys for bone infection and iv antibiotics in 2008, and still drs said it just happens sometimes. I even had an infectious disease dr in the hospital treating me with the iv antibiotics for 7 months before I convinced him after much much begging for ansewers to test my immune responce.
At every point of care u can not be to vigilant. Knowledge is power! Find out all u can and ask the drs, nurses ect, sometimes difrent drs the questions u need ansewered , and keep asking until u get an ansewer..don't be afraid to reask the same questions. Have faith and never give up hope.I will keep him in my prayers!!!
I am so glad I found this. I am 33 right now and was diagnosed with CVHG and IGA allergy when I was 24. They told me with how bad I was at diagnosis that I would only live for about 10 to 15 years. I had a baby at 19 and got super sick. 3 years later I had another baby and it was after that I found out what I had. They couldn't believe I lived through two births and told me not to have anymore. I got pnemonia 20 times in 18 months. It was aweful. I get really sick after every infusion for 1 week sometimes 3 weeks. It is so hard on my kids and husband but they are amazing. I have 3 kids now because there was a little boy spirit telling me he needed me. I just am at a loss. Thanks for the site!
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