Inherited Common variable immunodeficiency (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 undiagnosed 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)

This is not a cure, but it strengthens immunity by ensuring that the patient has "normal" levels of antibodies, which helps to prevent recurrent upper respiratory infections.
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 Inheritance
Genetic 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

Happy Easter

Love is my Easter Basket

Handle in hand it swings in steady movement as I walk it sways
Sweetness as candy a child's dream
Nibble its' goodness
Joy for every day
As a bunny prolific life it stays
for love of a Easter Basket

Donetta

I will soon be able to post again. Typing is getting easier. In the last 6 months I have had both of my shoulders repaired. The labium was torn off of both. They drilled holes and set screws into the bone. Then they stitched the labium to the screws. It has been a very long road of healing.
The childhood tares tore up to the top of the shoulder and stopped at the ligament. The recovery of the total knee replacement tore then the rest of the way. From arm pit to tip of each shoulder. I am now seven weeks out from the second surgery.
I have love in my basket for all things become new. Newness is often a painful journey. Love sees us through.

Know that you are loved simply for who you are.

Patricia left a comment

 Yes dear Lady it can be very lonely. I think it hard when folks see me the week after ivig. I look so normal it is like nothing is really warranting the isolation. They can not see the risk to our lives. It took a long time to teach those around me why I insist they wash their hands. I have two kids in school yet. Each day I risk exposure. 
There is a gift to loneliness and that is to reach out as you have. I am here. Only one woman but with a heart of compassion. I have been recovering from surgery of a torn cartilage in my shoulder. Now having to go to P.T. I risk. Yet I ask if he has washed his hands and he is careful of it for. Last Friday I masked, it is so awkward to do so. Saturday was infusion day. I am stronger.
I too worked with kids, never gave birth yet adopted two. Almost dieing after both trips to Russia from becoming terribly ill.
Patricia Commiseration is good for the soul.

Patricia has left a new comment on your post "Inherited Common variable immunodeficiency (CVID)":

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!

Frugality is timeless

 Do you ever purchase just to have the latest and greatest of gadgets? Consider the humble $20 coffee pot. We had our four cup pot for a whopping 30 years! Monthly cleaned with vinegar and a good triple flushing it served us very well. My darling and I only share a pot on the weekend, otherwise i may have a cup or two every other day or so during the week. A four cup pot is a self regulating measure as to not be over caffeinated. If we needed more for company we just brewed two pots.
 After my sweet man tore it apart and determined it was a goner we opt for a new one.

We like things to be compact and so we opted for a new $16 pot to replace it. No fancy bells or whistles to break down. Living with simplicity is a frugal option to think about. We do the same thing in many of our purchases.
A coffee pot with good maintenance lasting 30 years is really not that surprising if the design is simple. Money well invested even in the small purchases will take your dollar far.

 In the refer freezer was kept all the pre-made or ready made meals. The fast foods the family used during my recent down time from the shoulder surgery. The old refer is still running just fine. It is used for sodas and over flow storage.

In the spring we decided to retire our 25 year old refrigerator to the garage and get a new one. We waited on that purchase and did a study  to discover all the pros and cons. We saved a long time and bought a large capacity french door with freezer on the bottom. This has really been a back saver!

Being frugal is to save up for the best you can afford to budget for, research and learn about your item on line. Utube and other sites offer user reviews. Avoid extra features when your able, it is most often the mother board computer parts that gives out. They are $$$ to replace. Fewer extras also mean no need to concern yourself with extended warrentees.

CVID infusion tip

Wow what a difference dilution has made. Friday I had my monthly IVIG. We changed it up from 40/400 to 40plazma/800water that is full dilution. The day was wonderful. I have the best nurse/friend. She took me to treat me to a pedicure and manicure during infusion. I have a fanny pack that holds the iv bag and the pump. Having the port is wonderful, we used a folded wash cloth in my shirt so the sling did not rub on it.
Even still being in a sling we were able to have a really nice day. We discovered that the water dilution is so much easier for my organs to absorb. NO side effect at all!
So if you notice the thickness of infusion ask your doc about diluting it. Really made it like nothing occurred. Even today I was full energy.