Living with CVID (hypogammaglobulinemia) Helpful Hints
Illness and Diagnosis
I am creating this blog to help other's who have been diagnosed with CVID. There does not seem to be much information out there that is helpful. The doctor also has limited anecdotal information to help with the side-effects or help mitigate the impact of living without one branch of your immune fighting system.
Hopefully this is helpful to others. Your comments are welcome and appreciated as all of us suffering from this and are searching for the switch to reactivate our B-cell immune system.
There is hope. My doctor reports he has one well-documented case where the male patient's immune system turned back on. So that gives us all hope and purpose to keep searching. My guess is that all of us have different reasons that our immune system failed. So this is a personal journey. However if enough people provide their input, we might find some common linkages that point to solving our system's trigger.
Diagnosis Year 2002: Short History
I was diagnosed the year of my 40th Birthday. That year, I was routinely sick from infections, mostly pneumonia. I also had severe ear infections whereas one led to Bells Palsy. Each of these episodes would require trip to doctor and antibiotics.
I was getting sick over silly things. I would work in the yard for 2-hours and then have a 104F fever for next two days. I taught Sunday school and if one kid had a cold, I would get a fever within hours. On vacation at beach, ocean water entered my ear canal. That lead to a horrible ear infection. The ear infection ballooned into Bells Palsy – inflammation of the cranial nerve VII leading to paralysis of ½ of the face. I was like the Joker on Batman…only ½ of my face could make expressions. I could not pucker my lips; speaking certain words was also difficult, only half of my tongue could taste and my eye would not shut when I slept requiring me to tape it close at night. It was a wild experience. The doctor started me immediately on a steroid which reduced the long term impact and helped me fully recover over the next 4-6 weeks. Today, some ear pain still lingers and my right eyelid does not completely close when I sleep, but this is all minor.
I believed I was allergic to something in the mulch or yard. I made appointment with Allergist who was also Immunologist. He gave me vaccine for pneumonia, a battery of allergy tests (all negative) and asked for me to have blood sample drawn at local lab. Completed the blood draw same day (Friday). On Saturday, Dr called me and blurted out the dreaded words: “Acquired hypogammaglobulinemia.” He said I did not make any antibodies to the vaccine. One branch of my immune system was basically “turned-off” – the bacteria fighting branch of the portion that makes B-cells.
He said I must begin treatment immediately or risk sepsis (bacterial infection of the blood) which is almost certain death.
First Treatment Method: Intravenous Method (sorry no pictures of the equipment)
This method is where highly diluted immune globulins are main lined directly into the vein. For intravenous infusion of human blood product, the treatment required a nurse to administer. Good thing. I traveled through the list of reported reactions like a textbook. The worse of these was the deep bone shakes (like uncontrollable chills) in addition to extreme back spasms. I would try to relax into the chills in order to prevent the spasms from taking place. It is a horrible place to be.
Type of Human Immune Globulin: GammaGuard by Baxter.
Years using this method: 6-years
Pros of this method:
1) Infusions are 1x per month. Take about 3-5 hours.
2) Allows you to travel and not be a slave to weekly B-juice infusions
3) First few weeks, you are “hot” with immune fighting cells. However see “cons” of this as well.
4) Day after import, you feel great, strong – almost like superman. I exercise regularly and after infusion, I am always strong, faster and have more endurance. Maybe it is the liquid. Either way, I always liked the boost.
Cons of this method:
1) Risk of extreme reactions during monthly infusions (as outlined above)
2) Dealing with “peaks and valleys” with immune fighting cells are physically noticeable.
3) Discovered I encountered fatigue and more susceptible to infection when nearing the end of monthly infusion.
4) Veins are stressed out during infusion. Very painful at site of infusion. You have to find a large vein that can handle the amount of liquid pushed into the vein over 4-hours. I tried many different sites. Finally settled on major artery on forearm (see picture). I tried hand and wrist. The arm was a reliable artery that I used for years after discovering it.
5) After infusion, I experienced extreme moodiness. I am a very easy going person, but after GammaGuard infusion, I was very irritable and noise sensitive. Family beware – dog as well.
6) It is a great deal of drama. You have to schedule a nurse to administer; it takes 3-5 hours depending on seriousness of reactions; my nurse became a good friend, but if you get stuck with someone you do not like, it can be a long “visit” every 30-days.
Helpful Hints using GammaGuard Intravenous Infusion:
To minimize the reactions from intravenous infusions, I learned a few tricks during that period for those using this method. Hope this is helpful.
1. Take Benadryl one hour before infusion and one more tablet immediately before infusion.
2. Take one Tylenol immediately before infusion.
3. The nurse’s protocol was to slowly increase the pump’s flow rate every 15-mintues after taking blood pressure and pulse up to a top speed of 200ml/hr.
4. To minimize extreme physical reactions, at one hour into infusion, stop the pump. No matter if no reactions were manifesting, stop the pump for 15-minutes and allow body to rest. I only have theory for this, but it seemed like it gave my existing imported immune system time to either be overwhelmed by the new – think “new sheriff is in town”; or time for the previous existing B-cells and the new B-dells to accept the presence of the other (think of this as getting to know each other as "safe" ). In my experience, it is always best to prevent the reactions from manifesting versus letting them develop even if just barely perceptible.
5. Sometimes, I experienced ZERO reactions. I cannot explain this. It appeared to be totally random.
I experimented with many other methods and meds to minimize the reactions. For example, I injected Benadryl (prescription required). This knocked me out, but did not stop the bone chills or spasm. At one hour, I would jump from couch and suffer the reactions.
More Tylenol, more oral Benadryl: increasing these over the counter meds did not prove to be more effective. So I kept to a minimum – no sense taking something that you do not need.
Intravenous entry point
Subcutaneous Human Immune Globulin Method:
This method involves injecting into fat tissue (subcutaneous) at multiple sites. The idea is that immune globulins once in the fat tissue will absorb into the body and perform as expected. This is my preferred method now. Only one company is FDA approved for this method.
[the pictures above show the equipment and vials of human immune globulin].
Type of Human Immune Globulin: VivaGlobulin by CSL Behring.
Years using this method: 2+ years (and continuing today)
Helpful Hints on this:
Pros using this method:
1) No “peaks and valleys”
2) No mood swings after weekly infusion
1) It takes about 1-hour, 1x every 7-days or more often depending on your doctor’s orders.
2) I don’t know, but sticking yourself or having someone else stick you at multiple sites (I request 6) is something that is psychologically difficult to look forward to each week. It is kind of a drag. I have made Sunday my infusion day. I do it in the morning.
3) You need weekly injections. So if you travel longer than 7-days, you need to make arrangements. I understand Behring is working on a shelf stable version which does not require refrigeration. I will try that, but I worry about the preservative that must be used to keep the B-cells viable. Usually preservatives and I do not mix well.
4) Painful “quarters” at site of infusion where the B-juice is injected in large quantities under tinto the fat. When removing, some will bleed and seep fluids. But within hours, all is well. You might feel the quarters on the hind side when sitting. These quarters only last for 24-hours or less. Then all is well.
Managing without B-Cells
Managing without B-Cells:
Living with a missing branch of your immune system is challenging. Here are some things I have learned along the way that may be helpful. The challenge is to avoid bacteria, at least the bad ones.
1) Two-thirds of your immune system is dedicated to your digestive system. So be careful what you eat. I find that salads often cause diarrhea for me. It is important for the lettuce to be handled properly to limit bacteria growth.
2) Freshly cut fruit is fine, but avoid buffet service. The fruit has been given sufficient time to grow bacteria.
3) If in doubt of food handling, only eat foods that are well cooked. This includes meat. Medium-well is best for me.
4) I always carry Purell hand sanitizer with me. I avoid surfaces that have public traffic (door handles, knobs, etc). However, with having hand sanitizer handy, I don’ make a big deal out of this. Being aware of “when” you touch something is the secret. Then simply squirt a drop or two of Purell into your hand and all is good again.
5) I travel extensively without any issues. I have been in some dirty cities throughout Mexico, Thailand and China. So I don’t let the deficiency curtail my lifestyle.
When checking into hotels, I immediately sanitize the common areas with a special sanitizing cloth. I purchased this from a medical supply retailer [picture]. One wipe and everything is sanitized. Be sure to include toilet flush handle, remote control, phone, TV controls, all door knobs and light switches, including bedside lamps. I also wipe the desk chair seat and back. I just don’t trust what people do in those chairs. I think I would do this with a perfectly intact immune system.