Ninth Gammagard S/D Infusion

I had my infusion yesterday at my boyfriend's house. I wanted to do it early so that I would be able to have a glass of champagne on New Year's Eve. I found out the rough way that if I have alcohol within a day of my infusion that it will make me feel truly awful due to the dehydration that it causes.

I noticed that I was feeling a bit dizzy and anxious when we were getting ready to do the infusion, so I asked him if he felt comfortable doing the infusion if I were a bit dopey on Benadryl. I had been feeling sick and tired for the previous 2 days, and, as usual, there are too many variables to really know what was going on. I may be fighting off an illness. I noticed that I never had the anxiety issues with the Benadryl in my system and that it would calm me. I only took one so that I would still be able to coach him as needed. He was wonderful, and did all of the needles for me. Some of the needles hurt due to the Gammagard having gotten into them, but it didn't phase me. I took the 2nd Benadryl after the infusion had started and took all the regular premeds at the appropriate times. I had to take my rescue inhaler periodically during the infusion, and more Tylenol (as I had a headache that wouldn't go away, but didn't take more Benadryl. The infusion took about 7.5 hours with me closely monitoring the pump to change the syringes.

Benadryl (AKA Diphenhydramine)

Tylenol

This was the first time I used the 6mm needles in my thighs, and at first I was excited because it seemed to be so much more comfortable than the 9mm needles. I stayed awake throughout the infusion and didn't put any serious pressure on any of the needles, but about halfway through my infusion one of the sites started stinging severely. I asked my boyfriend to look at it and he noticed that the Tegaderm was lifting around the needle, and when I got to where I could sit down and look at it in good light I saw that the site was actually leaking. I tried putting a pressure bandage over the needle to stop the leaking, and it seemed to work at first, but then it started up again. I had to clamp that needle off and finish the infusion in 4 sites.

Today the sites still look white and swollen (which is unusual for my thighs), and I've come to the conclusion that I need to go back to the 9mm needles. I think that the extra stinging is due to the fact that the Gammagard wasn't getting into the fat layer but was going in between layers of my skin. With the 9mm needles I would have leaking after an infusion, but never had leaking during an infusion. I'm glad that Accredo let me try the 6mm needles without taking the 9mm needles off of my order.

I took my second Prednisone this morning and have had some irritability and gloomy thoughts today, but I seem to be breathing well and feeling relatively well. I still don't know whether I'm getting sick or not. I sincerely hope not.

7th Gammagard Infusion...An Odd...One

So I had my 7th infusion today and something very odd happened. After the needles had been in for about an hour I noticed blood under the adhesive. It wasn't a lot, but enough to soak all the way out and stain my clothes after about 10 minutes. I called my nurse to find out what to do as I'd never encountered that before. I did check the sites before starting the infusion so I knew that it wasn't in a vein.

My nurse told me to clamp off the tube and apply pressure to the site (which freaked me out a bit since that seems that it would mean pushing the needle in even further). I applied pressure directly next to the needle and after about another 20 minutes or so I could tell that the blood was starting to coagulate and look darker, I'm not sure when the bleeding actually stopped though. After about 30 minutes I unclamped the tube again and let it run.

During the infusion I had more shortness of breath and tight-chested feeling than I did the previous time. I took my inhaler a lot, and ended up getting the jitters. This time I'd taken 20mg of Prednisone and 2 extra strength Tylenol ahead of time, but forgot to take the 50mg of Benadryl until the infusion had been going for at least 30 minutes. I forgot to take my inhaler for about an hour.

This infusion only took 6.5 hours because I made a point of paying attention to the syringe-change time. I wish I'd noted the correct time in my log so I'd know exactly how long it took, but I was very tired today (I've had trouble sleeping since Monday night), and I just absentmindedly jotted down 4:00 PM, but it may have been as late as 4:15.

I took another 2 Tylenol about 3.5 hours into the infusion, as I felt a headache trying to start. I took another 2 Benadryl at about 11:30 (after the infusion), and I'd taken the 2nd prednisone around 9:00. The prednisone is kind of touchy, as I need to take it with food, but I'm supposed to take it 1 hour before and one hour after. Instead I take it with lunch (or whatever meal I have as I'm getting things set up), then if dinner seems too close to the start time, I have a good-sized snack later. Tonight I had 2 snacks: a bowl of uncooked oatmeal with chocolate milk (yummy...you should try it), and some leftover spaghetti about 3 hours later. It's abnormal for me to get that hungry that soon after eating something substantial, so I think the prednisone is increasing my appetite. 

The infusion ended at about 10:30 PM, and it's now nearly 3:30 AM. I'm tired and I hope that I'll be able to sleep through the night. That's doubtful though since I drank over 100 Oz of fluids today and will probably wake up for the bathroom a lot.

Sixth Gammagard Infusion

I've had kind of a rough week, so I'm behind on writing about this. It's actually exactly a week later and I'm doing my 7th infusion right now, but I'll post about this one separately.

My 6th infusion went very smoothly. The whole thing took 7 hours, but I made it through the infusion with only 50mg of Benadryl as well as the 20 mg Prednisone, inhaler, and Tylenol. As usual I had insomnia after the infusion. I'm still not sure whether the insomnia is caused by the infusion itself or the steroids or both. I know steroids can cause it, but I think the infusion itself does too. I took 20 more mg of Prednisone towards the end of the infusion too.

I noticed a slight headache (if I remember correctly) towards the end of the infusion, so I took some extra Tylenol later. The next day (Saturday) I woke up feeling tired and weak, but not to the point that I've come to expect. It was much milder overall, and I was actually able to have a weekend where I felt almost like a normal person!

Thanksgiving and my Fourth Gammagard Infusion

On Thanksgiving Day I had 2 asthma attacks and a migraine. I was doing ok, but the candles at dinner, smoke on people's clothing and perfume all served to aggravate my breathing and I ended up having a very embarrassing (all public attacks embarrass me) asthma attack in front of my boyfriend's family. They were great about it, and helped me get some tea and water (tea helps loosen my chest), and were very considerate and kind about everything. I just hate being the center of attention like that, especially when I don't know people that well. I took 2 Benadryl, had extra caffeine in the hot tea, and took my inhaler probably about 3-4 times.

Then we went to my home for dinner #2 with my mom. I was doing better and pretty much held my breath from the car to the house because of all the chimney smoke in the air (I was wearing a mask too). I sat up in bed to recuperate from the attack until dinner was ready. I felt hungry but exhausted and nauseous. When dinner was ready I was so nauseous I could hardly eat despite feeling hungry, and I had a migraine on top of it. I took some Tylenol for the migraine. On the way back to my boyfriend's house, when he put the car's heater on defrost the smoke sucked into the car, which triggered attack #2. Luckily it wasn't as bad as the first one.

The Infusion

Friday morning I woke up with the headache, but it wasn't as bad as the night before. I got ready to start my infusion and had just put the first needle in when I started vomiting violently and came very close to fainting. I was so sick that I thought I'd have to go to the hospital. My vision became completely obscured by what looked like snowflakes, and I wasn't able to focus for probably close to 5 minutes. My boyfriend got me a Zofran, and totally took care of me; about 10 minutes after that I felt better. I don't know why I threw up, but I do know it wasn't related to my infusion. I had to teach my boyfriend how to do my infusion because I was not functioning very well; he put most of the needles in for me. I had taken a prednisone about 40 minutes to an hour before I vomited, and I was hoping that I'd have enough in my system to keep me from reacting to the infusion. I also took my inhaler, along with the 2 Benadryl and 2 Tylenol as premeds. Once again I put the 60 ml/hr tubing in series with the 120 ml/hr, and the infusion lasted for a total of 6 hours. I had some swelling and pinching at the injection sites, but never enough that I had to turn the pump off. I didn't have any breathing problems either! I took my inhaler again 4 hours into the infusion, and I had a very slight tightness in my chest at the very end and took my inhaler once more. Overall, I felt that the infusion was very successful! :)

The Next Day

Today I woke up feeling very weak and shaky. I had trouble sleeping last night after the 2nd prednisone and ended up taking 2 Benadryl again to help me sleep and prevent reactions during the night. I eventually fell asleep about 4:00 AM and slept fitfully til noon. My chest hurts a lot. It hurts to breathe deeply or laugh or cough, but I'm hoping that's just from the vomiting yesterday, and not my lungs. I'm eating very cautiously and very small meals because I want to avoid a recurrence of yesterday. I'm still in bed because I feel so weak, but I don't have a migraine, and the shortness of breath I've had seems more related to the chest pain rather than asthma.

Visit to my Neurologist

The week  following  the infusion I was able to see my neurologist about the temporary blindness that happened after I vomited. He believes that the blindness was caused by a migraine and put me on a blood pressure medication called Verapamil to help relieve the pressure in my head. He said that there's a migraine specialist who he knows at UCSF who treats neurological migraines (ones with strange things like blindness, numbness, the room spinning, etc) with the short-acting form of it, so I have to take it 3 times a day. The medication seems to be helping because I've been having fewer headaches. 

First Solo Infusion of Gammagard S/D!

The people at Accredo were great and sent me tubing to help slow down my infusion rate, so I expected things to be much better.

I made some rookie mistakes this time:

• Took my premeds 20 minutes after adding the saline to the powder. By the time I was ready to put the needles in I was pretty dopey from the Benadryl. It took 2 hours for the powder to dissolve completely, and another hour for me to get the needles in. Next time I need to take my premeds when I'm ready to start hooking up the needles.
• My nurses had suggested that I clamp off all needles but 1 to prime them, so I tried that this time. I primed 1 needle and put it in. Then I primed the next one, forgetting to clamp off the needle that was in me before pushing on the syringe. It hurt quite a bit to get the medication in at that rate, so I learned my lesson quickly. Probably wouldn't have done that if I hadn't been loopy from 50mg of Benadryl.

Throughout the infusion I had pinching and swelling at the infusion sites that I hadn't clamped off during priming, and had to turn the pump off twice for 15-minute breaks to allow the medication to absorb. It took me 3 hours from the time I started mixing the Gammagard S/D until I started the infusion, then the infusion itself took 5 hours due to using 60ml/hr tubing and taking breaks.

Because I'd taken 2 Benadryl 2.5 hours before starting the infusion, I took another 2 Benadryl 4 hours later and really struggled to stay awake long enough to swap out my syringes in the pump. After another couple of hours I had a slight fever (99 degrees, but my normal temp is 97-ish), headache, and was starting to feel achy in my lower back, so I took 2 more Tylenol.

After the infusion was over I felt a migraine trying to start at the base of my skull. I didn't want to take any more Tylenol, but I took 2 Aleve (not supposed to because of my stomach) and a Clonazepam to help relax my muscles.

Next Day...
This morning I woke up with a migraine threatening (not too bad though, I was still able to work). I took 2 Tylenol without it helping much, and took to Ibuprofen after a few hours when I wasn't getting much relief. I finally called my chiropractor and he was able to help a lot by massaging at the base of my skull and my shoulders and neck. I've been tired all day and worked on my laptop in bed because I didn't really feel energetic enough to sit at my desk all day. Nevertheless, I think this is the best drug for me of any that I've tried so far. My reactions to Carimmune and Hizentra were pretty bad, with the Hizentra being worlds better than the Carimmune. I really believe that my non-existent IgA is a huge factor in how they affected me.

Two Days After Third Hizentra Treatment

For the Third treatment I took 2 Benadryl as recommended by the specialist, and also took 1 Tylenol. By the next morning all of the swelling in my belly was gone and I only had 1 tiny knot. I was so excited because that was so much better than the previous 2 treatments! I felt like I had some energy and got up, showered, and went about my day. By about 4:00 PM I felt really tired, and my belly had swollen up again and was starting to hurt. I put ice on it, and it really didn't seem to help other than soothing the itching. Knots started forming at the injection sites, and increased in size and pain until they were about 1.5" in diameter. Only 1 site did not develop knots, and I have no idea why that is. I wonder whether the ice contributed to the knots forming, or if my jeans irritated my stomach and caused them? I have no idea...

I realized that I probably should have taken more Benadryl the night of my infusion, that might have prevented the swelling and itching the next afternoon.

Appointment with Primary Immunodeficiency Specialist

Today I had my first appointment with a true specialist in immunology at a local research hospital.

He's a pediatric immunologist, but sees adults once a week. I saw a fellow first who was very nice and also seemed very competent, she asked me a lot of questions about my history, especially regarding infections. Then the doctor came in with another fellow and sat down, talked to me in-depth, and very patiently answered my questions.

The doctor is concerned that the original tests that I had at diagnosis were not sensitive enough to detect how low my IgA actually is. My test results show <25, but that just means that my IgA is lower than the test is capable of showing. Because I have a history of bad reactions to both Carimmune NH IVIG, and have had a few bad reactions to Hizentra SubQ, he suspects that I might have an IgA level below 6. He also said that if that's the case, and I keep having Hizentra or other high-IgA gammaglobulin treatments, my body could make IgA antibodies which would mean that I could eventually have very serious reactions like anaphylaxis to the treatments.

He plans to run more in-depth and sensitive tests on me to check out my IgA level, see what antibodies I have for various diseases, test for RA and Lupus, and see what I have in terms of Candida. There was probably more, but I'm having trouble remembering.

I am very excited about being able to see this doctor. I believe he will help me a lot! He recommended that for my next Hizentra treatment I take 2 Benadryl, and 1 Tylenol and see if I do better.

I will keep the Fioricet as an option later if I get a migraine, but because it has Tylenol in it, I would have to wait hours after taking the Tylenol before doing that.

He kept reiterating that CVID is actually a "blanket name" for many different diseases, and they will test me to find out more about what I actually have. I'm so excited! :D

My Questions for the Doctor, and his answers:
(Note, I may have not fully understood his responses, or not written fast enough and can't remember exactly what he said (I took notes as he answered my questions). This is my best summary of what he said to me today. I hope it helps, but check out what I have here with your own doctor)

Questions for PI Specialist

Question: Should I take steroids (or any drug that lowers the immune system) ever? (eg: nasal sprays, inhalers, eczema creams, prednisone, etc)

Answer: Yes, as needed for asthma, allergies, autoimmune disorders, etc.     Avoid over-use of oral steroids due to them affecting the blood

 Question: Should we get treatments while sick?

• What about with an infection or while on antibiotics?

Answer: Yes, unless you have a high fever which might mask a reaction to the medication

Question: Should I stay away from children (some doctors have said that I should)?

Answer: No, but use caution. If they are sick, wear a mask, keep distance, and wash hands frequently

Question: What is your "goal" IgG level for me?

Answer: Based on individual, but around 800 for me. He also said that some people are fine at 600, but he doesn't think that I'm one who would be.

Question: Will you test me for IgA antibodies?

Answer: Possibly, if I have very low IgA (under 6.0)

Question: If I have IgA antibodies is it still safe for me to have Hizentra?

Answer: No because I will make antibodies to the IgA, and will then be more likely to have a very serious reaction to my treatments.

Question: Is it safe for me to receive a blood transfusion?

Answer: It is unless I have very low (under 6.0) IgA, in that case a blood transfusion could be very dangerous

Question: Can/will you do genetic testing? I think there’s a family history...

Answer: In my case, yes because I’ve been sick for so much of my life. A very small percentage of people have the defective TACI gene, and the people with the other associated defective genes are even more rare. He will test me for the TACI defect.

Question: Is it possible to have CVID your whole life, but have your immune system crash at some point where your numbers fall, or will they always be low?

Answer: Yes, but because I’ve been sick for so long that’s probably not the case.

Question: Do you treat the patient or the IgG levels?

Answer: He cares about the patient’s well-being, and some patients are good at lower levels and there are treatments other than IgG replacement that work for them. He does not think that is the case with me.

Question: Are you currently researching CVID?

Answer: Yes, he has a budget for researching CVID and is accepting patients for the research

Question: How will you help me regain my quality of life?

Answer: By finding a more appropriate treatment for me.

Question: Can CVID patients make antibodies?

Answer: Yes, especially for things like Lupus and RA

Question: Can CVID patients  test positive to things like Lupus, RA, etc?

Answer: Yes (see above)

Question: Should CVID Patients wear a Medic Alert Bracelet?

Answer: Yes, especially if they have very low IgA, then they should specify CVID – No IgA (or something like that)

Question: Should I have antibiotics for dental appointments? (Fillings, etc)

Answer: No, the primary reason for that is to avoid heart valve infections.

Question: Can we eat honey safely? I read that it’s an issue w/ babies because of them not having enough good bacteria in their intestinal tract, but ours can be wiped out by antibiotics and other drugs...

Answer: Yes

Question: Should we get vaccinations?
Answer: There's not much point because of the vaccinations being covered by IVIG/SubQ--except the most recent flu vaccination. You especially need it if you're on Hizentra.

Question: Is a problem with the innate immune system (limited/no swelling, low body temp, diminished pain response) related to CVID?
Answer: Yes, the innate immune system is (can be? don't remember which he said) linked to CVID, but they don't know how yet. I also don't remember whether he said for all CVID patients, or just some. I do remember him saying that they see things like low body temp, and not swelling in some CVID patients. He also said that some patients don't heal at the normal rate.

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If any doctors who saw me today disagree with the information I provided here, please either send me an e-mail or write a comment below. I will correct the information right away. Thank you! :)

Fourth Day After Hizentra

I woke up this morning and my headache had returned with a vengeance. It was nearly as bad as when I woke up Saturday morning, but not quite as intense. I really regret not hydrating...I think I learned my lesson!

I called Accredo's 24-hr helpline, and they put me through to the pharmacist at my local branch. The pharmacist suggested that I talk to my doctor and:

1. Take Tylenol in addition to the Benadryl
2. Use the skin protection patches to see if that helps
3. Call my doctor to find out whether she can give me a Rx for prednisone that I can just take during the infusion. She said I need to tell the Dr about my reaction including the flu-like symptoms.
4. I already take Rx allergy meds, which was her other suggestion.

Immediately after hanging up with the pharmacist the phone rang again, this time it was my nurse. I thought she was calling because of my call to the pharmacist, since they're out of the same office, but it was just a coincidence. She was calling to make arrangements to see me on Friday for my next infusion. We talked about my side-effects and she mentioned that shorter needles might help me. I guess if the needles go into the muscle, they can cause more of a reaction and trigger some of the inflammation that I experienced. She said she will talk to the team about what I experienced, and see what they can do to help me.

Later...
My nurse called me to say that she talked with the team at Accredo, and they decided that it would be best to slow my infusion down even more. I'm absolutely in favor of that since infusion speed was a huge factor in how I felt after my IV infusions. She also reiterated that as my body adapts to the treatments I should experience fewer side effects. She said that they're concerned that if they use shorter needles there's a potential that they might miss the fat layer and pump between layers of my skin--which would be a problem, so they're going to stick with the current needle size. I haven't yet received the new tubing, but I hope that I will get everything tomorrow or Thursday at the latest.

I also saw the chiropractor today to get help fighting the headache. He found a lot of really tight muscles in my back, neck and head that were contributing to the intensity of the headache. He worked them over and told me to rest. He also said the heat on my neck and shoulders was better than ice, and would help with the headache.