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.

Bad Reaction...but to what?

I did my infusion yesterday and finished about 11:00 PM. Today I felt ok other than exhaustion and a headache...until about 2 hours ago (8:00) PM. Suddenly I started swelling all over my body and having breathing trouble. I'm afraid it's from the treatment, but hoping it was just a food reaction rather than to the treatment. I took 2 Benadryl and my breathing has improved somewhat, but I'm still swollen enough that my fingers feel stiff, and still a bit short of breath. I know I probably should do the epi-pen and 911 thing, but I'm afraid that the epi-pen will trigger seizures. I keep monitoring my breathing with my peak flow meter and even with the feeling of shortness of breath, I'm blowing over 400 on it (which is good). My tongue isn't swollen either.

I didn't eat anything that unusual today, except some home-made oatmeal/coconut/chocolate cookies that my mom put some milk in. I'm allergic to milk, but have never had this kind of reaction to it before. That was the most recent thing that I ate before the reaction, and the reaction came on very suddenly. I also had some spaghetti with fresh basil on it from my mom's plant, and while that's not something I have all the time, it's still frequent enough that I didn't think twice about it. I had that about an hour before I noticed the reaction.

The only other time I've had a reaction like this to something was to shellfish, and it was similar except this time I don't feel like my tongue's swollen. I'm wondering whether there's shellfish in the cat food.

Will post more later...

False Alarm?

Sunday, 11/19/10 2:00 PM

So...I ended up not going to the hospital, but took an additional 2 Benadryl and was finally able to sleep despite the fact that I was really scared. I feel a lot better this morning, and I realized that the Prednisone could have made me puffy instead of it having been an anaphylactic-type reaction. I know, I'm an idiot for not going to the hospital because they could have at least confirmed whether it was anaphylaxis or not, but I was afraid to go because I was scared I'd catch something. Silly huh?

I took my blood pressure when I was going through all of that and it was 140/89 which is very high for me, but it made me more comfortable to sleep because one of the danger signs of anaphylaxis is low blood pressure in addition to the other signs. It made me realize that maybe something else was going on and I calmed down. This morning I asked my mom whether I'd been puffy earlier in the day before I went to get her and she said my face was definitely puffy. That's another reason I think it was the prednisone. Also, I never felt like my throat or my tongue were swollen, and I think the prednisone explains that. 

I feel like a fool for getting so scared and putting my mom through hours of worry, but it was scary and I've had the swelling thing before with my tongue and throat swollen from an allergic reaction. I should have remembered that prednisone makes my face look puffy too though and calmed down when I realized my tongue and throat weren't swollen.

I do have a rash where the Tagaderm patches were, and that's something new. I took picture to show the doctors if they want to see them. 

So confusing...too many variables...

Update...

Monday, 12/20/2010 10:00 AM

I tried to call my doctor but they are out of the office until January...I guess that's the drawback of a teaching hospital. When I couldn't reach anyone there I called Accredo and spoke to both the head nurse and the pharmacist. They were both of the opinion that it probably was the prednisone causing the swelling because I didn't have a swollen throat or tongue. The pharmacist suggested that I take 20 mg the day of my infusion and then try either taking 10 mg the next day and 10 the day after that, or 20 the day of and 20 the next day. She said she's noticed a pattern to me having breathing trouble about 24 hours after the infusion and believes it to be a backlash reaction from the prednisone suppressing everything. She said missing a few weeks of my infusion wouldn't kill me if I'd rather wait to talk to my doctor. I truly think that's what it was though. 

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. 

Third Gammagard S/D Infusion

My doctor called yesterday afternoon, and told me she was calling in a prescription of Prednisone for me to take to help ward off headaches and breathing problems with my infusions. I'm to take 20mg 1 hour before my infusion and 20 mg more 1 hour after my infusion. 

I started mixing the Gammagard at around 5:00, and it took an hour and a half to mix this time because I paid more attention to it than I did last time. I rolled it gently between my hands. turned it over & over, and did that at more frequent intervals than the previous infusion. I took the first Prednisone when it was mixed. and took the 2 Benadryl at about the same time.

I've been having a problem with my infusion going too fast, and the pharmacy ships slower tubes to me, so I have a wide range of rate tubing (the only way to control the speed with my pump is by the size of the tubes...smaller tubes=slower infusion). Last week I used the 60ml/hr size tubing, and while it was better, it was still too fast, so today I got an inspiration for my infusion--I put 2 rate tubes in series to make the infusion even slower than last week! For those of you not familiar with the "in series" thing, it simply means that I attached 1 tube to the pump as usual, and then at the other end (where the needles would normally go) I used another rate tube, then attached my needles to the other end of that. The longer tubing makes the infusion slower than it would otherwise have been, so the whole infusion took about 6 hours.

This time when I primed the tubing, I clamped all but 1 needle off, and primed them all before putting any in my skin. Unfortunately, thanks to being dopey from the 2 Benadryl, I made a totally stupid mistake last night and forgot to unclamp 2 of my tubes before starting the infusion. I didn't realize the mistake until it was over, so I got the entire infusion in 3 sites instead of 5! I kept wondering why 1 leg hurt while the other one didn't. Thank God I'd slowed it down with the extra rate tubing, otherwise it would have been intolerable! 

I also had breathing trouble during the infusion despite the Benadryl &; Prednisone. It's so strange, I hope that goes away. The doctor told me that systemic side effects are rare with SubQ, but for some reason my body doesn't agree with that. The Prednisone did help with the headaches. I woke up with a slight one, and haven't felt too bad all day. I had a severe ice-pick type headache that lasted for a few minutes (normally they're just seconds), but it faded and I was ok. I took 2 Tylenol this morning for the headache though.

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! :)

Days leading up to Second treatment...

So I called my doctor and talked to her about my side effects that I experienced since the last Hizentra treatment.

She doesn't want to give me steroids because for the last year I've had IV Hydrocortisone every 3 weeks during my IVIG, and I recently had a 12-day course of prednisone. She said that it will have long-term negative effects on me and wants me to adapt and find other ways to deal with the side-effects. On top of that, I had some stomach bleeding a few months ago, and she's worried that the steroids could be a factor there. There has been some discussion in my support group about whether or not CVID patients should even have steroids due to the fact that the suppress the immune system even more than ours are already suppressed, so I'm not going to make an issue of it.

I'm not supposed to take Aleve, Ibuprofen, or Aspirin because of my stomach issues, but then she doesn't want me taking Tylenol either because it has a drug interaction with another drug that I take.

I felt much better on Wednesday morning...I still felt the threat of a headache and flu-like/sinus infection type symptoms, but I'd started using my neti-pot, and that has helped a lot. I plan to post the recipe that I use here. It seems to work better for me than oral antibiotics at getting rid of sinus infections sometimes. I found the recipe online.

Thursday...
Thursday morning I woke up feeling decent. No raging headache, and sinus symptoms pretty much under control. I got up and showered and began working. I started hydrating with water, smart water, and gatorade right away. After lunch I got very nauseous, but the symptoms went away after awhile .I thought maybe I'd been drinking too much water too fast, so I slowed way down. Then about 5:30 in the evening I got so nauseous again that I vomited violently and felt close to having a seizure. I took some Zofran (prescription sublingual nausea medication) and some clonazepam (prevents seizures). I was so weak and sick from the vomiting that I had to get directly in bed. My mom said I was very pale and neither she nor my boyfriend could find my pulse. I had to take Zofran again that night, and also took some acidophilus. I don't know what made me sick...maybe it was the CVID?...food poisoning?...delayed Hizentra reaction? Who knows...at least I didn't have any seizures and didn't have to go to the hospital either. I decided that unless I ended up in the hospital I would go ahead with my nurse appointment for Hizentra the next morning.

Hizentra Day!!

I woke up this morning and remembered that Accredo had sent me a box of latex gloves, so I called the number that they gave me (where I'd talked to the nurse coordinator), and asked them if the nurse could bring a box of latex-free gloves. The nurse coordinator actually answered the phone directly and told me that my nurse was reading my chart last night and noticed that I'm allergic to latex and decided to bring me some non-latex gloves of her own accord. I thought that was so cool! :)

I also asked about pre-meds since no one had mentioned them and I wasn't sure whether I should take anything before the nurse arrives. They told me that the nurse would have me take a Benadryl 30 minutes before we start the actual treatment, and that she would tell me when to do it. For my IVIG I currently get 50mg of Hydrocortisone, 25mg IV Benadryl, and 0.5mg Clonazepam, so it will be interesting to see how this goes with only Benadryl. They told me to go ahead and take Ibuprofen or some other painkiller, but I'm not supposed to take them due to stomach issues and drug interactions, so I'm just going to see how it goes without. I have had a headache all morning though, so I might give in and take something.

The nurse coordinator has called twice to tell me that my nurse will be later than expected. I don't mind, since she's helping someone else, and I know I don't want to be rushed through my treatment so I can't get upset about her not rushing someone else's. I also planned in advance to take the whole day off of work, so I don't have any pressures.

Seventh IVIG Treatment

Infusion #7

Friday, June 25, 2010 

I'm getting my infusion right now, and it's been kind of strange so far. My doctor cut the hydrocortisone down to 50mg rather than 100, and then told me to cut out the Ibuprofen. 

I got really nauseous and nearly threw up and fainted when they first started the infusion. I don't know why, but I know that it was anxiety this time. It may have been because they blew a vein for the first time, and that just kind of freaks me out and hurts like the dickens! Mom got me some ice for my head, and I had to cut out the built-in bra on my tank top because it was putting too much pressure on me and aggravating the nausea.

The nurse started me at 25 cc/hr and raised it to 50, then 75, then 100. When it appeared that I would be done in 4 hours again, she slowed it back down to 75. I'm a little annoyed because I'd told her that I wanted to take the full 6 hours for it to see how much better I'll get.

Mom made a point of telling the nurse that she's recorded my infusion start time as 10:05, but that's more like the time they started the Benadryl drip. When she complained about it, the nurse dropped the speed back to 50. I'm really trying to find out what works for me, and them trying to rush it and going all over the board for speeds is probably not going to show me what an "ideal" infusion can be like.

I'm really gearing towards Sub-Q now :)

So, the infusion finished at 50 and took about 7.5 hours. I felt great when I left and was able to walk out of my infusion center for the first time rather than leaving by wheelchair! I'm so happy about that! I feel normal or maybe better than normal and I have some hope that these treatments are going to work for me now. Yay!! :D

I think next time I'll tell them to max out at 75 & just leave it there.

Update

It's now the morning after my infusion and I feel the best that I've ever felt after one. In fact, I feel like if I were working, I'd have no problem going to work within a day or so. The only things I notice today that might be from the infusion are lower backache and a headache. I took a Benadryl before bed to help with any lingering side-effects.

I'm amazed!  

Tuesday, June 29, 2010 

I had my infusion (#7) on Friday, and as I said in the previous journal entry, I tried to keep the speed of it down. I can't believe what a difference that has made to how I felt afterwards! I mean, normally I have some trouble breathing, I'm a zombie, itchy, and can barely walk during and after the infusion. Then, when I get home I have to sleep for at least 3 hours, and I'm so weak and exhausted that I can't even lift a normal glass of water. 

By way of comparison, when I left the infusion center, I felt good enough to walk out on my own 2 feet, then I only slept for an hour afterwards. I went home with my boyfriend that evening, and wasn't even concerned about being far from the hospital. Then, Saturday I went shopping at Best Buy and bought a router and upgraded my cell phone...so I was there for 3 hours, and had no problems. I did sit down while upgrading my phone, otherwise I wouldn't have been doing so well. Sunday I went to a baseball game with my boyfriend. Granted, he took me in my wheelchair to help stave off exhaustion and I wore a mask, but nevertheless it was such a tremendous improvement over any other time at least since I've started treatments, but more likely for the last 3 years since my seizures started. I started getting tired towards the end of the game, but after we got home I still felt well enough to go for a swim (another first in 3 years) before dinner. All in all it was a wonderful weekend, and I'm very hopeful that I will continue to improve! :D