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.

Eighth Gammagard S/D Infusion

I had my infusion today, and overall it went pretty smoothly. I tried new areas and shorter needles today, and 1 site leaked during the infusion. I also vomited again before starting the infusion and I'm starting to think that's due to a combination of migraine with mild nausea, PMS and a reaction to the first needle hurting and maybe a bit of anxiety just to make things fun. Both times that I vomited were about 5 minutes or less after having the first needle was put in and it hurting. I don't know why that would be a factor, I mean, I don't faint or throw up when I get my blood drawn or IVs, so why should this be different? I also don't vomit every time a needle hurts. It doesn't make sense that it's just anxiety, but perhaps a combination of all of the above just makes a bad mix. I've also started hydrating with 50% Gatorade and 50% smart water in case the Gatorade has been contributing to my nausea.

I had to put the needles in a place other than my thighs today because I have a rash all over them from last Friday's infusion. This time I chose the love-handle area and my lower back. I couldn't see to put the needles in so my mom had to do most of them. I took a nap and laid on some of the needles (they didn't hurt since they're the 6mm ones) and the one with the most pressure leaked onto my sheets. I have what looks like a water stain on my sheets and I hope it will wash out. I finally just clamped off that one site for most of the infusion since it wouldn't stop leaking. None of the other sites leaked. Although none of the needles were in my stomach area, my belly still bloated from the infusion. The needle sites aren't painful now, but I'll have to see how they are tomorrow. I hope the rash on my legs goes away soon because I really prefer my thighs....I wonder how the calves would be?

I'm doing pretty well. My pharmacist suggested that I take 20 mg of prednisone when I first eat tomorrow rather than tonight, so that might prevent the back-lash reaction that I seem to get the day after my infusion. I'm going to give that a shot...she also said I could try 10 tomorrow and 10 the next day...we'll see how tomorrow goes.

Update...
Thursday, 12/30/10

I didn't have any bad reactions the next day, and I've come to the conclusion that the pharmacist was right about the Prednisone causing a backlash reaction. The Prednisone affects my mood and makes me irritable, but it's better than migraines, breathing trouble, and rashes. I felt pretty good during the week and was able to have a nice Christmas. I think we are finally on the right track! :D

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. 

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!

Appointment with Immunologist

Last week I saw Dr. Lewis at Stanford again and he gave me the results of all the tests that they ran last time. IMy IgA was undetectable, IgM was up, and IgG is now over 1500! They said they don't want it to go higher. I responded well to Diptheria, Tetanus, Pertussis, and had normal results for Tuberculosis.

Dr Nelson, the Fellow who works with me most, said that they might run the IgA antibody test on me, at least, I think that's what she said. At first I thought she meant they'd run a test on me to see if I'm allergic to IgG, which she said I could be since I'm having so many reactions to my treatments, so I'm really not certain what test they mean to run.

Dr Lewis recommended various specialists for me who at least know him, and hopefully are familiar with CVID too.

We discussed ways to deal with my treatments, and for now we're going to continue as is with taking my asthma medicine regularly, taking my rescue inhaler 20 minutes before treatment and again 4 hours later, taking 2 prednisone, 2 Tylenol, and 2 Benadryl. I get exhausted just writing about it. ;) He also recommended that I start pushing hydration, even harder than I normally would for an infusion, starting the day before and continuing for 2 days afterward.

If those suggestions don't work, then they will add in using my nebulizer before/during treatment, and that would mean I'd also have to take Clonazepam to stave off seizures. What fun! I'm hoping and praying that it won't come to that. If that doesn't work, then they'll move on to about 20ml treatments / day probably for 5 days of the week. If I still have problems with that, then they will take me off of Ig therapy and put me on prophylactic antibiotics.

So, now that I know all the possibilities, I'm more coming to terms with my fears over the worst case scenario. Dr Lewis thinks that I should adapt to the current treatment and that the realistic worst it will be for me is to add in the nebulizer. That's much more acceptable to me than trying to wing it with antibiotics.

Here are some of the questions I asked while I was there:

Question: Can I eat sushi safely?

Answer: Yes, although eating exclusively saltwater sushi is safest, and salmon is the most dangerous.

Question: What wording should I use on my medic-alert bracelet?

Answer: IgA deficient with CVID

Question: What is IgM?

Answer: IgM is the body's first line of defense against infection, but IgG covers for it.

Question: IgA is for protecting our eyes, mouths, and mucus membranes from bacteria/viruses, do I need to be extra cautious of infection in those places due to not having IgA?

Answer: IgG will protect those places also

Dr Lewis told me that many of his CVID patients get daily migraines, and also migraines that precipitate IVIG. He recommended a drug called Diamox, but when I mentioned it to my neurologist the neurologist said that Diamox is for brain swelling, and gave me Verapamil instead. Apparently Verapamil is good for neurologic migraines.

Gammagard 5 (almost sounds like a band name)

I had my fifth Gammagard infusion yesterday. It went pretty well, and there was no vomiting this time. We got off to a late start with it, and so the infusion didn't start 'til 6:00 PM, then I spaced out on changing the syringe halfway through, and so the infusion didn't end until 2:00 AM. I think I need to set a timer for changing it.

I took my benadryl at the very start of the infusion. That's the best way that I can insure that my brain won't be foggy when I'm setting everything up. I'm a small person, and 50mg of benadryl is mentally overwhelming for me. I have a hard time following a plot on a tv show on 50mg of benadryl, let alone giving myself an infusion!

This time though, my boyfriend put all the needles in me. He's squeamish about needles, but does it to make it easier for me. I very much appreciate it, as it relieves a lot of my stress.

I had some breathing trouble during the infusion, but it was very slight and I took my rescue inhaler at the start and every 4 hours during until I went to bed. I also took another 2 benadryl and 2 tylenol 4 hours into it.

Today I feel weak and tired, but after 4 benadryl yesterday, I'm really not surprised by that. I'm continuing to hydrate well, and don't have much pain other than waking up with a headache that more tylenol seemed to help. I've pretty much spent the day in bed.

I'm continuing to hope that my infusions will get better and easier and I'll start needing less medication during them. 

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.

Doctors and Insurance Companies

Yesterday I was chatting with a friend who has been fighting with her insurance company for a year to try to get coverage for infusions, and she told me that they had finally agreed to cover her for a year!!! I'm so happy for her because she's been so sick for so long, and constantly had trouble finding doctors who would help her. It shocks me that so many members in my favorite support group have had doctors refuse to treat them once they found out that the person had CVID! Makes me think they swore a hypocritical oath rather than the Hippocratic oath! Sometimes these doctors will admit that the case is too complicated for them...but don't they consider referrals at least??? I mean, we CVID patients are desperately seeking relief from pain, gastrointestinal problems, breathing problems, skin problems and doctors just say "You're too complicated, get out of my office!"? What's wrong with this system?

Visit to the Pulmonologist

I went to the pulmonologist today and mentioned that I feel like I'm starting to come down with something. He asked me what my symptoms are, and I told him I feel a bit congested in my chest, I'm spitting up yellow/brownish phlegm in the morning, I have an urge to cough but am not coughing much yet, and I'm exhausted. Based on those symptoms and the fact that I need to avoid getting any more lung damage,  he prescribed a 10-day course of antibiotics for me. Unfortunately, it's one of those antibiotics that requires avoidance of all calcium and magnesium, so I have to quit all vitamins for 10 days because the warning on the label says "Do not take antacids Iron, or Vitamins/Mineral Supplements within 8 hours before and 4 hours after this medicine". I expect I'll be really tired without my vitamins!

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.

My First Gammagard SD Infusion...or....When You Feel Ready to Give Up On People, Read This!

Today I did my first infusion with Gammagard SD. I left the supplies and Gammagard in the box until yesterday morning, then I decided we were close enough to infusion time to set things out. I pulled out the gammagard boxes and got a bit of a scare. I knew that the medication was in powder form, and one of the ice packs had burst inside the styrofoam ice chest that it was shipped in. The Gammagard was packaged in plastic, but nevertheless, the ice-pack goo had soaked into the boxes--at least two of the boxes were moisture-soaked and splitting open. I was honestly afraid that the powder would be ruined and I wouldn't be able to do my infusion. I called Accredo to tell them what happened, and spoke to the warehouse manager. She informed me that the medication would not be compromised because it was sealed in a bottle within the package. I opened it up while on the phone and confirmed that the medication was all fine.

It was really interesting mixing the Gammagard. It is a powder that comes in a glass bottle with another bottle that contains sterile IV water. The nurse had me transfer 96mls of water to the powder bottle using a 60ml syringe. After combining the two, we had to let the powder mix in to the liquid. We couldn't shake or stir the powder in, and simply had to roll the bottle to gently mix the powder in. It took about an hour from the time that we combined them until the Gammagard was ready to infuse. The total volume that I infused today was 110 ml, and I'm honestly not sure how many hours it took.  The rate tubing that they gave me was very slow (as subQ rate tubing goes) at 120ml/hr, yet the infusion was much too fast for me. My legs (where I put the needles) were stinging and turned red and swelled significantly, although it probably wasn't as bad as on my stomach. I also was having some breathing trouble. When I showed my nurse the infusion sites, she had me turn off the pump because she said it was going too fast and that I wasn't absorbing the medication fast enough to keep up.

So...in addition to my infusion, my mom was having a crisis. For about 10 days she's been having trouble walking with one leg hurting and feeling heavy. Suddenly, yesterday before the nurse arrived, her leg started hurting so intensely that she couldn't put any weight on it. She came to my room using a carpet sweeper as a crutch. I had her lie down on my bed and we propped her leg up on pillows. I didn't know what to do as I was unable to drive (license taken away due to seizures), and I couldn't leave before my nurse showed up. When the nurse arrived I filled her in on my mom's condition, and she was concerned too. She said that if no one else could take mom to urgent care, that she would take both of us in after we got my infusion started.

The nurse arrived at 3:15, and because of waiting for the medication to mix and some things I had to do to get ready, it was at least an hour (maybe 2) before we started my infusion. She wheeled my mom out to the car in my wheelchair, and I put together an emergency bag for my infusion to take with me. Because I was infusing 110ml of fluid and the pump only accommodates 60ml, I had to take an extra syringe full of medication with me and ended up swapping out the syringes in the office at urgent care. We were at urgent care for 2-4 hours, and then sent to the ER. My nurse then drove us over to the ER, and she told the check-in nurse that we had to leave my mom there (mom was all for this) because of my immune deficient state. My nurse insisted that we get something for mom to eat before leaving, as by that time it was about 10:00 PM and none of us had eaten. She and I ran over to In & Out (nearest open place selling food) and she bought dinner for all 3 of us, then as we got in the car, a man knocked on her window and asked if she could jump-start his car. She turned the car so that the headlights were on his car and gave him a jump-start. She was very smart about it though, she popped the hood of her car from the inside and locked the doors. Turned out he was just an  honest guy with a dead battery.

After we dropped the food off with my mom, my nurse took me home and then headed home herself. I couldn't believe how gracious she was about everything all day, and she maintained a good sense of humor too!

I was worried about how my mom was going to get home. My brother lives in the area, but I couldn't reach him by phone, and my boyfriend lives 50 miles away and would have come except that he was sick and in pain himself. I'd given mom cab fare, but wasn't sure how she was going to get in when she wasn't even able to walk. I heard one of our roommates get home, and I told him what had happened and asked him  if he happened to hear her outside if he would let me know (his room is by the front door). He promptly offered to go pick her up and asked me to call her. I did, the doctor had just finished seeing her, and they released her. He went to pick her up and they got home about 1:00 AM.

People were really wonderful yesterday! 

About 2:30 AM I started getting a migraine (despite lots of hydrating in advance), so I took a Fioricet, and aside from some weakness and tiredness, I feel pretty good today!

IDF Patient Education Meetings Throughout the Country!

IDF Patient Education Meeting Join us for a one-day educational meeting presented for individuals, families and health professionals. IDF’s Local Patient Meetings offer an introduction to IDF, and feature local immunologists and allergists addressing the treatment and management of primary immunodeficiency diseases. These meetings are the perfect occasion for patients and families to meet and share ideas. Meetings are happening soon in different locations throughout the country! RSVPs seem to be required 3 days in advance, so go to the website and find one in your area. (Sorry, I didn't realize that one happened yesterday, but others will be happening Nov 6th and later). http://www.primaryimmune.org/IDFCalendar/default.htm Update The meeting was great! It turned out that my specialist was the guest speaker, and it was great to meet other CVID patients in my area. My doctor spoke about the various problems that CVID and PIDD patients encounter, different conditions associated with the disease, and the genetics involved. I video taped a lot of her presentation, but I wasn't feeling strong enough to hold my phone up to record, so the video isn't great, and the main reason to "watch" it would be to hear the information. However; I didn't get her permission to release it online, so I will have to do that before posting it here. If I'm unable to get her permission, I may be able to transcribe some of it.  The IDF invited representatives from pharmaceutical companies who had free gifts for attendees. Some of the things they gave away were visors, water bottles, pens, thermometers, and antibacterial hand sanitizer. In addition, the IDF representative provided pamphlets and books with information about PIDD, including a children's booklet that is very informative. I believe these books and pamphlets can be ordered from the IDF at the their website (see my reference page).  The meeting included a buffet continental breakfast as well as lunch. All in all it was a very enjoyable and educational experience, and I'm very glad I went!

I Just Got Approved for Gammagard SD!

My nurse from Accredo called today to schedule an appointment to train me to use Gammagard SD as subQ! I'm so excited! She will come next Thursday 11/4. She said to expect her late, which is fine with me because I will be working that day.

The pharmacist from Accredo also called and she said that I will be getting 10g of powder once a week. I don't know what volume that will reconstitute to, but she said I will still be using the Freedom 60 pump (which only holds 60ml of fluid, the same dose of Hizentra that I'm on).

Update

The specialist called and told me that they've started the process of authorizing the switch from Hizentra to Gammaguard SD with my insurance company. They are planning to have me start at one treatment a week. She also said that the specialty pharmacy (Accredo) said that if my insurance is cooperative then I could be transitioned in a as little as a few days. I'm planning to just skip treatment this Friday since even if it all gets approved this week, they won't be able to get the supplies to me in time.

If my insurance company causes problems or drags their heels too long, I might have to go back to doing Hizentra, but the doctor said that they'd give me steroids so I could get through it without a reaction. I really hope that doesn't happen! Wish me luck people! :)

Even though it's now days after my last infusion, my legs where I inserted the needles are still very itchy, and I'm getting a rash there. Last night the sites were lumpy, not from a build-up of medication this time, but from the rash.

Hizentra Reactions

I did my 4th Hizentra treatment on Friday, and I think it will be my last one. Despite taking 2 Benadryl in advance, I started itching all over my body and having breathing problems within about 20 minutes of starting my infusion. 

The bright note of my infusion was that I did it in the outside of my leg...on my thigh, about half-way between my hip and knee. the swelling was minimal, and went out about 2". Today (Sunday) it's itchy and sore, but better than on my stomach. My stomach tends to bloat and get really painful afterwards, but I haven't experienced that with my legs.

The specialist from Stanford called to say that my IgA is below 6--which is virtually 0, and when I'd had my appointment there he said that if my IgA is that low I could be building antibodies to IgA. Anyway, they believe that my low IgA is what's causing my reactions to Hizentra and Carimmune. I think I will stop taking the Hizentra until they can get my insurance to approve the switch to Gammaguard SD.

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.

**********************************************************************************

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! :)

Reactions since 2nd Hizentra Treatment

I haven't posted since 2 days after my last treatment, and I'd have to say that this most recent treatment was amazingly better than the first one.

I had a few headaches since then, but none as intense as last week, and I made a point of checking that my muscles weren't tense, and taking a clonazepam if so. I saw my chiropractor again on Tuesday and he said that most of my pain was due to muscle spasms or knots, and worked many of them out. I'm starting to think that the Hizentra (and other gammaglobulin treatments IVIG or SubQ) cause the muscles to tighten up, and may even contribute to knots. Just my theory, and I don't have any kind of medical degree, so take it with a grain of salt.

Overall this week has been much better than last week.

Two Days Later

I woke up feeling decent today...not great, but not horrible either. I had to take a Fioricet last night because I did feel a headache trying to come on, but it was gone by this morning. I even felt well enough to do my 5-minute workout today! The knots on my belly are down to 1" in diameter or smaller today! :)

After I'd been up for awhile I got very tired and had to take a nap. I've remained tired all day. I plan to try to sleep early tonight.

Second Hizentra Treatment! :)

I woke up infusion day with my stomach still feeling unstable, allover body weakness, and sore muscles from vomiting. I showered, got dressed, and was just reading my support group page when my nurse arrived. This time I knew what I needed for the treatment and set everything out, only checking with her once I thought I had everything ready. She showed me an alternate way to get the medication into the syringe that I like better than the way she showed me the first time because it puts fewer bubbles into the medication and is still easy enough for me to do it.

I really appreciate that Accredo overnighted the extra supplies that I would need to have a more successful treatment to me. The package included larger needles, quadfurcated tubing for 4 needle sites, and smaller rate tubing so that my infusion would go more slowly--allowing the medication to be absorbed while it's being administered. My nurse requested the larger needles so that it would be easier for me to get my medication into the syringe, and I'm so glad she did!

For this treatment I took a Zofran for nausea as well as Benadryl, and after awhile I decided to take a clonazepam as well because my nurse had said that it helps relax the muscles, and after the last treatment my neck and back muscles were so stiff and sore that I think it contributed to--or maybe even caused--my migraine.

Because of the slower rate tubing it took about 3.5 hours for my infusion this time, but it was so worthwhile! I have very small welts at some sites, and none at all at others!

I woke up this morning (first day after) with no migraine, but weakness and tiredness, however; those could be due to the vomiting from the day before my infusion. Overall it's been a good day and I'm feeling highly encouraged! :D

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.

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.

Third Day after Hizentra

I felt pretty bad when I woke up this morning, the headache is nearly gone, but it's more like...like I'm really getting sick. I didn't want to wake up, and didn't want to get out of bed either. I got up, showered and worked, but felt like I was really pushing myself to get through the day. I have chest congestion, a sick taste in my mouth, weakness, sinus pain and pressure, slightly sore throat, I keep feeling short of breath, and feel like I might be getting a cough. A friend from my support group said it might be a reaction from the Hizentra, as she had a similar experience after her first treatment, but I guess time will tell. I took a Benadryl just in case it might help.

I took my temperature. I usually don't bother since even when I'm really sick with pneumonia I tend to not get fevers--in fact, I'm usually at least a whole degree below normal, and so if I'm around normal I think of it as a slight fever. Tonight my temperature is 98.3.

My mom reminded me last night that my nurse had told me to use ice or heat for my injection sites when they hurt. I'd forgotten that, and was just using heat, but it seemed counterintuitive to use heat for swelling, so I tried ice. That was soothing. They have hardly bothered me at all today.

Second day after Hizentra

I woke up with a headache again this morning, and I realized that one reason I've been having such a rough time with this first SubQ treatment is that I didn't take it seriously like I do the IVIG. I had so ingrained myself with the idea that there are fewer side effects from it that I didn't even think to hydrate in advance! I realize now that I need to treat SubQ with more respect, just like I did my IVIG treatments. I need to hydrate the day before, avoid caffeine the day of, and hydrate they day of (and after too).

I have felt very tired all day with a strong desire to sleep. I wonder how long it takes to feel the increased energy that SubQ is supposed to provide? I just took it easy and stayed in bed all day. I hope that I'll be able to work tomorrow, and that I don't wake up with the headache.

Aside from the headache and fatigue, the swelling has gone down on my belly significantly. I almost look like myself again! The knots are about 1/3 the size they were after the infusion on Friday. I used to wonder what people meant when they talked about "knots" in regards to SubQ, and now I know! They are large welts (about 3" in diameter) that are hard to the touch, and painful too. They literally feel like a hard, knotted-up muscle, except they are more painful than that. I don't know whether they are caused by the fluid from the infusion building up under the skin, or if they are actually a histamine reaction. I need to do more research on that and find out.

Later the same day...
I feel like I might be sick. The question is, is it CVID, a reaction to the Hizentra, or am I actually getting sick? My legs feel like rubber and I have a "sick taste" in my throat.

The First Day After My First SubQ Treatment

I woke up with a splitting headache (migraine really) that seemed to reach from the base of my skull all the way up to my forehead. At first I didn't even want to try moving because I was afraid that the room would start spinning. I rolled over in bed and tried to go back to sleep but when I closed my eyes I would see what appeared be dim red lights throbbing, so I knew if I kept my eyes closed that would make me sick. I let myself wake up slowly, and after a few minutes had to get up to go to the bathroom. Luckily, the room didn't spin too much, but I kept hearing a high-pitched ringing that made my head hurt more.

I got back in bed and mom made me an iced tea to get some caffeine into my system, and also brought me some food so I could take some painkillers. I took a fioricet (prescription medication for migraines) and also took a medication for nausea.

I thought the knots had disappeared last night, but they're still there if a little smaller, and are still sore to the touch.


Hours After Waking...
I still have the headache, although it is less intense than when I first woke up, and the knots are still there and sore, but most of them are about half the size they were yesterday. I am also fatigued. Despite these side effects, It is not as bad as the first however-many IVIG infusions I'd had before realizing what a huge factor infusion speed is in how I feel afterwards. If I can get the side effects under control for this, I think it could really work for me. I still think it might be a good fit for me. :D

My First SubQ Treatment (Lots of details of what's involved)

My nurse arrived right about 2:00, and she brought another nurse with her for training. They had me sign consent forms, then double-checked the list of medications I'm allergic to. Then we went through the box of supplies and checked everything against the itemized receipt that came with my supplies, and she showed me some of the features of the things that were sent to me.

Once the paperwork was done, she had me wash thoroughly, and use a paper towel to dry my hands then turn the water off with the towel to keep my hands sanitary. We had set out the antibacterial hand sanitizer for "touch ups" in case I re-contaminated myself before finishing (I did...many times). We went through the box and selected everything that I would need for my treatment and laid them out where they were handy. Accredo sent me a thing that looks like a big bandage (it's about 5"x8" a flat package), but it was a sanitary paper to put down for a work surface. When it was laid out, it spread to about 18"x 24", and was really handy to have...I think they sent me 5 (1 for each treatment for the month, and 1 extra).

Prepping the infusion Sites...
I then took a dollop of the Lidocaine cream and pressed it onto the places where I wanted the needles to go, and pressed a Latex-free bandaid over each dollop of cream. I had to select 5 sites because the supply company had mistakenly sent me "hookups" (don't know what to call them) for 3 needle sites when I needed to have 4. I really appreciated my nurse because she refused to do the treatment with 3 needles, and called to straighten everything out and try to find a solution. I didn't understand the ramifications of using 3 needles, or why we couldn't just use 4, but they explained to me that the more needles you use, the slower the infusion rate. My doctor had ordered an infusion rate of 15ml/hr, and since my treatment is 60ml, that meant I needed 4 needles (because 60/4=15). They couldn't use 2 of the hookups at one time because the ends of the hookups have the needles, and they branch from a single tube. There was nothing in the box to allow for a Y-connection of the tubes. My nurse spent a lot of time on the phone until she and her boss (and the other nurse) all came up with the idea of giving me 2/3 of the treatment with 3 needles, then the rest with 2 needles.

Filling the Vials with Hizentra...
I'd chosen 4 treatment sites on my belly and 1 on my back in an area where I could still see by twisting. The nurse had me take a Benadryl about the same time as when I put the cream on, and then we began preparing the medication and pump. I first popped the cap off of the tops of the Hizentra vials and swabbed the membrane across the opening with an alcohol pad. The next step required me to remove the huge syringes from their sterile packaging and screw the large needles on to them (these are only for getting the Hizentra into the syringe...the needles don't go in your body). They had me save the needle cap to make removal of the needle more sanitary and safe later. Each vial contains 20ml of Hizentra, so the nurse had me pull back the plunger on the syringe until there was 20ml of air in it (Because the vials are vacuum-sealed, you have to pump as much air into them as the amount of medication you want to take out.) I am small and not very strong physically, so I found it a real challenge to even pull the plunger out on the syringe; the next phase was even more challenging.

Here's a link to a good deal on generic Benadryl (diphenhydramine):



The needle goes into the vial easily enough, but putting air into it and holding everything such that you don't contaminate a sterile surface will take some practice for me to master. The nurse had me turn the vial and syringe upside-down such that the plunger of the syringe was resting on the sterile surface and my left hand was holding the vial and needle end of the syringe such that I could see into the vial. With my right hand I pushed down on the shaft of the syringe and forced air into the vial until the resistance was so great that I couldn't push anymore. I would then ease up on the plunger and allow medication to pour into the syringe. Allowing medication out would let me push more air in, and so I repeated the process until I'd gotten all of the medication out of the vial. I repeated the process with the next vial until I had all of the medication that I needed in the syringe. I then replaced the cap over the needle and removed it from the syringe. The company had provided a sharps container for me to discard it in.

Connecting the Tubes...
I then connected the tubing. There's a small piece of tubing with a number on it like 1200 and 600 that determines the infusion rate (in addition to the number of sites). I forget now which is which, but one of them allows for a faster infusion rate. We chose the one for a slower rate, and  removed the sanitary caps from each end of the tubing and screwed it onto the end of the syringe. to the other end of that tubing I attached the 3-needle tubing. The 3-needle tubing kind of looks like a tree in that there are 3 tubes each with a needle at one end, and they all branch from a single tube. I was then ready to put the needles in.

Putting the Needles In...
If you're squeamish about needles and reading just for the sake of interest, you might want to skip this part...

The nurse had me remove the bandaids and wipe off the Lidocaine cream with a paper towel, then swab the area in a circular motion with an alcohol pad. I left the bandaids over the other sites until I was ready to put needles into them to allow them to get as numb as possible. While the alcohol was still drying I laid out the clear film covers (Tegaderm, by 3M) for each needle site, and peeled the protective layer off of the adhesive--these covers hold the needles in place so I can move around while having the infusion. The nurse had me pinch about an inch of the numbed skin between the fingers of my left hand, and spreading the butterfly flaps back with my right hand I stuck the tiny needle (it's only 4 ml long) straight into the middle of the pinched area. Thanks to the Lidocaine cream, I didn't feel it at all! I then covered the needle sites with a thin adhesive film that the supply company sent me. Finally, I had to make sure that I hadn't put the needle into a blood vessel because the drug is not yet FDA approved for intravenous treatment, so I pulled back slightly on the plunger and ensured that no blood appeared in the line. I repeated this entire process for each of the other sites, then prepared the pump.

Me with needles in. The tubes go to the syringe in the pump.

Starting the Pump...
The pump is a mechanical device, not battery operated. there is a large knob on one side to wind it up, and an On/Off switch. It comes with a black canvas bag to carry it in. The bag is pretty cool because it has a window  where you can see the syringe to monitor the progress of the infusion, and a convertible strap to make carrying it easier. The plunger end of the syringe fits towards the back of the pump (under the clear plastic cover), while the end connected to the tubing fits into a little ring at the front (see picture below).

Infusion pump. The big knob winds it up, the little one turns it on and off. The clear plastic covers most of the syringe plunger during the infusion.

Before putting the syringe it's necessary to wind it up first by turning the big knob clockwise. It's a close fit to put the syringe into the pump. You have to angle the plunger end under the plastic cover such that the numbered side faces up for easy readability. Then you have to push back on that as you push down to lock the tubing end into the circular holder (the lip at the end near the big knob). After it's in place, switch it on. It makes a really loud, obnoxious noise at first until the little black thing that pushes on the plunger reaches the plunger handle. As soon as it reaches the plunger handle, it's absolutely silent and remains so until the end of the infusion. My nurse said that the pump exerts a force of about 14 psi on the plunger...which is about the same as normal atmospheric pressure, so I think (if I remember my chemistry correctly) it should be about the same as getting a gravitational IV. I'm not positive about that though...feel free to let me know if you know more about this. :)

The Infusion and Wrapping it Up...
The infusion lasted about 2.5 hours for the 60ml treatment (I had to repeat all of the above steps to finish mine, but this was an unusual situation). During the infusion I had some mild discomfort at the injection sites, but no itching. Once it was over, I prepared bandaids, peeled the adhesive film off of the needles, and pulled them out one-by-one. With some of the needles I inadvertently dragged them across my skin as I pulled them out, leaving scratches that started bleeding. At first I didn't realize what had caused the scratches, but then I realized I wasn't pulling them straight out. Once I realized the problem, I was able to stop it from happening. I covered each infusion site with a bandaid. After removing each needle, I cut the tubing near the needle and discarded the needle end in the sharps container, and threw the tubing and syringe away.

Post-Infusion Observations...
Each infusion site had a hard knot about 3 inches in diameter around the needle entry point and those sites felt tender to the touch. My belly was bloated to the point that I look pregnant, and I had a lot of gas. I didn't realize it was from the infusion until I was talking with a friend from my support group who told me that she was experiencing the same thing. Later in the evening I started having some shortness of breath and my chest was tight. I measured my peak flow, and found it was lower than normal, so I took my inhaler, which helped. The heating pad helped with the soreness of my belly.





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