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.

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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! :)

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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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.