This morning Ryder, Ben and I headed out the door around 7:15am. Ben took his truck to work and I picked him up in our car. We then made our way to Hopkins and got there by about 8am. We got checked in and quickly taken to the back. I love having appointments first thing in the morning because it is empty and usually they are not running behind!
Friday, January 28, 2011
Just before leaving I out some EMLA cream (topical anesthetic) on Ryder's side and covered it with some Tegaderm (clear dressing to go over IVs and wounds). Once we got into the exam room the Nurse Practitioner (Beth Younger) brought in a big box. Ryder got excited because he was convinced it was full of toys for him. She pulled out the pump and let Ryder play with it because it is pretty durable. She showed Ben and I all of the supplies and gave us more information on the Hizentra. Then she walked us through the steps to set up the infusion. It seems pretty simple to me because it is fairly similar to getting my insulin pump set up.
We then put Ryder on the exam table so we could get started. As soon as we lifted up his shirt he pretty much went crazy screaming, crying, and arching trying to get away from us. He really flipped out when we pulled off the Tegaderm. Ben was standing by his head and holding him arms while talking to him. The Nurse Practitioner and I were standing by his feet/stomach so she could walk me through how to get the infusion started. I took the needle and quickly pushed it in and Ryder hit the ceiling. I felt really bad, but we quickly got a piece of Tegaderm on top of the needle and let him sit up. He was pretty upset, but once we got the infusion going he got to sit at the computer and play games. At that point he pretty much forgot about the infusion and was a happy guy. The infusion only took about 15-20 minutes. Once it was time to take the needle out he freaked out again and didn't want anybody to touch him. The Nurse Practitioner showed me to just quickly pull the Tegaderm and the needle would come with it and it was over.
I know I will be able to do the infusions at home because I will do anything needed for Ryder (or Zak). I will need Ben to help me hold him down until he gets more used to the whole process, but the Nurse Practitioner told me that kids will get used to it and I do believe that. He didn't have any reactions to the Hizentra other than swelling and redness at the injection site which is completely normal and will get less and less as his body gets used to the Hizentra.
During the infusion I talked with the Nurse Practitioner to get some questions answered which was really nice. After it all kind of settled in I had come up with a few questions so we went over them. Some things we discussed included...
1) Side Effects: She said that most of the people who have side effects are receiving the Immunoglobulins through IV and then get switched to the subcutaneous and no longer have issues. She explained the local injection site side effects are normal.
2) Need for Future Vaccines: She explained to me that since the Hizentra is an actual blood product it contains all of the antibodies from those who donated the blood. Because of this the Hizentra includes all types of antibodies so he will no longer need to get any vaccines while getting these treatments. She also advised me that he needs to definitely stay away from ALL live vaccines even when not on the treatments. This could definitely explain Ryder's pretty big reaction to some of his earlier vaccines. The reason he needed to flu shot this year is because the Hizentra he is receiving came from donated blood from 2 years ago (that is how long the whole process takes from donation to injecting the product) so there are no antibodies for H1N1 in. Because of this they are requiring their patients to get the flu shot this year, but in future years he will not need it. We also learned that he should avoid having contact with people who have recently received any live vaccines (glad we chose to keep him home from school the day they administered the FluMist!).
3) Actual Diagnosis: I asked what exactly was Ryder's diagnosis, I knew he had Primary Immunodeficiency, but also knew that there were tons of different subgroups. The Nurse Practitioner told me he was Common Variable Immunodeficiency (CVID). This is one of the more common types of PID. She explained that if you lined up 100 people with CVID they would all pretty much have different symptoms and blood results which is why the word variable is in there. For Ryder he even more specifically has a lack of memory cells within his immune system. This means when he has a vaccine his body shows a slight response, but it disappears very quickly which leaves him with no antibodies. When a normal person gets a vaccine (or even a strain of a virus) their body automatically produces antibodies. Then when you encounter that exact same illness/virus your body has memory cells which produce more of the antibodies and fights off the infections/disease/virus. Ryder is not able to do this. Along with his lack of memory cells he lacks IgG and IgM antibodies along with low IgE.
4) The Nurse Practitioner is going to send a letter from the Immunologist along with a packet of information regarding CVID and being in school to Ryder's school nurse. The information will basically outline what Ryder has and what precautions need to be made. The school will need to contact us ASAP if there is an outbreak of any type of illness/virus/disease so we can pull Ryder from school. Also, we will need to keep Ryder home when the school administers the FluMist each year. It will also give the nurse information about any reactions or side effects Ryder might exhibit from his treatments.
5) Monitoring: The Nurse Practitioner said in 6 weeks Ryder will need to have blood work done to check his antibody levels. Based on those results they will know if they need to adjust his Hizentra dosage. The dosage is based on weight so as Ryder gets older his dosage will increase. They will want to see Ryder in the office every 6 months and do blood work to check some organ functions to make sure he is not showing any signs of reactions or problems from the Hizentra. She said we will have contact through phone and e-mail so if we feel Ryder starts getting multiple infections again or if we have any other issues they can be dealt with quickly.
Once we were done with the infusion the Nurse Practitioner broke the news to Ryder that he would need some blood work done. Usually Ryder happily gets on my lap and watches the needle "drink his blood", but today he wanted nothing to do with it. He screamed the whole time. I felt so bad, but I know it is for the best. The Immunologist also gave me a lab slip to have Zak's antibody levels checked since CVID is a genetic thing. This doesn't mean Zak will have immune issues, but it is better to check it out and make sure. They also want me to have Zak's vaccine record faxed to them from the pediatrician.
Ryder has been slightly irritable today, but I admit I have pretty much been spoiling him rotten since we got home. As he gets more used to the treatments this special treatment will have to slow down, but today I felt so guilty for putting him through this ordeal.
Posted by April at 12:57 PM