First Hizentra Treatment

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!

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.

Immunologist Update

This morning Ryder and I were in the car and heading to Hopkins by 7:10am. Ryder's appointment was at 8:15 and we were checked in and he had been weighed and measured by 8:30am so that wasn't too bad. We only had to sit in the exam room for about 3 minutes before Dr.Lederman came to see us which was really good.

He started off by asking how Ryder's infection history has been since we saw him in May so we went over all of that. Then he did a physical exam on Ryder looking at everything. Once he was finished with all of that he sat down and showed me Ryder's blood work results. He had it all in a nice chart so it was pretty easy to understand once he explained what the numbers meant.

Apparently they use the Prevnar vaccine as the main diagnostic tool for kids when looking at primary immunodeficiency issues. His initial antibody values ranged from 0.1-0.4 which Dr.Lederman explained meant he had no antibodies from the vaccine (even after having the normal 4 doses babies receive). Then he had a booster shot given and more blood work 1 month later which showed values ranging from 10-33 which Dr.Lederman explained shows a response from his immune system, but not to the normal extent that would be expected (pointed out a 6-7 month fetus would have that type of response). Then we looked at the antibody values from 6 months after the booster shot was given which ranged from 0-0.4 which once again shows no antibodies. It was obvious to me at that point that something was not right about Ryder's immune system.

Dr.Lederman told me he feels with the number and type of illnesses/infections Ryder gets along with all of the blood work results that Ryder definitely has a Primary Immunodeficiency Disease. The only actual name he mentioned was the Transient Hypogammaglobulinemia, but said he is no longer transient. He then went on toe say he feels it is in the best interest of Ryder to start him on IV Immunoglobulin treatments which are basically IV antibodies to help increase Ryder's immune system.

The actual product that Ryder will receive is called Hizentra. They went over all of the information about this, showed me a video and discussed the options we have for administering it to Ryder. We decided to go with the weekly at-home infusions (another option was monthly IV treatments at the clinic, but they said many younger kids have more side effects from this option and due to my knowledge of needles from my Diabetes they felt it was best to try the in-home infusions). The first infusion will be given to Ryder at the clinic so they can make sure he doesn't have any reactions to the product. I was told the most common reactions are soreness, redness or swelling at the infusion site, headache, and nausea. They told me if he experiences the nausea they will be able to prescribe a medication to give ahead of time to reduce it. During the first infusion they will teach me how to administer the infusion so all future ones can be done at home on a weekly basis. Dr.Lederman said he was is not able to tell us right now if this will be a life-long thing for Ryder, but it is a very high possibility he will require these infusions for the rest of his life. They also gave me a prescription for EMLA cream which will numb the skin where Ryder will receive the infusion so he shouldn't actually feel the needle insertion, but they told me it will take a while for him to "get used" to the whole process.

I am waiting to hear from the Immunology office, but out hopes are to have the first infusion on Friday as long as insurance moves quick enough and we don't run into any issues with insurance. I have tried looking up the Hizentra online through the insurance company to find out if it needs pre-authorization or what not, but can't find it listed anywhere so not sure if that is a bad sign or not.

Snow

Yesterday in the evening it started to snow and sleet. Robin was going crazy about the snow so she spent a ton of time outside last night. She loves snow! This morning we woke up with about 3 inches of snow. Schools in our county were closed today even though the roads seem to be fine and many other counties only had delays. As soon as Zak woke up this morning he started yelling "It snowed! I need go out and play now!". So I bundled them up and we went outside to play for a while this morning.

I took a bunch of videos of the boys playing in the snow. Zak liked throwing the snow. Ryder was funny because he wouldn't really pick his feet up so he would slide his feet along the ground when he was walking.

Gymnastics

On Saturday morning we took the boys to the first class of the new session at YMCA. This session we decided to have them do a class together. We chose a mini gymnastics class because it was something new for them to try and we felt it might be good for Ryder to work on some coordination skills. They both had a great time. It was extremely obvious that Ryder is delayed physically compared to the other kids, but the class had kids ranging from 3-6 years old also. Zak still needs to work on waiting in line for his turn, but hopefully participating in these classes will help with that.

Zak doing forward rolls

He actually almost does a head stand at one point

Ryder doing his forward roll

Ryder doing his pencil roll

His old OT told me he doesn't keep his head down because of some vestibular insecurity he has, he doesn't like the feeling of rolling or being thrown or swinging

Zak doing the pencil roll

On Sunday I got really sick once again and was unable to hold any food or drinks down. I knew I wouldn't be able to function n Monday so the boys had a sleepover with Nanny & Poppy. They went to the state park which I think they loved. They told me all kinds of fun things they saw and did. This is a picture Rich sent to me from his phone.

Health Update

I feel like our house will be filled with germs forever! This morning I decided to go to the Target clinic for Zak and I to be checked out. I have been dealing with this cold for what seems like weeks and Zak has also. The doctor looked Zak over and decided to put him on antibiotics for a sinus infection. She then looked me over and said it was viral. I hate that word...viral! She did give me a prescription for Flonase to try and help clear out my sinuses so they don't get infected.

While there she said this viral thing going around is taking 2-3 weeks or more to run its course with people. That is not what I wanted to hear. She said for me to take Mucinex along with the Flonase. Then she told me to use the Children's Mucinex for the boys to help clear up some of the side effects of this viral thing that they have in addition to the infection going on. I am trying hard to get rid of the germs in our house so we are being very diligent about hand washing, hand sanitizer and using the sanitize function on our dishwasher!

I also have an update from Ryder's most recent lab work he had done for the Immunologist. He had booster shots done about 6 months ago and then some blood work done the beginning of December. On Monday I called to see if they had the results yet since I had not heard anything yet. The nurse practitioner told me they had the results, had discussed them and the head doctor (Dr.Lederman) had them on his desk because he wanted to call me himself. She said she would talk to him and let him know I called so that he gets back to me ASAP. This afternoon I got a call from a scheduler saying she was calling to make an appointment for Ryder with the Immunologist. I was confused so asked what the appointment was for. She told me the note said to schedule the first available appointment with Dr.Lederman for follow-up to results from recent lab work. So Ryder has an appointment for 8:15am on January 24. I can't believe we have to wait until then to find out what is going on! I am thankful we some how got in with one of the top Immunologists at Hopkins, but it is so hard since he only sees patients once a week and is nearly impossible to get in touch with. I am going to give the nurse practitioner a call tomorrow to see if she can give me any idea as to what is going on. I will make sure to update the blog when ever we find anything out.

Happy New Year

This post is a few days late because I have been in bed for the past 3 days battling a stomach bug. That was no fun at all! Actually, everyone in our house has been sick with something. It all started on Saturday December 18 when I took Ryder to the pediatrician because he had been coughing for about a week. He ended up on Omnicef for 10 days to treat pneumonia. We had his follow-up on Monday December 27 which went well with his lungs being much better. He still had a cough so they told me if he got worse to bring him in to get looked at again. Well, on Thursday December 30 I ended up taking both boys to get checked out by the pediatrician. Zak had started coughing and Ryder was coughing along with very snotty and just looking sick. We left that appointment with Zak having a virus and Ryder starting a course of Zithromax for a sinus infection. Today was the last day for Ryder's antibiotic, but it stays in his system for 10 days. If in 5 days he is still sick (which is is today still) then we are supposed to refill the Zithromax and do another course of it for him.

Meanwhile, Ben has been battling a really bad cold which I think could probably actually be a sinus infection. I started having a cold right around Christmas. I woke up around 4am on Friday with a stomach bug which stuck around all weekend. I was able to keep down some small stuff last night and feel better this morning. Ben went back to work today so I am forced to function today. I am hoping after I eat some more over the next few days my headache will go away and I will be feeling much better. I am still dealing with this cold as well. Zak seems to be pretty much the same at this point with his cough and runny nose. Hopefully we will all be better soon!

Today my plan is to get the house back into order. It pretty much feel apart over the weekend!

Now on to the good stuff, I have a bunch of pictures I wanted to post from the last few days. Laura and Haleigh came over for dinner on Thursday so I took a bunch of pictures then.

Zak is practicing to have twins of his own!

Playing with their monster trucks

Zak building with Trio blocks

Ryder with Handy Manny tools

The boys inside of their tent

Haleigh playing with the boys' toys

Ryder trying out Zak's skates

Ryder playing with a new toy

Haleigh was pushing Zak around on the skates

Haleigh

Zak dressed up as a Teenage Mutant Ninja Turtle

Tweedle Dee & Tweedle Dum

Ryder goes back to school tomorrow and I need to get Zak signed up for a new session at the YMCA. Ben starts back with his Monday night volleyball starting tonight. So things are picking back up and we are slowly getting back into our normal routine.

An update on Zak and potty training. It has been going really good the past few weeks! He has been wearing underwear all day and a diaper at night. He is getting really good about just going to the potty to pee during the day when ever he needs to go and he will tell us while we are out and about when he needs to pee. The big news is that he actually pooped in the potty 2 days in a row this past week!! It is still a work in progress, but he is making progress so that is what matters. It is amazing how having one of them in underwear during the day has cut down on our number of diapers!