12-24-13 Sarah's 4th infusion

Sarah had her 4th infusion yesterday on Christmas Eve. She still has a runny nose and a bit of a cough, but overall seems to be doing better on the antibiotic. She was worried about this infusion because she was still bruised and sore from her last one. I decided it change the location a bit and moved it down on her abdomen to see if it would help. The second infusion went so well that I was looking for that result again.

Moving it seemed to do the trick!  She hasn't complained at all about being sore, and the swelling from the infusion was already down significantly last night. She went to our Christmas Eve service and was even playing with her brothers last night and today.

Her next infusion will probably be Wednesday, January 8. I am waiting to hear back this week about upping Sarah's dosage and adding a third infusion site.

I will add pictures from this infusion tomorrow.  

Still sick and eye issues

Sarah has still not gotten over her crud.  After running a fever off an on, and coughing a ton, along with a runny nose, I took her back to the doctor Friday.  (She gets frequent sinus infections and left untreated, they drain into her lungs and then turn into pneumonia.  Her lungs were getting to the point that they would sound "junky" and she would be wheezing, but if she coughed, she could clear them.  Often within a few days, it will turn into pneumonia.)

The good news is that her numbers from her spirometry test were the best they have ever been!  So her maintenance inhaler for her asthma is working really well.  But, her lungs did sound icky until she coughed, her nose is full of "gunk", and you can tell just by looking in her eyes that she feels crummy.  The doctor put her on an antibiotic for another sinus infection.  She just doesn't get over stuff on her own.  Ever.

This doesn't have anything to do with Sarah's immune deficiency, but Sarah had an eye appointment this morning.  It turns out that one of her eyes is way worse than the other.  She needs to see a pediatric optometrist to find out if he wants to do visual therapy on her or just put her in glasses.  We will call Monday to make an appointment.

Sarah's next infusion will be Tuesday, December 24.  When I took Sarah in on Friday, the doctor and I talked about upping her dose on the subQ IG, and also maybe adding a 3rd infusion site instead of the 2 she has now.  We also talked about moving her to weekly, but still upping her dose too.  She was going to talk to the nurse and the pharmacist and see what the plan is.  We should know more next week.

12-10-2013 Sarah's Third Infusion

Sarah hasn't been feeling too great lately.  She has had a fever off and on, and her nose has been running and she has been coughing.  In speaking with the doctor today, we decided to hold off on antibiotics and see if she can fight this off on her own.  We may still end up on antibiotics later this week.

Today, we did Sarah's 3rd infusion.  It is the first one that I have done by myself without any help at all.  It went fine, and the emla cream really helps the needles not hurt going in.

There are a lot of pieces to doing this infusion.

This is the medicine itself.  It is made from human plasma, and has the antibodies to fight infections.

First, you hook the syringe

 

up to the spike

 

 

Then you push the spike into the first container of medicine.  You pull back on the syringe until you get all of it in the syringe.  Then you repeat with the 2nd vial.

Then you hook the flow control tubing (I believe this is the tubing that tells the pump how fast to infuse the medicine) up to the syringe.

Then you hook the infusion tubing up to the other tubing.

 

 

After that, you prime the tubing by slowly pushing down on the syringe and watching the medicine go through the tubing until it reaches just above the needle.  Then I insert the needles into her skin and hold in place with Tegaderm.  Then I put the syringe in the pump and turn it on. The infusion takes about an hour and a half.  Then we remove the needles, cover the infusion sites with gauze for leaking, and we are done.

 

I am hoping she will feel as good after this one as she did after the last!

 

 



2013-11-26 Sarah's Second Infusion

I did not have a chance to post after Sarah's second infusion this week as we left town the next morning and had a ton to do to get ready to leave. Sarah's belly was really sore for quite a while after her first infusion (like close to a week and a half), and she was thinking "I just am feeling better and now have to do this again?"! But, we gave her Benadryl to prevent the itching, and I put emla cream on her to try and ease the pain of the stick. The home health nurse came to watch me set the whole thing up and start the infusion (because this was her last visit).

This is the pump 

 

 

 

This infusion took about an hour and a half again, and she tolerated it really well.  She even got up and walked around some while it was happening.

 

 

 

 

By that, evening, she was even running around.  And even though her stomach is still a little sore to the touch, she is doing much better than after the last one.

 

And, she finally has color back in her face.  I don't think she has not been pale for years!  And you can just tell being around her that she feels better and is more perky.  I am so thankful this seems to be working already!

 

Her next infusion will probably be Wednesday, December 11.  Thank you for your continued prayers and for following Sarah's story.





2013-11-13 The First Infusion

After doing research, we chose to do the subQ Infusion instead of IVIG. It is every other week instead of once a month. But...

- The infusions are shorter (1 1/2 hours at a time as opposed to 4 hours at a time).

- She can move around while it is being done instead of having to be monitored.

- There are less chances of side effects.

- They do not have to find a vein every month for potentially the rest of her life.

- I can do the infusion myself instead of having it administered, so it can be on our timetable and in our home.

- With the IV, since it is once a month, sometimes people have a "crash" sometime around the 3 week mark and your next infusion isn't for another week and a half to 2 weeks.

So, last Wednesday, I got a box with all of the supplies and medication for 2 infusions. Wednesday afternoon, the home health nurse came to show me how to administer it. She set everything up and put the needle in the first infusion site. I did the second site.

She said some people pre-medicate with Benadryl and Tylenol, but she likes to see how they handle it without meds the first time. Within 60 seconds, she started complaining about it itching. So we gave her Benadryl, and after 25 minutes or so, she said it didn't itch anymore. Next time, we will pre-medicate her.

This picture is during the infusion.

The first time, the nurse had to take her vitals and make sure she tolerated it okay, which she did. After that one time, we won't have to do that. Her pump came with a bag that she can throw over her shoulder or hook around her waist, and she can go about whatever she wants to do. Obviously she can't go play a soccer game, but she is not confined to the couch or anything.

This picture was taken a couple of hours after the infusion.

I am told that after 2-3 infusions, her body should learn to absorb the medicine a lot faster and it shouldn't be as bad. She was really sore the rest of the week, though she didn't complain much. But she voluntarily stayed inside from recess and didn't participate in PE the next day, so I know she was hurting more than she let on. By the evening, the swelling was already down significantly.

Sarah's next infusion will be the Tuesday before Thanksgiving. The home health nurse will come out just to watch me get it set up and start the infusion to make sure I am good with it. That will be the last time she will come. Then we will be able to do it on our own schedule.

I am told we should start to see a difference after 2-3 infusions (so 4-6 weeks). However, they start at a certain dose because of her weight, but that doesn't mean that is HER therapeutic dose. So if it doesn't work, that doesn't mean it won't work (if that makes sense). They may have to up the dose. The pharmacist for the home health company also told me that she should only get one to two infections PER YEAR! I cannot even imagine what that would be like with her!

Making the Decision

Since the doctor had been trying to talk me into doing IVIG for almost 5 years, I knew that this chat was coming. A month ago, she told me that she thinks I should really REALLY consider trying it with Sarah. I had already done my research and the idea of doing this terrified me. She asked that I would let them start the process with the insurance company. She told me it would take weeks to get things approved through them and they would probably have to appeal it and everything.

At this point, I started praying for a sign that it was the right thing to do. The following week, Sarah was getting worse instead of better. So after a morning of a big dose of steroids and several breathing treatments, I took her into school late. As I was talking to the nurse, I found out that she had done this therapy when she was younger. Then I called the doctor's office to chat about what was going on with Sarah, and the doctor told me that the insurance company had approved the therapy for Sarah within 2 hours, and they approved it for 10 YEARS! So not even as a trial basis or anything. Then, my friend who used to be the IVIG coordinator for the office she worked at called me because we had been trying to get together to chat about this and it hadn't worked out. And then, I met a lady whose daughter had just the therapy 6 weeks prior. All of this happened within about 4 hours, so I would say that I got the sign that I needed that this was the right direction. We just know she cannot continue to go down the same path with illnesses that she has been. She is almost never well.

Sarah's Story

Sarah was born on Monday, March 27, 2006 after a quick induction (by a doctor who said she wanted the baby born before her office hours were over) because she was 11 days late and we decided she wasn't coming on her own. Around 3 1/2 months, she started having respiratory issues. Having gone down the same road with Zachary at almost the same age, we knew what was coming and did not want to have the same struggles. I went to the pediatrician (who was a different pediatrician than I had with Zachary) and explained my concerns. I was told that even though her lungs sounded "junky", babies do not get asthma, an we didn't need to worry about anything. Having dealt with asthma and multiple hospitalizations with Zachary when he was little, I took Sarah to the asthma and allergy doctor immediately. We got her started on a daily "maintenance inhaler" around 4 months. When she got RSV and pneumonia at 9 months, she did not end up hospitalized like Zachary, even though we thought she might. I attribute it to starting the asthma meds so early.

Also at 9 months, Sarah was diagnosed with failure to thrive. She was 8# 14oz at birthday, 15 pounds at 6 months, and 16 pounds at 9 months. She had gone from being big down to the 5th percentile. My boys and my husband have celiac disease, so the assumption was that she did too. We did not remove gluten from her diet, but she did not get a lot of it as we didn't usually have it in the house. (Where you might have fried chicken or rolls with dinner, we never did that because no one else could eat it.) The day of her 9 month checkup, we had her blood drawn for celiac disease, and I took her off gluten. The test came back negative, but it can be inaccurate under the age of 2 due to exposure, and probably more so in a case where she didn't get a lot anyway. After taking her off gluten, she grew like crazy. After gaining 1 pound in 3 months, the next 3, she gained 8!

Throughout all of this, we dealt with several cases of pneumonia, strep, other respiratory infections, fevers, etc. She was always sick. When she was 19 months, she ended up with pneumonia again, in spite of the fact that she had already been on antibiotics for respiratory "crud" for a couple of weeks. We changed antibiotics and keep waiting for her to turn a corner with breathing treatments and steroids. Be the middle of December, she had been fighting it for 2 months and was just getting worse and worse. We ended up in the ER, and it turned out she had become septic (the infection got into her blood) from it. She was on IV antibiotics (vancomycin and rocephin) and spent 6 days in the hospital (including her brother's 7th birthday). It turns out that the bacteria from the sepsis is usually related to a cardiac problem, so then we also had to spend time at the cardiologist and getting echos done and everything.

By January, she was doing better. Then she was sick most of February and into March. Mid-March, we went to Denver over spring break with a little girl who was "sort of" sick. By the second day, we ended up in urgent care and she had pneumonia again and her pulse ox was 90%. They let us go home because she looked "ok", but we had to take her back the next day to be rechecked. We thought she was doing better, but this time, her pulse ox was 87% and ended up dropping to 83%, even on oxygen. So my baby girl got to take her first ambulance ride that day to the hospital and was admitted in respiratory distress. She spent a few days in the hospital out there (including my then 5 year olds' birthday), and then we drove home.

Four days later, Sarah was sick again. Her limbs were blue and she was struggling again. We took her to the ER where they got her breathing under control, and diagnosed her with an ear infection. They also did some other tests, and the xray ended up looking like she might have a problem in her bowel. It was determined she didn't and they send us home on more meds. Then the next morning, she had diarrhea and was throwing up so much that I had changed her clothes 6 times in an hour. After calling the pediatrician, we went back to the ER. They did tests, and her liver was enlarged and she had rotavirus. Because of the celiac disease, it is even harder for her to get over it. So she spent another 6 days in the hospital (including her own 2nd birthday) hooked up to an IV.

While she was in the hospital, they drew her blood for an immune workup. Her IGG and IGA were "normal". The main thing that stood out was that 13 of the 14 strep pneumo titers did not even register. This would be the cause of all of the infections. We gave her an extra pneumonia shot, and talked about doing IVIG, but I wanted to try other things first. Two months after that, we discovered she had a chronic sinus infection in every sinus cavity, and we did 60 days of antibiotics to clear all of that up. After doing that, she actually had a streak where she was relatively well. She did get some illnesses, but it was nothing like the previous year had been. I was hoping maybe we had turned a corner.

Right before she turned 5, Sarah started getting illnesses again. In March of 2011, she had been running a fever for a week and then it finally broke. A day later, she woke up complaining that her legs were sore. I thought she maybe had a Charlie horse or something and wasn't too concerned. Basically I told her to eat a banana and get some potassium to she'd be okay. By 730 that evening, her fever had spike back up to 104.5 and she couldn't walk. If you even touched her legs, she would scream. I'd spent enough years working in the ER that it freaked me out a bit. So, we went to the ER. After many xrays and blood tests, they thought they saw a spot of something on her leg, but it would require a bone scan. They were worried enough that they sent us to the Children's Hospital Downtown, which is more equipped to handle stuff than the "branch" out South. By the time we got there and got her bloodwork back, we found out that her creatine kinase was extremely high. Creatine kinase being high can be a sign of muscle degeneration (non-specific) or kidney failure. Based on her age, her normal should have been less than 50. Her was almost 3,000!

They determined that the cause was not a kidney problem, so we waited on the results of her virus panel. In the meantime, she was hooked up to an IV and they were trying to "flush" her system and get her ck to come down. The virus panel came back positive for influenza B. Apparently, sometimes when the virus was leaving her body, it attacked her muscles. Then her blood culture came back positive again! This time, though, they determined it was because of a contaminant. She spent 5 days in the hospital for this (again was hospitalized on Zachary's birthday).

In the end of August 2011, Sarah spiked another serious fever and was diagnosed with mono. I am told that kindergarteners "don't get mono", or if they do, you don't even know it because it just presents as a cold and then they are fine. She ran fevers of 104-105 for 6 weeks, missed weeks of school, and also ended up with an abscess behind her tonsils. So that stunted her already compromised immune system even more.

At this point in time, she has had already had 4 extra pneumonia shots, and only 6 of her 14 strep pneumo titers are low. The doctor says that just because she has the correct "quantity" of IGG doesn't mean she has good "quality". Clearly it is not right that she runs a fever for a week, coughs for a month, goes through 2 rounds of antibiotics, is well for 4 days and then starts all over again.

Sarah is a trooper though. She doesn't let much get her down. After having pneumonia and coughing like crazy and running a fever all week, she still wanted to go play in her soccer game. I think she will be amazed at how good she will feel because she has never felt well!