August 2, 2011

We have a very busy several months. We went to Pheonix for the IDF convention and, once again, got a lot of good information.

Lately as Jacob has been infusing he gets these hard knots under his skin, that he call nickles, that stay for several days to a week after infusing. Theses knots also hurt him a little. While at the convention we learned that the needles were to big for him, adults were on the same mm. We have gotten new needle sets and he still gets the, nickles, but they don't hurt. (One more thing to ask the Dr)

His most recent IGG levels came back at over 1300 and he hasn't been sick, on antibiotics, since February! He has had a few asthma flare-up but only very minor.

He is still doing the infusions like a champ and take approximately 2 hours to complete the infusion. We have have discovered if we go any faster than the rate we are currently at he gets migraine headaches that will knock him out for a long while.

We are scheduled to go to Alaska in September, just 1 month, and we are all so excited.

April 4, 20

It's been a long time since I have posted but Jacob has been doing very well. He has a few infections but nothing severe and most importantly we have been able to stay out of the hospital since June! His asthma been flaring up a little more than usual lately but with a few tweeks to his mediction and it appears to have gotten under control. His migraine headaches have finally started to subside. He was getting full migraines atleast once a week with all the classic symptoms. rough on anyone but especially a 7 year old. His doctor has drastically slowed down his infusion from 45 minutes to 2 hours. In the beginning we had very little change in the headaches but in the last 6 months they have started coming less and less. Also, a huge step this year is we finally (so far) have not gotten the flu OR RSV. I monitored the flu outbreak and held off on the flu shot until as late in the season as possible, since everyother year he had lost all his titers to the shot and got the flu in Feb. every year since he's been born. In June we go to the IDF seminar and I hope to have a lot more knowledge about the disease my son has.

August 30, 2010

The article above is from the IDF (Immune Deficiency Foundation), they featured Jacob and his friend Ethan . They both suffer from CVID, Common Variable Immune Deficiency Diesease). The boys finding each other has been such a powerful and supportive experience for both of them. Finding someone that has this disease is rare much-less someone your same age. The boys started communicating via SKYPE and then started doing their infusions together over SKYPE. The IDF thought this was so remarkable that they did and article featuring SKYPING and the boys. I am so proud of them both. I have always said Jacob was given this horrible disease for a reason and he is helping others by bringing awareness already at 6!

It has been a while since I posted, we have had a busy summer!
After several hospital visits this summer and a change in our infusion medicine Jacob is finally on a good path again! The Hizenta seem to working very well for him he has very few side effects and his migraine headaches he gets from his infusion has decreased drastically and he hasn't gotten sick since starting it!!

Next month I will be running a half marathon with my sister in Washington state. This will be my first one ever. I am going to get shirts printed to support my son and immune deficiency disease to help raise awareness!!!

June 23rd

Oh what a fun month we have had!!!

On June 9th we had to go back to see his immunologist because his ear was still hurting and he had just finished his last round of antibiotics the day before on the 8th. He had a CAT scan done where everything looked good (for Jacob) but his ear was still infected so we were given another 10 day supply of antibiotics, Zithromax.

That night and the night of the 10th he screamed and cried ALL night long. After being unsuccessful with getting him in with his ENT doctor, he was on vacation, and by suggestion of his immunologist we took him to the childrens's emergency room. Well long story short his eardrum had ruptured and at that point cased as outer ear infection where his ear canal had swollen closed. He was given antibiotic/steroid drops and pain drops. We had a follow up appointment today with his ENT and his ear is healing nicely and he suffered no hearing loss!


We started Hizentra last week an so far the infusion results are fantastic! Jacob told me there was no pain during the initial start and it infused much quicker. He had a mild headache about 2 hours after infusing so we gave him some Tylenol and that was the last we heard of a headache. We do infusion #2 with Hizentra tomorrow and I hope the outcome is just as good.

May 17, 2010

I had to take Jacob back to the Dr. on April 27th. He woke up a couple days prior to this and told me his ear hurt but then never said another word. Last night he was crying in pain so I made an appointment at his pediatrician. He had the "Worst infection I have seen in years", I would love to go to the Dr. just once and not hear those words! Anyway, he was given a shot of Roceftin (he said it hurt a lot) and a 10 day supply of Suprax. She only give one or the other but since it's Jacob she wanted to make sure we got everything cleared up.

On May 7th we had an appointment with his Immunologist to have his ear checked out and to talk about switching him to Hizentra. His ear looked good and we are going to get him switched over ASAP, pending insurance approvals. We will have a infusion nurse sent to our house for the first two infusions on Hizentra to make sure he has no adverse reactions. He will also be getting an increase in his Immunoglobulin since he has been on a downward spiral since he got the Swine flu in February. He gets his IGG levels check again in September and if he gets another infection within the next several months he will be getting a CAT scan to make sure the infections are getting cleared and he is just not "reinfecting" himself with the same infection that hasn't been getting cleared up 100%. He will also go through round 2 of the allergy scratch test this summer. He had it done 2 years ago and only showed an allergy to Aspergillis, mold, but he ALWAYS has some sort of post-nasal drip and is constantly clearing his throat.

Jacob has made a online SKYPE friend that has CVID like him and we will be meeting in person in July. Jacob is so happy to have met him.

March 25, 2010

Well we have been off of antibiotics for about 24 days and had to start theme back up again. We are on a 14 day supply of Ceftin and on all the nasal sprays and of course the nubulizer treatments throughout the day. If we don't show signs of improvement by the beginning of next week then back we go to see our immunologist.

Jacob made a MAJOR break through last night during his infusion. For the first time ever he didn't fight and it only took one person to do it. I am so proud of him!

March 15, 2010

Well we broke our long streak of not being sick. On February 10th Jacob shot up with a 104.9 degree fever. I took him to the Dr., unfortunately his regular pediatrician was not in the office and we had to see another one in the office. I HATE seeing another doctor in the practice, they don't understand him! Anyway, she wanted to test him for strep and I insisted on getting him tested for the flu and guess what? He came back positive for the flu that came back as H1N1. Great. I asked for an antibiotic since the flu ALWAYS turns into a more severe bacterial infection. Well she refused since the antibiotic doesn't help with the flu..No kidding!

Several days later we had to go see him immunologist since he turned bacterial. We got started on on a 14 day supply of Ceftin and had to go on nebulizer treatments since of course it is in his lungs. He got lung function test and it looked good but a little low. Anyway, he has recovered and told me "The swine flu wasn't as bad as thought".

He started FINALLY not fighting us over his infusion, we had been having to sit on him to hold him down. I tried putting a blanket over his head so he couldn't see what was going on and that did it...two weeks in a row no fighting!!!!

December 2009

We are gearing up for Christmas and to see grandma in Florida. Jacob has had to make sure he tells Santa that he won't be at home but instead in Florida.

He has been doing so well lately. He last levels came back at 1196 and he has managed to stay well. We have also gotten many phone calls from his school altering us that kids in his class have gone home sick with the flu and so far we have dodged the bullet several times.

He is still having a lot of anxiety over the needle part of the infusion and he is now seeing a play therapist that is helping him understand and help him work through his fear and anxiety.

His next scheduled appointment isn't until February and if he makes it that long with out being sick then he has made it well over 6 months. February will be 1 year since we started his infusions and we have gone from being constantly sick unable to be off any antibiotics for more than 2 days with out getting sick to an astonishing 3 total infections in a 1 year time span. If only we could achieve that goal without the infusions but it will never be. I am just thankful that we caught his disease at an early age so he could start getting treatment he now has the potentional of living a full life instead of a painfully short, that would have occurred due to irreversibly damaged internal organs caused from repeated and severe infections his entire life.

Everyone have a Merry Christmas. I know we will !!

October

Here we have made it through another month with out any infections! So far we have dodged the flu but it's still early in the season. His "pokies" aren't going as good as they were, we hit a bad spot one week that caused a lot of pain and now the fear has set back in again. We are still making small steps from where we started. I have discovered though the further I deviate from the middle of him belly the more it hurts, so I try to stay as close to the belly button area as I can.

He took his 36th infusion this week! I can't believe that much time has past!

In these 36 weeks we have been on antibiotics twice. Not to bad considering he started on antibiotics at 6 weeks old and has had very few breaks off of anitbiotics in his 6 years of life up until 36 weeks ago.

He is still being very cyclic. We infuse every Thursday. By Tuesday he is getting the congestion and the coughing that lasts until around Sunday. This morning, Saturday October 10, he had to get up early because he said laying down was hurting his lungs and making him cough. I suspect if this week goes like all the prior weeks he will be "well" again for his two days of the week then start his "coming down" with something cycle on Tuesday again.

He goes to get his blood tested on this Wednesday to see if the amount of Vivaglobin needs to increase. If we do have to get an increase Jacob is going to be MAD since he is at the max amount of one site and will have to move to two sites each week.

I go get the rest of my blood work done in 2 weeks to see if he has XLA instead of CVID.

We have been put on flight restrictions with him over the winter months and we can do NO international travel. Since his body doesn't make the proper titer response he can become an open book to all the odd diseases that we as a nation get vaccinated against. Many people don't understand this!

September 2, 2009

Jacob is doing much better and it has only take 1 course of antibiotics for 14 days instead of 3 months of antibiotics and switching to a new one every 21 days!

We had a little accident that turned in to a something great. My grandmother, Mimi 88 years old. she fell at the house and broke 2 vertebrae in her spine. Well long story short she had to have a spinal procedure and Jacob told Mimi if she was brave for her needle pokes he would be brave for his. He held up his end of the bargain and for the FIRST time ever we didn't have to chase him down or hold him down to put the needle in his abdomen. He lied down and tome me when he counts to 10 do the "pokie" on 10. I did exactly as he asked. We were SO proud of him!

Mimi had her procedure and did fantastic and after over a week in the hospital was able to fly back home with no pain and a complete sign off from here doctor!

August 19, 2009

The good news and the bad news!

Good news Jacobs IGG total came back at 1096!

The bad news, Jacob couldn't fight the sick bug any longer:(

6/24/09---Started coughing and getting that general "gurggly"lasting throughout the week

7/31/09--Minor Coughing back and constant throat clearing lasting throughout the week

7/7/09--still coughing and constantly clearing his throat and lasting throughout the week

7/12/09--started coughing again (had a couple day reprieve after his infusion) also had minor
sinus congestion. He had his infusion on 7/15/09

7/29/09--Serious sinus congestion starting

7/30/09--Woke up at 0430 with Croup sounding cough, sore throat and severe sinus
congestion. Started getting headache and gave him Tylenol at 0830. All symptoms
continued to last throughout the entire week with out much change.

8/2/09--serious sinus congestion and constant cough. That night at 2200 he began getting a
migraine headache and abdominal cramps. He started projectile vomiting several times
throughout the night.

8/3/09--Woke up in the morning with headache continuing and still having severe sinus
congestion and "wet" cough.

8/6/09--Today his sinus congestion turned from clear to yellow/green.

8/10/09--Sinus congestion continued and drainage would toggle from clear drainage to yellowish
drainage. On this day his drainage turned greenish and having wet, choking cough.
Called his Immuno and was put on a 14 day supply of OMNICEF.

He is stating to do a little better with his infusions. The last 2 weeks he hasn't cried with the "pokey". I have been asking him question during the "poke" minute that he has to think about so has started thinking about the answer instead of the "poke". I have discovered that if I undo the clip to stop the flow on the tubing he dosen't get the initial sting with the start of the flow, this has helped also. However, we still have to hold him down but progress is being made. We are currently entering into week 28.

His immuno Dr. has done a complete run-up of all my levels. There is some indication that he might possibly be XLA as opposed to CVID so I am getting al sorts of tests done to see if I am the carrier.

July 5, 2009

Here is a picture of him getting his infusion. It lasts about 1.5-2 hours. The infusion really makes him tired but he tries to fight the tiredness this week he gave in and about 5 minutes after taking this picture he was sound asleep.

Well Jacob has still not gotten sick! We are getting very close to the two month mark without any infections! I think this is close to the longest ever time period without an infection. This week he didn't have any headache side effects but he has complaining that his legs have been cramping don't know if they are really cramping or if he just wanted out of the car. His body is still trying to recuperate from Disney. He starts showing signs of starting to get sick on Wednesdays so we have moved his infusion day up to Thursday and so far the IGG boost on Thursdays have kept him from getting sick. His follow up appointment is in the end of July. I hope we can make it that far.

June 24, 2009

Well we got the results back from his most recent blood tests and his IGG levels are perfect. So we keep infusing the same amount weekly 16ML. We will go back and get his blood tested again in 6 months!
We just got back from Disney for the IDF Conference and what a bunce of great information we received. The best part about the entire conference is my son met another little boy the same age as him the receives the Vivaglobin also. He thought is was great to not be the only person it the world getting infusions.
The bad side of the Conference is it was at Disney so any and every "sick bug" was out and present so he is starting to get sick. He gets his infusion tomorrow so I hoping it will knock the "sick bug" out of his system!

June 10, 2009

We are still going strong over 1 month now infection free! Jacob got his blood tested yesterday, 6-9-09, we will see how his IGG numbers are in about 2 weeks.

His last infusion went very well no side effects and only took 1 hour 23 minutes. The infusion prior to that one May 29, he had horrible migrains that started on Monday around 5 pm. I kinda had a feeling he was going to get one because we didn't push him to drink on Saturday like we normally do. His Migrain was awful nausea, sensitivity to light and of course the headache part.

I gave him some Tylenol and he passed out on the couch. I woke him up 2 hours later to see if he wanted any dinner and to get him to drink. His head felt better but of course about 3.5 hours later the headache was coming back. He woke up Tuesday with a headache but not a migrain. He had a slight headache for the rest of Tuesday but by Wednesday it was gone! This infusion for some reason took 2 hours and 3 minutes to complete.

We are packing up and leave tomorrow for the IDF convention in Orlando. I know we are going to get a lot of information and Jacob is going to meet a lot of kids that goes through the same stuff as him.

I will update his current IGG levels when we get them.

2008 IG Levels

This year our levels dropped. I will indicate the levels below the effective range to fight infections in blue.



IGG Serum 634 (Range 308-945)

• Subclass 1 406 (Range 592-1723)


• Subclass 2 97 (Range 2.0-112)


• Subclass 3 46 (Range 10-122)


• Subclass 4 6.7 (Range 61-345)

IGG Type B .22 (Range 1.0)

IGG Streptococcus Serotypes: Effective range for ALL serotypes 2.0 The <0.2 means there was not enough antibodies to register a total.

• Type 9 (9N) <0.2
• Type 8 0.6
• Type 68 (9V) 0.5
• Type 56 (18C) 0.5
• Type 51 (7F) 0.2
• Type 5 0.3
• Type 4 1.5
• Type 3 <0.2
• Type 26 (6B) 1.0
• Type 23 (23F) 0.6
• Type 19 (19) 7.3
• Type 14 0.5
• Type 12 (12F) <0.2
• Type 1 <0.2

IGA 58 (Range 33-235

IGM 40 (Range 36-314)

2006 IG Levels

We received the Pneumovax in 2006 to see hom his body responded the IGG boost. This test was taken exactly 6 weeks after the Pneumovax was administered. The numbers in itilacs & red are the previous years numbers.

Pneumococcal Antibody IGG
12 Sterotype Panel range for all 2.0

• Type 1 3.1 1.7
• Type 3 3.5 .06
• Type 4 >10.4 2.3
• Type 26 (6B) 25.8 1.2
• Type 8 >9.2 0.5
• Type 9 (9N) 2.6 0.5
• Type 12 (12F) 0.7 1.3
• Type 14 12.2 2.3
• Type 19 (19F) >23.2 7.5
• Type 23 (23F) 27.9 1.6
• Type 51 (7F) 4.3 1.8
• Type 56 (18C) 16.7 1.8

IGA Serum 40 52 (Range 24-121)

IGA Subclasses

• IGA1 33 (Range 46-378)
• IGA2 7 (Range 13-91)

2005 IG Levels

2005

IGG Serum 691 (Range 533-1078)
IGG Subclasses

• IGG 1 492 (Range 315-1100)
• IGG 2 79 (Range 35-285)
• IGG 3 60 (Range 11-66)
• IGG 4 5.9 (Range 1.0-120)

Type B IGG .12 (Range 1.0)

Pneumococcal Antibody IGG
12 Sterotype Panel (Range for ALL sterotypes 2.0)

• Type 1 1.7
• Type 3 .06
• Type 4 2.3
• Type 26 (6B) 1.2
• Type 8 0.5
• Type 9 (9N) 0.5
• Type 12 (12F) 1.3
• Type 14 2.3
• Type 19 (19F) 7.5
• Type 23 (23F) 1.6
• Type 51 (7F) 1.8
• Type 56 (18C) 1.8

IGA Serum 52 (Range 24-121)

IGE Serum 9 (Range 93 or less)

May 4, 2009

We have finished our full course of antibiotics so now it's a waiting game to see how long we can get in between infections. The infusions are going well and his body is tolerating them VERY well. We still need to work on his mental stress from "the sharp needle". Once the needle is in he is fine. He still infuses 16ML of Vivaglobin every Friday. We are doing our best to avoid this "dreaded" N1H1 Flu. Many of the schools around us have closed due to the flu but so far we are in the clear.

He recently participated in a Karate competition, he place third in the sparring . His other extracurricular activities for the week were canceled due the N1H1 Flu.

We are gearing up for the IDF Convention in Orlando. For myself and my husband this is going to great learning experience. For my little guy its going to be an opportunity to go see Mickey Mouse. He also will get to meet other kids that share the same story as him. Hopefully this will help him realize he is not alone. We are in the process of getting him set-up with a pediatric counsler that specializes in children with chronic conditions.

Next month he gets his blood drawn to see where his IGG levels are and to see if his Vivaglobin dosing needs to adjusted.

April 15 2009 -starting a new antibiotic (not new for the first time taking it)

On Monday we had to start a 21 day supply of Omincef given 2x daily. We have to take 21 days because he has built up such a resistance to antibiotics standard amounts no longer work. Once we finish our 21 day supply we will have to go back to the Dr. for an X-ray and if there are still signs of an infection then we are back on another 21 day supply. Jacob hasn't been on antibiotics for 3 weeks and this is AMAZING for us.
Our current medications are:
Xopenex (nebulized)
Pulmicort (nebulized)
Astalin
Nasonex
Zrytec
singulair
Omincef
and nasal wash
On Friday 4/17/09 we will do our weekly infusion of Vivaglobin.

He has tolerated the transfusions very well. He has very few side effects. The one thing we did learn early on is (1) don't premedicate with Motrin only use Tylenol (Motrin gave his severe headaches) (2) FLUID...FLUID...FLUID it takes 48 hours for your body to adsorb the Vivaglobin the more you drink the less likely to get headaches and abdominal cramps.

He is living the life of any regular 5 year old boy. He is in school, plays soccer and is an orange belt in Karate.

Our journey with CVID

My son Jacob has been diagnosed with CVID. This has been a difficult journey in his 5 years of life. He has currently started (8 weeks ago to be exact) SCIG, Sub-q. He transfuses 16ML of Vivaglobin every Friday. His transfusion takes around 1.5-2 hours through 1 site.

My goal for this blog is to help create a level of awareness and to document our journey. The first thing I did when Jacob got diagnosed was scour the internet for anything I could find from someone living the experience. I wanted real life experience not necessarily medical advice.

I want to post an information sheet I have put together from many different resources to help people understand CVID and what some of its signs and symptoms are.

Primary Immune Deficiency
CVID

CVID (Common Variable Immune Deficiency) is a disorder characterized by low levels of antibodies and an increased susceptibility to infections. CVID is the most common immunodeficiency, hence, the word common. However, CVID is registered with the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that CVID affects less than 200,000 people in the US population. The clinical signs and symptoms also may vary from severe to mild. Frequent and unusual infections may first occur during early childhood, adolescence or adult life. In the majority of patients, the diagnosis is not made until the 3rd or 4th decade of life. However, about 20% of the patients have symptoms of disease or are found to be immunodeficient under the age of 16.

The cause of CVID is unknown. In some cases, more than one individual in a family may be affected, while in other cases this is no not the case. There is a decrease in the number of immunoglobulins (antibodies) in the affected person. Immunoglobulins are produced by the body and are necessary in fighting infections.

The following are the most common symptoms of CVID. However, each person may experience symptoms differently.
Recurrent infections that can affect the ears, sinuses, and lungs (the more these infections occur, the greater the risk of scarring and permanent damage to the lungs and breathing tubes) these infections respond poorly to antibiotic treatment
inflammation in the joints of the knees, ankles, elbows, or wrists
stomach and bowel disorders
increased risk of developing cancer
poor titer levels in response to vaccination
Other symptoms of CVID include:
chronic swelling of lymph glands
enlarged spleen
pernicious anemia
tiredness
bacterial overgrowth of the intestine
Primary Immune Disease can be easily diagnosed by blood test ordered by an Allergy, Asthma & Immunologist. You can also request blood test be done on a newborn in the hospital.

The well know case of Immunodeficiency Disease is “The Bubble Boy Disease”. Medical treatment has improved that patients no longer live their life in a bubble. Patients can now receive several different treatment options. Treatment usually consists of immunoglobulin therapy, which is an infusion of human antibodies harvested from plasma donations. This is not a cure, but it strengthens immunity by ensuring the patient has “normal” levels of antibodies, which helps to prevent recurrent infections.
Intravenous immunoglobulin (IVIG) done in a hospital setting, usually done every 3-4 weeks and takes 8 hours to infuse
subcutaneous immunoglobulin (SCIG) done at home, done every week and takes 2-8 hours to infuse depending on how many sites (needles) are used
intramuscular immunoglobulin (IMIG) not a popular treatment method, less effective and painful

http://www.primaryimmune.org/

http://www.jmfworld.org/