On July 4th, 2016, I embarked on a journey that would take an entire year to complete. I wanted to show what goes into managing Gavin's immunodeficiency for one full year. I wanted to show everything that Gavin has to go through, simply to keep from getting seriously ill. After discussing this with Gavin, he liked the idea and together, we began preparing for this post, one infusion at a time.
What is Common Variable Immunodeficiency? Before we get into this, let's first explain what *Common Variable Immunodeficiency *is, and how it impacts Gavin's life.
The below information is courtesy of the NIH
Common variable immune deficiency (CVID) is a disorder that impairs the immune system. People with CVID are highly susceptible to infection from foreign invaders such as bacteria, or more rarely, viruses and often develop recurrent infections, particularly in the lungs, sinuses, and ears. Pneumonia is common in people with CVID. Over time, recurrent infections can lead to chronic lung disease. Affected individuals may also experience infection or inflammation of the gastrointestinal tract, which can cause diarrhea and weight loss. Abnormal accumulation of immune cells causes enlarged lymph nodes(lymphadenopathy) or an enlarged spleen (splenomegaly) in some people with CVID. Immune cells can accumulate in other organs, forming small lumps called granulomas.
Approximately 25 percent of people with CVID have an autoimmune disorder, which occurs when the immune system malfunctions and attacks the body's tissues and organs. The blood cells are most frequently affected by autoimmune attacks in CVID; the most commonly occurring autoimmune disorders are immune thrombocytopenia purpura, which is an abnormal bleeding disorder caused by a decrease in cell fragments involved in blood clotting called platelets, and autoimmune hemolytic anemia, which results in premature destruction of red blood cells. Other autoimmune disorders such as rheumatoid arthritiscan occur. Individuals with CVID also have a greater than normal risk of developing certain types of cancer, including a cancer of immune system cells called non-Hodgkin lymphoma and less frequently, stomach (gastric) cancer.
People with CVID may start experiencing signs and symptoms of the disorder anytime between childhood and adulthood; most people with CVID are diagnosed in their twenties or thirties. The life expectancy of individuals with CVID varies depending on the severity and frequency of illnesses they experience. Most people with CVID live into adulthood.
There are many different types of CVID that are distinguished by genetic cause. People with the same type of CVID may have varying signs and symptoms.
As far as we know, Gavin doesn't suffer from any form of autoimmune disorder. He's undergone extensive genetic testing, participated in a major study at the Cleveland Clinic, and yet the cause of his condition remains unknown.
Regardless of the cause, the treatment is IVIG infusion therapy. Without going into the complex mechanics of this process on an immunoglobular level, let me explain it like this.
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What is IVIG Therapy and how does it help Gavin? Every Monday and Friday, Gavin receives a subcutaneous infusion of donor antibodies, directly into his belly. These donated antibodies, essentially function as a temporary immune system, replacing his own missing or defunct antibodies.
Each batch of medication (called GAMMAGARD) is created from the blood serum of 10,000 - 15,000 different people.
In Gavin's particular case, the antibodies he needs are IgM and IgA. For whatever reason, Gavin's body produces less than half of the IgM it should, and what it does produce is defunct or non-functioning. As things have progressed over the years, his IgA has become low as well.
Over the last year or two, we've also been keeping a close eye on his platelets because they've been dropping as well, sometimes significantly enough to be concerning.
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*Click the above image to view the gallery* Each infusion provides him with an acting immune system for about a week. The timing and space between infusions are purposeful. We want to provide enough overlap that should there be a delay in a single infusion; he will be okay. This buffer of sorts has come in useful on many occasions over the years.
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What goes into an IVIG infusion? The process of the infusion itself is relatively simple, but it can be very painful for Gavin. There are several pieces of equipment that go into each infusion. There's an external pump, two subcutaneous needles, tubing, extension tubing, alcohol prep, obsites, three vials of GAMMAGARD, one 18 gauge needle, and a 60cc syringe.
We assemble the tubing, draw up the solution, prime the pump, disinfect his belly, place the needles into his belly, cover them with obsites, and turn on the pump.
Before beginning the infusion, Gavin needs to take medication to help prevent or limit side effects. He takes Motrin for pain, Zofran for nausea, and Benadryl to help his body accept the donor antibodies. We used to numb the areas where the needles were placed before the placement, but it took much longer, and as Gavin has gotten older, he decided to skip that part.
Each infusion can take anywhere from one to six hours to complete. It depends on needle placement, and honestly how fast his body absorbs the medication.
While we do this procedure at home, it can be very painful at times. When the infusion completes, there are two almost half racketball sized lumps at the infusion sites. These go away over the next few hours, as his body absorbs the medication.
After each infusion, Gavin will usually sleep for several hours. These medically necessary infusions take a great deal out of him, and his body is exhausted afterward.
Most of the time, he doesn't experience any significant side effects, but sometimes he gets sick to his stomach. Sometimes he gets a bad migraine, and other times he deals with both. Amazingly, in all the years he's been receiving these infusions, either at home or in the infusion lab at Akron Children's Hospital, he's never once complained. I find that truly inspiring.
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This is what goes into one year of IVIG infusion therapy? Over the last year, Gavin and I have been saving up all the empty GAMMAGARD vials used for each infusion. We wanted to be able to show you how much goes into managing this serious and sometimes life-threatening disorder.
We've tallied up everything that has been needed to make it through the last year of treatment, and it goes as follows:
- 314 vials of GAMMAGARD
- 192 injections, not counting any re-sticks needed
- 96 syringes were used to draw up and infuse the medication
- 192 subcutaneous needles
- 192 sets of tubing
- 96 alcohol preps
- 96 eighteen gauge needles
- 2 extra large Sharp's containers
- 96 total infusions
- 3 or 4 trips out of town to see his immunologist
- Roughly $274.73 per week
- Roughly $13,187.04 per year
I've also researched the cost of this treatment over the last year. Gavin's on disability, and we are blessed because everything has been covered. The cost listed above is what Medicaid has paid, however, the actual cost is significantly higher. Without insurance, these treatments could cost roughly $26,000 a year. This number may vary depending on actual treatment needs.
Keep in mind, that I performed these infusions myself. Therefore there wasn't the additional cost of medical staff, visiting nurse or hospital stay. The cost with that included, both with and without insurance would be astronomically higher.
Gavin will need these infusions for the rest of his life. This will never get better, and will likely continue to get worse over time, requiring additional infusions per week.
We've been holding steady over the last year and for that, I'm incredibly grateful.
I'm hoping that this article helps to raise awareness for people with disorders like Common Variable Immunodeficiency. I wanted to show you what Gavin endures over the course of a calendar year, and hopefully, provide insight into all that goes into managing this kind of disorder.
Gavin hopes that anyone going through something similar, will read his story and know they aren't alone.
*A side note, that may or may not be relevant by the time you read this. *
*At this moment in time, the Republicans are currently trying to repeal and replace the ACA or Affordable Care Act. What they have presented the American people as a potential replacement is considered an absolute disaster by all major experts and medical organizations in the field. *
*If this bill passes in its current or even similar form, millions of people will lose their insurance coverage. No one can tell me for sure, whether or not Gavin will be impacted by this new bill, should it pass. *
*I pray that whatever ends up happening, doesn't negatively impact the lives of those most in need. People like Gavin need IVIG therapy covered in order to survive. *
*There are a great many things to worry about if this bill passes, and until we know anything for sure, people like Gavin and millions of others will be living with great uncertainty as to what the future holds for them. *
