This is an x-ray (actually a Myelogram from 1997) of my lumbar fusion. Much has changed since then - newest addition: a fibrous tumor on my spinal cord
This was properly diagnosed in 1980. In 1995, I discovered that my neutropenia had converted to a rare form of Neutropenia called Severe Chronic/Congenital Neutropenia, which also included Kostmann's Syndrome (Agranulocytosis), which caused my white cells and neutrophils to bottom out to near nothing and stay that way. This left me with no protection against germs, illness, bacteria and such. My bone marrow stopped making these important cells. This was confirmed with a bone marrow biopsy. I have had many bone marrow biopsies. They hurt like hell even with the sedation. Click here to learn how this is done.
Kostmann's Syndrome is a congenital disorder that causes an impairment of myeloid differentiation in the bone marrow characterized by severe Neutropenia, which can be treated with recombinant human granulocyte colony-stimulating factor (G-CSF) name brand Neupogen.
One in 1 Million people have what I have and it is not contagious. A person can contract this only if they receive my bone marrow, which isn't even a remote possibility.
In 1995, by the time they found I had mutated to SCN, through a series of daily blood tests for 4 weeks straight, I was sick, down to about 125 pounds and very anemic. They started me on an IV iron infusion delivered in the chemo room for 8 hours to help resolve the anemia, which after an infusion takes a couple weeks for the hemoglobin and hematocrit cells to raise to normal levels. Then the following week I was started on Neupogen.
The first injection put me in the E.R., with a low BP and rapid heart rate, vomiting, fever, migraine, severe muscle aches (flu like symptoms) and seizure like chills. The E.R. attending told my mother he wasn't sure I would make it. But, being stubborn, I did.
Neupogen still makes me sick to this day but only that severe each time I get a new lot/shipment of Neupogen.
Now, some think I have to be exaggerating or faking how sick Neupogen makes me because they know someone who is on it or they work with patients who are on it, who don't have the reaction to it that I have.
I say to those people, kiss my ass.
If drugs worked the same in everyone, no one would have an allergy to any medication. Everyone would react the same. Hell, everyone would be able to take penicillin. We know that's not true, right?
It stands to reason that just because so and so doesn't react to Neupogen the way that I do, doesn't mean it doesn't make me very sick. I am one of the few people who has this kind of an adverse reaction but I am far from being alone. I believe the SCN registry has 2 others on board having the same reactions. Trust me, after 12 years of this shit, I am tired of feeling like hell after an injection and if I had a choice, I wouldn't give them to myself because of how they make me feel.
I have an adverse reaction to every injection, every time. To what degree depends but I can never foretell when it will be very bad. For the most part, after any injection I give myself, I feel like I have the flu, extreme nausea, chills (sometimes so violent it's like small seizures), everything hurts to the touch, big time migraine headaches; I am very fatigued and I have strong bone pain in my rib cage and pelvis.
Yet, I cannot stop it - my very life depends on it. If I ever got bad enough to want to commit suicide, I wouldn't have to take any pills or use a bullet; I just stop my Neupogen and wait to catch something then let it end me.
But, I press on. My side effects are treated with daily anti-emetic (nausea) medication, Phenergan 50mg, lots of Tylenol and Aspirin and a strong slow release pain medication, which is also used to help my chronic back pain.
Since March of 1995, I self inject Neupogen daily using an insulin syringe. The medication goes sub-q just under my skin. I am a member of the Severe Chronic Neutropenia Association Registry, whom arranged for me to get my medication free through the maker Amgen.
This is the only way I can afford my medication as a small 480 mic vial, which is a one use vial, costs around $200. I go through one per day. A 3 month supply would normally cost me $18,000.00. That's eighteen thousand dollars.
Kostmann's Syndrome (a congenital neutropenia) can evolve to AML leukemia and require a bone marrow transplant and chemotherapy. I could find out if this is the next phase for me, the mutation into AML leukemia if I take a chromosome test. The culprit is a defective monosomy 7 chromosome.
I am not placing myself on the Bone Marrow Registry for a variety of reasons. One being that doing so means that I might be inviting a problem. I can get on it in an instant if I do convert to AML, so why tempt fate? The other is, unless there is a problem, I doubt I could sign up.
AML Leukemia is a very nasty leukemia. It's treatment is a bone marrow transplant, chemo...which in itself is risky enough to kill people with fairly good immune systems, even though your existing one must be killed off first before the person can receive another's (donor) bone marrow.
The catch 22 of this whole thing is that they have found that Neupogen exacerbates the problem meaning makes the threat of mutating to AML worse...but again, I cannot stop the injections. And I just learned in the last 18 months that Neupogen causes bone thinning, which is great news for my fusion (I am being sarcastic) beings that I have 6 pedicle screws as big around as a little finger, in 3 of my vertebra. Yippie! One more delight from Neupogen use! (still being sarcastic here)
Since 1995, I have been going every 2 months for a CBC with differential blood test to check how the Neupogen is working and every 6 months for my check-ups.
There you have it. I didn't post this for sympathy; just for a little understanding because though I offer to help others and maintain the website, I simply cannot be everything to everyone, as hard as I may try. I am having to take more and more time for myself than ever before and I need my visitors to understand why I cannot answer their email right away or tend to their needs as quickly as I have in the past.
I am doing my best but now it's time for me - for once.
Nick