Living With Neutropenia (or) God Dammit Not Again!

If you know me, you probably know that I'm sick a lot. You may also know that I have a low white cell count. You may even know that the disorder I have is called Neutropenia. What you may not know is how this disease "works" and how low my cell counts are. Well, for a starter here's a link to webmd that might give you some idea of what the disorder is. In a nut shell, Neutropenia is a relatively rare blood disorder in which the patient presents with an abnormally low neutrophil count; usually less than 1,700 neutrophils per microliter of blood, although this can vary from MD to MD. Cases can range from mild, moderate or severe and from acute, chronic, and cycling. Where am I on this fantastic voyage? WELL...

When I was 15 I got sick. I slept for 14 hours straight, felt like crap, and had a strange rash on my hands and feet that didn't itch. My mom took me to the doctor where my strep test came back negative. My doctor wanted to check my blood to see if I had Mono, which is pretty common in 15 year olds. I got the labs done and was sent on my merry way back home.

4 hours later, at 6pm we got a call from my doctor stating I needed to run, not walk, to the nearest ER and be put in isolation for more labs to be drawn. Either there was an error in the lab, or there was something REALLY wrong with me. I think I donated nearly a pint of blood that night. Apparently my white count that day was 1,200 which sent off major alarms bells for my doctor. The next few months flew by in a flurry of tests and prolonged periods of quarantine. I was not allowed to go back to school for the remainder of school year. This happened in November. I was referred to a Hematologist/Oncologist where I was tested for leukemia, HIV, AIDS, and a number of other diseases that I can't even remember. We tried so many different treatments tests that I almost lived at the office. I was known by name by the other patients who were all pediatric cancer patients. There's an eye-opener for ya. My official diagnosis as of 2000 was chronic, acute, idiopathic neutropenia. Basically: I will always have it, it's pretty severe, and we don't know why I have it.

Your White Cell Count                   My White Cell Count

Roughly between 4,500 and 10,000                                        Floats between 1,900 and 2,100 cells
cells per microliter of blood                                                       per microliter of blood

              Your ANC                                                  My ANC
(Absolute Neutrophil Count)              (Absolute Neutrophil Count)

A normal ANC is above 1,500 cells                               Hovers at 1,050 per microliter of blood, but
per microliter of blood.                                                    can drop to 950 if I have an active infection.

What does this mean? It means that when I get sick, I get **SICK**. There was one year that I had gastroenteritis because the bacteria in my gut decided to throw a party and didn't invite me. And then when I stubbed my toe and it got a little cut on it that turned septic. Oh, and the UTI from hell I got after I had Paul; that landed me in the hospital for three days. Can't forget the ten day stay I enjoyed in isolation after I got bit by a cat AND NEARLY FUCKING DIED. Recurring pancreatitis, cellulitis, upper respiratory infections, and general "infections" are all reasons I've been admitted to the hospital. This is not a good thing for me.

See, hospitals are little petri dishes of disease that love to fester and multiply and morph into scary superbugs that could kill me in a matter of fuckin' HOURS. We don't do the hospital unless we absolutely have to. I will usually try to talk my way out of a trip to the hospital if I can. I'm usually strong-armed into going, however. It's a miracle I ain't dead yet.


Normal treatments for Neutropenia are either a bone marrow transplant (only if it would be beneficial), Neupogen (aka GCSF or Granulocyte colony-stimulating factor), or IVIG (aka Intravenous immunoglobulin).
Yes, it is as painful as it looks.
Bone Marrow Biopsy/transplant: I've had two biopsies in my life (and I recommend you NEVER EVER do that if you can avoid it) and basically my bone marrow is peachy keen! My body produces enough white cells and neutrophils, but something is killing them off. So a transplant is of no use to me.

IVIG: Basically like injecting me with water. Yup, it does nothing. Not even a nudge to my counts. So, that's off the table.

Neupogen: Ahh, this wonderful fuckin' drug. This...this is my nemesis; my crutch; my golden shackles. This drug has been given to me for the last 15 years and usually works. By "works" I mean my cell counts go back to normal. However, it only works for about a week. After the first day my counts begin the rapid plummet and I end up back where I was. The price for all this roller coaster fun? A list of possible side effects (the ones with the * by them are the ones I get to enjoy):

Routine Side Effects

According to the Drugs website, Neupogen routinely causes myalgias* (muscle pain), bone pain*, joint pain* and alopecia (hair loss). It can cause unintentional weight loss and a poor appetite. Neupogen's other common side effects include a headache*, epistaxis (nosebleeds), itching*, swelling*, irritation* and a lump-formation at the injection site. Since Neupogen affects your immune system, you are to expect these side effects. However, tell your doctor if these effects worsen and remain for at least one week.

Serious Side Effects

MedlinePlus reports that Neupogen can cause fever*, dizziness*, wheezing, trouble breathing and abnormal bruising. It can cause eye or mouth swelling, rashes, hives, fever and left upper stomach pain. Neupogen's other dangerous side effects include bleeding gums, bloody urine or stool, hemoptysis (condition where you cough up blood), facial swelling, menstrual irregularities, dysphagia (trouble swallowing) and pale skin, says the Drugs website. Neupogen can cause difficulty urinating, paralysis, a sore throat, fatigue*, chest tightness, profuse bleeding and tongue, lip or mouth sores. Inform your doctor when you develop these serious effects.
In a study of 384 patients receiving Neupogen, the National Library of Medicine says that 57 percent of the patients developed nausea and vomiting, 22 percent had bone pain, 18 percent had hair loss, 14 percent had diarrhea, 13 percent developed a neutropenic fever (type of fever due to low white blood cell counts) and 12 percent developed mucositis (inflammation of your mucusal linings of your digestive tract). Additionally, 11 percent had fatigue, 9 percent experienced anorexia and shortness of breath, 7 percent developed a headache and 6 percent had a cough and skin rash. In the same study, 5 percent had chest pain and constipation while 4 percent had a sore throat.

Other Side Effects

According to the National Library of Medicine, Neupogen can cause the spleen to rupture. Symptoms of an enlarged and possibly ruptured spleen include left upper stomach pain and shoulder pain. Additionally, you should avoid Neupogen if you suffer from sickle cell disease, a condition in which your red blood cells are abnormally shaped. This prevents them from carrying adequate amounts of oxygen to your tissues. Neupogen can worsen this condition.
It is not known if Neupogen can cause harm to your nursing or unborn child. Tell your physician if you are pregnant or breastfeeding. Also, you should not take Neupogen if you are allergic to filgrastim. You can develop potentially fatal complications such as swelling of your throat, hives and difficulty breathing. Finally, avoid combining Neupogen with vincristine or toposome. These drugs may interact with Neupogen and cause the aforementioned side effects.
Read more:

Wanna know what's NOT listed there? Uncontrollable shaking and tremors. In order for me to get a Neupogen treatment I have to be admitted for at least 24 hours to receive IV fluids, zofran for the extreme vomiting and nausea, dilaudid for the pain from headache, joint, muscle, and bone pain (usually in my back), and IV Benadryl to lesson the side effects. By the way, IV Benadryl fuckin' BURNS. If you ever want to see a nurse freak the hell out, you should come to my room when I get my Neupogen treatment. The constant shaking and tremors usually gathers quite a crowd.

You saw where I wrote "usually works"? That's because the last three treatments I got didn't do anything to my counts. It appears to have lost it's efficacy in me. And that, my friends, scares the hell out of me. If I don't have some way to shoot my counts up to normal I am like a sitting duck. So while I've been getting little head colds, paper cuts, or sinus infections I'm really wondering "Is this going to be the one that kills me?" 

Some people think I'm a hypochondriac and I honestly get kinda pissed about that. I *wish* this were all in my damn head. I *wish* I didn't worry day and night that my kids might get it when THEY turn 15. I *wish* I didn't wonder if I'm going to leave two kids and a husband behind because I just couldn't fight an infection any more. I want a normal life. A want a life where a cold just knocks me on my butt for a day or two and then I'm fine. I want a life where I can have routine dental work done, like a cleaning, and not need massive amounts of antibiotics to prevent me from getting some sort of horrific infection. Or being able to not worry about that little scrape on the back of my hand. I want all those things...God, how I want them. But, unless some new miracle drug comes out, it ain't gonna happen. 

Neutropenia is pretty rare. From the Neutropenia Support Association, Inc
The true incidence of severe chronic neutropenia is now known, but these are rare haematological disorders. Based on case findings in the Puget Sound area of western Washington in the US, it is estimated that there is approximately one case per 100,000 population (Dale, unpublished observations). Cyclic neutropenia is probably the rarest syndrome, with an estimated frequency of one per million. Congenital and idiopathic neutropenia are more common, with an estimated frequency of one per 200,000 population. Estimates in other regions suggest perhaps a somewhat lower incidence. Mild neutropenia is more common in certain racial and ethnic groups; it is not known if severe chronic neutropenia is more common in these populations.
There are some doctors who have never even heard of it. Most doctors who HAVE heard of it assume I have it from Chemo. I've never had Chemo. Never had cancer, either. I tested positive for Mono once when I was 15. The generally accepted theory thus far is that the Mono managed to attach itself to one of the proteins in my neutrophils and modified the cell to make it a "foreign invader" to my immune system. Now something is eating the cells thinking it's fighting a disease.

So, when you hear me grumble or see me writing about what may be a perfectly trivial health concern for you, try to remember that it could actually be something that could kill me. 
And I ain't ready to go yet.