myhistiostory

Not only is LCH a very unusual disease and that I'm 1 in a million adults who get it. Not only is it hard to define what it is (autoimmune or cancer). They treat it as both. 

The chemotherapy is for the cancer part of the disease. The steroid injections are for the autoimmune part of the disease. The side effects of both...JOY!!!

Most Common side effects of the particular chemotherapy drug I will be taking (medium-high dosage):

I can feel how weak my body is. My whole pelvis is screaming of pain at the moment. I can feel how lose my hip is, it's so fragile. It doesn't help that I've got a cold. My kidney's are hurting too. AAAAAH...everything is hurting me...I'm in pain...everywhere...

Codeine my love, come to mama. Let's get high shall we??

/H

My dad calls me after getting the rest of the pictures from the scan. My small intestine is highly inflammed and we need to know why? Is there internal bleeding that we cannot detect? Has the histiocytosis infiltrated my intestine? What's going on? Abover that, my muscles in my back and my chest are very very inflammed. Of course, this could be because of tension or walking with crutches. But why? The suspected lesions he found from the CT were also highly contrasted. Additional lesions or something else. The bone marrow in my spine was also highly contrasted. Something to do with my anemia? I DON'T FUCKING KNOW!!! 

Let's find out in a couple of hours. I'm dressed. Had my coffee. Nails painted and pretty. Just going to fix my hair and head off to the hospital. I'm starting to hate hospitals.

/H

OK...so yet again, I started my morning with shitty news. But first, let's turn back time to about mid-August. A radiologist put me through a CT-scan that isn't as advanced as the one's used today. Basically, technology has advanced. That was the first mistake. Mistake number two: she sliced up the pictures really thick. When examining oncology-related scans, you have to slice the pictures up really thin to capture all dimensions and aspects of the tumors/lesions under examination. Mistake number THREE: her conclusion was that the lesion hasn't developed, that it's smaller than what the dr's evaluated in Egypt, and that I have no risk for fracture, AT ALL!!!!

OK...so far so good.

My gut instinct was that something didn't make sense. So, I demanded to do a CT-PET before I started my treatment. They made me wait for 2 and a half weeks.

My dad calls me this morning and tells me not to move, not to go anywhere, and not to do anything. The lesion in my hip has spread so much, there's 9mm left of it. There's nothing left. One wrong movement and I will snap in half. We thought it was bad before, but this is worse. My disease is acting quite aggressively...more than we imagined.

I've been laughing all day. It's either that or crying. I chose to laugh. It's the easier way out of the chaos I'm in.

I came to Örebro walking. I'm going back to Stockholm in a wheelchair.

SMILE...you'll live longer and you'll look prettier in whatever state you're in :)

/H

What do you do when you just don't have the energy one day. Who will fight for you on that day? Who will pick up the pieces? Who will be your voice? Or is it REALLY ok to have an off-day?

I'm going to try to explain this as simple as possible.

Langerhans cells are a type of dendric cells of the skin. They can also be found in different parts of the body.


Histiocytes are cells that belong to the immune system. They are produced in the bone marrow along with white blood cells and red blood cells. They travel through your system to protect your body from infections. One type of these histiocytes are Langerhans cells.


Histiocytosis is the overproduction of histiocytes. Histocytosis is also an umbrella name for a group of rare diseases that share the same characteristics. Histiocytosis can attack any part of the body: skin, bones, muscles, organs (liver, kidneys, spleen, stomach, lungs, etc.) Histiocytosis also belongs to a family of other blood related diseases (diseases that derive from the bone marrow) such as leukemia, lymphoma, and erdheim-chester disease).


So combine the two, Langerhans cell histiocytosis (LCH) = the overproduction of Langerhans cells.


All types of histocytosis are very rare. LCH in particular, is most commonly found in children. The prevalence in adults is even more rare.


Cancer or Autoimmune disease?


Medical researchers have to date not agreed on how to classify LCH. Some argue that it is a cancer because it behaves and acts like a cancer. That is, the abnormal proliferation of cells which is by definition a tumor. Others claim however that it is an autoimmune disease because Langerhans cells are a part of your immune system. So, in this case the Langerhans cells are attacking your own body instead of foreign objects. The most recent research is leaning towards cancer however. But, some still stay firm claiming it's an autoimmune disease. Why people develop this disease is unknown. LCH in particular is a mystery and is commonly known as Histiocytosis X - cause unknown. Maybe that's why it's hard to define what it is.


Regardless of what it is, Oncologists (cancer specialists) and Hemotologists (blood specialists) treat this disease. Why? Read below...


There are several types of LCH.



Type I: unifocal. Benign. Affects only one part of your body, in one system. It is a slow progressing form. It usually involves bones, most often the skull. Can be solved easily with surgery and mild radiation therapy if necessary. In some cases doctors have found that the bone regresses and heals itself. Excellent prognosis.


Type II: multifocal unisystem. Malignant. Affects several parts of one system. For example, several area's of the bone. Other complications follow. Usually chemotherapy in combination with steroids is necessary to treat this type. Prognosis: around 60% chance of relapse.


Type III: multifocal multisystem. Aggressive malignanancy. Affects several systems of the body. If it affects your risk organs such as the liver or spleen, then the prognosis declines severley. This type progresses fast and should be treated with aggressive chemotherapy. The mortality rate is around 10%.


I was first diagnosed with type I. Then with the lesion in my hip, they diagnosed me with type II.


So why am I going through chemotherapy?


Because the lesion found in my hip developed within two months. This is fast progressing which could indicate I'm in a danger zone. If it managed to progress this fast within 2 months, it could mean that there's a high risk it will spread to other bone parts or in worse case, start developing in my organs (type III). So the solution is to start with chemo.

Hope this information helped :)

/H

For all of you that know how this all progressed don't really have to bother with this part ;)

November, 2010

It was a Thursday. I remember it like yesterday, a cozy Novermber Thursday evening. I was on the phone with a friend, laughing, talking, and playing with my hair. I felt a bump on my head. I told my friend laughing. I tried hard to remember if I had bumped my head lately. Nope, no memory of that. You know me, I’m a clumsy ass...I mean, I've literally fallen off a bus...yes..OFF A BIG RED BUS!!! So a bump here and there isn't unlikely in the life of me.

Christmas-January, 2011

Time passed and the bump remained and painfully so. I couldn't sleep on the left side of my head. After a well needed vacation in Egypt over Christmas, I went to the hospital and got a scan. I didn't think much of it.

February

Weeks later, I got a letter home. It was freezing that day, chilling. "bla bla bla....high suspicion of Langerhans cell histiocytosis or a neoplasmic mass." WHAT THE DUCK? (Yes, I love saying that expression!!!). I called my dad, read the letter. He couldn't believe it. I didn't understand. He made me read it over and over again as if he had no memory of hearing me the first time. "Come home immediately, H." In my world, he was just being dramatic. He wasn't.

All I really remembered was my dad showing me the scan of my head on the computer. As he scrolled down, he pointed out the piece of my skull missing. Imagine someone took a bullet and shot it through your head with no exterior wounds, just a clean hole through the skull. He explained what the letter said. Langerhans cell histiocytosis is a rare disease of the immune system. A neoplasm is a tumor. So basically, my alternatives were Bin Ladin or Palin for president of the world (JOY TO MY LIFE!!!). Let’s not even get into the fact that I got this letter sent to me, at HOME, ALONE!!! I was in shock, I couldn't speak. Every word I said was like talking under water. I cried. A lot. For two days straight, I cried.

So a few biopsies later, the Dr's confidently diagnosed me. Langerhans cell histiocytosis (yes it's a mouthful to read). At first I didn't really understand what it was...hell, I could barely pronounce the damn name. I was too busy dealing with the whole ordeal. Reading about it didn't really make any sense either. It took a while for me to understand. But I was naive at the time. I mean, if I was diagnosed with such a rare disease that could have serious consequences, surely the doctors were going to prioritize ME (HELLO!!! CENTER OF THE UNIVERSE SPEAKING!!!!). They would take care of everything. I laugh just thinking about it, I chuckle.

They did NOTHING!!!

March - June

So began the journey of frantically calling doctors, trying to find the right doctors to deal with this, and the worse part, having to explain the entire story from A to Z to every new person I spoke to. So, I did the research. After all, I am a researcher, that's what I do. Finally, I took all the most important research articles, from the high impact journals, the most cited ones, and highlighted the most important findings in each article. I walked up to my doctor and smacked the articles on his desk, and told him "There, I did this for you. Now what are YOU going to do?" Finally, I was immediately sent to the Oncologist and he did a full body scan of my bones and a bone marrow biopsy. The bone marrow biopsy was done to exclude the possibility of leukemia or lymphoma (LCH belongs to a family of diseases, usually diseases that derive from the bone marrow). The results came back negative, I was free of other cancers, and the scan came back negative of other lesions. I felt relieved, I could breathe again. All I had to do was to have surgery on my skull. I chose to postpone it for after August though for personal reasons. It would all be over soon. Or so I thought.

A couple of days after the bone marrow biopsy I started feeling an excruciating pain in my hip and leg. It was the kind of pain that takes your breath away. Every night, I'd feel the pain. I went to the ER, they waved it off as an inflammation from the bone marrow biopsy, "Don't worry, it will disappear, just take a few ibuprofens and you'll be fine." I went home thinking that would solve all my problems. It didn't. As time went, I kept calling the oncologists at the hospital and complained about this horrific pain, the pain that made me want to put a bullet through my head to spare me. "It's ok H, it's probably just an inflammation, it will disappear in time. But just in case, we'll prescribe you some codeine to relieve the pain."

July

Time passed. The pain didn't. It got worse. I had a gut instinct that something wasn't right. So, on my vacation in Egypt, I got a CT and an MRI scan done of my hip and pelvis. "H, your histiocytosis has spread to your hip, almost half of it has dissolved." I didn't believe what the Dr's there were saying. I honestly thought they were being stupid. They weren’t. I was in denial. "Nooooo, I'm telling you, I had a bone marrow biopsy done recently, it's an inflammation!!!" In the heat of my frustration, I grabbed the results and went to my cousin who's a doctor to take a look at them. "H, I'm sorry, but a bone marrow biopsy is usually very small and it's taken in the pelvis. This is your hip bone. They're far apart. And a bone marrow biopsy is usually just about 1cm wide, this lesion is 5x4 cm wide."

August

Nervously, I contacted my Dr over mail and sent him the scans. He booked me in immediately for an appointment the day after I arrived in Sweden. "This is not good, H. This lesion must have developed since your last scan. This means it's become malignant, it's spreading." He started talking, lingering around with his words. That's never good. "Just tell me, what is the treatment?" "Chemotherapy, 6 months". I cried.

I walked out of his office with four legs. He wanted to put me in a wheel chair first but I refused. So he gave me two crutches to walk on. He forbid me to go to London for one of my best friend's wedding. He forbid me to go on a work-trip to Croatia. "Stay put, stop moving. Your hip is ready to snap. You're 27 years old, you shouldn't have a hip replacement at this age."

I called my friend, I was in shock. I tried to laugh it off, but inside there was a child that was terrified. How did this all happen to me? When did this all happen? Why?

I went to the two people I knew could calm me down immediately. My professors. I cried, I talked, and I laughed. And they let me. It was exactly what I needed. They supported me, made me feel better. We talked about it all, everything. We joked about it. We talked about ways to cope with it. And it helped. And honestly, I think that that conversation alone, is what made me feel OK right now. Sure I still have ups and downs. But the downs aren't so far in the gutter. They pass. And I bounce back faster. I see things in a new light now. A positive light.

So here I am. 9 months stronger. Three seasons wiser. A lot more happened, trust me, A LOT MORE!!! This was the SHORT version believe it or not!! I'm writing this blog, mainly to vent and to express myself. I need to. It is what it is. I'm not the type that gives up easily. I will cry. I will laugh. I will think it's unfair. I will develop. I will have my dips. But the best part of it is that I will come out of this, stronger, happier, and healthier, better, and fit for fight. It's been quite the journey, but at least I'm half way through.

At least I can put it on my CV. I've had chemo before the age of 30. What have YOU done before you turned 30? MUUUAHAHAHAHA! Yes, get use to it. Anyone who knows me knows I have a very twisted humor!!

Good night!