Category: Bleeding Disorder

Listening to the Unseen

Close-up view of an elephant’s eye, showing detailed skin texture and wrinkles surrounding the eye area.
~ Linda Wright

Inspired by reading “The Invisible Kingdom: Reimagining Chronic Illness,” by Meghan O’Rourke 

The physician’s assistant asked, “On a scale of one to ten, how bad is the pain?”

I responded, “Four… maybe?

Pain is a solitary experience, something no one else shares, it is always hard to describe. For me it is more like a noise getting louder or softer. It comes and goes and when it goes, it mostly disappears from my memory. Words never quite convey the feeling… the ache, the sharp stab, the feeling that something inside is pulling tissue deep inside my gut. 

Not only that, how could I possibly categorize this pain on a scale with one I have had before… like the times I have sprained an ankle, or the time I could barely breathe after the car accident when the airbag cracked my sternum. I’m glad I cannot bring those sensations back clearly to my mind. I remember them only as events that I want to forget.

On a scale of one to ten, how bad is the pain? This question does not ask me to describe the complexity or the variability of what I feel. It is not one sensation, nor is it the same from minute to minute. In frustration, I mutter, “I hate this question.”

Still I want others to understand that I am in pain. It takes energy to hide or pretend. When I try to communicate with friends, it stirs something in them. Many feel some responsibility to respond. Some encourage me to think more positively. One offers a suggestion, intended to be helpful. “Try writing from your non-dominant hand and see what you learn about the source of your pain.”

“I haven’t been able to write easily even with my dominant hand since a hemorrhagic stroke left some Wernicke’s aphasia two dozen years ago,” I mutter. Frustrated, I struggle to show appreciation for all suggestions, but I can’t always summon genuine gratitude.

There may be nothing someone else could say that would truly help, though people try. Those who are uncomfortable with illness or disability often respond with sympathy, or worse, praise for my endurance. I feel the gap between us widen, a chasm between us that words cannot bridge.

When my primary care doctor enters the examining room. I say, “I imagine that this pain might be caused by adhesions from the liver transplant I had nine years ago. It has been sporadic now for a few years, usually lasting a few days or more, then disappearing. This time it is not going away,”

She believes me and I am grateful. There have been lots of times when doctors have not believed my uncharacteristic symptoms.

She orders an X-ray of my lungs and ribs. The pain seems to be radiating from my lowest rib and it doesn’t increase or decrease when she presses on my side and abdomen. She also orders a short term dose of Prednisone.

I know that all medications, even those that help, come with consequences. Today the pain is gone. Even the chronic ache in my shoulders has quieted for now.  I’ve been more restless. I crave sweets. 

Yesterday I took the last dose. The pain is gone. Even the chronic ache in my shoulders has quieted. The side effects are diminishing. And now that the medication is finished, I can admit what I couldn’t before. I am tired of pretending I’m fine; tired of reassuring my loved ones, my friends… and myself. Because what cannot be seen is still there, waiting, whether anyone else knows how to measure it or not.

Rare Disease Day 2016

~ Linda Wright
 I see a dermatologist once a year just for a check. It happened that my appointment this year was on Rare Disease Day. The doctor said, “How long has this spot on your face been there?”

“I don’t remember,” I said.

“I don’t like the look of it.” He left the room and came back wearing magnifying goggles. After he stared at it through the glasses, he said, “I’m going to biopsy this spot.”

“Remember, I have a bleeding disorder.”

“It won’t be a problem,” he said. “I’d sleep better tonight if I biopsied it.”

If my brain had not shut down after hearing the word “biopsy,” or if he had not made it sound so urgent, I would have said, “I’ll need to get an infusion before you do any cutting.” When I feel afraid, it shuts down my thinking temporarily. I was in flight response and I just wanted this suspicious spot on my skin off.

The doctor left the room and the nurse snapped a photo of the spot. Then she turned her back to me while she uploaded the photo into the computer. Much to my surprise she had a syringe and was pointing the needle at my cheek. “This will sting a little, but it will numb the area.”

“No,” I said switching to fight mode. “You’re not going to inject anything. It will only make a second place to bleed.”

She made a face and said, rather gruffly, “I’ll have the doctor come speak to you.”

When the doctor came in he said, “What do you want me to do?”

“Either use a topical anesthetic or nothing.”

“We can put a topical cream on and wait several minutes,” he offered. I could hear the hesitation in his voice. Was I slowing down his efficient routine?

“I’d rather you used nothing for pain.” I glanced at the nurse who visibly cringed.

“Ok,” and off he went to get his surgical kit.

In two seconds he was done. Then he put a gauze pad on the wound and told the nurse to hold pressure on the spot for five minutes. “It’s just oozing,” the doctor said confidently.

That was when I began blaming myself for allowing this procedure to be done before getting a dose of fibrinogen first.

Five minutes later the nurse pulled up the pad and peaked. “Still oozing,” she said.

I refrained from saying, “I told you so.”

After another five minutes the cut was still oozing. Again in another five minutes, it was still oozing.

“Can you hold the pad while I get the doctor?”

“Sure,” I said. I sighed heavily, realizing that half of tomorrow would be spent getting an infusion. It was too late in the day to get it done now.

The dermatologist came in looking a bit concerned, but not apologetic. What was normally easy for him, looked more challenging now that he understood the consequences of what he believed was a simple procedure.

“We could put some jell foam on it and bandage it securely,” he offered.

“That would be a good idea,” I responded trying to cleanse my tone of sarcasm.

The nurse muttered, “I’ve never used jell foam.”

Before the doctor could respond, I said “You’ll have to sterilize scissors and cut a small piece to put over the cut. We used to keep jell foam in the house when I was a child and my mother sterilized the scissors with rubbing alcohol.”

“Right,” the doctor said, “and crisscross the tape to keep the bandage on tight.” Off he went to another patient.

I left the office with a bandage that covered more than half my left cheek. The waiting room was standing room only by that time. I got in the car and called the hematologist to see if I could get an infusion the next morning.

The hematologist removed the bandage after the infusion so she could see the cut. It was less than a half-inch slit, but three days later she had me come back for another infusion, just to make sure the cut healed fully.

The following week the report came in the mail. Benign.

“It never fails to amaze me how so many doctors – your’s in particular – always know so much better and refuse to listen. I mean, you’ve lived with your body for six decades. Is it a guy thing?” my cousin commented afterwards.

In truth, I think it is just part of the territory for having a rare disease.

Not Today

~ Linda Wright

One of the youngest members of our Fibrinogen Free community recently had a conversation with his cousin that went like this.

Cousin: “Do you have Hemophilia?”

Response: “Umm, not today.”

I think I know how he feels. I began this blog six years ago, and I haven’t posted in almost two years. Each time I think about posting I think to myself, “Umm, not today.” At 66 years of age I actually feel like I don’t have a bleeding disorder except for the few hours I spend each week being infused with Factor I.

The biggest change is that fibrinogen concentrate is now available and cleansed of known viruses. The standard of care is to use this concentrate on a prophylactic basis so that it prevents the deterioration of joint tissue and life threatening bleeding. And, gene therapy is in the works. Then there will be no more concern about what undetectable hazard has entered the blood pool. I believe it will not be long before Factor I deficiency is a thing of the past.

When I began this blog I hoped to find some more people who either had a fibrinogen deficiency or had someone who was a family member with little or no fibrinogen. We are a small group yet we know there are more. We learn from each other, support one another, and advocate together. I am grateful that folks younger than I are using social media to reach out and say, “Hello, you are not alone.”

Scars from bleeds in the past now limit my walking. I don’t climb stairs with ease. Knees, ankles, shoulders, and back aches remind me daily that being born with no fibrinogen has taken a toll. My brain does not function the way it did before a hemorrhagic stroke in 2002.

The more we know about preventing unnecessary bleeding incidents the healthier we will be. Because I am currently the oldest member of our online community I remember the bad old days viscerally. My body and mind signal the reminders of insufficient treatments or delayed medical attention. There are still many people who experience this needlessly. There are still doctors who do not properly diagnose or treat those of us with Factor I deficiency.

We all deserve to have more days when we don’t feel like we have hemophilia.

Pop-Top

~ Linda Wright

“You really need to get a port,” the hematologist said. He kept up this mantra for more than twenty years.

My veins have always been small, the best ones seem to automatically retract or dodge away from an oncoming needle. By the time I was twelve years old I had three scars where a doctor had cut the skin to get an IV line started. Some times it took a few tries but they always succeeded eventually.

By the time I was an adult the IV nurses who knew me best wouldn’t come near me until I had soaked both arms in hot moist towels to plump up the veins. The nurse would choose a child-sized butterfly needle and take a deep breath. I tried all the tricks I knew for my part of this drama. I drank several glasses of water to hydrate. I meditated and relaxed. I wouldn’t let them try to stick me until the blood products were hung beside me, just in case it took several tries. I didn’t want too many pinholes oozing all at once if the first few attempts to start the IV failed.

When I had a hemorrhagic stroke in 2002, I awoke to find that they had inserted a line into my femoral vein. The doctor increased his nagging, “You need a port.”

“Not yet,” I said, “Not yet.”

I couldn’t quite explain why I kept putting it off. I knew there was a risk of infection, ports could get clogged and have to be replaced on a regular basis. I new it was surgery and my automatic response to surgery had always been, no. I knew once I made the decision, there would be no going back. Unlike people who have a port for chemotherapy, I would have one for the rest of my life.

Nurses said, “You’ll love it.”

“Doubtful,” I thought. I guessed that the nurse might love it, but I couldn’t imagine that I would.

“You’ll wonder why you didn’t do it earlier.”

“Probably, I won’t,” I said.

I held them off until I was 64. By that time the only veins that could be felt or seen were on my hands and they were dotted with scars from previous use.

“Ok,” I said after one day there were four failed attempts and the one that succeeded was on the base of my thumb. “Ok, I’ll have a port put in.”

I almost reconsidered when the surgeon explained that he was going to cut my jugular vein and run the plastic tubing to just above my breast where the port would be implanted. Then the line would continue downward just above my lung.

It took a few weeks for the scars to heal, but once it was in they could easily test my clotting levels and give me additional fibrinogen.

Now there is a round bulge about the size of a quarter just under the surface of my skin. I can also feel the plastic tubing that runs from my neck to the port. It is a foreign object and I do not love it. Nor do I wish I had done it earlier. The nurses don’t shudder when they see me walk in the door. But, it only takes one skin stab to start the infusion or draw a blood sample and I have to admit there are some benefits to having a pop-top.

The Devil I Know

~ Linda Wright ~ 2 Comments

Often I have found myself explaining to people who ask, “So, if you have no fibrinogen, do you bruise easier?” What I want to reply is, “No, I have very thick skin.” Of course I would be thinking figuratively, not literally. I’ve answered so many of these questions in my lifetime I have adopted a defense system that shields me from blood phobias.

The questions were scary for me when I was a child and someone would ask, “Can you bleed to death from a cut?” The questions were embarrassing when I was an adolescent, “How do you stop bleeding from your monthly periods?” As a young adult the questions seemed ridiculous, “What happens when you shave your legs?”

Most people fumble about trying to phrase the question they really want to ask, “Will you start to bleed in front of me and collapse in a pool of blood?” Eventually, I grew weary of the questions.

I learned a skill set and developed a level of confidence. More than that, being a “bleeder” became part of my identity. It did not define me, but I knew how to handle it. Then came the game changer in the form of an email message from a friend in California. She had been born with a fibrinogen deficiency and had reached out internationally online to find others who also had low levels or no fibrinogen.

A small group of us had been exchanging information on an email list for several years when she wrote saying that she was now having problems with thrombosis. At first there was disbelief, It gave me the heebie-jeebies when she reported that they were giving her clotting factor and anticoagulant medications at the same time. Then in 1997 a young woman in Switzerland sent a message that she had several embolisms in both of her lungs. When I was hospitalized not long after that for an internal bleed in my lower intestine, my hematologist laughed when I told him that an embolism was my biggest concern. It was no laughing matter to me though. Since then one of the members of our group has died from complications of thrombosis and one has recently reported that she lost the use of her right hand and some vision when clots formed in the arteries of her arm and neck.

Having long passed the fear of bleeding I now found myself afraid of the opposite. The irony is that I am starting all over with the pattern of vulnerability, misunderstanding, and angst. It felt like it was a new devil sticking its tongue out at me. It’s not, though, it’s just that same old devil testing me. It smiles and whispers in my ear. “You think you are in control, but you aren’t.”