Tuesday, February 28, 2017

Hope - Rare Disease Day

This past weekend we went to San Antonio for The Atypical HUS Foundation conference. It's always such a good time. Seeing those that have become family. Seeing those that KNOW what we go through. Knowing that we are not alone.

Since we have been home, I have been thinking about where I was 2 years ago in this journey called Atypical HUS.

I thought I would share my thoughts and feelings for others. What better day to do this than on International Rare Disease Day?!

Two years ago, I was fairly new in my diagnosis of aHUS still. I was diagnosed Sept/Oct of 2014. That first year was HARD. There were a lot of changes. Life saving meds were administered. I was literally fighting for my life.

After learning what I could from the very limited information on this disease, I was overwhelmed and hopeless. I told those closest to me that I would not be alive in 5 years. Whether it be the disease taking me or me taking my own life after tiring from fighting the disease.

At that time, it was the truth in my mind. I had no hope.

In the late fall of 2015, Ben and I attended a luncheon put on by Alexion (the pharma company that makes my life-saving drug, Soliris). We heard a patient story, we learned a ton from the doctor that spoke, we met others that are in the same journey as us. WE FOUND OUR HOPE!

I knew at that time that with the support of those with this disease, the information that we could find by going to these conferences, the people we would meet...that I would survive this. I had found my hope.

Since that first luncheon we attended...I have been to and spoken at so many conferences. I have shared my story in hopes of helping others get through the rough patch and grieving stages of a rare disease diagnosis.

Without these conferences and people to share this journey with, I was honestly lost. I was staring a life threatening disease right in the face. It's a very scary journey to be on with rare disease.
There aren't many people who have the disease or understand what you are going through.

But the people at these conferences, these people get it! They are currently living it as well!

There is hope out there. There are researchers working to find treatments and cures for this awful disease. We are one of the few rare diseases that actually has a treatment. We have each other. I have made some incredible friends that are now family. I have hopefully helped others somehow along the way.

We met several people this past weekend that were very recently diagnosed with aHUS. You can always tell how overwhelmed people are with this disease. It's so much to take in.

To those new patients, and even the experienced patients, please don't give up.

Please reach out to those that you trust. Reach out to those that are experiencing it as well. Hang in there. We are a team, a family. A family...that will fight together and love you through each set back and step forward. We are here!

Thursday, February 2, 2017

spleen infarction

I wanted to post an update here as I have had a lot of people asking why I was having scans done earlier this week and for those in general who want an update on my recent health scare.

Please know that I don't post this type of updates for attention. It is strictly to help others that might have a similar situation, or to update those who want an update.

Also, having an ultra rare disease, it is helpful to see the issues that others have, to know you aren't alone, and to see what type of treatment others have been through.

I really do appreciate all of the thoughts and prayers. They really do mean more to me than I can tell you all.

With that said...my last week has been interesting, to say the least.

At the middle of last week, I started having pains on my left middle back and left side. I just assumed it was my Crohn's, because I have been having some minor issues with it. 

This past Monday night, the pains traveled to the abdomen area as well and became extreme. I was doubled over in pain. So, I called my GI's office first thing Tuesday morning, thinking that I may have a blockage or obstruction. My GI ordered an emergent CT scan for that day. I went, had the scan. Came home. Wednesday morning, I had an appt with my GI. 

The good news is, I didn't have an obstruction. As a matter of fact, my intestines look pretty good. There are some things we are keeping an eye on, but that is typical for Crohn's patients.

The bad news is, I have an infarction on my spleen. This means that a blood clot traveled to my spleen and cut off the blood/oxygen supply to part of my spleen. This portion is about the size of a walnut. Because it cut off the blood/oxygen supply...this part of my spleen is dying. 

This is something that can happen with aHUS (atypical Hemolytic Uremic Syndrome). It's not too common though. I am actually my hematologist's first patient to have this particular issue.

I have learned a lot about the spleen in the last few days. Your spleen filters the red blood cells, platelets, and helps your body fight infection. Because I am immuno-suppressed, I need this help! You can live without a spleen, but for someone with my conditions, it's not ideal.

So, the dying/dead part will remain, and the rest of the spleen will do it's normal job. 

The worse part about this is the pain. My hematologist told me that he cannot believe that I am able to work while having this pain. I just do what I need to do. 

On a normal person, they would recommend aspirin and/or blood thinners to prevent clots. Because of my GI issues, we are choosing not to go this route.

However, my treatment for aHUS should be preventing this. 

At the beginning of January, I was doing really well with my treatment and my blood levels were where they should be. So we moved my infusions from every 10 days to every 14 days. My hematologist believes this is what caused this to happen. My body just isn't able to handle 14 days in between infusions.

So, I am going back to every 10 days on infusions. All we can do at this point is watch closely for symptoms of another clot. And with a spleen infarction, the pain will probably last several weeks. My hematologist is very concerned but said basically the only thing they can do in this situation is remove the spleen if the whole thing dies off. He said with a partial infarction, the pain is the worst part. The pain, it can mimic a heart attack. It hurts to take deep breaths, etc. 

So there it is. Effective, immediately, I am back to every 10 days on infusions. I am to immediately call my doctor and head to the ER if anything changes. I am to take pain pills as needed for the pain. We will do scans again in a few months to monitor the spleen. 

Please, please, please listen to your body. If you have a weird or different type of pain, please have it checked out ASAP.

Thank you all again for the thoughts and prayers!