From There to Here

 

1479020_10201819868972906_734981903_n
Pott. County Democrats Lunch (Photo Credit: Linda Nelson)

Chapter 1

 

A child lays in a hospital bed with tubes running into her allowing medications to flow into her. This child is very sick, and the doctors do not know what she is suffering from or how to treat it. Her family is there waiting, not knowing what to do or how to help their daughter.

After hours of waiting, the many doctor’s visits, and multiple hospital stays they have an answer to what their daughter has and a possible treatment. The doctors informed her family that she has cystinosis, a metabolic disease that causes cells to crystallize causing early cell death. The disease slowly destroys the organs in the body including the kidneys, liver, eyes, muscles and brain. They tell them that their child has an incurable disease that will eventually take her life.

I am that child. I, Mika Jayne Covington, am the daughter that lives with that fatal illness. I am 25 years old and still living thanks to the doctors and researchers at the National Institutes of Health (NIH). I am still alive.

Cystinosis has been a struggle, and it has been a challenge for me including those who care about me. Yes, perhaps I’m a survivor. However, I’m still living with it every day. There is no break, and I do fall apart from time to time because of it.

When I was a kid, I was always seen as different. Every day, I would go to the nurse’s office to take medications. Many days I had bad breath and body odor from my medications, and I felt sick to my stomach, which usually ended in me vomiting.

Elementary and middle school were particularly challenging for me because of this. Not to forget all the doctor appointments and hospitalizations that I had which caused me to miss a lot of school. Growing up with cystinosis is challenging and quite the journey.

Cystinosis is not the only thing that defines me. I am more than my diagnosis. I am a progressive democrat, a feminist, a human rights activist, an organizer, and a student, beyond all of this, I am a person.

However, cystinosis is part of everything. It is with me every day and intersects everything I do. Because I must take medications daily and I must make sure that I have health insurance no matter where I go to school or work. I even cannot leave for a weekend vacation without packing medications, and it is not just a couple, it is thirty different drugs. Because of them, I am reminded of the disease every several hours.

In the second grade, there was a boy in my class; we would go to his house and play games together. We frequently played at school during our recesses. I was drawn to him. He always stood up for me against the big bad bullies. He would hug me and tell me, “Don’t cry, I won’t let’em hurt you.”

At that age, I did not think that I was different or see that there was anything wrong with me. I had no idea what being gay was. I was me, and he was my best friend, that was all I saw back then.

Later, my family moved to Wahoo, Nebraska and I never spoke to him again. I was in the third grade when I first heard the word ‘gay.’ I did not understand what it meant and I was too busy playing and being a kid to worry about it. Until my classmates started calling me gay and a sissy because they saw me as weak and I did not do or like what most of the other boys did. There were also these feelings that I did not quite understand. I thought that they were normal, so I did not pay much attention to them.

I was busy trying to be ‘normal’ while living with cystinosis. Taking medications and dealing with side effects. Making up school work from missed classes because of hospital stays and doctor’s appointments. I just did not have the time or want to deal with another thing that made me seem different from everyone else.

Around the end of third grade, someone called me a ‘faggot.’ I cried that day for hours. I could not understand what it meant but knew that it hurt me that people would call me these names. I began to realize that I was different and I had no idea what they saw in me that was bad or wrong with me.

It was in the fourth grade when I began having sexual feelings and started to think that I might be gay. I tried to push them away and tell myself, “No, it’s wrong to think like that.” I believed that I was sick and depression set in. I had no idea what to do. I wanted to go back to second grade and be with my friend; he would make all the bad things go away. I tried to pretend that I was sick all the time so I would not have to go to school or be able to leave early to get away from the bullying.

In 2010, my senior year in high school, I turned 19 and lost my health insurance. In Nebraska, a person becomes a legal adult at age 19 and per Medicaid rules, one must re-apply for coverage. I did just that and was denied. I was told that I was not eligible for coverage for having a pre-existing condition (cystinosis). I tried applying four times with the same results.

Thus, while attempting to be a regular high school student and completing my senior year, I needed to figure out how in the world I was going to get health insurance. I needed the insurance to cover all my medications that kept me alive and keeps the disease at bay.

At this point, I was working at J.C. Penney, but not nearly making enough money to cover private health insurance. Not to forget, I was beginning my process of coming out as transgender. And finding my political affiliation as a Democratic Socialist.

While many of my friends were talking, and laughing about who they were dating, talking about what college they planned to attend, and what classes they would take, I wasn’t, no not me. They made me feel angry, hurt, and mad at the world and God. Nevertheless, I understood that perhaps I saw the world a bit more clearly than they did. I guess they all may have made me stronger, and that they made me into the person I am today. I was even forced to make sacrifices that I did not want to make, but I knew I needed to so that it would protect me and get things done.

National Institutes of Health (NIH)
National Institutes of Health (NIH)

With no avenue to appeal their decision, I decided to focus on graduating high school and going to college until my scheduled trip to the National Institutes of Health (NIH) that fall. I went most of that year without any medications, which resulted in me going into end stage renal or kidney failure and it probably cut a few years off my life. I went from needing a kidney transplant in 2-4 years to needing one in six months to one year. If I had had health insurance and access to the medications, I might have been able to wait until after college to have a kidney transplant.

After I had graduated from high school, I knew I wanted to contribute to society in any way possible and work to create the change I seek. Unfortunately, having cystinosis and going on dialysis forced me to stop working. To stay busy, I volunteered on issue and political campaigns, such as fighting for full federal LGBTQIA (lesbian, gay, bisexual, transgender, queer, intersex, and asexual) equality, voting rights, and healthcare for all. I am passionate about these because I look forward to a future where everyone has the right to vote, has full and equal human rights, and access to high-quality health care. For me, these causes are important because I know first-hand how not having access to health care can cause a chronic disease to get worse like mine did.

Chapter 2

In 2011, I faced more challenges that made it more difficult for me to attempt to put cystinosis in a box as just one detail of who I am because my kidneys failed. I would need a kidney transplant or start dialysis. Therefore, my doctors put me on hemodialysis and at the time I did not have an organ donor.

hemodialysis1Hemodialysis is a form of kidney dialysis that can be done with a catheter (a plastic tube) placed in the chest into the superior vena cava (a very large vein right about the heart) that is used to cycle blood into a machine that cleans it and returns it. I was on this form of dialysis every other day for four hours in a medical center for about ten months.

I knew that I would need to look for a kidney donor because I could not indefinitely live on dialysis. After a couple of months of searching, I found one, and her name was Erika von Kampen. She was a match, and we had the transplant scheduled at the University of Nebraska Medical Center (Nebraska Medicine). Unfortunately, the operation was unable to happen there, and I had to move on.

PD Bag
PD Bag

Therefore, in February 2012, I switched to peritoneal dialysis. This form of dialysis uses my body, the peritoneal membrane in my abdomen as a filter to clear wastes and extra fluid from my body and returns electrolytes.

I was on peritoneal dialysis for fifteen months. This form can also be continuous, which I did go to when I had a cycler so that it could cycle the fluid at night while I slept. I was on it from 2012 to May 30, 2013, when I received a kidney transplant from the University of Iowa Hospitals and Clinics (UIHC).

PD Cycler
PD Cycler

Sophomore year at Wahoo High School was sort of the best and worst year I had ever had in Wahoo. I decided that I could be ‘normal.’ I worked to open up to people and put myself out there. I just wanted to be like everyone else, to be normal. I decided that to be normal, and I should find a girlfriend. My cousin had a friend who I also knew, and we had several things in common. I asked her and my cousin to go to the homecoming dance. I thought it would be a safe way to attempt to belong.

New Year’s Eve, I was with my older sister and cousin, we were celebrating the promise of the New Year, and I told them that I was indeed bisexual. Both my cousin and sister said, “Yea, we know.” They poked fun for a bit, in a loving way, and we continued chatting as we always had. I finally felt like I could be myself.

The next day was a new year; I felt it was time to live openly, and I experienced firsthand what living honestly meant. Including the pain of being different in a small town high school in the United States, and especially in Nebraska where it was legal to bully and harass a fellow student based on their sexual orientation. To this day, there are no state or federal laws banning bullying or harassment based on sexual orientation or gender identity.

As an openly ‘gay’ student, I experienced bullying and harassment in a new way, but I decided I would have to live with what was thrown at me. Soon the bullying escalated to the point where I needed to leave Wahoo. I became very depressed and angry. I didn’t completely understand why I felt that way. Especially since I just came out and from my research, I should be feeling better. Nevertheless, I didn’t feel any better. I still felt wrong in my body.

One Summer day, after watching my older sister have her nails done, something happened. It was like a light went on. I started to wonder what it would be like to have my nails painted. Thus, when I went back to school a couple of my friends, and I painted my nails, and I liked it.

Soon, I felt more comfortable and started looking at expanding my gender expression. I looked at wearing tighter jeans from Hot Topic. I knew that guys were not ‘supposed’ to paint their nails or wear so-called ‘girly’ clothes. However, I wanted to wear them, and they made me feel more safe and comfortable. They also scared me to death. Then, I became active in other things to express myself such as choir, drama, and activism.

 

As I’ve said, cystinosis is only a part of my life, and peritoneal dialysis changed my life to make it a smaller part of it. It was like I had some of my freedom again. I was able to follow my passion and fight for what I believed in again. I was able to become active in politics, and I met many wonderful people including Amber Bordolo a Field Organizer with Organizing for America (OFA).

While I went to college at Iowa Western Community College (IWCC), working on my Associate’s degree in psychology, Amber invited me to a couple of her events. I finally attended one of them, became friends with her, and she recommended that I apply to become a Summer Fellow (intern) at OFA. Little did I know how big of an impact that was going to have on my life.

398777_10150940759848430_1366498105_nIn the beginning, I worked to organize house parties and other events to get supporters together, fired up and ready to go to work to get the President re-elected. Soon, I moved into a different stage of the campaign where I made many calls to convince voters to support him. Additionally, on the campaign, I had the chance to go to Des Moines, Iowa to see the President speak to a crowd of supporters at the Iowa State Fairgrounds where I was able to stand on stage behind President Barack Obama.

While I worked with OFA, I considered going to UIHC to get on their transplant list and perhaps have a living donor transplant.

University of Iowa Medical Center
University of Iowa Medical Center

In September of 2012, I went to Iowa City, Iowa for medical tests and evaluations at UIHC Transplant Center to see about being placed on their list. I passed and was placed. Additionally, I went out to find a living donor. This included me sharing my story and health condition with people. Then I sent out packets of information and forms to eight individuals who were interested. However, only three were returned to the transplant center, and one was a match, but had complications and could not donate. I had to make a choice, spend more time sending out packets or see if Erika was still willing to donate her kidney.

I was lucky because Erika was still interested in donating. Once she filled out the forms, and they were returned to the transplant center, they immediately scheduled her for an appointment to be evaluated since we already knew she was an excellent match. The operation was scheduled for May 30, 2013, which I dubbed adoption of Serenity because I named the new kidney Serenity.

Chapter 3

IV meds post-op transplant.
IV meds post-op transplant.

I wake up feeling pain and choking on the dryness in my throat. The nurses around me are talking and doing things with my iv lines. I couldn’t understand exactly what they were doing.

One of them asks me, “Mika, what is your pain level?” I don’t respond, and I just groan because I still am fighting the hoarseness and dryness in my throat. I try to swallow the saliva that isn’t there and fall back asleep.

I wake again hearing someone ask me, “Mika, can you tell me what your pain level is?”

I clear my throat and say, “I feel pain. A lot of pain.”

The person replies, “Yes, what is the pain level? One to ten.”

I reply, “It is an 11.” I then feel someone touching my arm, and I fall asleep once again.

Jon and me after our surgeries!
Erika and me after our surgeries!

The next thing I remember is people talking and being in a different room but I was not wearing an oxygen mask. Someone in the room was speaking but not towards me. I ask, but to no one in particular, “Water, I need water.”

At one moment of my consciousness, before I was fully awake, I turned to my grandmother and weakly said, “No more dialysis.”

I am a bit more conscious and aware, and I see that I am in a private patient room with family around. A nurse is trying to talk to me about the surgery, the morphine pump, only being allowed ice, and needing to get up to walk by 10:30 pm.

I am living with full kidney function thanks to Erika and the staff at UIHC. The transplant gave me my life back literally and figuratively. I started the journey to kidney transplant on November 22, 2010, to May 30, 2013. Those were three very long years of two types of dialysis, doctors’ visits, and hospital stays. All I must deal with now is immune – suppressants, cystinosis medications, blood thinners, transgender hormones, and fighting to continue to have health insurance.

But, it is still hard. I don’t know what it is like to just live. To live without there being a struggle or some challenge to overcome. Ever since I graduated high school, I have been living from one struggle or challenge to overcome to the next. No time to breath, no time to relax or get comfortable. Or at least the moment that I start to relax something new happens, another challenge confronts me. I know how to survive but do not know how to live.

Nevertheless, cystinosis and most of the challenges that I have faced have not halted my endeavors. I wanted to do more for society, and I have done that. Therefore I volunteered with the Iowa Pride Network and volunteered to be on their College Leadership Team.

On their team, I organized and ran the IPN Southwest Regional Gay-Straight Alliance Coalition. This Coalition was a great way for LGBTQIA youth and students to come together and have a safe space. Additionally, the coalition consisted of one meeting a month, and the meetings were educationally based on filling the education gap on LGBTQIA studies and issues.

While I was a member of the College Leadership Team, I was a college student at Iowa Western Community College, and in the summer of 2012, I had my first taste of political campaigning.

As I spoke about before, I became a Summer Organizer for Organizing for America (OFA) or the Obama campaign. In that position, I had many responsibilities including working closely with volunteers and talking with Iowa voters. I recruited, managed, and trained volunteers to organize their communities and neighborhoods into teams that registered, persuaded, and motivated voters. I organized my turf, which included most of Iowa House District 16. I planned and successfully organized one of the largest Equality Nights in Iowa.

During that Equality Night, I lead a discussion on the accomplishments of President Barack Obama and the Democrats for the LGBTQIA community. Additionally, I worked to grow neighborhood teams on a grassroots level and managed team leaders.

For me, this was an excellent experience to develop as an adult, learn how to handle several tasks at once and focus on total goals. It was also fun. I loved talking to voters and making connections with them about what is important in their lives and how Democrats can work with them to better their lives.

In 2007, I left Wahoo, Nebraska with my family. Most of that summer we lived in a subdivision of Omaha, Nebraska. When school started, I moved in with my cousin and her family, that way I was able to attend school with her at Millard South High School. There I met many new people. People were finally accepting of my sexual orientation and gender expression. I even met other openly LGBT students. Millard South was a safer environment for me. I was able to open up and become the person who I truly was all along.

Millard South even offered many additional academic opportunities than the small-town school. I was able to take part in the German program at Millard South. I was able to thrive there, and I gained some confidence.

Students and teachers at Millard South thought something of me. They believed that I could accomplish some awesome things in my future. I had a couple of teachers who believed in me especially when I did not believe in myself. They kept giving me encouragement. They told me to keep fighting for what is right. They believed in me when I needed someone, even when my family was not there for me. I will never forget all that they have done for me, and I hope I can repay them.

My new pride and confidence did not help me at home. As I become more open about myself, my mother and her boyfriend became negative towards me. My mother not only did not understand who I was, but she also seemed not interested in trying to learn. Both thought that I needed mental help for who I was.

On that particular issue, perhaps they were right. I needed help dealing with the damage that they were causing. I also needed someone who could help me understand myself. I’d known for years that I was different and my time at Millard South allowed me to develop and embrace parts of my identity. But, I felt that there was something more. I could not piece together why I felt that I was incomplete.

The therapist that I did see understood that the problems at home were multifaceted and did have a lot to do with my mother and her boyfriend. She understood that some of the challenges I faced were related to being queer and dealing with bullying and harassment in my life. These sessions helped me begin to accept a fact about myself that I had previously been unable to come to terms with. I could identify the roots of some of the problems and discovered more about my identity. I realized that the only way to overcome the feelings of injustice I had was to continue to fight for my rights.

At one of the sessions where my mother and I were at, I remember yelling, “Well, I am sick of listening to you cry about your problems! We are here about me! I am transgender!”

That day forward, I began coming out to people. On October 11, 2009, I told the store manager at J.C.Penny that I was transgender. I told her that from that moment on I would be identifying as female, using female pronouns, and prefer being addressed as Mika. This is the time I began the process of coming out at school and requesting my teachers to address me the same.

Chapter 4

Coming out as transgender was one of the hardest things I’ve ever done. I was terrified. I knew my family didn’t like me being queer. I was concerned about how my friends at school and work would treat me. I even expected to lose my job.

It has been a long journey. I wonder why I did not notice or understand sooner. Coming out opened my mind to new possibilities. It helped me accept myself, and I could improve my academics and work beyond what I thought I could.

After nearly six years of living openly as myself, I have started the medical transition to assist me in confirming my gender. I recently started transgender hormone therapy. The hormone therapy helps in establishing my secondary gender characteristics.

During the hormone therapy, I take two medications spironolactone (Aldactone) and estradiol (Climara). The spironolactone is used to suppress the male sex hormone (testosterone), and the estradiol is used to add the female sex hormone (estrogen).

Furthermore, I have hypogonadism, which causes my body’s sex organs not to produce healthy levels of testosterone. Because of the hypogonadism, my body was never exposed to large amounts of testosterone. Therefore, I could transition into my real gender much easier socially.

Coming out as transgender, has abled me to become a full person. I can be who I was all along. Yes, there were many challenges that everyone including myself faced. For example, many of us cannot access high-quality health care, find health insurance, change of identity documents, fair housing at colleges and universities.

In my case, I had numerous experiences of health care providers refusing to recognize my gender identity. This led to many occasions of embarrassing moments where a nurse would call my name, and I would walk over. The nurse would then ask me who I was, and I would tell them. They would say, “You aren’t a male, I am looking for a male.” Yes, that kind of occasion. There were also those events where the doctor comes in and asks me, “Where is Mika at?” I would tell them, that I am the patient. Then, I would get, “Oh! Well, I guess I have the wrong room.” Yeah, it was wonderful!

In regards to gaining health insurance, transgender individuals can get health insurances regardless of their gender identity. Despite that, most insurance policies do not cover any gender confirming treatments, and they are unlikely to include gender-confirming surgeries. Consequently, transgender individuals do not get the health care they need and in many cases or must pay out of pocket.

Thus, many transgender people are not getting the health care they require. Hence, why I waited six years to start any form of gender confirming treatments. Only recently did government policies change to allow Medicare and Medicaid to pay for some gender confirming procedures. At the moment, all of my gender confirming procedures are covered under Medicare part D. I am lucky because many transgender individuals do not have cystinosis that qualifies them for Medicare and Medicaid.

Furthermore, changing your identity documents is not a walk in the park either. It costs money and if you do not have that money you just cannot change them. As a transgender person, it destroys you every time you must show that card to someone, and it doesn’t represent who you are.

Imagine, how would you think you feel if you go to an airport to go on a vacation and going through security you must show a card that says you are male. But you apparently look like a female, and on top of that, you must use a name that isn’t you. This is the situation for many transgender individuals.

Additionally, transgender individuals face many difficulties when applying for on-campus housing at colleges and universities. Many college and university housing policies say that they make placement decisions based on the legal or biological sex of a student. This is unfair and a problem.

In my case, I first applied to live on campus at Iowa Western Community College, and the campus housing administration had to have a discussion with me on my “trans issue.” They told me during that conversation that I could live on campus if I lived in a male dorm, and they said I would probably need to dress as a male.

Ever since getting involved in politics in 2012, I stayed involved because I felt that it was an important endeavor. Therefore, I became more active within the local party and learned more about Iowa politics, my new home. I stayed involved in Nebraska as well, where several Nebraska citizens were interested in fighting for second parent adoption.

It was in the winter of 2012, and I worked with these residents who did not have the ability to secure their families through second parent adoption or join adoption in their state. After we had done extensive research, we began working with Nebraska State Senator Sara Howard (District 9) to introduce a bill in the state legislature.

photo (1)Senator Howard introduced LB380 in February 2013 to correct Nebraska law. I then ran a grassroots campaign for the bill that consisted of an online petition, emails, and calls to State Senators. And I organized postcard making parties in several cities throughout eastern Nebraska, which was sponsored by MoveOn.org. Unfortunately, the bill did die that session, but Sen. Howard continues to fight for families in the state legislature.

While, I was working on the second-parent adoption bill, I was the field director of Forward Equality. The organization was formed by myself, several friends of mine including a former professor of mine. Forward Equality worked on progressive issues ranging from workers’ rights to civil rights. I worked (non-paid) at Forward Equality from April 2010 to April 2014 when it dissolved.

Chapter 5

As I said, I continued to be involved in the Iowa Democratic Party. I was elected to serve as the Pottawattamie County Affirmative Action Chair, and I served from February 2014 to October 2015.

11391246_981335545240787_107062442060574773_nAs the Affirmative Action chair, I organized an Affirmative Action Committee in Pottawattamie County for the County Democrats. We worked to ensure that our party was following the Democratic Party’s rules and regulations on the Americans with Disabilities Act of 1990 and their diversity requirements. This included organizing our involvement in several Pottawattamie County events such as Celebrate Council Bluffs and Heartland Pride LGBTQIA Parade and Festival. We also assisted in ensuring that the Pottawattamie County 2016 Caucus locations were ADA accessible to the best of our abilities, and we worked to bring those who historically felt unwelcome back into the party.

Moving forward, I have been involved in the 2016 Democratic presidential primary. I supported Senator Bernie Sanders for President, and I first met him back in September 2014 at an event he was speaking at in Des Moines, Iowa.

From that moment on I knew that he was a person that I was willing to fight for and put in long hours campaigning for. Sen. Sanders wasn’t running for president at that moment, though. He was only considering it. I decided that I had to work to get him to run. Thus, I volunteered with the Run Bernie Run campaign to convince the Senator to run for President as a Democrat.

On May 26, 2015, Sen. Bernie Sanders surprised many people including myself by announcing that he was indeed running for president and that he was going to run as a Democrat. I knew at that moment that I had to learn how I could help him while living in Council Bluffs, Iowa. However, I was still the Affirmative Action Chair, and I was forced to make a choice. I had to decide if I wanted to throw my full public support behind him.

The decision was thrust upon me the last week of June when I received a call by the Sanders campaign asking me if I would be interested in introducing the Senator at his town hall in Council Bluffs on July 3rd. I felt overwhelmed with excitement and terror. I have never spoken publicly to over 2,000 people. Thus, my first response to the staffer who called me was to give me some time to think about it. I said this even though in my heart I was screaming to go and do this.

On July 3, 2015, I gave one of the first speeches kicking off the Bernie Sanders campaign in Iowa. I was nervous, but I did my job and did it well for Bernie. That day forward, I began volunteering for the Senator. I did this for his campaign for months until December when I was hired on to the campaign as an Organizing Fellow. I was officially on the Bernie Sanders campaign payroll.

Introducing Sen. Sanders
Introducing Sen. Sanders

I was an OF for two months before I was promoted Field Organizer. I that position, I recruited managed and trained volunteer. I built and led several volunteer canvass and phone banks.

14102677_10153641460691923_3415136486597262791_nOn the campaign, I worked in Iowa and Nebraska on their caucuses. While in Nebraska, my turf included Lincoln but I also knew people in three other counties. Thus, I helped to organize them as well. Two of the three counties went for Bernie.

After the Nebraska caucuses, I was sent back to Iowa to work on our county conventions making sure that Sen. Bernie Sanders got a fair representation by having all his delegates show up.

Between working on the Iowa conventions, I was sent to Kansas to help organize the campaign’s participation at their state district conventions. Afterward, I went back to Iowa to work on the district conventions. Nonetheless, I was sent to Colorado to assist with organizing for their State Convention. In Colorado, I helped in several ways including monitoring the official ballots and observing the counting of those ballots. I also signed off on the initial hand count of the official ballots as the Sanders, campaign staffer.

I am proud to have worked for Senator Bernie Sanders and voted for him in the Iowa caucuses. I worked for him as an FO from February 2016 to May 3rd, 2016. Nonetheless, I was not finished working for and representing the movement for a progressive future and the political revolution started by the Senator. At the 3rd Congressional District convention, I ran for national delegate to represent Iowa at the Democratic National Convention. I did not win enough votes to go.

Later at the State Convention, I ran for Democratic National Committee member to represent the movement of progressive voices and the youth voice of Iowa. I won a significant number of votes to make me a strong challenger against the party establishment. Unfortunately, I did fail to gain a majority of the votes. Moreover, we did show the establishment that their politics are no longer what many in the party want and they should begin to listen to us otherwise, they may lose their power.

Today, I am living my life. I am out and open about who I am. This is who I am, a human being who has a tremendous passion for making this world a better place for all. I am a person who lives with cystinosis and who is a transgender woman. I am a sexual assault survivor, a human rights activist, a feminist, a student, and a fighter.

This is my story, and it continues.

Advertisements

How Do You Live With An “Orphan” Disease?

Have you ever wondered what it is like to live with an “orphan” disease like cystinosis?

rare-disease-infographic

It is difficult but it is an amazing journey. The following information will help you understand what a person living with cystinosis must go through while growing up.

Cystinosis is a rare disease that primarily affects children. It is a rare genetic disorder in which continuous cellular accumulation of the amino acid cystine rises to toxic levels, resulting in irreversible tissue and organ damage if left untreated even for a short time. Cystine crystals accumulate in the kidneys, eyes, liver, muscles, pancreas, thyroid, brain, and white blood cells. Without treatments, children with the disorder develop end stage kidney failure at approximately age nine.

Cystinosis is treated symptomatically and ongoing, which makes it possible for patients (us) to live longer lives. Consistent care is essentialeven when the patient (we) look or feel fine. The disease does not rest. If a patient delay, miss, or stop taking medicine, cystine levels can rise very quickly. The damage caused by toxic levels of cystine cannot be reversed. *A kidney transplant is not a cure for cystinosis.*

MYN90450The following are ways cystinosis is treated:

  • High intake of fluids and electrolytes to prevent excessive loss of water from the body.
  • Sodium bicarbonate, sodium citrate, and potassium citrate may be administered to maintain the normal electrolyte balance.
  • Phosphates and vitamin D are required to correct the impaired uptake of phosphate into the kidneys and to prevent rickets.
  • Cystagon or Procysbi (cysteamine bitartrate)cystaran_374803
  • Cystaran (cysteamine eye drops)
  • Kidney transplantation

The disease first affects the kidneys and it is a primary concern to ensure they last as long as possible.

kidney-transplantation

 

The kidneys are fist-sized organs, shaped like beans. They act as filters to clean waste (what’s left over from breaking down from food and your body’s other activities) from the blood. In addition to removing wastes from the bloodstream, the kidneys also make and regulate hormones and chemicals in the body. When the kidneys are not working correctly, the body can develop several problems, including: fatigue, bone problems, sleep problems, and anemia.

Dialysis may be needed if a donor kidney is not available, or if a kidney transplant fails. Dialysis serves as an artificial filtering system that mimics a person’s kidneys by removing waste from the blood.

microbeadsCystinosis patients (we) are now living longer lives because of the new treatments and advancements in medicine. Living longer means that cystinosis patients must transition from pediatric care to adult care. As a patient myself this is excellent news because we had have only a 20 year life span, now we have a 50 year life span. Once they become adolescents they must learn about treatment options, know their medications and side effects from those medications.

C._Everett_Koop,_1980sThis period is known as “transitioning,” which was inspired by Surgeon General C. Everett Koop. He described it as the “one major issue” for chronically ill adolescents that had not been adequately addressed by the healthcare system.

A consensus statement from pediatric and adolescent health professionals in 2002, in the journal Pediatrics defined the goal of transition:

“To maximize lifelong functioning and potential through the provision of high-quality, developmentally appropriate healthcare services that continue uninterrupted as the individual moves from adolescence to adulthood.”

There are many factors that affect the overall well-being of adolescents with cystinosis:

  • Medical/physical concerns
  • Psychosocial functioning
  • Long-term disabilities
  • Multiple hospitalizations
  • Treatment compliance
  • Comorbidities such as gastrointestinal complications with possible swallowing problems.
  • Hypertension (elevated blood pressure)
  • Photophobia (lights hurting the eyes) due to corneal crystalscystinosis
  • Visual impairment due to retinopathy (involvements of the back of the eyes)
  • Metabolic bone disease with skeletal deformities as consequences of rickets
  • Persistent polyuria (large urine output) in pre- and post- transplant patients
  • Endocrine involvement including diabetes and hypothyroidism (low thyroid function)
  • Various degrees of myopathy (muscle involvement) affecting strength
  • Delayed puberty (sexual maturation)
  • Male hypogonadism (low male hormone levels) and infertility (inability to conceive)

Disease_chart_big1

The transition process that Koop spoke about is broken down into three stages by their age. Early adolescence (12-15), late adolescence (15-18), and young and “emerging” adults (19-25).

cysteamine-bitartrate-figure-3In early adolescence, cystinosis patients (we) begin understanding their medications and taking responsibility. They should recognize what their medications look like and should be able to tell their parents and healthcare team the following:

  • Name of each medication
  • Dosage and schedule for taking each medication3aab3b39-52b6-4a79-9a5c-7baa7574f24f-03
  • Purpose of each medication
  • Side effects of each medication

Cystinosis patients (we) have the unique role of being their own advocates, and at this age they should learn how to self-advocate. Advocating for themselves will be crucial as they face many circumstances where they need to stand up for themselves in the healthcare field and in general. Parents should encourage children to practice self-advocacy while still supporting them in their decisions.

Often children and young adults with chronic illnesses may be hidden victims of bullying.

These are some potential bullying warning signs that we all should look for:

  • Loss of interest in school and extracurricular activities
  • Frequent complaints of illness to avoid attending school
  • Sudden decrease in academic performance
  • Few or no friends with whom he/she spends time with
  • Unexplained bruises, scratches, and cuts
  • Fear of going to school, riding the bus, walking to school, or taking part in organized activities with peers
  • A preference for a long or illogical route to school
  • Increased moodiness, sadness, or depression
  • Loss of appetite
  • Trouble sleeping
  • Anxiety or low self-esteem

Going to school with any illness can be daunting, especially with a serious “orphan” disorder. Cystinosis patients and their parents should not be intimidated by the “educational professional,” and the school administrators should NOT be intimidated by the parent! Patients and parents should NOT let teachers, support staff, or administrators tell them that a request is “impossible.”

Navigating the school system can be difficult living with cystinosis, and communication is key for parents and patients. Parents should get to know key individuals – including a child’s principal, guidance counselor, nurse, psychologist, social worker, AND ALL teachers (including those who teach special classes like art and physical education) as the school year begins. Parents should provide the school with packet of information about cystinosis.

Honesty is the best policy. Not sharing information does not allow understanding, and can create problems. Encourage school staff to ask questions. Urge them to ask questions, both academic and medical.

Cystinosis patients (we) are constantly faced with difficult situations, therefore young patients need to build a self-confidence. Building self-confidence is imperative for them. Having a self-confidence can help patients think positively and deal better with the daily stresses of having cystinosis.

Here are some ways that can be used to help build self-confidence:

  • Set standards for independence
  • Focus on building confidence, resilience, and strong communication skills
  • Recognize their child for doing a good deed, doing well at school, or completing a goal.
  • Point out their child’s strengths
  • Applaud their child’s efforts to improve on an activity he or she enjoys (even if it’s not the parent’s favorite!)
  • Listen to their teen when they speak and use open-ended questions rather than ones that require only a yes/no response

During transition, these young patients should spend a few minutes talking privately with their healthcare team at the beginning or end of each visit. This can help them feel more in control of their life and the cystinosis. They should start siting in (or picking up the phone) when their parents are making medical appointments or calling for prescription refills.

Sample Questions to Ask Your Medical Team

  • At what age does transition happen at this hospital?
  • Are there teen- or young adult- specific clinics?
  • Who will help coordinate the transition?
  • Do you have adult specialists that you recommend?
  • Why do you recommend them over others?
  • where are specialists located?
  • How will we meet them?
  • Do we need referrals?
  • Are there adult physicians/specialists who are knowledgeable about cystinosis?

Cystinosis patients face a unique set of stressors.

The disease can interfere with the patient’s comfort in moving toward independence and their parents may be resistant to a patient’s efforts to become independent.

  • In these situations the parent’s job is to provide information and resources to help adolescents think and live as independent adults.
  • One difficult, however necessary job of the parent is to seek a balance between “normal” adolescence and the unique healthcare needs that cystinosis patients (we) face.
  • Parents should start involving their teens in all health-related discussions (treatment choices and current concerns about their illness).
  • Work together, the patient and parent to teach self-care skills related to the patient’s illness (from medications to calling the pharmacy or scheduling doctor’s appointments).
  • Work on the development of coping skills to address problems or concerns that may arise related cystinosis.
    • Talking to friends about cystinosis
    • Participating in support groups
    • Expressing frustration or anger when needed
    • Using humor to defuse frustration or anger
    • Researching a problem
    • Participating in social support or religious activities

Medications are vital to cystinosis patients (our) survival. If they miss a dose of cysteamine or anti-rejection drugs, it could damage their health. Over time this can lead to the progression of the disease and rejection of the transplanted kidney.

  • Think about how much privacy is important to them when taking their medications
  • The patients need to speak honestly with their parents or their healthcare team if there is a problem with taking medications – whether because of side effects, a busy schedule, or sheer frustration. There may be options that could make things easier.
  • Parents need to understand that their teen will be taking many medications, to control cystinosis and to protect a kidney transplant.
  • If a cystinosis patient illness reaches an unstable state due to “nonadherence with treatment recommendations,” parents and healthcare teams should aim for discussion of what’s happening rather than arguing or punishment. The parent and healthcare team should decrease their frustration and replace it with support. 
  • Both the parent and the healthcare team should be honest about the results of not adhering to medication schedules, but avoid scare tactics. 
  • Communication is vital between the parent and patient. Talk about what the nonadherence is really about – taking medications can be a burden, it may interfere with social activities, the side effects may be too troublesome, etc.
  • Parents should know that not taking medications can also be a way of “acting out,” which other teens might express with skipping school, using drugs or alcohol. This can be a way of expressing rebellion or a way of trying to feel in control. The parent and patient must work together as a family and with the healthcare professionals to find a treatment plan that works for the young patient.

Gears_big1

Cystinosis patients (we) must learn coping skills to deal with the side effects of Cystagon or Procysbi. These patients (us) cannot stop taking these medications. Cysteamine bitartrate is the lifeline for individuals with cystinosis. Cystinosis patients may often feel many side effects from their medications, especially the cysteamine bitartrate. As these patients reach adolescence and young adulthood, it becomes frequently tempting for them (myself included) to skip taking the medication due to the side effects, social concerns, and self-conscious feelings from the drug’s odor, and complications such as gastrointestinal distress.

CELLS_b-02-big1

Cysteamine bitartarte is vital, without it , the cystinosis will take its natural course, and more complications of the disease can happen at an accelerated rate. Patients may not be immediately aware of the damage to their health.

Living with any illness is difficult, being a teenager as well, makes life even more so. If a teen or young adult has five or more of these experiences, he or she may need more intense and immediate support than just talking with a friend or family member:

  • Feeling sad all or most of the time
  • Fast to get angry
  • Lose of interest in activities that were formerly enjoyable
  • Sleeping too much or too little
  • Missing school frequently or drop in grades
  • Worrying about being rejected or not doing something well
  • Feeling anxious and shaky
  • Feeling worthless or guilty
  • Feeling isolated; avoiding or not having friends
  • Having problems concentrating
  • Thinking about running away from home
  • Deliberately skipping medications

Parents and or patients should talk to your healthcare team and seek help if:

  • The patient seems overwhelmed with emotional issues related to living with a chronic illness. This could take the form of extreme sadness/depression, anger, or isolation/withdrawal
  • A pattern of “nonadherence,” or risky health consequences occur. Not taking Cystagon or Procysbi can result in the development of cystine buildup in all organs over time. Not taking immunosuppressants can result in acute rejection of a kidney very quickly, or cause a slow decline in kidney function due to chronic rejection. Sabotaging their health can become a powerful way to get attention or act out.

Young cystinosis patients must grow up learning all of the experiences of being a teen while they must learn how to take care of their health. 

  • These patients should spend a day or weekend independently preparing and taking medications and eye drops – without parents reminding them. Set up their own medication box for the week.
  • Parents should work with patients to start exploring academic and work interests that they want to pursue after high school.
  • Patients should keep a journal or write when they feel strongly about something. Write an essay about the best and most challenging things they have experienced (which might have nothing to do with cystinosis).
  • Patients should practice and try out different ways to tell “their” story may be useful for college and scholarship applications, or enable them to be an advocate for a cause they care about.

As cystinosis patients (we) become adults, they face many new challenges such as: furthering their education, exploring career paths, forming relations, experiencing different living situations, and dealing with car, home, and the big one health insurance.

Some call this age group young or “emerging” adults (18-25).

Jeffrey-Arnett-150“Emerging” adulthood – coined by researcher Jeffrey Arnett, Professor in the Department of Psychology at Clark University in Massachusetts, which used the term to describe the experience of 18-30 year olds. They are sometimes called millennials. They are unique, because in the United States and Europe, the years were once  thought of as a time of settling down, starting jobs, and building families; now it is a time of extended exploration, trying out educational and career paths, traveling, forming relationships, and experiencing individual living situations.

Consequently, growing up with cystinosis, young adults usually are eager to become independent and might be anxious about what lies ahead for them. They may also be facing challenges that their friends or healthy family members are not experiencing.

These young patients:

  • Negotiating what it means to them to be independent
  • Allowing friends and family to provide help to support when needed
  • Traveling, studying, moving away from home
  • Making educational and work choices
  • Taking complete (or more complete) responsibility for their health and health care

The following is a couple quotes by some cystinosis patients.

“Generally I’m pretty happy with my life . . . but it’s so much harder than anyone realizes.”

“It [cystinosis] totally affects how I feel about myself – for the best. I am proud of all that I do in spite of cystinosis. It gives me a story of triumph and hopes to share with others.”

Relationships and dating are even  bit more unique for individuals with cystinosis.

Just listen to these patients.

“An important issue is the smell of Cystagon. As I’ve talked with some of the young adults in our community. I’ve found more than one person who has stopped taking Cystagon when embarking on a serious relationship. You cannot talk about dating without approaching the subject of close contact, kissing, etc. and Cystagon. Quite frankly, if you’re with someone who is going to make a stink (no pun intended!) about your lifeline, he or she isn’t worth it.”

“I usually shared on the second or third date. My feelings is that if he doesn’t stick around, then he is not the kind of person I would want to be with anyway. One time, I waited too long, and they guy bolted shortly after I told him. Although he didn’t admit it, I felt that was why. By then, I was more attached, so naturally it hurt more.”

The Future


Sexual Reproductive Health and Cystinosis

by Dr. William A. Gahl and Dr. Galina Nesterova, National Institutes of Health (NIH)

Adolescents and young adults with cystinosis are faced with a challenge of sexual maturity and reproduction. If cystinosis is untreated people nearly always exhibit late sexual maturation (puberty).

Cystinotic males reach puberty at about 16-17 years old, and may demonstrate a primary decrease in testosterone (the hormones produced by the testes) due to cystine accumulation in the testes. Poorly treated males exhibit high levels of luteinizing hormone and follicle stimulating hormone (other hormones needed for sexual maturation), as compensation for the low production of testosterone by the testes. Male patients benefits from testosterone supplements to restore secondary sexual characteristics such as facial hair growth, muscle mas, and increase in testicular (testes) size.

The ability to have an erection (ability to have sexual life) apparently remains intact, no cystinosis patient is known to have fathered a child.

Cystinotic females poorly treated reach puberty at about 14-15 years old. Ovulatory cycles and gonadal endocrine parameters are normal in females, and several successful pregnancies and deliveries have occurred in women with cystinosis; the children have all been completely normal.

It is recommended that women with cystinosis withhold cysteamine therapy when trying to conceive or during pregnancy. Currently, there is no data o the teratogenic effects (risk of birth defects) of cysteamine in humans.

*Adapted by Mika J. Covington to fit word limit.


Cystinosis patients face challenges when it comes to education.

They have many doctor’s appointments, lab visits, and sometimes hospitalizations all forcing them to miss school.

The Rehabilitation Act of 1973 (Section 504) and the Americans with Disabilities Act of 1990 (Title II) prohibits discrimination based on disability. “Disability” can include “invisible” conditions like cystinosis, and includes more “obvious” disabilities such as vision problems, learning disabilities, difficulty walking, etc.

Universities and colleges are required to have an Office for Students with Disabilities (actual names will vary). Cystinosis patients should consider documenting their health condition with their educational institution. However, if the patient doesn’t document their healthy condition ahead of time, their professors and administration will not be required by law to make special exceptions.

Additionally, new federal regulations, require insurance offered by universities and colleges to offer the same coverage as commercial plans – such as not imposing limits on lifetime costs or pre-existing conditions.

Go here for more information: http://www.ed.gov/ocr/transition.html

Employment and Insurance with Cystinosis

Cystinotic individuals entering and who are in the workforce may experience a variety of issues and concerns. Career choices for young adults with cystinosis should be based on their intellect, ability, interests, and life goals. These individuals (us) are not limited in choice of employment and work in a variety of professional and technical jobs.

How should cystinotic individuals handle interviews? First, they should understand all the state and federal laws related to questions that the employer can legally ask (www.ada.gov or www.wwoc.gov).

Should an applicant disclose information regarding their cystinosis at the interview? Usually, no. Sharing this information about their cystinosis can put both the interviewee and the employer in a difficult position. The decision to hire should be based primarily on the individual’s ability to perform the tasks of the job.

Health Insurance

Health insurance is confusing for most people, especially for people dealing with a serious illness. Cystinotic individuals will need to have a vast knowledge about health insurance. They should have the answers to these questions:

  • Does the plan cover your prescriptions (anti-rejection, Cystagon, Procysbi)?
  • Are your medications (anti-rejection, Cystagon, Procysbi) on the “formulary” or must they be ordered through as specialty pharmacy? Will you have to pay out-of-pocket for specialty pharmacy orders?
  • Is there a mail-order option? Cystagon, Procysbi, and Cystaran will likely require extra steps.
  • Does the plan permit you to see the providers you already have established relationships with and allow you to be hospitalized at the medical center of your choice ?
  • What does it cost to go “out-of-network”?
  • For medications that may not be FDA approved, what will be the cost to you?

Legal 

Cystinosis patients should understand the basics of the many laws that assist them in the workplace and with their health insurance. Go here for additional information: www.younginvincible.org

The following are brief descriptions of some of those laws:

  • Section 2714 of the Affordable Care Act of 2010 (ACA aka Obamacare), requires all plans offering dependent coverage to allow individuals up to age 26 to remain on their parent’s health insurance.
  • Section 2301 of the Reconciliation Act of 2010, includes a requirement for existing health insurance plans to provide coverage for adult children up to age 26 and eliminates the requirement that adult children be unmarried. The extension also means that adult children do not have to be in college full-time to maintain under their parent’s coverage. The following is a resource for information is the Commonwealth Fund (www.commonwealth.org).
  • Consolidated Omnibus Budget Reconcilation Act (COBRA) of 1986, allows you to continue your insurance coverage for up to 18 months through your employer if your job is terminated or your hours are reduced (www.cobrainsurance.com).
  • Americans with Disabilities Act (ADA) of 1990, requires that employers to provide reasonable accommodations for individuals with disabilities who can perform the essential functions of the job (www.ada.gov).
  • Ticket to Work and Work Incentives Improvement Act. If you receive Social Security Income or Social Security Disability Benefits, This law may allow them to work and continue some of their insurance benefits. It also has a voucher system, where they can gain rehabilitation and other services to assist them in getting a job (www.ssa.gov).

 

*This blog post is dedicated to all those fighting “orphan” diseases including my sister Mary Covington, who like myself lives with cystinosis.

 


This blog post used information from an amazing resource, “Bridge to the Future,” a transition guide for teens and young adults with cystinosis and their families, created by the Cystinosis Research Network. Additional information used within this blog post came from the website http://www.knowcystinosis.com. I, Mika J. Covington, take no credit for collecting and putting together any of the information herein. However, I, Mika J. Covington, do take credit for the addition of my opinions and summing up some of the information here within this blog post from the sources listed above.

Orphan Disease

Do you fear your birthday? Do you worry about the fact that your days are numbered? Do you fear aging because you know death could be nearby?

I do, and many others like me do too. We do this because we have “orphan” diseases and know that our lives are numbered unless a miracle occurs and a cure is found.

1528646_689146354459709_255445209_nMy name is Mika J. Covington; I’m 24 years old and I have cystinosis. Cystinosis is a rare “orphan” disease that causes the amino acid cystine to accumulate in the cells. As the cystine accumulates in the cells, it slowly damages organs including the kidneys, liver, thyroid, eyes, muscles, and brain. Pharmaceutical companies’ control which diseases are to receive attention and funding from the medical and research communities by determining how much financial gain they will make from the disease. Pharmaceuticals created the term “orphan” disease.

An “orphan” disease is a disease that has not been “adopted” by the pharmaceutical industry. Critics maintain this because there is little financial incentive for the private sector to make and market new medications to treat or prevent them. There are almost 7,000 “orphan” diseases in the United States that collectively affect nearly 30 million people. An average of about 4,288 people for each disease. Specifically, in the case of cystinosis, only 2,000 people in the world are affected and in the United States there are only 500.

ki2011301f1Cystinosis has three forms, nephropathic (infantile), late-onset (intermediate), and ocular (adult). I have nephropathic (infantile) cystinosis, which is the most common and severe form of the disease. Patients with nephropathic cystinosis appears normal at birth, however before one year of age they have excessive thirst and urination, and failure to thrive. They are smaller than others their same age and often tend to be in the lowest percentile or even off the pediatric growth chart. And they have delays with walking and bearing weight. Late-onset cystinosis, kidney symptoms typically become apparent during adolescent years. With ocular cystinosis, cystine crystals are present in the eyes but kidney function remains normal.

970200_581143018593377_1471588441_nUnfortunately, cystinosis has only one treatment, cysteamine. Cysteamine slows the progression of the disease by removing the cystine from the cells. There are only two forms of the medication, Cystagon and Procysbi. Cystagon was approved by the Food and Drug Administration (FDA) in 1994, and must be taken every six hours every day and has many side effects. Procysbi was approved by the FDA in 2013 for the treatment of only nephropathic cystinosis in adults and children six years and older. Procysbi is a delayed-release form of cysteamine that must be taken every 12 hours every day with many of the similar side effects as Cystagon. However, there is only one treatment for the corneal cystine crystal accumulation, Cystaran. Cystaran was approved by the FDA in 2012. The medication must be put in the eyes, one drop in each eye every hour while awake.

I have been on Cystagon for nearly 22 years of my life. I was on the drug for a part of the clinical research trial before it was approved by the FDA. Cystagon kept me alive, however it made me sick. It caused me to have nausea, vomiting, gastrointestinal issues, headaches, bad breath and body odor. The medication did this because Cystagon dissolves quickly in the stomach causing the harsh chemical make-up to affect the stomach, which has been commonly known to cause stomach ulcers. Taking Cystagon, we don’t get a break because it must be taken every six hours every day. Now, I am on Procysbi.

Jon and me after our surgeries!
Jon and me after our surgeries!

Another major treatment for cystinosis is a kidney transplant, which has become a standard in the treatment of cystinosis because cysteamine only slows the progression of the disease. If not treated by cysteamine, kidney failure occurs at about 12 years old or younger. With cysteamine treatment, the damage of the disease commonly leads to kidney failure by the late teens. Most of the patients must be placed on dialysis to keep them alive before they receive their kidney, which causes more pain, and suffering; sometimes death occurs while waiting for the kidney.

1_HIV-AIDS-StatisticsNevertheless, being an “orphan” disease, cystinosis does not get the attention or research funding it need to help find better treatments or a cure. All the while, children and young adults are dying from this disease. Compare cystinosis to the Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome (HIV/AIDS), which affects 1.1 million people in the United States. Persons with HIV/AIDS tend to have a longer life-span than cystinosis patients. HIV/AIDS has more attention and gets more research funding for two reasons. First, it affects more people and thus is more profitable for pharmaceuticals to do so. (I fully recognize that this use to not be true and many people with HIV/AIDS face stigma.) Second, because of the larger number of people with HIV/AIDS, the more people are buying their products. (This is not a good thing; I believe that we should rid the world of HIV/AIDS too.) This is unlike many “orphan” disease, and especially unlike cystinosis.

what-is-hiv-aids-2

National Institutes of Health (NIH)
National Institutes of Health (NIH)

We must find a cure for all of these “orphan” diseases and certainly for cystinosis. Thus, I have been participating in a long-term clinical research study at the National  Institutes of Health (NIH) in Bethesda, Maryland. In the study, I see Dr. William Gahl, one of the leading researchers in the world on cystinosis. Dr. Gahl’s study was one of the first studies of its kind on cystinosis. I currently see Dr. Gahl for the specialized study of the progression of the disease, which I hope will assist in finding a cure. I began seeing Dr. Gahl when I was a baby, shortly after I was diagnosed with cystinosis. I would spend a couple of weeks at a time there. Basically, I grew up there from 1992-1996. In addition, I have participated in several other studies, including at the University of California at San Diego Medical Center, where they studied the neurological and psychological effects of cystinosis.

Today, I am raising funds to help find that cure for my terrible disease. The money raised will go directly to the Cystinosis Research Foundation (CRF). CRF is a non-profit 501c3 organization that was started by the amazing Stack family in 2003. this was after their daughter Natalie Stack made a wish on the eve of her twelfth birthday,

“to have my disease go away forever.”

cystinosis_research_foundation_partnerCRF today supports bench and clinical research that is focused on developing improved treatments and a cure for cystinosis. CRF has funded every bench and clinical research study that led to Procysbi, allowing cystinosis patients like me to take the drug every 12 hours instead of every 6 hours, which has improved our quality of life. They established the CRF Cystinosis Gene Therapy Consortium, whose mission is to bring stem cell and gene therapy to clinical trial. They also work on effects of cystinosis on neurological function and cognitive development, causes of muscle-wasting and potential therapies.

Finding a cure may save my life, as well as others with cystinosis including my sister Mary, or even persons with other diseases. Pharmaceutical companies may not make a profit off the knowledge discovered by studying one “orphan” disease, however often those discoveries leads to advancements in other diseases.

To help find that cure, please make any kind of donation you are able to the Cystinosis Research Foundation (CRF) on my behalf here: Hope Through Research

*All funds donated going directly to research.*

Thank you! Still time to Give HOPE through Research!

FundaCureHuge thank you to everyone who have already donated to the Cystinosis Research Foundation to help Give HOPE through Research!

It means so much to me! I realize that it was during a weekday and that not everyone who wanted to attend was able. Therefore, there is still time to donate! Go here to: DONATE

If you also were interested in hearing other cystinosis patient’s stories please watch the youtube video below of Tina’s story.

If you were quite interested in what I was going to say in my little speech you can check that out right bellow!

599457_111877858954314_119659440_nMy name is Mika Covington; I’m 23 years old and hope to live 23 years more. I was diagnosed with cystinosis around age 10 months old. Cystinosis is a rare “orphan” disease that causes that amino acid cystine to accumulate in the cells. As the cystine accumulates in the cells, it slowly damages organs including the kidneys, liver, thyroid, eyes, muscles and brain. An orphan disease is a disease that has not been “adopted” by the pharmaceutical industry because it provides little financial incentive for the private sector to make and market new medications to treat or prevent it. Almost 7,000 rare or “orphan” diseases in the United States collectively affect nearly 30 million people. In the case of cystinosis, only 2,000 in the world are affected.

Cystinosis has been a struggle. It has been a challenge for me and those who are about me. Yes, I maybe a so-called survivor; however I’m living with it every day. When I was a kid, I was always seen as different. Every day, I went to the nurse’s office to take medications. Many days, I had bad breath and body order from the Cystagon, and I felt sick to my stomach that usually ended in me vomiting. Elementary and middle school were especially difficult for me because of this. Not to forget all of the doctor appointments and hospitalizations I had to go to which caused me to frequently miss school. Growing up with cystinosis is difficult and quite the journey.

Cystinosis has been difficult on my family, too. I cannot even comprehend how heartbreaking it must have been for them to get the news that cystinosis is an incurable disease. Then, to see me go through all of the hospital visits, side effects from the medications, and just daily life with cystinosis. However, I am proud because I am one of the only cystinosis patients to make it to 19 years old before needing a kidney transplant.

In 2010, my senior year in high school, I turned 19 and lost my health insurance. This happened because in Nebraska, you are an adult at age 19 and you must re-apply for Medicaid. I did just that and I was denied. I was told that I was not eligible for coverage for having a pre-existing condition (the cystinosis). I tried applying four times with the same results each time. With no avenue to appeal their decision, I decided to focus on graduating high school and going to college until my scheduled trip to the National Institutes of Health that fall. I went most of that year without any of my medications. this resulted in me going into end stage renal or kidney failure and it probably cut a few years off my life. I went from needing a kidney transplant in 2-4 years to needing one in six months to one year. If I would have had health insurance, I might have been able to wait until after college to get a kidney transplant.

International Day Against Homophobia and Transphobia Rally
International Day Against Homophobia and Transphobia Rally

When I graduated from high school, I knew I wanted to contribute to society in any way possible and work to create the change I seek. However, because of having cystinosis and going on dialysis, it forced me to stop working. To stay busy, I volunteer on issue and political campaigns, such as fighting for Full LGBT Equality, voting rights, and health care for all. I am passionate about these because I look forward to a future where everyone has the right to vote, has full and equal human rights, and access to high quality health care. For me, these causes are important because I know first-hand how not having access to health care can cause a chronic disease to get worse like my disease did.

I was on dialysis for almost three years. I began dialysis in May of 2011, when I was a patient at the University of Nebraska Medical Center (UNMC) being evaluated for kidney transplant. I first was on hemodialysis, a form of dialysis that is performed with a catheter placed in the chest that is used as an access to cycle large amounts of blood into a machine that cleans the blood and returns it to the body. Fortunately, I was only on this form of dialysis for nine months, until I switched to peritoneal dialysis.

I started peritoneal dialysis in March of 2012, because I was denied getting a kidney transplant at UNMC from my living donor and would need to be on dialysis much longer, in addition to hemodialysis not working out for me. I had many complications with hemodialysis and cystinosis. For example, cystinosis patients are not your typical kidney failure patients because we still need access to water and potassium. This is why our specialists recommend having the kidney transplant as soon as possible. Most health care professionals are not fully educated on cystinosis. Therefore, cystinosis patients like me must take it upon us to help educate our health care professionals.

 554643_454772334563780_354924217_aEven with the additional education sometimes, mistakes are made. While I was on hemodialysis, it caused me to continuously become dehydrated, have hypotension (low blood pressure) and tachycardia (fast heart rate). In addition, I had other complications like the catheter itself falling out of my chest and a couple of times where there were infections. By the end of the period of me being on hemodialysis, I had seven hemodialysis catheters placed in my upper right chest. I can show those afterwards. 

Peritoneal dialysis is performed using a catheter placed in your abdomen that cycles a dextrose mixture fluid into your peritoneal cavity that uses the wall of the cavity as a natural dialyzer that cleans your blood. Peritoneal dialysis was much better for me because I was able to better control how much fluid I take off my body and my health care was more in my own control. It also was done at home. This way I did not have to go to a dialysis center. It gave me more of my life back. I was on peritoneal dialysis until May 30, 2013, when I received the Gift of Life from my living donor. I had the kidney transplant at the wonderful University of Iowa Hospitals and Clinics (UIHC), in Iowa City. At UIHC, I received excellent care and compassionate treatment. I personally feel they are the best in the Midwest. 

Today, I am living with a new kidney, which I named Serenity after the ship on FireFly a scifi show, and doing wonderfully in aspects of my kidney health. The transplant team at UIHC was impressed with how well my body accepted the kidney and recovered from surgery. My creatinine level, which determines how well the kidney is doing, is 0.9. This number is awesome! You don’t always see transplant patients do this well at this point post-surgery! Even my incisions are healed so well you can hardly see them. I can show those to you afterwards too! I am now down to only having blood draws once every other month to check my levels and only need to visit UIHC once a year. 

Currently, I am not employed because even though my kidney issue is resolved for the moment. I still have cystinosis; remember it affects my entire body. Because of cystinosis, I have Fanconi Syndrome, where I constantly must fight the loss of water, important minerals, salts, and nutrients. I have issues with my eyes being extremely sensitive to sunlight and light in general. I also have some issues with my heart and frequent headaches. This all leads to a lot of stress and still feeling sick. 

I must point out, I don’t only have cystinosis. I have nephropathic or infantile cystinosis. There are three forms of cystinosis: nephropathic (infantile), late-onset (intermediate), and ocular (adult). The most common and severe form is nephropathic cystinosis. Patients with nephropathic cystinosis appear normal at birth. However, before one year of age have excessive thirst and urination, and failure to thrive. They are smaller than others are their age and often tend to be in the lowest percentile or even off the pediatric growth chart. There may be delays with walking and bearing weight. With late-onset cystinosis, kidney symptoms typically become apparent during adolescent years. With ocular cystinosis, cystine crystals are present in the eyes but kidney function remains normal. 

Fortunately, we have the drug cysteamine to slow the progression of cystinosis by removing the cystine from the cells. There are two forms of cysteamine Cystagon and Procysbi. The FDA approved Cystagon for the treatment of cystinosis in 1994. Cystagon must be taken every six hours, every day. I was on Cystagon during the trial and had been taking it for nearly 21 years of my life. It caused me to feel sick almost every day. 

Last year, the FDA approved Procysbi, a delayed-release capsule for the treatment of only nephropathic cystinosis in adults and children 6 years and older. I was lucky enough to start Procysbi about four months ago and I have nearly no side effects from the new form. There is only one medication to treat the corneal cystine crystal accumulation in patients with cystinosis, Cystaran. Cystaran must be used every hour while awake in order to remove the cystine crystals from the cornea. Patients who begin cysteamine treatment early enough, and are compliant in taking cysteamine as prescribed, generally delay the need for kidney transplantation for several years. 

250px-NIH_Clinical_Research_Center_aerialWe must find a cure. Thus, I am participating in a long-term clinical research study at the National Institutes of Health (NIH) in Bethesda, Maryland. In the study, I see Dr. William Gahl, one of the leading researchers in the world on cystinosis. Dr. Gahl’s study was one of the first studies of it’s kind on cystinosis. I currently see him for the specialized study of the progression of the disease in my body and to aid his research in the long-term effects of the disease, which I hope will assist in finding a cure. I began seeing Dr. Gahl when I was a baby, shortly after I was diagnosed with cystinosis. I spent several weeks at a time there. Basically, I grew up there from 1992 to 1996.

In addition, I have participated in several other studies, including at the University of California at San Diego Medical Center, where they studied the neurological and psychological effects of cystinosis. I was also a participant in the study at the NIH, which proved to the FDA that the eye drops work to reduce the cystine crystals on the corneas. 

Today, I am here raising funds to help find that cure for my terrible disease. The money raised here will go directly to the Cystinosis Research Foundation (CRF). CRF is a non-profit 501(c)3 organization that was started by the amazing Stack family in 2003, after Natalie Stack their daughter, made a wish on the eve of her twelfth birthday, “to have my disease go away forever.” CRF today supports bench and clinical research that is focused on developing improved treatments and a cure for cystinosis. 

CRF has funded every bench and clinical research study that lead to Procysbi, allowing cystinosis patients like me to take the drug every 12 hours instead of every 6 hours, which greatly improves our quality of life. They established the CRF Cystinosis Gene Therapy Consortium, whose mission is to bring stem cell therapy to clinical trial. The CRF is currently funding investigators in eleven countries. Some of the areas of focus include stem cell and gene therapy, effects of cystinosis on neurological function and cognitive development, causes of muscle-wasting and potential therapies, etc. 

Finding a cure may save my life, as well as others with cystinosis including my sister Mary, or even persons with other diseases. Knowledge discovered by studying one “orphan” disease often leads to advancements in other diseases. 

Can I count on you to join me?

Go here to donate online: Fund a Cure 4 Cystinosis 

Help Fund a Cure for Mika’s Disease

599457_111877858954314_119659440_nMy name is Mika Covington and I live with Cystinosis. I am 23 years old and hope to live 23 more.  I was born with Cystinosis and diagnosed around age 10 months. Cystinosis is a rare metabolic disease that causes cells to crystallize causing early cell death. This happens because the amino acid cysteine accumulates in the cells, but has no transporter out. Cystinosis slowly destroys the organs in the body including the kidneys, liver, eyes, muscles, and brain. Cystinosis is a progressive disease. As I age, the disease affects my body further, inflicting damage to multiple organ systems. The medications I take only slow the progression of the disease but there is no cure. I have already been through the kidney failure, next up is thyroid insufficiency, calcifications on my brain, muscle wasting, and swallowing difficulties.

Cystinosis has always been a struggle for me and my friends, and my family. It of course continues to be a struggle. It has caused a lot of trauma not only to me but also to those who care about me. I cannot even comprehend how difficult it was for my family to get the news of my disease and how it will eventually take my life if we do not find a cure.

Post-Op Kidney Transplant
Post-Op Kidney Transplant

A year ago, I had a living donor kidney transplant at the University of Iowa Hospitals and Clinics (UIHC). At UIHC, I received excellent care and treatment. I personally feel they are the best in the Midwest. I was on dialysis for almost three years. I began dialysis in May of 2011, when I was a patient at the University of Nebraska Medical Center (UNMC). I first, was on hemodialysis, a form of dialysis that is usually performed with a catheter placed in the chest used as an access to cycle large amounts of blood into a machine that cleans the blood and returns it to the body. If you are on hemodialysis for a long-term period, a surgeon will create a port in your arm or leg called a fistula. However, I was only on this form of dialysis for nine month, until I switched to peritoneal dialysis.

IV meds post-op transplant. Not many compared to most transplant patients.
IV meds post-op transplant. Not many compared to most transplant patients.

I started peritoneal dialysis in March of 2012, because I was denied a kidney transplant at UNMC. Peritoneal dialysis is performed using a catheter placed in your abdomen that cycles a dextrose fluid into your peritoneal cavity that uses the wall of the cavity as a natural dialyzer that cleans your blood. I was on peritoneal dialysis until May of 2013, when I received the kidney.

Today, I am living with a new kidney and doing wonderfully in aspects of my renal (kidney) health. The transplant team at the UIHC was impressed with how well my body accepted the kidney and recovered from surgery. My creatinine level, which determines how well the kidney is doing, is 0.9. This number is awesome. You do not always see transplant patients do that well after transplant. My incisions are completely healed. I am down to only having blood draws once a month and only needing to visit UIHC once a year.

Me in Pre-op with Diane and family
Me in Pre-op with Diane and family

In 2010, my senior year in High School, I turned 19 and lost my health insurance. This happened because in Nebraska, you are an adult at age 19 and you must re-apply for Medicaid. I did just that and was denied. I was told that I was not eligible for coverage for having a pre-existing condition. I tried applying four times with the same results each time. However, I was still in High School, therefore I stopped trying and instead focused on graduating High School. I went most of that year without any of my medications. It is a fact that because of this, it cut a couple of years off my life and made me go into renal failure more quickly. I went from needing a kidney transplant in 3 – 5 years to needing one in six months to a year. If I would have had health insurance, coverage I would have been able to wait until after college to get a kidney transplant.

Healthcare event in Council Bluffs, Iowa.
Healthcare event in Council Bluffs, Iowa.

Since I graduated from High School, I have wished to contribute to society in any way possible and work to create the change I seek. However, because of having Cystinosis and going on dialysis forced me to stop working. To stay busy I volunteered on issue and political campaigns such as fighting for Full LGBT Equality, voting rights, and health care for all. I am passionate about these because I look forward to a future where everyone has the right to vote, has full and equal human rights, and access to high quality health care. For me, these causes are important because I know first-hand how not having access to health care can cause chronic diseases to get worse like my disease did.

International Day Against Homophobia and Transphobia Rally
International Day Against Homophobia and Transphobia Rally

I am currently not employed because even though my kidney issue is resolved for the moment. I still have Cystinosis. Remember it affects my entire body. Because of Cystinosis, I have Fanconi Syndrome, where molecules that should be reabsorbed into the blood stream are instead eliminated in the urine. This leads to the loss of important minerals, salts, fluids, and many nutrients. I also have issues with my eyes being extremely sensitive to sunlight and light in general. I have some issues with my heart and starting to have some neurological issues and frequent headaches. This all leads to a lot of stress and feeling sick. Not to forget, during my time on dialysis, I gained a lot of weight, which in of itself is causing problems.

National Institutes of Health (NIH)
National Institutes of Health (NIH)

I am participating in long-term clinical research study at the National Institutes of Health (NIH) in Bethesda, Maryland. I see Dr. William Gahl, one of the leading researchers in the world on Cystinosis. Dr. Gahl’s study was one of the first studies of its kind on Cystinosis. I currently see him for specialized study of the progression of Cystinosis in my body and to aid his research into the long-term effects of the disease. I first saw Dr. Gahl in 1992 shortly after I was diagnosed with Cystinosis. Since then I have participated in several studies elsewhere including at the University of California at San Diego Medical Center (UCSDMC) where they studied the psychological and neurological effects of Cystinosis.

There has been a lot of progress in treatment and management of Cystinosis. For instance, I take Procysbi instead of Cystagon. When I took Cystagon, I had to take 13 capsules four times a day and the medication made me sick. With Procysbi, I only take five capsules two times a day with nearly no side effects. There has also been progress made in studies using stem cells, which could cure Cystinosis. Therefore, there really is hope.

I am raising funds to help find that cure for my terrible disease. I hope you all will join me in this effort. Finding a cure not only will save my life but will save my little sister’s life and many more. Can I count on you all to join me?

You can donate by visiting www.gofundme.com/9ibcmo or click here: Fund a Cure

*Funds will go directly to the Cystinosis Research Foundation (501(c)3 non-profit) that has no paid staff and sending all funds to cystinosis research.