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.*

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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. 

Why Obamacare is important to me

1479020_10201819868972906_734981903_nMy name is Mika Covington and I am a first generation Iowan. I grew up in a small town in Nebraska in a middle class family with three siblings. Life was difficult for my family because I was born with a rare disease called Cystinosis. Cystinosis is a rare metabolic disease that causes early cell death and it slowly destroys my organs including my kidneys, liver, and eyes. There is no cure for Cystinosis, however there is some amazing research being done at the National Institutes of Health (NIH) and at the University of California at Los Angeles (UCLA) with stem cells. Because of having this disease my parents always were worried about how to pay for my medical care and still pay for all the other bills. It was always a struggle for them.

I am now 22 years old and live in Council Bluffs, Iowa where I am working on getting some college classes done at Iowa Western Community College while recovering from a kidney transplant that I had last year at the University of Iowa Hospitals and Clinics (UIHC). Because of my disease and having the kidney transplant, I cannot work and so I am on disability. Luckily, because of the Affordable Care Act (Obamacare), my insurance cannot put a lifetime cap on my coverage and when I am able to work, again I cannot be denied coverage because of having a pre-existing condition.

Image from google
Image from google
Image from google
Image from google

This spring I volunteered to be an organizing fellow with Organizing for Action because I believe that healthcare is a human right and I want to be sure that as many people as possible can get covered through the ACA. I believe it is important to make sure Iowans are educated about what the ACA is and how it is already benefiting them. Unfortunately, many Congressional Republicans want to repeal and defund the ACA. They refuse to help kids and young adults like me to get the lifesaving healthcare services because their plan is to let insurance companies put lifetime caps on insurance and deny people like me coverage because of having a pre-existing condition.

As an organizer with OFA my goal is to make sure that doesn’t happen and make sure everyone understands what the ACA is and how they can get covered through it. I want to stand up for those who cannot speak for themselves and to stand up for the least of these.

My Journey with Cystinosis & I am a Survivor

Here I am in the hospital sick because I have Cystinosis!
Here I am in the hospital sick because I have Cystinosis!

I am Mika Covington, I’m 22 years old, and I have Cystinosis. I was born with this disease and diagnosed around age 10 months. Cystinosis is a metabolic disease that causes cells to crystallize causing early cell death. This happens because amino acid cysteine gets into the cells, but has no transporter out. Cystinosis slowly destroys the organs in the body including the kidneys, liver, eyes, muscles and the brain. Cystinosis has always been a struggle for me and my family and friends. My disease has caused a lot of trauma to myself and my family and friends. I know that it must have been very difficult for my family to learn that their child has an incurable disease that will eventually take their life. I personally cannot comprehend how they could have handled getting that news.cistinosis_-432x300

My family first learned of my disease from the University of Nebraska Medical Center (UNMC) Nebraska Medical Center where they took me to try to figure out what was going on with me. Before they took me to the UNMC they took me to many different hospitals in the Omaha Metro area. UNMC was the one to diagnose me with Cystinosis because one intern happened to read an article talking about a mysterious disease. Nevertheless, at the time UNMC did not know exactly what to do with me and said that they did not think I would live much longer then about 6 years old.

250px-NIH_Clinical_Research_Center_aerialLuckily, because of that intern my family heard about the trial going on at the National Institutes of Health (NIH) in Bethesda, Maryland with Dr. William Gahl. During the trial, I started taking oral Cysteamine four times a day, and later I began doing the Cysteamine eye drops that are taken every hour while awake. Moreover, of course many other medications for all the different problems that come with Cystinosis, like low sodium, potassium, phosphorus, protein, and excessive thirst.

Now, I would like to fast forward to when I started dialysis because I finally went into end stage kidney failure (ESKF). I started dialysis at the end of May in 2011. I first started on hemodialysis. Hemodialysis is a form a dialysis that is usually done with a cardio catheter placed in the chest that is used as a port to cycle blood into a machine that cleans the blood and returns it. If you are doing a long-term hemodialysis, a surgeon will create a port in your arm or leg called a fistula or graft to do dialysis. It is kind of like creating a thick vein to use to access the blood. I personally was on hemodialysis for a little over a year and then switched to peritoneal dialysis. More on that later.

However, before I started hemodialysis in 2011; I was being worked up at the University of Nebraska Medical Center (UNMC) Lied Transplant Center to get on the waiting list for a cadaver kidney (dead person kidney). I did pass all of the tests and was placed on the list as of March 2011. Then I started to look for living donors. I found Jon von Kampen who happened to be a match. Therefore, we set up a surgery date but eventually were unable to do the transplant at the UNMC Lied Transplant Center.

PD Cycler
PD Cycler

Therefore, I would be on dialysis longer and I did not like or do well on hemodialysis. That is when I concluded that maybe peritoneal dialysis would be a better choice for me. Therefore, in February of 2012, I was set to have a PD catheter place in my abdomen. Then in March of 2012, I started peritoneal dialysis. This form of dialysis was the best for me. I got many parts of my life back and was able to become more politically active once again. I liked it a lot. I also felt better on PD.

My journey would soon take a major turn. The summer of 2012, I met Amber Tracy a Field Organizer from Organizing for America (OFA) or the Obama Campaign. She invited me to a couple of events she was holding at the college I was attending for political science and psychology. After she became to know me a little more and all of things I was doing and active in. She recommended 398777_10150940759848430_1366498105_nthat I apply to become a Summer Fellow (intern) at OFA. I agreed that would be fun and a great learning experience and so I applied and came to Council Bluffs, Iowa to first volunteer on the campaign until I was accepted and interviewed for the position. Once, I was accepted, I began doing a lot of work to elect the President. I organized house parties and other events like our Equality night. I made many calls talking to voters about what President Obama has done for the State and me and why they should consider voting for him. I also had the chance to go to Des Moines, Iowa to see the President of the United States of America speak to a crowd of supporters at the Iowa State Fair Grounds where I was able to stand on the stage behind the President. During this entire time, I was doing Peritoneal Dialysis four times a day. The Obama Campaign even arranged for me at the President’s event to get dialysis done before he spoke. They were so considerate of my condition and me.

University of Iowa Medical Center
University of Iowa Medical Center

During my time in Council Bluffs, Iowa working with Organizing for America (OFA), I started looking into the University of Iowa Hospital and Clinics (UIHC) to see if I could possibly get on the transplant list there and maybe get a live donor kidney transplant. I did a bunch of research on the hospital and their transplant team and then I finally decided to call them and get a packet of information and paperwork to fill out.

In September of 2012, I was set to go to Iowa City, Iowa to be worked up at the UIHC Transplant center to see if I could be placed on their transplant list. I did pass and was placed on their list the following month, then set out to find another living donor. I sent out about eight packets of information to people who expressed an interest in donating their kidney to me. Out of the 8 packets only 3 where returned and only one was chosen to come to Iowa City, Iowa to be worked up to see if they were a match. That donor was a match but was eventually denied by the committee to donate and so I had a choice to make, send out more packets or see if Jon was still interested in donating.

Luckily, Jon von Kampen was still interested in donating his kidney to me. So, he filled out the packet and sent it in to the UIHC Transplant Center. They immediately saw that he was a match because he was the original donor when I was at the UNMC Lied Transplant Center. Within a month or so Jon was called and asked to come to Iowa City to the UIHC Transplant Center to have some tests done to make sure he was still able to donate. Later of course, Jon was ruled able to donate his kidney and so they scheduled the transplant for May 30, 2013.

Last month, Jon, I, and our families went to Iowa City, Iowa to the University of Iowa Hospitals and Clinics (UIHC) Transplant Center to have the kidney transplant. We arrived at Iowa City a day before our pre-operative appointments because it is about a 4.5-hour drive to Iowa City. We both passed all our tests well and went back to hotel to wait until 5am the next morning to go in to have the surgery.

10250On the morning of the surgery, we all had to get up very early in the morning to get to the hospital by 5:30am, Jon’s surgery was scheduled for 7:15am, and mine would be about 20 minutes after. My surgery took about 5 hours to finish.  The staff at UIHC was wonderful to both Jon and I. Once I was moved to stage 2 right before they took me to the operating room the family started taking photos of me while I was waiting. The staff was able to get the IV started very easily. The staff also explained everything to me and I understood what was going to happen before it happened. After the staff explained everything they rolled my bed out of the room on our way to the operating room. They kept me calm and relaxed almost the entire time. When we got to the operating room they started to give me more medications, got me up on the table, and then started to put me to sleep.

After the surgery and after they had me in recovery for awhile, I finally wake up with the new kidney that I named Serenity and Serenity was working very well. Of course though, I was in a lot of pain but I was already feeling much better.  After surgery that night my medical team wanted me to get up by 10:30pm to do my first walk. My goal was to walk every 4 hours and sit in my chair 3 times a day for each meal. I was able to get up and walk at about 9:30pm. It was very painful but doable. This went on for a couple of days and then I was discharged on June 3, 2013.

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

Now, I am living with full kidney function and I am no longer in End Stage Renal Failure. My incision has finally healed and my health is so much better. Nevertheless, I had to have another surgery to remove my peritoneal dialysis catheter. That surgery was also done at the UIHC by my transplant surgeon. This surgery had some complications because of how the UNMC Transplant surgeon put it in. My UIHC

Pre-op with family
Pre-op with family

Transplant surgeon has never seen a PD catheter placed like mine was so it wasn’t a laparoscopic surgery and they had to keep me over night because of my pain levels. I was discharged the next afternoon and went back to the hotel to rest before we would head back to Council Bluffs, Iowa.

Thus, today I have pain from the two incisions for the PD catheter removal and one of them was left partly open to drain in case of infection. But other than that I am much better but I do sleep a lot and I have been able to decrease the amount of pain killers I am on to about 2 tabs at bed to help sleep more comfortably and fully without the pain. Those two incisions currently have constant pain.

Any questions about my journey or about kidney transplants or Cystinosis feel free to email me at mika.covington@yahoo.com

I am a survivor and there is nothing that can take that away!

I would like to also tell you about how my days go medically.

7am: I take medications

  1. Sertraline 2 tabs – for depression/anxiety
  2. Phospha 250 Neutral 1 tab – for Cystinosis
  3. Mcyophenolate 1 tab – for prevention of kidney transplant rejection
  4. Sulfamehoxazole-TMP-SS 1 tab – for transplant to prevent infection prophylaxis
  5. Rapamune 4 tabs – for prevention of kidney transplant rejection
  6. Prednisone 1 tab – for prevention of kidney transplant rejection
  7. Levocarnitine 2ml oral solution – for Cystinosis
  8. Cystagon (LTD DIST) 10 caps – for Cystinosis prevention of additional organ failure
  9. Cysteamine HCI BAC eye drops one drop in each eye – for Cystinosis to prevent corneal crystal buildup (I try to do these every hour while awake like prescribed)

9 or 10 am: I get out of bed to really start my day

  • I weigh myself and record it
  • I take my temperature and record it
  • I take my blood pressure and pulse and record it
  • I fill all my water bottles (need to drink at least 2 liters of water a day, my doctors want me to drink between 3-4 liters of water a day)

11am or 12pm: Meds again

  1. Levocarnitine 2ml
  2. Cystagon 10 caps
  3. Cysteamine drops
  4. Ondansetron 1 tab – for prevention of vomiting as needed (many of my medications especially the Cystagon make me very sick some days)
  5. Multivitamins
  6. Vitamin D 200 IU

6pm: Meds YAYS!

  1. Mcyophenolate 1 tab
  2. Phospha 250 Neutral 1 tab
  3. Levocarnitine 2ml
  4. Cystagon 10 caps
  5. Cysteamine eye drops

8 or 9pm: I get ready for bed on days that I don’t have any events for LB380 or other

  • I take my temperature and record it
  • I take my blood pressure and pulse and record it

11pm or 12am: Yays meds again!

  1. Levocarnitine 2ml
  2. Cystagon 10 caps
  3. Cysteamine eye drops
  4. Ondansetron

That’s how my days on average go.

Finally, here are some videos that I found that I find moving and inspirational.

Several hours after surgery. Serenity is home!
Several hours after surgery. Serenity is home!
My new Med box with some of my meds!
My new Med box with some of my meds!
All my current meds
All my current meds

Below are pictures of my incisions so don’t look if you might get sick. I cannot be held responsible.

Not too long after the kidney arrived
Not too long after the kidney arrived
Incision one from PD Cath removal
Incision one from PD Cath removal
Incision two where the PD cath use to be.
Incision two where the PD cath use to be.