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.
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Medical Update

Hi,

So as everyone by now knows I need a kidney transplant. Yes, I have friends working very hard to try to get me a pool of donors for the transplant. Nevertheless, I would like to share some information from  the University of Iowa Organ Transplant Center.

First I must point out that, organ donation to another person must be done on your own free will; that means no one can force you into this decision. Second, the donation must be given altruistically; this means that there is no illegal financial gain by anyone. Third, the Transplant Center must always maintain the best health interests of the donor. If there happens to be a clear – cut reason for the donor not to donate, the Transplant Center may deny the donor that option, even though the donor may still wish to donate.

There is no director medical benefit to donating a kidney. Although, the benefit that you will receive as a living kidney donor is the opportunity to give another person the possibility of better health compared to if they received a kidney from the list of cadaver donors. Living donor organ transplants usually increase the chance of a successful transplant. Successful transplants result in improved rates of functioning kidneys at one year, and especially in the long term, at five, ten, or more years. Additionally, there is often a longer waiting time for deceased donor transplant in which time the recipient can become more ill. (By the way I am going on my third year of waiting for a kidney transplant. My specialist on Cystinosis at the National Institutes of Health (NIH) in Bethesda, Maryland wanted me to get a transplant almost two years ago.)

It is a rare possibility that you could develop kidney failure in the future, either as a result of the donation or more  likely, due to unrelated causes. Another, less obvious benefit of donation, is the discovery of an undetected illness that might benefit from treatment.

There are some risks to living kidney donation. The kidney removal for donation is usually done by using surgical instruments and a small incision (laparoscopy). There are some short-term risks of surgery for donation include, but are not limited to: death, bleeding, need for blood transfusion, wound infection, pain, bowl symptoms, and development of a blood clot in the legs after surgery. The risk of operative death from this kind of surgery is about 1 in 10,000, the same risk for any elective general surgery procedure. Donors can expect to experience a mild to moderate degree of pain for several weeks. Rarely, patients experience pain for up to two to three months. Occasionally, patients have some discomfort for up to six months, but pain beyond six months is rare.

Before donating, you must be evaluated to determine if it is medically appropriate for you to donate a kidney. You must be 18 or older. You must have a number of blood tests. This includes screening for HIV (the virus that causes AIDS), Hepatitis B (which I am immune to, so I can receive Hep B positive kidneys), and Hepatitis C (viruses that can cause liver disease). It is a possibility that an unexpected finding during the evaluation might affect your ability to get health, life or disability insurance in the future.

Some of the long term risks of living kidney donation include the possibility of a greater risk of developing high blood pressure as you get older. This is particularly true in African-American donors. Kidney donors may also have higher chance of increased protein in the urine. Everyone loses a small amount of kidney function as they get older, even with two normal kidneys. The rate of loss of kidney function after removal of one kidney is the same as before surgery. With no kidney disease, one kidney should be adequate for the normal human life span. At present, no studies have shown any abnormal loss of kidney function in kidney donors, even up to 15-20 years after surgery. Having only one kidney puts a person at a disadvantage in cases of kidney cancer or injury that results in the need for removal of that kidney. These are rare problems, but you should tell us if you think they may cause specific trouble for you. Kidney stones can also be more difficult to treat.

You cannot have diabetes if you wish to donate. There are no tests that can predict with certainty if you will get diabetes as you get older. Diabetes is particularly of concern if you have a family history of diabetes. Your risk of diabetes later in life will be influenced by your diet, weight, and level of exercise. If you have a higher likelihood of developing diabetes, and your laboratory tests indicate some level of concern, you might not be able to donate.

The process to donating a kidney starts with a simple health status questionnaire and a blood sample to determine your blood type and compatibility. If it is determined that you are a potential donor, you will be invited to Iowa City to complete your evaluation. The evaluation includes interviews and examinations with physicians, a clinical psychologist or social worker, and a donor advocate. There will be other blood tests, and routine medical screening tests such as an EKG and chest x-ray, and a CAT scan of your kidneys. We expect your routine general health maintenance tests (e.g. mammogram, Pap test, colonoscopy, etc.) to be up to date.

In order to complete the evaluation, the transplant team will check your ’tissue type’ or ‘HLA type’ which is the complement of proteins that your cells have that are recognized by your immune system as your own and by your kidney recipient’s immune system as foreign. Since these proteins have a strong influence on the success of the transplanted kidney, it is important for the team to identify your HLA type. This is done via a blood test and involves genetic testing and by Iowa law requires your informed consent.

Once all the testing has been completed, the transplant team and your donor advocate will meet to determine whether or not it is in your best interest to proceed with donation. The transplant coordinator will let you know the result of the meeting.

You may decline to donate at any time before, during or after the evaluation for any reason. If you chose no to donate, the reason for your decision can only be disclosed if you allow it. On occasion if you chose to donate, portions of your medical information may need to be shared with the recipient so that they can consider the risks and benefits of accepting a kidney from you.

After the donation surgery and after being discharged from the hospital, you will be asked to return to the University of Iowa Hospitals and Clinics for a post-op visit with your surgeon. This is usually scheduled 1-2 weeks after discharge. The primary reason is to ensure that your wound is healing well and that you do not have any other problems. Ideally, the transplant team would like to see you at 6 months, 1 year, and 2 years. You will receive a letter with the team’s recommended follow up at the 6 months, 1 year, and 2 year intervals. The team can work with your primary care physician to accomplish this if travel to Iowa City is too difficult.

All medical costs of your evaluation as a potential kidney donor, and the cost of the actual surgery, will be covered by the recipient’s insurance. There will be no charge to you or your insurance carrier for these medical expenses. However, sometimes during your evaluation, the team may find conditions that may require further tests and treatments for your own benefit. Whether you become a kidney donor or not, paying for these tests and deciding where they should be done, will be a choice made by you and your insurance company.

The cost of your first follow-up visit after surgery will also be covered by the recipient’s insurance company and/or Medicare. In addition, any problem occurring during the first 6 months after surgery, which the UIHC transplant physicians believe is related to the donation, will be covered.

After 6 months post donation, the Transplant Program will no longer assume responsibility for the costs of care related to complications of donor surgery or the recommended follow-up care described above.

So there is much of the information that is most important to the donation process. If anyone is interested in donating please send me an email at mika.covington@yahoo.com.

*All information provided above was provided by the University of Iowa Organ Transplant Center.

Queer Goes to the NIH!!

Me in the hospital because of getting really sick with my good friend Nancy!!

Today I will be leaving for Bethesda, Maryland to go to the National Institutes of Health (NIH). I am going to the NIH because I am in a clinical research trial for my Cystinosis. We are working on a cure and better treatment for Cystinosis. I will be at the NIH through Wednesday October 17. While there, I will have almost a full work up. I will have some bloodwork, Echocardiogram, Pulmonary Function Test, Ultrasound of my kidneys, Dental exam, EKG, and Eye checkup. On the last day of the visit, I will meet with Dr. William A. Gahl and his research team.

National Institutes of Health (NIH)

I have been seeing Dr. Gahl from since I can remember. I have basically grown up at the NIH. Some of their nurses have taken care of me from when I was a binky baby to now being a 21-year-old person. So many memories there. Some of them are some really awesome memories and some of them are not so much. See the last time I was there I got the news that I would need to get a kidney transplant and this year I am worried what I will hear. Nevertheless, I know that there must be something negative because I am in the final stage of Cystinosis but yes, it is the longest stage depending on how well your medical team and support teams are and how compliant you are as a patient.

University of Iowa Medical Center

Well, I have been a compliant patient but there was a period that I did not have insurance to cover my medications and so I did not get the medications that I need for almost a year that would help prevent the illness getting worse. Now, I could have had my kidney transplant a year ago but I face discrimination based on my gender identity at the UNMC Transplant Center in Nebraska. Therefore, I am now working to try to get the transplant done this winter at the University of Iowa Medical Center.

So anyways, I happen to be very nervous for this trip that I am actually having an anxiety attack worrying about that will happen while I am there and if they will tell me some really bad news. So please everyone please keep me in your prayers, thoughts, and send me some positive energy. Thank you so much!

BREAKING NEWS!!

 

 

 

I have some very good news! The University of Iowa Medical Center will transplant me!!!!!! At this time, I am mailing out information packets to all my prospective donors for them to review the information and fill out the paperwork and mail it in to the UI Medical Center. Once the UI Medical Center receives the paperwork and call them and set up appointments for them to come in and get worked up to see if they are a match to me, we will then start talking about transplant surgery dates!!!! This is some fucking awesome news people!!

In addition, they do want to see the results from my endoscopy and my TB tests and call the notes from my dialysis center. Plus, they do want me to have on going sessions with a psychologist. Here are their exact words for why they want me to see a psychologist, “you got a lot of problems.” LOL I think that is a LOL moment because they are talking about my illness, relationships, family, being queer, and being genderqueer. I have a lot of stress in my life and I agree with them I should see a psychologist to work through all the stress and major life decisions I will need to make.

Therefore, I am working on all that stuff while preparing to go to the National Institutes of Health in Bethesda, Maryland on October 14th. There they will work me up to see how the Cystinosis has progressed and where I am at right now. I really hope to have at least some good news to bring back but IDK if I really will. I am in end stage Cystinosis and Cystinosis patients usually only live to be about 30 and I am 21.

Well some other news, I have not fought the University of Nebraska Medical Center Transplant Center yet for all the shit they put me through but their time is coming up fast. I am still so hurt and pissed off at them. They put me through so much shit and I really did hold it together. I did not fall apart when they told me that they were canceling the surgery that would save my life and I did not fall apart when they told me that they would not treat me there any long. Now that I have moved over to Iowa and I am a patient at the UI Medical Center, the time might be now to take action against the hateful UNMC Transplant Center! (I am not going to take any violent actions against them. There might be a protest but that would be about it. Maybe even a sit in sometime after my transplant at the UI Medical Cetner.)