Sunday, November 18, 2012

Evans Syndrome: A Family Affair

Contributed by Joe Camilleri


In 2007 and again in 2010, I was diagnosed with Evans Syndrome - a rare autoimmune disorder in which the body produces antibodies that destroy the red blood cells, platelets and white blood cells.  It has no known cause and is not believed to be hereditary. However, my situation appears to be unique even for this relatively rare disease.


I was born on the island of Malta and immigrated to the United States in the 60's with my family as a child.  My older brother, who was also born in Malta, was diagnosed with ITP as a baby.  The disease went away without any treatment.

I had an uncle in Malta, who passed away from an unknown blood disorder before I was born.  It is now speculated that the disease might have been ITP and/or hemolytic anemia.  His son, who eventually immigrated to Australia, was diagnosed with ITP several years ago.  The disease went into remission after splenectomy.  He passed away last year but I don't have details because I had only recently discovered that he was my cousin.

My sister, who was born in the United States, was diagnosed with ITP about 10 years ago.  She was treated with Prednisone and the disease went into remission where it has remained.

My Struggle

I was diagnosed with Evans Syndrome in 2007.  It was initially treated with Prednisone but its effects were very short-lived.  My hematologist recommended splenectomy but I refused since my spleen was healthy and functioning normally.  A very low platelet count helped my argument against the surgery. 

At that time, Rituxan was being used as a cancer treatment but not widely used for the treatment of other diseases.  My hematologist said it had to be a last resort but I convinced her to try it anyway.  The treatment appeared to work after several weeks.

The disease went into remission for six months, after which, my platelets dropped sharply.  Once again splenectomy was recommended and once again I refused.  Rituxan was tried again and, after several weeks, the disease again went into remission.

In September of 2010, I was once again diagnosed with Evans Syndrome.  This time the disease was a little harder to get under control and I received many blood transfusions.  I was given a dose of Cytoxan to stop the hemolysis and once again treated with Rituxan.  After several weeks, this miracle drug once again came to my rescue.

My Daughter's Battle

Immediately after I had recovered from my illness, my daughter started complaining about tingling in her legs and occasionally seeing spots.  She had a sinus infection and we thought that it was somehow related.

 Her physician recommended a CT scan in order to rule out a brain tumor.  The day after the CT scan, my daughter started vomiting.  We took her to the doctor again after this continued for several days.  We were told that it was simply the flu and that the results from her CT scan were normal.

The vomiting continued on a daily basis and many tests were done but each time we were told that everything was fine.  The nurses at the physician's office were clearly becoming annoyed with my many phone calls.  My daughter was referred to a surgeon (not really sure why) who simply said she did not need surgery but recommended another CT scan.  My daughter had not been able to hold down food for weeks and now she was asked to drink the Barium shake.  That was horrible!

With still  no answers and increasing desperation, we decided to take my daughter to Emergency because her finger tips appeared shriveled.  I thought that she might be dehydrating.  After a very long examination, the ER doctor said my daughter's kidneys were functioning at 16% and that she would be admitted for treatment.

The Real Nightmare Begins

 The sinus infection could not be treated until my daughter's kidneys had recovered to a safe level.  After a couple of weeks of treatment, her kidneys stabilized and antibiotics could be administered.  Immediately, my daughter started complaining about intense tingling in her legs and trouble moving them.  The antibiotics had triggered an autoimmune response.  After the diagnosis was repeatedly changed, it was finally considered to be ADEM - an immune mediated disease of the brain.  Left untreated, the paralysis which started in the legs, would work its way up the body until the respiratory system was affected resulting in death.

Treatment for ADEM was identical to that for Evans Syndrome - Prednisone and IVIG.  After her condition stabilized, my daughter was released to a rehabilitation hospital to try to regain the use of her legs.  She was told that she may always have a mobility problem.

My daughter went to physical therapy and returned to school in a wheelchair after missing much of her senior year of high school.  Her goal was to walk again when she graduated.  She achieved her goal and made plans to attend college but not before following in my medical footsteps.

Evans Syndrome Strikes Again

Everything seemed to be returning to normal when my daughter complained about feeling badly.  We assumed it was due to her impaired kidney function and took her to the hospital.  Tests showed that her hemoglobin had dropped sharply.  Since she was still technically a pediatric patient, her treatment was a little different than mine.  Her hematologist had her do plasma pheresis, then prescribed Immuran, Prednisone, IVIG, countless units of blood, and finally Rituxan. 

My daughter's hemoglobin was 3.1 at its lowest point.  It took about 8 months until her hemoglobin approached the normal range.  After it did, it was her platelets turn to crash.

Since she had turned now 18, we transferred my daughter's care over to my hematologist.  He started with Prednisone but, like me, she showed only a modest initial response then nothing.  She started a Rituxan treatment and ultimately responded as I did.

Fighting the Disease

Rituxan appeared to play a key role in combating Evans Syndrome for my daughter and I.  However, I believe that a good support system and faith are also keys to overcoming this disease.  To me, the psychological stress was far worse than the physical symptoms.

For anyone interested, I kept a diary of my second battle with Evans Syndrome.  You may read it or watch the video presentation at   It was my intent to record my most intimate thoughts as I once again battled this terrible disease.  I was sure that God would once again answer my prayers.  What I experienced was beyond my wildest imagination.  My story is hard to believe but none of it is fabricated or exaggerated.

I pray that you may find a bit of comfort or inspiration in what I have written.  If you or a family member is experiencing Evans Syndrome, it is easy to feel overwhelmed and desperate.  However, never lose hope.  God answers prayer.  I am not sure I honestly believed that before I got sick.  I am quite convinced of it now.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Monday, August 6, 2012

Carter's Story

Contributed by Carter's Mom

Carter's Family
Our son, Carter, is a fun, energetic, absolutely adorable almost-3-year-old little boy. His story began when we found out we were spontaneously expecting not one, but two babies at our 11 week ultrasound. It was a smooth, uneventful pregnancy until my water broke, out of the blue, at 25 weeks.

Fraternal twin boys were born 3 days later, 15 weeks early, weighing just under 2lbs each.  The first 3 months of life were spent in the NICU (neonatal intensive care unit) at the University of Michigan. It was touch and go for a while and a roller coaster of ups and downs but we were extremely blessed that our boys came home strong and healthy.

One year from bringing the boys home, feeling settled into our life as a family, we were out in the yard on a snowy winter day for the boys' first sled ride. They were all bundled up in baby-blue snowsuits, and wearing sunglasses.  We had fun pulling them around. When we came inside and were taking off Carter's hat, we noticed some purple dots where the hat sat on his forehead and around his chin.  We chalked it up to either some sort of a heat rash from being bundled up or to having sensitive skin like his Dad and blamed the hat for causing the irritation.  This was on a Saturday.  

A curious and nervous mom who is the need-to-know type, I jumped on the computer to figure out what those purple dots likely were. On many pages it stated that petechiae (broken blood vessels forming tiny dots under the skin) is the only rash that is a medical emergency. Carter's spots did resemble those in the pictures, but he only had a few, unlike the pictures showing head-to-toe coverage.  As the weekend went on and the spots remained we decided to give the pediatrician a call on Monday, feeling OK that we waited based on the fact that Carter was exhibiting no other signs of being ill. The call prompted an appointment and the pediatrician immediately recognized the dots as petechiae.  However, from the minimal amount of spots, he assumed the platelet count might come back around 60,000 - 80,000, and that it was likely pediatric ITP (immune/idiopathic thrombocytopenic purpura) from which Carter would bounce back from quickly.  We were sent for a blood draw in the lab downstairs, and told we'd either get the results the next day or after hours if it was urgent. The day went on as normal with the precaution to make sure Carter avoided major impacts/falls.

Around 8pm the doc on-call phoned. In a calm manner, she asked where Carter was and how he was doing (he was peacefully sleeping in his crib) and then she informed us that the platelet count came back at 5,000. We had read enough to know this was critically low. Carter was happily asleep in his bed, so once we had family on board to take care of his brother, we were off to the ER for a hospital admittance and treatment with IVIG. There were talks of possible leukemia. We experienced immense amounts of stress from being back at the hospital we were discharged from just one year ago. We were now on the hematology/oncology floor, sharing a room with a very sick little boy fighting brain cancer.

Carter responded to the IVIG, and we were sent home with a platelet count of 73,000. Five days later the count was 90,000 and we thought we were on the way up and in the clear; so did the docs. Four days after that we noticed some spots (petechiae), and went for a blood draw that came back at 7,000. What?

The story went back and forth like this for most of the next 4+ months, as Carter was treated with both prednisone and IVIG. Finally, in May, a remission was hit after bottoming out with a platelet count of 1,000 around Mother's Day.  Carter has held strong with platelet counts ranging from 200,000 to 500,000 since (14 months).  Fingers crossed and lots of prayers still being said. The remission was finally hit after trying IVIG and prednisone combined, having tried them separately with transient response. We/the docs considered Rituximab, but we were skeptical/nervous and opted for a trial of IVIG and prednisone first, and it worked (along with many prayers).   

The first two blood draws when this all started in January 2011 revealed neutrophil counts of 300 and 1300. Carter had just overcome a cold, and the lower counts were thought to be from fighting that. Other than doing a bone marrow biopsy to rule out leukemia (ok 3 bone marrow biopsies — first sample was bad (terrifying/devastating), second was for a better sample, and third was after treatment wasn't working to make sure nothing was missed), not much attention was given to the low neutrophil count...In fact they didn't even order it on a handful of blood draws. However, as time went on and Carter's neutrophil count remained in the low to extremely low range, the docs' concern grew. The bone marrow biopsies both revealed that Carter had 10 times the amount of "baby" neutrophils than the average person. More blood tests revealed an antibody present to his neutrophils, as well as one to his platelets.  So, his body was essentially tagging and destroying the neturophils and then working in overdrive to make replacements.

Coombs tests were run twice, the first negative, the second positive. However, so far we have been blessed to stay clear of hemolytic anemia.

A diagnosis was given after ruling out many other things and going for more blood work than one could imagine a 15 month old could have gone through.  We also took Carter for a second opinion appointment at Children's Hospital of Detroit (Carter is seen at the University of Michigan), and received two opinions from docs out of state via phone and sending medical records. It took a few months to unravel Carter's case, but Evans Syndrome is his diagnosis. 

To keep a twin baby-turning-toddler boy safe from falls, bumps, and bruises was extremely challenging. He and his brother were just learning to stand and walk, and so up went adhesive bumpers on all the corners of the house and furniture. The entire first floor of our house has hardwood, so we covered that in those colorful play mat tiles you can buy. More time was spent on walks and in the pack-and-play to try to keep the bumps and bruises at a minimum. Helmets were purchased to protect Carter's little head from falls, especially when we played outside. And, we limited tumble time with his twin brother as much as we could without restraining him from being the fun, energetic little boy that he is.

Carter became really puffy from the prednisone and had petechiae and bruises from head to toe, as well as a large hematoma on his arm (that we called 911 for when we were new to all of this). It was extremely hard, to say the least.  We did all we possibly could to protect him and keep him safe. We thank God every day for the remission we hit with the platelets, and pray the neutrophils will follow in time.

The miracle about all of this is that Carter is beaming with energy and other than the blood draws, which he tolerates very well, he shows no other signs of being "sick.” He runs, jumps, plays, and wrestles with his brother just as he should. We are now on an every-other month blood-draw routine, which is fantastic compared to when we were going multiple times a week.

We know at almost 3 years old Carter's story has barely begun, but it has been so eventful from the start. 

To cope with having sick children is not easy, from a simple cold or virus to things much more complicated such as extremely premature birth and Evans Syndrome.

My mother has Lupus and Rheumatoid Arthritis, and so do three of her cousins, as did her Grandmother. I personally believe there is/can be a genetic link to autoimmune diseases, even if not completely proven by science (yet). With that said, who knows what the future holds. Obviously we hope and pray for a clean bill of health.

When faced with the ITP and neutropenia without it being leukemia or other suspects, the docs struggled for answers. I took it upon myself to read medical journal articles, contacted those who wrote ones that seemed relevant and found some wonderful, extremely helpful docs out there. All I want is for my son to be OK and live a long, healthy, happy life. I want to do all I can to help him because, although we have found some wonderful doctors, there seems to be a kind of disconnect out there in the medical world. Although I did not find it until Carter hit a remission, The Evans Syndrome Community Network has made me feel so much less alone. I kept saying to my husband and family when this was all happening that Carter could not be groundbreaking medical information; there had to be someone, somewhere who had or was going through this, too. I wanted to go on the national news and find somebody! I did even better finding this group.

May all who encounter Evans Syndrome be blessed with long remissions and may we one day find a cure.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Saturday, July 21, 2012

Conner's Story

contributed by Amanda Ballesteros

Conner Ryan Lloyd 1999 - 2012
I would like to share with others the story of my Angel, Conner Ryan Lloyd, in the hope of helping others struggling with Evans Syndrome, and to let them know they are not alone. 

My son was born a healthy 8-pounds 9-ounces on September 12, 1999.  He was a perfect baby and always happy. 

In 2004, our lives changed forever. Conner started to develop bruises almost overnight. Some thought this was a case of abuse, and an investigation was opened. We ended up at Primary Children's Hospital in our hometown of Salt Lake City, Utah. 

After undergoing blood tests, we discovered Conner had ITP. He was given an infusion of IVIg, and I thought everything was going to be OK. 

A few months went by. Conner developed a cough. I took him for a check up, and they found his hemoglobin (red blood) was low. We went straight to the hospital for additional testing. After the bone marrow biopsy came back negative for cancer, we were able to sigh relief. The blood test came back Coombs positive, and the disease rare: Evans Syndrome. 

Conner was in and out of the hospital every couple of months for mostly minor low blood counts, and he always responded quickly with a treatment and prednisone. It was difficult for our family to adjust to hospital stays and treatments, but after a while we adjusted to the situation knowing it would be a chronic illness.  

Eventually we decided to give rituximab a try. Conner went into remission for 8-9 months at a time! My son was actually able to live a normal, healthy boy's life. 

In May 2012, Conner wasn't feeling well. Stomach pains, anemia, and fatigue. We were admitted into the hospital. After 11 blood transfusions, IVIG, 500 mg prednisone, Cellcept, rituximab, and a splenectomy, Conner lost his battle with Evans Syndrome. It was June 8th, 2012. His kidneys were very stressed and not able to release all the toxins from treatments, and his potassium count rose to a level that stopped his heart. 

A parent never prepares themselves enough to lose a child. I honestly did not think Conner would have passed from this disease and its complications of treatment. I don't know if there was another underlying disease that he may have had, but the pain that Conner suffered is now over. May he rest in peace. 

Conner wanted to be a doctor so he could help other kids going through a chronic illness. I believe sharing his story is a way of helping him achieve this goal. He is an amazing, brave, and dearly missed little boy. 

As part of the Evans Syndrome Community Network, I have met some wonderful families in support of raising awareness and promoting research on autoimmune blood diseases including Evans Syndrome. I want to be a part of the support that families and individuals need when suffering from this illness. I want my son's name to always be remembered. Hopefully his story helps another. 

Amanda Ballesteros
Mother of Conner Lloyd 09/12/1999-06/08/2012

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Wednesday, July 11, 2012

Our Family Vacation

contributed by Janet Shull Shepherd

Our journey began four years ago when Brenna was 10 years old. She had a bad virus which resulted in hemolytic anemia. She was hospitalized, given blood transfusions and steroids. Doctors assured us that it was a freak thing, and that it would never happen again. 

Fast forward--two years later...Brenna was 12 years old, Zach 16, and Jared 9. My husband and I took the kids to a beach for vacation that August. Brenna started to have bruises pop up, and purple dots on her legs, arms, and the inside of her mouth. She also started her first period, and was bleeding heavily. We took her to an urgent care clinic and they sent us for blood work. 

The doctor called our rented condo and told us Brenna's platelets were 2,000, and her hemoglobin was 5. He told us to go to the nearest hospital, and that we would be transported two hours away to a children's hospital. I went into shock and started packing up our clothing for the trip. My husband drove us to the hospital. We went on to the children's hospital, and Bill went back to the condo to stay with the boys. We were about 11 hours from home, and I had no idea what was really happening with Brenna. I just knew it wasn't going to be good. 

Once we arrived at the children's hospital, they started two IV lines to give Brenna blood, platelets, estrogen, steroids, and IVIg, plus some other things to try to stop the bleeding. She was hemorrhaging: It was more blood than I had ever seen. The doctors said she lost all of her blood volume. 

To make matters worse, my youngest son had a seizure that same night. He'd had one other seizure the year before. When my husband called and told me about Jared's seizure, I broke down. 

My parents drove down and picked up the boys. Bill came to the hospital to be with Brenna and me. It was 6 days before Brenna was stable enough to make the trip home. 

Brenna remained on steroids and was repeatedly hospitalized for IVIG and other treatments before she went into remission three months later. She missed 67 days of school that year. Our lives had become a living nightmare, and we have been riding a roller coaster ever since. 

Brenna was diagnosed with Evans Syndrome and Lupus. She has chronic hives, chronic fatigue, and is frequently too dizzy to function. She has also developed depression and anxiety issues. She is very worried about starting high school this year, and missing days due to being sick. This is so overwhelming at times, and the worry and stress I have for her consumes me. I try to stay positive on the outside, but seeing my daughter suffer tears me up on the inside. 

I keep all who are suffering with Evans Syndrome and Lupus in my prayers, and pray that there will someday be a cure for autoimmunity. 

To parents who are dealing with this: I think our strength just comes from God, as difficult as it is at times we have to be strong for our children. We can curl up and cry when we are alone, but when we are by our childrens' sides, by their beds, in hospitals, during doctors appointments, we have to be their advocates, their rocks! Stay strong--You're not alone!

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Monday, July 2, 2012

My Story

contributed by Kim Wright

It was 2005. I had flu-like symptoms, was very lethargic, feeling sick. I also had a terrible headache. No matter what I did, I just couldn't get rid of the headache. After five days of laying in bed in agony, my mum said enough was enough, and called for my GP. 

The doctor came to my home and tested my urine for possible pregnancy, but that was negative. My mum is an insulin-dependent diabetic, so the doctor also tested my blood sugar levels. This revealed I was slightly hypoglycemic but that was because I hadn't been eating for several days. After the tests, my doctor rang for an ambulance, as I was on the verge of losing consciousness. 

At the hospital, I had more blood tests, and was placed in quarantine: Originally they suspected meningitis. They put me on 100 mg of prednisone folic acid, as well as a vitamin tablet. That's when I met my haematologist, Dr. Watson. He came out of semi retirement because I was a cause for concern. 

Four days later, I was moved onto the hospital's blood ward, where I was told I had a condition called autoimmune haemalytic anemia. My haemoglobin had dropped to 2, which is what caused the headaches. I was given five units of blood. 

I remember this as a very distressful time. I broke my ankle three weeks prior, so I was still recuperating from that when I was in the hospital. To this day, I suspect the trauma of breaking a limb triggered my Evans Syndrome. I was given morphine for pain, and my condition was downgraded, but when I tried to go to the bathroom by myself I collapsed. There was not enough blood to circulate through my body. The second time it happened, my heart had to be restarted. 

After three units of blood, I started to feel a lot better, and was sent home after 9 days on 80 mg of prednisone. When I returned to Dr. Watson for my follow-up appointment, he told me that I had Evans Syndrome. 

In June of 2006, my platelet count dropped dramatically. I bruised at the slightest touch, and my menstrual period was unusually heavy. Dr. Watson put me on a treatment called rituximab. Thankfully, I responded.

In November of that same year, I miraculously fell pregnant. The baby was due in August of the following year. However, not long into the pregnancy, I was told by a doctor that I should consider terminating my pregnancy: The effects of rituximab on the unborn were unknown, and my child could end up with disabilities. 

I didn't even have to consider the possibilities: I would continue the pregnancy and give my child a chance. I gave birth to a perfect baby boy weighing 6 pounds 9 ounces, 6 days overdue, by c-section. We will celebrate his fifth birthday this year. 

I have been on and off steroids now for 7 years. I have been given somewhere around 40 blood transfusions. Personally, I have found Azathioprine to be the best treatment for me. Currently, I'm on 200 mg daily, with no side effects. 

I do suffer in other ways: I have irritable bowel syndrome, rheumatoid arthritis, and I battle with my weight because of the steroids. Sometimes I get very tired, but I'm still up-beat, and I don't let this get to me. 

The main thing is that I'm alive. I don't worry about relapse, and I pray for treatment success…I don't think I would be human if I didn't. I fight…and I win. 

In between all of these years, I contracted Legionnaires Disease. I was in intensive care, and was left with weakness in my left lung. I am now prone to chest infections, which lead to pneumonia. I always have back up antibiotics at home, as infections seem to be a main cause of allowing my platelets to drop. I've learned the signs, and I'm quick to react.

I owe Dr. Watson a great deal. He is not just my consultant: He and the members of his team are my friends. I can turn to them any time. 

I am now 29 years old. I hope my story helps. Thank you for reading.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Thursday, June 7, 2012

Regarding Natural and Holistic Approaches to Treatment of Evans Syndrome

by C. McNamara Romanowski

Over and over we hear inquiries about natural approaches to treatment of Evans Syndrome. Our answer always has to be the same: Please check with your doctor before making any changes to your diet and/or medication. 

It would be nice if we could find a cure for Evans Syndrome in nature. And to be completely honest, a lot of the medicines we use today actually got their start in nature. Here are some interesting facts  (from David T. Teachey, MD):

  1. Most modern drugs are originally from nature. For example, Rapamycin is a drug that is currently being investigated in clinical trials for treating children with Evans Syndrome. Rapamycin comes from a bacteria that lives on the shore of Easter Island (known as Rapa Nui to the natives—hence the name of the drug). 
  2. Not all things from nature are good for you—arsenic, cyanide, and hemlock for example. These substances are poisonous…and they are found in nature. 
  3. With alternative medicine, you don't really know what you are getting. There are no FDA regulations on alternative medicine approaches.  Because of this, you could buy two (2) bottles of ginko from the same company, and one might have 100-times more ginko per pill than the other. 
  4. Herbal medicines can interact with other drugs. Check to make certain that there are no drug interactions before starting anything new (natural or prescribed by your doctor). 
  5. Some herbal products that are advertised as recommended for Evans syndrome can cause bleeding, and are therefore a bad choice in someone with Evans Syndrome.

Ultimately, the most important thing is to talk to your doctor before starting anything. is a great website to check for drug interactions and side effects.

Be proactive by double-checking information you get from your pharmacist. Stick with the same pharmacy all of the time: Your pharmacist knows what drugs you are taking and can make sure none of them will interfere with each other. Even talk with the pharmacist about non-prescription remedies, to make certain your prescriptions won’t be impaired by something over-the-counter.

For example: St. John’s Wort, in the US, is recommended by herbalists for treatment of depression. There is no standard dose or regulation that prevents a person from taking as much as desired. St. John’s Wort can cause an increase in serotonin levels, leading to a condition known as serotonin syndrome. Withdrawal symptoms can be extreme, and include excessive tremors, hot flushes, increased anxiety, and restlessness. On top of that, St. John’s Wort can interfere with prescription serotonin reuptake inhibitors such as Zoloft, Prozac, and so on. In the US, St. John’s Wort is viewed as a benign herb, however in the Republic of Ireland a prescription is needed for St. John’s Wort. ('s_wort) are your own best advocate, and the advocate of your children. It’s up to you to make sure you get the most appropriate care, are on the appropriate meds, and eating the appropriate diet for your circumstances and health.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Every Day Is a Gift

Contributed by C. McNamara Romanowski

My name is Constance. I am 51 years old. Seven years ago, I was diagnosed with Evans Syndrome.

There were signs that I had a problem with my immune system for many, many years, actually beginning around age 2 when I was first diagnosed with a severe allergy to penicillin.  Around age 7, I was diagnosed with eczema. At age 15 I was placed on birth control pills to control crazy menstrual cycles. At 29, it was Hashimoto’s thyroiditis. In my 30s, I developed numerous allergies to various medications, primarily sulfas. Also, I started having problems with unexplained pain throughout my body. Sometimes I wondered if I would see my 40th birthday.

When I made it to 40, I thought, “Wow, every day from here on out is a gift!”

On my 44th birthday, I was diagnosed with Evans Syndrome following a lengthy period of decline that culminated in a hospitalization.

Treatment  consisted of blood transfusions, IVIg, and finally rituximab. Not so very long after my last rituximab infusion, my blood counts skyrocketed to normal but then I started having trouble breathing.

On my 45th birthday I was diagnosed with massive pulmonary emboli in both lungs due to Factor V Leiden and a Lupus trait to hyper-coagulate. Once my Evans had been brought under control, it gave the hyper-coagulation tendencies in my body a chance to wreak havoc. My life was saved by a catheter into my lungs, injecting strong anti-coagulants directly into the clots.

Sometime during my 45th year, I was diagnosed with fibromyalgia, and then Lupus. I have been lucky, though. So far, Lupus doesn’t appear to have affected any of my organs.

Throughout this journey, I have sought information with regard to what’s happening to my body. In particular, it was difficult to find much information on Evans Syndrome. Because of that, my husband, William, and I decided to start a network of people who shared this diagnosis. The Evans Syndrome Community Network was born.

Speaking figuratively…We are from every continent; we are every race; we are every age; we are every sex. We are the Evans Syndrome Community, and we are united. We may be rare, but we are not alone. Together we will make a difference, standing shoulder to shoulder.

I have been asked, “Who are your heroes?” 

I really have to answer: "My heroes are my doctors and nurses and all of the other individuals who have participated in my healthcare. My heroes also are all of the moms and dads and grandparents and spouses out there who are caring for a person with Evans Syndrome. My heroes are those who have Evans Syndrome and fight every day to overcome its obstacles."

Today I am on blood thinners, and I am in remission from Evans Syndrome. Every day is indeed a gift…every breath, a joy.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Wednesday, May 30, 2012

With All My Heart

Contributed by Joshua Rice

Hello all. My name is Joshua. I’m a 34 yr old that has been battling Evans Syndrome.

My story begins back in 2005. I was working as a millwright in a foundry in Birmingham, Alabama, when I came down with my “problem”. It started like a stomach virus, so naturally I stayed out of work for a couple of days to rehydrate. When I didn’t get better, I decided to go to the Emergency Room for fluids.

When the receptionist called me back for my insurance info, she asked me if my color was usually yellow. I turned yellow as a school bus right in front of her, so they rushed me back and immediately did a CBC, and told me they were keeping me. Over half my blood was gone, and they couldn’t find out why. My wife and I were horrified.

Later, I was moved to a private room and was told I might have bird flu. I was quarantined, and all they would do for me was give me Tylenol for the major temp I was running. While all this chaos was going on, my whole family was called in, and were given no answers.

I ended up staying the night before I was transported to University of Alabama Hospital in Birmingham. It was a Sunday. I don’t remember much but the ambulance ride that day.

Early Monday morning I woke up short of breath. The color of my skin was pale-grey. My blood had hemolyzed to the point I had very little left. My hematocrit (blood volume) was 5. I remember the nurses getting a little blood but before they made it to the lab it would coagulate. They pumped me full of steroids and gave me what they thought was the best match in blood. Needless to say it’s a miracle I’m here today to write this biography. During this time, I felt the Grace of God surrounding me: It was a tangible presence. The next morning, the nurse came in and told me it was good to see me still here. After 5 days of working to get me stable, I was sent home with a diagnosis of hemolytic anemia, not knowing that my life would be forever changed. What I didn’t realize was that hemolytic anemia is not exactly a diagnosis: It is a symptom of a deeper problem.

After a couple of weeks of taking mega doses of steroids, my counts stabilized and became normal. I went back to work. After a month, I was given the option to work in Ohio or quit, so I just quit. My boss at the time was worried I would sue him, I guess.

It wasn’t long after that I found another job here in our town, and for a year I was going strong—all was good. Then I started waking up with nosebleeds. I thought maybe it was just sinusitis. I stayed fatigued and worn out all the time.

Finally, I decided maybe I should see a doctor. He did a CBC that showed my counts were dropping again. Back on the steroids I went, and it helped…But my platelets wouldn’t come up past 70. I still had no definite diagnosis, so I started looking for another doctor.

My new doctor did a bone marrow biopsy but still gave me no diagnosis. I felt like I was just floating along waiting to die for four more years, until 2009, when the steroids completely quit working.

I found another doctor, and had to go through the process all over again. That meant another bone marrow biopsy. This guy told me I had myelofibrosis, a disorder with a prognosis of about 5 years. All I could think of was, “At least I know what I’m facing now.”

In 2009, I slept in the hospital more than I slept in my own bed, and all my doctor would do was pump me full of steroids and transfuse me. Nothing was working, so my family doctor advised me to go to the Mayo Clinic in Minnesota to see Dr. Ayalew Tefferi. I agreed, so my doctor gave me three pints of blood, and told me “Good luck.” To be honest, I didn’t expect to come back.

My wife and I arrived in Rochester, Minnesota, in February. It was COLD! My appointment was at 10am, and we were both anxious. We walked into the clinic and were amazed: The first thing that caught my eye was a grand piano surrounded by women in wheelchairs and wearing do-rags on there heads. They were chemo patients. The pianist started playing a familiar song—Amazing Grace. As I watched the faces of those ladies beam with Glory, tears flowed down my cheeks and hope arose in my spirit. I knew all would be OK. After my wife and I collected ourselves, we went on to my appointment.

We walked into the doctor’s office, and he asked my name and what was going on with me. I described my medical history, and he listened with a concerned look on his face. He told me to get some blood tests and to see him in two days.

The morning after a day of testing, we arrived in Dr. Tefferi’s office hoping to hear any kind of news. He sat me down and told me it wasn’t myelofibrosis at all. I had Evans Syndrome, or I was in the beginning stages of aplastic anemia. We were glad that I would live longer, but we weren’t out of the woods yet.

After our conversation with the doctor, we went out of that office happier than we had been in a long time. We walked across the street to have a smoke. I looked into the heavens and asked God, “What are you doing?” While walking back across the street a green ball of light the size of a volleyball began to dance at my feet, and I knew all would be OK.

That year I was laid off from work and had to draw unemployment.  During that time, my counts dropped once again, and I was infused with a bag of platelets. From that bag of platelets I contracted e coli. It found its way to my back and destroyed half of my L5-S1, and obliterated a disk. I was paralyzed for two months. I am currently disabled.

The point of my story is not to scare or depress anyone. I want to encourage you: Never give up. Never loose hope. There is a light at the end of your tunnel. I had every reason to give up and call it quits, but I didn’t. Many times I wanted to give up and I would…for a while. But then I would find the faith to get back up and fight, and Grace just never, never gave up.

I love you all, and pray that you find comfort and peace in knowing you are not alone.

With all my heart,

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Friday, April 6, 2012

It's My Story...

Contributed by Laura Thurman Shaffer

As I was walking through the mall with my mom and daughter, I was thinking to myself, ‘How lucky I was to be buying baby clothes again!’

I had wanted this baby for years, and I was finally showing!! I also just got over a scare: I had started bleeding, and they weren’t sure why. They did do an ultrasound and found a little tear in my uterus, so I was put on bed rest for a few weeks.

Earlier that day, I had cut my finger while working in the kitchen. I looked down at the cut, hours later, thinking it was funny that it was still bleeding. It bled so much when I first cut it that I ended up with blood all over the floor, as well as covering at least a dozen paper towels! My concern led me to stop in at my husband’s station to get it looked at, and I got laughed at!

“Laura, its just a little scratch!” Nick said. He wouldn’t have thought so if he saw the mounds of paper towels and blood on my kitchen floor! Oh, well.

Back at my mom’s house a few hours later, I had to pee again—another fun perk of pregnancy! As I got up, there it was again…peach-tea urine. I called my mom in to see if I was crazy, and she thought that I needed to call my OB doctor.

“Laura, that’s weird, plus you do not look right…Are you sure your going to be OK while I am in Florida?” she asked.

“Moooooommmmmm…I’ll be just fine!” I said. To be safe, I called my OB and got an early-morning appointment.

That next day, I went to my doctor appointment, and got to hear my little guy’s heartbeat! The doctor came in, took one look at me, pushed on my stomach (which was always hurting me), and said that I needed to get to the emergency room right away.

I called my husband, but I was not panicked. I told him to stay at work; that it was probably just so I could get checked out, and that I would be home later.

I sat there alone for hours on a hard bed in the triage unit of the OB floor. They came in for blood every hour. Finally, after 5 hours of waiting, a nurse came in and said a doctor would be in shortly to see me.

That nurse did not tell me that a team of doctors was coming, but that’s what I got. I was shocked to see so many white coats standing beside the bed. They asked me all kinds of questions. Finally, one asked, “Mrs. Shaffer, have you ever had problems with your blood?” I shook my head, indicating that I had absolutely no history of blood-related problems, and then they told me they were admitting me to the hospital.

The next morning was a blur of specialists, and a statement that I will NEVER forget. “In Order To Save You, We May Have To Take Your Baby.” I was barely hitting 19 weeks: The baby would never survive delivery.

I thought, ‘After all that I had been through to get him, you want to take him away from me?? NO WAY.’

That day, to my surprise, my skin turned very yellow. Jaundice! They told me that billirubin would cross the placenta and cause problems. All day long, specialists provided me with more bad news, but not one could tell me what was wrong with me. It wasn’t HELLP (related to the very serious pregnancy complication of pre-eclampsia); although similar, some of the key symptoms were not there.

Finally, after a week in the hospital, a hematologist was able to stabilize my blood, and I went home on bed rest and monitoring. I visited my hematologists and my OB every few days, and I made it to 38 weeks…despite all the speculations of a premature birth.

I gave birth via a caesarean, which was a terrible experience for my OB! Then my hematologist diagnosed me with me Evan’s Syndrome. He seriously thought that maybe this was all pregnancy-related but my blood did not recover after birth. In fact, ITP and hemolytic anemia were getting worse. The steroids were no longer helping; blood transfusions were not helping; Iron Transfusions were not helping. Rituxan was my next step, however, the pain in my stomach was severe and the rapid growth of my spleen could no longer be ignored.

On June 3rd, 8 months after giving birth, my overly enlarged spleen was removed. I almost did not make it through the surgery. I had several blood transfusions and, if not for the patience of my surgeon and anesthesiologist, I would not be writing this today. Not only did the surgeon remove from my body a spleen the size of a newborn: He also took out an accessory spleen!

One week and a drain tube later, I was released to go home. I still felt terrible and the pain on the opposite side of my stomach got worse. One very early morning when I got up to check on the baby, I nearly passed out.

I called my husband and he took me to the hospital. They did a CT scan, which not only showed that the drain tube crossed over my stomach, but that I had several more spleens. To this day, I still have pain where my spleens are, but they are not being monitored and we do not know if they are working.

A few weeks after surgery, I was told that my Evans Syndrome was in remission, and that maybe I never had it; They just couldn’t be sure.

Today is March 28, 2012. I still start to turn yellow. I get very pale, very tired, and my joints hurt. I stutter, and I have terrible headaches. My hands, arms, feet, and legs are always numb. I am not allowed to drive because its possible that I may be having mini seizures, and I have momentary memory loss. I feel swollen and bloated. But my blood is still in remission.

In November, I was hospitalized for a critically high level of ammonia in my blood. Still no one can tell me what’s wrong with me. I need someone to tell me. I want someone to tell me. I despair.

My life has completely changed: I feel weak and not in charge of myself anymore. I have no freedom, no career path, no money, and no insurance. Things have gone downhill so quickly. When I go to my hematologist I am made to feel like a hypochondriac, as if I can NOT be validated because my numbers are good. Luckily, my family doctor is listening: on April 11th I will see a rheumatologist. My family doc suspects that lupus may be creeping through my body. FINALLY…an answer. I am awaiting an MRI—because I am an American without insurance, it’s on hold.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Monday, April 2, 2012

Ella's Evans Syndrome Story

Contributed by Libby Gray

For the first two and a half years of her life, my daughter Ella Rain was healthy. She was my first child and watching her grow up has been an experience sad and thrilling and terrifying all at once.  For the first two years of her life she a healthy, happy, beautiful, outgoing little baby girl.

New Year’s of 2004, when Ella was 29 months old and I was pregnant with her little brother, we went to a friend’s house for dinner.  My toddler ate nearly as much as my friend—a large man in his late 20s! That intense hunger was probably the first symptom we noticed.

Over the next several days she began asking for cup after cup of water, and was going through more diapers than she had as a newborn.  I contributed it to a urinary infection, or a growth spurt, or her being jealous of me and the baby.  I took her to the doctor, who said she just had a yeast infection in her diaper area and gave me some steroid cream that did no good.  My little girl was becoming different…she was whiney, tired, clingy and thirsty, thirsty, thirsty.

On February 9th, 2004, Ella woke up vomiting in the middle of the night. She cried incessantly, begging for water, drifting in and out of sleep and complaining her eyes hurt, her legs hurt, her head hurt.  I tried to brush it off as just the flu, but my heart wouldn’t let me. I didn’t know what, but I knew it was something bigger. That afternoon, my husband got on the phone with the doctor who, in turn, told us to go straight to the hospital.

To shorten this long story, it was on that day that Ella was diagnosed with Type One Diabetes, an autoimmune disorder in which the immune system destroys the insulin-producing cells of the pancreas. Her blood sugar was nearly seven times the normal level, her blood chemistry was messed up, she had lost 8 pounds in six weeks, and was less than 12 hours away from a diabetic coma.  Suddenly we were thrust onto a steep learning curve of insulin injections, carbohydrate counting, and constant monitoring and vigor to balance a delicate hormone that could save a life or take it.

As time went on, diabetes became our “new normal”, and started to fade into the background of our everyday life. Ella was diagnosed with other autoimmune conditions over the next few years: Hashimoto’s hypothyroidism, severe allergies, and asthma. 

But our Evans story begins around December of 2008, when Ella was seven years old. At first I thought she might be getting sick, or in a growth spurt: She began experiencing relentless high blood sugars, having nosebleeds and being so weak and tired during the day she was taking napping more than her baby sister.  Next came the bruises that covered her body as if she’d been in a fist fight, the cold and ear infection that never quite went away, and the way I noticed her working just a little harder to take each breath.   

I was used to seeing a rainbow of colors from Ella: pink cheeks flushed from excitement and playing in the sun; ghost-like paleness from a low blood sugar; greens and browns of dirt and grass staining her knees from a hard day of play; marker, pencil, and paints of various shades covering her hands because her creative brain felt like making something.  But this was not a good rainbow to spot: she appeared deathly grey, with dark circles under her eyes; covered in purple bruises; and the whites around her hazel eyes just slightly yellowed.

Shortly after the New Year of 2009, Ella woke up with a 103F fever, blood in her urine and out her nose, and 450 blood sugar (her range is 80-150, and we rarely see numbers that high unless she’s very sick, we seriously miscounted carbs, or there is a problem with her insulin pump). She just looked like death warmed over.  Completely terrified, we decided not to waste time at the pediatrician’s office, and I took her straight to the ER.
They immediately started running blood work and found her hemoglobin and platelet counts to be severely low. She was admitted, given antibiotics and a blood transfusion, and the whirlwind of tests began to find out what was wrong.

I remember the first night: I sat by her bed and just sobbed. Why Ella? Why my family? She’s such a good girl and she’s already been through so much. Why can’t she just be left alone? The C-word was thrown around, but I refused to let it enter my mind. Lymphoma and leukemia—such big, horrifying diseases that just don’t happen to little girls who spend their time crafting, reading for pleasure, and doing cartwheels through the grass.
On January 10, 2009, after almost four days of testing on her urine, blood, and bone marrow, a direct Coombs test revealed the duel diagnosis of ITP and Autoimmune Hemolytic Anemia—Evans Syndrome.  I asked my husband, who was at home with the other two kids and the computer, to Google this bizarre disease.  He found nothing but horror stories and brief descriptions. I so wish we had found the Evans Syndrome Community Network (ESCN) at that time.

The pediatric hematologist explained the vicious attack her immune system was inflicting upon her body, and that the best way to stop it was a strong course of steroids to combat the attack. Steroids can make even a non-diabetic resistant to insulin, so her already-high blood sugars skyrocketed to five and six times the normal levels. I worried about the formation of dangerous ketones. Ketones are the by-product of high blood sugars as the body attempts to break down fat instead of glucose for energy.  She was already sick and developing trace ketones. I didn’t want to risk DKA (diabetic ketoacidosis:  a life-threatening level of ketones in which the blood is acidic and there is no way for the body to get energy from food.)

She was released from the hospital four days later, and for the next several weeks we walked a dangerous tightrope. The steroids stabilized her hemoglobin and platelets to around 10 and 30,000 respectively, but they sent her blood sugar soaring. I doubled, tripled, quadrupled her insulin dosages around the clock, but days were rare we saw a number under 200. In addition to the immediate risk of DKA, the paralyzing fear of future heart, eye, kidney, and limb damage from relentless highs kept me wide awake at night. As soon as we began tapering her off the steroids, her blood sugar would come down, but so would her hemoglobin and platelets. She ended up back in the hospital three times in two months from low platelets, low hemoglobin, or scarily high blood sugars with ketones.

By March, we’d all grown frustrated beyond belief with the battle between diabetes and steroids and I longed to see my daughter well.  It’d now been four months since she felt like a normal child—she was always tired, nauseous, thirsty, and dizzy from the high blood sugars or the Evans.  We both cried a lot in that time. Our whole family was missing the spunky, creative, energetic little girl we’d known just a few months prior. I begged our hematologist for another option. He suggested IVIG.

In April, we began the monthly infusions. After some trial and error with working out the horrendous headaches she would get from them, she started to respond very well. Her platelets went up to 60,000; 80,000; even 110,000 and her hemoglobin climbed, too.  While she didn’t particularly enjoy sitting for 5 hours in a doctor’s office every three weeks, we were all thrilled with the progress she was making. With the exception of our treatment days every few weeks, life was getting back to normal. Ella was getting back to normal.

On June 15th, exactly one week before her eighth birthday, we began seeing dramatic drops in Ella’s white blood cell counts. We knew it increased her risk of infection, so we tried our best to keep her isolated…but in a houseful of small, social, germy children that was nearly impossible.  So began an uphill battle with chronic sinus and ear infections that would take two and sometimes three rounds of antibiotics to clear up before they would just return again. The infections also would drop her platelets and hemoglobin, trigger her asthma, cause roller coaster blood sugars, and make her just feel miserable. She went back on short courses of steroids, during which we struggled to control her blood sugar even more.  I tried every method I knew to protect and clean her sinuses and ears to prevent these infections: Neti pot washes, saline spray, fresh juice, homeopathic ear drops, garlic oil.  It helped some, but her weak immune system just couldn’t fight off these infections.

At the end of the summer, a nurse at our endocrinologist’s office recommended papaya leaf extract for low platelets, saying she knew a young leukemia patient who had great results with it for his platelets. A big fan of homeopathy, I ordered a bottle and gave it a shot. I gave her a large spoonful each morning  on an empty stomach, and her platelets shot up just as well as any IVIG treatment. We used it for about four or five months and her platelets haven’t been below 80,000 since then, even after stopping the extract.

While the papaya leaf extract stabilized her platelets, Ella still struggled with repeated infections, low WBCs and recurrent anemia. Her numbers would go up for about two weeks, then crash steadily in the remaining week or two before her next treatment. In October 2009, she was hospitalized for a week and a half for bronchitis, complicated by the Evans Syndrome, diabetes, and asthma. What a nightmare! In the prior months, we’d also had several overnight trips and 24 hour stays in the hospital to correct high or low blood sugars, ketones, and dehydration from stomach viruses and other infections.

We continued with steroid bursts, papaya leaf extract, IVIG, and round after round of antibiotics for the next year. She had a period of about two months in early 2010 when her numbers were very stable and treatment was working well; then her counts started to fall again.

In May of 2010, shortly before she turned 9, Ella had sinus surgery and ear tubes placed in an attempt to relieve the chronic infections. It didn’t get rid of them completely, but greatly cut down on the number of infections she got, and improved her quality of life.  The IVIG kept her stable for another month, so much so that her hematologist wanted to begin lowering her dose. That didn’t work well at all. Her counts fell drastically, her symptoms returned, and it took another round of steroids (and super high blood sugars) to pull them to safe levels.

After a failed, vomit-filled trial of Cellcept in August and September, Ella began the drug Rituxan in October 2010.

(Now might be the time to mention that in the midst of this chaos, we had a baby girl in August, and began fostering a four-year-old boy the week before Ella’s first Rituxan treatment. Instead of dragging all these kids with me to the hospital every two weeks, my husband graciously took Ella to all of her twice monthly infusions.)

Rituxan was a wonder drug for Ella. That, in addition to all our other measures, stabilized her counts drastically in a very short amount of time. For the first time in almost two years, she could play like a real little girl again—she could run outside with her siblings and not get tired. She could turn cartwheels and not be covered in bruises and blood. She wasn’t so sick anymore. She was Ella at last.

She took her last IVIG on December 10th, 2010, and her last Rituxan shortly after the New Year.

On February 9th, 2011—ironically the 7th anniversary of her type-one diagnosis—Ella was officially declared in remission and has remained there for just over a year. 

I thought having a toddler with diabetes and a new baby was the hardest thing I ever went through. Little did I know I would deal with diabetes, Evans Syndrome, and five young children (now six—we adopted again in March 2012). 

Ella Rain is a trooper through all of it and the strongest person I know.  On our journey with Evans, she cried, she got angry, and she got scared. But through all of it she found a way to be her quirky, outgoing, positive self, even when dealing with the worst of it all. She’s been recently diagnosed with Celiac Disease (January 2012) and is adjusting to a gluten-free life with grace. 

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

Wednesday, March 28, 2012

Colby's Journey

Contributed by Amber Wakefield

Our journey began in April of 2010, so we are “babies” on this trip! My son, Colby was in 7th grade--a normal 13-year-old who liked to play sports, was busy with his church, and was, overall, a great kid. He still is!!

Colby started coming home from school with huge bruises on his arms or legs. To be honest, I thought he was being bullied at school, and somebody was hurting him. He kept on insisting that the bruises were from basketball or games in PE. Well, the mom’s intuition kept nagging in the back of my brain and time went on for a bit more, but I always felt as if something was wrong.

The next things we noticed were huge-looking hives, along with the bruises. Benadryl would make them go away for the duration of the medication but they would come right back. Finally, I took him to our family doctor, and he, thinking Colby was allergic to something, referred us to an allergist. So…to allergy testing we went: No allergies were revealed, so a blood test was written up. We went later that same day to have his blood drawn, and then headed home.

So began our entry into the world of a thing called Evans.

At 4:00 a.m. the next morning the allergist called to tell us Colby needed to go immediately to Children’s Mercy Hospital. My husband didn’t understand what the doctor was saying, so I took the phone. The doctor proceeded to tell me that Colby's platelet count was at 4 (or 4,000, as I would later come to learn). Too many numbers and not enough explanation; they are all still so confusing at times! The doctor also explained that if we didn't go, Colby could bleed to death. So we lay in bed and, of course, I cried: The immediate thought in my head was that it was cancer or leukemia of some sort.

We decided Colby was safe in bed, and so waited until his two older sisters were up for school and told them what little we knew. They were a little upset but headed off to school with lots of texts going back and forth. We got Colby up, and told him about the phone call. We explained that we needed to head to Children’s Mercy in downtown Kansas City.

As testing continued, I realized how blessed we are to have a Children’s hospital within 20 minutes of our home. I ran into many people that traveled for hours for their child’s treatment, or would have to use the wonderful downtown Ronald McDonald House facilities. I will never complain about traffic, or the drive, because we could always come home or have family come to us.

By this time, I am a mess but trying to contain it for my son’s sake. I didn’t want him to worry or be afraid. They kept taking more blood, and finally put in a port. My prayers increased. They started off telling us Colby had ITP but more assumptions were to come. Cancer was on the list but pretty far down. The doctor-on-call saw my reaction to the work and, again, tried to reassure me that cancer probably wasn’t causing the anomalous blood counts.

Eventually they took Colby up to the hematology/oncology unit—no comfort at all. My heart ached for these parents and all they have had and will continue to endure. I still pray for those families and what must undergo. As a side note, my freshman daughter, who plays soccer, was participating in the annual Cancer Battler Cup against their rival high school. I had that T-shirt on and was still reminded of what could be.

Colby received two bags of platelets that evening but they were eaten up within 12 hours, his counts returning almost back to where he began. This gave the doctors the direction to lean to Evans and away from cancer. My husband, who never cries, broke down in tears. He had been in Colby’s room praying for him and was relieved as we all were!!

So now the Google searches began! We did exactly what they told us not to do because there wasn’t much (and still isn’t much) information out in cyberspace. We also found out his red and white counts were low but not too bad.

They sent us home the next day with orders to come back later the following week for a bone marrow aspiration. We learned this was needed in order to confirm the “no cancer” diagnosis, since steroids are the first treatment generally given for Evans. With that test behind us, and again no cancer, Colby went on an horrendous regiment of steroids, to the tune of 300mg.

It is appalling what this drug (corticosteroids) does to the body!! Colby lost a part of himself during that time. My son who normally is loving, outgoing, and will laugh at anything, became withdrawn, depressed, and quiet. He hated it, I hated it, but most of all kids at school found something for them direct their stupidity towards. He started his 8th grade year, looking like somebody else and feeling abnormal. We almost pulled him out to home school but he wanted to keep on going and things eventually calmed down. His school was great and did whatever was needed to accommodate him.

“Remission” lasted almost a year. Colby was and still is good about pointing out bruises or rashes. We went back in and blood work showed a big drop again so this time they did rituximab but without steroids!! Colby asked me in the car on our way home after finding out his counts had dropped, “Do I have a say in all of this?” I said, “of course.” This was the day he decided, never again on the steroids unless it is the only treatment!!

Four rounds rituximab of later, Colby has been in another “remission” for almost a year now!! His last set of blood work showed even more of a raise in platelets, red, and white cell counts. We don’t have to go back to the doctor for another 6 months, unless he shows signs of its return.

All during this time, our church prayed and continues to pray for Colby. There is much comfort in knowing others are on this road with us, and that no matter what happens, the Lord will never leave us. The end result may not yield what we want, but the lessons learned will always be priceless.

Colby has given up the sports but has found a new passion with acting and singing. He found his voice in a new way and has a heart that is bigger than a mom could ever imagine. For that, I am grateful for this path called Evans.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.