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.

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