The story of Ami
It is the final day of #HistiocytosisAwarenessMonth, and this is MY #HistioWarrior, Sean. Our journey has been long, so please bear with me.
Our journey began in September of 2004 with a collapsed lung. He was only 6-1/2. Then it happened again a month later, and a procedure was done to make that lung stick to the chest wall. On a visit to the pediatric cardiologist for an unrelated issue, it was discovered his thyroid was enlarged. The following spring was testing for CF and A1AD, June brought another collapsed lung, and in July a trip to Minneapolis for surgery to repair a hole in his heart and see a pediatric pulmonologist. They also took lung biopsy samples.
That August (2005) he had his fourth collapsed lung and we were sent straight to Minneapolis where I was informed they found nothing fixable on the biopsies. He had end stage idiopathic pulmonary fibrosis and would need a lung transplant. He was only 7-1/2! By this point he hasn’t grown or gained weight in a year and would need to do so before they could list him.
While doing the transplant work up, they discovered his liver was also sick and testing eventually indicated he had Primary Sclerosing Cholangitis (#PSC) and would eventually need a liver transplant, also! Each was functioning too poorly to hold him through transplanting the other, so they worked on getting the liver more stable.
February 2006 he received his new lungs, 2 weeks after his 8th birthday. Over the next 17 months he had repeated infections, bouts with sepsis and pneumatosis (air in the intestinal wall), chronic c diff infection, surgeries, etc. We did IV meds at home. He developed Diabetes Mellitus from the prednisone, and Diabetes Insipidus for some unknown reason. Along the way we suspected LCH was the reason for all this, but could never prove it.
In July of 2007 he received his liver transplant and things improved greatly from there. (You can read about that here: https://www.facebook.com/HistioMom/posts/1787920448116255 )
July 2010 we got our confirmation of LCH when it returned, this time to his transplanted lungs and his mastoid. He had chemotherapy for 6 months and it went well.
July 2012, however, we again discovered the #Histiocytosis had returned, this time to the same mastoid with the new addition of skin lesions. He had 3 radiation treatments to his mastoid and a year of chemo.
As of now, Sean is almost 19 years old and just over 3 years NAD (No Active Disease). Histio has taken his lungs, liver, and thyroid and damaged his pituitary and mastoid, but it has NOT taken his life.
He will forever take drugs to suppress his immune system so he does not reject the transplanted organs. He takes hormones to replace those lost from the thyroid and pituitary damage, a daily antibiotic to prevent a particular type of pneumonia, a medication to thin the bile in his liver, allergy medication, an antidepressant, and an appetite stimulant. He’s smaller than most kids his age, only at the 10%, but considering he spent 8-1/2 years not even on the chart, we’ll take it!!
This journey has taught me a lot, brought me many friends, and many opportunities to help others through their own journeys. Sean’s story has given others hope and strength.
I have become a part of an amazing family – my Histio family – that I never would have had. I am honored to have them in my life and to be a part of their joys and sorrows. There have been too many heartbreaks along the way, and that is why I fight to make others #HistioAware, to know the signs, to push for testing and treatment. When another Histio Warrior I’ve never met becomes a #HistioAngel, a part of my heart goes with them.
Thank you all.