Histiocytic disorders are a related group of diseases caused by over-production of white blood cells known as histiocytes, which can lead to organ damage and tumor formation. This group is made up of a wide variety of conditions that can affect both children and adults. In order to reduce confusion, the Histiocyte Society in 1987 classified these disorders into three groups based on the types of histiocyte cells involved:
- The first group is called a dendritic cell disorder, and the most common disease in this group is Langerhans cell histiocytosis (LCH). Also included in this group are more rare diseases, juvenile xanthogranuloma (JXG) and Erdheim-Chester Disease (ECD).
- The second group is called a macrophage cell disorder, and includes primarily hemophagocytic lymphohistiocytosis (HLH) and Rosai-Dorfman Disease (RD).
- The third group is called malignant histiocytosis and includes certain kinds of leukemia and malignant tumors.
Each of these diseases is very different, and the symptoms, rate of occurrence, diagnostic testing, and treatments vary widely. For more information about a particular histiocytic disorder, please see the corresponding pages on the website www.histio.org.
My son, Brayden, had Langerhans Cell Histiocytosis (LCH) which they question if his actually transformed into Hemophagocytosis at the end. Below is the description of LCH from the www.histio.org website:
LCH in Children
Langerhans cell histiocytosis (LCH) is the most common of the histiocytic disorders and occurs when the body accumulates too many immature Langerhans cells, a subset of the larger family of cells known as histiocytes. Langerhans cells are a type of white blood cell that normally help the body fight infection. In LCH, too many Langerhans cells are produced and build up in certain parts of the body where they can form tumors or damage organs. The cause of this disease is unknown, although many possibilities have been explored, including viruses, exposure to toxins in the environment, family history and geography. Most data support the concept that LCH is a diversed disease characterized by a clonal growth of immature Langerhans cells that appear to have mutations of BRAF in about half the cases. Excess production of cytokines (immunoregulatory proteins secreted by cells) also appears to be important. LCH is not considered a true cancer and is not caused by a known infection. It is not contagious, nor is it believed to be inherited.
LCH is believed to occur in 1:200,000 children, but any age group can be affected, from infancy through adulthood. In newborns and very young infants, it occurs in 1-2 per million. It is, however, believed to be under-diagnosed, since some patients may have no symptoms, while others have symptoms that are mistaken for injury or other conditions. It occurs most often between the ages of 1-3 years and may appear as a single lesion or can affect many body systems, such as skin, bone, lymph glands, liver, lung, spleen, brain, pituitary gland and bone marrow.
(All of the above information was taken directly from the Histiocytosis Association website. Please check out www.histio.org to find out more including more detailed information on the disesases, how they are diagnosed and treated, information on some of the testing done on histiocytosis patients (Brayden had pretty much all of them done and more), and so much more!!
On August 10, 2003 the most important day of our lives was here. We became the proudest parents to a beautiful baby boy. We instantly were in love and looked so forward to all of our hopes, dreams, and expectations coming true with this new little member of our family. He was our first child. On October 22, 2003 those hopes, dreams, and expectations were shattered when our son, Brayden Alan Sands, became an angel in heaven and was no longer able to be held in our arms but was now in the arms of the Lord. Brayden was truly an amazing and brave boy with a very encouraging soul. Every time you looked into his eyes, it was so comforting to be able to see him “talking” to us. Amazingly, this lil baby boy had a way of reassuring us that what was meant to be would be, to have no worries, place our concern in the Lord’s hands and assured us that he himself was “ok.” Instead of a life full of playing with little trucks, learning ABC’s and 123’s and many other lessons, having baseball and other sport practices, Brayden had a life full of tubes, needles, medications, chemotherapy, surgeries and everything else that is not fun or the “norm”. Going through all of that, he never let anything bother him and always let us know that deep inside he was going to be okay. His life was most importantly full of so much love and dedication from his loving family that truly adores him. He knew what his destiny was and where he was going to be in the end. As much as we (his Mommy and Daddy) love him and he loves us, we would love to have him here with us to spend our every second with. We would love to hold him in our arms and feel his warm and soft skin, run our fingers through his hair and have his little fingers wrap around ours. We would love to tuck him in every night after our bedtime prayers and get him out of his crib every morning. We would love to see him smile and hear him coo. There are so many things we would love to be doing with him, but they are all things that we are at a loss with him and will only be able to dream about until it is our turn to go to our eternal home. He knew we would give him the most perfect life we could, but in heaven there are no illnesses and nothing to fight. There is no pain and no tears. It is just a beautiful place!
A very horrible disease called Langerhans Cell Histiocytosis took over our little Brayden’s life. He was diagnosed when he was approximately three weeks old after being in two different hospitals for two weeks. We were able to have our baby boy at home for only 5 days before he was readmitted for further testing. We brought him home thinking he was a perfectly healthy baby until we got a phone call from his pediatrician later that night after arriving at home with our new bundle of joy. He informed us that Brayden’s bloodwork from the hospital showed his platelet count was low. We took Brayden to the doctor and to get more bloodwork done the very next day and every other day following until he was hospitalized due to the continual decline in his platelet count. He was at St. Rita’s Medical Center in Lima, OH for one week where they were unable to diagnose him after the many tests they ran on him. They then decided to transfer him to Children’s Medical Center in Dayton, OH where he was diagnosed with Langerhans Cell Histiocytosis one week later after performing many, many additional tests and having some surgeries. This disease is extremely rare which makes it very hard to diagnose. It took a nationally known pathologist to make the final diagnosis. It affects 1 in 200,000 children a year between the ages of 2 and 10. It affects 1 in a million when it affects a newborn like Brayden. Brayden’s skin, liver, spleen and digestive tract were affected. The only hope to put this disease in remission was to try chemotherapy. Brayden went through three different combinations of chemo before he went to heaven to be with the Lord. Brayden ended up in the ICU on a ventilator when he was four weeks old and was there until he passed away at 2 months and 11 ½ days old. We were unable to hold our child in our loving arms this entire time he was in the ICU because of all the tubes and the ventilator. The night the doctors told us they did not think he would make it through the night we requested to hold him knowing this would be our last chance, and we wanted him in the most comfortable place he could be during his last minutes on earth. Brayden was being held in his Mommy’s arms with his Daddy’s hand on his chest feeling every last breath and every last heart beat when the Lord reached out and grabbed his hand to guide him through the gates of heaven. We were able to be right by our son’s side the entire time he was ill. This is the only place we would be at such a time. Every second was very precious and meant everything to us not knowing what was going to happen. Our son, Brayden, was a very special little boy that holds a very special place in many hearts and is loved by thousands. Our son changed so many lives and encouraged so many people to look at life in a totally different perspective during the short time he was here. It is amazing how strong the impact is that he made on so many people, even the ones that were unable to meet him but got to hear his story. It really takes an amazing, strong, and brave person to do this. There are so many important lessons that he taught us about life that we really never realized before.
We continue to live our lives day by day trying to cope with the loss of our son. It is very difficult to get through our days but we always manage to in some way. The best thing you can do during a difficult time like this is to have faith in the Lord and pray for strength and comfort. It is important to lean on your family and friends for support and to always talk to someone when you feel like you need to talk even if you are saying the same things over and over. We do all that we can to keep our son alive here with us including doing things in honor of him whenever we get a chance. We love to share how amazing he was and tell his story to everyone. We want everyone to know how truly precious he was and will always be. Our little Brayden was sent to earth on a mission and got it completed in a very short time. He touched more lives than many people will in a long lifetime. Once his mission was complete that the Lord sent him to do, he got to go back home. We have asked our son to come and hold our hand and show us the way through the gates when it is our turn to go be with him once our mission is complete. What a joyous day that will be!
Thank you for taking the time to read our story! Always remember how precious life is!! Many blessings to you and your family!
Jennifer (Hirschfeld) (Sands) Gaston
-Siblings and entire Family-
St. Marys, Ohio
According to the American Diabetes Association, there are a total of 25.8 million children and adults in the United States; 8.3% of the population has diabetes. Of these individuals, 18.8 million people are diagnosed and approximately 7.0 million go undiagnosed. Prediabetes is estimated at 79 million people. In 2010, 1.9 million new cases of diabetes were diagnosed in people aged 20 years and older. I, Jamie Heitbrink, am one of those people. Here is my story.
In 2004, I was diagnosed with Polycystic Ovarian Syndrome. According to www.medterms.com, Polcystic ovary syndrome, abbreviated PCOS, is a condition in women characterized by irregular or no menstrual periods. Acne, obesity, and excess hair growth are common problems associated with PCOS. PCOS is a disorder of chronically abnormal ovarian function and hyperandrogenism (abnormally elevated androgen levels). It affects 5-10% of women of reproductive age. Most women with PCOS do not ovulate or do not release an egg every month. They are at significantly higher risk for high blood pressure, diabetes, heart disease, and cancer of the uterus (endometrial cancer). Like many patients, I was told much of this risk can be reversed by exercise and weight loss. However, I was also told I didn’t fit the “typical mold.” All my blood work and lab results were normal. There was no indication of diabetes and I was not overweight. After many months of tracking my cycles and journaling every detail, my OBGYN was able to use this information along with ultrasound to confirm the PCOS diagnoses. For years my husband and I worked with doctors to conceive a child. After many unsuccessful attempts, in early 2008, my husband Michael and I were successful in conceiving our first child.
Like any pregnant woman with PCOS, I was carefully monitored throughout my entire pregnancy. I did Yoga, watched my diet, and got plenty of rest. With taking every precaution possible, early in my third trimester, I failed my 1 hour glucose tolerance test. So, I had to go back and for a 3 hour glucose tolerance test. Whoever thought it was a great idea to tell a pregnant woman they had to fast for 12 hours then sit in a lab for another 4 hours while they drew blood every hour was crazy. Hungry and cranky, I left the lab with anxiety over what the lab test would reveal. Sad to say, I was diagnosed with gestational diabetes. A diagnosis of gestational diabetes doesn't mean that you had diabetes before you conceived, or that you will have diabetes after giving birth.
Soon, I met with a dietician, learned how to count carbohydrates and test glucose levels. At 34 weeks I went to my OBGYN twice a week for non-stress testing. After being 3 days overdue and enduring 32+ hours of labor, I delivered a healthy 8 lb 7 oz baby. After our child was born, I asked the doctors and nurses in the hospital if I need to keep testing for diabetes. I was told that it would go away. I stopped testing, stopped counting carbohydrates and focused on nurturing my new baby and enjoying time with my husband.
As time went on I began to track my cycles again in hopes of having another child. At this point, I was still carrying 15-20 lbs of baby weight. I just couldn’t shed that weight! Then I started feeling highs and lows again like I did when I was pregnant. I knew something was wrong. After visiting with my OBGYN, I started taking metformin again, a drug used for diabetes and fertility. Within 4-6 weeks of being on metformin, I lost that 15-20 lbs and was having highs and lows more regularly. This of course created concern. My OBGYN referred me to a Specialist. Once again, I was back at the lab taking another glucose tolerance test. In April of 2010, I was diagnosed with Type II Diabetes. What I thought was only going to be an issue for the term of my pregnancy turned into a lifelong battle.
A common misperception is that people with Type II Diabetes are overweight. Once they lose weight, they will no longer have diabetes. WRONG! Overall, I am healthy. I am not overweight; I eat a balanced diet, and keep active. For me, Type II Diabetes is hereditary. My grandmother and my great grandmother were both diagnosed with Type II Diabetes. In some respects, I feel blessed to learn of this condition at such a young age. Many people go undiagnosed and continue live an unchanged lifestyle. Diet and exercise are so important to regulating glucose levels. Continuously, I am reading articles, learning about foods, and trying to prepare meals that are diabetic friendly and that my whole family will enjoy.
Living with diabetes is another reminder that our generation and future generations are also at risk and we need to do what we can to make a difference for ourselves and all the others in the world that are faced with diabetes. Like any mother, I worry about my child, his future, and want him to live in a better world. Will we ever live in a world free of diabetes? Probably not, but we can get one step closer. It is a goal of the Hirschfeld Foundation to raise funds for diabetes research in hopes to find a cure; help the lives of those affected, and educate people on this rapidly growing disease. Thank you for reading my story.
The American Diabetes Association is one of the many organizations we hope to help in our efforts. Please check back to our website regularly. As we grow, we will post more information about our battle against diabetes.
To learn more about diabetes, please visit www.diabetes.org