Langerhans Cell Histiocytosis (LCH) – Rare Disease Day 2012

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Since today is Rare Disease Day, I decided to recap our experience with Langerhans Cell Histiocytosis (LCH). Langerhans cells are immune cells that are normally present in the skin and mucosa. LCH happens when immature Langerhans cells behave like cancer cells – proliferating, and sometimes travelling through the blood stream to end up in other parts of the body. It is still debated whether it is considered a cancer or auto-immune disease. Occurrence of the disease is about 1 in 200,000, but occurrence in very young infants is closer to 1 in a million, and often presents in a single place, such as the skin.

Elijah was born with LCH, involving his skin, lymph nodes, bone marrow and lungs. His liver and spleen were also enlarged. My understanding is there are not enough cases to really speak to the odds of his particular presentation. The lungs are considered a “risk organ,” significantly lowering the prognosis to about 35% for those who do not respond well to treatment in the first 6 weeks, or about 50% in the first 5 years.

Elijah during his first week

Elijah was diagnosed in 9 days and was transferred to the care of specialists at Children’s Healthcare of Atlanta. He had a Broviac central line placed immediately (because his veins would be too fragile for the caustic medicine) and started a “mild” chemotherapy of Vinblastine and Prednisone. In addition to being born with the skin lesions, he had some difficulty breathing comfortably and breast feeding. This was because the lesions were also in his airway, and lungs. The first week, he had Oxygen, then after his central line surgery, he couldn’t get off the ventilator.

Elijah after surgery to put a medicine line into a major vein - the tail hangs out of his chest, kept under a sterile dressing.

Elijah extubated (removed his breathing tube) himself in the third week. Over the next 8 weeks, he bopped around the NICU and the step-down NICU, suffering one more brief intubation when his left lung collapsed. That lung partially collapsed several times, requiring a chest tube or a needle aspiration. The staff rigged up a stopcock on his chest tube that allowed them to suck out leaking air when needed. He had one blood transfusion and multiple transfusions of IVIG (Intravenous Immunnoglobulin) to help his immune system. We wrestled around with the hospital’s need to measure his milk intake via NG tube (a feeding tube in his nose), versus Elijah’s and my need to breastfeed. I did a lot of miserable pumping and had some small breastfeeding victories.

We struggled with all sorts of heartache, including how to feel like good parents to our then two- and three-year-old boys at home. CHOA was immensely caring and did their best to care for us as a family, but we couldn’t have done it without the generosity of our families, friends and church members. They fed us, watched our boys, spent time holding Elijah in our absence, brought handmade gifts of love, prayed with us, and countless other helps.

The hospital sent us home together when Elijah was 11 weeks old, with a pump to administer tube feedings, but we used it less than a week before he was able to fully breast feed. We had a home health nurse come weekly to help me change his sterile dressing. That became a terrible ordeal as his skin became raw and rashy from constant exposure to the adhesive.

Poor little guy with the raw chest

We drove up to the AFLAC Cancer Center at CHOA for weekly chemo treatments and IVIG infusions, and ultimately antibiotics, when he became allergic to the one we administered at home. On chemo weeks, we had to give him Prednisone (a steroid) daily, which made him crazy, and upset his tummy. The treatments tapered on a schedule to every 3 weeks (a maintenance dose). When he was 9 months old, his central line broke and it was later replace with an implanted port on the other side of his chest. He continued treatment until he was a year old, and experienced well over 50 radiological exams. Finally, at age 18 months, his port was removed and it felt so amazing to have him free of medical devices!

Elijah's PET Scan showing active disease. Notice the bright yellow in the lungs and neck - gone by 4 months.
This CT scan shows the damage to his lungs (black circles) from his diagnosis in January through October. The first photo shows the collapsed lung with the black crescent on the bottom right.

 

Elijah modeling his beads of courage. He got one bead for each poke, treatment, surgery, scan, etc. he experienced his first year.

As of now, his lungs look healthy. I understand that since all this happened before the age of 2, his lungs were growing enough to completely heal. He has some mild chicken pox-like scarring on his body, and a few other scars from surgery, biopsies and chest tubes, and is a little low on the growth charts, but the biggest long term side effect he has experienced is damaged teeth. Two bottom teeth fell out in the NICU (odd to see those so soon). Later, the top front teeth and bottom roots had to be extracted because they were severely damaged – broken or malformed from the disease. At least 3 other teeth have had fillings or repairs. We think most of the damage was done while the teeth were forming and the disease was active, although chemotherapy can cause significant dental problems. No problems are anticipated for his adult teeth.

There is so much more to learn about this disease and its variants. No one is sure of the cause, but it’s not contagious and doesn’t seem to be hereditary or have any other obvious triggers. All we know is that this illness brought many people together to pray for our little man and to help us in countless ways. God is so good, and has blessed us with a normal, healthy little treasure.

Our healthy 2-year-old!

 

For more information about LCH, visit: https://www.histio.org/sslpage.aspx?pid=379.

2 Comments Add yours

  1. KIM Lambert says:

    Thank you for sharing your story. You are truly blessed to have him in your lives. I too never heard of this disease until our youngest daughter was diagnosed the week before Christmas, when she was 5 months old with LCH affecting her lymph nodes. She is now 10 months old and has completed chemotherapy. We still go to CHOP weekly for tests and CAT SCANS monthly to monitor but we too could not have gotten through this, especially in the beginning, without the help of our family, friends and community. It is amazing how much people, some strangers, step in to help when you need it the most. God Bless your precious gift and thank you again for sharing and helping to raise awareness for this horrible disease.

    Like

  2. Lisa says:

    Thank you for sharing your story. I love what you did with the beads.

    Like

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