A Hope for a Cure: Juvenile Myositis Affects a Quaker Ridge Family

Sending a child to kindergarten marks a momentous occasion for all parents. For most, it includes picking out the perfect outfit for the first day of school and making sure a favorite lunch or snack is packed. For the Hahn Family of Quaker Ridge, and specifically for 5-year-old Addie Hahn, it's different. Even in 95-degree weather, Addie has to wear long sleeves, pants, a hat, and thick layers of sunscreen. That's because Addie has Juvenile Dermatomyositis, or JDM. She also has Celiac disease which means she is on a strict gluten-free diet.

Juvenile Myositis, which includes JDM and Juvenile Polymyositis, is a group of rare and life-threatening autoimmune diseases in which the body's immune system attacks its own cells and tissues. Weak muscles and skin rashes are the primary symptoms of JM, and the body becomes easily fatigued. Every day activities like standing up, walking up stairs, or opening a door become Herculean efforts for many kids and they are hypersensitive to the sun. Many children with JM get mouth sores and are at risk for calcinosis or build-up of sheets or lumps of calcium under the skin- it can be very painful. The muscles of the digestive tract, heart, and lungs can also be affected. There is no cure for JM, only treatments that come with severe side effects themselves but also the hope (not the promise) that the disease will be treated aggressively and early enough that a child will go into remission.

Nikki and Drew Hahn and their four children are long-time residents of the Quaker Ridge section of Scarsdale. They love being a part of the Scarsdale community. Drew is the Vice President of the Volunteer Ambulance Corps and a small business owner. Nikki just completed her term as president of the Junior League of Central Westchester. She is the PTA President-Elect and has served as both Girl Scout leader and class parent.

I had a chance to talk with Nikki to learn more about the disease that Addie and the rest of her family have been forced to confront:

It sounds like diagnosis of JM can be challenging. Tell me about Addie's diagnosis.

JM is very rare. Between two to four kids per million are diagnosed with it in the U.S. each year. It's considered an orphan disease. Addie was first diagnosed with Celiac disease last July and went on a strict gluten-free diet. She didn't get better. She actually declined rapidly. Within weeks she started falling while she was simply walking. She had no stamina. She couldn't climb stairs. I was carrying her everywhere and I used a stroller a lot. She had a hard time eating because her mouth was full of sores. She was classified as "failure to thrive" at four-and-a-half. It was devastating. The lowest point was when she couldn't get out of her bedroom in the morning because turning the handle was too difficult for her due to her muscle weakness. She developed a rash everywhere. It was symmetrical and only on her joints. We had been hiking in Michigan so I took her to a clinic and it was treated as an allergy. Back in Scarsdale I took her to a dermatologist. The dermatologist biopsied her hand and two weeks later, actually when we were on our way to a rheumatologist at Columbia in the city because Addie was just fading away, I received the definitive diagnosis. During those weeks, I literally thought Addie was dying and technically she was. She was curled up in a fetal position instead of acting like a normal, happy child.

Sadly, there is no cure for JM, but thankfully there is treatment. What is Addie's treatment like?

If kids are treated early and aggressively, within four months of the onset of symptoms, they have a greater chance of going into remission within two years. However, they still always have to avoid the sun completely. Scientists believe that in addition to a child's genetic make up (including a parental history of auto-immune diseases), exposure to the sun and/or a virus can trigger JM. I clearly remember the day before Addie's rash appeared and we were in a place that was highly exposed to the sun.

Addie was immediately placed on a huge dose of steroids. She became enormous. People looked at me. I actually heard one person say, "Look at that really fat kid." We have a good friend here in Scarsdale who didn't recognize Addie a week after she started steroids. We've been able to taper the drug so she looks like herself again, but the drugs used to treat JM have serious side effects. Addie walked with a limp for a long time. She was big and puffy and we had to buy all new clothes. Her bones are affected. She had eight cavities and this is a kid who brushes her teeth and flosses always. Her eyes are monitored because the steroids can cause glaucoma. Once a week Addie gets a chemotherapy injection of Methotrexate that we administer at home. Unfortunately it can cause hair loss and it has for Addie. Once a month she goes to the hospital for an all-day infusion of IVIG- an immunoglobulin treatment. She gets a really bad headache from this and it takes her a few days to get back to feeling like herself.

How is living with JM affecting Addie and your whole family?

It affects all of us but Addie is really the one directly dealing with it. This is a lifelong disease- she'll always have it. Because of the intolerance to the sun, Addie can never go to sleep away camp. There are so many things she can't do like a normal kid. It was so hot at the Quaker Ridge open house last night and Addie had to wear a hat, long pants, long sleeves, and caked-on sunscreen. She must have been so hot and she never complains! As a family we usually have to avoid outdoor activities. My other kids worry about Addie a lot, especially my fourth grader. She asks things like, 'what happens if the treatment doesn't work?' She fears what could happen. My oldest daughter came home with a drawing in her notebook last year with the caption 'cure JM' and Addie's name underneath it with a heart which means she has gone to the curejm.org website. Penny Randall from the JCC has been an unbelievable resource for me helping me with how to talk to my kids about this as well as Addie's teachers. She always said to be upfront and honest but just to give them the information they need. The CureJM.org website says a lot more than I thought they need to know. It's all accurate- kids die from JM- but it might be more than they need to know at this time.

Addie has asked why her siblings don't have to go to the hospital for infusions. On infusion days, well, those are the longest days ever. We get to the hospital at 7:45 in the morning and then they take vial after vial of blood. Then she is hooked up to machines until 6:00 PM. The infusion center is actually an old closet that they converted into an infusion room. It's so sad to see your kid having to do that. I know it's saving her life but it's so, so depressing.

How has Addie's diagnosis affected your outlook on life?

I don't sweat the small stuff as much and I've realized the things that are really important in life. I'm less type A! I'm also less judgmental; I like to think I'm nicer. You just never know what other people are dealing with. It makes me think of when Addie was on high-dose steroids- when she had a puffy face and looked obese, even had a hard time walking. I'm sure people were so quick to judge.

We've also been so lucky to have Addie's rheumatologist at Columbia, Dr. Eichenfield. He has saved her life, I really believe that. He's such a good person, a really smart diagnostician, and he is so thoughtful with how he approaches each case.

You and Drew have given so much to the Scarsdale community over the years. How can people help Addie and the Hahn family as well as other kids who have had their lives changed forever by this disease?

People can donate money through the CureJM Foundation. CureJM funds research seeking to find a cure for JM. On Saturday, September 19th we are hosting a fundraiser along with another mom from New Rochelle who has a 17-year-old son named Russel with JM. It's at the Coliseum in White Plains and will be a fun, meaningful night for all. Tickets can be purchased at: www.biddingforgood.com/curejm.

If you'd like to donate a service or item for our silent auction, we would be forever grateful. All the money raised at the event will go toward funding research to helping kids like Addie and Russel. We are also raffling off a 2-year lease for a Mercedes sedan and tickets can be bought through the www.biddingforgood.com/curejm site as well. Email nhahn@newsroomsolutions.com for more information on in-kind donations or questions about the event.