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Cure JM Family Survey: Early Diagnosis Initiative

Summary of Responses: Jan. – March 2016

Number of Respondents

403 respondents representing 18 countries and 47 U.S. states

Gender

  • 73% of patients are female
  • 27% of patients are male
Female vs. Male

Type of Myositis

  • 98% have JDM versus JPM
JDM vs. JPM

Age of Diagnosis

  • 37% were diagnosed between the ages of 3-5
  • 28% were diagnosed between the ages of 6-8
  • Only 10% were 13 and older and 10% were 2 and under
  • 75% of patients are 8 and under
Age at Diagnosis

Muscle-Related Symptoms Prior to Diagnosis

  • Top Response:
    70% complained of being tired more often
  • More than 50% of patients also had:
    • Difficulty climbing stairs
    • Loss of ability to do physical abilities, i.e. bike riding, running, soccer
    • Fatigue before normal and/or after physical activities
    • Complaints of aches/pains
    • Difficulty in keeping up with friends at school and in sports
  • More than 40% of patients:
    • Asked to be carried more often
    • Difficulty walking
    • Fell more frequently
  • 26% had stomach discomfort and pain

Skin-Related Symptoms Prior to Diagnosis

  • Top 2 Responses:
    68% reported rash on cheeks and/or bridge of nose
    56% reported red or purple coloring on eyelids
  • More than 50% of patients cited:
    • Overall redness on hands (joints, knuckles, nail beds)
    • Redness and/or bumps on knees
    • Redness and/or bumps on elbows
  • More than 40% reported:
    • Redness and/or bumps on knuckles
    • Red/swollen fingernails/cuticles
    • Gottron’s papules

General Symptoms Prior to Diagnosis

  • 84% reported fatigue
  • 48%: sleeping more often
  • 40%: low-grade fevers
  • 35%: weight loss
  • 25%: difficulty with bowel movements
  • 25%: contractures
  • 10%: lung issues
  • 7%: lipodystrophy
  • 6%:  heart issues

Types of Doctors Consulted Prior to Diagnosis

  • 81% consulted with their pediatrician
  • 47% consulted with their dermatologist

Tests Used Prior To/During Diagnosis

  • 97% bloodwork
  • 65% MRI
  • 63% strength testing
  • 33% muscle biopsy
  • 27% skin biopsy

Number of Times Patient Consulted a Doctor Prior to Referral to Specialist and/or Diagnosis

  • 33% of patients saw 2-3 doctors prior to diagnosis or referral
  • 29% of patients saw 6 or more doctors prior to diagnosis or referral
Doctor visits before diagnosis or referral

Type of Doctor who Ultimately Made the Diagnosis

  • Rheumatologists made the diagnosis in 60% of the cases.
  • Dermatologists made the diagnosis in 16% of the cases.
  • Pediatricians made the diagnosis in 7% of the cases.

Did Patient Have Infection or Virus Within 3 months of Onset of Symptoms?

  • 49% of patients had an infection or a virus within 3 months of the onset of skin rash or muscle weakness.

Photos

  • 69% of patients are willing to provide photos of signs and symptoms.

Month of First Symptom

  • The incidence in every month is between 6% and 12%.  
    • Highest month is June at 12% and lowest month is July at 6%.
    • The last 3 months of the year have the lowest incidence, aside from July.

Month of Diagnosis

  • The month of diagnosis ranges between 6% (May) and 11% (October).

Length of Time Between Patient’s First Symptom and Diagnosis

  • 27% were diagnosed between 1-3 months
  • 27% were diagnosed between 4-6 months

Is the Patient in Remission (two years with no symptoms/no medications)?

  • 78% of patients are NOT in remission
In remission

If Patient Went Into Remission, Did the Patient Have Any Relapses?

  • 21% had relapses
  • 23% did not have relapses
  • 56% not applicable

Takeaway from this survey:

  • ¼ of patients experience full remission
  • ¼ of patients chronic with at least one remission
  • ½ of patients chronic without remission
  • Overall, ¾ of patients have chronic disease
    (this is similar to Norwegian study that showed 61% of JDM patients had chronic disease after 16.8 years)

Medications Prescribed at Time of Diagnosis

  • Top 3: 
    89% oral prednisone
    62% methotrexate injections
    58% IV solumedrol/prednisone

Most Important Outcomes Desired

  • Top 3:
    84% a cure
    58% a “normal” lifestyle
    20% better treatments

Types of Misdiagnoses Received Prior to JM Diagnosis

  • Eczema 46%
  • “Nothing is wrong” 32%
  • Allergy 26%
  • Flu or other virus 20%
  • Psoriasis 13%
  • Fifth’s Disease 11%
  • Muscular Dystrophy 5%

The very FIRST symptom for the majority of patients

  1. Facial rash
  2. Non-specific rash
  3. Pain
  4. Fatigue/tiredness
  5. Rash on fingers, hands, knees, and/or elbows

First Symptom

The most DEBILITATING symptom for the majority of patients

  1. Muscle weakness
  2. Pain
  3. Fatigue/tiredness
  4. Skin involvement/rash
  5. Falling/difficulty walking

Most Debilitating Symptom

Geographic Area Where Child First Developed Symptoms

Top 5 States in the U.S.

  1. California
  2. Texas
  3. Illinois
  4. Florida
  5. New York
If in US, State residency at time of diagnosis

Top 5 Countries outside of the U.S.

  1. Canada
  2. United Kingdom
  3. Australia
  4. New Zealand
  5. South Africa

If outside of US, Country of Residency at time of diagnosis

Complete Survey Results and Graphs in PDF format