One Family’s Struggle with Danon Disease, a Rare Disorder Affecting a Mother and Both of Her Two Young Sons

In a story published in The Hernando Sun, Dawn Dybiec, age 31, describes her disbelief when she and her husband were told that her two sons, Gabriel and Steven, were diagnosed with Danon disease, a rare genetic disorder. Dawn and her husband were trying to work through the process of accepting this devastating news when they were told that Dawn was not only the carrier, but had symptoms of the disease herself.

Dawn’s 34-year-old husband, Steven, may not be affected physically with the disease, yet he is struggling emotionally as he witnesses the suffering of his wife and children.

Danon Disease: A Rare Genetic Disorder

One of the key characteristics of Danon disease is the weakening of the heart muscles (cardiomyopathy) causing the heart muscle to become thicker and more rigid than normal. Skeletal myopathy (weakening of the muscles used for movement) occurs less frequently and affects the upper arms, back, shoulders, neck, and thighs.

Other symptoms may include problems breathing, vision abnormalities, and gastrointestinal disease.

Signs relating to the heart may include chest palpitations, an abnormal heartbeat (arrhythmia), or chest pain.

Males seem to be affected more severely than females and tend to develop symptoms earlier. The disease is not well known among physicians and easily misdiagnosed. Additional information about the disease may be found here.

The Discovery

The first signs of the illness began with Gabriel’s stomach pains, then both boys developed severe leg pains causing them to wake up screaming at night.

Suddenly their son, Steven, had to be admitted to the hospital, as his heart rate was 298 beats per minute. He was immediately immersed in ice water which shocked his body and brought his heart rate down. The doctors performed heart surgery. They told his parents that if he had been middle-aged, he would not have survived.

This ordeal was followed by six months of genetic testing involving each strand of DNA. The doctors discovered that both boys, as well as Dawn, had the same condition. She had a pacemaker implanted in March of 2016.

Both boys will require a heart transplant in the very near future. The disease has prevented them from playing sports or just being able to play with their friends.

Their daily life involves therapy three times a week, counseling and regular doctor visits.  Gabriel has been most affected emotionally as he pleads with his mother not to have the heart transplant, to have the doctors “fix Danon another way” because he does not feel that she will survive.

As there is no known cure, the transplants can only prolong their lives.

Dawn’s husband Steven says “I just push myself through it. I have to work [to support my family], be strong for everybody and take care of the family.”

Dawn adds “I know I have to be strong because I have to show the boys that it’ll be okay. It will be worth it in the end because they’ll still have me. I have faith. It’s in God’s hands.”

The Family’s Financial Struggle

Dawn’s condition is critical.  Her heart is functioning at only five percent. Although she is “on the list” for a heart transplant, she is scheduled to receive an Implantable Cardioverter Defibrillator on  January 31st while she waits for a heart donor.

In addition to the obstacles Dawn has had to overcome just to get on the heart transplant list, an even greater obstacle is their very serious financial situation.

The heart transplant will cost anywhere between $1.2 to $1.4 million and Steven’s health insurance will cover only 70%. The Dybiecs also have to prove they are capable of affording the anti-rejection medications at a cost of approximately $22,000 per year.

Then there are the medical expenses that have been adding up due to the numerous hospital stays for Dawn and the two boys. Dawn has had a pacemaker implanted and currently, in addition to various other medications, there are weekly charges of $150 for medication that has to be administered via intravenous infusion into her heart 24/7.

Steven works 60 hours a week as a manager for a building supply company and has taken over household tasks due to Dawn’s illness.

Dawn’s mother has set up a GoFundMe account to help defray some of the costs.  

Dawn’s mother explained that “This is Dawn’s life we are talking about. I have never had to beg for help before but I have to now. The boys need their mommy, I need my daughter, and Steven needs his wife.”

If you would like to contribute, you can find the crowdfunding campaign here: https://www.gofundme.com/6ie19bs

Another fundraiser is being spearheaded by Shirley Lowe, a friend, and co-worker of Steven’s. On March 16, a drag show featuring Divas and Dudes will be held at the Heather House (the community building) at Clover Leaf Farms Mobile Home Park in Brooksville. Doors open at 6:30 pm and the show starts at 7:00 pm. Tickets are $15 and are expected to sell out fast. They can be purchased at the door. However, for a guaranteed seat, purchase in advance by calling Shirley at 978-273-9736 or Phillip at 904-273-9736. Credit card payments are accepted. You can also email lowe22@gmail.com.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia four years ago. He was treated with a methylating agent While he was being treated with a hypomethylating agent, Rose researched investigational drugs being developed to treat relapsed/refractory AML.

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