Magazine article The Exceptional Parent

Strength in the Face of Adversity

Magazine article The Exceptional Parent

Strength in the Face of Adversity

Article excerpt

Last month, we introduced our readers to the Crowley family from Pennington, New Jersey, our newest inductees into the "EP Hall of Fame." Parents John and Aileen spoke to his about caring for their three children, John Jr., 5, Megan, 3, and Patrick, 18 months. Megan and Patrick both have Pompe's disease, a rare muscular disorder.

Pompe's disease is caused by a genetic defect that prevents the body from producing a crucial enzyme called acid maltase. In healthy individuals, acid maltase breaks down sugars that are stored in the body's muscle cells. In Pompe's disease, the absence or insufficiency of acid maltase forces the body to continually store sugar in the muscle cells in the form of glycogen. Glycogen storage rapidly causes the muscle cells to deteriorate. This results in progressive, weakening and deterioration of the muscles. Glycogen also accumulates in the liver, muscles, nerves, and heart, preventing them from functioning properly.

The demands of the disorder have invaded every, aspect of the family's life and prompted their efforts to raise fund for research into the disease. It has forced them to be their own advocates when dealing with their insurance carrier. This is the second part of our interview with the Crowley family, as they relate their experience in their own words.


The level of care that Megan and Patrick need every day, around the clock is extremely costly. John and Aileen have managed to convert the insurance companies from adversaries into partners, by making them see the human side of Megan and Patrick's illness.

JOHN CROWLEY: The care for Megan and Patrick at home and the 24-hour nursing care alone is a quarter of a million dollars a year. Beyond that are the equipment, visits to the doctors, and hospital stays. Fortunately we have not had many of those this year. All together, Megan and Patrick's medical care runs close to $500,000 per year. We are very fortunate in working for Bristol Meyers Squibb. It is a company that is dedicated not only to its customers but also to its employees in its mission of extending and enhancing human life. They have helped tremendously with the financial burden of this.

The insurance company, United Healthcare, has been great, too. But this has required many discussions.

When we first took Megan and Patrick home, the insurance company told us we will get 24-hour nursing care for a couple of weeks, then 16 hours, then they said they believe that having a child on a ventilator is a home care skill they could teach us and then we won't have nursing care. I just said from day one that is absolutely not possible, not acceptable, and fought very hard. I think they realized the uniqueness of this situation and the unique needs, and since that time have been very supportive. For example, the nurses that United Healthcare originally provided through their agency were uniformly horrible. We fired about 22 of them for gross incompetence.

After a couple of months I just said stop. We can find better nursing care with the money you are giving these agencies.

The insurance company was very flexible in working out an agreement with us to allow us to directly hire the nurses and to pay them directly.

AILEEN CROWLEY: There is no middleman getting a cut and therefore we have excellent nurses.

JOHN: As a result, we interview and choose our own nurses ourselves.

John and Aileen have been their own advocates with their insurance carrier.

JOHN: When they (the insurance carrier) wouldn't listen, I went to our HR (human resources) people and our benefits people and told them the stow. You have to put a very human side to it and try to explain to them in very layman's terms what it's like living in the house without nursing care, and it's impossible. The alternative is to institutionalize the child, and that's something that is completely, as a lifestyle, completely unacceptable to us. …

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