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Strength in the Face of Adversity.

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.

It also did not make economic sense for them. So you have to make arguments on a number of different grounds and realize that at the end of the day, these are people with kids, too.

AILEEN: They have their certain rules that are outrageous. When Patrick came home from the hospital, they said they would pay for two nurses in the home at a lesser price. I said I don't want two nurses at a cheap price, I want one nurse that we can pay good money to. They said we don't do that. That doesn't make any sense, they were going to pay double, pay for two nurses, when they could have paid for one nurse.

JOHN: Once it was explained to them, they understood and they became very flexible and very supportive. Without that support I don't know what we would be able to do. There are other programs, there are state programs, the state catastrophic medical insurance fund, Medicaid, but fortunately we have been able to navigate through the private insurance right now.

We probably have $30,000 a year unreimbursed, uncovered medical expenses.


The Crowley household benefits from the services of two live-in caregivers, Sharon (a nurse) and Ann (an au pair), who live downstairs in the Crowley's house. They are always available to provide whatever help is needed.

AILEEN: Sharon is wonderful with the children. She also deals with the surgical supply company, and she is tough, she is on their case if they are not here.

She always has been a travelling nurse. She and Ann have become good friends. It's hard having strangers in your house all the time. It's so rare that John and I are ever alone. They give us our space and they get their own space downstairs.

It's hard to have non-family members living in your house, but they are very respectful as we are of their privacy, too. Downstairs is theirs pretty much, they have their own TV and couch and refrigerator and stuff. Sharon needs her time away, too; she puts in 12-14 hour days with kids every day that she is here. They have become part of the family. The kids are very excited about Ann's birthday.

We lucked out with Ann, so many people have stories with au pairs and how things just don't work out. She wanted to learn how to take care of Megan and would take shifts at the hospital. Megan was in the hospital for six weeks first, and she and Patrick were in the hospital last March for 3-4 weeks. We ail took shifts. Ann took the night shift some nights. She did more than most family members.


JOHN: People have different ways of coping, different ways of taking care of their children; different ways of dealing with it. There is no one right way of doing it.

We were determined to fight the disease and I guess my recommendation for parents in similar situations is to be as curious and as challenging as you can, always respectful, but curious and challenging.

The doctor in California, the pediatric neurologist who diagnosed Megan, told us that day he did not know of any research going on. It was an extremely rare disease and, according to a footnote in his medical textbook, Megan had about two months to live. I didn't know anything about rare genetic diseases. I knew there were hundreds of thousands of them. I didn't know how they got cured, how they got treated, who works on them.

Then we found out very quickly on the Internet, first of all, that there were doctors working on it. So my first advice is to find out who the top doctors are, who are writing the papers on these diseases, which have done PhD theses on them. Then contact the doctors, see where they are in their research, see where their funding levels are. I had always assumed that if the researcher thought he had the cure to it, or was close to a cure, that he would find research money--from industry, government, families, foundations, somewhere. I found three or four doctors working on Pompe's disease who had virtually no funding at all.

It was very frustrating. You are hopeful, knowing that there are doctors working on a cure for Pompe's disease. But because there wasn't enough funding, your child isn't going to have access to it, and it may never be developed, or not for a long time. That was something that we are determined to change. For parents in that situation, I would say never give up, keep driving, always challenge, and always learn. You will find that doctors and researchers will welcome your support, even just to tell you and teach you about their research.


JOHN: The past year, we started our fundraising in November of 1998. Altogether we have raised about a million and a half-dollars in a little over a year. It started with a letter-writing campaign to friends and family. When they know about it, they'll tell ten friends, and they will tell ten friends. The media exposure has been helpful, too.

AILEEN: We get lots of encouraging letters, too, which is nice.

JOHN: Some letters are from people who can sympathize or empathize, because they've had a child who died. We've gotten letters from people who've read the story in national magazines and they will tell us that they had a brother who died of Pompe's disease in 1960. Their dream is that this gets cured, and they send $50 in his memory. Or we hear from parents of children who have disabilities or disorders who can empathize with us. Then we will get letters from people who are thankful who say, they have beautiful, healthy grandchildren, and couldn't imagine what we are going through. I thank God that we are healthy and we are sorry your family isn't and here's what we can do to help.

AILEEN: It's amazing. One Sunday school Nursery School all wrote letters. It was nice.

JOHN: They had a bake sale, it was a preschool, and they raised about $90, in fact I have to tell you because after they raised $97.10. They each sent a letter; we had no idea that this was going on.

One of the 5-year-olds wrote in the letter, Dear Mr. and Mrs. Crowley, I am sorry to hear about Megan and Patrick, I hope they get better soon. We tried to raise a lot of money for you, but we didn't do really good, we only got 97 bucks. She signed it, and the teacher made her put a line through the part that said we really didn't do really good, we only raised 97 bucks. From the mouths of babes. I've kept all those letters.

AILEEN: I want Patrick and Megan to read them.

JOHN: Megan and Patrick better be pretty good kids when they grow up. I'll say to them, "You want to stay out how late? Let me tell you about what I did for you when you were a kid ..."

For more information, contact the Children's Pompe Foundation: The Children's Pompe Foundation 66 Witherspoon Street, Suite 341 Princeton, NJ 08542 Telephone: (609) 924-7192. E-mail: Web site:
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Title Annotation:part 2
Publication:The Exceptional Parent
Geographic Code:1USA
Date:Mar 1, 2000
Previous Article:Sites from the Heart Special Needs Advocates Share Their Caring Online.
Next Article:Can We Talk?

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