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Bridging the Challenges
Marilyn S. Hair
abc
a
Wisconsin Conference, United Methodist Church, USA
b
International Fibrodysplasia Ossificans
Progressiva Association, USA
c
Carroll College, Yale Divinity School (MDiv 1977), Waukesha, WI, USA
To cite this Article Hair, Marilyn S.(2000) 'Bridging the Challenges', Journal of Religion, Disability & Health, 3: 3, 5 23
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Bridging the Challenges:
The Effects of FOP on the Family
Marilyn S. Hair, MDiv
ABSTRACT. A personal account of family life with a disabled child.
The author describes her experience of the grief process following
diagnosis, the particular challenges associated with the rare orthopaedic
condition, fibrodysplasia ossificans progressiva (FOP), and the effects
on the family when one member has extensive needs. She speaks to the
value of a support group and summarizes with a list of ten things she
has learned as the mother of a disabled child. [Article copies available for a
fee from The Haworth Document Delivery Service: 1-800-342-9678. E-mail
address: getinfo@haworthpressinc.com <Website: http://www.haworthpressinc.
com>]
KEYWORDS. Bone, coping, craniopharyngioma, family, FOP, ortho-
paedic, Sarah
FIBRODYSPLASIA OSSIFICANS PROGRESSIVA
FOP is the acronym for a rare disease called Fibrodysplasia Ossifi-
cans Progressiva. Its a mouthful that came uninvited into my vocabu-
lary when it was written as a diagnosis into the hospital chart of my
daughter, Sarah, in April, 1985.
Marilyn S. Hair is a clergy member of the Wisconsin Conference of the United
Methodist Church and a member of the Board of Directors of the International
Fibrodysplasia Ossificans Progressiva Association. She is a graduate of Carroll
College, Waukesha, WI (BA 1973) and Yale Divinity School (MDiv 1977). She and
her daughter, Sarah Steele, are the authors of What is FOP? Questions and Answers
for the Children (Casselberry, FL: International FOP Association, 1995). She lives
with her family in Redmond, WA.
Journal of Religion, Disability & Health, Vol. 3(3) 1999
E1999 by The Haworth Press, Inc. All rights reserved. 5
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6 JOURNAL OF RELIGION, DISABILITY & HEALTH
FOP is a progressive musculoskeletal disorder. It is characterized
by normal bone that grows in abnormal locations. An aberrant gene
instructs the body to grow bone in the skeletal muscles, tendons and
ligaments of the body. Gradually a second skeleton develops. As the
bone replaces tendons and bridges joints, the person with FOP be-
comes gradually frozen in position. A particularly chilling article I
read soon after Sarah was diagnosed describes FOP as stone man
deformity.
1
The joints become fixed in whatever position the patho-
logical bone forces them, sometimes in comfortable and functional
positions, but often in useless, awkward and painful poses.
The second skeleton is not manifest at birth. A typical distinguish-
ing feature of FOP which is present at birth is short and characteristi-
cally misshapen great toes. FOP progresses along four well-defined
gradients: top to bottom, back to front, spine to limbs, and young to
old. It does its damage by fits and starts. Although the timetable varies
widely, the advance is relentless. Usually by age five, warm painful
swellings or lesions develop in the back and neck. The swellings
recede but develop into cartilage, then mature bone. Spine, neck and
shoulders are affected first. Hips are typically affected in the third
decade of life, eventually necessitating full-time use of a wheelchair.
Efforts to remove FOP bone surgically result quickly in re-growth of
the bone.
FOP is an autosomal dominant genetic condition. This means that a
person who carries the gene manifests the condition. Someone who
has FOP has a 50% chance of transmitting it to his or her child. Most
people who have FOP develop it as the result of a spontaneous muta-
tion in the DNA.
2
Based on the population of Great Britain and the
number of people there who have been diagnosed with FOP, it is
believed that FOP affects one out of every 2 million persons. Approxi-
mately 600 patients have been described in the worlds literature since
1792 and currently there are fewer than 200 individuals known to have
this disease.
3
I am unspeakably grateful to Dr. Frederick Kaplan
4
at
the University of Pennsylvania and the other doctors who have spe-
cialized in understanding FOP and who have examined many FOP
patients. Sarah and I have traveled to Philadelphia several times so that
she could be examined by the experts.
Now that I have introduced FOP, let me introduce myself. Profes-
sionally, I am an ordained United Methodist minister. I pastored con-
gregations for 17 years before Sarahs health took a nosedive. I am
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Marilyn S. Hair 7
currently appointed to Family Leave. I have been married for 20 years.
My husband and I are the parents of three children, Sarah, Jonathan
and Mollie, ages fourteen, eleven and eight.
Of the several roles in life that I play--clergywoman, wife, mother--I
am best known by far as Sarahs mother. I once telephoned a parent
from Mollies preschool and tried to tell her it was Mollies mother
calling. I couldnt get it out. I said, I am Sarahs mother. I am
always Sarahs mother, because Sarah has FOP. I will begin by telling
my familys story. Then I will address some of the issues that I see
facing FOP families, as well as other families who live with chronic
illness and disability.
5
THE GRIEF OF THE DIAGNOSIS
My husband and I were both 32 years old when Sarah was born. We
had been married for six years and she was our first child. Sarah had
symptoms of FOP at birth. Her great toes were misshapen. She had a
lesion on her spine. In the first weeks of her life, golf-ball shaped
lesions swelled up on Sarahs head, then disappeared. The back of her
head grew into a dull pointed shape. Her pediatrician examined Sarah
several times but was not immediately concerned because the symp-
toms kept disappearing and, from a cognitive perspective, Sarah was
developing normally. However, when Sarah was four months old and
new lesions kept appearing, the pediatrician referred her to a neurolo-
gist. The specialist observed immediately that Sarah held her head
towards the left. He palpated a hard mass on her neck and sent Sarah
and me to the Childrens Hospital. The neurologist thought she had
cancer. This is a typical first impression of symptoms which are even-
tually recognized as FOP.
We were extremely fortunate that after having bloodwork and x-
rays, an alert radiologist identified Sarahs symptoms as early man-
ifestations of FOP. After just 24 hours in the hospital, we were sent
home, first-time parents with a baby who was more fragile than ever,
an unpronounceable, catastrophic diagnosis, a little bit of information,
and hardly a suggestion for treatment. As one parent put it, the diagno-
sis was an eight on the Richter Scale.
6
One of my first thoughts was, Now our family is going to be
screwed up! My husband said, No more children! as if he thought
our entrance into parenthood had been a mistake from the start. For
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several weeks my grief was manifest by physical symptoms: I had a
pit in my stomach, nausea and no appetite. FOP made me sick.
We learned a new vocabulary: birth defect, orthopaedic, heterotop-
ic, endochondral. We read Dr. J.M. Conners 1982 article about 34
FOP patients from Great Britain.
7
We made a list of the medical terms
we didnt know, carried it to the pediatrician, and made him explain
what all the big words meant. We were told we were lucky to have a
diagnosis so quickly, that a biopsy had not been done,
8
and that the
only trauma was venepuncture. We did not feel very lucky, but as time
went on, we heard other FOP families tell stories about spending years
not knowing what was wrong, being misdiagnosed, and receiving
inappropriate medical treatments.
9
Only then did we understand the
worry and uncertainty we had been spared.
The doctors said that anything we could do to prevent trauma would
be good. In the face of untreatable, incurable disease, I was desperate
to do something. My cause became preventing Sarah from being
bumped. I sewed pillows, determined to pad everything that Sarah
would touch. I bumper-padded her crib, inside and out, made pillows
for the back of her high chair and the baby gate, even custom-pillowed
the piano bench. Our house was like a padded cell, cushioned within
an inch of its life. I did it because I could not sit there passively and
watch the disease progress. Futile as it turned out to be, I did the only
thing I could think of to protect her. I had to combat my sense of utter
helplessness, because I could not make FOP go away.
It was six years before we found anyone else with FOP. In 1991,
one of Sarahs doctors contacted the National Institutes of Health.
Through the NIH we were put in contact with an organization that had
formed in 1988, the International FOP Association. To find other
people who have and know about this unusual condition, and to dis-
cover that even though FOP is rare, it isnt an orphan disease, changed
our lives. We were no longer alone. When Sarah was diagnosed, the
geneticist told us to take her home and love her. There is no treatment
and this disease is so rare that no one understands it and no one is
studying it. But that is not true now. A number of eminent doctors
and scientists are seeing patients and doing research into FOP.
When Sarah was eight years old, in 1993, she was found to have a
congenital brain tumor. It was a benign pituitary tumor called a cranio-
pharyngioma, among the rarest of all pediatric brain tumors. This
second rare disease had been present since birth and was now large
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Marilyn S. Hair 9
enough to cause symptoms. Sarah, her Dad and I traveled to Chil-
drens Hospital of Philadelphia where the tumor and her pituitary
gland were removed. Her hormones are now medicinally supplement-
ed and she is under the care of an endocrinologist. In the history of
medical literature, no one with FOP has been known to have had a
craniopharyngioma. The chance of these two rare conditions coinci-
ding in one individual has been calculated at less than one in 100
billion,
10
which is more people than have ever been alive. One would
expect it never to have happened at all.
When Sarah was diagnosed with FOP, my husband and I were
plunged into grief. It is normal to grieve over losses ranging from the
death of a family member to disappointment at being passed over for a
job promotion. When we learned that Sarah had FOP, Sarahs Dad and
I lost the child we were expecting, the perfect child we were wait-
ing for, hoping for and dreaming about. Working through our grief
took a long time and was emotionally exhausting; but we had to
endure it. We had to let ourselves hurt over what we had lost and let go
of what we expected before we could accept what we had.
Twenty-five years ago, Elisabeth Kbler-Ross wrote her well-
known book, On Death and Dying.
11
I used Kbler-Ross stages as
markers to evaluate how I was adjusting to my childs diagnosis. I
tried to deny the situation when we first learned that Sarah had FOP. I
would think: There must be some mistake. The records got mixed up.
The doctors thought they were talking to somebody else. This is a
nightmare. Soon I will wake up and find out it is not true. In the
movie Dead Poets Society, a prep school student shoots himself.
When his parents find him, his mother screams, Hes all right! Hes
all right! Denial is instinctive, like raising your arms in front of your
face to fend off a blow.
Denial soon gave way to anger. I wondered, Why me? Why our
family? Why should this happen to a child? Why cant God take it
away? Why does there have to be suffering at all? But I could not
feel angry for long before I turned the anger inward and felt sad and
guilty. I questioned my every action and conviction: Did I step off
the path somehow, stray from the straight and narrow? Is there some
hidden rule to the moral order of the universe that I have unwittingly
transgressed, such that Sarahs having FOP is the punishment? What
did I do wrong to deserve something like this? It is not fair! I was
mad at the doctors who gave us such terrible news. I was mad at life,
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which had tricked me--white, middle-class American that I am--into
believing that if I followed the rules I would get smarter and more
successful. I was mad at God who had not protected me from this
catastrophe.
I also bargained. We should re-do the tests. Lets get a second
opinion. Maybe another doctor will see it differently. If Im really,
really careful--if I sew pillows for every surface in the house--maybe I
can get control over this and she wont get any more lesions. If I am
super-attentive and super-careful, maybe the FOP will stop. I was
haunted by the if onlies. If only I had gotten pregnant at a different
time. If only different cells had come together to form her, we could
have avoided that bum germ cell and she would not have FOP. I
imagined having married somebody else so maybe conception would
not have led to FOP. I rehearsed my life script over and over again,
searching for some different path which might have avoided this horror.
Friends told me to bargain with God. Shortly after Sarah was diag-
nosed, one of the women who belonged to the church I pastored told
me, Pray! God can take this away! Prayer has helped me tremen-
dously in the years since FOP came into our lives. But I sensed that to
ask God to take away Sarahs FOP would only leave me stuck in the
denial stage of my grief. To pray that her FOP would disappear would
be to deny that it was real, and would prevent me from accepting it. In
the short months before Sarah was diagnosed, as I sat up in the wee
hours of the night to nurse her, I would kiss her lesions. I didnt have a
clue about what they were, and in those dark hours I believed that
Sarah would die soon. But the lumps were part of her, and I felt that I
had to learn to love them if I was going to love Sarah. I felt that the
way for me to be whole, and to be the mother Sarah was going to need,
was to work through my grief and learn to accept everything about her,
including FOP.
About this time it occurred to my husband and me that since FOP is
genetic, it is part of every cell in Sarahs body. To wish that Sarah
didnt have FOP was like wishing she was a different person, not
Sarah, and we did not wish that. FOP is completely intertwined with
Sarah, and for us to have Sarah has to mean having Sarah with FOP.
That insight helped us move toward accepting her condition.
There was also depression. Oh my gosh, I thought, This is for
real. This is not going to go away. It is going to get worse and go on
forever. I wondered, Is there something wrong with me, or my
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Marilyn S. Hair 11
husband, or our marriage, that we had a child with FOP? Is something
wrong with our family now? Guilt stalked me: Did I do something
to cause this? Even if it was not on purpose, even if I did not know I
was doing it, did I do something wrong? Was I unknowingly exposed
to some toxin? Did I transgress some physiological or moral law I did
not know existed? Was there anything I could have done to prevent
it? I grasped at straws trying to make sense of this turn of events in
my life. I lay awake at night tortured by obsessive thoughts: Have I
done anything to hurt her, or failed to prevent someone else from
hurting her? Should I have known that the tests the doctors were doing
would be harmful? There has to be some reason this has happened,
because if there is no rhyme or reason, the way I have always under-
stood life to work has been turned inside out.
But finally, acceptance came. This is not to say that these stages
kept themselves in neat order, 1-2-3-4-5, then my grieving was done.
If only grief were like a game of golf, and I could have pushed onto
the 18th hole and been done with it. I have been from depression to
anger, and from bargaining back to denial. Old memories and new
events have sent me into different stages of grief. But as I let myself
feel the sadness and the pain, there have been moments when I think I
can see light at the end of the tunnel.
When Sarah was newly diagnosed we had friends who invited us
for dinner and told us what had happened to them. Fifteen years
before, their first child, healthy five-year-old Philip, caught himself on
fire on the gas stove, burned out his lungs, and died. Our friends
served as examples of people who had borne unspeakable pain, and
survived. They said things that evening I will never forget. This is so
awful, they said, you would never wish it on anyone else. I wasnt
sure I wouldnt have given FOP to somebody else if I could have.
Terrible things happen to other people every day. Their words chal-
lenged me to buck up and learn to carry the small share of the worlds
pain that had been dealt to me.
I asked the woman how long it had taken before she felt normal
again. It was a year. One day she noticed that she had not thought
about Philip once all day. I thought to myself: If it took a year for her
to begin to recover from the death of a child, and my child has not
died, then maybe it would take less time for me to recover from this
catastrophic diagnosis. I had the expectation that within a year, things
would get better. And that is what happened. After six months, I felt a
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small surge of hope. I could think about something besides FOP. I was
beginning to learn that Sarahs FOP was only one part of our family
life.
Acceptance, for me, means realizing both intellectually and emotion-
ally that this situation is for real. It is not going to go away, and I can
place it in perspective alongside other factors in life. When I take Sarah
to the doctor, I almost never cry now. I can look at a swelling that I
know is going to turn to bone and further restrict the movement of her
joint forever, and not get broken up about it. It is as if I have developed
calluses so that truly awful things do not hurt the way they used to.
Fourteen years into this experience, I could postulate some answers
to the questions, Why? Why me? Why is there suffering? But thats
not really the point. As I have lived with my grief, gradually the
questions have become less compelling. I have reconstructed my
theory about the way life works: Having a disability is not a punish-
ment. No one did anything wrong. That Sarah has FOP is an accident.
Sometimes bad thing just happen. My husband said that God hurt, too.
He said, God has a tear in his eye for Sarah. Everyone who faces
loss must wrestle with these questions. Each of us has to come to terms
with what suffering means.
PROGRESSIVE, RARE AND CHRONIC
When it comes to FOP, these three words, progressive, rare and
chronic, are profanity. The P word is progressive. Fibrodysplasia
ossificans progressiva is three long words, and progressiva is the
most awful. We wait for FOP to get worse. Progressive means there is
going to be another site of involvement, a new physical limitation, a
further challenge to mobility. Progressive means that each frozen joint
is here to stay, that every new flare-up will add to the physical limita-
tion that was already there. I stopped taking Sarah to quarterly physi-
cal therapy appointments because the only treatment the therapist
provided was to take range of motion measurements, and every time
we went, Sarahs range was less.
When Sarah was a toddler, some of the effects of FOP I had read
about but never seen made me sick with worry. I agonized especially
about hip and jaw involvement. Sarahs right hip joint froze in 1991
when she was six years old, and she began to walk with a limp. I
prayed that God would hold back the FOP bone from her other hip. I
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Marilyn S. Hair 13
prayed not for a miracle, but for time, for Sarah to walk her way into
adulthood. We didnt realize FOP bone had begun to grow in her left
hip. Dr. Kaplan examined Sarah in 1993 and observed that the patho-
logical bone extending downward from her pelvis had nearly attached
to her left femur. Her second hip became fixed in place after neurosur-
gery when she was nine years old. That was a major set-back, and it
complicated Sarahs life and our family life when she couldnt walk.
But I was shocked to discover that I was also relieved. The progressive
part of FOP had done its damage. All of Sarahs major joints were now
affected. There was nothing left for me to fear. What I imagined would
be the worst part of FOP happened, and we are still coping.
The R word is rare. It is an added burden to have a rare
disease, a disease that our friends, family, teachers and even doctors
have never heard of. Sarah is the only patient with FOP most of her
local doctors ever see. I am constantly explaining what it is, not only
to family and neighbors and schools, but to medical people. This
means that well-meaning doctors who do not understand some of the
unusual features of FOP can hurt my child by doing routine medical
procedures. I have to know the implications for FOP of every sugges-
tion and procedure. I feel responsible to make sure, within the limits of
medical knowledge, that the procedures which Sarahs doctors, thera-
pists and medical equipment providers do for her will help relieve the
effects of FOP, not make it worse.
The C word is chronic. There is particular stress to having a
chronic disease. The demands, the difficulty and the pain never let up.
They consume us all day and worse, all night, every day of the year.
There is no respite. It has been suggested that in some ways chronic
illness is harder even than terminal illness, because chronic illness
goes on relentlessly. It wears us all down; the person who suffers from
it, and the caretakers as well.
ON BEING DIFFERENT
One Sunday afternoon our family took a long walk to the duck
pond. Then five-year-old Sarah rode in a borrowed wheelchair. Jona-
than, age two, walked or was carried. At the duck pond we tossed stale
bread and started to walk home. The sun was hot, we were tired, and
far from home. We had overextended ourselves and it wasnt the first
time. Jonathan was too tired to walk and my husband and I were both
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too worn out to carry him. We were thirsty and began to imagine
ringing strangers doorbells, telling them we were dying of thirst. We
piled Jonathan onto Sarahs lap and staggered home wondering, How
do we get ourselves into these things? That day we adopted our
nickname; the Nerd Family.
We all use this name for our family. We have a child with an unusual
body shape, a power wheelchair, and an assistance dog. She has two
active siblings. The Nerd Family names the fact that we stand out in
a crowd. My husband says, I just hate being on parade all the time.
Having a sense of humor helps make an awkward situation more
bearable. But it does not change the fact that my family is different.
People turn their heads to watch us. We spend our time and money on
atypical things. We have unusual worries and concerns compared to
most families. Because we spend our efforts disproportionately on one
child, our family life can feel skewed, not fair to the other members of
the family, and out of step with the rest of the world.
I have found that there is risk to the well-being of the family when
one member has greater needs for a long time. There may be love
enough for everyone, but time, energy and money have limits. Sarahs
lengthy bedtime routine leaves me exhausted. When Mollie was two,
by the time I had put Sarah to bed I had no energy to help a recalcitrant
toddler settle down for the night. Jonathan, our middle child, com-
plains that he does not get enough attention. Another family refers to
the next-older sister of their child who has FOP as their neglected
child. After listening to how I try to make it up to my other children,
a social worker warned me that there is no way I can shelter her
siblings from the effects of Sarahs FOP.
I have noticed as Sarah has grown older and her FOP has pro-
gressed that she has developed an attitude we call the Princess Syn-
drome. Sarah expects to be waited on. A great deal of our familys
effort is focused on Sarah. When we bought our home, we chose it to
meet her needs for accessibility. She sleeps in the master bedroom, a
concession to her convenience that was hard for me to make. We have
spent a great deal of money on things a person would not choose to
buy: Wheelchair, ramps, van with wheelchair lift, roll-in shower,
shower chair and barrier-free lift. Sarah has a full-time aide at school,
and I have taken leave of my vocation to be with her at home. I put the
tray table on her wheelchair and hand her what she asks for. I wash
her, dress her, groom her, and get up at night to scratch her itches, roll
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Marilyn S. Hair 15
her to a new position, and administer medications. Yet much of the
time, her response to my devotion is to expect this kind of service. She
quickly gets frustrated if I dont drop everything when she calls. It is a
sad thing that she requires so much help and it makes me feel guilty
for resenting someone who is truly needy. But Sarahs expectations are
a burden for the other members of the family. Her dependency takes a
toll on all of us.
Each family member has the right to have their needs met. To make
Sarah the sole focus of our family life makes her too powerful and that
is unfair to her and to everyone else. One way we to try to keep our
family healthy emotionally is to give each person permission to talk
about their feelings. We try to let our younger children complain about
Sarah (out of Sarahs hearing) and about the restrictions FOP puts on
our family, without being made to feel guilty.
It has been observed that parents often have a harder time dealing
with FOP than the FOP patients themselves. As Sharon Kantanie has
written, Having lived with FOP for most of my twenty-five years, I
would have a hard time envisioning life without FOP. It is a part of
who I am: not a part that I would purposefully choose to have, but a
part of me nonetheless.
12
The person who has FOP knows no other
way. But my husband and I each had a life before FOP. We grieved the
loss of a healthy first baby in ways that Sarah did not. As parents, we
need to vent our frustrations, and finding ways to do that is not easy.
FINDING SUPPORT
Every family who lives with FOP, or any other chronic illness, must
cope in its own way. No one can tell anyone else how to do it. It helps
me to sort out my concerns when I talk about them, and so I have
sought people to talk to about FOP. When Sarah was diagnosed and
we did not know how to find anybody who knew about FOP, I at-
tended a Support Group sponsored by the March of Dimes for parents
of children with birth defects. The childrens problems ranged from a
missing forearm to terminal illness.
Those parents seemed strong. I admired how knowledgeable they
were about their childrens complicated needs, and how they could
talk matter-of-factly about devastating disabilities. I felt that the way
to come to terms with my situation was to become like them. I had to
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learn to think of myself as the mother of a child with special needs,
and I have.
Then we discovered the International FOP Association. We learned
the names and heard the stories of others in the FOP community. The
first International Symposium on FOP would be held in six months, in
October 1991, and we were both scared and excited to think of meet-
ing other families like ours.
In our hotel in Philadelphia, before the Symposium began, two
young people knocked on our door, a nine-year-old and a fourteen-
year old who both had FOP. After six years of being alone, it was
extraordinary to be in the same room with three FOP children. Sarah
had told us that every time she met somebody new, she would look at
their arms to see if they were bent permanently at the elbow, like her
arms were. How glorious for Sarah finally to meet other children like
her.
At the Symposium the next morning, we met the adults. A young
couple who both have FOP were on their way; my anticipation built. I
felt nervous about seeing how they looked, how Sarah would look
someday. Finally, they wheeled into the small meeting room. They
looked very big: Big people with big equipment. Each time I see them,
they seem to shrink. They looked big to me because meeting others
with FOP was an overwhelming, larger-than-life experience.
Sarah was examined in clinic by Dr. Kaplan and Dr. Michael Zas-
loff. It was the first time a doctor had ever told us Yes or No about
FOP. For six years we had listened to, What about . . . ? Do you think
. . . ? Heres an idea. Finally someone had answers, at least more
answers than we had ever heard. Sarah hopes her doctors will discover
what causes FOP bone to grow and a way to make it stop. One of her
goals is for the FOP bone in her elbows and wrists to be removed so
she can move her arms. Knowing people who know about FOP, both
doctors and other families who live with FOP, has been the single most
helpful thing in my coming to terms with Sarahs FOP.
There are other important supports too, and my family and I have
used them. Professional counseling has helped me to sort out my
feelings and pulled me through rough times. We have occasionally
found financial support through local service organizations, the public
schools, hospitals, and state and county social services.
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TEN THINGS I HAVE LEARNED
1. Not everything doctors predict will happen. Soon after Sarah was
diagnosed, we were told that if she could not move freely because of
her restricted joints, she might not be able to orient her body in the
world and would have perception problems. She never had a problem.
A doctor noticed that Sarahs face is asymmetrical and asked if she has
double vision. She never has. These questions planted seeds of dread
and worry which I have learned to put into perspective. Not everything
doctors predict will happen.
2. Parents have to stay in charge. We cannot let pity or guilt get in
the way of setting limits and giving direction to all of our children.
During those times my husband and I have become too tired or con-
fused to make and enforce rules, the children have become ill-behaved
and demanding and the whole family has floundered. It surprises me
how well our children respond when we enforce firmer guidelines.
Even during periods of flare-ups, when Sarah is in severe pain, she
reacts positively when we tell her how she can and cannot behave. All
three of our children have responded positively when their father and I
are able to be proactive rather than reactive towards our family situa-
tion.
3. The highs are higher and the lows are lower. Another parent of a
special needs child said this at a March of Dimes Support Group
meeting. A lot of special needs parenting is the same as parenting
healthy kids. There will be ups and downs, like anything in life. It is
just intensified when you are raising a special needs child.
4. Take one day at a time. Matthew 6:34 says, Do not worry about
tomorrow, for tomorrow will bring worries of its own. Todays trouble
is enough for today. I have learned the hard way not to let troubles
that may or may not happen in the future, even troubles that most
likely will happen someday, overshadow what could be a perfectly
good today. Even if the dreaded thing happens, experience has taught
me that often it is not as bad to face the reality as to imagine it.
When Sarah was two, she developed a lesion that made her neck
swell up. One of my nightmares was imagining Sarahs jaw freezing
shut, and I was frightened. It was a Friday afternoon: Our medical
emergencies always happen on weekends. We could not reach the
pediatrician so we went to the emergency room. Sarah was admitted
and put on intravenous antibiotics for two days. When the antibiotics
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did not reduce the swelling, she was discharged. The pediatric resident
who was taking care of Sarah thought I was crazy to have held togeth-
er while she was in the hospital, then dissolve in uncontrollable tears
when she was being sent home. The doctor did not understand that by
unhooking the antibiotics he was telling me that Sarah had a lesion, an
FOP flare-up, and nothing could be done.
The lesion resolved and Sarah did not lose any movement in her
jaw. Later, her jaw became slightly restricted, but she still managed to
eat and clean her teeth. Sarahs jaw locked completely after neurosur-
gery. When the manifestation I dreaded most happened, my anxiety
about her jaw turned out to be smaller than the new anxieties brought
on by the brain tumor. Sarah needed extensive brain surgery. She
might be blind or have brain damage that would destroy her personali-
ty. When the surgeon listed these horrible possibilities, I passed off
losing the movement in her jaw as something that would happen even
if she did not have surgery.
Sarah has passed her fifth year with a clean CT scan. The brain
tumor is gone, and she did not lose her vision or her personality. We
consider the surgery a fantastic success. Even coping with her frozen
jaw has been manageable. Sarah can eat soft food and small pieces of
meat and fruit. That part of FOP has not been the nightmare I imag-
ined.
Struggling through experiences like this one has taught me that it is
easier to address real concerns than hypothetical ones. From marital
stress to hip involvement, imagining what it will be like is harder than
actually coping with it.
But now I must qualify what I have said. The night after I drafted
these paragraphs, I lay awake worrying about how I would manage in
a few weeks to transport Sarah, her power wheelchair and her dog, by
airplane, from Seattle to Philadelphia to attend the Second Internation-
al Symposium on FOP. Why this anxiety? Had my own philosophy
completely failed me? I have concluded that, although taking one day
at a time is an important aspiration, it is not quite that simple. When I
am faced with a problem or challenge, I need to feel my way into it,
even be overwhelmed by it for a while, before I can own it and put it
into perspective. Only then do I seem to be able to address it construc-
tively, break it into smaller pieces, and attempt to solve it. To take one
day at a time does not mean not thinking about tomorrow, which might
even be irresponsible. Rather, it means to focus on todays issues and
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not to borrow trouble. I need to address real concerns that have to be
faced now, not squander my energy worrying about things that have
not happened and that might not happen at all.
5. I have learned to take care of myself. If I am not well, physically,
emotionally and psychologically, I will not be able to take care of my
family, and soon they will not be well either. I am doing something for
all of us when I exercise and eat well, get enough rest and spend time
with my husband. And, though I preach this one better than I practice
it, I believe in respite care. I cannot let compassion, guilt or pity
prevent me from taking time for myself or attending to the other
members of my family. By its nature, FOP demands a lot of attention,
but it should not become the only factor in family life.
6. Faith helps a lot. When Sarah was first diagnosed with FOP, I
wondered, in a detached way, whether I would question my faith. I
read Rabbi Harold Kushners well-known book, When Bad Things
Happen to Good People,
13
and agreed with his thesis that God does
not cause suffering. As I navigated the stages of grief, anger was the
emotion I had the most difficulty expressing. I never dared to feel
outright anger at God. I knew God would not abandon me if I got mad
at God, but I did fear that I would separate myself from God and I
needed God to be my firm foundation when nothing else seemed
dependable. I clung to the conviction that God would see me through
this crisis.
Eight years later, after Sarahs neurosurgery, we had several family
calamities that motivated me to seek therapy with a social worker.
After hearing me recite the stresses our family was under, she said, I
think at some point this is going to shake your faith. I thought long
and hard about her comment. The circumstances of Sarahs life and
the effect it has had on my life have challenged my assumptions. I
have had the luxury to live by the philosophy that a lot of middle class
Americans live by: If we work hard, we will get ahead. If we go to
college and get a degree, we will get a good job, earn a good salary,
buy a nice home, maybe even have a happy marriage and family. My
hard work always yielded positive results, until Sarah was born. Deal-
ing with progressive disease has given me a set of challenges that does
not respond to hard work, education or money. Nothing I do can make
Sarah get better, or relieve me of the responsibility of being her care-
taker. This makes me question the work ethic, but it does not, as the
social worker presumed, shake my Christian faith. Shortly after re-
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turning from Philadelphia when Sarah had neurosurgery, I saw a pic-
ture in the newspaper of the body of an old woman in Bosnia who had
gone out to buy water and was shot to death. Nowhere are we prom-
ised that life is going to be fair. The social worker thought my circum-
stances were going to shake my faith. No, I told her, The Christian
faith is about suffering. If I lose touch with that, all I have to do is
remember who suffered and died for me.
Early in the grief process, well-meaning parishioners quoted I Co-
rinthians 10:13, which says, God is faithful, and he will not let you
be tested beyond your strength, but with the testing he will also pro-
vide the way out so that you may be able to endure it. But my church
friends reversed the meaning of the passage. Instead of asserting that
God would strengthen us for this test, they suggested that since God
had chosen us for this test, our family must be very strong. I balked at
this pseudo-flattery. I did not want to be chosen for such a dubious
honor, nor did I feel strong. Between the progressive quality of FOP
and the subsequent diagnosis of the brain tumor, this scripture felt like
a contest to find out how much we could bear before the load crushed
us. It felt like Satan and Gods contest over Jobs suffering, except that
Jobs story has a happy ending and I do not know how our story will
end.
Far more rewarding has been another passage by Paul, Romans
8:28: We know that all things work together for good for those who
love God. FOP has brought pain and suffering into our lives, but
many blessings have come from it as well. We have been saved from
the illusion that we are immune from the suffering and hardships of
life. We have been forced to examine our assumptions about life and
faith. We have learned that relationships are more important than
accomplishments or possessions, and that small things can be of great
significance. Though Sarah wishes she could be more independent,
she sees herself as an example to others of faith and perseverance in
the face of adversity.
7. Life with FOP gets more demanding as the disease progresses.
Physical limitations and caretaking needs gradually but constantly
increase. Parents get older and more tired, but the disease progresses
in spite of it. Older parents are universally concerned about who will
care for their afflicted child when they can do it no longer. There are
many families who were unable to attend the Second International
Symposium on FOP because they could not manage the difficulties of
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travel. One adult who has FOP was so determined to attend the Sym-
posium that he traveled from his home in New York City to Philadel-
phia by ambulance.
8. Disability does not mean that life is not worth living. Having a
disability does not mean that victories are not possible, or that gloom
and doom must be the mood of each day. Disability does not mean a
person cannot lead a productive life. Everyday things just take more
work, and we have to give ourselves credit for the effort it takes to
accomplish what we do.
9. I have learned compassion. There is a lot of pain in the world. I
have a colleague in the pastoral ministry who was praised by his
parishioners as an exceptional preacher, teacher and administrator. His
only shortcoming, they claimed, was that he had not suffered and he
could not fully identify with their suffering. Then his wife was diag-
nosed with breast cancer while she was pregnant with their first child.
Now he has suffered; now he understands. Suffering changes a person.
I would even suggest there are two kinds of people in the world; those
who have suffered and those who have not. There is a name for my
camp. My family and I belong to the community of those who bear
the mark of pain. There are some advantages to belonging to this
group. Suffering makes us struggle and reflect about what we believe
and what life means. This struggle can make us richer, deeper people.
We learn things about whats important in life that can only be learned
by suffering. My own suffering has given me compassion for others.
10. There is no one right way to do this. Each person has different
resources and coping strategies. Each family finds techniques that
work and others that do not. Fathers and mothers often deal differently
with the pressures and demands of having a child with special needs,
and that can add stress to the marriage. One mother said, My hus-
band and I have our differences, but we both know that neither one of
us alone could take care of our (adult) child with FOP. We need to
give each other space to handle the pressures of having or living with
disability in our own way. Living with FOP is the challenge of a
lifetime. There is no one right way to cope.
CONCLUSION
Being the mother of a child with special needs, or chronic illness,
or, as I have come to name it, complex medical needs, is the hardest
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thing I have ever done. I feel vulnerable all the time. How can I gain
control when I am at the mercy of Sarahs pain, flare-ups and sleepless
nights? Just as Sarah will never be the same because she has FOP, I
will never be the same either.
Yet being the mother of a child with FOP has not been all bad. It has
made a better person of me. I am less proud and self-sufficient, and
more patient, compassionate and understanding than I used to be.
Coping with FOP has shown me that I can face disappointment and
grief, and survive. It has proven to me that my faith does not depend
on favorable circumstances but rests on solid ground. It has given me
and Sarah and the rest of our family an international network of
friends and soul mates through the FOP community. At the Second
International Symposium on FOP, a middle-aged woman was attend-
ing a gathering of persons with her disease for the first time. In a
discussion for adults who have FOP she told what her flare-ups are
like. Suddenly she looked at the others and exclaimed, But you
know, dont you? There is nothing like being with other people who
have and cope with FOP. It is a glimpse of what heaven must be like:
The place we are understood like nowhere else.
NOTES
1. John Segrest Taylor, Stone Man: Fibrodysplasia Ossificans Progressiva,
Alabama Medicine, The Journal of MASA (October 1984).
2. See Richard B. Steele, Accessibility or Hospitality? Reflections and Experi-
ences of a Father and Theologian, Journal of Religion in Disability & Rehabilita-
tion 1:1 (1994): 11. This article was written about my daughter, Sarah, by her father,
my husband.
3. A. Shafritz, H. Janoff, F. Gannon, E. Shore, L. Sutton, J. M. Conner, M. Zas-
loff, and F. Kaplan, Craniopharyngioma in a child with fibrodysplasia ossificans
progressiva. Abstract presented to the Society for Bone and Mineral Research
Annual Meeting. September, 1994.
4. Dr. Frederick S. Kaplan, Issac & Rose Nassau Professor of Molecular Ortho-
paedic Medicine, University of Pennsylvania Medical Center. Dr. Kaplan, along with
Dr. Eileen Shore, Dr. Michael Zasloff and others, is doing research into what causes
FOP at the FOP Molecular Biology Laboratory at the University of Pennsylvania
School of Medicine.
5. This paper was first presented to persons who have FOP and their families in a
workshop at the Second International Symposium on Fibrodysplasia Ossificans Pro-
gressiva in Philadelphia, PA from October 29-31, 1995. One hundred physicians and
scientists and 43 FOP patients and their families came from throughout the world to
share cutting-edge scientific discoveries about this mysterious condition. As the phy-
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Marilyn S. Hair 23
sicians discussed medical and scientific research, family members talked about living
with FOP. As the physicians puzzled over FOPs physically immobilizing bridges of
bone, patients and families reflected on the spiritual, social and psychological immo-
bility caused by this disabling condition. Participants discussed how to bridge the
challenges those bridges of bone present, challenges faced not only by the person
who has FOP, but by the whole family. Hence, the title of this article: Bridging the
Challenges: The Effects of FOP on the Family.
6. Naomi Angoff Chedd, Genetic Counseling: The science is the easy part,
Exceptional Parent Magazine (August 1995): 26.
7. J. M. Conner and D. A. P. Evans, Fibrodysplasia ossificans progressiva. The
clinical features and natural history of 34 patients, Journal of Bone and Joint Sur-
gery 64-B (1) (1982): 76-83.
8. Biopsies are routinely done on tumors to determine malignancy. But in the
presence of malformed toes, the microscopic examination of tissue from a character-
istic FOP lesion is not necessary to establish the diagnosis, and the trauma of a biopsy
likely would trigger the growth of FOP bone and actually make the condition worse.
9. In the mid-1980s, the right arm and shoulder of one young child with FOP
was amputated following misdiagnosis of a desmoid tumor. Another child was given
chemotherapy and now suffers from iatrogenic kidney disease.
10. Shafritz et al., op cit.
11. Elisabeth Kbler-Ross, On Death and Dying (New York: Macmillan Publish-
ing Co., Inc., 1970).
12. Sharon Kantanie, When the Wanting Is Not Enough: Living with FOP,
What is FOP? AGuidebook for Families (Casselberry, FL: International FOP Asso-
ciation, P.O. Box 196217, Winter Springs, FL 32719-6217, 1995) p. 56. The IFOPA
website is located at <www.med.upenn.edu/ortho/fop/>.
13. Harold S. Kushner, When Bad Things Happen to Good People (New York:
Avon Press, 1983).
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