Brittle Bones Prequel: Aaron's story

Prologue:

Aaron Flynn sat on the hard, filthy floor in the back corner of a one-man jail cell. His dirty fingers locked together around his skinned and bruised knees, which were curled up and pressed tightly against his spasming chest. Loud sobs shook his entire heavy frame. He rocked back and forth in rhythm to the wailing prayer coming from his own lips.
He wasn't thinking about the words; those came automatically from Hebrew school recitations and years of practice during other times of distress. Instead, he was focused on his family, recalling everything he'd put them through over the past three years, ever since the birth of his beautiful, precious, and extremely fragile daughter--Madison Crystal.
She was going to be called Madison Pearl, until in-vitro she was diagnosed with a multi-syllabic death sentence: Osteogenesis Imperfecta. Madison Crystal seemed to fit better.
Also called OI or "the brittle bone disease, this disability is not fatal in the way some are. OI is never listed as the cause of death on any coroner's report. In fact, if the world was made of pillows, a person with brittle bones might live a happy, long life--but it wasn't. Even in such a magical world, the sudden jolt of an unexpected sneeze could break a rib. In the harsh real world, the turbulence of air travel could fracture the sensitive long bones, an arm or a leg. Plopping Madison down on any hard surface--a kitchen counter, a park bench, or a wooden chair--could break the tail bone. Each of these facts was learned by one terrifying ER trip after another. Aaron recalled every bone he was personally responsible for breaking.

Prologue 2: sometime between 1992 and 1997 (about 1992)

Aaron James Jacobson sat on the hard, filthy concrete floor in the back corner of a two-man jail cell. He was the only occupant. His dirty fingers were locked together around his skinned and bruised knees, which were curled up and pressed tightly against his heavy frame. He rocked back and forth in rhythm to the wailing prayer coming from his own lips.
He wasn't thinking about the words; those came automatically from Hebrew school recitations and years of practice during other times of distress. Instead, he was focused on thoughts of his family, recalling everything he'd put them through over the past three years, ever since the birth of his beautiful, precious, and extremely fragile daughter--Madison Crystal.
The name Madison was planned even before Laura was pregnant. They decided Crystal would be a fitting middle name when in-utero the baby was diagnosed with Osteogenesis Imperfecta. Also called OI or "the brittle-bone disease," this was a multi-syllabic death sentence, though not fatal in the way some conditions are. OI is never listed as the cause of death on any coroner's report. If the world was made of pillows, a person with brittle bones might live a happy, long life--but it isn't. Even in such a magical world, check the pillows to be sure they are hypo-allergenic--the sudden jolt of an unexpected sneeze could break a rib.
In the harsh, real world, the turbulence of air travel could fracture the sensitive long bones, an arm or a leg. Plopping Madison down on any hard surface, a kitchen counter, a park bench, or a wooden chair could break the tailbone. Each of these facts was eventually learned by one terrifying ER trip after another. Aaron recalled every bone he was personally responsible for breaking.

Prologue 2 - 1988-1989: The Announcement

Aaron, a California native used to dry heat and the cool, clean ocean breeze, had never gotten used to the muggy atmosphere of Oklahoma. The air was thick and the night dark. The broiling clouds passing by overhead were heavy with rain. A tornado watch had been issued for Cleveland County by the National Weather Service. No funnels were in sight and the rain had yet to fall, so Aaron continued his vigil, tending hot dogs and hamburgers on the back-porch grill. In the distant southwest, lightning crackled and thunder shouted across the sky.
Along with the smoky, strong aroma of grilling meat, Aaron smelled a conspiracy. Laura and Barbara were inside, giggling like schoolgirls, the sound carried through a crack in the sliding glass door by the strong storm wind. He caught his wife looking his way. He waved the grilling fork at her, then from a plate resting on the grill's table attachment he picked up a hotdog with his free hand and chomped it in half. She smiled and directed at him a gesture she often used with the kids. "Shame, shame, shame," she mouthed.
Just then, Aaron felt the first large raindrop fall from the overstuffed blanket of clouds above. He stabbed the meat on the grill, stacked dogs and burgers together on the plate, and rushed it inside. When he crossed the threshold, he used his elbow to slide the door shut behind him. He paused in the entryway. The house was quiet, hushed. Laura and Barbara were suspiciously silent; he had the distinct feeling that a conversation had ended when he entered. Conspiracy indeed,
Barbara looked sideways at Laura and casually wandered out of the kitchen. "I'm going to check on the kids," she said, retreating into the living room.
Standing alone now behind the counter, Laura looked at him expectantly. He looked back, wondering if she cut her hair or was experimenting with new makeup. Something was different, and it looked Good. She looked happier than she'd been in a while.
"Come here," she said, beckoning him with the crook of her index finger.
He hurried as fast as he could without dropping food on the carpet. He set the plate on the counter and she took his calloused hands in her delicate small ones, not minding the grease that coated them, and she kissed him long and slow. She hadn't kissed him like that since Martin was born. The business of taking care of two young kids had kept them apart more than either liked.
"What's gotten into you?" He asked, playfully running his fingers through her curly, long hair. He kissed her back, competing with her passion.
Coyly evading the question, she led him into the bedroom. Later, as Aaron and Laura snuggled together under the covers, she whispered in his ear, "I'm pregnant."

Track B.1 (an unfinished summary segment): Dec 1988-Aug 1989
They will be having a disabled child; ultrasounds showed her limbs curved like ribs, not straight as normal arms and legs should be. Both of her arms and her left leg were fractured. Diagnosis is confirmed later in utero DNA test. Osteogenesis Imperfecta.
Psychologically, this is a big blow to both parents, but Aaron takes it hardest. He has been in competition with his brother Josiah, following in his footsteps, trying to measure up to his older sibling. He recognizes Josiah's poor choices, but unconsciously still craves validation and approval from him. He had dreams of returning to Cali with his new wife (to show her off) and a child is seen as one more achievement in his favor. The idea of having a disabled child makes him feel like a failure. He researches OI himself and it tears him up inside. He knows as much medical knowledge as is necessary to interpret what he finds, and wishes he didn't. Knowing the details, being able to read the medical lingo, leaves him with no shelter of denial.
Barbara, Laura's friend, comes by often for support and both Aaron and Laura are grateful. She listens to their frustrations and worries. Aaron is afraid to talk about his fears with Laura, knowing she has her own and not wanting to burden her with his, too. He and Barbara become close friends.

Scene 3: Discharge Day: 10/09/1989

Though doctors and Aaron's colleagues in social work express to them the challenges inherent in raising a child with OI, nothing prepared them adequately for the reality they would face. At two months old, Madison was stable enough to be brought home.
Doctor Bliss, the physician in charge of her case, instructed them on the dangers of putting Madison in an uncontrolled setting. He explained how to avoid injury in a thousand different circumstances--how to hold her, how to feed and burp her, how to change her diaper, how to bathe her, and how to put her to bed. These daily tasks required the greatest care.
On the day Madison was to be discharged from the hospital, Dr. Bliss handed Aaron a piece of paper and said, "Carry this always." On it was a description of OI, focusing on the common ways that children with the condition could break a bone, and a paragraph identifying Madison Crystal Jacobson as having the disability.
"We've talked about this already, doc," Aaron said.
Dr. Bliss frowned. "It's not for you. It is for the myriad of doctors and nurses your daughter will be treated by throughout her childhood." Aaron raised his eyebrows in a quizzical gesture. Bliss continued. "What would you think, if you'd never heard of OI, of a mother who brings a child into the emergency room who says a fractured arm was caused by bumping into a low counter and the day before the same child fractured a leg by bumping into the coffee table?"
"Oh, Jesus," Laura sighed. Aaron put a reassuring arm on her slumped shoulders.
"Let's not get ahead of ourselves here," Aaron said, tucking the document in Laura's purse. "Nevermind dealing with a bumbling, clumsy toddler. I need to know how to get here from here to home."
[Author's note: where are Martin (now 4) and Charles (now almost 2)??]

Scene 4: The 1st Year

The first year of Madison's life was hardest for them all. At two-months-old she was stable enough to go home from the hospital. Dr. Bliss, the physician in charge of her case and the man who diagnosed Madison based on the color of the normally-white sclera of the eye, instructed them on the dangers of putting Madison in any uncontrollable setting. He explained how to avoid injury to her in a thousand different circumstances--how to put her to bed, how to hold her, how to change her diaper, how to feed her, and how to bathe her. Those daily tasks required the greatest of care. On the way home and for the next six months she slept in a cocoon of extra-plush hypo-allergenic pillows arranged in an ordinary plastic tub bought from Walmart. When driving to or from the hospital, the tub was placed on the floor in the back of the car. Seatbelts were not OI-approved, and a crash would break their precious cargo into a million pieces regardless, so Laura never sped.
Once a week it seemed Laura returned to the hospital with some new fracture of near-disaster that needed an x-ray, just in case. As the visits increased abuse allegations became ordinary, though only once did the slip from Dr. Bliss, which explained the fracture causes and which Laura carried in her purse always, fail to prevent cops from being called.
The medical bills were astronomical, even with Aaron's work insurance, so he spent extra hours at the office while Laura managed the house and took care of Madison. Dr. Bliss recommended that they take Madison to Utah and enroll her in a quarter-annual clinic for OI patients. They'd pay for medical x-rays, DEXA bone-density scans, MRI's, and other tests not routinely performed elsewhere. They'd also grant access to specialists who had treated OI before. Dr. Bliss was 'happy to help' locally, but Madison was his first OI patient and calling colleagues for advice was not the best treatment plan. In return for treating Madison's symptoms, the OI clinic would study the condition in hope of better treating future patients. Without this level of treatment, Dr. Bliss said, her chances for survival were minimal.
Looking back, it was during this time that Aaron started to build a wall between himself and his daughter. The idea of her not surviving wasn't new. The idea that he'd move away and sacrifice everything to her survival because he loved her--and that she still might survive--this had just occurred to him and it angered him. When Dr. Bliss first mentioned it, he lunged at the man.
"Just tell it like it is, you pompous prick." Aaron shouted. "With this OI clinic's experimental drugs and fancy equipment, my daughter's probability of dying before her fifth birthday is what, eighty, ninety percent?"
Laura had calmed him down, but the wall's foundations had been laid. If didn't sever the attachment he already felt for his baby now, when she died he knew it would be as if ripping off his own arm. It was better to let go now, gradually. He could avoid this pain if he didn't allow himself to care so much.
Yet, at her one-year checkup, one doctor reported that she had a strong will to survive and she exceeded all expectations thus far. Her growth was stunted, but she was a fast healer and her history of multiple breaks had caused no early signs of brain damage or other disabilities. As she grew and surprised doctors with fortitude, Aaron swelled with hope. Each checkup began to echo the last: she was doing well. He hugged her and told her how proud he was of her. She smiled back as if she understood.
However, the wonderful feeling that everything would be okay only lasted from the time the doctor praised her progress until the moment of her next fracture, often the same day. He suppressed the hope they were feeding him, especially the part about what the OI clinic could do. Laura was hopeful enough for the both and he told her to go without him. After the first trip, Laura came back raving about new treatments and experimental drugs which were achieving "some success" in early trials.
"They can't cure her," he had said. "And they cannot make the bone produce what collagen it's missing. She will never walk, she will never drive, she will never get married and give us any grandkids. If she's lucky and she lives that long, her children would be cursed with her disease. The clinic will study her like a lab rat hoping to find a genetic marker they can use to provide early warning to other mothers -- so they can abort before it's too late."
Laura didn't bother to respond to his drunken tirade. During the next trip, she traveled to Utah with Madison, her mother Gina, and her best friend Barbara. Aaron was not invited.

Scene 5: Two to Six Months (1989ish)

On the way home and for the next six months she slept in a cocoon of extra-plush hypo-allergenic pillows arranged in an ordinary plastic tub bought from Walmart. Seatbelts were not OI-approved, and a crash would break their precious cargo in a million pieces regardless, so when driving the tub was placed on the floor in the back of the car. Laura never sped.
It was always Laura. The medical bills were astronomical even with Aaron's work insurance, so he spent extra hours at work while Laura managed Madison's care. Once a week it seemed Laura returned to the hospital to take care of some new fracture or near disaster that needed an x-ray just in case. The house maintenance was low on their priority list but between the two of them they managed to keep it from total disrepair. {the boys!}
As the hospital visits increased, so did tensions between Aaron and Laura. Though Madison's long-term prognosis is not good, she continues to survive. Aaron proudly believes that she will continue to make fools of doctors as long as they keep telling her she "wont" or she "can't" and set limits on her survivability. He feels split in two. One half looks at his daughter with pride and hopeful expectation, while the other half feels helpless, afraid, and resentfully bitter of the seesaw as her prognosis changes daily.
Laura feels much the same as Aaron. She is frustrated as no one in Oklahoma seems to know much about OI, and the note Dr. Bliss had written is waved like a magic wand to fend off abuse charges more than once. Madison's doctor suggests they fly out to Utah and take her to Primary Children's Medical Center when Madison is stable enough. Laura and Madison fly out. Aaron is unable to come because of work.

Scene 6: PCMC: sometime appx. Feb 1990, Madison appx. Six months old. {Martin appx. 5yo, Charles appx. 2}

During the flight from Oklahoma, Madison is bumped by a careless flight attendant, so immediately upon arrival she is evaluated by a team of doctors. She fractured the radius of her right arm. The chief clinician is called Dr. Nielsen. He discusses enrolling her in a semi-annual clinic for OI patients. They'd pay for medical x-rays, MRI's, DEXA bone-density scans and other tests not routinely performed elsewhere. In addition, they'd also grant access to specialists, ones who had experience in treating OI specifically.
"I've spoken to Dr. Bliss," Dr. Nielsen said. "Though he is happy to help in any way he can, Madison is his first OI patient. We have a first-rate facility here and quite frankly without this level of treatment your daughter has minimal chances for survival past five years."
"What do we have to do in return?" Laura asked. "There has to be a cost somewhere down the line or you couldn't afford this 'first-rate facility"
"Well, yes, we do ask for something in return, but not money. During each visit we'll schedule a series of tests. Eyes. Ears. Heart. Lung. Etcetera. We will monitor the progress of Madison's disease as she ages, repeating many of the same tests from year to year and tracking the changes."
"If she's going to live a short time, I don't want all of her memories to be of doctors and tests. Poking and prodding in addition to all of her bone breaks seems like unnecessary torture."
"Except for the unpleasant but necessary poke of a needle to draw blood, the tests will not usually be painful, and the more we discover about OI the longer we can extend her life. We collect data to develop new cutting-edge treatment, which we'll offer first to Clinic patients participating in the studies. For example, we are testing bisphosphonate drugs. They've shown to increase bone density, and by tracking the density changes from year to year we learn how to improve the drug for the next generation. The lack of collagen inherent in OI patients--the cause of brittle bones--is countered by greater bone density."
"Right now, at six-months-old, all your daughter's memories are limited by her lack of mobility. She can't roll over, she hardly rocks from side to side, and that can be improved before your seven days with us is over. If we can improve her quality of life by even a fraction at this point, isn't it worth it?"
"It'd be a blessing, yes, but I still don't know if it'd be worth it. How long before she can crawl if we are improving a little bit every six months?"
"Our rehab specialist, Holly Jensen, will teach you how to make fractional improvements on a daily basis, continuing at home after your visit, so she can be rolling around the room in no time at all, and crawling only a few months behind schedule. We'll have to do surgery on her arms and legs, inserting telescoping rods in the long bones so she can bear weight on them, and they'll grow as she does, but we'd do that anyway after the next major break. The rods stabilize the bone from the inside--an internal splint, if you will--so when she breaks it won't be a compound fracture."
"But will any of this extend her expected lifespan? That's what's important. If she lives six years but she spends half in the hospital recovering from surgeries to minimize breaks, how is that better than six years half-spent in an emergency room recovering from the breaks themselves?"
"Trust me, you'll learn how to avoid most breaks after you have more experience, and when she makes it to two we can begin the bisphosphonate drugs for bone density, which by then will no longer be so 'experimental.' We'll also be able to do the rodding surgeries. Keep doing what you're doing, come back every six months, and after a few more visits you'll see that her life will not, as you say, be half-spent in hospitals. And it is possible her life will in fact be greatly extended by treatment and rehabilitation. If nothing else, consider that we'll be able to catch problems early, and correct them. I'm not giving you a guarantee, I'm giving you a chance."
Later that night, on the phone Laura tried to explain to Aaron the benefits of enrolling in the OI clinic, but he was having none of it. She patiently explained the possible new treatments and excitedly shared the good news that experimental drugs were achieving some success in early trials, because of other patients' past enrollment.
"They can't cure her," he argued. His words were slurred and she knew he'd been drinking. "They can't make the bone produce what natural collagen is missing. You don't get it. She'll never walk. She'll never drive. She'll never get married and give us any grandkids. If she's lucky and she lives that long, her children would be cursed with her disease. The best the clinic can do is study her like a lab rat, hoping to find some genetic marker they can use to provide early warning to other mothers--so they can abort, before it's too late."
Laura, choosing not to respond to this drunken tirade, hung up. {where are the kids, dammit!}

Summary B.2

After the birth of Madison, Aaron falls apart completely. He simply doesn't know how to handle the cold fact that he's the father of a disabled child. Madison is flown to Baptist Medical and is hospitalized from birth until she's two months old. Upon release, she still requires endless doctor visits and hospital stays. Though her prognosis is not good, she continues to survive. Aaron believes she will continue to make fools of doctors so long as they keep telling her she "wont" or "cant," setting limits on everything from her mobility to survivability. This touches his heart and makes him proud, but his fear of losing her tempers his joy. He feels split in two--one half looks at his daughter with pride and hopeful expectation, while the other half feels burdened and is resentfully bitter. Her prognosis changes daily. He pushes Adonai away completely, except in prayers of desperation, which seem to be unanswered. He feels like a victim in some cosmic joke. He spoils his older boys, overcompensating for his feelings of helplessness. No one in Oklahoma seems to know how to handle OI and Madison's doctor suggests they fly out to PCMC, in SLC, UT. After Madison is stable, at 4 months, Laura flies out with the kids. Aaron cannot leave work to join them. Turbulence breaks an arm. Utah docs "strongly suggest" moving to Utah, mentioning St. George as a warm place where there is already one girl with OI. Upon her return, Laura fights with Aaron about moving. 3 months later, Laura again flies to Utah with Madison. This time, Aaron (and Laura's mother, Gins (or Barbara)) also go/es. The kids stay home with Barbara (or just Bob). Docs confront Aaron, telling him directly that Utah is best. Madison continues to grow past expectations. She "makes" both parents feel inadequate b/c they break her. Aaron turns to drugs to cope, while Laura turns to Adrianna, the mother of the other girl with OI in Utah, which often alienates Barbara. Maddie reaches her 1st birthday and is accepted into PCMC's research study. In agreement that Madison will visit every six months, they will cover expenses and provide continual care. This allows them to stay in Oklahoma, at least for now. (Note that most of this is outdated, but only because unclear where the boys are).

Summary B.3 : Feb 1990-1992 (appx July)
Since Aaron cannot take a week off of work every six months, he is left alone with too much time to think. Laura's best friend Barbara is invited, but she also cannot miss work to come. Aaron's depression worsens. Laura discusses Aaron's problems with Barbara: he's great most of the time and is romantic and loving, unless the children's needs are put before his, which is often in Madison's case. Just before trips he is irritable, but afterward he is sweet. His drug use increases.
[Note: 2nd trip was July 1990 in Summer break then again in late Dec. 1990 in Winter break, and subsequent trips June 1991, Jan 1992, and July 1992.)
In January of 1992, Barb's mother passes away. She needs support and Aaron is available. He listens to her, comforts her, and shares his stash of drugs with her, promising they will take her mind off of her problems. They end up getting high together, then end up having sex while stoned just before Laura comes back from the most-recent trip. Privately, Aaron and Barb agree to limit the affair to just the one time and keep it secret. But... Barb discovers she is pregnant. She and her husband Robert (Bob) have tried and failed to conceive, so she decides to pretend it is his. Bob is overjoyed at the news of her pregnancy while Aaron is rightfully suspicious. Fearing he will interfere, she waits until the next med trip in July, then makes an anonymous call to the police, informing them Aaron is a drug dealer. They raid the house, find drugs where she said they'd be, and Aaron is arrested. Devastated, Laura asks Barb for advice. Barb says, "Think of the children's welfare. He'll never change. Leave him and get to Utah as fast as you can go." Aaron pleads guilty and is sent to prison on a 5 year sentence. Madison turns three in August of 1992 and the clinic visits are extended to yearly ones. While in Utah, Laura looks at potential real estate, seriously considering the option to move. When she returns, she's made up her mind to "do what's best for Maddie and the boys," and files for divorce. She moves in with Barbara until she can arrange to move.

{Note: by Jan of 92 (5th trip) the boys are 6 (almost 7) and 4. During the Feb 90 trip (first trip) Martin is likely in preschool and turns five in March. 2nd trip the boys make come on summer break of Martin's kindergarten (July 90) and return again for 3rd trip the same year in winter break (late Dec: Martin 5, Charles 3). 4th trip in June 91. And Note: the kids MUST be with Laura on 5thtrip but may stay home others. The 6th trip is july 92. The boys are HOME when the police raid house and are allowed to stay with Barbara and Bob until Laura returns. Barbara calls Laura to tell her the news of the raid, also tells her she (Barbara) is NOT bailing him out and suggests when Laura returns that she think twice before bailing him out also. She possibly stays with him clear through trial, but leaves when he is sentenced.)

Future segment scene:

As Aaron was sitting and pondering, Laura and Barbara were watching three-year-old Madison roll on the floor. They'd just returned from a trip to Primary Children's Medical Center in Salt Lake City.
"Isn't her progress wonderful?" Barbara said.
"Miraculous," Laura responded.
After only a handful of visits to PCMC's OI clinic, Madison was able to roll on the floor. Though most children were walking at that age, Laura knew without the physical therapy offered at the clinic, without the balance of patience and pushiness from the Holly, Madison would still be stuck in whatever position she was laid. If she was put on her back she was able to roll on her stomach now, and if she was on her stomach she was close to being able to crawl. One more visit.

Track B.4: more summary: Aaron 1992-Current (born 1958)

Aaron spends 4 years and 4 months at the Lompoc Federal Correctional Institution (FCI) and is released in Dec. of 1996 at 38yo. During his prison stay, he writes to Madison, but gets no response from Laura. He is utterly alone. Until year 2 he didn't even tell his parents where he was. He believes Adonai is punishing him and is ashamed. When he did tell them, they were very forgiving and offered him a place to stay when he got out, until he gets back on track. Upon his release, he stays in Cali and works at the family's Cali pawnshop 8am-4pm. From 8pm to 12am he drives a taxi. No hospital will hire a felon social worker. At the pawnshop he works M-F and takes off the Saturday Sabbath as well as Sundays. At the taxi job, he has Friday and Saturday nights off.
In 1998, at 40, he meets Nichole, a Hawaiian-skinned 34yo from Maryland, on a series of taxi-runs. In December of 1999, the day after his three-year probation expires, he marries her. He's no longer tied to Cali, to his PO Brandon Pixley, and they move immediately to Silver Springs, MD. They live 3 years near her parents. Her father is a radiologist-consult/health-care-physicist and her mom is a pediatric oncologist. When he finds out the NIH in MD studies OI, he is reminded of the daughter he abandoned. He's not sure she's even alive, but he believes she is. On Sept 4, 2000, at 42, he has a healthy baby girl. He names her Jessica. He writes again to Laura, telling her that he is moving on, that he is sorry he wasn't there for her. He doesn't mention his new wife and child.
In 2003, after Nichole finishes an Internship at the NIH, they move to Vegas. In 2007, he realizes Madison will be turning 18 and he writes to her again. Nichole is eventually unhappy. They divorce in 2009. He is 51. Nichole takes their daughter back to MD. He stays in Vegas, working at a pawnshop his brother Josiah manages. In 2010, Adonai directs Madison back to him.

Track B.5: more summary: Laura Carlson (Jacobson) 1992-current?

After moving to St. George, Utah, Laura loses touch quickly with Barbara. Barb, feeling guilty about the past affair and ashamed of her actions against Aaron, pushes Laura away, ignoring her calls. Laura becomes very close with Adrianna Beck. Though Madison continues to break, Laura accepts this as part of the OI routine. The older boys learn to take care of their sister, given chores like winding up unrolled splints, emptying bedpans, dispensing oral pain medication, and above all keeping all toys off the floor that the toddler may bump into and trip over or, later, the wheelchair-bound child may run over. Also, no rough-housing with sister.