My Life Story

Our 3rd child was born right on time, on January 20th, of 1983, weighing in at 6 lbs 10 oz, and 19". Within normal limits as babies go, but small compared to her two older siblings. We honestly had no clue that anything was wrong with her until she made that appearance (not that it would have really made any difference to us).

Her head and eyes were notably small. Eyes were also tightly shut with very long lashes. One ear was floppy. She had a small mouth with a very high arched palate. Doctors heard a loud "click swish" heart murmur. Her 3rd & 4th fingers on her right hand were webbed. And she'd also aspirated amniotic fluid during delivery, so she was a blue baby with apgar scores of 5 & 7. The doctors immediately suspected that it was genetic, but none of them had ever seen anything like it before. And that wasn't our only surprise.....

We were actually expecting a boy, and had intended to name him Philip, after my step dad. So we hadn't even picked a girl's name. And it took us a couple of days to come up with one we felt we could live with. The NICU nurses however assumed we weren't naming her because we thought she was going to die, dispite my having told them otherwise. So they were all quite surprised when we walked in and announced that her name was Philina.

The official female derivative of the Greek name Philip, is Philana, but a childhood friend had already named her daughter that, so we went with it's English variation, Philina, which means: "Loving. Lover of all God's creations, this girl was beloved by all." And the extra time needed to choose her name was well worth it, as the name most certainly fits the girl.

She only spent a week in the hospital, but came home still with no over all diagnosis. She grew poorly, and threw up constantly. It was customary to lay babies on their stomachs at that time, but we couldn't do that, as she would totally congest up. And we couldn't put her on her back either, due to the vomiting, so she slept sitting up in her car seat until we could come up with a viable solution. We finally just put cinder blocks under the legs at the head of her crib, and laid her on her back with her head elevated.

At one month, she opened her eyes, but just barely. Since there was obviously something wrong with her, even if we didn't know exactly what, we enrolled her in our school district's early intervention in-home infant program. And when we got the preliminary genetics test results back, of some unidentified extra piece of genetic material on one of her #6 chromosomes, she became a client of The San Diego Regional Center for the Developmentally Delayed.

Due to continual ear infections, ventilation tubes were surgically placed at 5 mos. But once the tubes were in, we got a bonus, she suddenly stopped throwing up. Seems the poor child had just been suffering from motion sickness all that time from the pressure on her ears.

She was also fitted with glasses around the same time, and we started to patch the lenses for 10% crossed eyes. Progress was very difficult and slow, because she constantly took her glasses off. But with perseverance, we eventually had total success, and avoided eye surgery.

It took 8 months, and 3 chromosome tests to finally get a conclusive result of a de novo Partial Trisomy 6p (PT6p). Very little information was available on it, and nothing beyond the age of 3 yrs. It seemed they tended to die of either pneumonia or heart failure by then. And she too had a bout of congestive heart failure at 11 months, but a Patent Ductus Arteriosis repair was all she needed at that time.

At 1 yr and 11 lbs 8 oz, she still only wore size 3 mo clothing. And frankly I was starting to get tired of looking at the same old clothes. But with the heart repair came a big improvement in her oxygen circulation, and she was finally at the point of what almost qualified as thriving.

Then we discovered that she was actually allergic to both milk and soy, so we changed her to Gerber's Beef Heart Formula. ( I don't think they even make it any more. ) It was vile smelling and much thicker then regular formula. And she'd always had a weak suck due to that high arched palate. But since she'd never suffered from reflux, I just cut bigger holes in her baby bottle nipples. We also increased her calcium intake at that time, with ground up Tums, and suddenly we DID have a growing, thriving child.

We had put her down on the floor on a blanket at 6 mo, but all she had ever done was roll around the house a little. But now we were seeing army crawling. By 18 mo she sat up all by herself. Then at 19 mo she began being bussed to our local early intervention in-school infant program. So when she crawled on hands & knees at 2, we put all of her toys in a big wooden wagon. That required her to have to pull herself to a standing position in order to be able to get at them. Incentive did wonders.

Hearing aids were fitted at 2 1/2, her fingers were surgically separated, and she began to learn sign language. And because she was identified as hard of hearing by our Children's Hospital Audiology Dept, the school district labeled her Deaf, Hard of Hearing, Multi-Handicapped (DHH-Multi), and moved her out of the infant program 6 months early.

They placed her in a preschool program within an all handicapped school. I liked her new school (teacher & program). And she was developing well within that enviorment too. So with added private insurance funded OT & PT from the local Children's Hospital, and an orthopedic walker, she began to walk at age 3. But she discarded the walker at 4, because it slowed her down too much.

She loved her school, was attending swimming lessons, and The Park's Dept Disabled Preschooler's activities . She was a very happy little social butterfly.

When she was 4 however, our district's policy dictated that Philina's school had to be changed again. So she was moved on to a DHH-Multi preschool program at a regular elementary school. But despite the fact that her new teacher keep telling me how cute Philina was, and how much she adored her, she was also so desperate to get her out of her classroom, that she turned us in as negligent parents to Child Protective Services, when we resisted her efforts to totally strip Philina of her DHH label. She wanted to make her a Severely Handicapped - non verbal. But I knew a child in that program, so was already way too familiar with it. Tied in a chair & just playing with toys all day, and it sure wasn't happening to our child. No way were they going to ship her off where nobody would even know sign language, especially after they'd taught her to communicate that way.

But based on all of the documentation that I'd saved from the school and our doctors, the CPS worker who was assigned to investigate our case closed it as fraudulent on her first visit to our home. It was her opinion that we were the victims of harassment by the school, so she suggested that we should hire a lawyer. We didn't initially though, as we still hoped to work things out amicably, since we felt Philina was truly in the best placement for her.

In retrospect though, we finally figured out what the real problem was. Her teacher had kept complaining that Philina was sick all the time, because her nose ran every day at school. But it didn't run at home. Of course she didn't believe us. Our ENT kept saying it was just allergies, and she should be in school. But the school nurse sided with the teacher and kept saying, "No she was sick", and calling me to take her back to the ENT for another note, before she could come back to school again. But it WAS allergies. Her teacher was a smoker, we aren't. So every day about 10 minutes after her teacher would get back from her 10AM cigarette break, Miss P's nose would just start to pour (left nostril only). But hindsight is always so perfect.....

Before it was over, the school had build their very own hearing testing booth, tested her themselves, and declared that she didn't have a significant hearing loss. That was news to us, the local Children's Hospital Audiology Dept, and her ENT's office, who had both tested her numerous times, with a consistent results of a bilateral, moderate to severe, mixed loss. But they had justified to themselves going ahead and stripping her of her DHH label behinds our backs. That's when we hired the lawyer!

Unfortunately though, by the time we were able to get a handle on it all, Philina was so upset and frustrated, that she ended up having to spend the next 10 years in a private school placement specializing in severe language disorders and behavior modification (something she REALLY needed after what the teacher had just put her through).

Precious developmental & educational time had to be sacrificed to get her back to where she could even be worked with again, without her shrieking every few seconds, ripping out fist fulls of her hair, and biting her wrist. Even her social activities were impacted by these behaviors. But thanks to the lawyer, at least the school district had to foot the bill for the private school.

We insisted on an "Other Health Impaired" label for her during those years. The advantage to that, was our district had no set program for that label, so any further attempts by them to move her to another school, would have to go through an IEP review first. And about 5 years into that placement (or age 11) she had improved so much that they thought she could probably go back to the DHH - Multi classes, but at the middle school level, since I felt we had NO basis for a working relationship with the elementary one. But after only 3 weeks of dual enrollment, of just a couple of hours a day spent there, she broke out in a pityriasis rosea rash (due to nerves). Plus by then, the DHH middle school had declared that they didn't EVER want to see her face again either. So back full time to the behavior modification program, to spend the next 5 years undoing the damage caused by that 3 week trial.

During those years she had also suffered from lots of ear infections, and sinusitis (due to another smoking teacher, but at least this one really did adore her), had several ear and finger surgeries, and a mysterious episode of temporary paralysis (that had spontaneously corrected itself within hours). She'd also had one of these as an infant, but on the opposite side of her body. Tests were inconclusive however, and all the neurologist was able to find was some mild damage in the balance center portion of her brain. We still had some very significant developmental milestones though, so by 8 she was independent in her toileting, day and night, could tolerate texture, and had even begun to feed herself.

At 12 yrs she needed an aortic angioplasty (feeding a balloon up through her valve from the groin area, inflating it, and pulling it back through the valve to rip it open), when puberty made her heart unable to keep up with her growth. She was turning blue, & tired out easily. But after the procedure, once again her only heart restrictions were antibiotics before dental checkups & yearly EKG & ECHO evaluations.

15 saw her behavior so much improved again that she was able to try to return to the regular DHH-Multi class at the high school level. And I figured, "Fine, if she doesn't make it this time, we just won't ever have to face this again." But she did make the transition, though she was unable to do the regular school curriculum, and continued to have to work off of an IEP. She also lacked good finger dexterity, but could sign pretty well in spite of that, & knew hundreds of signs. So she fit into the class well, made new friends, and they gave her a job helping in the school cafeteria.

By 16, though she was still smaller then both of her siblings, she had reached 5' 1 3/4" tall, & 111 lbs. And once again she was her same old happy, social butterfly self. A very active girl, who loved to play on the computer, listen to music, and watch videos. She could ride a bike (with training wheels) and roller skate. She was back at her swimming lessons, was now on a bowling league, went to Disabled Services Teen Group activities, and Respite Camps. Her life was pretty good again.

Then at 18 there were some VERY big changes in her world. Testing placed her developmentally at around an overall age of 3 yrs. But despite that, she was now officially an adult. And on top of that, her aortic valve had begun leaking. So she would be needing a valve replacement surgery, but not until it became absolutely necessary. She still didn't require any medications for it, but her doctor was keeping a closer eye on her. Then due to the relocation of the company her dad worked for, we baled on big city life, and the only home she'd ever known, to move to a farm that we call Philina's Pharm. She lost her almost daily contact with her best friend Ryan (Down Syndrome) with that move. They had virtually grown up together, as I'd been his daycare provider since he was 4 and she was 2. Her swimming lessons also had to go too, but at least she did get to remain with her bowling league and respite camps. I had hoped that would soften the blow enough, but I also kept her home that first summer here to let her get use to her new home & town before hitting her with a new school district (school, teacher, classmates, bus driver). Her brother had stayed behind for his job, and then at the end of August her sister transfered colleges to one in New York City, making her in effect an only child now too. She had lost almost all of her "normal" day-to-day life.

On top of that, she was traumatized just after starting her new school, when a coyote ate her cat, leaving behind copious amounts of hair as evidence. After that she refused to go outside to play, hung tambourines on the door knobs for "coyote alarms", and insisted on sleeping in the middle of the living room floor. It was pretty obvious that she didn't really understand what a coyote was, but nothing "bad" was going to get past her.

It continued to be a hectic fall. On 9/11 her sister was also traumatized, but trying desperately to hold herself together with the help of Zoloft. Then on Halloween Philina had to be sedated for her annual cardiac ECHO, so we decided to have her drooping eye lids fixed at the same time. I've always coordinated as many procedures as possible per anesthesia. The surgery was a total success, but the ECHO showed it was now time to see about that valve replacement. She had a heart cath the end of Nov, and the open heart procedure was scheduled for Dec 11th, unfortunately with transiting Pluto conjunct both her dad's and my Ascendants. But as slow as it moves, and as bad as her valve was leaking, waiting just wasn't an option.

The cardiac surgery itself was also a total success, but everything went wrong post op. It became an over 3 month ordeal that we still refer to around here as "The Cardiac Surgery From Hell" (aka Pluto). It all started the very first night with a clogged chest drain, so they reopened her chest and left it open for a week. Once closed she had 3 collapsed lungs. But we started giving her Reiki, and that problem disappeared. But then she had a bad reaction to Nipride (a blood pressure regulator) and she fully "coded" twice in one day.

Her sister was now freaking out back in NY. She had taken an emergency leave despite the school trying to talk her out of it, but she didn't get here until after the surgery had started, and she'd had to go back to NY after Christmas, with Philina still in bad shape & in the coma. By this point Philina had been in the medically induced coma, on Fentanyl (a heavy duty narcotic pain killer) for about 7 wks, so now she had to be detoxed with Methadone too. The poor child was suffering from some pretty severe shaking during the detoxification treatment. And someone obviously decided she needed a neuro consult for it. (Sure wish that people would have bothered to either read her chart, or at least talk to us.) And then the neuro evidently assumed it was due to seizures (even though they'd had her hooked up to an EEG for a week after she'd coded, and saw nothing wrong with her brain waves). So now he added massive doses of Phenobarbital into the mix too. Next she came down with sinusitis, and the Lasix she was being given post op turned her normally pasty mucous into cement-like consistency plugs which blocked her airways. So she failed extubation 3 times & ended up with a tracheotomy.

Just before her 19th birthday, Philina was finally awake, detoxed, and moved into the Intermediary Care Unit. That was when it was first suspected that she must have sustained some pretty serious brain damage. She drooled, listed badly to the left, was unable to smile, communicate, or even lift her head off of the pillow by herself. But she sure could pull out the tubes, so they had to keep her hands tied (even with a sitter).

And so it was that we got a new diagnosis of Moya Moya Syndrome added to Philina's chart, compliments of an MRA. Moya Moya is Japanese for "puff of smoke", and it represents a structural problem with the blood supply to the brain. The veins in her neck are restricting blood flow, and she's totally missing the vessel that should transport the blood through the center of her brain. It wasn't anything new of course, she'd been living with this all along. But now we had a logical explanation for the paralysis episodes, and the damage in her balance center. In fact, the only reason she had even lived to be born, was that she had set up her own custom "puff of smoke" capillary system to oxygenate her brain in gestation. Usually doctors look at Moya Moya as a bad thing, but in her case it was now actually encouraging news. Since her body was already use to needing to compensate, it was hoped it would keep it up, and correct the recent damage.

So it was at this point that we had to make one of the hardest decisions of our lives. But basically we had no choice. The only way we could possibly hope to get her back, would be to send her back into surgery for a g-tube and fundoplasty, so we could build her strength back up. We desperately needed to get the ng & trach out, her hands untied, rid of the Phenobarbital and Lasix, & her moved to the rehab unit.

A couple of days before the surgery her sister totally fell apart, and ended up in a mental ward back in NY on Respiridol and Paxil. Now we had one in hospitals on each side of the country, and needing our attention. In fact, I was so stressed out by this point, that I actually told their brother, "If you need anything, you're going to have to take a number". Fortunately he not only didn't need anything, I met my future daughter-in-law over Philina's hospital bed. And the day after Philina got her g-tube, I held down the fort here while their dad flew off to collect the older one, and bring her home.

The g-tube surgery did wonders, & within a couple of weeks Philina was off the Lasix, the ng & trach were out, her hands were untied, and she was finally being moved into the rehab unit, where I was her sitter and therapy aide. I'd also finally found another neurologist who would listen to me about the Phenobarbital, and he wrote an order to begin weaning her off of it. She exhibited many of it's worst side effects: nausea and gagging (but she couldn't throw up with the fundo), a severe acne like oozing rash all over her face, chest & back, and such intense drowsiness that she was really too tired to do the therapy. But with the lowered dose she made amazing progress, and was able to come home on March 16th '02, after just 3 wks in rehab.

She was still a little unsteady on her feet, dependent on the g-tube for most of her intake, and had lost 8 lbs, but she had stopped drooling, was out of the diapers, and communicating like nothing had ever happened. (They had really expected her to spend the rest of her life in a wheel chair and diapers.) But by her rehab follow-up one month later, she was even steady on her feet again, and my only concerns were some lost mobility in her left shoulder, getting her the rest of the way off of the Phenobarbital, & to get her eating by mouth again.

Part of the problem was she'd refused to cooperate for a swallow study, so we had to put Thick-it in all of her liquids, and then they tasted bad, so she didn't want to drink them. But finally I found another doctor (an ENT) who saved the day. Sometimes you just have to get creative with these kids. He cleared her to drink the old fashion way. She drank a 8oz glass of water for him without so much as a gurgle. Her lungs sounded clear, so he said let her go ahead and drink & eat what ever she wanted for 2 weeks, and then take her in for a chest x-ray to make sure. And her lungs were actually clearer looking on that x-ray then they'd been in the one a month earlier at her cardiology follow-up.

It took us until the middle of June to finally get her totally weaned off of the last of the Phenobarbital, but every time we lowered the dose, we would see an improvement in her within about a week. And once off it, Philina's appetite started to improve, so she is eating orally again. We have therefore, slowly lowered her tube feedings from the initial 8 cans/day down to just an occasional can as a supplement now. And Philina always let's us know when SHE feels she needs one. With this arrangement, she has managed to regain all of the weight she'd lost, but it took her about 3 yrs. In retrospect though, I really have to wonder just how much (if any) was brain damage, and how much was just another bad side effect from the Phenobarbital.

We also started her on some recommended exercises for her shoulder while soaking it in a hot tub. And she can now reach over her head and behind her back with it again. She's a very happy camper too, because her new hot tub sort of made up for not getting to go to her swimming lessons any more.

She had returned to her school and bowling league in April of '02, but her respite camps took about another year. Her heart is working beautifully. And since she was weaned off the Phenobarbital, she's only required the antibiotic prophalaxis before dental check ups, some topical items, nutritional supplements, and 1 chewable children's aspirin daily as a precaution for the Moya Moya. (Her sister also weaned herself off of all the drugs that the psych hospital had put her on, and was so much better without them, that her psychiatrist declared her miraculously cured.) At least for my girls, "less has always been more" when it comes to drugs. It's not that I don't believe in them, I mean I did use to be a medication treatment nurse, but I just don't believe in giving anybody anything they don't really need.

At 22, our Miss Philina is an odd mix of age and developmental appropriate. She likes "Kim Possible" and "Arthur" cartoons, but then again, the last movie she saw with her sister (and I hear loved), was "The 40 Year Old Virgin". She "reads" (looks at) "Cosmo" and children's musical books. Top videos favorites are "The Little Mermaid" and "Encino Man" (and she does a pretty fair Pauly Shore "excellent weasle" impersonation too). Music choices range from a "Rug Rats Movie" sound track to The B 52's, Oingo Boingo & The Cranberries. That is when she's not tuning in golden oldies, salsa, country classics or cool jazz on her clock radio....

Philina "graduated" (with a certificate of completion) from Escondido High School on June 8th, '05, and she was the culmination of a family tradition. Her grandpa Phil was our 1st alumni (class of '45), and her dad was a member of the class of '65. Notice the 60 yr stretch and all those 5's? Not only that, but her full name is also made up of a combination of both of their's. You'd almost think we actually knew what we were doing!

While she'll never be really independent, I do still want the fullest life possible for her, so her Regional Center worker and I selected an appropriate adult day program for her run by the local United Cerebral Palsy. (It's virtually identical to the one she just left at the high school, except with better hours.) So she began attending it in the fall of '05.

I'm sure it's obvious that life hasn't always been easy for her, or us. But she is living proof that doctors don't always know how things are going to turn out. She was never suppose to accomplish any of the things she has, and even her life expectancy was only 3 years tops. But I think that the most amazing things about my girl are her resilience & determination. Not only did she beat the odds, she beat them twice.