Ruminative Ponderings - There's Life Jim, but not as we know it...

Written in Melbourne 2009 as a contribution to a home education magazine ...

My expectations of what life would be like by this stage in my life; middle aged with two adult children, and the actual reality of life at present are poles apart. Aspects of the future I had imagined have prevailed including the happy fact that one of my children is at university and is making an independent life for himself out in the adult world. Following on from this is where a big chasm between the imagined and the actual presents itself. Standing with me on my side of the chasm is my wonderful daughter. In my past imaginings I thought that by now she would either be at university, working or at least be living a reasonably independent life. But there are massive barriers standing in her way and keeping her on my side of the rift. By now, I also thought I would be free to do what I liked, to follow my dreams, to play in a sandpit of my very own making... but for both of us, our current reality is something else entirely and probably isn't going to change any time soon. This is a story that explores the journey to our here and now.

It has been a hard, emotional, challenging, frustrating, rewarding, enriching, wonderful, at times terrifying and life altering 20 years. Would I change any of it? Some of it maybe, perhaps not home educate? Once; after shifting from Hamilton to Wellington, the answer was yes and the kids went to school... but not for long! In retrospect, knowing what I know now and beginning to understand the complex picture that is Geri, the answer is a resounding NO. Hindsight can be a wonderful thing!

Take Home education. It is a big part of the reason I am the person I am today. Because I home educated, I learned to believe that my instincts and convictions matter. I learned to listen to them and follow them. I learned to look beyond society's prescribed norms and how to work my way around the barriers they can present. I learned to question and question and question again until I found an acceptable answer. I learned to understand what really matters and to persevere and fight for it. I learned tenacity. Without being a homeschooling mum I doubt I would have developed the strength and the skills to do what I have had to do over recent years; which simply put, is to save Geri's life.

As I ponder, I think that the biggest question that floats to the surface for me and that I'd like to address here is “What if? This question applies to both Geri; 20 and Pat; my son who is 24.

What if I had ignored the instincts that told me my deeply unhappy son wouldn't survive school ; quite literally, or at the very least, wouldn't survive with his true self in tact? What would have happened to Pat's wonderful spark of invention and his ability to find creative ways to solve technical problems?

Pat was a challenging child from day one. He was a multi side peg that the system simply couldn't accommodate in the few holes it had to offer. He was, and still is our 'mad scientist'. I can still remember with great clarity (yes, I still have glimmers of it every decade or so) the day that my friend Cally sat in her van; as she was leaving my place after a visit, and informed me that I would end up home educating Pat who was about 2 ½ at this stage. My response?

“NO WAY!!!”

Well guess what? Yes, she was right. We did try Kindy for a while but it became apparent very quickly that it wasn't going to work and we found that home education quickly became our preferred norm. Pat's foray into the world of school at around 8 was an experience I would never want to repeat. He was bullied mercilessly and the Pat I knew withdrew from the world and then disappeared. His unique qualities were extinguished by school and took 4 years to fully return once he returned to home education.

Home education gave Pat the opportunity to play... primarily with science; which was and still is his passion. Over the years we did the basics; maths and english, on a regular basis. He was a late reader so when he was little, I would read to him for hours every day. However I stopped truly understanding what I was reading to him (it was nearly all science) when he was about 5 years old. We'd have to read yet another chapter in order to answer the questions he was chucking at me. We never studied science formally, we simply read, played and experimented. When we eventually extended the garage to make a bigger workshop it was built extra strong, as bangs and 'letting the steam out' of various pieces of equipment and devices were regular occurrences. Our elderly neighbour, on hearing a blast or boom learned to merely open his back door, peer over the fence, and raise his eyebrows with a grin to confirm that it was simply Pat at work. One year these neighbours came over for a drink on New Year's eve. The culmination; and absolute highlight of the evening, was watching Pat and our frail neighbours hooting, crowing and jumping up and down with glee. Their faces were split with the biggest grins ever as the three of them shot Pat's spud gun off out over the valley... ... it was only loaded with packed paper, but was still lots of fun!

Pat is proof that an unconventional education can work. He never did any school exams, but we built a strong alternative portfolio from various sources; Open Polytechnic, Boat Masters Certification, Duke of Edinburgh's Award etc. When he was 18 he was admitted to Victoria University on a part time provisional entry. He did really well, but after that year's finals he decided that university wasn't right for him at that time. For the next couple of years he worked at a Montessori college as a computer tech and mentor, then went to Weltec to do an NDE in electrical engineering. Weltec employed Pat on a part time basis during the second year of his NDE, and after graduating he continued with them in a multifaceted role as a technician/tutor/student. He is currently studying towards his degree at another university and has once again been offered a part time tutor support role there, as well an continuing to work at Weltec in his holidays. Through his contacts at Weltec Pat also landed a stint as a technician for Stone Street Studios on James Cameron's movie Avatar and has recently been done the special effects for a number of music videos.

Until recently, I had always thought that Pat was the most challenging of my children and in many ways he was – those who knew Pat when he was a little guy know what I mean! Looking back I suspect he was actually the training wheels for what was to come.

And so to Geri...

What if I had left Geri in school to sit at the back of the class and miss mostly everything that was said and was supposed to be seen?

What if I had believed and accepted the opinions of various educational 'experts' when they gave me 'that' look and told me I was the cause of Geri's learning problems?

What if I had left her in school whilst she become progressively ill and accepted the statement from her teachers that Geri was always sick because she had no immune system due to being home educated?

What if I hadn't learned to ignore 'that look' – you know the one... the 'oh, this is an over anxious, over protective, pushy, paranoid, etc, etc, etc mother' look. What would have happened to Geri if through my years of experience home educating I hadn't learned to be a stroppy cow who trusts her instincts and could turn away from teachers and medical specialist after medical specialist over and over and over to continue my search for answers because I knew they were wrong?

The answer is very simple...Geri would have learned to fail and she could have died.

Geri went to school for a few years whilst Pat was there. It was never a comfortable fit, with my concerns about her vision, hearing and learning being ignored. We pulled her out after Pat had been back out of school for 6 months; she couldn't see why she had to go whilst Pat didn't, which was fair enough! Geri was also suffering from illness more and more often which which meant she was missing ¾ of the school year anyway. My husband Chris and I also realised that all of the effort and energy we were expending in trying to get her issues recognised and addressed through the system would be better invested directly in her on a one to one basis at home.

Geri was also a late reader who at school, and had already learned that she was a failure. She couldn't see the print in most of the readers provided so was left with 6 readers to use over and over for a whole year. She was told she wasn't allowed to write any more animal stories; which were her passion at the time, so she simply stopped writing all together. Once she came home I sourced books for her by her favourite author; Dick King-Smith, in a print size and layout that she could cope with and then I found the same books on tape. I left her alone with them so she could simply enjoy the stories with no pressure to read. She completely immersed herself in the tapes and 'read' the books along with them for the next six months ... and taught herself to read.

Over the following years whenever I tried to address her health and learning difficulties, I got a truckload of 'that look' and was told it was all because she was home educated. We soldiered on and with a lot of hard work she did ok until she was about 14 - 15. I was acting as her 'reader' with her school work by this stage (she was doing 4 subjects with the Correspondence School) as she was having real trouble understanding the booklets when she attempted to read them. We also had to balance any extramural activities Geri was doing very carefully as illness was still an issue as was exhaustion, and it was very apparent when watching her attend a dance classes for instance, that she did indeed have learning issues. It would take Geri 10 hours of hard work and practice to learn and remember a move that would take the kids in her class 10 minutes to pick up. To help her with this I started videotaping her classes so that once she was at home she could watch the lesson as often as she needed to to understand it.

Because of these issues it was much more difficult; if not nigh on impossible, for Geri to put together a portfolio as Pat had done so we decided to try the more formal NCEA route. At 15 ½ she was doing NCEA L1 and educationally we hit a brick wall. I realised there was no way that she'd ever be able to read through and fully comprehend an exam paper in three hours, let alone finish the exam. So we pulled the plug and I explored other options for her. I found a STAR course at Whitireia Polytechnic. It didn't take long for them to say that they thought there was a learning problem... Oh! Really!!?? Well! Blow me down!! But they didn't point the finger at home education or at me. They got their disability coordinator involved and were fantastically supportive. She had a learning assessment which pointed the way toward information processing problems.

This began a cascade of discovery which almost drowned us over the next year. As part of the testing I took her to a new audiologist to have up to date hearing tests. She'd been under the care of an ear specialist since she was 4 due to problems with ear infections and deafness. He had responded with 'that look' whenever I had raised concerns about her hearing, so I decided that there was no point in flogging that particular dead horse any more and changed tack.The audiologist discovered that she had sensory neural hearing loss and suspected an auditory processing problem. She sent us to a specialist audiologist. At the beginning of our first appointment we got 'that look'...you see the thing is, Geri is a very bright young woman and when you meet her you'd never guess that there are problems - she fools people! The audiologist started testing and then slowly turned around to me with shock on his face and said,

"Oh!"

When he had finished the testing he reported that she had a rare form of auditory disorder on top of the deafness. The audiologist asked us,

"Does she work really, really hard but nearly always fail or underachieve? Does she easily get stressed and anxious in new or busy, noisy places? Does she get very tired?"

YES! This is Geri in a nut shell. Then he asked ,

"So she was home educated?"

Well, here we go I thought, get ready to duck 'that look'! He continued with,

"Well, she is as articulate as she is because she was. You've done a great job!”

He explained that Geri's brain works many, many times harder than normal just to get by and to function out in the world. When she is trying to learn something new, is in a new place with new people, is in a bad hearing environment etc it gets overloaded, stops coping and she gets confused and tired etc. He said it has no built in redundancy because it is running at full capacity all of the time just to get by and that it has been compensating like mad all of her life, but now it had run out of capacity to do so.

In the meantime I took her back to the excellent optometrist I had just found for her, to have a current check up. I told the optometrist about the learning assessment, during which Geri had asked,

"Isn't it normal for the words to move around on the page?"

What??!! We knew she had reduced corrected acuity; she wears a +11.5 correction. I had also been bleating my head off to her ophthalmologists forever that she wasn't seeing well and had been raising concerns about her weird looking eyes since she was 2 weeks old, but got ignored.

The optometrist said,

"You do know that she probably has a developmental syndrome?"

What??!! So the optometrist referred us to yet another ophthalmologist (the 4th she'd seen) and also a behavioural optometrist. The ophthalmologist confirmed that she did have a genetic developmental condition; which is very rare, though he thought it was Axenfeld-Rieger Anomoly which affects only the eyes, not A-R Syndrome which affects other areas of the body as well as the eyes. Because of the way A-R affects the formation of the eyes, she had been at very high risk of developing glaucoma and had been a ticking time bomb! All of her previous ophthalmologists had missed this condition and ignored my questions and concerns.

Next was the behavioural. optometrist.... who initially gave us 'that look'...yup, she fooled him too! Then he started testing..... and it was his turn to turn around and say,

"Oh!"

It turns out she has significant visual processing problems; for instance her brain only 'sees' about 15% of what she is looking at. Then he asked

"So she was home educated?"

Here we go again... get ready to duck 'that look'! But he went on to say,

"There is no way she should be as literate as she is and she is because she was home educated!"

Geri's ill health began escalating in a frightening way throughout this assessment process and both the audiologist and the bevavioural optometrist asked if there was a way to ensure she wouldn't be so sick for the duration of the testing. The only answer was to withdraw her from the STAR course. The polytechnic understood and agreed that the most important thing was to get the puzzle that is Geri sorted.

About this time I finally realised and accepted that Geri wasn't going to 'get better' in regard to her ill health no matter how long I waited for it to happen, and that I had to do something proactive about it because she was getting sicker and sicker. Due to previous experience I trusted doctors about as much as I trusted the school system, but with the recent success we'd had I felt the time was right to take on this new fight. I took her to our GP and opened the Pandora's box that I knew would be the reality of trying to find out why she was sick. As I live with ill health too, I dreaded having to lift that lid...I knew what this battle was going to be like. But what else could I do?

We took Geri to see an immunologist in Auckland as there wasn't one in Wellington at the time. He ran lots of tests, was magnificently dismissive and concluded there was nothing wrong – even though we reported some improvement in the symptoms she had at that time with the largish dose of antihistamines he put her on at her first appointment. His explanation for this was that she must be imagining the improvement because all of her allergy tests were negative, therefore the antihistamines could not have made any difference at all. He never questioned why the antihistamines had improved things even though she had no allergies...

Geri's health went from bad to worse until by March of her 18^th year, she was bedbound and verging on suicidal. Her abdominal symptoms had worsened dramatically and additional symptoms had appeared. Her fatigue and dizziness had intensified and she had started sleeping 16-20 hours out of 24 and was a zombie for the hours she was awake, her fragile appetite had disappeared, an intense urticarial rash had appeared around her eyes accompanied by angioedema and she developed flushed areas on her chest and face. Her eyes became very dry so that she could only wear her contact lenses very occasionally. Her lips would swell so much they would crack and her nose became swollen and blocked up. She started having sudden collapses with a racing heart beat and numbness in her extremities.

These symptoms followed a pattern and when on an upward cycle she would be jumpy and agitated and sometimes aggressive. She developed cognitive problems (confusion, couldn't read, couldn't remember how to write, spatial judgement disappeared, felt spaced out... etc). She had periods of blurred vision which meant she couldn't read, use the computer or watch TV. She also developed generalised urticaria from the sun, heat and showering. On some days, when things were settled, she could be almost normal and then it would hit her out of the blue again.

She saw a Dermatologist who made a number of snap diagnoses (all three were wrong). He also took over 6 months to accept that her symptoms seemed to be linked to each other and stated that,

“There is no illness that has all of these symptoms and if there was I'd know about it.”

He finally put her on steroids which only just controlled her symptoms. The steroids gave her severe acne and were dangerous for her because of her already increased risk of Glaucoma with Riegers.

We had just gained an appointment with Wellington's only immunologist (9 month wait) when we moved to Australia partly for Chris' work but mostly to look for new doctors as we felt we had exhausted all possible medical avenues in New Zealand. We consulted yet more specialists in Melbourne; a Rheumatologist who drew a blank and said Geri was a true enigma but agreed that she was indeed very ill, and an Immunologist/Rheumatologist/Allergist in private practice who followed the same ilk of the immunologist in Auckland. He was the first doctor to mention Mastocytosis and ran more tests; which were all negative, and tried yet more drugs which didn't work and caused some frightening side effects. After he stated that the rash and swelling around Geri's eyes was caused by steroid withdrawal I challenged him and told him yet again that the rash predated the steroids by at least 6 months. His response was to flippantly tell us that there was nothing serious wrong and that it wasn't life threatening. He referred us to a research immunologist at the Royal Melbourne Hospital. At least we have this to be grateful to him for if nothing else! By this stage Geri and I were in an incredibly fragile state and I just didn't know how many more times I could pick us back up to continue the fight if we were dismissively told by yet another doctor as he gave us 'that look' that because the tests were all negative Geri simply wasn't sick....

After a delay caused by the referral disappearing into thin air a number of times we finally obtained an appointment and saw Geri's new doctor. I handed him my pile of notes and we slowly went through Geri's many and confusing symptoms. We talked about her past medical problems and he stated that at any time in the last 19 years she could have died. He said he needed to run a pile of tests... and when he caught me rolling my eyes he said,

"Yes, I know they'll probably be negative but I have to run them! Just because they are negative doesn't mean she's not sick, just that we don't have the right tests yet and that we aren't good enough!! We won't have really reliable tests for this for another ten years...."

Oh! It was music to our ears, and yes, all of her tests were normal.... he told us that some doctors call it MCAD (mast cell activation disorder) but in his opinion that is semantics and that as far as he was concerned it was most probably a mast cell illness which presents the same symptom picture as Mastocytosis (yet another very rare condition)Then he looked Geri in the eye and very firmly said to her,

"You are not mad..."

This doctor is our hero!! He is honest and frank, a very caring physician, has a wonderful sense of humour and he connects with Geri as a human being. There is no cure, and successful treatment is difficult, but the monster now had a name so we could stuff it into a box, put our boots on and kick the box around with glee when Geri felt well enough to do so! She now sometimes has 1-2 good days a week where she can leave the house for a few hours, instead of 1 day every 3-4 months.

In fact one day Geri wanted to go out. I couldn't as I was still getting over a virus we'd just had. Since starting treatment for her illness, her life long problem of getting extremely sick from virus' has eased and she was fed up with being stuck at home. She took a deep breath and announced that she was going to go for a walk! Now, this was significant because she hadn't been well enough to be out without someone with her for a very long time. It's also a big deal for her to be go out alone even when she feels well because of her hearing/vision issues. Having her mast cell illness on board as well makes it is almost impossible. But, she did it! Her battery banks ran flat really fast though, so she was wiped out for the next week but the look on her face when she walked in the door was simply wonderful. This sort of event provides the rare glimmers of hope that keep us going when the going is tough and Geri's illness gains the upper hand.

About 16 months into her treatment, Geri underwent a bone marrow biopsy. This was the first test she'd had through all of this that showed something abnormal. It apparently tells her doctors something but they aren't entirely sure what yet! It is a very grey area. This result seemed to move her medical team further along the Masto treatment protocol road. Just prior to this they had moved the goalposts on us and were querying their approach and diagnosis. This was pretty upsetting for us as up to that point they had only been talking about her illness within the context of misbehaving mast cells. However we now realise that they were simply being very thorough and were exploring every possible diagnostic avenue open to them. The doctor's current stance is that they can't give us a formal diagnosis of Mastocytosis because her test results don't conform with the WHO diagnostic criteria, but that that is what they are treating her for. They've said that it can be very easy to prove someone has does Mastocytosis, but that it can be very hard to prove that someone doesn't have it as negative tests don't necessarily mean no Mastocytosis.

Just before we saw the immunologist I had found a support group for Geri called ChIPS; this stands for Chronic Illness Peer Support. She was able to start attending occasional meetings once she'd started her medication regime and her illness had settled a little. Through this group Geri has been able to make a few friends and even attend a 3 day camp. The great thing about ChIPS is that all of the kids involved are sick in some way so they understand what it is like to feel unwell and to be tired etc.

We've also made some progress in finding out more information about Geri's Axenfeld-Rieger. Via an online group I belong to I was able to contact another A-R family here in Australia. They recommended a Geneticist at the Royal Children's Hospital who; when I made contact, was very interested to see Geri even though she was an adult. He confirmed that most of the 'odd' stuff of Geri's that I had for years suspected of being linked to her A-R was indeed attributable to it. He said she definitely had the syndrome; which involves multiple body systems, not just the anomaly; which only involves the eyes, as had been stated by the Ophthalmologist in NZ and that her general appearance is consistent with the very few A-Rs that they see. He called it Riegers Syndrome.

The things that we can now place in Geri's RS box are: Eye formation abnormalities, poor vision, high glaucoma risk, sensory neural deafness, unusual facial composition; eyes wide set, small, prominent and back angled, flattened front to upper jaw, short upper lip, asymmetrical face shape, long fingers; arachnadactly, very small feet for her height; she's tall for RS as they are often short/small - but he said she is short/small compared to the rest of the family, underdeveloped musculature, lack of coordination, possible myopathy. He wasn't sure about brain involvement and her processing/learning issues. However I think with the improvement we've seen with her masto meds it is likely that her mast cell illness may have been a significant contributor to these problems all along. 

So, she has a mild; but a fairly wide spread, expression of one of the RS genes. The geneticist thinks she is a new spontaneous mutation (well we always knew she was totally original and one of a kind!) so didn't inherit it, but can pass it on as it is a dominant gene. He contacted a researcher in America and they agreed to add Geri to their study, so her DNA has been sent over and we await any further information that may result from that.

We are still hopeful that we will be able to get Geri's illness stabilised to the point that she can plan ahead a little and perhaps manage some regular activities. The latest addition to her medication regime; which was introduced after her bmb test results, has seen a dramatic improvement in her cognitive function. Although it is an unstable improvement; as are most of the improvements that her medications have brought about, it is still very exciting. (This has since been discontinued due to a bad reaction to it, but may be tried again in future)

Amongst other things, home education especially helped equip Geri with some of the tools that she has needed to survive this challenging journey, chief among them: self belief and self reliance. She also has a rare wisdom for her age. Now that her health is a little more stable she is cautiously starting to think about the future. One of her interests is drawing and is the only activity she has been able to sustain throughout her ordeal. She now accepts that she does have talent and is starting to make plans involving her art. Her once lost love of writing has reappeared and she has found that ideas for books and graphic novels are now competing in her head for attention. She has teamed up with her oldest and best friend Jeff; via the Internet, and together they are working on a children's book. Once her health is more stable she is hoping to be able to start working consistently on all of the graphic novels whirling around inside her head.

So, our journey continues. We have no idea what the future holds for Geri and I have no idea what it holds for me. Will we ever have the freedom to do what we would choose to do without the constraints placed upon us by this situation? Will Geri ever be able to fly solo so that I am 'home alone'? Who knows. In the mean time I devote my time and energy to acting as Geri's case manager; now there's a job I never thought I would hold, helping her stay focused on looking forward and staying as positive as circumstances allow. Any remaining scraps of my time and energy are spent playing in my own personal sandpit which holds a collection of my interests and passions. I've been revisiting my old passion for photography , have discovered genealogy, am doing a bit of writing and practice TaiChi regularly. I also enjoy pattern making and fine sewing/tailoring. This is another re visitation of an old interest. I used to sew for the kids and was a quilter for a number of years. I've always been interested in the technical side of pattern making and had some private tuition for a few months before we left NZ. Since then, I've been home educating myself and am making steady, if slow progress. I often get frustrated with what I perceive to be a lack of progress or the frustrating pace my circumstances force me to work at. However, before I chuck my toys out of the sandpit I remind myself of what I say used to my kids when they hit the similar barriers as they were growing up,

“Life's not a race, it's the journey that matters.”

I have discovered the benefits of being in control of how I use the 'bar' which redefines who I am and what I can do. Sometimes the bar is so low that it is almost on the ground, and that's okay. I've learned that when I feel the time is right the bar can be raised, but cautioulsy. This enables me to step over it with one foot whilst keeping the other foot hooked under that bar so that I don't lose sight of it. If the bar is put up too high too quickly then it has a tendancy to fall down and crack me over the head. The trick is to keep pushing it up a little at a time so that the goal is attainable.