"Bad is never good until worse happens"

Truer words have never been spoken! I completely understand that in life perspective is everything. There is always something worse than what we are facing. We cling to, "it could always be worse," in times of struggle and somehow it makes our bad seem a little easier to bear. When worse happens the process starts over; lowering our expectations, reevaluating our hopes and dreams, adjusting short and long term goals, surviving through the grief and convincing ourselves that "worse" might become just bearably bad in time.... but always hoping that we don't ever have to find out how much worse it could be. No one wants worse!! But I'm just rambling...

My little princess has had more than her share of bad; many thousands of finger pokes and needle sticks day and night since infancy. A constant infusion of insulin is required to keep her alive, though not adequate to spare her from the endless highs and lows of type 1 diabetes. We fear the very real dangers of life threatening DKA and hypoglycemia every day. For a young child who makes no insulin there is little room for error and it is a full time job to keep her safe and happy and healthy. There are carbs to be counted in every bite of food that passes her lips, calculations to be made for illness, stress and excitement... even normal growth, and extreme caution with exercise which can drop her dangerously low in an instant. It's pretty bad as far as chronic illnesses go and seriously labor intensive since it must be managed around the clock. It's expensive to treat and life changing for everyone involved. There is no cure, but with new technology and medical advances these kids have a great long term prognosis and can lead relatively normal lives.

A few months after her diagnosis with D Emma began having severe GI problems. She had chronic diarrhea, her stomach was bloated and hard and she would start vomiting for no apparent reason, then stop eating for days on end. It would've of been bad enough in an otherwise healthy one year-old, but in one with D it was serious enough to land her in the hospital for days at a time. She was admitted numerous times for dehydration combined with "acute anorexia" and large ketones in the year following her D diagnosis. I expected Celiac was the likely cause, but I was not prepared for the news that she had "very strong" auto-antibodies for Crohns Disease, and ~if~ this wasn't the beginning of it, she had an 80% chance of full blown disease activity within 5-10 years. Wow. Full blown Crohns disease in a five year-old? A five year-old with type 1 diabetes? Really? The pedi GI seemed as stunned by the results as we were, but her intestinal biopsies looked normal so we waited... She had a few more severe episodes of vomiting and refusing to eat that required lengthy hospitalization, but eventually she started to improve. We are still waiting for that shoe to drop, and hoping that those lab stats are wrong.

We started her on an insulin pump around 14-months, just before she started to walk. The stiff nature of her steps each morning didn't alarm me until it became clear that they were very different than her normal steps later in the day. Thinking back, I don't think I've ever seen her walk out of her room in the morning without limping or shuffling down the hall. There were days that she would simply refuse to walk at all and revert to crawling. (She still does this on particularly bad mornings). She had endless problems with twisting her ankles or taking "a funny step" that would cause her severe pain and send me running to her pedi for help. She always improved and went on as usual, but watching her try to walk after sitting in her car seat became gut wrenching, and the mornings only continued to get worse.

I asked her pedi about the possibility of JRA when she was only 16 months-old, she wasn't officially dx'd by a pedi rheumatologist until just before her third birthday. The only problem with the JRA (Juvenile Rheumatoid Arthritis) diagnosis was that by the time she got it she had numerous other issues going on in her little body. She was having almost daily fevers, fatigue, bizarre rashes, turning blue when she was cold. She had huge, hard lymph nodes that had to be surgically removed from her neck. She was having problems with severe high blood sugar anytime she spent time in the sun, and she would be sickly for days if we tried to take her swimming outdoors. She also had a horrible look that came and went depending on how she felt; dark circles, sunken eyes, poor color in her face. I was certain that there was more going on than JRA, and I presented all of my findings to the pedi rheumatologist that we had waited so long to see. I questioned him about Lupus and Stills disease, but he arrogantly and adamantly refused any possibility of it. He started her on high doses of NSAID's to treat her arthritis and promised that she would improve quickly. She didn't.
She did, however, take high doses of NSAID's and low doses of chemo, (that he kept increasing because she wasn't getting better), for many months before I insisted that something was very wrong and that I wanted a full work-up from her local pedi before we upped her meds any further. I had been complaining to every Dr. we saw that her feet were swelling up like balloons and her BP had been high at her endo appointments, (the only place they ever tested BP on a 3 year-old), but they assumed the foot swelling was related to JRA and the elevated BP was a fluke or simple white-coat anxiety. That workup was the first time she'd had a simple urinalysis done in over a year and the startling results would change everything. Her kidneys were in serious distress, spilling out huge amounts of protein and blood into her urine and she was in real danger.

She is now facing a probable diagnosis of Lupus (SLE) after more than two years of disturbing symptoms. Her labs are finally starting to match those symptoms, and combined with her A-I kidney involvement her new doctors are taking this very seriously. If you are not familiar with Lupus in young children, let me assure you that it qualifies as worse than anything we have ever faced, and combining it with the already bad concerns of type 1 D makes it unknown territory for all involved in her care. It is becoming more unlikely that her arthritis was ever truly JRA, but simply one of the many signs of Lupus, and the high doses of NSAID's she has taken certainly contributed to her kidney issues. I plan to have a lengthy discussion with that rheumatologist...

Emma had an MRI today to finally decide the severity of her arhtiris and help the docs decide on further treatment. It is within the realm of possibility that she has ANA positive JRA, combined with autoimmune kidney disease (IgA nephropathy), combined with autoimmune diabetes, combined with other various autoimmune issues that look like Lupus but aren't... anything is possible I suppose.

The medical description of Lupus:
"SLE is a chronic, inflammatory, multi system disorder. It presents with a wide scope of symptoms and signs due to auto antibody mediated tissue damage. SLE can cause arthritis, fever, skin rashes, nephritis and damage to vital organs such as the lungs, heart, kidney, and brain, leading to organ failure and death. Renal involvement in SLE is common and often predicts poor outcome."
The prognosis:
"Although survival in childhood lupus has improved, complications of therapy result in significant morbidity with distressing frequency. Immuno-supression may result in mortality and morbidity due to opportunistic infections. Individuals with otherwise successful control of renal manifestations of systemic lupus erythematosus may still be left with significant morbidity due to disturbances in growth due to long-term corticosteroid treatment. Psychosocial development may be adversely affected both as a result of chronic illness as well as due to the effects of therapy. Meticulous attention to detail over decades of treatment is necessary to optimize patient outcome in childhood lupus nephritis."

Unfortunately, the primary treatment for Lupus nephritis (or IgA nephropathy) is chronic steroid therapy which has profound complications for a child with diabetes. Without this treatment the survival rate of Lupus is grim and the progression of IgA is certain. With aggressive treatment using a combination of steroids and immunosuppression you have a good chance of survival, and possibly even a relatively normal life.... other than the fact that you have a weekend immune system that leaves you open to severe infection, you are short and round, have brittle bones and are most likely sterile. Non diabetics often become type 2 because of the severe insulin resistance caused by long term use of corticosteroids and require high doses of insulin. The complications of the treatment are not appealing, but in most cases they are better than the alternatives.......
In an insulin dependant child, who is already at risk for nasty complications if blood sugar is not well controlled, the prognosis is frightening and uncertain. There are few long term studies on Lupus, none that involve children as young as Emma, none that involve type 1 diabetes. I assume it's probably for the best that I don't know how much worse it could be.... worse totally sucks!