Tuesday, October 5, 2010

The Medical Side of Josh

Just heard that one of the websites for chronically ill kids that featured Josh back in 2006, MACS (Make A Child Smile), is expecting a big media event soon. They say we may expect a little more traffic looking at our kids' sites with the links to our page there. So, I thought I'd write up a little about what my son, Josh has and how it affects him.

Josh likes to think of himself as a normal boy.
His mind is often on football, basketball, his friends, games or movies. He dreams of driving his own sports car one day. But whether he likes it or not, his rare medical condition always looms over him and limits him.

Everyone thought Josh was perfectly healthy and normal when he was younger.

His first problems were:
sweating more than normal - which he's done since he was a baby,
failing hearing screenings - as early as 3 years of age (they said the trouble was in the high frequencies and he can hear most normal speech just fine),
and a learning disability (discovered in 1st grade when he could not seem to grasp basic math and reading).
Otherwise, Josh was active, rarely sick and usually quite happy.

It wasn't until he was 8-and-a-half that we discovered there was something seriously wrong with him. He'd had a throat infection and finished a course of antibiotics. He would get up and go to school ok, but come home and fall asleep on the couch and not be able to wake back up and stay awake. He was missing out on playing with his friends, going to a favorite church function, and he wasn't eating much. This lasted a few days. I thought he was just still recovering from that throat infection. But after having a bath to try to wake him enough to finish his overdue homework, still wrapped in a bath towel, he collapsed in front of me.

I got him to his bed, realized he was very hot, took his temperature and found he had a fever of 103. I gave him motrin, got him dressed and took him to a minor emergency clinic - thinking he likely needed more antibiotics. Instead, the doctor found his blood oxygen level was very dangerously low (50 percent and it should be 97-100 percent). They called an ambulance that rushed him to the hospital. The next morning, he went into respiratory failure and had to be resuscitated 5 times before getting him onto a ventilator.

Doctors did not find the real cause of his problems until months later and another hospitalization. His diagnosis was a combination of a few things: central hypoventilation (a part of the brain causes the body not to ventilate properly).... because he did not have that particular problem from birth, they called it Late-Onset. But his body was not producing cortisol as it should either. An endocrinologist said he also had hypothalamic dysfunction.

You can see more about how we learned of Josh's disorder by watching the documentary produced for Discovery Channel and Discovery Health. "Life or Death : Battling to Breathe" aired in 2007. I have a taping of it here and on the bottom of my blog page.

The name of his disorder was changed in 2007.

What Josh has is now called ROHHAD (Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation). There are now more than 50 people known to have this disorder in the world and most of them have different symptoms or at least different degrees of severity of the symptoms. Here's how it affects Josh:

His body does not produce growth hormone or testosterone so he must take those hormones by injections. Growth hormone he gets 6 nights a week in a tiny needle just under the skin (sub-q) of the back of his arm, testosterone once every four weeks (in a longer needle deep into the upper buttock area).

His metabolism is slowed down, so that he gains weight much more rapidly than others his age, despite diet changes and exercise. So, he sees an endocrinologist and a dietician frequently, uses fat free milk, sour cream, etc. and we make sure to add as much fiber and reduce the amount of sugar he takes in.

His body does not regulate temperature correctly. If he is in the cold, his body temperature goes down. It's been as low as 96 degrees. If it's hot out, he is also hotter than he should be - usually up to 100 or so with no infections present. So, we take his temperature several times every day to make sure it doesn't get too high or too low. He often has to take ibuprofin or cortef if his temp gets too high. If it gets too low, his hands will turn a scary shade of purple/blue and we have to use hand warmers or blankets, etc. to help warm him up.

His body does not regulate hydration properly. He will sweat often even if its cold, pee out way too much, and he never really feels thirsty when he should. This causes his sodium levels in his body to skyrocket which can be very dangerous/deadly. He takes a nosespray called DDAVP that helps him retain fluids to help prevent him from becoming overly dehydrated. We also encourage him to drink alot. He carries G2 (low sugar gatoraid) and diet green teas with him to school to help the nurse gauge how much he is drinking.

His body does not detect oxygen or carbon dioxide levels. He can physically breathe just fine, but never feels the sensation of suffocation that he should when his body gases are out of whack, so he doesn't realize he needs to breathe in and out bigger. Because this is an automatic thing your body is supposed to do and you can't just sit and think about breathing all the time, Josh needs a ventilator or some other device to force the bigger breaths. He was implanted last summer with diaphragmatic (breathing) pacemakers to help with that. He can use them when he is awake, but needs to be hooked up to a ventilator (respirator) when he sleeps, to give his body rest.

Josh takes a variety of medicines/supplements to help replace nutrients he looses when he sweats or pees out too much and to reduce stress on his body from all the weight and other issues going on inside of him. He takes a low dose steroid called cortef (hydrocort is the generic name), synthroid, potassium, calcium, magnesium, zinc, fish oil, acetyl l-carnitine, alpha lipoic acid as well as allergy meds.

To hook up to the ventilator, Joshua had surgery just before he turned 9 on his neck where he has a tracheostomy tube placed. That tube must be changed weekly or when it gets too plugged with mucus. And it must be suctioned out frequently to try and keep it nice and clear.

His breathing pacemaker has small wires running through his chest that can break easily, so he has to be careful not to get hit in his chest area too hard, as this could break the wires and cause his pacemakers to stop functioning - which would mean he'd have to hook back up to the ventilator all the time to breathe enough to be healthy.

Josh also has that high frequency hearing loss - which means he misses some sounds like "sh", "th", "sk", and "ch." He has hearing aids for both ears to help, though he doesn't feel the need to wear them, and we don't fight with him about that.

Josh requires somebody trained as a nurse, with experience using a ventilator and changing a trach, etc. to care for him basically 24 hours a day.

They need to know about diaphragmatic pacemakers (now) so they can help him with his. When we do not have a nurse on duty, I perform this task. And he'd like it to be 24 hours a day - but that is just not possible.

Josh contracted H1N1 swine flu January 2010 and developed double pneumonia.

His health since that time has not been as stable as it was before.

He had been able to balance everything pretty well and stay out of the hospital mostly. But he has been in the hospital a few times this year. And he has to take breathing treatments called IPV (Intrapulmonary percussive ventilator) now 3 time a day.

Our goal is to get and keep him as healthy as possible while helping him live as close to a normal lifestyle as possible.

Sunday, October 3, 2010

I'm a student again


You may have noticed I haven't had as many posts lately here on the blog. That is because I have been even more busy than usual - I'm back in college.

This semester, besides working nearly full time at a local daycare and keeping up with the bills, laundry, dishes, trash, etc. at home, I am also taking two of my pre-requisite classes I need to get into the respiratory program at St. Phillips College next fall.

I am taking College Algebra and Human Anatomy & Physiology.

Since I have not seen the inside of a college classroom in 15 years, I am really having to put my nose to the grindstone to master this material. But I think I am doing pretty well. I have made 100's on my math quizzes and on my first lecture and lab exam in A&P, I made a 101 on each! I took my first math exam on Thursday, but have not seen my grade on it, yet. I do know I missed at least one of the 17 problems on the test because it dealt with something that we did not specifically cover in that class, but something that we should have remembered about graphing from a previous math course (which I had not taken since high school).

Here are some of the interesting things I'm learning about in my classes so far:

And here are some of the subjects we've covered in Anatomy & Physiology:

Words used to describe body areas and position, etc.

Body Cavities (areas in the body where different organs go)


and now we are working on the skeletal system


vertebra (spine)

ribs & sternum




I have a big exam on all the bones coming up Wednesday, Oct. 6th. Wish me luck!

I really enjoy taking Anatomy & Physiology because I can use what I'm learning as I study on the medical problems Josh has with his ROHHAD. I learn the medical terminology and functions more specifically. I'm all for learning as much as I can to help Josh and others with this crazy disorder.

Josh is 14

Wow, 14 years old!

We planned the details of Josh's birthday party for several weeks. However, it did not go off the way we planned.

Josh wanted as many kids as possible to come to his party ... which was to be held at the same park where we had his last two parties ... so they could play all the football, basketball and baseball they liked.

One of his nurses had recruited Pizza Hut and Starbucks to help supply the food and drink.
We picked up the decorations. And we had several offers of folding tables to use to set up everything at the park. Invitations were out and we even set it up as an event to invite friends on facebook to come. It was all coming together and Josh was super excited.

BUT .........mother nature did not play along. We were watching the weather forecast and the sky as the time neared to go out and set up at the park. At one point, it said there was a 100 percent chance of rain for the time of the party and the next hour. And it started pouring before we could even get out the door to start toward the park. So, we contacted everybody we could and let them know we would change the location and have the party here at the house instead. Josh was not happy to have to ditch the park and all the sports he was ready to play with his friends.

My sister and I worked relentlessly to get the house ready for a party that we had not planned to have here... and just in time, it looked like we had planned to have it here all along.

We picked up the coffee, etc. from Starbucks (a huge container full with a sleeve of cups, lids and all the different sweeteners you can imagine plus a half gallon of half n half).

Another of Josh's nurses arrived and brought with her a very large, beautiful birthday cake she had worked on for hours for him.

Then, the guys from Pizza Hut popped in to unload several large pizzas, tins of pastas, breadsticks, sodas, plates, cups, etc.

And our guests got here despite the downpours outside. Josh had a few boys his age show up, his nurses and their families, some great friends from church and some neighbors as well.

We all ate and drank well.

The kids had a great time throwing a little soft football around.

Josh received several nice gifts.

And we all had a great visit.

It seemed the party was a huge success even if it couldn't happen at the park this year.


Josh and I worked on some thank you poster cards for Pizza Hut and Starbucks.
Here are a couple of pictures we took of Josh to use on those cards:


Besides the party, I had hoped to be able to give Josh a gift he has been wanting a while - his own laptop computer.

He has a pretty severe learning disability. But he loves to look up information on the computer about pretty much anything he learns about in school.

He had been trying to save money from selling cookies, etc. but still had not collected very much.

So, I put out a request on a local yahoo group called Freecycle for a used laptop computer.

I did not get any responses back before Josh's birthday, so had given up on receiving one for Josh.

But more than a week past Josh's birthday, I was contacted by email. A lady asked if I'd ever gotten a computer for Josh from a fellow freecycler. I told her I had not. And she said she and her husband had one they were no longer using. She would ask her husband to get it prepared and would bring it to us for Josh.

They did and brought it over yesterday (Saturday, Oct 2nd).

Josh was still asleep when they came by with it. My husband, sister and I started looking at it to make sure it would be able to get on the internet and everything. We loaded programming into it that would allow Josh to get online wirelessly. And we found that this was one of the nicest computers we'd seen. The couple even gave us their nice leather case for it.

The wife told me that her husband had walked in telling her they should think about getting rid of that computer because they no longer used it right as she'd noticed my post about wanting a computer for Josh. We decided it was God's moving that brought this all together.

Since Josh's birthday was already 2 weeks behind us, I thought about holding the computer as a Christmas present for Josh. But after looking it over and getting it all ready for Josh, I was too excited to wait. I went up and woke him. I told him we had something for him downstairs, but he had to do his breathing treatment, take his medicines and get dressed before he could see it. He did all that and was so thrilled to see his gift. It was kind of like Christmas morning!!!

Discovery Documentary - Life or Death : Battling to Breathe