Sam's Condition (just the bad stuff)

I had to write this for a social worker.  It was hard.  So I thought I'd post it, because it felt like a challenge like this should be memorialized - or something.  Either I'm just feeling sorry for myself or else I'm really proud of my ability to spell arcane medical words.

Sam Forsberg is a 29 year old boy with the mental capacity of about an 18 month old.  He is about 4 1/2 feet tall and, on a good day, weighs 80 pounds.  If he goes too much below 75 pounds he gets sick from being too thin.  He has aphasia (which means he can't figure out how to talk) and dysphasia (difficulty swallowing).  He also has a very high arched palate which may be part of his swallowing difficulties.  He has bi-lateral choroidal coloboma (which means a hole in the back of both of his eyes) and strabismus and nystagmus (which means his eyes wobble up, down and sideways).  The last time we took him to the eye doctor, she said he also has some astigmatism.  He has a certain degree of ataxia (which means he shakes or trembles a lot especially when tired.)

All of this is because something went wrong about a month before he was born.  Maybe he had a stroke.  He was born with brain damage in the back part of his brain.  He can't walk because he has damage to his vestibular nerve and so he has very little sense of balance.  He isn't very strong, tires easily and has very low muscle tone. He also doesn't have a very good temperature regulator, so I can't take him outside if the weather is too cold or too hot.  He has asthma, so I am not supposed to take him outside on "ozone action days".

He can't chew, which means everything he eats must be pureed or mashed.  According to the dentist, this means his teeth are MORE likely to decay than if he actually chewed, so I have to be very careful to brush his teeth often.  If he swallows something too thin, he chokes and some of it goes into his lungs.  If he swallows liquids that are too thick, he chokes and stuff goes up his nose.  Sometimes things that he has swallowed half way come back up into his nose and then, we think, go back down into his lungs.  He has very extensive nasal polyps because of this nasal regurgitation.

All of his life he has had a lot of congestion.  No one is quite sure why.  He doesn't seem to have any allergies, although he does have asthma because his lungs are so chronically full.  His nose is always running and he often has huge amounts of mucus clogging his nose and throat.  He had almost constant ear infections as a child.  The muscle that is supposed to help his ears drain is either missing or non-functional because of his high palate.  We lost track of how many different ear doctors tried to put tubes in his ears.  (I can think of 5 off the top of my head)  The tubes never lasted long.  But his ear drums have been perforated so many times that he seems to have developed permanent holes in them now, so we don't have as many ear infections.  This is good because he is resistant to some of the antibiotics and allergic to others because he's been on them so often.  Now we try to avoid using antibiotics as much as possible.  He usually gets pneumonia about once a year and we give him antibiotics for that. 

About 6 years ago he had a very bad bout of pneumonia and had to be hospitalized.  At that time we met Dr. Mutlu.  He has been caring for Sam's lungs ever since.  He discovered that Sam also has Central Sleep Apnea  which causes his Co2 levels to rise.  Sam is in compensated respiratory acidosis.  This means his body is working overtime to compensate for the extra Co2 in his blood.  If the Co2 rises too much, he dies.  So bringing down the Co2 has become a central focus of our lives the last 6 years. 

We tried for about a year and a half to get him to wear the bi-pap machine, but finally gave up because I wasn't getting any sleep and started to see snakes in the corner of the room!  Instead, I keep a close watch on his blood oxygen level with a pulse oximeter.  I try to keep it between 87 and 95.  If it gets too low, I have an oxygen condenser at home and I can give him oxygen.  It never gets too high naturally, but sometimes it can get too high with the oxygen condenser.  If it gets too high, the sleep apnea gets worse and his Co2 levels rise.

He has always seemed to sleep best between the hours of about 4 am and 11 am.  I used to dress him in his sleep and take him to the car to get him to school or program.  He was always late.  I would feed him in the car when he woke up.   Dr. Mutlu said this should stop.  It is vital that Sam sleep whenever he can.  When he sleeps deeply, he breathes deeply and this is the best way to bring Co2 levels down.  So we let him sleep.  He often takes a nap in the afternoon as well.  This means that he has been unable to attend Esperanza very often.  When we have a very good day, I bring him to visit for a few hours from about noon to when they close at 2:15. 

In addition to letting him sleep and preparing all his meals carefully so he won't choke or regurgitate, there are also his breathing treatments.  I give him chest PT with a chest vibrator 2 or 3 times a day.  This takes 30-45 minutes each time.  I also give him a nasal irrigation twice a day and nebulizer 4 times a day with medications prescribed by Dr. Mutlu.

I have three recent examples of how necessary all this is.  First: in December my husband had surgery for prostate cancer.   He came home with a catheter and was very weak.  I spent a great deal of time taking care of him.  I skimped on Sam's treatments.  Sam got pneumonia.  Second: whenever I fail to keep all the food and sleep and treatments in balance, Sam is up all night whimpering.  Third: When we went to see Dr. Mutlu on February 22 of this year, Sam's blood gasses were stable!  After 6 years of rising blood levels, and all my efforts to slow the rate of rising, we have finally stabilized him!  His blood levels are still much higher than normal, but this is a great victory.

So, in summary, a brief list of his many diagnoses would include: profound mental retardation, ataxic cerebral palsy, chronic hypercapnic respiratory failure, bronchiectasis, central sleep apnea, asthma, dysphasia and aphasia.  There's probably a several more, but that's all I can think of at the moment.  Dr. Mutlu speculates that he has Joubert's Syndrome.  His small stature is due to failure to thrive in his first year.

Exciting day

Sam and I went to Starbucks today to see are good friend Eileen.  Eileen worked very hard for many hours trying to get Sam to say her name, and he used to say it.  But he hasn't said anything in years apart from the occasional "Sushi" request!

Later this evening we went to CVS to pick up some meds.  Eileen is the manager of a CVS store.  Just outside the CVS, Sam stopped and said, "Eileen"!!!!  Inside I was showing him a toy I thought he'd love, but he didn't seem too interested.  I said, "Sam don't you want this?" and he said, "No."  When we got home I was a bit encumbered with some packages and, of course, Sam.  I stood at the back stairs and wondered outloud, "How are we going to manage this."  Sam said, "Up."

Of course the really sad part is that he has these moments every now and then.  And then they are gone again the next day.  But it was really great while it lasted. 

It confirms in my mind that he really does know what's going on outside his head, he just doesn't remember how to talk very often.  One doctor told me that is very common among stroke victims.  His theory was that Sam suffered a stroke just a few weeks before birth.  Certainly, Sam has many things in common with people who have had a stroke in the same part of the brain that Sam has damage and treating Sam as though he was a stroke victim has been a very good plan.  It's probably the best theory we have about what is wrong with Sam.  This inability to speak even though he understands what is said to him is called aphasia.  He doesn't understand everything, but he understands a lot.  I never know how much, but it seems safest to assume he understands just about everything. 

I wish we knew.  I wish a lot of stuff. 

I went to the Esperanza Community Services Annual Banquet last night.  It was great, as usual.  Amazing food, great art, saw a lot of people I really love and laughed a lot.  Bought a quilt made by my friend Margaret.  That's a real treasure! 

But there is a real problem with going out in public.  People say stuff and I don't know the correct response.  For example, several people last night said that old line "I don't know how you do it.  You are amazing."  I don't know what I'm supposed to say to comments like that.  I have tried many responses and rejected even more.  Like I have never actually said, "Well, my parents are from the Planet Krypton."  Last night, I came up with a new response.  I said, "Well, he's just so darn cute.  It really makes things easy."  I haven't slept in two nights, but he's so cute that I don't mind... much...  Well I do mind.  A lot.  But I don't know what else to do.  Oh! Oh! There's a good response: "I'm just too stupified with exhaustion to think of an alternative to doing what I do."

Another response I've been thinking of using is to tell that story of the frog in the boiling pot.  You know the one: If you put a frog in a pot of boiling water he jumps right out again.  If you put him in a pot of cold water and gradually turn up the heat, he'll just swim around until he's cooked. 

If someone hands you a kid with profound and multiple disabilities and tells you the whole litany of problems all in one go, you run for the hills.  On the other hand, if someone gives you a beautiful baby boy who just has trouble swallowing, you learn to cope.  Then they say, "well he seems to have one or two other problems."  And you notice a few more as the weeks and months go by.  Each time you say, "Well, that's just one more little thing." After awhile the doctor can say, "well, he seems to have spinal meningitis and he's probably going to die in the next few hours.  Oh no.  Our mistake, it's just an ear infection."  That's a great strategy because you hardly notice the next 42 little new things wrong because you're so happy he made it through the spinal meningitis thing.  After that, they keep on adding stuff gradually for 28 years!  So when someone comes up to you and says, "Wow!  You're swimming around in boiling water and you look just fine."  You say, "Well, he just so darn cute."

I don't believe that story about the frog.  Who would do such a thing to a poor frog anyway?  Or why?  Maybe I should go with the one about my parents being from Krypton.