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.

Pureed Food Recipies

Sam's a "big boy" and doesn't like to eat baby food.  He wants to eat whatever I'm eating.  This is a bit of a problem because I like to chew and he can't.  He has dyphasia (swallowing problems) on top of that.  When he gets too much food in his mouth, or hoards food he can't chew in his palate, it produces an amazing amount of saliva which goes up his nose.  When he swallows liquid that's too thick, it clogs his nose and he just about suffocates.  We ordered him a milk shake once and I thought we'd lost him.  Not going to repeat that experiment.  He does like Wendy's Frosty with a spoon.  If it's too thin, it comes out his nose when he swallows.  Sometimes the food that is just right only goes half way down his throat and a couple of minutes later it comes out his nose.  Sigh.

Numerous swallow studies have not really given us a solid picture of what is going on or how much this is related to his ever diminishing lung capacity, but it's a pretty safe bet that something that isn't supposed to be there is getting into his lungs and clogging them up.

So I have to feed him very carefully, but it has to be attractive, nutritious and taste good.  Culinary challenge they don't often cover on the food network!  One bonus: calories and fat are not an issue with this boy.  I'm always trying to keep his weight UP.

Of course calories and fat are a huge issue for his mom who knows how to chew!  So my grocery cart is a very peculiar mix of high and low fat stuff.


But over the years I've discovered a lot of things that work for Sam.  Some things I can serve to everyone as is (which thrills him) and some things require some last minute adjustment for Sam, but still are essentially the same thing we are eating.  Sadly, most of the time he just has to put up with purreed whatever we are eating because I just don't have the time or energy to make it special.  But I try to make him food he enjoys.


If I'm having a sandwich for lunch, I put the same stuff in the food processor, meat (or baked tofu or whatever), tomato, pickle (lettuce doesn't work very well).  Then I add a goo factor.  He likes Kraft Olive Oil reduced fat mayo or that Italian sandwich mix (gardineria?) or ketsup.  (I like fancy mustard.)  I put in one slice of bread and mix it for 30 seconds.  It turns into a kind of sticky glop that holds it's shape.  Then I make breadcrumbs (often toasted) of the other slice of bread.  I roll tbsp bits of the filling glop in the bread crumbs and shape them into little rectangles.  Voila! Finger sandwiches he can eat with me.


Really fluffy pancakes can be eaten just cut up (not pureed) if they have enough syrup on them.  Cheese cake is the ideal desert.  Cake is not very smart as it crumbles and chokes him, BUT if you add some cream or other liquid it becomes Dulce de Leche and very soggy.  Perfect for Sam to consume with a spoon.  Looks like cake but doesn't crumble!  If you take Starbucks Classic Coffee Cake and pour mocha frappicino over it, you get something similar to Tiramisu!  (Be sure to ask the Starbucks Barrista to give you the cake on a plate!)


I recently tried a Butternut Squash Soup with variations that was a huge success.  I got the recipe from someone named Gabster Roolz on Cooks.com.  Here's my variation:


1 chopped sweet onion
4 cloves garlic
tsp dried basil (or thyme)
Some fresh ginger (about 1/2 a thumb's worth)
Evoo
1 cubed butternut squash
1 C chicken stock
3 C water
1 bay leaf
pepper
2 medium sweet potatoes
1 pt heavy whipping cream  (NOT Whipped Cream)


You don't have to chop the garlic and ginger very much since it will all get pureed.  Just peel them and chop coarsely.  Saute onion, garlic, ginger and basil in the evoo (extra virgin olive oil).  Add squash, stock, water, bay leaf, pepper and sweet potato.  Boil 20 minutes.  REMOVE BAY LEAF.  Puree the rest.  At the last minute add the cream.  The whipping cream adds a lovely bulk to this soup and makes it exactly the right texture for proper swallowing.  BIG hit.

The chef Simply Ming says that heavy whipping cream is different from heavy cream because they add something to it to make it whip up better.  I don't know but I did love the way the heavy whipping cream bulked up the puree and made it gorgeous.  Of course, I suppose I should have the soup without the cream and just add it to Sam's portion... 


Here's another thing, I've been reading labels and Carnation Breakfast Essentials has pretty much the same nutrition as Ensure or Boost.  It also tastes a LOT better and is MUCH cheaper.  You have to add a bit of Thicken or Thick-it to make it the right consistency, but it's still cheaper.  Sam consumes 3 or 4 nutrition drinks a day so this is really good news.

What Have I Accomplished?

Yesterday my therapist and I were discussing my diagnosis of PTSD. This is a bit of a misnomer. I've never been in a war zone or been attached by dogs or raped or anything like that. I actually suffer from CHRONIC Traumatic Stress Disorder, except there isn't any such thing. There should be. It's the result of being the oldest daughter of a WWII vet who DID have PTSD, which gives me a vulnerability to developing it, combined with 27 years and counting as the mother and primary caregiver of a profoundly sick and disabled son. (In this case, Person First language is inappropriate. Sick and Disabled are the point of that sentence). And then, the final blow, the brutal murder of my PTSD Dad 4 years ago. These things combined have transformed me into someone I am struggling to get to know. Someone who apparently deserves the diagnosis of PTSD.

So we were talking about my life and my guilty feeling that I haven't accomplished much. I'm no Madame Curie and I sort of grew up thinking I should/could be. She (my therapist) said that I'm too close to my life to be able to stand away from it and say, "WOW. That's a lot of work." All I can see is that I'm getting more and more tired and frightened and that there are days when I really don't want to leave the house. I just want to stay home, take care of Sam and do something creative. And, the way my life is constructed, I can do that. Which makes me grateful but I wonder if I'm really pulling my own weight. Maybe I'm so far from being Madame Curie that I'm actually a wimp.

I should mention here that I shared this fear with my husband at dinner and he actually snorted! (A comforting sound) and said, "You are NOT a wimp. No one is Madame Curie." God Bless him. Since he gets up every morning and goes to a very difficult job without complaining, his opinion means a lot.

Another thing that happened yesterday was I got to sit in with Sam's Spred Group at Agape and watch while someone else struggled to keep him clean and keep him from aspirating food and keep him from stealing food from someone else's plate. It's a frantic activity. She's really good at it and I trust that she takes the whole thing very seriously. It was like looking at myself as other people must see us when we eat at a restaurant. It looked really hard. It looked really beautiful because they obviously enjoyed each other. For the first time, I sort of understood why perfect strangers think it is okay to interrupt us in restaurants and say drippy things like "God had sent you a little angel". ("The Stars are God's Daisy Chain" - Madeline Basset) "Angel" is not the first image that comes to mind when you are wiping chocolate milk and snot out of your son's nose.

So I was thinking about this "standing back from my own life" thing. The closest I can come to that right now is the secret thoughts I have and shove away because they are not nice. For example, when I am talking to a new mother. She will talk about how tired she is and how the baby never stops crying and how nasty poopy diapers are. She is afraid the baby might have a little cold or an ear infection. I will give her grandmotherly sympathy and, if appropriate, suggestions. Usually she just needs encouragement and approval. I'm good at giving those.

But here are the thoughts I'm struggling NOT to think: "You WIMP! Your baby will be fine and 6 months from now you will be sleeping soundly." MY baby has grownup poop and pubic hair. Try changing THAT. (grumble, grumble) I have been changing diapers every day for the last 30+ years.

For 27+ years, I have gotten up in the middle of the night to be sure my child is still breathing and often sat beside him and waited too long for the next breath. It is routine for my husband or myself to get him up and do some physical therapy or connect him to some machine to stimulate his breathing.

I have ceaselessly watched my child with an eagle eye to see that he doesn't aspirate too much liquid. I have tried to get his other occasional caregivers to understand the life and death importance of feeding him appropriately. When I drop him off for a few hours at school or Adult Day Care I worry that they think I am over-reacting and will not take my instructions seriously.

I have slept in hospital beds beside him when he is hovering between life and death. I literally pull him back from the brink of death 3 or 4 times every year. I check his oxygen blood levels every night at least once, usually more. I have 3 machines which I use on him 2 or 3 times a day to keep him breathing. I have 3 more I can use if he needs them. I grind up everything he eats and then try to form the pureed food into something that doesn't look pureed because he doesn't like to eat pureed food. I have developed a kind of mental telepathy with him because he is non-verbal. I am the captain of a team of doctors who advise me, but defer to me as the primary care-giver. This is a responsibility I accept gravely and with terror. I just barely have a Bachelor's Degree! But I guess I deserve a doctorate in "Sam".

I worry that the roofers will light a cigarette while I am giving him oxygen and blow up the house. I have told them how dangerous it is over and over again and they say, "Yes, yes." But I still find cigarette butts in the garden under the eaves.

I clean blood and chocolate milk out of my son's ears so routinely that it doesn't even occur to me to call a doctor.

There are so many things that I deal with calmly at home without even thinking of calling the doctor. These are things that would have any other parent calling 911! But I know how to treat them and a trip to the ER is so hard on him. I went to the ER so many times early on. Now its only once every year or even less. This is partly because he is stronger and partly because I know what to do at home. It's much easier to treat him at home where we can all watch Kung Fu Panda and sleep in our own beds.

That ER hiatus may be drawing to a close, however. His health is very slowly deteriorating and there may come a time when frequent ER trips once again become routine. I try not to think about the future very often. If it happens, it will happen when it happens.

He looks so happy and "normal" because I have dedicated my life to keeping him that way. But people don't understand how fragile he is because he doesn't have tubes coming out of him. I have fought against tubes because I want him to be happy. I hope I have made the right decision. When he dies, I will worry that it was because I refused the tubes. But no one ever knows what would have happened if... He looks so happy, doesn't he?

Sam Care is wearing me out and I'm getting older. I see what happens when a mom dies at 82 or something and there is no provision for her child. It's terrible. Yet, here in Illinois, there really isn't any way to plan ahead. I worry about what happens if he doesn't die first. I worry about how I will continue to live without him if he does die first. I can't imagine living without him.

Wow. That is a lot of work. Maybe I am not a wimp.

But there are occasional days when I am too tired or too depressed to do his machines, or only do them once instead of 2 or 3 times. There are days when all he will eat is Ensure and I give up and take him out to IHOP because I know, nutritious or not, at least he will not go to bed hungry.

For 27+ years I have struggled with the question, "Is it really okay to take a break?" "If/When he dies, will it be because I missed a treatment here and there?" People are always telling me it is important to take care of myself. I see the logic of this. Where would Sam be if I got sick or died? Sam himself knows how important I am and worries if I seem to be sick or if I walk away from him in a public place. But the fact remains that every instant I spend on myself is time I didn't spend on him. What is the cost of that?

Dysphagia

It's always a challenge to figure out what to feed Sam. He doesn't chew and he has all these aspiration issues. Food goes up his nose and down into his lungs if we aren't very careful. So I puree everything and thicken liquids. But he doesn't like food that looks different from everyone else's or is just plain grey and no fun.

Tonight we had Panda Express. You get two entrees and one side per person. So that makes 6 different entrees. I split them up so we each had a bit of each of these. I ordered one side of rice and two steamed vegetables. For Sam, I made a big platter with steamed rice in the center. Then all around the outside I had a little bit of all the different things we had. I ground them all separately and spooned them over the rice. It looked very fancy and delicious. Each entree had a little bit different color.

When something is too dry, I add a little bit of half and half and it gets nice and smooth. Calories are not his issue...

He was very interested and happy to eat with us!