Monday, February 13, 2017

Chronic Sorrow

Losses are an integral part of chronic illness and disability. The term chronic sorrow,can be described as  "long-term periodic sadness" when you experience ongoing times of grief.   I know that it happens to us as we try to raise our son or daughter with PWS in a world that doesn't seem to notice or care.  It can also happen to our sons and daughters too, as they travel life's uneasy path.  They too, experience sadness for themselves as they see contemporaries fulfilling their lives in ways our kids can never do.

Chronic sorrow can hit you at any time without warning; it doesn't need  prompts or reminders, although these are around us all the time; it can just well up inside and make you feel vulnerable, alone, misunderstood, and cause sudden and intense grief.  Often it is cyclic and occurs when another son or daughter or just a random child you see, surpasses the ability of your child with PWS and you realise that no matter how hard you try, you cannot cure your child.

Having said that, I am not for one moment, not for a milli-second, saying you should ever stop trying to help and support your child, learn as much as you can about PWS, advocate, teach others, and never stop hoping and working towards a better understanding and even a cure.  What I am saying is that it is ok to grieve, ok to accept that grief doesn't ever go away, and ok to be kind to yourself when it hits.  I hope that the following article helps.

Chronic Sorrow is a term coined by sociologist Simon Olshanshy to describe the long-term reaction of parents who have a child with a disability. This pervasive reaction is often not recognized or understood by those around the parents--professionals, family and friends. These feelings of chronic sorrow are normal and to be expected and accepted, given the life-long implications for the family and child. 
Many factors can affect the intensity and exhibition of chronic sorrow: the parent's personalities, the severity of the disability, the nature of the disability and the adequacy of support and services provided. 

Chronic sorrow does not mean that the parents don't love or feel pride in their child. These feelings, and many other feelings, exist alongside the sadness. It is as if many threads are woven side by side, bright and dark, in the fabric of the parent's lives. They co-exist; they do not blend into one color, or feeling.  

Because ours is such a "can do" society, there is pressure on parents to quickly put their feelings of sadness away or deny them. Parents are told to "think positively" and "to get on with your lives." They are told that God has "selected" them to receive this special child because they are such strong people.  

These kinds of comments, while well meant, deny the validity or parental long-term grieving. The discomfort of observing pain in those we care about can be part of the reason for such comments from others. 

Grieving, however, is a process that takes time, often years. It's a prickly bush that one must go through, not jump over. However, there are ways to support the process of grieving. Most parents find support in a community of people who understand because they, too have lived the experience. It is lonely to be the only family on the block with a child with a disability. Being part of a support group or organization helps to combat feelings of isolation. 

Engaging in personal activities that do not center on the family member with a disability can help increase feelings of competency and self-worth. Counselling, especially at times of significant stressful milestones, can be useful. 

Chronic sorrow becomes a permanent part of the personality structure of most parents who have a child with a disability. It's a normal response. Its thread narrows and widens depending on life situations; most often it is accepted with courage. And, although permanent, if is not the dominant force in interactions with our children.  
The dominant forces are love and feelings of connectedness to them.  

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The article comes from the Hydrocephalus Association Newsletter, Spring 1998, page 9, and was originally adapted from Meeting Ground, a publication of the Courage Center and is used with the stipulation that the original source be credited. 
For more information on the Hydrocephalus Association: 
870 Market Street # 955 San Francisco, California 94102  
            (415) 732-7040        
Website http://neurosurgery.mgh.harvard.edu/ha   
Email: hydroassoc@aol.com  




Friday, February 3, 2017

Gastrointestinal problems - the hidden enemy



Gastrointestinal problems – the hidden enemy
By Linda Thornton

(please note I am not a physician; I am a parent whose daughter with PWS has had some severe gastrointestinal problems which some of you may also have experienced or been unsure about)
In 1999 The Gathered View included an article about the discovery by PWS specialist Rob Wharton, M.D., of what he termed Acute Idiopathic Gastric Dilation. What Dr Wharton saw in his patient was that for some unknown (idiopathic) reason the stomach (gastric) was quickly (acute) pushed out (distended), causing the stomach tissues to die. If not immediately treated with surgery, this condition may lead to death.


Recently a great deal has been written about the issues around GI problems.  This is something that as a parent, I read and thought, “well, I’ve not seen any of this, so we’ll be fine”.  How wrong was I?  It’s not an easy topic to discuss among friends… (“well, now, tell me how many times do you poo a day and what do these look like?” is the best starter, but probably not a conversation-opener for many.)

I have always checked to see that my daughter is at least regular in her bowel movements and it’s always seemed so.  Looking back, and hindsight is a wonderful thing, I realise that she has had many bouts of diarrhoea which I put down to food that she had obviously stolen, or secreted away somewhere and eaten too much of, or she hadn’t been washing her hands thoroughly and has caught a bug.  
After all, we’ve always been told that people with PWS don’t vomit and that if there is a bug going around, it’s more likely to present as diarrhoea.  My daughter is now 31, and I started to notice the trend for diarrhoea about 10 years ago now.  And as I say, I always just put this down to her theft of foodstuffs.

I think I was wrong.

The PWS Diet
Diet in PWS has always been a corner-stone of how we manage the syndrome.  We’ve been told for years that the best way to help a person with PWS is to bulk up the meals, to add high-fibre foods that take longer to digest and stay in the tummy longer, thus keeping the notion of being ‘full’ more pronounced.  So, over the years I’ve happily watched my daughter eat the cores of apples (only the stalk was thrown away), the skins of Kiwi fruit, the gristle from chicken bones, nuts, seeds, berries, and I’ve loaded her plate with coleslaw, salads, tomatoes and similar.  I’ve been delighted at her ability to cheerfully eat broccoli stalks, the thick part of a cabbage, carrots, and other raw vegetables and fruits.  

I’ve counted calories, I’ve placed high-fibre foods in front of her all the time thinking I was doing the right thing.  Willingly she has eaten the skins, stalks, seeds, pips, of just about anything I have put in front of her, raw, or cooked.

I think I was wrong.

Suddenly in the past year, she has developed very scary acute abdominal pain.  So bad that she had more than once taken herself off to the Emergency Department of the local hospital – along with a staff person carrying all relevant information about PWS.  She has spent hours in the waiting room, patiently and painfully wanting to see a doctor.  She has been admitted overnight on several occasions and tests done for appendix, constipation, and various other extraneous things.  All of which have had no conclusion and all of which resulted in her being discharged from hospital, mostly with painkillers and laxatives.  On one occasion she was crying out for morphine to help with the pain, and this was put down to ‘attention-seeking’.  It didn’t matter how much information on PWS was taken for doctors to see, it simply was skimmed over, or disregarded.  Both her staff person and, on the occasions when I travelled down be with her, were ignored.   It is the greatest put-down you can experience… that knowing look from the medical expert when you try to tell them about PWS.  They’re not interested.  They are merely the ambulance at the bottom of the cliff.

However, after each time at the hospital things looked a little better and the laxatives did their job.  So back onto the high-fibre diet we went.  

I now know I was wrong.

The first time she told me she had vomited, I thought to myself, “she’s exaggerating, she’s never vomited before, why should she start now?”  It was a cup of coffee, last thing at night.  Went all over her sofa as it was so unexpected she really didn’t know what was happening.  The second time it happened, she scared herself badly, that she and her staff person went back to ED.  The wait was too long for her to cope with so, feeling better, she simply left and went home at 3am.

The third time, she was at home with us.  She’d been complaining of a stomach ache and later that evening, she asked me to feel her stomach.  It was distended and as hard as a rock.  I was very worried, particularly so as we live way out in the country away from medical help.  I put her to bed.
Within an hour she had vomited.  Projectile, compulsive, chaotic vomiting.  Fortunately this time she had made it to the bathroom, but no further than the door.  I could see everything she had eaten that day, nothing seemed digested at all.  

This time, I was scared.

The very next day I turned to our experts for help.  Asking Janalee Heinemann first, and then being guided to Dr Jim Loker, I can only say that without this immediate support and help, we could have been in a far worse position than I ever had imagined.

The first thing I was advised to do was to get her to a gastroenterologist for tests for gastroparesis.  Then at the same time I was to reduce her meals to 6 small (palm size) per day and to make these low-fibre.  She was not to eat meat, skins of fruit or vegetables, grain bread, salads, tomatoes, onions, anything from the cabbage family, or anything with pips which could stick in the lining of the stomach.  

Within hours, I had found a paper by Kate Beaver RN, and Barb Dorn, RN CNN, with a menu suitable for gastroparesis in PWS.  I am so grateful that this had already been done!  So together with Jim Loker’s dietary advice, I  mapped out a basic plan for the new diet.  The staff were fantastic and immediately put this into action, discarding from the cupboards and fridge all the foodstuffs she could no longer have.

What causes Gastroparesis
How on earth had this happened?  Surely I’d followed the rules?  Surely I’d understood about PWS and the diet’s special needs – after all, I’d run workshops on this very topic many times in the past, encouraging high-fibre foods, and foods slow to digest in the stomach.  

How could I be so wrong?

It seems there are many contributing factors which add to the misery of PWS.  Kate Beaver, MSW, CSW,  and Barb Dorn, RN, BSN cite in their paper:

Risk factors for Gastroparesis
Risk Factors Seen in Persons with PWS
Diabetes – most common cause
Diabetes – many persons with PWS have diabetes.
Adrenal and thyroid gland problems
Many persons with PWS have been found to have low functioning of their thyroid gland.
Certain drugs weaken the stomach – many antidepressants and heart medications
Many persons with PWS take antidepressant medications as part of behavior management and some may be taking heart medications.
Neurologic or brain disorders such as Parkinson’s, stroke and brain injury
We continue to learn the effects of PWS on brain functioning.

There was an immediate result with the small meals.  Well, two, if you like.  The first was that her ability to digest the smaller amounts was much, much easier.  The second was that she thoroughly enjoyed having these snack-meals, “I feel like I’m eating all day!” and it kept her bright and happy.

Convincing the doctors
I still needed to convince the doctors.  I’d already convinced myself, and her staff were grateful that something positive was forthcoming and this could be the end of the midnight sieges of the Emergency Department.
So I made an appointment with a dietician at the hospital, and another with her GP. The dietician was dubious.  “I think you should stick to the same diet as before.  We don’t know whether your guess at gastroparesis is the right one.”  She was right about one thing; we didn't know whether gastroparesis was the right diagnosis, but entirely wrong about sticking to the former diet.

Next appointment, the same day, was with her GP.  I asked the staff person to keep my daughter occupied while I did this appointment alone.  I had taken with me the GI papers from Ann Scheiman and the latest one from Barb Dorn and Kate Beaver.  I sat down and quietly told the doctor that I didn’t plan to leave his office until he had listened to me.  I said I didn’t need a diagnosis of “over-anxious mother” as I already had that, but I really and truly just wanted someone to listen.

Younger than me (they all are, these days), he assessed the situation and probably decided it would be quicker if he listened!  He wrote notes, he read the papers.  I sat there and waited.  He looked up and said, “I believe you are on to something.  We need to organise a gastroenterologist appointment.  We need to rule out this for a start."

I could have kissed him.  

I asked whether I could wait until he’d confirmed the gastro appointment as I had to travel some distance home and wanted to have things sorted before I left.  He said I should hear within the next 4-5 days and to keep up with the new diet.  It seemed so much better than just a few days before.
I waited the 4-5 days – just to be polite – before I rang the hospital gastroenterologist department and asked when I would be hearing about my daughter’s appointment.  The nurse searched the waiting list and said it would be about 3 months.  I cried.  She listened.  She said she would talk to the doctors and see whether my daughter could be squeezed in.

I waited the obligatory 2 days and phoned again.  Braver this time.  The nurse said she was about to call me and that she had been able to get an appointment within 3 weeks. 

I felt as though I was walking on eggshells for those 3 weeks.  Among all of us – the staff, my daughter and myself, we had worked out a menu that was satisfactory (and full of wonderful stuff, she thought, starting with white bread and ending with custard).

During this stressful time, she had to move house.  I decided to bring her home so that she wasn’t living in a house full of boxes and packing strewn all over the place.  This was a good move as I could also see how the new menu was working out.  It did mean that I was constantly in the kitchen preparing delicious little mini-meals for most of the day, but it was worth it.  There were new rules in place:  she was to sit up to the table to eat and not sit tucked up on the sofa.  This was to help with the digestion.  She could stand up to eat, if she wanted.  This is helpful especially after soup as it doesn’t sit in the stomach so long.  She was to walk after each little meal, at least 10 minutes which could add up to an hour’s exercise each day.  A good start.

Together, and with a staff person, we went to the gastroenterologist appointment.  Three days before our appointment I wrote to the doctor and sent him the two attachments which had become like my Ten Commandments.  I had carefully couched the email so that it didn’t sound like the mad mother from hell, but unemotionally and factually centered just asking him if he could read these as I was not sure how well PWS was understood in his world.

He was grateful!  I spent my Saturday reading and researching!”  Respect!  He listened, asked questions, did a physical examination and then said he would do a biopsy of the stomach followed by a “radio active scrambled egg breakfast” to follow the movement through the digestive system.

We started with the biopsy of the stomach – she was sedated, but not anaesthetised (which was good since she has an appalling record of coming out of an anaesthetic, fighting), and said she “felt the camera going down her throat” but thankfully she managed to stay with it.  

A couple of weeks later she underwent the gastric emptying study which meant she had no breakfast, arrived at the clinic and was given a small amount of scrambled egg whites, told to wait an hour when she would have another small meal of scrambled egg whites (during this time there were plenty of phone calls to me begging me to allow her to "have something to eat"!)  She lasted the distance with great support from her staff.  


And now for the interesting part

  • The results of the biopsy show no necrosis.  
  • There was no evidence of delayed gastric emptying  - in fact if anything, the gastric emptying was on the border of slightly rapid emptying.
So just what had been causing all the endless gastric problems that she was suffering for months and months?

A clinical visit to the Gastroenterologist to review the results of her tests  brought up the possible prognosis of Irritable Bowel Syndrome (IBS).  The Mao Clinic states:

"Irritable bowel syndrome (IBS) is a common disorder that affects the large intestine (colon). Irritable bowel syndrome commonly causes cramping, abdominal pain, bloating, gas, diarrhea and constipation. IBS is a chronic condition that you will need to manage long term."

Wrapping it up

 We have looked carefully at the diet, eliminating all high-fibre and wheat.  We've eliminated onions, and all the cabbage family as these for years had been causing really revolting wind problems; eliminated all fizzy drinks; kept coffee to a minimum and introduced decaffeinated drinks, we've kept to the 5 small meals a day and very slowly we've re-introduced fruits (peeled), soft vegetables and kept away from high fibre food.

There have been no further visits to the Emergency Department, no vomiting, no constipation and no gut pain.  We have decided to follow a diet which will manage the IBS condition.



I know this is no dinner-party topic, but I thought I would blog it just in case you notice a change in your son or daughter’s gut habits and to look at the amount of high-fibre we use in their diets.  It’s not gastroparesis, but it is definitely a part of what is a critical piece of the intestinal workings for all of us.
 

Read "Rethinking the PWS Diet" by Barb Dorn, RN and Kate Beaver, MSW, CSW.  On the same link, read more about Constipation in PWS, and Gastroparesis.

Read more about gastrointestinal problems in PWS including constipation