5 Stages of Dying


It became crystal clear to me when Vic yelled at me in the ICU that she had deep-seated anger issues with me and where she was in her journey.  Dying is unknown territory and none of us have walked this road. Yet we are all morbidly curious about dying. Everybody has some shadows of uncertainty.

According to Hospice there are FIVE stages of dying.

1.      DENIAL: “I’m too young to die. I’m not ready to die (is anyone ever really ready?)”  It became clear to me that despite the fact that Vic was sentenced ten years ago she is still in denial. Even after Brendan had informed her that nothing more could be done for her she still clings to her lifelong defiance of death. The prediction from one’s physician of imminent death can do several things. It can give you time to prepare, take care of business, close doors, make amends. The shock begins to ebb as you come to grips with approaching death.  Vic has some doors to still close.  Her business is in order.  She has written letters to her loved ones, bought major birthday gifts and cards for the boys

 2.      ANGER: Suddenly the terminally ill person is no longer in control of their life.  They have no choice any more – the die is cast….. They are going to die. This is really where Vic is now.  She has lost control of her life.  At the age of 37 she is living in her mother’s home, decisions are made for her she has become a child again.  Her anger on Sunday morning was directed at me.  Her profuse sense of helplessness and loss of control is however not a new feeling.  Vicky has endured a long, debilitating illness.  Doctor error has robbed her of a life.  Illness has robbed her of her dignity.  She is angry with God for allowing this to happen to her.  She is angry for God not taking her.  On Monday afternoon she said “Mommy, God does not even want me…”She has been robbed the opportunity to see her sons complete school, university, get engaged and eventually marry……  Many people die too early but at 37 it is mainly due to misfortune, wrong place wrong time, an accident…..  At the age of 27 Vicky was sentenced to death…..

 3.      BARGAINING: I do not know what bargaining Vic has done with her God.  I know that I have made lots of bargains with my God.  Just one more Christmas….. Just one more birthday…..

 4.      DEPRESSION: Knowing that you are dying must be depressing!  This must be a normal part of the process of preparing to die. Vic is depressed about her inability to deal with her responsibilities. Vic is too ill to get away from the symptoms of her illness …..  She waits for death every day. Vic is depressed about the pain that her illness is causing her sons, friends and family.  Vic is depressed because she feels that she has failed her sons.   Vic is depressed because she is lingering…. Vic is depressed about the loss of love that she has suffered.  Vic is depressed period!  But with good reason.  Antidepressant’s are part of the pain control regime.  If it is helping for her pain that is great.  I hate to think what her mental condition would have been if she had not been on antidepressants.

 5.      ACCEPTANCE: Vic is not at this stage yet.  This is one of the main reasons why we need Hospice.  Brendan (her Gastroenterologist) referred her to a councillor last year to guide her into this phase of her journey.  Vic went a couple of times and then it became a matter of budget – medication or counselling.  Medication won.  Hospice defines acceptance as follows…”Acceptance is NOT: doing nothing, defeat, resignation or submission.  Acceptance IS: coming to terms with reality. It is accepting that the world will still go on without you. Death is after all, just a part of LIFE.”

I see absolutely no peace in Vic.  She is still kicking and fighting.  At times she may fool herself into thinking or believing that she has accepted her situation but it is crystal clear to me that it is not the case.

We met as a family on Tuesday evening to discuss all our frustrations.  It becomes difficult to handle one’s day to day frustrations as we have different agenda’s.  A while back Vic asked my permission to give up.  She spoke to the boys.  We cried and gave her “permission”. 

The family immediately went into palliative mode.  No demands or expectations for any normal functionality from us to Vic….. Therein lies the issue.  We became an “Us” and “Her”… “Us” became the protectors and “Her” became the invalid.  We tippy toed around Vic.  The boys stop bothering her with everyday issues like “Can we go to the movies”; “please pick me up at 16:00”; “Do I have to go to extra lessons?” When we walked into her room and she was sleeping we would turn around and walk out.  We would show no concern for the amount of pain medication Vic was on. 

It is so easy to slip into a “mode”.  I took all responsibilities out of Vicky’s hands, we as a family organized our lives around her pain levels and energy (or lack thereof) levels, we stopped laughing and living in our house.  We were all dying!

When she lashed out at me in ICU I realized that we had serious problems.  Vic was not ready for Stage 5.  She started kicking against death – again.  Her fight is back.

It is however a difficult and delicate balance between pain-free and functional…..  Vic said that she wanted to reduce her pain medication as maybe she would not be so tired all the time.  By Monday evening she was in so much pain that she was vomiting.  She could not keep tablets down….. It took two days to get her pain under control again.

I have also read Katie Mitchells Blog on Chronic Pain and the way that brave lady articulates her battle with pain truly opened my eyes.  I realize now that I cannot take living away from Vic whilst she is still breathing.  I have to let go.  I cannot protect her against pain.  I cannot protect her against death.  I have to try to look at her through her pain filled eyes as I don’t understand pain or her frustration.  I am active and busy.  Today I joined a gym so I can train with the boys.  I do the things with her sons that Vic would LOVE to do!  A couple of weeks ago Vic said “You are the fun person in the family.  You do all the fun things with the boys” Obviously there must be resentment and anger (Stage 1). 

But we live in a civilized home.  We don’t scream, shout or curse.  We bury things under the carpet.  We walk away from conflict so we don’t know what the other is thinking or feeling.  We only see the veneer..… How terribly sad!!  We have lost our ability to function properly.

So from now when I am frustrated with Vicky attempts at doing things for herself or the boys I will leave her be!!  PLEASE God help me!!!!  I am such a control freak!!!  I will endeavor to not stop her from going for a cup of coffee with one of her friends.  I will just pick up the pieces afterwards. 

I have to stop being selfish.  I realize that I was trying to keep Vicky pain-free as it is easier for me to handle!!  Pain free means medication on the strict regime, no strenuous activities, protected and wrapped up in cotton wool.  Vic must make her own painful decisions.  If she wants to take the boys to school who am I to stop her?  Of course she must but not on 400 mg of morphine! 

The problem is that I look at Vic and all I see is that fragile little toddler…… And she is sick.  Very sick!  I want to protect her, breathe for her, die for her.  Vicky is my baby.

Jurnista – hope or looming disaster? 19.7.2012


I woke up early this morning with a very heavy heart.  It was Pain Clinic Day again.  There is no appointment system – it operates on a first come first serve basis.  Wonder above all wonders no traffic delays… I was first to arrive!!!

The amazing thing is that one of Vic’s ICU doctors ran the Pain Clinic today.  He immediately recognised me and we spent an hour discussing Vic.  He looked at her X-rays and was mortified by the fracture.  He was so sympathetic.  Over the past 5 years he has often been part of the ICU team fighting for her life.  He is well aware of the prognosis and confirmed that the arm would be terribly painful and that there was no chance of it mending.  He knows exactly what her little body has been subjected to over the years.

 We will try an additional tablet, Jurnista, which apparently works on two different pain receptors.  Hydromorphone controlled-release belongs to the family of medications known as opioid analgesics (narcotic pain relievers). It is used to treat chronic severe pain. Hydromorphone works on the brain to increase the ability to tolerate pain. Hydromorphone controlled-release works by gradually releasing the medication into the body to help control pain that requires the prolonged or continuous use of an opioid pain killer.”

 

The side effects seem severe.  But he double checked with the head of the department so I am sure they know what they are doing!  I know that I will not have a peaceful night’s sleep at all!  But so far so good – it is a slow release tablet so no effect at all yet.  Oh, I forgot – it is highly addictive!

I am cautiously optimistic!  Just a little reprieve would be great!

 Poor Jared.  Just when we thought he has recovered so well from the Nissin Repair, chest pain strikes again!  (Nissen fundoplication is a surgical procedure to treat gastroesophageal reflux disease (GERD) and hiatus hernia. http://en.wikipedia.org.)

Jared was born with a reflux problem.  He was the best projectile vomitter ever!  As he grew older he stopped vomiting (in public) and never complained.  He then started chewing Rennies.  No matter who he went shopping with, he always came back with a pack of Rennies.   Obviously nobody reconciled his Rennies intake…  Until he developed chest pains!

Long story short is that after six months of treatment, the decision was made to do the Nissen repair.  I may have blogged how well Jared did post-operatively, how brave he was and how wonderfully he recovered.  So it came as quite a shock when I received a call from school yesterday asking me to pick Jared up as he had severe chest pains again…

 We spent the afternoon at the doctors.  Chest X-Ray, ECG and then a CT scan.  CT showed up several kidney stones… Average size 6.6mm.  The chest pain was caused by something called Costochondritis.   Costochondritis is inflammation of the junctions where the upper ribs join with the cartilage that holds them to the breastbone or sternum. 

Today Jared went for blood tests.  Once the results are back we will start seeing some specialists to resolve the kidney stone and calcium build-up problem.  The Dr suspects Jared has either an autoimmune and/or connective tissue problem.

 I was thinking today, that if we only have one life, how come it is so crappy?  Why can I not be the ill one?  I have made many mistakes in my life and stepped on a couple of toes in my career.  People love me or hate me.  Vic has never deliberately gone out to hurt anyone.  She was born with this dreadful disease.  And now my beautiful Jared… I could not bear going through this again with him.  He has such a pure heart.  The scariest thing is that he has witnessed his mother’s struggle and steady decline over the years. 

ImageThere are times that Vic cannot remember – the times that she was in ICU, ventilated, sedated, critically ill – all of this the boys lived.  Vic does not know what tomorrow holds but if Jared is diagnosed with OI he will know exactly what lies ahead of him.  I see the helplessness in his eyes when he looks at her.  I hear his frustration with her battle…  Please, if there is a God of Mercy, let Jared be spared this. 

Tonight I am not in a happy place.  I spoke to the UK kids and feel content that they are settled and doing well.  Spoke to the little ones but the longing is worse than ever.  Chloe graduates tomorrow.  I am not there to share this milestone in her life.

 Life sucks.

Image

I wish for Vic….10.7.2012


Image I found the blog of a 19 year old girl – Katie Michele who suffers from Marfan Syndrome.  Marfan is a connective tissue disorder.  This is a very special young lady who deals with Chronic Pain and the fear of aneurysms every day of her life…I have copied part of one of her blogs addressed “Dear Marfan”  because I think this is what Vic could have  written when she was younger… Please read her letter to Marfan… it will give you some insight into the heart of an ill teenager.

 “I  wish I didn’t have to wonder if you’d cause any future child of mine suffering, or if I’d lose my own life in the attempt to have one.

 “I wish you hadn’t taken my sports and many of my friends, and replaced them with medications and doctor appointments. You’re constantly reminding me that no part of my body is safe from your consequences and that it’s only a matter of time before something else goes wrong. You hurt me, day in and day out, standing or sitting, year after year, from head to toe. Because of you I talk more often to doctors than I do to people my own age.”  http://connectivetissuedisorders.wordpress.com/2012/05/02/dear-marfan/

I wish I did not have to wonder what my child’s life would have been like if she had not been born with OI.  Dr Frank S, I wish I did not have to wonder what life would have been if you had not chosen to ignore your colleagues warning on how poor Vic’s tissue is.  Dr V, I wish I did not have to wonder what my child’s life would have been if you had not perforated her small bowel, not once but twice!  And the biggest wonder of all, I wonder what my child’s life would have been like if I had insisted that Dr S spoke to Dr Coleman.

I wish that Vic could have enjoyed her son’s toddler years; that she could have played ball with them, gone on holiday with them.  I wish the boys could have known Vic happy and carefree.  I wish the boys could remember a day when their Mom was not doubled over in pain, vomiting and sad.  I wish they could remember their Mom going to work…looking forward to a normal tomorrow.  I wish they could sit and enjoy her playing the piano.  I wish that I could wish for Vic to see her son’s finish school, graduate at University, fall in love, get engaged and get married.  I cannot.  My biggest wish is that my poor child’s suffering will end.  That she will truly make peace with giving up…  That Vic will stop breathing and that she will float pain free and joyously to join her father and grandparents in Heaven.

Life has many questions.  Far more questions than answers.  I wonder about the fairness of life.  I wonder why Vic has to go through so much pain and agony.  I wonder why her boys have to watch her slow decline and painful journey. 

I wonder why the bad people, the rapist and murders, thieves and criminals get to live good lives.  Why do they have good health? 

What has Vic ever done to harm anybody?  She was sentenced to a life of pain and misery from conception.  PLEASE!  I don’t want to hear about the “sins of the fathers” sermon. 

A convicted murderer has many appeals before the sentence is executed.  A last appeal to the governor or the Supreme Court or who ever for clemency.  A terminally ill person does not have that luxury.  Their final appeal is to Hospice.  Now, what is left for the terminally ill person to do if their last appeal is denied?

They are not kicking and screaming, fighting the sentence anymore.  Hospice is their own hope of dying with some dignity and quality of “life”  But, like the Supreme Court in the USA and Governor, appeals are rejected quite often.  As in Vic’s case.

I will do everything in my power to ensure that Vic is allowed to spend, what is left of her life, as pain free as possible.  I will do everything possible to allow her to die with dignity. 

I am babbling.  And today Vic is having a good day… But tonight lies ahead with it’s pain and terror…

Facts about Osteogenesis Imperfecta


 I am posting this extract from the Osteogenesis Imperfectawebsite.  It is informative and will give you some idea of the terrible disease called Osteogenesis Imperfecta.The problem with Vic is the Connective Tissue issues.  If she had not had the blotched pro-disc surgery she would have been fine.

Vic is terminal due to doctor error!  Vic will probably die from her frozen abdomen and the issues that arise from a frozen abdomen. That is the short and the tall of it.

 

Osteogenesis imperfecta (OI) is a genetic disorder characterized by fragile bones that break easily. It is also known as “brittle bone disease.” A person is born with this disorder and is affected throughout his or her life time.

  • In addition to fractures people with OI often have muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, blue sclerae, dentinogenesis imperfecta (brittle teeth), and short stature. Restrictive pulmonary disease occurs in more severely affected people.
  • OI is caused by an error called a mutation on a gene that affects the body’s production of the collagen found in bones, and other tissues. It is not caused by too little calcium or poor nutrition.
  • OI is variable with 8 different types described in medical literature.
    • The types range in severity from a lethal form to a milder form with few visible symptoms.
    • The specific medical problems a person will encounter will depend on the degree of severity.
  • A person with mild OI may experience a few fractures while those with the severe forms may have hundreds in a lifetime.
  • The number of Americans affected with OI is thought to be 25,000-50,000.
    • The range is so wide because mild OI often goes undiagnosed.

Genetics

  • The majority of cases are caused by a dominant mutation to type 1 collagen (COL1A1 or COL1A2) genes
  • Other types are caused by mutations of the cartilage-associated protein (CRTAP) gene or the LEPRE1 gene. This type of mutation is inherited in a recessive manner.
  • OI occurs with equal frequency among males and females and among all racial and ethnic groups.
  • Approximately 35% of children with OI are born into a family with no family history of OI. Most often this is due to a new mutation to a gene and not by anything the parents did before or during pregnancy.

Testing and Diagnosis

Diagnosis for OI is primarily based on signs seen in a doctor’s examination. When there is uncertainty about the diagnosis, it is best to consult a physician who is familiar with OI. Genetic testing is available to confirm a diagnosis of OI through collagen or gene analysis—a skin sample or a blood sample are used to study the amount of Type I collagen or to do a DNA analysis.

Types

Since 1979, OI has been classified by type according to a system based on mode of inheritance, clinical picture, and information from x-rays. The characteristic features of OI vary greatly from person to person, even among people with the same type of OI, and even within the same family. Not all characteristics are evident in each person. The OI type descriptions provide general information about how severe the symptoms probably will be. Health issues frequently seen in children and adults who have OI include:

  • Short stature
  • Weak tissues, fragile skin, muscle weakness, and loose joints
  • Bleeding, easy bruising, frequent nosebleeds and in a small number of people heavy bleeding from injuries
  • Hearing loss may begin in childhood and affects approximately 50% of adults
  • Breathing problems, higher incidence of asthma plus risk for other lung problems
  • Curvature of the spine

See Types of OI for a detailed description.

Treatment

Doctors who see children and adults with OI include primary care physicians, orthopedists, endocrinologists, geneticists and physiatrists (rehabilitation specialists). Other specialists such as a neurologist may be needed.

  • Treatments focuses on minimizing fractures,  maximizing mobility, maximizing independent function and general health
  • Treatments include
    • Physical therapy and safe exercise including swimming
    • Casts, splints or wraps for broken bones
    • Braces to support legs, ankles, knees and wrists as needed
    • Orthopedic surgery, often including implanting rods to support the long bones in arms or legs
    • Medications to strengthen bones
    • Mobility aids such as canes, walkers, or wheelchairs and other equipment or aids for independence may be needed to compensate for weakness or short stature.

Treatments Being Studied

  • Medications
    • Bisphosphonates such as ©Aredia (pamidronate), ©Fosamax (alendronate) or ©Reclast (zoledronic acid)
    • ©Forteo (teriparatide injections) for adults only
  • Growth Hormone
  • Increased vitamin D intake
  • Physical activity
  • Potential for gene therapy

At this time, there is no cure.

Prognosis

The prognosis for a person with OI varies greatly depending on the number and severity of symptoms.

  • Life expectancy is not affected in people with mild or moderate symptoms.
  • Life expectancy may be shortened for those with more severe symptoms.

The most severe forms result in death at birth or during infancy.
Respiratory failure is the most frequent cause of death for people with OI, followed by accidental trauma.

Despite the challenges of managing OI, most adults and children who have OI lead productive and successful lives. They attend school, develop friendships and other relationships, have careers, raise families, participate in sports and other recreational activities and are active members of their communities.

Managing OI

  • Techniques for safe handling, protective positioning and safe movement are taught to parents
  • Infancy, early childhood and the pre-teen years are often challenging
  • Growth and hormonal changes can affect the frequency of fractures
  • Children and youth learn which activities to avoid and how to practice energy conservation
  • The number of fractures usually decreases in adulthood
  • Following a healthy lifestyle including not smoking, and maintaining a healthy weight is beneficial

History of OI in Medical Literature

There is evidence that OI has affected people throughout history. OI has been recognized in an Egyptian mummy dating from 1000 BC. It has also been identified as the medical condition suffered by Ivan the Boneless who lived in 9th century Denmark. Prince Ivan, according to legend, was carried into battle on a shield because he was unable to walk on his soft legs.

Case studies of fragile bones and hearing loss have appeared in the medical literature since the 1600s.The term “osteogenesis imperfecta” was originated by W. Vrolik in 1849, and the condition was loosely divided into “congenita” and “tarda” by E. Looser in 1906. Van der Hoeve in 1918 described the occurrence of fragile bones, in combination with blue sclera and early deafness as a distinct inherited syndrome.

In the 1970s, Dr. David Sillence and his team of researchers in Australia developed the system of categorization using “Types” that is currently in use. His original four classifications (Type I, Type II, Type III and Type IV) combine clinical symptoms with genetic components. This listing is based on the number of people in the study who had similar symptoms. The types do not go from mildest to most severe. This classification system has been generally accepted world wide since 1979 but continues to evolve as new information is discovered. In recent years, evidence from bone biopsies and other research led to the addition of Types V, VI, VII and VIII.

More Information

http://www.oif.org/site/PageServer?pagename=AOI_Facts