Another birthday…..


South African grandchildren

South African grandchildren

Yesterday I celebrated (another) birthday.

Late Saturday night Vic’s restlessness was indicative that she was determined to be the first to wish me.  At 11.30 pm she came through and said “another half hour….. I want to be the first to wish you Mommy.  I just want 30 minutes alone with you on your birthday…”

“No problem angel.  I’ll switch the kettle on.” I said

“I will be back in a minute” she said

I made coffee and checked some e-mails.  At 12:00pm I expected her to come through singing “Happy Birthday” but no Vicky….

I went through to her room and the poor baby had fallen asleep on her bed…

Jon-Daniel came through and brought me a cup of tea on a tray, with a gift and card and a rose!  “Happy birthday Oumie” he said.

He had bought a book I have wanted to read for a while “The Elephant Whisperer” – It is an inspiring, true life drama of a herd of wild African elephants on an African game reserve. The herd is destined to be shot for dangerous behaviour when this special human being, Anthony, intervenes to try to save their lives.  I was so thrilled that he remembered.

Just before 01:00 am Vic shuffled into my TV lounge.

“Oh Mommy, I am so sorry I fell asleep.  I thought I would just close my eyes for 5 minutes whilst you make the coffee…”

We sat and chatted for a while.  Vic shared her good wishes with me and we just sat and spoke.  We spoke about our very special mother-daughter relationship.  We spoke about years gone by and how blessed we are to have this time together. (I cannot imagine Vic married and living in someone else’s home on her final journey.)

The girls, Esther and Lani, arrived at 10:00am with gifts, a cooked meal, dessert and cake.  The grandchildren set the table…  My sister Lorraine and dear friend Judy arrived bearing armloads of gifts.  The grandchildren had written me letters and cards – it was so special.  Vic bravely cooked a pot of rice and had lunch with the family.  All the grandchildren swam and played tug-a-war!   We laughed and joked.

It was a perfect day.

Esther and Lani planned the day to start early whilst Vic is at her best.  As the day progresses so her energy levels decrease.  Immediately after lunch Vic went to bed.  She was in so much pain and absolutely exhausted.

All the grandchildren wanted to stay.

Sunday evening we Skyped my son and his family in the UK.  Vic and Danie spoke.  Vic and Danie Jnr have a special bond.

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Twenty two years ago I married Danie Sr and his four children; Esther 23, Lani 18, Liza 16 and Danie 11…  Danie married me and one, sick, very protected, spoilt brat, Vicky, aged 16.  Vic and Danie Jnr were the two kids who lived with us.  Vic embraced her new family.  (I was petrified of the children!)

Vic’s siblings have been amazing over the years.  I could never have coped as well as I do if it was not for their love, support and encouragement.  The siblings are fiercely protective of their little sister.

Vic and Danie Jnr spoke for at least 10 minutes last night.  It was a sad conversation between a brother and his older, little sister.

“I miss you so much Little Brother” Vic said

“I miss you too Vic.  How are you feeling?”  Jnr asked

“I am battling Boetie (Little Brother) Vic said

“We are coming to visit in April then I will see you Vic”

“I don’t know if I am going to make it to April” Vic said

“Just hang in there Vic.  It is not that long to April…” Jnr consoled her

“I know but I am tired.  I am just missing you” Vic cried

“I will fly over for a weekend.  I want to see you again” Danie promised

Vic was so tired last night.  Her little body cannot handle parties anymore.  She tries so hard.  This weekend we will have Jared’s 16th birthday.  It is only his birthday on the 26th but most of his friends are away for Christmas so we have his friend party an early in December.

I know this will more than likely be another last for Vic.

Esther, Vic and Lani

Esther, Vic and Lani

 

 

 

 

 

 

 

 

 

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.

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

Chronic Illness versus Terminal Illness


Chronic Illness versus Terminal Illness.

Chronic Illness versus Terminal Illness


Vic’s other parents visiting…

What a lovely surprise.  Gillian and Len popped around for a cup of tea!  What makes it unusual is the fact that they live in White River and have just been to the Sani Pass.  Vic was so happy.  She cried tears of happiness when she saw Len.  For a precious hour today we were able to laugh and visit with dear friends.

Len is the second “oldest” living male role figure in Vic’s life.  My brother Johan is the “oldest” living male role model.  Gill and Len have been my dearest friends for the past 34 years.  Is oldest the correct word?  Len is older than Johan and yet Johan is the “oldest” male…So “oldest” in this case means the “longest around”…

Gill was so amazed.  When she last saw Vic she was convinced that she would not see Vic alive again… In the meantime Vic has had her fall and technically speaking should be so much worse off.  But Vic has once again bounced back.

I decided that maybe I should check the difference between critical, chronic and terminal illness.  Maybe Vic is just chronically ill and the doctors got the terminology wrong.

“A chronic condition is a human health condition or disease that is persistent or otherwise long-lasting in its effects.[1] The term chronic is usually applied when the course of the disease lasts for more than three months.[1] Common chronic diseases include arthritis, asthma, cancer, COPD, diabetes and HIV/AIDS”.  From Wikipedia

Critical illnesses are serious illnesses that put the afflicted in danger of possibly dying. The illnesses that are considered critical tend to vary. Typically the big four critical illnesses that are covered would be heart attack, cancer, stroke and coronary artery by-pass surgery.

“Terminal illness is a medical term popularized in the 20th century to describe a disease that cannot be cured or adequately treated and that is reasonably expected to result in the death of the patient within a short period of time. This term is more commonly used for progressive diseases such as cancer or advanced heart disease than for trauma. In popular use, it indicates a disease which will eventually end the life of the sufferer”.  From Wikipedia

So according to Wikipedia Vic squarely falls into the terminal illness category.

The experience of death and dying has been transformed over time by significant advances in medical care and technologies, from a short-term event into one that usually involves a prolonged time of slow decline from chronic degenerative conditions.

Let’s look at Vic.  Vic was diagnosed with Osteogenesis Imperfecta as the age of 18 months.  By her 3rd birthday Vic had had 41 fractures.  The prognosis: – Vic would not live to be older than 12

Vic survived her 12th birthday and got married at the age of 21.  Vic fell pregnant 6 weeks after the wedding.  The prognosis:  Vic would not survive the birth of her baby.

Vic survived the birth of Jared.  When he was 7 months old Vic needed surgery to both her wrists.  She had fractured both her wrists and torn the ligaments picking up her baby.  When Jared was 13 months old Vic fell pregnant for the second time.  The prognosis:  Vic and the baby would not survive the pregnancy.  Vic was on bed rest when she was 3 months into her 2nd pregnancy.

It was a difficult pregnancy.  Vic survived and so did Jon-Daniel.

The pregnancies took a severe toll on her health.

At the age of 26 Vic had a hysterectomy.  Age 27 Vic had her blotched pro-disc surgery.  Life threatening sepsis resulted in 80, mainly abdominal surgeries. Now in 2012 there is no further treatment or surgeries available.  Over the past 10 years Vic has bounced between life and death.  She has certainly had good times as well as bad times over the past 10 years.  She has loved and hated, laughed and cried.  She has had good days and real bad days.  She has raised two beautiful boys to be compassionate, caring, responsible young men.  Boys that have brought so much joy to their mother’s life and that make us all so proud.  Young men that we know will honor their mother and their upbringing for the rest of their lives.

The difference however is that this year I believe Vic went from chronic illness to terminal illness.  This year the medical profession has given up hope.  Brendon has categorically stated he will perform no further gastrointestinal operations on Vic, The orthopedic surgeon refuses to pin her arm and the physician cancels blood tests…  On Friday we went back to the orthopod as her hand is so swollen and the pain is getting worse in the arm.  The X-rays showed that the fracture has been aggravated.  The displacement worse and yet he cannot do anything.  The sepsis from the spine and abdomen will spread to the arm.

Everyone has given up on Vic.

It is so soul destroying that no-one is prepared to try anymore.  This year every darn doctor has refused me.  I have always been able to bully them into trying one more time…just one more time.  I cannot take Vic to another gastroenterologist because someone who does not know the condition of her abdomen will certainly cause her death.

Tonight, after Vic’s 23:00 medication she just sobbed and sobbed.  I know her arm is really hurting.  She asked me to lay with her and I held her until her sobs subsided and her breathing became deep and peaceful.  She kept murmuring “I am sorry Mommy…”

Sorry for what?  Vic is sorry that she has “let me down”.  That she has failed at surviving and truly bouncing back. Being able to maintain a “life” and assuming responsibility for her own household and family.

Gill gently suggested that I blog happy moments…  So when I sat down tonight I tried to think of happy moments to share but I could not!  Gill says my blog is sad and I suppose it is.  The blog however mirrors my deepest feelings.

I am sad for my beautiful little girl.  I am sad seeing Vic deteriorate and her suffering increase.  I am sad for my beautiful grandsons and the despair in their eyes when they look at their Mom…

 

 

Who is taking care of the caregiver? 5.7.2012


Who is taking care of the caregiver? 5.7.2012.