Tag: Conditions and Diseases

Aging….


I am so grateful that I am no longer young. I received this in an email and thought I should share it!!As I’ve aged, I’ve become kinder to myself, and less critical of myself.

I’ve become my own friend.


I have seen too many dear friends leave this world, too soon; before they understood the great freedom that comes with aging.


Whose business is it, if I choose to read, or play, on the computer, until 4 AM, or sleep until noon? I will dance with myself to those wonderful tunes of the 50, 60 &70 ‘s, and if I, at the same time, wish to weep over a lost love, I will.


I will walk the beach, in a swim suit that is stretched over a bulging body, and will dive into the waves, with abandon, if I choose to, despite the pitying glances from the jet set. They, too, will get old.


I know I am sometimes forgetful. But there again, some of life is just as well forgotten. And, I eventually remember the important things.


Sure, over the years, my heart has been broken. How can your heart not break, when you lose a loved one, or when a child suffers, or even when somebody’s beloved pet gets hit by a car? But, broken hearts are what give us strength, and understanding, and compassion. A heart never broken, is pristine, and sterile, and will never know the joy of being imperfect.

081113_2215_Aging6.jpg

I am so blessed to have lived long enough to have my hair turning grey, and to have my youthful laughs be forever etched into deep grooves on my face. So many have never laughed, and so many have died before their hair could turn silver. As you get older, it is easier to be positive. You care less about what other people think. I don’t question myself anymore. I’ve even earned the right to be wrong. So, to answer your question, I like being old. It has set me free. I like the person I have become. I am not going to live forever, but while I am still here, I will not waste time lamenting what could have been, or worrying about what will be. And I shall eat dessert every single day (if I feel like it). 

!cid_15_31527390@web162103_mail_bf1_yahoo

There is pain after death



On the 10th of October 2012 I posted this:

Two days ago I reblogged a post “Is there pain after death” written by a Dr James Salwitz. This post elicited some comments – mainly from Vic. Vic has started reading the odd post of my blog. In a way I am truly okay with it but on the other hand I find it difficult to blog my fears and emotions knowing that Vic may read the post. I find that I have become guarded in what I am writing. I am thinking that I should blog about stuff that may allay Vic’s fears….

Yesterday Vic asked “Mommy, I know what we believe in but what if there is more pain after I died?”

“You read my blog?” I asked.

“Yes” Vic replied.

“Sweetie, I believe that when the time comes our loved ones will be our guardian angels and hold our hands whilst we cross over….”

“I know that Mommy but what if I am still in pain… What if the pain does not stop?”

“Sweetie, the pain that continues after death is the emotional pain that belong to the loved ones that are left behind. That is what the post is about…..”

Tears welled up in Vic’s eyes and she said “I know that Mommy but what if I am still in pain… What if the pain does not stop? What if your pain does not stop?”

Andrew, http://lymphomajourney.wordpress.com/, commented as follows… “Even before one leaves, I always thought it more difficult on my family to watch me go through what was pretty aggressive treatment than on me.”

sbcallahan, http://thedrsays.org commented…”this is one of the difficult things about being the one who leaves. to know that your loved ones are going to suffer more than they already have is heartbreaking.”

“how to die? I have watched many die over the years and the range is as you would imagine. there were those that just could not let go and suffered every indignity to their body and soul. of course others went quietly with love around them. I have not decided if I want to be alone or with loved ones by my side. is there a way to make it easier for them? would they rather receive a phone call with the news or be at bedside? either way it will hurt them, not me of course as I am the one leaving. I would be lying if I said I don’t think of how I will miss so much. the thing is I have had so much, so much more than others and it seems selfish to complain. what they will go through is tremendous compared to what I will go through. I will sleep eternally and they will live. the best I can hope for them is peace of mind and future happiness. I want them to think of me and smile as I do now thinking of them.” http://thedrsays.org/2011/03/

I am beginning to think it is easier to be the person leaving than the one being left. I have always known that about relationships and breaking up but now realize that it is the same when someone you love is dying. My husband became suddenly angry and I knew there was something wrong. it is so unlike him to get angry over nothing that I was completely off guard. we had been watching the movie “steel magnolia’s” and he asked me what Julia Roberts was dying from and I told him kidney failure. later when he was able to talk, he shared that it had reminded him of my own kidney failure and near death. we live in limbo waiting each week for blood tests to know if I am back in failure or good for a few more days. I don’t really think about it and when he shared his fear my heart ached. The sad thing is I have no fear and realize more and more how hard this is for him. I know that he will be fine in the end but it is hard for him to imagine he will be fine without me. It is so much harder to be the one being left behind. http://thedrsays.org/2011/03/25/the-one/

Vic so often tells me how worried she is about the family. She worries about how the boys, her dad and I will cope. Whether we will cope…. whether we will be able to get over her eventual passing…. Andrew and sbcallahan write about their fears… for their loved ones. It is a fear that all terminally ill people appear to have.

My Mom died a bad death! Two weeks after major surgery she died an agonising death from septicaemia. We could see the gangrene spread…. She was burning up with fever and no amount of pain medication could dull or relieve the pain. God alone knows what went through her mind because she was ventilated. When my Mom finally died we were so relieved. We were relieved that her suffering was over. We were traumatized by the dying process not her death.

As a family we have lived with Vic’s pain and her excruciatingly slow journey towards death for the past eleven years. For eleven years we have heard her scream with pain, moan with discomfort, we hold her hair back when she is doubled up over a toilet bowel, vomiting until she fractures a vertebrae. We have nursed open wounds, changed colostomy bags…. We have watched our daughter and mother suffer the most horrendous symptoms.

So baby, if you read this post, know that we will miss you. We don’t want you to leave us behind but we want your suffering to end. We will continue to love you until we are reunited one day. You have to trust us that you will always be “my baby” and the boys’ mummy. But know that we will be grateful when your little body is freed from its pain and suffering. You will be at peace… You will not suffer more pain after death. We will mourn you but we will also be at peace… We will think of you and smile…

It is okay to let go my angel child.

Preparing for Vic’s death was not easy. It was however a breeze compared to the actual pain after Vic died.

In a way I supposed I almost romanticized Vic’s passing. I knew that I would miss her. What I did not know is how much….I did not know that my mind would block out the suffering beforehand.

I thought I would always remember her cries, her tears, and her pain. I did not realize that I would forget her cries, pain, tears…. I remember her shuffling little footsteps down the passage, her soft kisses on my cheek, her gentle nature, her laughter…

I thought I would be relieved that her suffering was over – Nothing and nobody could have prepared me for the huge void in my life.

Today I know that there is excruciating agony after death. For the living…

I wrote “We will think of you and smile…”

Vic, today I know we think of you and cry… selfishly I don’t have peace.

Chaka’s is not the same without you. Nothing will ever be the same without you my Angel.

I miss you so much!!!

“The pain passes, but the beauty remains”.


"The pain passes, but the beauty remains". --Pierre-Auguste Renoir (1841-1919)
“The pain passes, but the beauty remains”.
—Pierre-Auguste Renoir (1841-1919)

I have flu.  I get ill once in 5 years and I am a ninny.  I do not handle pain and discomfort well… I whinge, moan and groan until I am better…

This time I have embraced my flu.  I can stay in bed and there is no pressure for me to get up…. I can just lie in bed with my eyes closed and it is okay!  For the first time since my precious child died I don’t have to communicate, talk or pretend.  I can just be sick!!!!  I have spent four days in bed not having to talk, smile or pretend….

I am sick to the core of my soul – not only from the flu but from heartbreak.  I was able to feel the loneliness and emptiness that permeates my life since Vic died.  For four days I have not had to smile or live – I could lie in bed and hope to die.

There are days that I really do cope.  There are days that I am able to smile and laugh.  Most of the time I live a lie…

I have made peace with the fact that my precious child’s suffering is over.  I know that never again will she fracture vertebrae from vomiting, scream from pain, whimper because she does not have the strength to scream… But all I have done is learnt to accept that Vic was amputated from my life.  I still have to learn to live without the amputated part of my body; the pain of the missing part of me continues to taunt me…

I miss my child so much!  I want to love and hold her.  I wish I could have protected her from the ravages of Osteogenesis Imperfecta and doctor error!  No, I don’t have guilt…I did everything I possibly could for my baby girl. I just desperately miss her!  I miss her company.

I am hoping that the pain will pass…I do remember the beauty of my precious Vic all the time.  The wonderful mother and daughter that she was; kind and gentle; incapable of malice…Her beauty will remain with us for ever.

Tomorrow I will get out of bed and carry on living.

Vicky Bruce 31.8.1974 to 18.1.2013


Vicky Bruce, brave warrior, beloved mother of Jared and Jon-Daniel Sadie, beautiful daughter of Tersia and Danie Burger, sister and friend lost her brave battle against Osteogenesis Imperfecta on 18 January 2013. Finally, you can run angel child!  Your incredible will to live and your beautiful soul will live on in your amazing sons.  They are truly monuments that will honour you forever.  You are finally free and reunited with you Daddy, Moekie and Gramps.!  Run Vic run! Love you now and forever baby!

Vicky Bruce
Vicky Bruce

Tomorrow may be a rough day


Alberton-20121206-01427

 

Vic’s arm is very painful.  The antibiotics have not started working yet.  Dr Sue will come and see her tomorrow morning, and we will then decide whether it warrants hospitalization.  Obviously Vic need intravenous antibiotics and her tissue is too poor….

Tonight Vic had one of her worst vomiting spells yet.  It happened after 02:00am and her dinner of 7.30pm had not digested yet.  It is obvious that the oral antibiotics are not being absorbed.

Vic was very tired today, but insisted on going with Jon-Daniel to the orthodontist.  In years to come will he remember that his mommy was with him when he heard his orthodontic treatment ends on the 1st of February at 09:15am?

My sister phoned tonight.  She categorically told me that I have no business injecting Vic.  Nurses go to College for 4 or 5 years so they know what they are doing…. I did not even bother to explain that it is the Hospice site that is bad… My two sites are only in the beginning stages of going septic…  I wonder whether she remembered that Vic has sepsis in her spine and abdomen…

The pethidine has kicked in.  My child is in a pain-free sleep.  I will now try to sleep.  Tomorrow may be a rough day.

 

The Liver….


 The liver works with the endocrine system to regulate nutrients in the body and remove toxins. An enlarged liver means that it is endangered due to disease or other factors, which can lead to toxic shock. The proper medical term for an enlarged liver is hepatomegaly.

A healthy liver helps fight infections and filters toxins from the blood. It also helps to digest food, store nutrients for future needs, manufactures protein, bile and blood-clotting factors and metabolizes medications. A healthy liver has the ability to grow back, or regenerate, when it is damaged. Anything that prevents the liver from performing these functions – or from growing back after injury – can severely impact health and very possibly length of life.

What Are the Symptoms of Liver Failure?

The initial symptoms of liver failure are often ones that can be due to any number or conditions. Because of this, liver failure may be initially difficult to diagnose. Early symptoms include:

  • Nausea
  • Loss of appetite
  • Fatigue
  • Diarrhea

However, as liver failure progresses, the symptoms become more serious, requiring urgent care. These symptoms include:

  • Jaundice
  • Bleeding easily
  • Swollen abdomen
  • Mental disorientation or confusion (known as hepatic encephalopathy)
  • Sleepiness
  • Coma

http://www.webmd.com/digestive-disorders/digestive-diseases-liver-failure

Causes

Fatty or enlarged livers are caused by infections, certain medications, general toxicity, hepatitis, autoimmune disorders, metabolic syndrome and genetic disorders that affect the liver. Abnormal growths, such as cysts or tumors, impact liver size. Blood-flow symptoms, such as heart failure, can cause liver enlargement. Conditions also exist that restrict blood to the liver veins, such as hepatic vein thrombosis.

An enlarged liver indicates a problem with the liver itself or your overall endocrine system. If you suspect that you have any of these symptoms, you should seek medical attention. The causes of an enlarged liver vary in their severity and complexity. Only a medical professional can make the proper diagnosis for the cause of an enlarged liver.

Read more: http://www.webdiagnosis.com/causes-of-an-enlarged-liver#ixzz2CzDrLFH2

If not addressed and stopped in the earlier stages, the damage resulting from these multiple causes leads to scarring of the liver, known as cirrhosis, where large portions of the organ begin to lose their capacity to function or regenerate.

Treatment of patients with liver failure is specific to the unique symptoms and conditions experienced by each individual. Any patient with liver damage will be asked to abstain from alcohol. For patients with cirrhosis and end-stage liver disease, medications may be required to control the amount of protein absorbed in the diet. If there has been a build-up of toxins, particularly high ammonia levels, medication will be offered which lowers these levels. Low sodium diet and water pills (diuretics) may be required to minimize water retention. In those with large amounts of ascites fluid, the excess fluid may have to be occasionally removed with a needle and syringe (paracentesis). Using local anesthetic, a needle is inserted through the abdominal wall and the fluid withdrawn. Sometimes surgery is performed to minimize portal hypertension and lower the risk of gastroesophageal bleeding.

At this point, a person may become a candidate for liver transplant of part or all of the liver. Transplant success has improved in recent years with 1-year patient survival rates of up to 87%.  Due to the severe organ shortages, patients who are listed for liver transplantation have an estimated wait time of 1 to 3 years, depending on blood type and illness severity. Many patients are never able to be considered for transplants due to severity of their disease, other medical problems, or social considerations such as ongoing alcohol use or non-compliance with treatment recommendations. Others die while waiting for a transplant as their disease continues to progress.

The impact of these various symptoms and conditions on suffering and quality of life are profound, and ESLD patients can benefit greatly from hospice and palliative care. Even, when an ESLD patient is on a transplant list, this does not automatically prevent them from being on hospice services.

According to Medicare/Hospice  guidelines, patients will be considered to be in the terminal stage of liver disease (life expectancy of six months or less) and eligible for hospice care, if they meet the following criteria (1 and 2 must be present; factors from 3 will lend supporting documentation):

1. The patient should show both a and b:
a. Prothrombin time prolonged more than 5 seconds over control, or International Normalized Ratio (INR)> 1.5
b. Serum albumin <2.5 gm/d1

2. End stage liver disease is present and the patient shows at least one of the following:
a. ascites, refractory to treatment or patient non-compliant
b. spontaneous bacterial peritonitis
c. hepatorenal syndrome (elevated creatinine and BUN with oliguria (<400ml/day) and urine sodium concentration <10 mEq/l)
d. hepatic encephalopathy, refractory to treatment, or patient non-complaint
e. recurrent variceal bleeding, despite intensive therapy

3. Documentation of the following factors will support eligibility for hospice care:
a. progressive malnutrition
b. muscle wasting with reduced strength and endurance
c. continued active alcoholism (> 80 gm ethanol/day)
d. hepatocellular carcinoma
e. HBsAg (Hepatitis B) positivity
f. hepatitis C refractory to interferon treatment

http://www.hospiceofthecomforter.org/en/post/medical-perspective/understanding-endstage-liver-disease

 

“Mommy, I have the hiccups again”


Photo courtesy of http://www.mindentimes.ca/2012/07/11/non-profit-facility-provides-vacations-for-dialysis-patients

 

Dr Sue has been.  The lung infection has cleared and Vic’s saturation is back up to 98%!  What a little trooper she is!

She is however in Stage 4 renal and liver failure.

Chronic Kidney Disease is diagnosed by the eGFR and other factors, and is divided into five stages:

Stage of Chronic Kidney Disease eGFR ml/min/1.73 m
Stage 1: the eGFR shows normal kidney function but you are already known to have some kidney damage or disease. For example, you may have some protein or blood in your urine, an abnormality of your kidney, kidney inflammation, etc. 90 or more
Stage 2: mildly reduced kidney function AND you are already known to have some kidney damage or disease. People with an eGFR of 60-89 without any known kidney damage or disease are not considered to have chronic kidney disease (CKD). 60 to 89
Stage 3: moderately reduced kidney function. (With or without a known kidney disease. For example, an elderly person with ageing kidneys may have reduced kidney function without a specific known kidney disease.) 45 to 59 (3A)
30 to 44 (3B)
Stage 4: severely reduced kidney function. (With or without known kidney disease.) 15 to 29
Stage 5: very severely reduced kidney function. This is sometimes called end-stage kidney failure or established renal failure. Less than 15

Only last week Vic complained to one of her siblings that she is battling with hiccups.  We laughed about it and reminded her of the old wives tale that if you steal you will get hiccups… We wanted to know what she had stolen… If any person in the world told me then that hiccups is a symptom of kidney failure I would of thought they were taking the Micky out of me!

Renal Failure Symptoms                                                                   http://www.mayoclinic.com/health/kidney-failure/DS00682/DSECTION=symptoms

Signs and symptoms of kidney failure develop slowly over time if kidney damage progresses slowly. Signs and symptoms of kidney failure may include:

Signs and symptoms of kidney failure are often nonspecific, meaning they can also be caused by other illnesses. In addition, because your kidneys are highly adaptable and able to compensate for lost function, signs and symptoms of kidney failure may not appear until irreversible damage has occurred.

 Chronic kidney failure can affect almost every part of your body. Potential complications may include:

 Treating the cause of kidney failure

Depending on the underlying cause, some types of chronic kidney failure can be treated. Often, though, chronic kidney failure has no cure. Treatment consists of measures to help control signs and symptoms of chronic kidney failure, reduce complications, and slow the progress of the disease. If your kidneys become severely damaged, you may need treatments for end-stage kidney disease.

Your doctor will work to slow or control the disease or condition that’s causing your kidney failure. Treatment options vary, depending on the cause. But kidney damage can continue to worsen even when an underlying condition, such as high blood pressure, has been controlled.

Treating complications of kidney failure Kidney failure complications can be controlled to make you more comfortable. Treatments may include:

CONCLUSION:  Vic’s kidney failure is irreversible.  There are no drugs to reverse the process.  The question can only be whether Vic will go onto dialysis….  The decision will be her’s to make.

 

 

 

 

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“Worrying doesn’t empty tomorrow of its sorrow, it empties today of its strength.”


“Worrying is carrying tomorrow’s load with today’s strength carrying two days at once. It is moving into tomorrow ahead of time. Worrying doesn’t empty tomorrow of its sorrow, it empties today of its strength.” ~ Corrie Ten Boom – I read this on Dr Bill Wooten’s blog http://drbillwooten.com/2012/11/17/todays-strength/

This is so true.  I have been so worried about Vic’s latest symptoms   The body is such a complex intertwined mechanism…. I am absolutely fascinated at how everything links in… I just have to discover the function of the appendix and it’s interwoven functions…

Vic is having a lousy day.  She is exhausted and very swollen.  Despite the injections she has been nauseous all day.  This evening she had another vomiting bout.  Tomorrow we will see Dr Sue again.  Just maybe there is a 3rd anti-nausea type injection available

Vic also complained of a terrible “acidic” feeling.

I Googled her symptoms and found something that matches her new symptoms and blood test results.

 Metabolic acidosis

From Wikipedia, the free encyclopedia

In medicine, metabolic acidosis is a condition that occurs when the body produces too much acid or when the kidneys are not removing enough acid from the body. If unchecked, metabolic acidosis leads to acidemia, i.e., blood pH is low (less than 7.35) due to increased production of hydrogen by the body or the inability of the body to form bicarbonate (HCO3–) in the kidney. Its causes are diverse, and its consequences can be serious, including coma and death. Together with respiratory acidosis, it is one of the two general causes of acidemia.

Other causes of metabolic acidosis include:

 Dr Sue said that Vic’s sodium levels are very low.  So I Googled “Low sodium Levels”

Abnormal sodium levels can be due to many different conditions.

A lower than normal sodium level is called hyponatremia. This may be due to:

  •  Addison’s disease – CHECK
  • Dehydration, vomiting, diarrhea  – CHECK
  • An increase in total body water seen in those with heart failure, certain kidney diseases, or cirrhosis of the liver – CHECK
  • Ketonuria – NEGATIVE
  • SIADH – POSSIBLE
  • Too much of the hormone vasopressin – VERY POSSIBLE
  • Use of medications such as diuretics (water pills), morphine, and SSRI antidepressants – CHECK

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001397/

This truly scares me.

Earlier tonight Vic and I chatted over a cup of tea and I said to her “Baby, I am a little worried about what is happening in your little body”

“Do you think I am in trouble Mommy?  Please be honest!” she pleaded.

*Sweetie, I think you are in more trouble than you realize” I answered

“Mommy I can feel it in my body.  I am just so tired.”

“Sweetie, if Dr Sue says you must go onto oxygen tomorrow you MUST”

“I don’t think so Mommy.” Vic replied

“You have to make it to Christmas Baby…”

“Do you think it is that close Mommy?” Vic asked

“It could be Vic….” I answered

“Do you think we will get to Italy?” Vic asked.

“We will go in January” I promised

“I will stay in bed and rest for a whole month if it enables me to go to Italy….” Vic promised

I have increased Vic’s Addison’s medication.  Prevention is better than cure and Italy awaits!

 

 

 

“

 

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