I loved to be active like most typical boys:

Baseball

Street hockey

Football

Swimming

Basketball

Bowling

Choir.

I come from a pretty large family, maybe not as large as some of you readers, but for my Mother who raised all of us as a single parent after my Father died when I was 5, and my step-father left sometime after my youngest brother was born, it was a large group.

As for my Family Tree I shall give you a little information:
Donald my eldest brother.
Married with 4 natural children, and 4 children in which he raised with his wife; Linda when her sister passed away. Currently he has 16 grandchildren. He is approximately 10 years older than I.
Michael the next brother in line.
Not currently married, has no children. Approximately 5 years older than me.
Next, there is me; and I am James. Married with 2 children and 1 grandchild Romina
I have only one sister and that is Angelia.
Currently not married but has 2 children. She is 2 years younger than I.
My brother John was a twin.
Alvin did not survive. John has 4 children. He is approximately 18 months younger than me.
Thomas is the youngest of the clan, he is from our step father, he is our brother & we treat him like one. Married to Carol, has one step child and one natural child with Carol. Thomas is approximately 7 years younger than me.

We are a very close knit family, of course there are typical disagreements, but when it comes to an illness, or helping each other we are all there for each other.







March - 2002
I began getting some strange pains in my stomach, near my June, the pain had become worse. The family Dr. took x-rays of my abdominal area, stated that he thought there may be a blockage; such as a bowel obstruction. I was then set up with tests and a follow- up treatment plan. Very close eye was kept on me, since I am not the type to complain of pain. A little more time passed I asked my wife; (Kym), what appendicitis felt like. I was feeling nausious, plus when I would play a computer video game with my son and a friend of his, I would get dizzy and need to lay down, symptoms were very much like those with appendicitis. The pain shifted slowly from the naval area to the lower abdominal area near the groin.
September - 2002
My pain became so intense one night, I asked my wife to take me to the ER. Many tests were ran, we were there for several hours: Barium swallows, CT of the abdomin & lower extremities, urine test etc. Nothing was found, except for the fact that I had waht is known as plueracy . I was sent home, the pain continued. As instructed by the ER, I went to our family doctor for my follow-up; I then was referred to a general surgeon. The surgeon got me into the office 1 week after I had been to the ER. With only the results from the family Dr., his knowledge and skill of feeling my abdomin and groin area, it was detected that I had an Ingunal Hernia. A discussion of how things were done so that it could be repaired, plus when I would be able to return back to work after the repair, as I do no heavy work. My surgery was scheduled, I awaited with mild pain medication until the date of September 27th, 2002. This was a time frame of approximately 17 days from the ER visit to the hernia surgery. I came home several hours after surgery and felt fine, a little tired, but that is to be expected after surgery.


OUR LIVES AS WE KNEW IT CAME TUMBLING DOWN.


September 30, 2002

An active 43 year old man; with a wife, two adult sons, a grandchild on the way, was in for the fight of his life. At the time of this writing the children were ages 22 and 20.




27th of September 2002, Inguinal hernia repair complete.
Came home with no complications.
Feeling slightly tired, with the surgical medicine, but is expected after any type of surgery. Now out of the agony of the original pain and on my way to healing. Happy and ready to return to work come Monday. I rested up that day, ate a light meal for dinner, as I do like to eat for me a light meal is a bowl of home cooked chicken noodle soup, some crackers with a glass of water and milk.




On Saturday, September 28th, 2002 - I phoned my wife to come home as I was not feeling well. She had gone to the health fair at the Silverdome, which is about 15 minutes or so from our home. I was home with our son who at the time was 20 years old, so I had not been left alone. When my wife arrived, I asked her to take me directly to the emergency room. Being told I was pallor in color, clammy, slow with my speach (kind of like a record on slow speed), and I informed her that I was lightheaded, nauseated, and I felt like an elephant was sitting on my chest, plus in agony with a headache, like I have never had before in my life. My wife, (Kym) immediatly took me to the ER., they had me sign in, but once it was stated that I was having chest pains I was immediately taken to the examination room. Never had I been probed and proded so many times in my life, scared and in pain they took my temperature, blood pressure, etc., Kym was filling out the paperwork at admissions. I was given nirtroglycerin,low dose asprin,torodal injection(s), then was attached to a nitro drip IV, X-Rays, Pulse, Temperature, Oxygen levels, Blood work, EKG's, and more...... Nothing was found. I was however admitted admitted as my blood pressure was not stable. It was finally decided that on Monday, I would receive a heart cathiter to determine what may be going on, as I was still having pain, and not eating too well. Most of the time I would sleep, some sleepiness may have been due to the medications that were to keep me well rested. I received some visitors the next day, but I don't remember everyone who showed up. I do remember that my wife was there, and someone from work who brought me a book. My wife tells me that I had several visitors.

Monday morning: I (Kym) his wife, was given a pager at the hospital, then told that when the procedure was complete, I would be able to come and get my husband. At that time I would be given instructions as to what would be done next. I was told the procedure would last anywhere from 30 minutes to 1 1/4 hours. I received a page in approximately 20 minutes later. Being as it was so short of a time, I did not think anything of it, and proceeded up to the area in which the test was ran, through the shuffle I was told that Jim had to go for emergency surgery. He was being transported via ambulance to another facility. After making a call to our sons at work, we went to the other hospital. For many of you that do not know, there is a law called FMLA in which you are entitled to take a leave from work, whether it is paid or not to be with a severly or not severly ill family member, it: spouse, children, parents, and not be dismissed or fired from your job. This came in particularily handy knowing this law, as the manager was not going to let one of the children come to the hospital. Not knowing what was going on, or what type of surgery my husband was having made the wait seem like forever.

Members of the family, and friends patiently awaited answers from the surgeon. Once the 9 hour surgery was over, the surgeon came out to tell us what had been performed. The surgeon told us that with the condition that Jim was in, he was lucky he got there when he did. It was nothing less than a miracle. My husband was on life support system for 7 days in ICU. 6 days in a step down room at the hospital, and was still requiring oxygen.
Family, Friends, and Co-workers have been there to help with the recovery, to offer their kindness which has helped the family and Jim, keep their spirits up. A special thanks to all of you: blessings to you and yours, now and always.


The good Lord answered our prayers by letting us keep James here on earth. Thank you

Treasure the love you receive above all. It will survive long after your gold and good health have vanished.
Og Mandino



Ascending Aorta
n: The part of the aorta from its origin to the beginning of the arch .

Ascending Aorta: One of four sections of the aorta, the main artery that carries oxygen-rich blood from the heart. This section leaves the heart and then branches into the left and right coronary arteries that carry oxygen-rich blood back to feed the heart muscle.
When accompanied by a widening or ballooning part of the wall of the aorta (aortic aneurysm), the condition is referred to as a dissecting aortic aneurysm. Dissections can be either ascending or descending depending on whether the vessel damage occurred in the part of the aorta that rises up from the heart:

(type A) or the part that descends down through the chest (type B).

While both are serious, the ascending aortic dissection is considered the more dangerous of the two.

The incidence of an aortic dissection is about two (2) in 10,000 people. There are an estimated 2,000 to 10,000 aortic dissections a year in the United States.
However, the condition may be underreported because it is difficult to determine whether death was caused by an aortic dissection, a heart attack or (sudden cardiac death), without an autopsy.
When untreated, an aortic dissection is fatal to 33 percent of patients within 24 hours, 50 percent within 48 hours, and 75 percent within two (2) weeks of onset. This is why early recognition and treatment of an aortic dissection is crucial.

Aortic aneurysm
An aortic aneurysm is the dilatation (widening or bulge) of a portion of the aorta, usually at a weak spot in the aortic wall. The aorta is the largest artery in the body. It carries all the blood that is pumped out of the heart and distributes it, via its many branches, to all the organs of the body. The aorta projects upwards from the heart in the chest and then arches downwards, travelling through the chest (the thoracic aorta) and into the abdomen (the abdominal aorta). The normal diameter of the abdominal aorta is about one inch.

What causes an aortic aneurysm and who is at risk?

Most aortic aneurysms occur in the abdominal aorta, the main cause being arteriosclerosis. This is a condition in which fatty deposits are laid down in the walls of arteries, which are less elastic and weaker as a result. Major risk factors for arteriosclerosis are smoking and high blood pressure, although it also probably runs in families.
Other causes of aortic aneurysm include:

Trauma to the aorta, for example a crush injury to the chest following a car accident,

Inflammation of the wall of the aorta,

Rare hereditary conditions such as Marfan's syndrome (a major cause of thoracic aortic aneurysm),

Syphilis

There is another, more rare type of aneurysm called a 'dissecting aneurysm' which occurs mainly in the thoracic aorta. This is a very serious condition, in which the inner wall of the aorta develops a tear and then rips off inside the aorta due to the pressure of the blood flowing over it.

Many people can have an aortic aneurysm for years before any symptoms develop. When they do become evident, symptoms vary according to the type and location of the aneurysm.

Symptoms of an abdominal aortic aneurysm include:

A pulsating feeling in the abdomen.

Abdominal pain,

Back pain

If an aortic aneurysm becomes very large, it can rupture (burst). This causes excruciating pain in the abdomen and back. There is severe internal bleeding which is often fatal. The symptoms of a thoracic aortic aneurysm often do not occur until the aneurysm is quite large. They are usually caused by pressure from the aorta on surrounding organs in the chest and include:

Other causes of aortic aneurysm include:



Pain in the upper back.

Coughing and wheezing,

Horner's syndrome (drooping eyelid, constricted pupil and dry skin on one side of the face),

Hoarse voice,

Difficulty swallowing

If a thoracic aortic aneurysm ruptures, it causes excruciating pain high in the back and often in the chest and arms. The pain may then go down into the abdomen and lower back. Massive blood loss can rapidly lead to death.

The symptoms of a dissecting aneurysm include:



A ripping sensation within the chest.

Severe pain in the back, often between the shoulder blades (mine was in my neck & head)

Blockage of smaller arteries which lie in the path of the dissection leading to

Stroke

Heart attack

Nerve damage

Kidney failure

Hoarse voice,

Difficulty swallowing

How do doctors recognise aortic aneurysms?
Aortic aneurysms can be diagnosed from their symptoms when they occur but this is often too late. They are usually found on routine physical examination and chest and abdominal X-rays. On examination, your doctor may feel a pulsating mass in your abdomen which may be tender if an abdominal aortic aneurysm is large. If your doctor suspects an aneurysm, he or she will probably request that an ultrasound scan is carried out. Other scans such as computerised tomography (CT) and magnetic resonance imaging (MRI) may also be performed: these are very useful for determining the exact position in the chest of a thoracic aortic aneurysm.

Dissecting aneurysms are diagnosed initially from their symptoms and physical examination of the pulses in the arms and legs. Other tests include chest X-rays, ultrasound scans, CT (often using injected dyes that show the flow of blood) and MRI.

What is the treatment for aortic aneurysms?
Self-care action plan
There are several things you can do to reduce your chance of developing an aneurysm:

Do not smoke

If you have a family history of arterial disease, have regular medical check ups

Have your blood pressure checked regularly

The treatment of an aortic aneurysm depends on the symptoms that it causes. If it is asymptomatic, your doctor will advise you on the best course of treatment, as surgery carries a risk.
Symptomatic aneurysms require early or urgent treatment. The surgical procedure involves replacing the part of the aorta affected by the aneurysm with a synthetic graft a tube made out of an elastic material with properties very similar to that of a normal healthy aorta. This very major operation is usually quite successful with a mortality of between five and two per cent. The risk of death from a ruptured abdominal aortic aneurysm is about 50 per cent, even during operation. However, there is no other treatment and an untreated ruptured abdominal aortic aneurysm is always fatal.

A thoracic aortic aneurysm is repaired using a synthetic graft usually if it is wider than three inches. Surgery on this type of aneurysm is more risky, with a death rate of up to 15 per cent.
The treatment of a dissecting aneurysm initially involves lowering the blood pressure with drugs to reduce the force on the tear in the aorta. Then the surgeon must decide if it is safe to attempt to replace the dissected part of the aorta with a synthetic graft. The closer the dissection is to the heart, the more likely it is that surgery will be performed. The majority of people with an untreated dissecting aneurysm will die within a few weeks. However, if the operation is successful and the patient survives the first few weeks after the operation then the outlook is quite good so long as the blood pressure is carefully monitored and controlled

An aortic dissection is the primary cause of death for Marfan patients.

Now if the above information is not bad enough, I also had a type A Ascending Aorta dissection. All in all I had two dissections.

Type A:
All dissections involving the ascending aorta, regardless of the site of origin.

Diagnosis: Type A Aortic dissection.

Acute aortic dissection is the most common emergency affecting the aorta, with a reported fatality rate of 36-72% within 48 hours. Without intervention, approximately 62-91% of patients will die within the first week. Aortic dissection consists of a tear in the intima with subsequent development of an intimal flap secondary to flowing blood dissecting along the intima. There are multiple predisposing conditions including hypertension (most common), connective tissue disorder, cystic medial necrosis, and Turner's syndrome. In addition, pregnancy and aortic stenosis have been described as being related. Type A dissections generally require surgical intervention while Type B dissections can often be treated medically.

Patients who are suspected to have aortic dissection are considered a medical emergency. Hemodynamic monitoring, cardiac rhythm, and urine output become of vital importance. Surgery, often emergent, is generally required to correct the problem. Surgery would usually involve placing the patient on cardiopulmonary bypass, deep hyporthermia, and circulatory arrest. Dissected part of the aorta is then resected and a prosthetic graft is placed between the two ends of the aorta.

Since many of you reading this page are not familiar with the medical terms, I have put a simple Discriptionary of the terms used to help you understand.

Aneurysm - a ballooning-out of the wall of a vein, an artery or the heart due to weakening of the wall be disease, injury or congenital defect.

Artery - any vessel that carries blood from the heart to the body.

Catheterization - an examination of the heart by passing of a thin tube (catheter) into a vein or artery and pushing it into the heart area.

Congenital heart defect - a heart defect present at birth.

Mitral valve - the heart valve between the left atrium & left ventricle.

Nitroglycerin - a drug that causes blood vessels to widen and therefore increase blood flow, used in the treatment of angina pectoris.

Angina Pectoris - chest pain caused by insufficient blood to the heart muscle.

Dyspnea - shortness of breath.

Pleura - the membrance that envelopes the lungs and lines the chest cavity.

Inguinal hernia - a disorder in which a loop of intestine protrudes into the groin, often the result of strain from heavy lifting, coughing, or accidents.

Dysphasia - difficulty in speaking or understanding speech, due to a brain condition.

Peripheral neuropathy - The term peripheral neuropathy describes a problem with the functioning of the nerves outside of the spinal cord. The symptoms of a neuropathy may include numbness, weakness, burning pain (especially at night), and loss of reflexes. The pain may be severe and disabling. Peripheral neuropathies are fairly common and usually present in the feet initialy with numbness, tingling or pain. Approximately one half have a known cause. The two most common causes are alcoholism and diabetes mellitus. Rarer causes include kidney and liver disease, B12 deficiency, thyroid problems, and certain cancers. Drug treatments such as mood elevators and anti-seizure medicines have shown some benefit.

The scary part of the whole thing is that our family Dr. stated that he thought that I had an aneurysm of the heart (aorta) and sent me for many tests. I cannot even remember how many as there were so many. All came back negative. The family Dr. did however keep a close eye on me. I do not place any blame on the family Dr. as she/he does only go by what the people who read the results tell her/him.

Surgery for Marfan syndrome is aimed at preventing dissection or rupture and treating valve problems.

Aorta surgery

About 40 percent of patients will die immediately if aortic dissection occurs. The risk of death is between 1 and 3 percent per hour after the dissection event. Even with emergency surgery, the risk of death is between 10 and 20 percent. The goal is to perform surgery prior to the dissection occuring because:

he early results are better (better than 98 % survival)

The long-term life expectancy is better.

The normal aorta measures about 1 inch (2.54 centimeters). When the aorta diameter is more than 4.7 centimeters, or if the aorta is enlarging at a rapid pace, surgery is recommended. The decision to have surgery is based on size of the aorta, expected normal size of the aorta, rate of aortic growth, age, height, gender and family history of aortic dissection.
During non-emergency aorta surgery, the survival rate is greater than 98 percent.
Surgery involves a replacement of the dilated portion of the aorta with a graft. This can involve two techniques:

Traditional method: replace the aorta with a graft and the aortic valve with a mechanical valve

Valve sparing method: replace the aorta with a tube graft and re-implant the native valve

Our bodies are like computers, once offline, they must be fixed to put them back online. Many times it is frustrating to see a loved one who is ill, or does not comprehend what you are saying, for some being able to function in a semi-normal capacity is a challange. Family and friends are important to anyone who is ill, or recovering from trauma, as well as to the other members of the family. It is quite difficult when you have had an active lifestyle and then in a moment everything changes. Patience, Love, Understanding, Faith, Compassion, and Prayer can help not only the person whom is ill, but also the others that are involved. Medicine has come a long way over the past 20 years, but more still needs to be done, it takes many hours of research and money to find cures for diseases, if you can find enough money to buy a cup of coffee, a candy bar, or a pack of smokes, donate the same amout of money you are spending to find cures.
Coffee $1.25
Donate $1.25 etc.
When you have 5.00 saved up give your donation.
Thanks.

www.marfan.org Visit their website to find out more on how you can help save lives, and donate. Thank you from our family and from the many others that may be affected with Marfan Syndrome.









My Corkboard



kjnatzke@netscape.net