My Christmas Wish

My niece Ly, Kairit's sister, sent me this poem today:

May all beings have happiness
and the causes of happiness.

May all beings have no suffering
nor the causes of suffering.

May all beings dwell in supreme bliss
free from all sorrow and suffering.

And may all being rest in great equanimity
free from all attachment, addiction, indifference and aversion.

And may all beings wake up
to recognize their true self-nature.

An emptying glass...

There have been no words for a long time.

The lack of information is not unlike the lack of treatment and no cure when one is diagnosed with ALS. You might as well disappear into a black hole. Research has yielded some genetic markers that may one day lead to a cure but Kairit's glass is now less full. It will be too late for her. 

There is no good news these days. Kairit spends her days in bed and requires help to sit up. She is dependent on her mother and her boyfriend, Anto. Despite their blowup a few months ago, Anto has hung in and has been supportive. It has not been easy.

Kairit is in a very angry stage as she realizes that she is not going to improve. Her muscles continue to deteriorate and her mood darkens each day. There is no doctor, no treatment, no hope of getting better. Whatever is killing the motor neurons in her body is also killing Kairit's spirit.  The family feels a sense of frustration and the glass that was is now almost empty. Kairit insists on staying home for as long as possible. Without Anto, this would already be impossible as he is the only one strong enough to carry her to the WC and the shower. The desire of the patient can cause stress on everybody.  But love conquers some of the difficulties.

My half-sister, Aino, is also not well. She will soon undergo tests for severe stomach pain that has worsened over the years. Estonia's health care system needs an overhaul as well. It takes too long to see a doctor...too long to get tests...too long to get results....too long....while patients suffer and people die.

I am not in a particularly good mood. I try not to let the problems of loved ones so far away and so much out of my control overtake my optimistic self, but the holiday season of 2010 is not cheerful. What we need is a miracle.

From Bad to Worse

Kairit fell yesterday on the way to the bathroom in her apartment.  Her left leg completely twisted and bent backwards.  It is fortunate that Anto was there. He called for an ambulance right away. Kairit was taken to the local hospital where an x-ray showed she had torn the ligaments and tissue in her leg up to the knee.  The doctors sent her home with a brace and told her to keep her leg elevated and still for the next week. They recommended ice packs and offered her pain medication. Kairit refused the medication because she is concerned about the interaction with the Imuran.

I only heard about this terrible turn of events this morning as I skyped with my sister-in-law Margit and then Kairit's younger sister, Hurmi.  Health care in the USA is often determined by one's insurance coverage. It is no different in Estonia where universal coverage gets you some help but private coverage may get you more.  In addition, many of the doctors are not motivated to help when care is needed. One of my nieces near Tallinn had a fever for two weeks and despite calling her doctor requesting an appointment citing her fever, stuffy nose, blocked ears and pain in her face, it wasn't until she was well in her third week of discomfort that the doctor reluctantly gave her time. He then prescribed antibiotics after which she was better in a few days. In the meantime, she had lost more than two weeks of work and suffered unnecessarily.

In Kairit's case, she is only one of three patients in Estonia (with a population under 2.5 million people) who have ever been diagnosed with ALS.  There is little knowledge among doctors there about the disease and patient care and no incentive to learn more.  Kairit's doctors at Tartu University Hospital are doing the best they can but it is also up to patients to advocate for themselves and the country's citizens to demand more.

With that in mind, I suggested that despite it being a weekend, that Hurmi try to reach Kairit's main physician at Tartu to make her aware of Kairit's accident and acute pain and her reluctance to take any pain medication until discussing it with her doctor.  ALS is not a painful disease, although horrific, but taking strong medication usually given to cancer patients and then having severe pain from an injury and the psychological curveball in trying to stay positive is almost too much for Kairit to bear.

Life has gone from living in one room....to a nightmare.

A Visit to Estonia

The past two weeks have been a reality check in Estonia. Kairit's life now takes place in one room of her comfortable apartment in the small town of Haapsalu. My visit confirmed suspicion that Kairit's optimistic nature and sense of humor belied the truth of her situation. When her boyfriend Anto is available, he carries Kairit into the shower that has been newly redone to suit a disabled person for bathing. However, one needs to be able to step up about six inches to get into the shower

room and Kairit is unable to do that.

Over the past few months, Kairit's mother, my half-sister, Aino, has taken on more and more responsibility caring for her. She can still walk a short distance to the bathroom with the help of a walker and a strong hand guiding her. Her gait, however, is less steady. Kairit is also having trouble sitting up in bed without help. She has less use of her hands and her fingers appear to be thinner and atrophied. When she's on her laptop computer, she uses her curled fingers to type on the keyboard.

To make matters worse, she and Anto have used up their mutual goodwill and their relationship is tenuous. Kairit understands that he is healthy and well and needs to move forward with his life. She does not want to be a burden to him or unkind but sometimes her anger at her situation gets the best of her. Anto has been a good help but the time is coming when her physical situation will require more trained and able caregivers. Aino will care for her daughter as long as possible with help and occasional weekend relief from another daughter who lives an hour away but works and has a young son. Kairit wants to stay home as long as possible but ALS is taking its toll physically, mentally and emotionally.

There is also still no consensus on her diagnosis. One of her doctors believes that it is yet possible that she has MMN (multifocal motor neuropathy) and has prescribed a drug called Imuran. The drug that is often used by some cancer patients is being used off-label here. She may experience nausea, dizziness, and other symptoms or she may have no reaction at all. My understanding is that if the drug helps her in a 4-5 month period, then she has the autoimmune system disease. The drug could itself cause cancer in the long term but Kairit felt it was worth trying for a few months to see if anything changed in regard to the motor neurons. In any case, neither ALS or MMN is a good diagnosis although with MMN one can live longer. In Kairit's case, does that mean a continuation of life in a room or would she be able to work, get out of her apartment, and lead a more normal existence? Or is MMN simply three different letters to a similar health issue?

Life in a room. Eat. Sleep. Watch TV. Read. Google. Email. Occasional visits from friends. Wait to deteriorate. Cry...while loved ones also cry and hearts break.

The fight continues...

Kairit and I spoke a few days ago.  She had been to see her doctors at Tartu University Hospital. .)  It seems one of her doctors is still not yet sure that Kairit has ALS. They want her to try what she calls a "chemistry" treatment called cyclophosphamide (cytoxan), a chemotherapy drug normally used for some types of cancer. It is also used in treating MMN or multifocal motor neuropathy. There are many possible side effects to the cyclophosphamide

treatment.

Kairit then sent me the following email...

"I think I'm not doing this. I'm sooo weak. I need my good blood and good bone marrow.

And I need to eat and never be sick and throw up. I really don't know- can I throw up or do I suffocate in the first 5 minutes? Experiments?

Doc say- the chemistry I need do in to the vein and that I need to do it again in 6 months.

But I have 2 months to thinking about this. Today and tomorrow I know- I'm not doing this. If I need to die, then without throwing up. :)))  And - maybe stem cells started their work. I have time for this."

In the meantime, I will continue to send her protein powders and vitamins and mineral supplements to keep her body as strong as possible. She feels well, but for the muscle weakness in her hands and legs.

I cannot advise her as to what to do. I understand the reluctance at trying such difficult treatment that can cause toxicity and so much harm to one's organs. Life is precious but if the treatment is not a cure but ravages your body while possibly giving you some more time…

What quality of life is there?  What price?  These are questions only the patient can answer. I wish my niece the gift of time lived as well as possible.

"Hurry up, hurry up!"

My heart is breaking for my half-sister, Aino Krikk. She has already lived through so much pain in her life. Her father (my father) was forced to leave his first family behind during WWII when the Soviet army marched into Estonia in September, 1944. Had he not left at the urging of his wife (Aino's mother) the entire family may have been killed instantly or perished in Siberia. Aino and her three brothers and mother were led to believe that Paul Leek had died when his documents and wedding ring were found the nest day. Years later in 1962 they learned that he had remarried and lived in Toronto, Canada. Aino felt great sadness and feelings of abandonment by her father....and life in an occupied homeland under communist rule was hard and dark. But nothing can compare to watching her youngest daughter, 39-year-old Kairit,, on death's march with a disease in which muscles waste away. It is as if one's heart is whipped every day.

I spoke with Kairit this morning. Her disposition remains stable but her body is not. She is getting weaker. She is losing her ability to walk, even awkwardly. Her hands are weaker. The February stem cell treatment gave her a shot of optimism, but it did not have all of the right ingredients. I watched an episode of Charlie Rose that dealt with inventions and science. He quoted Einstein who said something like "If you ask the right question, you have 95% of the answer. " What is the right question about ALS? What is the answer?

I told Kairit about the event held this past Saturday at the Century Plaza Hotel, a fundraiser for the ALS Association that honored some notables in the fight against ALS. They included actress and ALS celebrity spokeswoman Kate Linder, whose brother-in-law had ALS; Jim Barber, an ALS patient and attorney who advocates with great success for patient rights; Dean Rasmussen, whose father had ALS, who is a tireless advocate, generous benefactor and meaningful fundraiser, and Dr. Clive Svendsen, PH.D, who is the Director of Regenerative Medicine at Cedars Sinai Medical Center that provides ALS care and research into the cause, treatment, prevention and cure for ALS. Dr. Svendsen illustrated with the use of slides the exciting promise of stem cell research for treatment of ALS.

I shout, "Hurry up, hurry up!"

I had the good fortune to sit next to a doctor working with Cambria, a pharmaceutical company that is developing a drug for ALS. It is not a cure but one that has shown in mouse studies to prolong life longer than Rilutek, the only drug currently available. Clinical trials are next.

I shout, "Hurry up, hurry up!"

I know, of course, that science does not hurry up. Sometimes there are "aha" moments that lead to further results but it's generally a long and tedious road, especially when it involves the NIH, the FDA, or any government institution. On one hand, they protect. On the other, they hold back on the side of safety. One death in a clinical trial, even if the patient was at death's door, can set back progress by years. In a world where just about everything is rushing at us at warp speed, cures for diseases seem to be in analog.

I shout, "Hurry up, hurry up!"

Can a dream become reality?

This past Sunday I attended a fundraising event for Medicine for Humanity headed by Dr. Leo Legasse. The non-profit group takes doctors and nurses from UCLA to underserved countries (most recently Uganda) and teaches the local professionals newer diagnostic and surgery skills.

However, the Westerners who do this invariably say they learn more than those they teach. They are reminded of why they became doctors and nurses. They work under difficult conditions at a small rural hospital where resources are limited and lives hang in balance. They do what they can and the baby steps lead to bigger ones.

It's not unlike the first trip to the Moon. People said it couldn't be done. NASA did a lot of planning and training. It took time. Then the astronauts did it. "One small step for man. One giant leap for mankind." The dream became reality.

In the beginning when I learned of Kairit's diagnosis, I went on warp speed. I devoured the internet looking for information on ALS. Surely there had been advances since Aunt Geri had it. I wanted to know everything about symptoms, clinical trials, and best doctors. I wanted to raise $25 million for research. I recently learned that is the same amount the ALS Association is hoping to get in government funds during its Advocacy and Public Policy days in Washington, D.C. on May 9-11. So, I have had a reality check. We are going to take baby steps.

I heard today that the friendly lawyer from Canada who also had stem cell treatment in Duesseldorf when Kairit did, is not doing so well. He, like she, has not had any noticeable improvement. But Kairit has remained stable. His condition has progressed and he now needs a wheelchair to get around. He works on a part-time schedule. He's going to Sunnybrook Hospital in Toronto to check out a clinical trial. Thus is the struggle of ALS patients...a constant search for hope. Unlike most diseases where we have a potential wellness partner in drugs, in chemo, in surgery or in some other kind of therapy, ALS has no such offering.

Kairit is bored at home. It's tiresome even though she does little and can do very little for herself. She keeps in touch with friends and writes emails to thank those who have contributed to a fund at an Estonian bank to help pay for her care. There are many generous people, strangers, in fact, who have sent money to help Kairit cover the cost of the stem cell treatment. She survives on a small government stipend for disabled persons, just enough to cover basic necessities.

We continue to look for a clinical trial in Europe that she can participate in but thus far, to no avail. Her next appointment at Tartu University Hospital is on April 19 but there is no joy in noting that on the calendar. What can the doctors offer? Her son, Kent, leaves Estonia that day for England where he will live with friends and seek his future hopefully as a trained chef. Kairit misses him even though he has not yet left. Being a single mom can be lonely sometimes. Being a single mom with a terminal disease and a son beginning his life's journey is hard to bear. On one hand Kairit is happy she gave her son wings to fly. On the other hand, it's hard fighting ALS without a smiling, familiar face and a child to hug.

We talked about it today...

Life took on new meaning today in my skype conversation with Kairit. It happened when we talked about death.

Kairit told me that Silvi died at the end of March. She was 62. In the summer of 2009, the former seamstress who retired to a country home was having trouble swallowing and talking. She had lost weight. She was at Tartu University Hospital for tests in November at the same time that Kairit was undergoing an IVIG treatment. At the time it was still unclear as to whether or not Kairit had ALS.

Kairit and Silvi shared a room and the older woman helped Kairit who had lost much of the use of her hands. Silvi opened her milk and juice cartons…cut her food if necessary and so on… They became close. Silvi’s daughter phoned Kairit with the news that her mother had died.

Kairit was stunned that her new friend had died within five months of their meeting. It shocked her how fast Silvi’s ALS had claimed her life.

Our conversation turned more emotional as we talked about life and death. Silvi leaves behind two daughters and a young grandchild. Kairit has a young son, 19, and many family members and friends who pray for her every day. Her mother, my half-sister, Aino is a religious person of great faith who attends church regularly. What must she ask of God? How do we seek a miracle or do they just happen? Will the ALS researchers find a treatment or a cure in time?

Kairit does not want to die. She says, however, that she does not want to live in a vegetative state, either, when the mind sees but nothing else is alive.

We cut our conversation short today. She, to go to bed. Me, to search the internet, looking for a miracle.

I’m going to get real today. Maybe $25 Million is a stretch. Let’s just work to get as much as we can to the ALS Association as soon as possible. I’m looking for a miracle. Got one?

To visit our Family Fund page and make a donation, please click here.

April update

It has been a crazy full moon week. I fell while walking two dogs, one large, one medium-sized, that took off with me on their leashes. As I was sitting in the orthopedic doctor’s office listening to my diagnosis (fracture of the rotator cuff bone) and the prognosis I felt fortunate. Wearing a sling for several weeks and physical therapy is inconvenient and not fun but my broken bone is not a life-threatening illness and there is treatment. Kairit is not so lucky.

In conversations with people who work in ALS research and support services, the key in finding treatment and a cure is money. The treatments for cancer that have resulted in remissions and cures for patients have come as a result of billions of dollars into research and development. ALS has been underfunded in part because it affects fewer people per capita.

The following information comes from the National Institute of Health website:

As many as 20,000 Americans have ALS, and an estimated 5,000 people in the United States are diagnosed with the disease each year. ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. ALS most commonly strikes people between 40 and 60 years of age, but younger and older people also can develop the disease. Men are affected more often than women.

In 90 to 95 percent of all ALS cases, the disease occurs apparently at random with no clearly associated risk factors. Patients do not have a family history of the disease, and their family members are not considered to be at increased risk for developing ALS.

About 5 to 10 percent of all ALS cases are inherited. The familial form of ALS usually results from a pattern of inheritance that requires only one parent to carry the gene responsible for the disease. About 20 percent of all familial cases result from a specific genetic defect that leads to mutation of the enzyme known as superoxide dismutase 1 (SOD1). Research on this mutation is providing clues about the possible causes of motor neuron death in ALS. Not all familial ALS cases are due to the SOD1 mutation, therefore other unidentified genetic causes clearly exist.

We need to find treatment and a cure now. We can do this. There is much data compiled over the past decade. We know what happens in the body of an ALS patient. Now we need to find out how to stop the disease from progressing, what causes it, and how to cure it. There are many clinical trials going on. If we can put the researchers into a room for a few days to discuss findings, give them the face-to-face time to go over data, then perhaps the goal of finding treatment and a cure might be closer.

Kairit is worthy of this goal. Everyone with ALS is worthy of this goal. It does not matter that fewer people in the world get ALS than cancer or some other devastating disease. Life is precious for all. Let’s give everyone a chance to live it to its fullest.

Humbling generosity keeping hope alive

Just for a moment, I'd like you to step into Kairit's shoes. One day you are walking in the sand at the beach, playing volleyball and riding a bike. You feel strong, vital, and healthy. Then, slowly, over a brief few months your muscles begin to fail you. The doctors put you through a battery of tests. They don't know what's wrong. One suggests you need to be in a psychiatric hospital because he never heard of such a thing as muscles feeling like popcorn in your body. Another says you are just nervous and need Xanax. You can no longer climb stairs, or walk with your head held high, nor quickly nor far. You can no longer work. The muscles in your hands are weakening, too. You can't brush your hair because you can not hold a brush.You hold a fork and knife like a child holding a tool. Someone needs to cut your food.

You finally find out from the doctor that you have amytrophic lateral sclerosis, or ALS. When you ask what it is, you learn that it's a difficult problem for which there is no cure. And, oh, there's no treatment either.

We need to change this. Several months ago Kairit's sister, Hurmi, reached out to friends and family to help Kairit fund the stem cell implantation therapy she underwent about ten days ago. Kairit lives on a small government stipend for which she is grateful but does not provide any extras. We have learned that people are generous. The family is grateful for all the assistance.

Just today Kairit learned that a very generous Spanish gentleman donated a large sum that will cover all of the medical expenses Kairit incurred at the X-Cell Center not yet paid. Such kindness is overwhelming! It enables Kairit to focus on staying healthy with an eye on the future.

We have skyped this past week. Kairit has not had any noticeable effect yet from the stem cell treatment but she looks and feels well. There is no noticeable deterioration either. Hope is still alive.

We have started a campaign to raise funds for the Los Angeles Chapter of the ALS Association for a symposium to bring together ALS researchers from around the world. There has never been such a gathering.

This year on June 27-28 the ALS Association is holding its first two-day symposium near Sacramento. It will bring together local and regional researchers who no doubt will welcome the face to face time to discuss the data that has amassed on ALS. I so hope it will bring new ideas and help Kairit and others live.

If you would like to donate to the Leek Family Fund and help us make this international symposium a reality, please click here.

Donations earmarked to the Leek Family Fund can also be mailed to the ALS Association at:

ALS Association - Greater Los Angeles Chapter
Attn: Leek Family Fund
28720 Roadside Drive, Suite 200
Agoura Hills, California, USA

On behalf of Kairit and the extended Leek family, thank you.

Note: Videos from our trip to the X-Cell Center in Germany are forthcoming...

Thoughts on the last night in Germany

Kairit's blue eyes filled with tears that fell on her cheeks. We were having dinner in the hotel restaurant on our last night in Duesseldorf. She talked about the impact ALS has had on her life. She was an artist who worked in a flower shop while honing her craft and searching for work. She studied theatre art and glass painting. Finally about five years ago the hobby that brought her joy turned into work that she loved and could live by.

She began illustrating children's books and greeting cards. For several years, Estonia's largest chocolate company, Kalev, hired her to design their Christmas packaging. She taught art classes at the Tartu Art Institute and found satisfaction in having students find their talents through her teaching. She was able to buy a small apartment in a rural area and life was good.

Estonia is known for its cultural activities and Kairit's favorite work was in the theatre where she painted masks and actors' faces. It's in a small regional theatre in the summer of 2008 where she met her partner, Anto Siimson. Anto, who works as a musician and furniture craftsman, was performing in a play. He has been by her side ever since - in the beginning when the struggle to get a diagnosis was so frustrating and painful, and since, acting as a caregiver against what could become insurmountable odds. He's loving, kind, thoughtful and strong, lifting her out of a chair, carrying her when she cannot climb stairs or get into a car. He's a helpmate at home, too, with cooking, cleaning, and whatever else he can do. Most important, he makes Kairit feel beautiful, and despite the muscles that have shrivelled and shrunk, that she, Kairit Krikk is the most wonderful woman in the world.

Kairit's medical experience has been good at Tartu University Hospital except for some confusion as to whether or not she has ALS or MMN (multifocal motor neuropathy, a non-fatal motor neuron disease.) Not all the doctors agreed on the diagnosis (not unusual in cases of difficult diseases in which the diagnosis results from the elimination of possibilities.) Kairit says when she had the IVIG treatment (Nov. 2009) usually prescribed for MMN she was doing better, able to climb stairs carefully and the muscle-wasting did not seem to progress. That benefit lasted until the end of January. However, her next appointment was not scheduled at Tartu until mid-April, and despite her many efforts to get it moved up, she could not.

The stem cell treatment, she thought, was the only option to try and hold off any further damage to her body. She is hopeful that it will help but it may take a few weeks to ascertain what, if any, improvement there is.

I was upset to hear Kairit's story about a Dr. Katrin Gross-Paju who is known as a top neurologist in Tallinn, Estonia's capital. She could use some sensitivity training. She's not unlike some doctors we have in Los Angeles who spend more time on their television careers than paying attention to their patients. I once waited in a well-known female ob-gyn's patient room for three hours...thinking she was dealing with an emergency...when she breezily walked in and said, "I'm so sorry. The front desk should have called you." Turned out the doctor had a last-minute TV appearance. I never saw her again.

Dr. Gross-Paju's office ordered a muscle biopsy done on April 7, 2009. When Kairit called repeatedly to get the results over a period of six months, she was told by the doctor that the results were not in and she needed to wait. Kairit did not know if such a long wait was normal. Kairit had no way of knowing that the lab had sent back the results by April 27 noting the muscle biopsy (a painful procedure) needed to be repeated because the arm tissue was not enough. It had to be done on the leg. It was only when Kairit's older sister emailed the laboratory that they found out the results had gone back to Dr. Gross-Paju's office six months earlier. Kairit then proceeded to have the second biopsy which indicated ALS.

Kairit's final appointment with Dr. Gross-Paju was demoralizing and difficult. When Kairit asked about her options she was told there were none. That's true, there aren't any.

When Kairit asked if she had any help to give, the doctor said, "I can get you a breathing machine."

Kairit: "But I don't have any problem with my breathing."
"You will, and then you will come back to me," says Dr. Gross-Paju.
Kairit: "But what can I do? What help can I get?"
"I can do nothing," says Dr. Gross-Paju, "You are going to die."

Here you have a young woman who has just been diagnosed with the most awful disease...in a country with no supportive ALS services...whose tests were delayed by six months...who had nowhere to turn, no knowledge of the death sentence that had just been pronounced. Some kindness would have been welcome, even in some gentle words, saying "I am sorry for your situation. I would like to help but to date there is no cure and no therapy. We will keep in touch and I will assist as the need arises."

Doesn't take away the shock but helps to cushion the blow.

Kairit's Blog for Estonian speakers

To read Kairit's personal blog, written in Estonian, click here.

Procedure done and now we hope...

Kairit, our family soldier in the fight against ALS, was released a day early from the X-Cell Center hospital. Except for the slight fever last night and a sore neck from the positioning of her head during surgery, she has had no complications.

When I walked into her hospital room at nine this morning, she had already showered, had breakfast, put on a little make-up, and settled into the comfortable hospital bed to watch TV. The doctor had been in to see her and was very happy with her progress.

I gave Kairit a little shoulder massage and we walked (Kairit with Anto's help of course) to the reception area for a good cup of coffee. I met Margeurite, one of the administrators at the center. There are two locations in Germany but the owners are from the Netherlands. Marguerite said many of the ALS patients they have seen have worked in jobs involving toxins. While not everyone who works with chemicals gets ALS, obviously the immune systems of some people

can't handle the poisons.

Kairit said her first symptom was the feeling that her muscles were like popping corn all over her body, especially her legs. They never stopped. The popcorn feeling was intense at the beginning but has abated over the past nearly two years. Kairit was an artist working with paints and pencils but she also worked in a flower shop for 5 years.

Is ALS caused by toxins? Doctors may say they don't know but we think it may be. Our environment is polluted and our workplaces and homes are filled with poisonous substances. We are not the first to think toxins may be responsible for many diseases such as cancer, immune system disorders, or motor neuron diseases. The list goes on.

Perhaps we could deal with the consequences if there were hope for survival but there is no treatment for ALS. Kairit knows that this amazing stem cell procedure she underwent is not a cure. We will have to wait and see how successful it was...but she has at least had a therapy that may bring some improvement if the detoxifying she has done the last several months has helped her, if it's even what's needed. In any event, Kairit - but for ALS - is healthier than she was six months ago. If the new stem cells are viable enough....So much emotion involved as I am

reading the doctor's report on the transplantation:

On 10:03:10 (Mar. 3/10)

stem cells were implanted in general anesthesia via neuroendoscopic

ventriculocisternostomie 3.0ml in high concentration: 1.0 ml in basilar cistern,

1/2 ml in third ventricle, rest in side ventricle an solution intraparenchymal.

We have transplanted 4 650 000* CD 34 cells (Vitality 90.1%)

In the United States they have begun clinical trials to see if the lumbar puncture procedure is safe enough. That phase will take two years. Then phase two another two years...then phase three...and maybe after 5 or 6 years the FDA will sanction this procedure which they are now doing at the X-Cell Center. Why can't the doctors in the USA come here with an FDA official and check it out? See what they do?

My new Canadian lawyer acquaintance who has ALS was also in the intensive care when Kairit was there. He was able to have the stem cell implantation through the occipital area. He was doing well and released to his brother for two days of rest before heading home. We saw other patients as well following stem cell procedures, including two young children. We don't know what medical problems they were being treated for but we can assure the reader they looked no worse for the wear. How well the stem cell implantation works is yet unknown for these patients but others have benefitted.

Surely when people's lives can be improved it would behoove the medical professions all over the world to work together, wouldn't it? ALS is a horrible disease that attacks the muscles, causes them to atrophy, with the most severe patients losing their ability to walk, use their arms or hands, swallow and breathe while keeping their minds intact. I don't care where the best therapy comes from to help my niece and other ALS patients. I don't care if the cure for ALS comes from China, Germany, Russia, Mexico, Canada, or the U.S.A.

I am going out on a limb here...but the lumbar puncture is safe, as is the occipital procedure and the neuroendoscopic stem cell implantation done by skilled neurosurgeons under FDA-type rules and regulations. At the X-Cell Center they use the patient's own bone marrow to harvest the cells. Maybe in the future stem cells from other sources may prove more beneficial but right now this seems to hold hope for some. Yes, it's a private facility and it costs money to do this but Kairit doesn't have anywhere else to go.

Kairit, Anto, and I will spend our next twenty-four hours watching television, reading, snoozing,

eating, and talking about the incredible week we have had in the multicultural clinic. There are people from many countries performing different specialties. The waiting room is just as multi-ethnic and when it comes to health and good care, we all look the same.

A step into the future...

Shortly after noon today, Kairit Krikk stepped into the future. She is not the first ALS patient to undergo neuroendoscopy stem cell implantation but to the best of my knowledge, she is the first Estonian to have it . She said this morning that faced with a dire prognosis it is a lot easier to take that step into what may one day be more commonplace surgery throughout the world.

A cure was not promised of course, but, if all goes well, Kairit will see improvement in her condition. She can have the therapy again in about a year and if we can fast track the ALS research data into meaningful treatment, perhaps we can find a cure for Kairit and other ALS patients.

Before she went into the operating room, she told Anto that if she came out of surgery and her favorite music was no longer Sting, Police and Billy Idol, he should have the doctor wheel her right back into surgery for a redo. Her mood was light and positive. Dr. Tamaschke told us that the minimum requirements for the stem cell procedure were two million cells at 80% viability. Word from the lab was that Kairit had 2.5 million cells at 88% viability. How could you not be optimistic?

In about an hour Dr. T came out with a smile. Everything had gone well. A small patch of Kairit's hair had been shaved...an incision made and a tiny endoscope would make its way in the "space" of the brain area carrying with it Kairit's stem cells to the area where the brain controls the motor neurons. I am oversimplifying this, no doubt to the horror of any brain surgeon, but I think it's good enough for us lay people. Suffice to say the "hole in the head" as Kairit joked was sealed and she was now going into the intensive care room where we could join her.

She received the first of two IV infusions this afternoon...some clear liquid that would help keep the brain from swelling. A tight green band was wrapped around the top of her forehead like a headband.

Kairit was awake and ready to talk.

"Anesthesia is not so bad," Kairit said. "I had a great sleep!" She said she felt fine but could feel the incision area and she had a slight headache...in her words "the kind you get sometimes in the morning, maybe after having too much wine."

The doctor came in to check on her and mentioned that Kairit could take home a dvd of the operation which shows everything going on instead the head. Kairit plans to show this to her doctor at Tartu University Hospital.

At about 2p.m. Kairit started drinking water...just about two hours after the surgery she ate a small sandwich...and a light dinner came about 6 for both Anto and Kairit. I returned to the hotel to Skype with my relatives in Estonia and my family in Los Angeles. News travels fast these days...

It is now nearly 9p.m. Anto just called from the hospital where he is spending the night. The doctor had checked in a couple of times. During the last visit she removed the tight headband revealing a small bandaid over the incision. Not much hair had been cut according to Anto. A nurse also took her vital signs...Kairit had the slightest temperature which we had been told could happen and was normal following such a procedure. She was given medication and would receive another dose at eleven. Kairit had also slept, used the bathroom, and was feeling okay and just a bit tired.

Being in the future is exhausting for all of us. Good night...sleep tight.

Anxiety and Anticipation at 2 a.m.

Can't sleep...My mind can not settle thinking about Kairit's bravery and others who deal with ALS or any motor neuron disease.

After dinner Kairit fell just outside her hotel room. Fortunately she did not hurt herself too badly...a little bruised on one arm. Anto had just opened the door but before he could reach her, the muscles, worn out after today's activities simply could not hold her any longer. I think of how active Kairit was prior to this disease. My Aunt Geri was, too...so are the men and women of the military who seem to contract ALS far too often. What causes the motor neurons to fail? How do we stop it before the muscles atrophy? Is stem cell treatment the answer? Is there something else? We need answers quickly. That takes money for research.

Kairit's friends and family in Estonia have wings on their shoulders for the donations they have raised and given to help her try the treatment she will have today. There are so many people who need help and who want to live. They deserve a chance, too. Maybe the big pharmaceutical companies don't put enough into research because there's not enough money in ALS for them. Most patients die rather quickly compared with say, a cancer patient. Do I sound cynical? It must be the late hour.

In the Name of Luck

One phone call can change everything. Our free day exploring the lovely city of Duesseldorf went up in smoke after Dr. T called to say she had gone over Kairit's MRI very carefully in preparation for Wednesday's procedure. She assured us that we did not need to be stressed but did want us to return to the clinic for another consultation.

One of the things that struck me about the X-Cell Center is how differently it operates from a doctor's office or clinic in the United States. You don't have to go through a number of secretaries or front office people behind closed walls with often-closed windows. There are nurses in the hospital area but the doctor comes in herself, even to the waiting area and greets you. Dr. Tamaschke invited us into her office but not before asking us if we wanted any water or juice.

It seems that Kairit has a sort of "sling" in the area near where the occipital implantation was to take place -- basically an obstruction that made it too risky to implant there. So we were back to either a lumbar puncture or a new option that was offered to her. Kairit did not need to make an immediate decision but a timely one was necessary. Dr. T said Kairit could undergo stem cell implantation via neuroendoscopy. This is a surgery requiring anesthesia and a hospital stay for 48 hours. Dr. T. has done over 600 of such operations during the last 10 years, many on children.

Needless to say, the thought of brain surgery can take one's breath away. However, as Kairit, Anto and I processed the explanation of the procedure and the potential of the help it might bring, we decided a leap of faith is sometimes needed in the face of dire circumstances. I asked Kairit how she felt after the doctor left us alone for awhile so Kairit could ponder her decision. She said now that she was here and despite the extra cost (an additional 10,000 euros) she wanted to try the newest procedure with results that have shown to be promising. No one said this was a cure but patients had shown improvement and could do stem cell treatment again. Perhaps in a year, new research would show even more promise.

When the doctor returned later, Kairit gave her the thumbs up. We then met a charming anesthesiologist who went through an intensive questionnaire with Kairit who had never had any surgery previously. He told her what to expect tomorrow and gave her the routine instructions prior to anyone having anesthesia. He promised me he would take good care of my niece.

Next, the driver took us to another facility nearby where Kairit was filmed as her walking and balancing abilities were tested. She also had to fill in a form asking many questions about the use of her hands, legs, etc. By this time Kairit was worn out physically and emotionally. Khalid Sousi, the physiotherapy specialist, showed us a film of a Norwegian man about 45 years of age who also had ALS and underwent the same procedure that Kairit is now scheduled to have. In the before footage, the man could not walk well and needed to hold the handrails. In the after (6 days later) he was walking much better without the use of the rails. Kairit was asked to return paperwork with the same questions and a film of her condition six months later if she were unable to return to Germany for the follow-up.

Just before the driver came to pick us up and return us to the hotel, Khalid asked if we knew the meaning of Kairit's name in Arabic. We did not. He said, "In Arabic, Kairit means lucky." I nodded, "From your lips to God's ears!"

The start of the week

This week began with great anticipation and emotional charge.

Kairit, Anto and I were picked up by a pleasant German driver, Reinhard Fischer. The X-Cell clinic was less than ten minutes from our hotel. It's located on two floors of a local hospital. We first filled in the paperwork.

Kairit had previously wired the funds for the procedure We then met with neurosurgeon Dr. Uta-Kristein Tamaschke. I wanted to ask her about her training and education but the efficient Dr. T put us immediately at ease. She clearly explained the procedures but recommended Kairit consider having the stem cell implantation in the occipital area at the base of the skull. She said that in her discussions with colleagues in other countries doing similar work with stem cells and ALS, the benefits of implantation in this area seem to outweigh the lumbar puncture. It takes a giant leap of faith to do any of these procedures but Kairit is determined to make the best use of the funds and time available to her. She will sleep on her decision.

There were others in the clinic as well including two boys who seemed to be in their teens. I spoke with a lawyer from near the Toronto area in Canada with a similar diagnosis as Kairit. He was first to have the bone marrow extraction and when he returned to the lobby reassured Kairit that it was relatively simple. I spoke with him for awhile. He, too, is frustrated by the lack of therapies for ALS. He was, however, grateful to live in a country where receiving healthcare was not a issue. I cannot imagine having motor neuron disease and no insurance.

We will rest this afternoon. Tomorrow is a free day. Reinhard secured a wheelchair for us and provided us with directions on the train into the city center. We will explore, eat, and take a day off from the ravages of a disease that needs research money and lots of smarts to fight it.

In Germany...

I arrived in Germany today, after a long flight from Los Angeles to London to Dusseldorf. Kairit and Anto met me here and we are just getting settled. There is a lot of anticipation, some fear, and definitely hope. Tomorrow, we go to the XCell Center where Kairit will get bone marrow extracted from her hip. The doctors and scientists there will then prepare the stem cells in a laboratory and implant them into her lumbar region on Wednesday.

Click here to read more about what the XCell Center is doing...

I've been taking video, photos, and notes and will be updating the blog with updates as to how the treatment and this weeks' journey goes. All we can say for now is that at the very least, it is keeping Kairit in good spirits and hopeful.

Notes following an ALS Association event last week

I went to the ALS Association Los Angeles Chapter event at the Skirball Center last Thursday night (Feb. 25). Billed as a "Decade to Remember," it highlighted the many achievements of the organization since it's early days, including legislation establishing a standard of care for patients, including those in the military. It seems those who serve our country are especially susceptible to ALS. Why is that? I want to know what causes this terrible disease.

My niece is the second loved one in my family to be diagnosed with Lou Gehrig's disease but the two relatives are not related. In the mid-1980's my Aunt Geri in Toronto was given the grim news. She was my mother's younger sister. I was working at KTLA News at the time so I wasn't able to get back to my hometown as often as I would have liked. I saw her at the beginning when she had trouble walking and used a cane. I visited a few times after that but she went downhill rather quickly, falling into a depression, and losing her will to live. The last time I saw her before she passed just after turning the corner on age 60, she was lying in a hospital bed, unable to move, refusing a feeding tube and the ventilator. I really had so little knowledge about the disease but I do recall being upset to know there was no therapy, no drugs, nothing available to stop or cure the problem.

I Skype with Kairit regularly so even though she is thousands of miles away, I feel closer to her than I did with my aunt. I also have searched the internet so I have a lot more information about ALS. Kairit is the youngest daughter of my half-sister on my father's side of the family. This is not a genetic link, merely bad luck to have this terrible illness strike twice.

One of the wonderful case workers, Jackie Neff, who is now retiring but has long been associated with the L.A. Chapter of the ALS Association was honored for her service at the Skirball event. She would go out of her way to do or get whatever it was that her patients and their families needed. Jackie exemplified the true meaning of a "Social Worker." I am glad that ALS patients in California have a caring and supportive network available to assist them. Many of the services are provided free by the ALS Association. I wish there were something like this in Estonia where Kairit lives. There is not. Even her doctor in Tallinn was insensitive to the diagnosis and what it meant. When Kairit asked her whether or not she should try stem cell treatment or the drug, rilutek, assuming she could get it, her doctor said, "I don't care what you do. You are going to die. Do what you want." I couldn't believe that a doctor could be so heartless! The patient and her family is left on their own.

Despite some moments of anger (why me?, why now?) and discouragement at the lack of therapy or a cure, Kairit has a positive attitude now. She has followed a healthy diet with lots of vitamins and minerals to boost her immune system. She has tried to detoxify as much as possible. Could it be there was some lead or other toxic substance in the paints she used to use for her work? She says she always bought the best paints, the most expensive ones...but who knows what unscrupulous manufacturers may have put in them? My aunt worked in the offices of the Reliable Toy Company. What toxins were there? Toys were made there...plastics, metals, rubber, and lots of other materials in an era when we did not know as much as we do today about air quality. Did whatever toxins there were cause the motor neurons to start failing? Is ALS cause by a virus or a bacterial infection? We don't know yet.

I want more answers. There is so much data that needs to be sifted through by those scientists who know about cause and effect...who can make educated guesses and hopefully find the answers we seek. That's why I want to raise the necessary funds to get them working together and trying something new. I want a cure for Kairit and all the other patients who have no hope at the moment.

A philanthropist who underwrites a baseball tournament that benefits the ALS Association and a family who raised $60,000 for the fight against ALS were honored at the Skirball as well. I left that evening encouraged by the warmth and commitment of the people in the ALS Association, content with the knowledge that even in today's economy, there are those whose hearts are huge. ALS kills but money for research can hopefully stop it in its tracks.

The faces of ALS

I found this video on YouTube and was moved. There are so many suffering from this terrible disease and not enough awareness. Please take a moment to watch. It's sad, but we need to be motivated by these stories and honor their lives or memories by continuing the fight to find a cure.
(If you can't read the script on the video because of the advertisements -- the link is here.)

Day One

My daughter Lindsay and I went to the ALS Association Los Angeles Chapter office in Agoura Hills today armed with questions and filled with concern. My 39 year-old niece, Kairit Krikk, the youngest of my half-sister’s three daughters in Estonia, was diagnosed with Lou Gehrig’s disease a few months ago. Just over a year ago, the muscles in her legs began twitching involuntarily as though the brain was not getting the message that the muscles had done their work and now could rest. Her doctor in the capital city of Tallinn told her she was just nervous.

“Is there something in your life causing you anxiety, my dear?” asks the doctor. She prescribed Xanax.

When the feeling of needles and pins began in her lower body, Kairit was sent to Tartu University Hospital in the heart of Estonia, a facility similar to a teaching hospital/university in the USA but without the resources. Estonia is the smallest of the three Baltic States, about one-quarter the size of Texas, with a population of less than one and a half million people.

After a battery of tests, including two painful muscle biopsies after the lab determined that not enough tissue had been taken the first time, doctors at Tartu pondered for a while as though they could not believe the results. They determined that Kairit has the motor neuron disease called Amyotrophic Lateral Sclerosis, commonly known as Lou Gehrig’s disease.

The younger generation may not be familiar with Lou Gehrig. He was a famous Yankee baseball player who at age 36 was diagnosed with the disease in which the motor function of the central nervous system shuts down, causing muscle wasting and paralysis while the mind stays alert. Patients usually die within three to five years. The vibrant, healthy-looking young man bore his cruel fate with such grace. You can watch Gehrig’s farewell speech from July 4, 1939 below.

Kairit is also full of grace, a gentle, creative soul…an artist who painted and illustrated cards and children’s books but has now lost the ability to do so. Her ability to walk is nearly gone and her hands no longer work. She survives on a small government stipend for disabled persons. In November, her sister Hurmi sent out an email to family and friends explaining Kairit’s debilitating condition asking for donations for Kairit’s care and angels to hear the prayers of Kairit’s 19-year-old son, Kent, her family and her friends.

Her photo is above.

There are absolutely no support services in the small rural area where Kairit lives nor anywhere in Estonia. We have a standard of care in California but there are no therapeutic treatments other than the drug, Rilutek. It is very expensive, has side effects, and only extends life for 2-3 months. There are clinical trials going on but nothing of consequence has yet been discovered.

However, according to what I’ve read on the internet and from emails I’ve received from research scientists I have reached out to, there is lots of data available. The key to unlocking the mystery of ALS may be in having these best and brightest of minds together for a duration of time to look at, decipher, and brainstorm ideas.

Kairit has decided to try stem cell treatment in Germany at the X-Cell Center. I am going to meet her and her longtime partner Anto, in Duesseldorf in early March. Not enough is yet known about stem cell treatments but a clinical trial is being undertaken in the USA at Emory University Hospital in Atlanta headed by Dr. Eva Feldman from the U of Michigan. The first phase of the trial is to determine if the procedure is safe and will take two years to study before Phase Two begins.

Who has time? Not Kairit.

The X-Cell Center is doing stem cell treatment now. So, Kairit is putting on her game face. She will undergo bone marrow collection from her hip bone. The stem cells will be developed in the X-Cell lab overnight and then implanted via lumbar puncture. Cost is almost eight thousand euros. Once there, Kairit has the option to decide to try the one in which the stem cells are implanted in the occipital area closer to the brain. Right now she is not sure if she’s up to having a hole in the head…too new…only two patients thus far have tried it with no results to report thus far.

“No ALS patient we know of has been cured or benefitted significantly from any stem cell treatment to date," Fred Fisher, President of the Los Angeles Chapter of the ALS Association told us today. “However, stem cells may very well hold promise in the future.”

This, of course, was not what I wanted to hear. Fred Fisher is proud of the achievements thus far by the organization he heads and pointed out a number of legislative and standard of care successes, particularly in California. In late June they are planning a two-day symposium in Northern California to bring together researchers to discuss data and clinical trials going on. This is where my interest lies.

Dr. Marie Csete, an ALS researcher, to whom I was introduced via email and telephone by a mutual doctor friend, mused to me one day in conversation…"It would be great if we could have all these best and brightest in a setting together for several months to really devour all of the findings to date."

When I asked how much money that would take, she answered, “Oh, about twenty-five million would be good seed money.”

That’s what brought Lindsay and me to Agoura Hills today. I may be naïve but I think we need to try and raise the money. There’s hope in that scenario where scientists can lock horns and throw ideas around. They can discuss the results of what has been tried thus far. If they just focus on this one disease, and not have to look at other diseases at the same time in order to make a living, then maybe someone or some small group of doctors will have a lightbulb moment - that “AHA! I think we need to try this. This might work.”

I want a cure but I would temporarily accept any therapy that would treat ALS as a chronic disease halted in the early stages when a patient can still walk, use hands, talk, breathe and swallow.

Lance Keene, the VP of Development for ALS in Los Angeles, explained how we can set up our own family page on their existing website, which we are working on...

Our goal will be to raise $25 million, specifically to fund a significant international research symposium and the subsequent research that must be conducted following the symposium's findings and conclusions. If over 100-million people can watch a televised football game, surely we can find 2.5 million people willing to send $10 or more to help people like Kairit who right now have no hope. There are angels out there…they just need to know where they are needed!