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.