"He is right here," said Pranav's mother, in her lilting Indian accent; "would you like to speak to him?"
For a moment I was actually afraid; I don't know why.
Pranav had Spinal Muscular Atrophy (SMA) a disease like a slow spinal cord injury. Like the paralysis which afflicts my son Roman Reed, SMA is not "catching", and in any case we were on the telephone.
"Hello," said a surprisingly deep voice, very dignified. I had a sudden thought -- he sounded like a politician, a good one, somebody who would use the power of government to help people.
We talked about Disneyland and Elmo, both of which he strongly approved.
But the conversation was tiring for him, and we soon said goodbye.
He was three years old.
During the next four years I learned a little bit about SMA.
"Similar to ALS/Lou Gehrig's disease, but in babies, SMA eventually impacts every muscle in the body, hindering the ability to walk, sit, stand, eat, breathe, and swallow. SMA is degenerative. SMA is brutal, the #1 genetic killer of young children. And as of today, SMA is terminal."*
Pranav's mother had given up her career as a Certified Public Accountant (CPA) to stay home to fight for her son's life. When he had a cold, she would sit beside his bed, all night long, keeping him breathing. She sent me a picture of him, a handsome little boy in a black-framed wheelchair, and I put it on the wall beside my computer, where I can see it every morning when I write.
Pranav was paralyzed; my son Roman Reed was paralyzed... from a college football accident instead of a disease, but still there were similarities, and what might cure one, might help the other.
Spinal Muscular Atrophy. SMA, is a disease of the motor neuron, the cells which are located in our spinal cord... If there is something wrong, if the motor neurons become sick or die, then the nerve no longer carries the ... message to the muscles, and the muscle cells atrophy and die....
The motor neuron is a long nerve from waist to heel. What if stem cells could make new motor neurons to replace the ones lost by SMA? This could be the answer to not only SMA, but also related conditions like spinal cord injury, ALS (Lou Gehrig's disease) and others.
The same scientist, Hans Keirstead, who developed the embryonic stem cell research now in human trials for spinal cord injuries, is close to another FDA approved human trials, this time for the motor neurons to help fight SMA.
The Roman Reed Foundation did a fundraiser not long ago, "An Evening with Hans Keirstead", specifically to raise funds for Keirstead's motor nerve project, where he is trying to purify embryonic-derived stem cells called motor neurons -- for both spinal cord injuries and for SMA.
It was my hope that Pranav could participate in those trials.
Four years had passed, since I first spoke to Pranav: four years of constant struggle, to raise funds for the research, and to protect scientific freedom so the scientists could do their work.
At every step, we faced the same political/ideological opposition. The Religious Right and their dominance over the Republican party are huge obstacles to cure.
One of many attacks was a major national lawsuit, Sherley v. Sebelius, an attempt to stop all federal funds for embryonic stem cell research.
This lawsuit threatened any child with a disease which embryonic stem cell research might help. If the money is cut off, the research stops. If embryonic stem cell research later is determined to have the answer to a disease, what will that mean to the children who died in the meantime?
The number one source of research funding in the world is America's federal government, specifically the National Institutes of Health (NIH). If it becomes illegal for the NIH to fund embryonic stem cell research, that essentially kills our hopes for cure in that area.
A few days ago, an appeals court made a decision which allowed the funding of research to go forward, pending the outcome of the trial.
The patient advocate community was naturally overjoyed with the decision. But jubilation is premature, because the fight is not over. The court decision only blocks the ban on funding research during the trial...
Royce Lamberth, the same conservative Republican Bush-appointed judge who had the case before, still has it now. He opposes the research now as he did then, and I fully expect him to decide against us.
I hope I am wrong about him, of course, as I was about the three other conservative Republican judges on the appeals committee. I figured we had no chance.
All three were Republican appointees: one put there by Ronald Reagan, the others by the first and second President Bushes.
But two of three judges agreed that a stem cell is not an embryo, and therefore the Dickey-Wicker Amendment (which forbids destroying embryos) did not apply. Since, in their view, the case was not likely to succeed, they decided government funding of the research could continue during the trial.
But the case is very much alive. The plaintiffs will doubtless appeal all the way to the Supreme Court.
Who is suing? Although backed by powerful Religious Right organizations, only two people are technically doing the suing: adult stem cell researchers Theresa Deisher, and James Sherley.
Dr. Sherley, an African-American, once sued his college (MIT) for not renewing his contract, and he charged the college with racism. He went on a hunger strike to dramatize his position.
Dr. Deisher (whose business, Ave Maria Enterprises, was recently blessed by her local bishop, who sprinkled her office with holy water) is well-known for her opinions--in addition to a seeming hatred for embryonic stem cells, she is also against vaccination for measles and other diseases, apparently believing that vaccines cause autism, despite overwhelming evidence to the contrary.
What are their grounds for suing? Self-interest, pure and simple. They claim they will be economically damaged, if the Federal government is allowed to fund embryonic stem cell research.
Both scientists want to be paid by the government to do adult stem cell research. They feel they would be unfairly hurt if any government dollars were spent on embryonic stem cell research, because that would mean less money for them.
On those grounds -- their opposition to increased competition for grants--they are suing the American government. If they win, it will illegal for the NIH to fund embryonic stem cell research.
This is nonsense. If allowed, their argument would set precedent for any scientist to sue the government to block federal funding of any new research which competes with the old.
Can you imagine? One alternative to nuclear fission (what we have now) is nuclear fusion, which apparently can give us all the positive energy, but without the radiation.
According to the argument advanced by Sherley and Deisher, it would be illegal for the federal government to ever fund the clean kind of nuclear, because it would mean less money for researchers who liked the old dirty kind!
Four years had passed. Four years...
Although type one SMA typically kills its victims before the age of two, Pranav had survived to the age of seven, in my opinion due to the heroic efforts of his family.
The Keirstead/Geron stem cell trials for paralysis are underway.
A young man named Timothy Atchinson received the world's first embryonic stem cells in a human trial. That research, which Roman's law funded--and which was opposed at every step by the Religious Right -- was headlined in a story in the Washington Post: "Stem Cells were God's will, says first recipient of treatment".
The Act which paid for that research, the Roman Reed Spinal Cord Injury Research Act of 1999? Its funding was removed. Although the program itself was unanimously approved by both sides of the aisle, with every Republican and Democrat voting that it be continued, there was no money for it.
But what if we funded it by driving violations (car crash is a major cause of spinal cord injury) like seven other states did?
I approached Assemblyman Bob Wieckowski, Democrat of Fremont, and asked him to champion this new way to fund the law begun so many years ago by Assemblyman John Dutra.
Wieckowski just smiled, and said "Absolutely", and has been fighting for it ever since.
If AB 190 prevails, a $3 fine will be added to bad driving tickets in California, which would bring in enough money (perhaps $11 million a year) to fund a serious spinal cord injury program.
The Religious Right came out against it. Why? Of the 191 projects the RR Act funded in our ten year history, 4 involved embryonic stem cells, and that was enough to get us attacked.
Naturally,I went through my stem cell phone book, emailing everybody I knew, asking their support.
I wrote Pranav's mother Kavitha twice, asking for a letter in support of AB 191. Always in the past, when I needed a letter written to fight for research, Kavitha was willing.
This time there was no answer.
I figured, she was either exhausted from the work of tending her son -- or maybe just tired of hearing from me! The latter would be understandable. Sometimes I get sick of the sound of my own voice, endlessly exhorting people to do what they don't want to do, interfering with their busy lives, asking them to do still more chores, to write yet another letter of support.
But I had to share our wonderful news... On April 26th, AB 190 passed our first committee hearing! The opposition showed up and talked against it, of course, and the two Republicans on the committee voted no, but still we still won: 4-2, with one abstention.
Roman spent virtually his entire speech talking about children affected by spinal cord maladies, and naturally he mentioned Pranav -- I just had to tell his Mom.
Kavitha wrote back the following.
Thank you, hard day for us-- one month since Pranav passed away. However. very glad that Roman mentioned Pranav and it had an impact.
I knew Pranav was getting weaker, he wanted to spend more time on the computer than going out. He was not sitting up so much. But he was so cheerful and happy.
On March 23th he started coming down with a cold - just a minor one, we had seen this a million times, managed it at home. His numbers were ok - good O2, Heart rate was slightly high. Just did more Chest PT, lots of rest, more bipap (oxygen mask) time - nothing unusual.
Night came and he was not comfortable, asked to be turned many times. Again not unusual. Numbers were fine, so we were not concerned.
March 24th was better. Had school at home, did painting project as he was studying about DaVinci and Monet.
March 25th, he woke up in the morning and asked me to cancel school, unusual for him.... For some reason Dad wanted to stay at home, saying he would work from home that day.
I stayed with Pranav by his bedside and he told me he was tired. I told him to relax and go to sleep. His numbers were ok, so no big worry.
Suddenly at 12:03 he said, "Mom, I can't breathe". That was the last time he spoke. It happened in seconds. We did everything possible. The EMT was there in 5 minutes. He still had a pulse, so was taken to ER and flown to Children's Hospital in Philadelphia. He had gone into a coma. They did everything, but there was no response.
We had to say goodbye on March 29th. His eyes, kidneys and tissue were donated.
I cannot believe yet what happened. He still lives here with us, I can feel his presence.
No child should be taken away so mercilessly by disease... He was so looking forward to do great things in life. He made me a better person, a better mom.
I wish I had just one more chance to make everything alright...
Kavitha and her husband are shining examples of all that parents can be. They gave everything, but the medical answers were just not there.
Here is a note from parents of another child, Gwendolyn, who has SMA.
Please know that Pranav...was an inspiration... the way you all lived your lives with him and his passing only fuels our passion to end this cruel disease -- for Pranav, for Gwendolyn, for those taken much too soon, for those who continue to fight for every single breath, for those whose parents will take them home from the hospital not knowing that they have a terminal disease called SMA. This is why we fight and we will not stop until SMA is no more. ~ Bill and Victoria Strong*, parents of Gwendolyn, age 3.
Pranav lived seven years, three months, and one day. He wanted to become President, and to walk on the moon (in the year 2035, his mom said, he always had a date for his goals). He got straight A's in his home school. He had planned on making a movie starring Barack Obama. He reasoned that when he was old enough to make the movie, Obama would no longer need to be President and would have the time...
The dignified little voice is silent. But in a larger sense, for we who support research for cure, Pranav's voice must never be stilled. Our struggle must and will go on. On its outcome depends the health and happiness of millions of children, all across the world, each with a right to live a full and happy life.
The opposition will continue to delay us. Their obstruction will be measured in the cost of lives and suffering: people who might have been cured. Those who use their powers to block research will win some battles along the way. At present, the Republican Party is unfortunately dominated by the anti-research Religious Right.
The victories of the anti-research few are like the victory of that miserable Bishop Pierre Cauchon in the middle ages, he who led the legal battle against Joan of Arc. He won the case. A fifteen year old girl was burned to death, and her ashes scattered in the Seine. Yet her name shines throughout the world, a symbol of faith and courage. And Cauchon? That word has the stench of an epithet.
The patients and their families will prevail. Already, a majority of Republicans are ahead of their political leadership, in full support of embryonic stem cell research. In time, mainstream Republicans will either migrate to a party which does support cure research, or (let us hope) they will change the Grand Old Party from within.
Everyone is affected by incurable disease. Already an estimated one hundred million Americans suffer chronic illness or injury. All of these people have families. The price of unending care for the chronically ill is so huge it is hard to grasp. Already, the medical bills of the incurably sick are so great they exceed the annual cost of the national debt, In 2009, the direct cost of chronic disease was $1.65 trillion, vs. $1.6 trillion for the national deficit in that same time. This is the reason health care costs for taxpayers are increasingly unpayable.
These are not statistics, but loved ones, our family members. They are our wives and husbands, our parents, our brothers, sisters, nieces, nephews -- and our children.
Like a dignified little boy named Pranav.
*At six-months-old, Gwendolyn Strong was diagnosed with a genetic disease called spinal muscular atrophy or SMA... We started the Gwendolyn Strong Foundation, a nonprofit public charity, in 2009 to fight alongside her. In less than two years, we have raised over $800,000, and have provided targeted, strategic funding for promising research programs including: Dr. Hans Keirstead's stem cell-derived motor neuron transplantation therapy currently awaiting FDA approval to commence a Phase I safety study ...--Bill and Victoria Strong.
For more information, please visit: GwendolynStrongFoundation.org