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Palliative Care - a personal story

In the five months between jacob's diagnosis and his death, i found myself searching for as many "sma baby stories" as i could find. somehow, i felt that it could prepare me for what had become my new life. as i embark upon the task of writing jacob's story, i have many mixed emotions. i want to record the information to help other moms the same way i desperately needed to read about other children. although his life story does have a beginning and tragically, an end- it is our commitment that his legacy will be unending. now, jacob lives through us, through his big sister jordan, his little brother max and through the many, many lives he touched. however, as you read this, know that i cannot possibly do jacob's life justice with words. there is so much in between the lines. so although this is a recording of sorts, please know that it is incomplete. although jacob was taken from us FAR too soon, know that he left us with many gifts. although he was dying, know that he lived each day to the fullest and he was showered with a lifetime of love. although the story has an end- please know that this is not the end.

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On december 27, 2001 our beautiful son jacob isaac was born. i loved him instantly. he was perfect. he was great at nursing, was very alert and had the sweetest cry. we went home and all was well. jacob got his first cold at two weeks. i was sick with worry. i brought him to our pediatrician three times in a single week to listen to his chest, but it was just a cold– jacob was "eating like a pig" and "growing beautifully." after a month, the cold went away only to be followed by a few more colds. in short, it seemed as though jacob always had a cold, but we thought nothing of it, maybe he was going to be an allergic kid.

Well, at around 6 weeks my grandma started mentioning that jacob wasn't holding up his head yet. i sort of dismissed her worries, and my pediatrician assured me that lots of babies don't hold up their heads at 2 months. his muscle strength was in normal ranges at his 2 month appointment, but as the weeks went on our concern grew as jacob still didn't seem to support himself as well or move nearly as much as other babies his age. he really only moved his legs in the bath and although he would bat at toys, it seemed as though his limbs were too heavy for him. so... at three and a half months i took him back to the pediatrician specifically for his lack of movement and our doctor said he shared my concern. he ordered a battery of tests- my poor little guy had to go through about half a dozen needle sticks (trying to get blood), sedation, and an mri that day. everything was coming back normal. our relief was short lived.

The next day we met with a pediatric neurologist who examined jacob and then sent us back to the hospital for more bloodwork. i read off the tests he ordered- prader-willy, muscular dystrophy, and dna for sma (these were just a few that i can remember). i forced myself not to get on the internet until we had a diagnosis. it took 2 weeks to get the results. those two weeks were excruciating. we prayed that jacob just had low muscle tone and would perhaps be late reaching physical milestones and need physical therapy. we took him to three physical therapists in one week. we immediately started him with both physical therapy and occupational therapy. we put him on a medication in case the problem was metabolic. in my heart, i felt that something was terribly wrong, but didn't want to believe it. of course, everything would somehow turn out okay, right? i mean, this stuff just DOESN'T happen to people we know- and certainly not to us! we were called back into the neurologists office, monday, may 6th and told that jacob had spinal muscular atrophy and that he probably had about a year to live. WHAT????????????????????? we were shocked. numb. devastated. disbelieving. how could this be happening to us?

We went home and read everything we could. i found that jacob was a textbook sma baby. everything that i read could have been written specifically about our jacob. we told ourselves that certainly there was something that could be done- of course, somewhere, someone HAD to be working on some experimental treatment that could save our son and wake us up from this nightmare. we were determined to unturn every stone until we found him. well, we did find a wonderful doctor and friend, dr. thomas crawford, at johns hopkins medical center. and he (along with many other doctors) is working on a treatment and a cure. but with sincerity and compassion he regretfully told us that there was no cure that would come in time to save jacob. our reality was becoming worse and worse.

In the weeks that followed, we continued to learn about sma. we travelled to baltimore to meet with dr. crawford, who proved to be an invaluable consultant on jacob's medical team, which in addition to dr crawford (a pediatric neurologist who specializes in sma), consisted of two pediatricians (our regular one and also a close friend who lived in our neighborhood and was able to make house calls), a surgeon, a pulmonologist, a gastroenterologist (we only had one appointment with him), physical and occupational therapists (which we stopped going to after about a month), and countless nurses. dr crawford told us that jacob was an average type one.

We also travelled to chicago to the families of sma national conference. we spoke to many sma families who graciously shared their stories with us. we met type one children whose parents had described them as typical two and three year olds. meeting the children allowed us to have a glimpse of what jacob’s life might be like. i felt that these type one children were anything but “typical.” that isn’t to say that i didn’t respect them, or feel that they deserved every chance in life, or that they were in any way inadequate people. in fact, i found them to be extraordinary. heroic. brave. exquisite. but it was clear that their lives were vastly different from the life of a “typical” child. i remember right after jacob was diagnosed, i took him to physical therapy and i saw a man in a wheelchair. he was talking to another man and he was laughing. i thought to myself, a month ago i would have pitied this person– and now i think how lucky he is. he is able to interact with others, have relationships and have a full life. i could make a list a mile long of the things this man was able to do. had jacob lived, he would not have been even a fraction as lucky as this man was. jacob’s list just would have been far too short.

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As a four month old, by appearances, it was impossible to tell that jacob had a terminal disease. he simply was a beautiful boy, alert and quick to smile. as the weeks went on it became clear that we would need to make some decisions about how we were going to manage jacob’s care.

After much research, many tears and great heartache we chose palliative care to manage jacob’s sma. this did not mean that i was choosing to “let my child die” or “do nothing.” i did EVERYTHING that i felt our son needed and it was sma that killed him- not palliative care. we did not make our choice out of selfishness, laziness, or because of any one doctor’s advice. we knew that we had options (such as the non-invasive protocol or a tracheotomy) but we also knew we could not save jacob from sma. had someone told me that there was something that could CURE jacob, i would have walked to the moon and back 10 times over to get it. there is no such thing. no matter what we did, jacob would always have sma. instead, we decided to cherish every minute and live each day to the fullest, for however long he wanted to be here.

I always looked at the choice between the non invasive protocol (niv) and palliative care as a choice between terrible and horrible. by choosing palliative care, we knew that jacob would not live long enough to do most of the things parents envision doing with their children. yet, we felt that niv or traching him would not give him “enough” of a life either. we asked ourselves if living with type one would be “enough” for us, had we been the ones diagnosed. for us, it was not. i felt that sma would rob him of a life with even the most rudimentary activities. it seemed as though jacob would have been hanging onto life, rather than really living. we felt that with palliative care, we could give jacob a full life without prolonging it.

Our first big decision came in early june. we decided that jacob would have surgery to put in a gastrostomy tube, which would allow him to receive nutrients directly into his stomach. because we knew that jacob would eventually lose the ability to suck and swallow altogether, we viewed this as a necessity. we felt that jacob would be able to spend a few more quality months with us by placing the tube- as he would be able to receive the calories he needed without having to use all his energy to do so. with dr. crawford's guidance, we decided to only put in the tube, without doing a nissen fundoplication, which would have been much more involved. the surgery was much more of an ordeal than expected. to begin with, we wanted to avoid general anesthesia. we were nervous that if jacob were to be intubated (as is the case with general anesthesia) he would have trouble being extubated and require a machine to help him breathe indefinitely. we knew that we did not want this for our son. our surgeon was willing to do the surgery under a local anesthetic. jacob was pretty miserable after the surgery and we spent three nights in the pediatric intensive care unit. being in the hospital was horrible. although we knew the best ways to treat jacob, we had to argue with the nurses about the simplest things. after three long days and nights, we finally brought our little guy home. he was so happy to be home and jacob was SO much more content in general, because he didn't have to struggle to eat. it turned out that we did the surgery at just the right time, because jacob slowly began losing the ability to swallow. about three weeks after the surgery (jacob was almost 6 months) he was exclusively tube-fed.

We celebrated jacob's 6 month birthday with a family party. after this month, we made it our family tradition to have a cake and sing to jacob on the 27th of each month. we constantly searched for light toys for him, sang to him, walked him around in his stroller, bought him balloons (which to his delight, he discovered he could tug on the strings and make the balloons move up and down), and took him outside. we gave him 2-3 nebulizer treatments a day, along with chest physiotherapy to help him keep his lungs clear. occasionally he needed suctioning (to clear his airways), but this was infrequent at this point. he was able to move his arms at the elbows, his head from side to side, and he spent a lot of time in his bouncy seat. eventually, he had to lay flat, as this was the best position for him to manage his secretions.

A few days after jacob's 6 month birthday (july 4th weekend), he contracted pneumonia. i remember that he looked awful, so unhappy! we were unsure if he would recover. we signed up with hospice and began giving him daily intramuscular injections of antibiotics. oxygen machines and a pulse-oximeter machine were delivered, and jacob used both. the oxygen was given through two tiny prongs that went about 1/3 inch into his nose, and the pulse-oximeter measured his heartrate and oxygen saturation level by a little probe which was taped around his big toe (this looks like a tiny bandaid). after a couple of days, jacob began to come around and smile more and act more like himself. we were so glad that jacob felt better. even after he recovered, we continued to give him oxygen and use the pulse-ox at night.

Over the next couple of months, jacob had good days and bad days- with far many more good days than bad. we celebrated his 7 and 8 month birthdays, played, sang, shot lots of video and took pictures and discovered new things about our little star everyday. jacob did get weaker in this time, and began waking up every hour throughout the night, sometimes to be turned (as he was unable to turn himself), sometimes to be suctioned, and sometimes probably just to say hi. around 7 months, we faced our decision regarding the bipap. we did try the bipap- jacob hated it and i hated seeing him wear it. he couldn’t suck his thumb. these, however, were not the reasons we chose not to use it. my older (sma-free) daughter is made to do many things she doesn’t like to do- if it is in her best interest. the reason that we decided not to use the bipap was because our philosophy was that once our child needed a machine to help him breathe- his quality of life would be compromised. we knew that the bipap would make jacob more comfortable in the short term, but we were afraid that it would keep him hanging on to what we felt was quantity of life- not quality of life. although i know that the bipap is initially used for sleeping at night and during naps, we found (by talking to other parents) that many children become more and more dependent on it and use it for more hours during the day than they are able to go without it. at that point, what is really the difference of being on a bipap 24 hours a day or being dependent on a ventilator? we had to look at what jacob’s life would be heading towards. we decided that jacob would not use the bipap.

The day after jacob's 8 month birthday, i walked out of the room for 90 seconds to hang a picture, and when i came back jacob appeared to be sleeping in his stroller. as i walked closer to him i could tell he was not breathing. i grabbed him out of the stroller and started screaming. i remember thinking– i knew that this moment would come, but i didn't know it would hurt so much! i wasn't ready! jacob wasn't ready! i remember thinking, there is NO way that adi is not going to see his son alive again. i started doing cpr. we increased his oxygen. my grandma called adi, who flew out of his office and raced home. after what seemed like an eternity (but was really only a couple of minutes) jacob finally started breathing again. that night he was miserable and we were unsure if he would make it. yet, he did. a few days later, jacob began acting like himself again. he lived another five weeks.

During those last five weeks, jacob's health declined. his breathing was more labored, and his "episodes" of turning blue and his pulse-ox alarm beeping (to indicate that his oxygen level had dropped) both became more frequent. he needed more suctioning and seemed to be weaker in general. he was only comfortable laying flat and we couldn't even really hold him, because this would cause him to choke on his secretions. i only left the house a few times during this period, and only if adi was home.

Through all of this, there wasn't a day that we didn't see jacob smile. we discovered a new way to bathe him on a small stack of towels, and float him around in the tub (which he loved). we were constantly finding new toys he could hold, books he liked to read and songs we could make up for him. i look at how many lives jacob touched in his last five weeks. we started an online journal about his life. a local tv station came out to do a story on him which aired september 20th. a south florida newspaper also interviewed us for a story . looking back, i think that jacob was spreading his message, and laying the foundation that would become his legacy.

I struggled with many decisions along the way- should we use oxygen? should we keep him on the pulse-ox? if he were to die at night, in his sleep– wouldn’t that be the way we would most want it? would the pulse-ox notify us that he was in pulmonary arrest, and thus keep him from dying in his sleep? if we didn’t use the pulse-ox, would i even be able to sleep at all- or would i live in even MORE fear than i already did that i would go to him in the middle of the night and he would have died? did we want the chance to save him? to be there when he died? would we go to the hospital? would we suction at the end? what was too much intervention? what was too little? would we know when he was “ready?” how would we know?

We decided to use oxygen. we decided to use the pulse ox– i felt that i needed to be there when jacob died. we decided we would not call 911 or go to the hospital. we gave him antibiotics and nebulizer treatments and cpt. we decided we would not preform cpr a second time. we said what we thought was “goodbye” to jacob at least twice before he died. yet, each of those times our boy fought back. and in the end- we knew that he was ready. he had fought. and fought and fought. he was tired. and nothing in our usual repertoire of treatments was helping him any longer.

The night that jacob died, adi and i took turns sitting up with him. we held his hand, sang him songs (especially his favorite, "somewhere over the rainbow") and adi later confessed to me that they watched baby bach together. we both sat with him during his last hour and i rocked him. i took in his smell, the softness of his skin, his every perfect feature. i knew that i didn't need to commit him to memory- for he is unforgettable, but i knew it was the last time that i'd be able to physically hold my son. we pledged to love him forever. he took his last breath in my arms.

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Immediately after jacob's diagnosis, i found myself focusing on jacob's death. at some point in the next few days or weeks (which are somewhat of a blur), i made a conscious decision that i wanted to focus on jacob's life, regardless of its length. while his prognosis was looming around us, i began to view our time with jacob, and jacob's life as a gift. we appreciated each day, each hour, each precious story we read him, each smile he gave us, every kiss, smell, and squeeze. i remember sometimes laying in my bed at night, thinking about wanting to go look at jacob sleeping, maybe gently kiss him or touch his head. i'd be so tired, that i didn't want to pull myself from my bed, but i did each time. i remember asking myself- if i don't go peek will i regret it? i am so glad i can look back on this simple act, and on so many other moments in jacob's life (some big, some small) without regrets. i knew that i would have a lifetime to mourn my son, and i vowed not to miss out on the time we DID have with him. i wish with all my heart that i could go peek in on him tonight.

Our friends and families supported us every step of the way. although we had great pain, we were surrounded by love. as a result, we were able to give jacob a full life in which he only knew love. we learned about the compassion of people. our love for each other grew stronger. i am so thankful that adi and i were like-minded on every heart-wrenching decision.

I don't know how we get through each day without jacob. i can't believe my boy is gone. it feels so unreal, but at the same time- so painfully real. i am proud of the way we faced our greatest challenges, and i would make the same choices with jacob all over again. our journey is filled with the deepest pains and the greatest joys. although i miss jacob terribly, i can honestly say that i have no regrets. i can look back on our time with jacob and find comfort in the knowledge that each choice we made was out of love for our son. even though it has been twenty months since i’ve held him in my arms i still think of him as often as i do my two living children, and his image is as clear to me as if i saw him yesterday.

I am a better person for being jacob's mommy, and i know that he will always be in my heart. even though we have a lifetime of grief, i wouldn't trade the nine months and four days that i had with jacob (on earth) for anything. he is my hero. the most courageous person i have ever met. he taught me more than i ever dreamed there was to know about life and love. we will mourn "our shooting star" for the rest of our lives. we will love him forever.

the jacob isaac rappoport foundation
fighting spinal muscular atrophy
in memory of our shooting star
www.ourshootingstar.com


 

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