She is a Star she is on the radio and in the newspaper, online she is a hit! She is lit.

WGN MVPP interview on the radio:

WGN Most Valuable Person on the Planet = Ivy Marcucci


Chicago Tribune article:

Chicago Tribus article about Ivy and her HSCT


Stay tuned for more info, there is more, she is not done.

Look out the window, oh how scenic, you can't go anywhere if you are Neutropenic

What is neutropenia? 
Neutropenia (noo-troe-PEE-nee-uh) is when your blood has too few neutrophils, a type of white blood cell. All white blood cells help your body fight infections, the neutrophils are important for fighting infections, especially the infections caused by bacteria.



Why would one become neutropenic?
Neutropenia, is a side effect of the chemotherapy given during critical conditioning regimen for HSCT. Thus, as opportunistic infections, including those caused by foods, can occur in the period during which patients are immunocompromised. Ivy will remain neutropenic until engraftment or bone marrow recovery. Once engraftment occurs her new immune system will start making WBCs including neutrophils.

I often take care of neutropenic patients who are febrile in the emergency department. Cancer patients who are neutropenic are instructed immediately go to the ER if they have a temperature >/= 100.4. This is because they are at increased risk of becoming septic and to be deathly ill due to the lack of immune system. These patients are treated with great concern because their condition is often tenuous and they are on the brink of death given their immunocompromised state. A neutropenic fever is no joke. 

Ivy is starting to show a slight increase in her neutrophil count and will hopefully be out of the dangers of neutropenia soon. Her neutrophil count will hit 0.5 which will mean she is no longer neutropenic and will be ok to be discharged home. Her count today in 0.1 which was positive as she has been too low to count or register a number for the past several days. Today is a good day and I predict that she will be above 0.5 tomorrow. 

A lonely neutrophil 

Stem Cells, What the &%$# are they and what do they do?

In order to explain stem cells we can start with bone marrow. Bone marrow is the soft, spongy area found in the center of some of the larger bones. The marrow makes all of the different cells that make up the blood, red blood cells, white blood cells (of many different types), and platelets. 

For Ivy's stem cell transplant we are dealing with the cells of the immune system. The cells of the immune system are also produced in the bone marrow. These cells develop from a specific type of cell found in the bone marrow, "hematopoietic stem cell."

The body signals the hematopoietic stem cells to create or differentiate into the different blood components needed at any given moment. This process is very active / busy, the bone marrow produces millions of different cells every hour. The stem cells stay in the marrow until they differentiate into mature blood cells, at which point they are released into the blood stream where they perform their specific functions such as carrying oxygen, providing protection from infection, and helping blood clot. There are small numbers of stem cells, however, that can be found in the circulating blood, this allows them to be collected under certain circumstances. Often medication as in Ivy's case she was given daily Neupogen injections the week before they harvested to increase the number of hematopoietic stem cells in the blood prior to collection. 

These stem cells that will go on to differentiate into mature adult blood cells of the immune system are what they harvested from Ivy. After the stem cells are infused into the patient’s bloodstream, they find their way to the bone marrow and begin the process of forming new, healthy blood cells including white blood cells, red blood cells and platelets. This new immune system will not remember to attack Ivy's myelin as they will not recall that the previous cells had mistakenly been programmed by Ivy's immune system to attack her myelin. The old immune system was wiped out by chemo and no longer exists so these new cells differentiate  into a brand-new immune system which also explains why Ivy will need to get revaccinated as her new immune system will no longer carry the memory of her previous vaccinations and she will no longer be immune to many of the diseases she was previously immune to by her past vaccinations she received as a child. The good news is that her immune system will no longer remember that it used to mistakenly attack her myelin as it thought it was a foreign substance that needed to be killed and removed from the body. 

Ivy is forming a brand new immune system that has forgotten that it was programmed to attack myelin and this is how the stem cell transplant stops MS from progressing allowing the body to repair the damage already done by MS and going forward her new immune system will ignore myelin and not try to destroy it anymore.

The actual treatment responsible for stopping MS the chemo which killed off the immune system and the stem cells that get transplanted will just help her new immune system grow quicker and help her recover in a more rapid fashion, without the chemo the stem cells would just differentiate into the same broken immune system and still attacks the myelin. Now that the broken immune system has been destroyed the stem cells will differentiate into an immune system that is no longer broken it will be like the immune system that was there before the MS developed effectively making MS just a bad memory that has been erased. 

My Stem cell poem 

Stem Cells, New Stem Cells,
So much potential have you.
reproduce, renew.

7 new Lesions are not very pleasin

Ivy had a new MRI recently of her Brain and cervical spine. We were wondering if she has had any relapses since her the MRI's she had when she was diagnosed. Since being diagnosed she had been feeling pretty good but has had some numbness that would wax a wane in different areas of her body but these symptoms would resolve and were not very severe at all. She had some facial numbness and some skin sensitivity to her chest was on her right side. She has not had any significant deficits or symptoms that were severe in nature like she had when she was diagnosed and she was seeing double due to the attack on the myelin of the  6th cranial nerve.

The good news is that she will has already received aggressive treatment to stop this flair in its path of destruction before it could do more damage and then her chemo and  stem cell transplant will facilitate termination of her MS and then allow her body to heal and repair the damage her body has already sustained from the MS attacks she has had up to this point. The goal is that she stops MS in its tracks and prevents it from coming back and then heals the damage that has been done and ultimately makes MS a distant bad memory never to return.


That is the update.

Today Ivy is have her stem cells harvested. I will write another post to explain the process soon.

Harvesting stem cells 

Ivy Launches her Blog

You should check out Ivy's blog about her journey and battle with multiple sclerosis. She is an amazing writer and her posts draw you in and leave you wanting and waiting for the next post. Follow the link and enjoy, learn, laugh, cry and live.


https://www.itsamess.me

The recent HSCT trial explained

I found an article that explains the HSCT treatment trial that was recently published in JAMA. Follow the link below for a great article discussing the treatment and trial findings that were done by the same team / doctors that are now Ivy's doctors. She is in good hands.



The treatment and recent study explained

Control-Alt-Delete a better approach to treating MS

HSCT is a procedure that aims to reset the faulty immune system. Haematopoietic stem cells are taken from your bone marrow or blood before your immune system is taken out with chemotherapy. The cells are then reintroduced into your blood, where they grow into a new immune system that will hopefully have forgotten the ways of the diseased immune system prior to being wiped out and no longer attack your  nervous system. 

While working as a Fellow at Johns Hopkins a doctor noticed that the patients with leukemia he was helping treat needed get their childhood vaccines again because the protection from diseases like the measles and mumps was being lost due to the treatments for their leukemia.  The cells impacted by transfusion treatments seem to lose their memory of the original childhood vaccinations. It was like they forgot. 

The doctor thought maybe we could get the diseased cells in patients with autoimmune disease to lose their memories they would forget to attack the bodies in people with these autoimmune diseases. This reprogramming would require adult stem cells. At first he had to test the concept in animals and quickly received FDA approval to test the adult stem cell therapy on people with Multiple Sclerosis. 

Although this stem cell therapy has not been approved for standard treatment for people with MS it continues to be studied in clinical trials at multiple centers around the world. The hope and the likelihood is that this therapy will become approved as a standard treatment for MS. In the meantime ongoing clinical trials continue and the results have been miraculous. The average person with MS can't get this therapy today as it is not approved and the only way to get this therapy in USA is to get included in a clinical trial and receive the treatment as a participant in a trial. 

There are a lot of people who would love to get the treatment but there are only a small number of spots in the ongoing trials. These trials have strict inclusion criteria and strict exclusion criteria thus making it likely that most will get rejected for inclusion in the trial and the 200 spots quickly disappear. Soon though these restrictions may be lifted and once approved stem cell therapy known as hematopoietic stem cell transplantation (HSCT) may become the standard of care or at least an optional therapy for individuals with MS meeting certain criteria that would make them a good candidate for the treatment. 

The past and current clinical trials done with HSCT have produced results that would make many call this therapy a cure, however the researchers / doctors refrain from calling it a cure at this point because more longterm data is needed. There have been many who have received HSCT and no longer have active disease and no longer require any medication and no longer have symptoms of the disease and have even had their symptoms reversed and eradicated. Some of these patients are not as careful with the word cure and they in fact joyfully consider themselves free of MS and cured. Many of these patients have been MS free for 5+ years after receiving HSCT. There are some who received HSCT 10 - 15 years ago in the early days of research who are still disease free. 

There are stories that are common for those who have been lucky enough to get HSCT.  One such example is a patient recently shared with journalists that he was diagnosed with Multiple Sclerosis, which is considered a type of autoimmune disease that attacks the brain and spinal cord. Before his adult stem cell treatment, could barely walk.  Today he is a healthy, active husband and father who coaches hockey and lives life to the fullest and has had no signs of active MS on all of his followup appointments since receiving HSCT. 

Here is a link to the recent clinical trial published in JAMA on January 15, 2019. https://jamanetwork.com/journals/jama/article-abstract/2720728?resultClick=1

Cure, when it comes to MS it is just an alternative band that was big in 1980's

Multiple Sclerosis is a diagnosis that brings a lot of questions and the answers are not that great. There is no cure for this disease. The cure has been dodging researchers and doctors for decades but there have been some great leaps and bounds with most of the progress being made in recent years. In recent years several medications have been developed and have had significant impact of MS and the course of the disease for those who have it.

Relapsing and Remitting multiple sclerosis (RRMS) which is the most common form of MS has to be treated in several ways, generally the the person gets longterm ongoing treatments, acute treatment for the relapses and finally treatment for various symptoms of the disease. RRMS has flairs or relapses where the patient experiences an "attack" which is an acute episode of symptoms resulting from the MS attacking the patients myelin. After the relapse the patient goes into remission and the body will heal over time initially the healing can be complete meaning the neurological symptoms resolve as the body recovers however over time and sometimes even in the beginning of the disease the damage caused by the flair does not heal completely and can become a permanant deficit. These deficits lead to problems for the MS patient like paralysis or weakness, visual symptoms, and a whole list of other neurologic problems that plague the individual. Patients need acute treatment when a relapse occurs tho stop the flair and limit the damage and then in-between relapses there are treatments to delay and hopefully prevent relapses from occurring.

Relapses
The relapses or flairs are generally treated with high dose steroids which have powerful anti-inflammatory properties. The goal is to stop the acute flairs or active disease that are actively attacking the myelin. This is an important time and treatment is needed to prevent and or slow the attack which will hopefully stop and prevent permanent damage. The steroids are usually given at ~20 x's the typical steroid dose and are given intravenously over 3 - 5 days and then the patient is put on a steroid taper and takes steroids in pill form and slowly taper the dose over 2 - 6 weeks depending on different variables. Time is of the essence during the relapse period as the disease is actively assaulting the nervous system.

Remission
These medications used to treat ongoing MS are called Disease Modifying Therapies (DMT's). DMT's are used to manage MS and the patient usually takes these the rest of their life and often have to switch between different DMT's as they stop working or just fail over time or cause complications and side effects that are intolerable and some can even lead to a fatal complication called  progressive multifocal leukoencephalopathy (PML). PML is an opportunistic viral infection of the brain that usually leads to death or severe disability. Some of the DMT's increase the chances of developing PML. Another issue is that these medications cost abut $6,000.00 / month and even with insurance still can run thousands of dollars / month. The cost of ongoing MS medications ~$70,000.00 / year for life and this does not account for acute medicines or symptom related medications, doctor's appointments, MRI's, ER visits, physical therapy or other medical costs. 

Misc Symptoms
There are many medications used to treat the symptoms of different MS related symptoms. There are pain medications, medications for bladder symptoms, medications for muscle spasms and whatever else the patient may develop. These medications are taken as needed or sometimes may be required for life depending on the symptom / problem being treated.

There are other approaches for dealing with MS that include dietary changes and exercise programs and stress relieving activities  that have also had varying degrees of success  in some patients. There are several dietary plans that have been touted as cures and have anecdotal stories of individuals finding varying degrees of success. Certainly we know that diet and exercise and lifestyle modifications can play an important role in managing MS and better controlling the course of the disease.
There are off-label unapproved treatments that are available but I will not go into the various treatments in this post but maybe in a future post.

There are also some treatments that are not FDA approved and are currently in clinical trials and evaluations to determine their effectiveness and safety. One of the most promising therapies is called HSCT (Hematopoietic Stem Cell Transplantation). I will discuss this in more detail in a future post. 

The funny bone isn't very funny. Numbness and tingling and other vague symptoms.

There were times when she had numbness and tingling in her arms and hands and sometimes in her legs. She would tell me about these sensations and said that it kind of felt like when you hit your "funny bone" but they would go away after a few days or sometimes a couple of weeks. She had a heavy backpack and carried it around all day on one shoulder and we thought maybe this was causing some of her numbness and she made some changes and the numbness went away, problem solved.

There were 3 or 4 different times over the last 1.5 years where she had these symptoms that were mild, would not last very long and always went away without any medical intervention. This past fall I took her out to college so we could find a car for her,  she told me that her hands were feeling numb again. I said well maybe we will get an MRI as we got her settled into school the sensations vanished but my threshold for getting an MRI was pretty minimal at this point because I feared that she may have some type of pathology that would explain the symptoms. She started school and life was busy and there were no numbness and no tingling. Soon these were just like a forgotten headache or cold and not a part of her life.

She returned home for Christmas this past December, 2018 and we were excited to have her back in the house. She brings an excitement and an energy that we had missed while she had been away at school. She was glad to be on a break from school and have the stress of school fade away for a couple of weeks. Not long after getting home she announced that her hands started to feel numb again and it seemed to start all of a sudden. She woke up one morning and felt some tingling and numbness in her hands. I watched her closely and started to think about having her come to work with me and get an MRI. After a few days I noticed her squinting and trying to focus her eyes while we were watching a movie. I looked a little more closely and after the movie I examined her and noted that she had a cranial nerve VI palsy which meant her eyes were not equally yoked together when she looked to the right. She told me that she started having double vision when she looked in certain directions and this started earlier that day.

This made me realize that my fears of something more ominous was causing these symptoms. Did she have a brain tumor or a neurologic condition like multiple sclerosis? I told her she would have to come to work with me so we could get an MRI and hopefully rule out any serious pathology. I remained positive but my experience and training told me this was not going to be a normal MRI....



cranial nerve VI palsy (abducens nerve):