Crossing 100K!!

We had another clinic visit today and Ibrahim's platelet count has reached 120,000. So now we will start reducing his steroids slowly and watch what his counts do. He still has to be cautious about being around other kids and particularly sick people since he has the central line in his neck. So he's missing out on his cousin's birthday party today. But inshallah he'll participate in many others in the future.

Again, all of our work up has remained normal, including the testing for penicillin antibodies and wiscott-aldrich. We'll check labs again in another week.

By popular demand.... a recent picture

Friday clinic visit

Mashallah 89,000 platelets today! We're really pleased with baby's progress. Still no clear etiology is identified as one by one our test results are coming back as normal. We'll check a platelet count again in one week and if Ibrahim's platelets are within normal limits (150-300,000) we'll start tapering his steroids.

Thank you for all your support, love and prayers.

Outpatient clinic visit

Platelets are 27,000 today which is great. Ibrahim's blood smear also looks great with a lot more platelets then we've ever seen. We're heading in the right direction... now if he'd only sleep through the night!

Our next platelet check is on Friday. Meanwhile, we continue our steroids and dressing changes etc at home. 

There's no place like home

Sunday 9:30 a.m.

Platelets 16,000. Yay!

We got the okay for discharge with plans to follow up on Tuesday in clinic for blood work and a platelet count (Monday is Martin Luther King Day ie a holiday). We go home with Ibrahim's central line in place and a bit of a low white count (which often becomes low when other disease processes are ongoing). So we've been told to be really careful about infection precautions and avoiding sick contacts. But it's all good. We're happy to be going home and Hamza will be thrilled to have his parents back (although his late night parties, daily presents and sleeping in mama's bed are now over!) 

Saturday 4 am

Platelets at 12,000 this morning. Again, this is with out a transfusion. We'll likely plan for discharge some time tomorrow. It will be so nice to be back home!

Friday 9:30 p.m.

Platelets holding strong at 9,000. We have now been without transfusion since Monday afternoon. After talking with our new attending physician, Dr. Rice, we will plan for discharge on Sunday inshaAllah (God willing). We will be sent home with the tunneled IV line, and have been thoroughly instructed on how to care for it. As long as baby's platelets don't fall further, all the remaining testing etc. can be done through the outpatient clinic. 

Friday 8 a.m.

Platelets 9,000 this morning. Waiting for doctors to round.

Thursday 2 pm

Platelet count is 12,000. The stability of Ibrahim's platelet count is a really good sign. We're planning to switch Ibrahim's steroids to an oral dose now and possible discharge some time in the near future if things continue to improve. 

We had a meeting with our hematologists and the head of the blood bank today. We reviewed all the testing that has already been performed and discussed the need for additional testing. Ibrahim's disease may never be clearly identified but our hope is that it will continue to resolve.

Note to Friends

Several of you guys have sent messages asking if it's okay to visit, so here's the deal. You are all welcome to visit us at anytime, there is no restriction as far as visiting hours, etc. Just call/text one of us to make sure we are around before you come. Enter St Jude from the Lauderdale St entrance (off of Jackson) and tell them you are visiting Ibrahim Farooqui, room 2013. Park where they tell you to, and then (after washing your hands at the door) come on in! 

Thursday 8 a.m.

So last night the platelets stayed at 8,000. This morning they rose to 11,000 WITHOUT transfusing! By the grace of God, this is the first true sign that Ibrahim is beginning to improve. Thank you everyone for all the calls, emails, texts, IM's, and most importantly, prayers. Please keep them coming, there is still a long road ahead. 

Wednesday 7 p.m.

Platelets from 4 p.m. were 8,000. We will check again at 10 p.m., and if they are still decreasing, Ibrahim will get a transfusion tonight. They sent genetic testing for Wiscott-Aldrich today, and will send the test for Penicillin -induced antibodies tomorrow. 

Saira's blood work came back today and confirmed she does not have anti platelet antibodies in her blood. We are not sure how this helps in Ibrahim's diagnosis, because it is obvious that he does have some sort of antibody that reacts to platelets. Not only is his own platelet count low, but his count does not rise appropriately after transfusions. We will try to call Wisconsin tomorrow to follow up on tests that his blood was sent for.

Wednesday 8 a.m.

Platelets this morning are 11,000. This is very encouraging since they have only dropped 1,000 in the past 12 hours, and no transfusion has been required in over 36 hours. We are waiting for Dr Mirro to round this morning. 

Tuesday 4 p.m.

Platelets are 12,000. This is progress considering we have not required platelets now in 24 hours, and the count has only dropped 5,000 in the past 12 hours. Inshallah this will get better soon. 

In the meantime, the immunologist talked to us about testing for another rare disease called Wiscott-Aldrich sydrome (http://en.wikipedia.org/wiki/Wiskott-Aldrich_syndrome). We are not convinced that this is what Ibrahim has, but will probably go along with the test anyway. 

We told our doctors today that Saira received a dose of Penicillin while she was in labor with Ibrahim and asked if this could be an unusual reaction to penicillin. This has been reported in the literature, but quite rare. When we first came to the ER, Ibrahim received 2 days of an antibiotic very similar to Penicillin. If this is indeed a reaction to that antibiotic, it might explain why there has been such a delay in improvement. Tomorrow they will send out a test to check this theory.

There will be no further lab result until tomorrow 4 a.m.

Tuesday

17,000 platelets at 4am.

9 pm

Platelets are 21,ooo. Our next blood draw is at 4am.

Monday 6pm

Our new central line is finally in, hopefully to stay this time. The catheter was tunnelled as his platelet count was slightly better today and he additionally was transfused with platelets twice before the line was inserted. We'll check another platelet count now that baby is tucked in. He was intubated (breathing tube placed down his throat) for this and so heavily sedated and is still not entirely awake yet. The plan is to work on feeding this evening since he has not had any milk since 6.30 am. 

Dr Mirro and our prior hematology attending, Dr Hankins are working on the long distance consultations that we had planned. We'll see if anyone has any other ideas. Meanwhile, Dr Mirro and I looked at the bone marrow biopsy sections today and they show sheets of megakaryocytes, which are platelet precursors. Simply put, that means that Ibrahim's body is responding to the peripheral platelet destruction and trying to produce large amounts of platelets on its own. This confirms the immune nature of his disease but unfortunatly thats all we still now at this point. 

Monday 9:30 a.m.

Unfortunately the new central line has come out as well, so Ibrahim is back to NPO for another line placement at noon. If the doctor agrees to it, we will try to have the new line tunneled under the skin to decrease the chances of it coming out again. 

4 am results

33,000 is the latest platelet count! This is about four hours after his last platelet transfusion, so that's promising. We'll check another count late this afternoon. Please pray that he continues to hold his own. 

We'll be transferred out of the ICU today. Ibrahim had to be monitored for twenty four hours after he was sedated (for line change out).  I'm looking forward to not having to spend another night on a chair!

9pm lab draw

Platelets dropped to 11,000 with a slight decrease in hemoglobin as well (no evident source of blood loss, including no bleeding during the line exchange). So baby gets platelets and red cell transfusions tonite. We'll check labs again in the morning. 

Sunday 2 p.m.

Baby had his central IV line changed, but is still asleep from the anesthesia. He has received one more platelet transfusion since morning, but they will not check the level until later this evening. In the meantime, we will probably be transferred out of the ICU and receive another dose of immunoglobulin (IVIG) to continue to help remove antibodies in the blood. 

Dr. Mirro came by today and said he wants to stay the course until tomorrow. Then, he plans to telephone consult Dr Victor Blanchette from Toronto's Hospital for Sick Children (Director of Hematology/Oncology) as well as the director of the Blood Center of Wisconsin, where samples of our blood was sent for advanced platelet testing. He wants to know if they have any more insight into Ibrahim's condition. 

We pray this ailment is removed from Ibrahim's blood just as quickly and unknowingly as it began, even if we never achieve a diagnosis. 

28,000 is our 4am platlet count this morning... this is the highest we've seen it all week! Maybe the combination of IVIG and the blood exchange is finally kicking in.. or else maybe this is just another transient post-transfusion bump. We'll just have to wait and see. I've requested a repeat platelet transfusion before his central line is changed out (which is scheduled for 11am) to minimize his risk of bleeding.

Meanwhile, baby Ibrahim thankfully continues to feel well. He will not be allowed to feed after 7am as they will sedate him for his procedure. So we've had a long night of bonding while trying to fill his belly to its maximum capacity so that the rest of the morning isnt so uncomfortable for him. 

We'll check another platelet count later in the afternoon and continue to watch those numbers. As always, we'll keep you posted. 

More labs

Ibrahim's 9 pm blood draw had a platelet count of 10,000 (post-transfusion) requiring yet another platelet transfusion. We'll repeat one at 4am again and transfuse accordingly. At 11 am he'll go back to interventional radiology for a  central line change out as his current one became faulty after the exchange yesterday. 

The plan for the weekend is essentially to see what his platelets do. On Monday, we'll have devise a new strategy. 

Lab Update

12 p.m. platelets are 9,000. Plan for platelet transfusion followed by IVIG. No one sure why they are still dropping. 

ICU Update

Platelets are now are 23,000 after the transfusion. The test now is to see if he can maintain this count and continue to rise on his own (Inshallah). We will have another platelet count done this afternoon to follow his trend. 

Dr. Mirro came in to see Ibrahim this morning and is planning to redose immunoglobulin today (IVIG) to remove any still-circulating antibodies. 

Please continue to pray and thank you again for all the love, concern, calls, emails and texts. 

Update

The platelet count from 3 a.m. was 7,000.  They transfused more platelets overnight and we are waiting for this morning's count to return. We are not sure if the low count indicates the blood exchange was not successful, it might just be too early to tell. After the ICU team rounds and talks with us we will have a better idea of what this means. 

The end of today

So today has been a long one for Ibrahim. Starting with his morning bone marrow biopsy in the operating room (since he  needed heavy sedation for it), his central line falling out requiring interventional radiology to replace it and then the whole blood exchange that was performed manually over two and a half hours (after some recalculations by the blood bank etc., the exchange did not actually need to be performed over 7 hours, thankfully). Thank God, all went well and baby is now resting peacefully. He was transfused with a little bit of extra blood during the procedure as he had some bleeding through the events of the day and had a hemoglobin of 6.6 at the beginning of the exchange. He was not able to feed most of the day so has fed like a glutton this evening. We are being monitored overnight in the ICU and will move back to our regular room in the morning. We'll just have to wait and see what his blood counts look like from here on. The hope is that the blood exchange will clear him of the antibody that is destroying his platelets. 

ICU

The central line has now been replaced and we are transferred to the ICU. Once baby feeds, the whole blood exchange will begin. This is a long process and is scheduled to take place over 7 hours, so will likely finish past midnight. One person will slowly draw Ibrahim's blood out a few cc's at a time while a second person will slowly infuse donor blood by hand. Once Ibrahim's entire blood has been exchanged the procedure will be complete, and we will transfuse more platelets overnight. We are praying for a good bump in the platelet count by tomorrow morning. 

In the meantime, Hamza is keeping himself busy with Bob the Builder reruns and flirting with nurses. He's off to a great start. 

Update

Bone marrow biopsy done. Platelets 20,000.  Prelim on the bone marrow is normal with increased platelet production, no sign of malignancy. Unforunately the central line has come out so the exchange transfusion can't happen. We are trying to get someone to put in another line today, otherwise there's no IV access for blood draws or platelet transfusions. 

So this morning our platelet count is still only 11,000 despite another platelet transfusion last night. The attending on service has changed and so our new staff is Dr Joseph Mirro, apparently a prior director of St Jude. He graphed out Ibrahim's platelet count to emphasize his lack of response to all our current treatment. So the plan for today, after much deliberation, is to perform the bone marrow biopsy and aspirate and after that perform whole blood exchange (plasma exchange itself is not a possibility in such a small child). We will be transferred to the ICU again to perform this procedure and will be monitored there over night. Please pray that all of this occurs uneventfully.

the next step

This disease has been a challenge for us in so many ways. The diagnosis of NAIT is under question with all our prior unresolved questions and particularly the lack of response to maternal platelets. Our expert opinion by Dr Bussell from Cornell recommended that we explore other diagnoses since Ibrahim's disease has not been behaving like NAIT. So tomorrow morning baby Ibrahim will have a bone marrow biopsy performed (a needle is inserted into his hip bone to get a biopsy of his bone for review under a microscope to evaluate the possibility of any other disease processes). Please pray for him. The biopsy will be performed under general anesthesia, since he is so small and Inshallah will happen at 8am. He continues to be transfused with platelets tonite and will receive another transfusion before the biopsy. Hopefully, this will provide more insight as to his diagnosis. 

Update

Today's platelet count is 25, the highest it's ever been since Ibrahim was admitted to the hospital.  The plan for today is another transfusion in the evening and more steroids.  If his counts are still low in the morning, we might have Ibrahim transfered to the ICU for a whole blood exchange transfusion in an attempt to remove the anti-platelet antibodies.

St Jude has a visiting professor today, Dr James Bussel from Cornell University.  He is a world authority on pediatric thrombocytopenia and came to deliver a Grand Rounds seminar.  Dr. Bussel's talk was on experimental therapy of thrombocytopenia.  Our team of physicians will meet with him today to see what his experience is with this disease, and if he has any insight into our case. 

Specifically, if this is NAIT, then why is it not responding to Saira's platelets? If this antibody is transferred from the mother to the baby at birth, then how did Ibrahim have a normal delivery and circumcision without any bleeding?  And if there is an antibody in Ibrahim's blood, why has it not been neutralized yet by IVIG and steroids?  These are all questions that have yet to be answered.

In summary...

Baby Ibrahim became ill on Friday, January 2nd.  On that day, we noticed several bloody diapers and a peculiar rash on his feet, so we took him to the ER at the local childrens hospital. Thinking this might be related to an infection, the doctors drew several blood tests and sent cultures.  On his blood count, Ibrahim had only 2,000 platelets (normal 150,000-300,000) and we were admitted to the ICU for extensive testing and treatment.

In the ICU, Ibrahim received blood and platelet transusions to replenish the blood he was losing, but the platelets remained very low.  The next morning, Saira and I went to the regional blood bank and had our blood tested for antibodies.  The results indicated that Saira's blood contained antibodies against my platelets, and that those antibodies were probably also inside Ibrahim and responsible for attacking his platelets. The working diagnosis was NAIT.

At the blood bank, Saira's platelets were taken and transfused into Ibrahim.  Since Saira's (and Ibrahim's) antibodies were not attacking her platelets, they should be safe in Ibrahim's blood.  That theory did not hold true, and several transfusions of Saira's platelets failed to raise his counts at all.

The next step was to give Ibrahim an immunoglobulin (IVIG), which would bind the anti-platelet antibodies inside his blood and clear them from his circulation, so that platelets would not continue to be destroyed.  Ibrahim received three doses of IVIG over three days, but we did not see any response in his platelet count.  At this point, we requested to be transferred to St Jude Children's Research Hospital for more specialized care.

We arrived at St Jude on Monday, January 5.  After failing IVIG, Ibrahim was started on steroids to suppress his immunity and block the effects of the anti-platelet antibody.  Now, Ibrahim has had over three days of steroids, and continues to receive about 2-3 platelet transfusions a day. By the grace of God, there has not been any further bleeding or bruising that we have noticed, and Ibrahim's hemoglobin has been stable.  We are beginning to see a slight response to the transfusions now, and we are hopeful that the counts will continue to rise each day.

We want to thank everyone for your concern, calls and emails. We're very sorry we have not been able to address everyone individually. We'll keep updates posted on this blog as well as recent platelet counts. Please continue to pray for our baby.

About NAIT

NAIT, or Neonantal Alloimmune Thrombocytopenia, is a rare disease of fetuses and newborns affecting the platelets, which are the cells responsible for normal blood clotting. In NAIT, the mother develops antibodies against the baby's platelets because of genetic differences in the mother's and father's blood. These antibodies are transfered to the baby before birth and begin to destroy baby's platelets to varying severity. Without platelets, the baby is prone to episodes of bleeding. Treatment usually involves multiple platelet transfusions and immunoglobulin (IVIG) to remove the circulating antibody. Prognosis depends on the severity of platelet destruction and the degree of bleeding, if any.

Read more about NAIT at http://en.wikipedia.org/wiki/Neonatal_alloimmune_thrombocytopenia.