Name 姓名: Mason Lee Yung Er 李滽和 Age 年龄: 4 Condition 病情: Krabbe氏症(球細胞腦白質失養症) Links: – Article: Nanyang Siang Pau – Article: China Press – Article: NTV7 – Article: 8TV

Updates 31/1/2019

Update from Mason’s mum on 31/1/2019 (欲阅中文请滑吓) :-

Hello all… how has everyone been doing? Hope you are all doing great.

I have some updates on Mason. Mason has been discharged from the hospital! Yaaay!! He is now being monitored twice or 3 times weekly (or whenever Mason is not well) as an outpatient at the BMT clinics. During these clinic visits, Mason will have his blood works done and all infusions required. Out of the 2 central lines that Mason had, 1 had been removed. All medications and feeds given at ‘home’ now are through his feeding tube.

Mason has officially been ‘engrafted’. That would simply mean the donor’s cells are growing in his body. The latest Chimerism test showed that the donor’s cells were 65% while Mason’s own cells were 35%. This test will be repeated in about 2 weeks time from now, to evaluate the chimerism status. The higher the donor’s cell percentage, the better it is for Mason, as more GALC enzyme is likely to be produced.

Mason is still ‘on isolation’ now and other than the clinic visits, he is basically homebound. He is not eating much yet, but we are hopeful he will not be dependent on his feeding tube for nutritions. At the moment, Mason has his ‘meals’ through his feeding tube using a feeding pump; 2 bolus feeds during the day and a 10-hour continuous feed throughout the night. The cost for the feeding pump etc is USD22+ per day, which is crazy!

Apart from these, Mason is also recovering from a nasty cough and flu now. We have faith that all will be well very soon.

We are also happy that despite his fatigue, which I believe is mostly contributed by his daily meds, Mason is always up and about. He will never settle to naps. No matter how sleepy he may be, he will still exclaim “I am not tired! I don’t wanna sleep!”.

Our deepest gratitude to YNP and everyone who has helped us in many many ways to make this transplant a reality for Mason. Thank you for being there for us during this difficult time.

Speedy recovery, our dear Mason!

来自李滽和的妈妈的来信:

大家进来好吗？希望你们一切安好。

我想跟大家说说关于Mason的进展。Mason已经出院了！Yaaaaay！他现在作为BMT诊所的门诊病人，每周被监测两次或三次（或每当Mason感到不适时）。在这些就诊期间，Mason需要完成血液输液作业。在Mason拥有的2条中心导管中，有1条已被移除。现在他在’家‘是透过喂食管来吸收所有药物和食物。

Mason已经正式’‘engrafted‘。这意味着捐赠者的细胞在他体内生长。最新的嵌合体试验显示供体细胞为65％，而Mason自身细胞为35％。该测试将在约2周后重复，以评估嵌合状态。对Mason来说，供体细胞百分比越高越好，因为可能产生更多的GALC酶。

Mason现在仍处于“隔离”状态，除了门诊外，他基本上都是在家的。他没能吃多少，但我们希望他不会依赖他的喂食管来获取营养。目前，Mason通过喂食管的喂食泵进行“吃饭”;白天2次推注，以及一整夜的连续喂食10小时。喂食泵等的费用是每天22美元，这实在太狂了！

除此之外，Mason现在也正在从令人讨厌的咳嗽和流感中恢复过来。我们相信一切很快就会好起来。

尽管他已经疲惫不堪，我们也感到高兴，因为我相信这主要是日常药效的原因，Mason总是很开心。他永远不会满足于小睡。无论他多么困倦，他仍然会惊呼“我不累！我不想睡觉！”。

我们非常感激南洋报业基金以及和所有帮助我们的人们，因为大家各种方式的帮助，让Mason可以实现这个移植。感谢您们在我们最困难的时候给予的支持。

亲爱的Mason，加油哦！

Updates 9/1/2019

Thank you very much for the support from all donors. Without your compassionate support, Mason might not receive the medical treatment before it was too late! We received the following updates from Mason’s mother. We are pleased to share it with all of you here:-

“My apologies that I haven’t been updating on Mason. It is Day +17 today from 1st transplant (18 Dec 2018) and finally we are seeing some signs of engraftment!:

“It was quite ‘stressful’ for me to wake up every day to be told that “we just have to wait, this is very normal…”. After transplant/ transfusion, it is a waiting game, while taking all precautions to make sure there’s no infection at all. What we all want, and waited, is for the white blood cell (WBC) count and absolute neutrophil count (ANC) to go up. I was told yesterday by the head of the BMT team, Dr Paul Szabolcs, that once the WBC get to 0.3, that’s the beginning of engraftment. Both WBC and ANC co-relates. When Mason’s ANC goes up to 500+ within 3 days consecutively, then we can be sure that engraftment will continue to take place over the next couple of months. Mason may also be then ready to be discharged as inpatient. We will be advised again soon on this.”

“Meanwhile, Mason has been having a tough time dealing with stomach pain/cramps and diarrheas ever since the 1st transplant day. He started on Morphine on Day +12 (30 Dec 2018) as Tylenol doesn’t seem to give him any comfort anymore. Mason is almost bald now. He is also fully dependent on his central lines for nutritions and hydration. The doctors are not using his feeding tube due to his diarrheas. With the central lines, the feedings need not go through and irritate the stomach. At the moment, the feeding tube is for all Mason’s oral medications. I was told that his appetite could be the last thing that he will experience. With the engraftment, Mason is also having frequent high fevers which is being monitored very closely to ensure it’s not fevers caused by infections.”

“I am very grateful that Mason also has his physiotherapy and occupational therapy since his conditioning period (chemo time) until now. He even started his speech therapy yesterday. These therapies motivate him to keep working on maintaining if not strengthening his core muscles and cognitive abilities especially during recovery.”

“As for the cost of the 2nd transplant, the doctors have advised that it could still be included as a ‘package’ in the original contract, depending on Mason’s recovery speed and condition. Will update on this again.”

“We are very hopeful that things will be better for Mason in every aspect very soon. Thank you YNP for giving Mason an opportunity to live a better fulfilling life.”

非常感谢所有捐款者的支持。没有你们的热心支持，Mason可能就无法在为时已晚之前接受治疗！我们收到了Mason母亲的最新消息。他们仍在美国医院陪伴着Mason接受骨髓移植后的后续治疗和物理治疗。

“我很抱歉，没有及时跟大家更新Mason的进展。今天是第一次移植（2018年12月18日）后的第17天，而我们终于看到了一些植入的迹象！：

“我每天醒来被告知“我们只需等待，这是非常正常的……“ 让我感到无比压力。移植/输血后，就是一个等待游戏，同时采取一切预防感染的措施。我们想要的、以及等待的，是白细胞（WBC）计数和绝对中性粒细胞计数（ANC）上升。昨天BMT团队负责人Dr Paul Szabolcs告诉我，一旦WBC达到0.3，那就是植入的开始。WBC和ANC是息息相关的。当Mason的ANC连续3天内达到500+时，我们便可以确定植入将在接下来的几个月里持续发生，Mason也可能做好出院的准备。医生很快就会再对我们作出建议。“

“与此同时，自第一次移植以来，Mason就一直经历胃痛/痉挛和腹泻的困难时期。他在第12天（2018年12月30日）开始使用吗啡（Morphine），因为泰诺（Tylenol ）似乎不再起任何安抚作用。Mason现在几乎秃顶了。他也完全依赖于大导管的营养和水份。因为他腹泻，所以医生没有让他使用喂食管。以大导管来喂食的话不需要通过和刺激胃。目前，喂食管适用于所有Mason的口服药物。我被告知他的胃口也许是他将要经历的最后一件事。Mason随着植入而经常发高烧，医生正非常密切监测以确保不是因感染引起的发烧。“

“我非常感谢从Mason的化疗时期开始一直到现在，他都有进行物理治疗和康复治疗。他甚至在昨天开始了他的语言治疗。这些疗法激励他继续坚持下去，尤其在恢复的过程中，能够加强他的核心肌肉和认知能力。“

“至于第二次移植的费用，医生们有建议说仍然可以被包括在原始合同中的“配套”，这将取决于Mason的恢复速度和条件。我们将会再跟大家报告进展。”

“我们非常希望Mason在各个方面都能很快地取得好成果。感谢YNP让Mason有机会过上更充实的生活.”

Updates 15/11/2018

Dr Escolar from the US have sent the following email to Mason’s parents on 8th of Nov 2018:

“We have been waiting to hear back from you. Do you have the visa? Time is of the essence and if he does not receive a transplant it will be too late. In this circumstance, it will hurt him more than help him. It is therefore very important that you make a decision so that we can continue to support you with whatever you decide. Just let us know, we are all ready to help. All the best.” – Dr Escolar

Mason Lee Yung Er who is almost 4 years old was diagnosed with an extremely rare disease called Krabbe disease (also known as globoid cell leukodystrophy), and he urgently requires USD500,000 (Approx. RM 2.2 mil. ) to undergo a bone marrow transplant procedure in the USA. However, as of 15th Nov 2018, Yayasan Nanyang Press only collected approx. RM 1 mil.

Considering the urgency of sending Mason for the bone marrow transplant as explained by Dr Escolar above, the top management of Yayasan Nanyang Press was very kind to take up the risk and guarantee the balance of the fund needed so that Mason and his family can proceed to the USA for the necessary procedures.

We met Mason and his family last Sunday. When his twin brother Carson was asked, “Are you ready to go through the surgery and donate your bone marrow transplant for your brother? Without further hesitation, he replied, “Yes!” . Their parents smiled with tears in their eyes. After a while, their mother told us that Carson told her a few times that he in fact terrified to go for surgery. But he truly loves his brother Mason. It is indeed a tough decision for an adult. What’s more to say for a four year old boy! Proud of you Carson!

Their USA Visa was approved this afternoon. The family will be departing from KL on 16th Nov 2018 and landing USA on 17th Nov 2018. The family and Yayasan Nanyang Press had done our very best to support Mason but we need all of your support to create the full conditions required for miracle to be actualized! Please hold on together and support us with this fundraising and sharing of this post! Our collective love and compassion can be a powerful wish-fulfilling tool to save a life and give hope to many other patients with the same syndrome!

Updates 22/10/2018

As of today, we collected RM 700k. We are getting closer to the target (RM2.2mil.) everyday. Please help to share this post and encourage more donation so that we will be able to send Mason for boon marrow transplant soonest!

Updates 13/10/2018

Mason Lee Yung Er who is almost 4 years old was diagnosed with an extremely rare disease called Krabbe disease (also known as globoid cell leukodystrophy), and he urgently requires RM2.2 million to undergo a bone marrow transplant procedure in the USA.

When little Mason was born with his twin brother, Carson Lee Jun Yue, his growth seemed as normal as the other children, although he was a slower learner than his brother. After he turned 1, he started to show symptoms that alarmed his parents.

His mother, Joyce Moong Lee Ping told reporters, when they went travelling to Australia in 2016, Mason had a seizure and had to be sent to the hospital. He was suspected of having cerebral palsy.

When her family arrived back to Malaysia, Mason was sent to HKL for further blood tests and imaging, and he was referred to many different doctors in relevant specialties. They later determined that Mason did not have cerebral palsy or was suffering from meta chromatic leukodystrophy.

“On January 9th this year, HKL clinic for genetic disorders told us they sent a sample to Japan for genetic testing and found that Mason was tested positive for Krabbe disease. The hospital recommended that we sent a blood sample to Australia to undergo galactocerebroside (GALC) test to confirm.

According to Joyce, after 2 years of scrambling for answers, the laboratory in Australia showed that there is a defect in Mason’s galactocerebroside enzyme, proving conclusively that he has Krabbe disease.

She mentioned that because the hospitals in Malaysia and Singapore lacks the medical expertise dealing with treating this disease, so under the guidance of family members, they have managed to find a doctor in New York that specialises in this disease.

When the young boy arrived in the US in September, the doctor told them that the progression of the disease is affecting his vision and said that he required surgery immediately to stop it. The procedure is estimated to cost about 500,000 USD. Joyce has tried her best but was unable to acquire that much money.

Joyce added that because his twin brother – Carson has a compatible bone marrow, there is a much higher chance of survival for Mason.

She had fought with her husband multiple times over the decision to let their sons undergo this bone marrow transplant. Knowing that there is a chance to save Mason’s life, she comes forward with a plea to the public for financial assistance to save her son’s life.

Krabbe disease, brief explanation:

Is a type of disease that affects the nerves. Due to a deficiency in an enzyme called galactocerebroside (GALC), nerves are not able to properly coat itself in the process called myelination. Myelin are coats the nerves in order to help conduct signals quickly and also to protect the nerves from damage.

This type of disease caused by demyelination is classified as a type of leukodystrophy. It is because in Krabbe disease, abnormal globoid cells are observed, hence it is also known as Globoid Cell Leukodystrophy.

The patient appeared normal in the first few months after birth, but started showing symptoms even before his first birthday.

The earlier symptoms were characterised by restlessness, weakness, trouble feeding, unexplained fever, rigidly straight posture, slow heartbeat and etc. The deterioration of psychomotor abilities can be deduced from the increase of involuntary movements.

The patient may show early signs such as restlessness, muscle spasms and slow development. As the disease progresses, the child may begin to lose control and experience long periods of muscle weakness. This will later also affect the ability to move, chew, swallow and breathe. The patient may lose his sense of sight and may also experience seizures.

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